82 results on '"İnci Nur Saltik-Temizel"'
Search Results
2. Catheter-related bloodstream infections in children receiving parenteral nutrition: Experience of a single center with 178 patients
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Zeynelabidin Ozturk, Hulya Demir, Asuman Nur Karhan, Ali Bulent Cengiz, Hasan Ozen, Inci Nur Saltik Temizel, and Aysel Yuce
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Nutrition and Dietetics ,Endocrinology, Diabetes and Metabolism ,Internal Medicine - Published
- 2023
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3. Esophageal squamous cell papilloma in a child with cystic fibrosis: A rare incidental endoscopic finding
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Ersin GÜMÜŞ, Diclehan ORHAN, and İnci Nur SALTIK TEMİZEL
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General Earth and Planetary Sciences ,General Environmental Science - Abstract
Esophageal squamous cell papilloma is an uncommon epithelial lesion of the esophagus. Epidemiological data regarding esophageal squamous cell papilloma in children is scarce and consists of few case reports. Although the etiology of esophageal squamous cell papilloma remains unclear, chemical/mechanical irritation induced hyper-regenerative response of esophageal mucosa and human papillomavirus infection have been suggested as most probable causes. A case of a 14-year-old girl with cystic fibrosis and chronic dyspepsia had a 0.5 x 0.5 cm sessile, multilobulated, whitish and verrucous polypoid mass close to the lower esophageal sphincter removed. Histologically, a benign squamous papilloma was confirmed. Her dyspeptic symptoms suggestive for gastroesophageal reflux resolved after papilloma removal and anti-acid treatment. Esophageal squamous cell papilloma is an incidental finding at upper endoscopy. Gastroesophageal reflux disease may be responsible for distally localized papillomas. Due to its rarity in childhood, there are not any well-established management and surveillance guidelines. Esophageal squamous cell papilloma should be removed, when possible, because of the ambiguity about its malignant potential.
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- 2023
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4. Evaluation of non-infectious complications in children receiving parenteral nutrition
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Zeynelabidin Ozturk, Hulya Demir, Asuman Nur Karhan, Inci Nur Saltik Temizel, and Hasan Özen
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General Medicine - Published
- 2023
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5. Assessment of swallowing function in pediatric patients with Wilson’s disease: Results of a videofluoroscopic swallowing study
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Aysel Yüce, Asuman Nur Karhan, Yusuf Aydemir, Numan Demir, Ersin Gümüş, Aynur Ayşe Karaduman, Inci Nur Saltik-Temizel, Selen Serel Arslan, Hasan Özen, and Hülya Demir
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Pediatrics ,medicine.medical_specialty ,business.industry ,Copper metabolism ,Incidence (epidemiology) ,Swallowing Disorders ,Gastroenterology ,Disease ,medicine.disease ,Deglutition ,Wilson's disease ,Hepatolenticular Degeneration ,Swallowing ,medicine ,Humans ,In patient ,Child ,Deglutition Disorders ,business ,Copper ,Subclinical infection - Abstract
Background and study aims Wilson’s disease (WD) is a complex disorder related to copper metabolism and neurological involvement may lead to swallowing disorders . The purpose of this study was to evaluate swallowing function in pediatric patients with WD by using videofluoroscopic swallowing study (VFSS). Patients and methods A total of 21 patients were included in the study, prospectively. The VFSS was conducted to evaluate swallowing function of the patients. The penetration-aspiration scale (PAS) was used to assess penetration-aspiration severity. Results According to the VFSS, abnormal results were detected in nine patients (42.9%) with WD. Of these nine patients, oral phase dysfunction was present in one patient, laryngeal penetration was present in one patient and moreover, abnormal esophageal body function was detected in all nine patients. Of these nine patients, five had neurological presentation at the time of diagnosis, and remaining four patients had hepatic presentation. Mean PAS score of the patients was 1. Conclusion The current study results suggest that subclinical swallowing dysfunction may be observed in patients with either neurological or hepatic WD. Further studies are necessary to reveal the real incidence of esophageal phase problems of swallowing function in pediatric patients with WD.
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- 2021
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6. Nutritional status of children with primary immunodeficiency: A single center experience
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Deniz Cagdas, Saliha Esenboga, Asuman Nur Karhan, Inci Nur Saltik Temizel, Ersin Gümüş, Hasan Özen, Ilhan Tezcan, Betul Karaatmaca, Hülya Demir, and Aysel Yüce
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medicine.medical_specialty ,Anthropometry ,business.industry ,medicine.medical_treatment ,Malnutrition ,Nutritional Status ,Disease ,Hematopoietic stem cell transplantation ,medicine.disease ,Body Height ,Combined immunodeficiencies ,Cross-Sectional Studies ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Primary immunodeficiency ,Humans ,business ,Body mass index ,Immunodeficiency - Abstract
BACKGROUND Nutritional status in primary immunodeficiencies (PID) is a major factor influencing immune defense. We aimed to evaluate the nutritional status of patients with PID. METHODS Demographic findings and anthropometric measurements of 104 patients were recorded for this cross-sectional study. RESULTS Combined immunodeficiencies (n = 49), predominantly antibody deficiencies (n = 28) and phagocytic system disorders (n = 17), were the major disease groups. In total, 44 (42.3%) patients had at least one anthropometric measurement below -2 standard deviations. Chronic, acute, and mixed-type malnutrition were detected in 18.3%, 16.3%, and 7.7% of the patients, respectively. No significant difference was detected among groups regarding anthropometric measurements however higher malnutrition rates were observed in 'combined immune deficiency less profound than severe combined immuno deficiency' (52%), chronic granulomatous disease (66.6%), and X-linked agammaglobulinemia (50%) patients. Severe malnutrition was present in 22 (21.2%) of the patients, although it was not significant. It was more common in the phagocytic system disorder group. All patients in the severe combined immunodeficiency group had undergone hematopoietic stem cell transplantation and 50% of them had malnutrition. There was also no significant difference regarding age, sex, anthropometric indexes (Weight for age, lenght/height for age body mass index Z-scores), malnutrition types, and prevalence of malnutrition among three major disease groups. Only the hospitalization history inversely related to body mass index and weight for age Z-scores (P
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- 2021
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7. Cytokine profile in serum and gingival crevicular fluid of children with inflammatory bowel disease: A case-control study
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Ersin Gümüş, Deniz Cagdas, Cansu Özşin-Özler, Buket Acar, Ilhan Tezcan, Ismail Yaz, Begum Ozbek, Ezel Berker, Hasan Özen, Inci Nur Saltik-Temizel, Meryem Seda Boyraz, Selcen Özcan-Bulut, Hülya Demir, Erdem Karabulut, Cagman Tan, and Aysel Yüce
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medicine.medical_specialty ,Periodontal examination ,Cytokine profile ,medicine.medical_treatment ,Inflammatory bowel disease ,Gastroenterology ,Interleukin-23 ,Crevicular fluid ,Internal medicine ,Medicine ,Humans ,Periodontal Indices ,Child ,Inflammation ,business.industry ,Case-control study ,Interleukin ,Gingival Crevicular Fluid ,medicine.disease ,Inflammatory Bowel Diseases ,Gingivitis ,Cytokine ,Case-Control Studies ,Periodontics ,Cytokines ,Interleukin-4 ,business - Abstract
Background To evaluate the cytokine profile in gingival crevicular fluid (GCF) and serum of pediatric inflammatory bowel disease (IBD) patients and determine the cluster patterns of cytokines. Methods Fifty IBD patients and 21 systemically healthy children were enrolled in the study. The GCF samples were collected from the participants during periodontal examination and periodontal indices were recorded. Based on activity indexes and response to conventional treatment, patients with IBD were further categorized into subgroups as: remission, active disease and treatment-resistant. Serum samples were obtained from IBD patients to determine serum levels of cytokines. The levels of pro- (interleukin (IL)-1β, IL-12, IL-21, IL-22, IL-23, IL-17A, IL-17F) and anti-inflammatory (IL-4, IL-10) cytokines in serum and GCF were measured using Enzyme-linked Immunosorbent Assay (ELISA) kits. Results Among 50 IBD patients, 58% were in remission, 20% had active disease and 22% were defined as treatment-resistant. The severity of gingival inflammation measured by the criteria of Loe had increasing trends in IBD patients with active disease and treatment resistance. Gingival crevicular fluid IL-1β level was lower and GCF IL-4 and GCF IL-23 levels were higher in IBD patients compared to healthy controls. In the active disease group, more cytokine clusters occurred compared to the control group and other IBD subgroups, as explained by increased cytokine-cytokine interactions. Conclusions Considering the increased complexity of cytokine interactions and the increased severity of gingival inflammation in patients with active disease, it can be concluded that disease activity might have an impact on gingival inflammation in pediatric patients with IBD. This article is protected by copyright. All rights reserved.
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- 2021
8. Clinical-genetic characteristics and treatment outcomes of Turkish children with Gaucher disease type 1 and type 3: A sixteen year single-center experience
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Asuman Nur Karhan, Hayriye Hizarcioglu-Gulsen, Hülya Demir, Aysel Yüce, Ersin Gümüş, Inci Nur Saltik Temizel, Hasan Özen, and Serap Dökmeci
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Male ,congenital, hereditary, and neonatal diseases and abnormalities ,Pediatrics ,medicine.medical_specialty ,Adolescent ,Turkey ,Anemia ,Hepatosplenomegaly ,Single Center ,Compound heterozygosity ,Genotype ,Genetics ,medicine ,Lysosomal storage disease ,Humans ,Enzyme Replacement Therapy ,Child ,Genetics (clinical) ,Gaucher Disease ,business.industry ,Infant ,General Medicine ,Enzyme replacement therapy ,medicine.disease ,Phenotype ,Treatment Outcome ,Child, Preschool ,Cohort ,Glucosylceramidase ,Female ,medicine.symptom ,business - Abstract
Data from 38 children were retrospectively analyzed to determine the patient characteristics of Turkish children with Gaucher disease (GD) and evaluate the impact of enzyme replacement therapy (ERT) in a pediatric cohort consisting of two different sub-types of the disease, Gaucher disease type 1 (GD1) and type 3 (GD3). Both types were represented equally (GD1/GD3 = 20/18). L444P (35.5%) was the most common mutant allele while L444P/L444P (34.2%) was the most common genotype overall. Compound heterozygosity of N370S and L444P homozygosity were the dominant genotypes in Turkish children with GD1 and GD3, respectively. None of the patients had moderate to severe thrombocytopenia at last follow-up while the percent of patients with anemia decreased from 60% to 5.7% (p 0.001). Significant improvements in mean liver (from 2.2 to 1.6 MN, p 0.001) and spleen (from 15.5 to 7.6 MN, p 0.001) volumes were observed in the first year of ERT. Linear growth was ameliorated as shown by the decrease in the percent of patients having short stature from 34.3% to 13.3% (p 0.01) at year 5. Erlenmeyer flask deformity, osteopenia and scoliosis were common skeletal findings. Although none of the patients had lung disease at diagnosis, 20% developed radiological findings suggestive of pulmonary involvement. This single center experience is the first comprehensive study from Turkey not only reporting clinical and genetic characteristics of GD patients but also providing information on the outcomes of ERT in two different sub-types of GD. Genotypic background of Turkish children with GD is similar to western populations. Although visceral and hematological therapeutic goals are reached as early as one year of ERT in both sub-types, achieving normal growth takes several more years than suggested in significant number of children with GD.
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- 2021
9. Nutritional characteristics of patients with functional constipation aged 4 years and older
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Ersin Gümüş, Inci Nur Saltik-Temizel, Asuman Nur Karhan, Gulnar Agakisiyeva, Hülya Demir, Hayriye Hizarcioglu-Gulsen, Damla Yildirim, Erdem Karabulut, and Hasan Özen
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Dietary Fiber ,Male ,medicine.medical_specialty ,Adolescent ,Breastfeeding ,Nutritional Status ,Internal medicine ,medicine ,Humans ,Nutrition information ,Risk factor ,Child ,Socioeconomic status ,business.industry ,Feeding Behavior ,Anthropometry ,Micronutrient ,medicine.disease ,Cross-Sectional Studies ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Defecation ,Functional constipation ,Female ,business ,Constipation - Abstract
BACKGROUND Dietary modifications may have role in prevention and treatment of functional constipation. Macronutrient, extra fluid, and fibre intake have been evaluated and the results are conflicting. The aim of our study was to define the nutritional features associated with functional constipation aged 4 years and older. METHODS This is a cross-sectional descriptive study. Forty-one patients with functional constipation and 55 age-gender matched controls between 4-18 years old were enrolled. Demographic data, duration of breast-feeding, defecation pattern in the first year of life, physical activity, socioeconomic parameters, and anthropometric measurements were noted. Mean daily macronutrient and micronutrient consumption from the 5-day dietary records were calculated by Nutrition Information System - BEBIS 7.2 version. RESULTS There were no differences between two groups in energy, water, protein, and fibre consumption. However, in 4-7 years old constipated female and male group, the percentage of carbohydrate was higher (p=0.010, p=0.049, respectively) but fat was lower (p=0.011, p=0.032, respectively). All patients except 4-7 years old boys of both groups got less energy than the reference values. The mean daily protein intake was higher than required in the 4-7 years old constipated and control groups. There was no significant difference in fibre consumption between groups. Breastfeeding >18 months was more common in controls (p=0.039). The constipated group used the squatting toilet more frequently (p=0.002). Lower family income (p
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- 2020
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10. Long term follow-up of children with chronic hepatitis B: a single center experience
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Hasan Özen, Aysel Yüce, İnci Yaman Bajin, Hülya Demir, and Inci Nur Saltik-Temizel
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Male ,medicine.medical_specialty ,Cirrhosis ,Adolescent ,Single Center ,Antiviral Agents ,Hepatitis B, Chronic ,Chronic hepatitis ,Interferon ,Internal medicine ,medicine ,Humans ,Hepatitis B e Antigens ,Hepatitis B Antibodies ,Child ,Retrospective Studies ,biology ,business.industry ,Lamivudine ,Infant ,Alanine Transaminase ,medicine.disease ,HBeAg ,Seroconversion ,Hepatocellular carcinoma ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,biology.protein ,Drug Therapy, Combination ,Female ,Interferons ,Antibody ,business ,medicine.drug ,Follow-Up Studies - Abstract
Yaman Bajin I, Demir H, Saltik Temizel IN, Ozen H, Yuce A. Long term follow-up of children with chronic hepatitis B: a single center experience. Turk J Pediatr 2019; 61: 846-851. Chronic Hepatitis B infection is an important clinical issue because of the associated risk of developing cirrhosis and hepatocellular carcinoma. Especially in children, there is no consensus about the optimal treatment. Clinical features and long-term outcomes of 165 children diagnosed with chronic hepatitis B at our institution between January 1993 and June 2012 were analysed retrospectively. Patients were divided into four groups according to their treatment protocols. The first group received Interferon (IFN) only, the second group started lamivudine (LMV) first then IFN+LMV combined and then continued with LMV only, the third group started with IFN+LMV combined then continued with LMV only and the fourth group received LMV only. After a median follow-up period of 7 years (1-19 years) the highest e-seroconversion (the loss of HBeAg followed by gain of anti- HBe antibody) rate, biochemical and virological response was observed with combined (IFN+LMV) treatment regimens. Patients with higher ALT levels were better treatment responders (p: 0.003). Identification of the patients who need to be treated in order to determine the most effective therapy with optimal treatment duration is important to reduce the risk of developing future complications like cirrhosis and hepatocellular carcinoma.
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- 2020
11. Amoebic colitis presenting with hypo-albuminaemia in an eight-month-old breastfed girl
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Ersin Gümüş, Inci Nur Saltik Temizel, Aysel Yüce, Asuman Nur Karhan, Hülya Demir, and Hasan Özen
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medicine.medical_specialty ,Population ,Disease ,Gastroenterology ,Asymptomatic ,03 medical and health sciences ,Entamoeba histolytica ,0302 clinical medicine ,030225 pediatrics ,Internal medicine ,parasitic diseases ,Humans ,Medicine ,Amoebic dysentery ,030212 general & internal medicine ,Amoebiasis ,Colitis ,education ,education.field_of_study ,Entamoebiasis ,biology ,business.industry ,Infant ,Dysentery ,medicine.disease ,biology.organism_classification ,Breast Feeding ,Pediatrics, Perinatology and Child Health ,Dysentery, Amebic ,Female ,medicine.symptom ,business ,Hypoalbuminemia - Abstract
Entamoeba histolytica is a protozoan parasite that affects a large proportion of the world's population and causes amoebic dysentery and extra-intestinal disease. Many individuals remain asymptomatic during colonisation; in 10% of individuals, the parasite breaks through the mucosal barrier and leads to invasive disease. An eight-month-old girl who was evaluated for hypo-albuminaemia and was diagnosed with amoebic colitis is reported. To the best of our knowledge, this is the first report of hypo-albuminaemia owing to amoebic colitis in any age group.
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- 2018
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12. A Spectrum of Clinical Findings from ALPS to CVID: Several Novel LRBA Defects
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Saliha Esenboga, Sevil Oskay Halacli, Kaan Boztug, Elifcan Aladag, Fatma Gumruk, Tuba Arıkoğlu, Helen F. Matthews, Ozden Sanal, Michael J. Lenardo, Inci Nur Saltik-Temizel, Cagman Tan, Bernice Lo, Hülya Demir, Visal Okur, Burcu Balci-Hayta, Baris Kuskonmaz, Fatih Süheyl Ezgü, Duygu Uçkan Çetinkaya, Betul Karaatmaca, Ilhan Tezcan, Pınar Gur Cetinkaya, Deniz Cagdas, and Hakan Goker
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Adult ,Male ,Evans syndrome ,Adolescent ,Regulatory T cell ,T cell ,Immunology ,Population ,Communicable Diseases ,Immunophenotyping ,LRBA ,Hypogammaglobulinemia ,Young Adult ,T-Lymphocyte Subsets ,Exome Sequencing ,medicine ,Humans ,Immunology and Allergy ,Genetic Predisposition to Disease ,Child ,education ,Genetic Association Studies ,Adaptor Proteins, Signal Transducing ,education.field_of_study ,business.industry ,Common variable immunodeficiency ,Autoimmune Lymphoproliferative Syndrome ,Hematopoietic Stem Cell Transplantation ,medicine.disease ,Combined Modality Therapy ,Common Variable Immunodeficiency ,Treatment Outcome ,medicine.anatomical_structure ,Genetic Loci ,Child, Preschool ,Primary immunodeficiency ,Female ,business ,Biomarkers - Abstract
Introduction Autosomal recessively inherited lipopolysaccharide-responsive beige-like anchor (LRBA) protein deficiency was shown to be responsible for different types of inborn errors of immunity, such as common variable immunodeficiency (CVID) and autoimmune lymphoproliferative syndrome (ALPS). The aim of this study was to compare patients with LRBA-related ALPS and LRBA-related CVID, to describe their clinical and laboratory phenotypes, and to prepare an algorithm for their diagnosis and management. Methods Fifteen LRBA-deficient patients were identified among 31 CVID and 14 possible ALPS patients with Western blotting (WB), primary immunodeficiency disease (PIDD) gene, next-generation panel screening (NGS), and whole exome sequencing (WES). Results The median age on admission and age of diagnosis were 7 years (0.3-16.5) and 11 years (5-44), respectively. Splenomegaly was seen in 93.3% (14/15) of the patients on admission. Splenectomy was performed to 1/5. Recurrent upper respiratory tract infections (93.3% (14/15)), autoimmune cytopenia (80% (12/15)), chronic diarrhea (53.3% (8/15)), lower respiratory tract infections (53.3% (8/15)), lymphoma (26.6% (4/15)), Evans syndrome (26.6% (4/15)), and autoimmune thyroiditis (20% (3/15)) were common clinical findings and diseases. Lymphopenia (5/15), intermittant neutropenia (4/15), eosinophilia (4/15), and progressive hypogammaglobulinemia are recorded in given number of patients. Double negative T cells (TCR alpha beta(+)CD4(-)CD8(-)) were increased in 80% (8/10) of the patients. B cell percentage/numbers were low in 60% (9/15) of the patients on admission. Decreased switched memory B cells, decreased naive and recent thymic emigrant (RTE) Thelper (Th) cells, markedly increased effector memory/effector memory RA(+) (TEMRA) Th were documented. Large PD1(+) population, increased memory, and enlarged follicular helper T cell population in the CD4(+) T cell compartment was seen in one of the patients. Most of the deleterious missense mutations were located in the DUF1088 and BEACH domains. Interestingly, one of the two siblings with the same homozygous LRBA defect did not have any clinical symptom. Hematopoietic stem cell transplantation (HSCT) was performed to 7/15 (46.6%) of the patients. Transplanted patients are alive and well after a median of 2 years (1-3). In total, one patient died from sepsis during adulthood before HSCT. Conclusion Patients with LRBA deficiency may initially be diagnosed as CVID or ALPS in the clinical practice. Progressive decrease in B cells as well as IgG in ALPS-like patients and addition of IBD symptoms in the follow-up should raise the suspicion for LRBA deficiency. Decreased switched memory B cells, decreased naive and recent thymic emigrant (RTE) Th cells, and markedly increased effector memory/effector memory RA(+) Th cells (TEMRA Th) cells are important for the diagnosis of the patients in addition to clinical features. Analysis of protein by either WB or flow cytometry is required when the clinicians come across especially with missense LRBA variants of uncertain significance. High rate of malignancy shows the regulatory T cell's important role of immune surveillance. HSCT is curative and succesful in patients with HLA-matched family donor.
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- 2019
13. Assessment of cardiac function and electrocardiographic findings in patients with Wilson's disease
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Ersin Gümüş, Asuman Nur Karhan, Hülya Demir, Hayrettin Hakan Aykan, Yasemin Nuran Donmez, Hasan Özen, Süheyla Özkutlu, Inci Nur Saltik Temizel, Aysel Yüce, and Dursun Alehan
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Cardiac function curve ,Male ,medicine.medical_specialty ,Adolescent ,Systole ,Heart Ventricles ,Diastole ,030204 cardiovascular system & hematology ,QT interval ,Asymptomatic ,Ventricular Function, Left ,03 medical and health sciences ,Electrocardiography ,Young Adult ,0302 clinical medicine ,Tissue Doppler echocardiography ,Hepatolenticular Degeneration ,Internal medicine ,Medicine ,Humans ,cardiovascular diseases ,Prospective Studies ,Child ,Isovolumetric contraction ,Echocardiography, Doppler, Pulsed ,Ejection fraction ,business.industry ,Infant ,Stroke Volume ,General Medicine ,Myocardial Contraction ,medicine.anatomical_structure ,Ventricle ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Asymptomatic Diseases ,cardiovascular system ,Cardiology ,030211 gastroenterology & hepatology ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background:This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.Method:Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.Results:The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.Conclusion:Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.
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- 2019
14. Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center
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Damla Hanalioglu, Inci Nur Saltik-Temizel, İbrahim Karnak, Asuman Nur Karhan, Ersin Gümüş, Feridun Cahit Tanyel, Hülya Demir, Saniye Ekinci, Arbay O. Ciftci, Aysel Yüce, and Hasan Özen
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Male ,medicine.medical_specialty ,Cirrhosis ,medicine.medical_treatment ,Portoenterostomy, Hepatic ,Liver transplantation ,Single Center ,03 medical and health sciences ,0302 clinical medicine ,Biliary atresia ,Biliary Atresia ,030225 pediatrics ,Internal medicine ,medicine ,Humans ,Survival rate ,Retrospective Studies ,Prothrombin time ,medicine.diagnostic_test ,business.industry ,Gastroenterology ,Infant ,Retrospective cohort study ,medicine.disease ,Prognosis ,Optimal management ,Liver Transplantation ,Survival Rate ,Treatment Outcome ,030211 gastroenterology & hepatology ,Original Article ,Female ,business - Abstract
Background/aims The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. Materials and methods Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. Results Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. Conclusion A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.
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- 2019
15. Distinctive Features of Hepatic Steatosis in Children: Is It Primary or Secondary to Inborn Errors of Metabolism?
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Ersin Gümüş, Hayriye Hizarcioglu-Gulsen, Hülya Demir, Inci Nur Saltik-Temizel, Diclehan Orhan, Zuhal Akçören, Asuman Nur Karhan, and Hasan Özen
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Pediatric ,Hepatic steatosis ,medicine.medical_specialty ,Nonalcoholic hepatic steatosis ,Hepatology ,medicine.diagnostic_test ,business.industry ,Gastroenterology ,Odds ratio ,medicine.disease ,Metabolism, Inborn errors ,Cholestasis ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Nonalcoholic fatty liver disease ,medicine ,Etiology ,Original Article ,Steatosis ,Family history ,Steatohepatitis ,Liver function tests ,business - Abstract
Purpose The incidence of hepatic steatosis among children has been increasing; however, data distinguishing simple steatosis from a more complex disorder are lacking. Methods This study identified the etiologies resulting in hepatic steatosis through a retrospective review of pediatric liver biopsies performed in the last 10 years. A total of 158 patients with hepatic steatosis proven by histopathological evaluation were enrolled in the study, and baseline demographic features, anthropometric measurements, physical examination findings, laboratory data, ultrasonographic findings, and liver histopathologies were noted. Results The two most common diagnoses were inborn errors of metabolism (IEM) (52.5%) and nonalcoholic fatty liver disease/steatohepatitis (NAFLD/NASH) (29.7%). The three most common diseases in the IEM group were glycogen storage disorders, Wilson's disease, and mitochondrial disease. The rates of consanguineous marriage (75.6%; odds ratio [OR], 26.040) and positive family history (26.5%; OR, 8.115) were significantly higher (p=0.002, p
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- 2021
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16. The hla groups and their relationship with clinical features in turkish children and adolescents with celiac disease
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Hülya Demir, Inci Nur Saltik-Temizel, Hasan Özen, Figen Gürakan, Cagman Tan, Necati Balamtekin, Nuray Uslu Kızılkan, Aysel Yüce, Gökhan Baysoy, and Ilhan Tezcan
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Turkish population ,medicine.medical_specialty ,Adolescent ,Population ,Disease ,Human leukocyte antigen ,Gastroenterology ,Gene Frequency ,HLA Antigens ,Risk Factors ,Internal medicine ,medicine ,Humans ,Family history ,Child ,education ,Allele frequency ,Alleles ,education.field_of_study ,business.industry ,HLA-DQ2 ,Celiac Disease ,Pediatrics, Perinatology and Child Health ,Female ,Histopathology ,business - Abstract
BACKGROUND We aimed to investigate the relationship between human leukocyte antigens (HLA)-groups and clinical features, and degree of intestinal injury in children with celiac disease (CD). METHODS Study group included 73 (50 females, 68.5%) children with CD. Demographic and clinical features, accompanying autoimmune diseases, family history for CD and degree of damage in small intestinal mucosa (according to Marsh classification) at the time of diagnosis were determined. Twenty-two siblings of celiac patients without CD (15 females, 65.2%) consisted control group 1, and 66 (40 females, 60.6%) people from the normal population consisted control group 2. RESULTS The allele frequencies of HLA B8, B50, C6, C7, DR3, DR7, DQ2, and DR3 homozygosity were higher in the patient group. HLA DQ2 positivity was 89% in the patient group, 73.9 and 45.5% in control groups 1 and 2, respectively (p < 0.0001). HLA A30, C14, DR11, DQ3 frequency were lower in patients compared to both control groups. HLA-DR15 alleles in patient and control group 1 was significantly lower compared to the general population (p < 0.05). Thirty (41.1%) patients had typical, 43 (58.9%) patients had atypical presentation. Thirteen (17.8%) patients had other autoimmune diseases. There was no association between coexisting autoimmune diseases and the HLA antigens. Fifteen patients (20.5%) had a positive family history for CD; patients with HLA A69, B41 and C12 alleles had a higher positive family history (p < 0.05). Intestinal mucosal damage was as follows: 5 patients (6.8%) had Marsh 2, 25 (34.3%) Marsh 3a, 28 (38.4%) Marsh 3b, 15 (20.5%) Marsh 3c. Patients with HLA-DR15 alleles had more frequent Marsh 3a lesions (p < 0.05). CONCLUSIONS B8, B50, C6, C7, DR3, DR7, DR3/DR3, DQ2 alleles were risk factors for CD in the Turkish population. HLA C14, DR11, DR15, and DQ3 alleles were found to have a protective role in the same population.
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- 2021
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17. A very rare cause of protein losing enteropathy: gaucher disease
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Inci Nur Saltik-Temizel, Safak Gucer, Hayriye Hızarcıoğlu Gülşen, Ersin Gümüş, Hasan Özen, Hülya Demir, Mehmet Akif Göktaş, and Aysel Yüce
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medicine.medical_specialty ,business.industry ,Protein losing enteropathy ,Disease ,Enzyme replacement therapy ,Abdominal distension ,medicine.disease ,Gastroenterology ,Diarrhea ,Internal medicine ,Edema ,Pediatrics, Perinatology and Child Health ,Ascites ,medicine ,medicine.symptom ,business ,Complication - Abstract
BACKGROUND Mesenteric lymphadenopathy is a rare manifestation of Gaucher disease (GD) in children and can be accompanied by protein losing enteropathy (PLE). PLE is a difficult-to-treat complication of GD. To date, only a few pediatric GD cases with PLE and massive mesenteric lymphadenopathies have been reported. CASE Here, we report a girl with chronic neuronopathic GD, whose disease course was complicated by massive mesenteric lymphadenopathies with resultant protein losing enteropathy despite a regular and appropriate enzyme replacement therapy of 60 IU/kg/biweekly until the development of mesenteric lymphadenopathies and 120 IU/kg/biweekly thereafter. CONCLUSIONS PLE is a devastating and life threatening complication of GD developing despite long term use of high dose ERT. Clinicians should be alert for this complication particularly in GD patients presenting with progressive abdominal distension, edema, ascites and diarrhea or in patients who have already developed mesenteric lymphadenopathies. Timely diagnosis may allow early intervention with previously suggested surgical or medical treatment options. Although there is no specific and effective treatment, surgical and aggressive medical interventions in addition to ERT were reported to relieve diarrhea and halt progression of mesenteric lymphadenopathies.
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- 2021
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18. Rare cause of protein losing enteropathy: Gaucher disease type 3
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Hasan Özen, Ersin Gümüş, Hülya Demir, Hayriye Hızarcıoğlu Gülşen, Aysel Yüce, Inci Nur Saltik Temizel, and Mehmet Akif Göktaş
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Endocrinology ,business.industry ,Endocrinology, Diabetes and Metabolism ,Protein losing enteropathy ,Immunology ,Genetics ,medicine ,Disease ,medicine.disease ,business ,Molecular Biology ,Biochemistry - Published
- 2020
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19. Biliary atresia and Niemann-Pick disease type C: Coincidence or a mimic?
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Inci Nur Saltik-Temizel, Hülya Demir, Pinar Simsek-Onat, Meryem Seda Boyraz, Hasan Özen, Saniye Ekinci, Hayriye Hizarcioglu-Gulsen, Aysel Yüce, and Duygu Demirtas
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Pathology ,medicine.medical_specialty ,Endocrinology ,Niemann–Pick disease, type C ,business.industry ,Biliary atresia ,Endocrinology, Diabetes and Metabolism ,Genetics ,Medicine ,business ,medicine.disease ,Molecular Biology ,Biochemistry - Published
- 2020
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20. Clinical and histopathological features of immunoglobulin G4-associated autoimmune hepatitis in children
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Inci Nur Saltik Temizel, Zuhal Akçören, Aysel Yüce, Hasan Özen, Hülya Demir, and Yusuf Aydemir
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Male ,medicine.medical_specialty ,Time Factors ,Adolescent ,Inflammation ,Autoimmune hepatitis ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Liver Function Tests ,Fibrosis ,Internal medicine ,Immunoglobulin g4 ,Biopsy ,Medicine ,Humans ,Alanine aminotransferase ,Child ,Transaminases ,Retrospective Studies ,Hepatology ,biology ,medicine.diagnostic_test ,business.industry ,medicine.disease ,Hepatitis, Autoimmune ,Liver ,030220 oncology & carcinogenesis ,Child, Preschool ,Immunoglobulin G ,biology.protein ,030211 gastroenterology & hepatology ,Female ,Immunoglobulin G4-Related Disease ,medicine.symptom ,Antibody ,business ,Immunostaining ,Biomarkers - Abstract
Background and aim We aimed to examine the frequency and the characteristics of immunoglobulin G4 (IgG4)-associated autoimmune hepatitis among pediatric patients with autoimmune hepatitis. Methods Immunostaining for IgG and IgG4 was performed in liver biopsies of 40 pediatric patients with autoimmune hepatitis. The patients with more than 10 IgG4-positive plasma cells/high-power field were defined as IgG4-associated autoimmune hepatitis. Clinic, laboratory, and histopathological results were compared between groups. Results Among the 40 pediatric patients, 34 patients were type 1 and 6 patients were type 2 autoimmune hepatitis. Six patients (15%), four of the type 1 and two of the type 2 autoimmune hepatitis patients, were diagnosed with IgG4-associated autoimmune hepatitis. Clinical, laboratory, and histopathological data were initially similar in both forms. There was a positive correlation between IgG4-positive plasma cell count and degree of portal (r: 0.406, P: 0.009) and lobular inflammation (r: 0.37, P: 0.019), grade of interface hepatitis (r: 0.33, P: 0.03), and fibrosis (r: 0.318, P: 0.046). Time required for normalization of liver transaminases and serum IgG level was significantly shorter in IgG4-associated autoimmune hepatitis (3.3 ± 0.5 vs 6.6 ± 3.5 for alanine aminotransferase, 3.7 ± 0.8 vs 6.7 ± 1.2 for aspartate aminotransferase, 4.3 ± 1.2 vs 7.1 ± 2.7 for gamma-glutamyl transpeptidase, and 7.2 ± 3.1 vs 12.8 ± 4.5 for IgG). Conclusions Immunoglobulin G4-associated autoimmune hepatitis can be found in pediatric age group and also in type 2 autoimmune hepatitis patients. As steroid response may be better in IgG4-associated autoimmune hepatitis, biopsy specimens should be evaluated for this entity at diagnosis.
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- 2018
21. Type 3 Gaucher disease presented with cardiac manifestations
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Ersin Gümüş, Lale Olcay, Asuman Nur Karhan, Inci Nur Saltik Temizel, Hülya Demir, Hasan Özen, Kürşad Tokel, and Aysel Yüce
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Pathology ,medicine.medical_specialty ,Endocrinology ,business.industry ,Endocrinology, Diabetes and Metabolism ,Genetics ,Medicine ,Disease ,business ,Molecular Biology ,Biochemistry - Published
- 2019
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22. Detection of parasites in children with chronic diarrhea
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Inci Nur Saltik-Temizel, Hayriye Hizarcioglu-Gulsen, Salih Maçin, Ilhan Tezcan, Yakut Akyön, Sibel Ergüven, Hülya Demir, Deniz Cagdas, and Filiz Kaya
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medicine.medical_specialty ,030231 tropical medicine ,medicine.disease_cause ,Gastroenterology ,03 medical and health sciences ,fluids and secretions ,0302 clinical medicine ,Internal medicine ,parasitic diseases ,medicine ,Giardia lamblia ,Enterobius ,Dientamoeba fragilis ,Immunodeficiency ,Feces ,Blastocystis ,biology ,business.industry ,medicine.disease ,biology.organism_classification ,Diarrhea ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,Immunology ,medicine.symptom ,business ,Entamoeba hartmanni - Abstract
The aim of this study was to investigate the frequency of intestinal parasites in patients with chronic diarrhea and clarify the importance of these parasitic pathogens in such cases. A total of 60 pediatric patients with chronic diarrhea between June 2012 and October 2014 were enrolled in the study. Out of 60 stool samples, five were positive for Giardia lamblia, two, Dientamoeba fragilis, and one, Blastocystis hominis. One stool sample was positive for Entamoeba hartmanni and B. hominis, another one was positive for G. lamblia and B. hominis, another, G. lamblia and E. hartmanni and one sample was positive for Enterobius vermicularis, D. fragilis and B. hominis together. Parasitic infection, which decreases quality of life and increases susceptibility to other infections, should not be neglected, particularly in patients with chronic diarrhea. Accurate diagnosis decreases morbidity and mortality in patients with parasite infection.
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- 2016
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23. Risk factors associated with clinically significant gastrointestinal bleeding in pediatric ED
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Hülya Demir, Ozlem Teksam, Ayse Gultekingil, Inci Nur Saltik-Temizel, Burcu Berberoğlu Ates, and Hayriye Hızarcıoğlu Gülşen
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Male ,medicine.medical_specialty ,Gastrointestinal bleeding ,Lower gastrointestinal bleeding ,Adolescent ,Anemia ,Peptic ,Pallor ,03 medical and health sciences ,Hemoglobins ,0302 clinical medicine ,Melena ,Risk Factors ,030225 pediatrics ,Internal medicine ,Tachycardia ,medicine ,Humans ,Prospective Studies ,Child ,Intubation, Gastrointestinal ,business.industry ,Infant ,General Medicine ,medicine.disease ,Hematochezia ,Child, Preschool ,Emergency Medicine ,030211 gastroenterology & hepatology ,Female ,medicine.symptom ,Gastritis ,business ,Emergency Service, Hospital ,Erythrocyte Transfusion ,Gastrointestinal Hemorrhage - Abstract
Introduction Gastrointestinal bleeding is a common problem in pediatric emergency department (PED). Some of these patients can lose significant amount of blood which may lead to shock. The aim of this study is to determine the risk factors predicting clinically significant gastrointestinal (GIS) bleeding in patients presenting to PED. Methods This study was performed prospectively from January 1st 2013 to December 31th 2013 in patients with upper or lower GIS bleeding. Clinically significant GIS bleeding was defined as > 2 g/dL hemoglobin decrease at any time during observation in PED, need for erythrocyte transfusion or need for rapid endoscopic evaluation. Results 105 patients were enrolled, 81 of which were eligible for the study. Twenty two patients (26,8%) had clinically significant GIS bleeding. These patients have significantly more commonly have upper GI bleeding and symptoms of melena, pallor and tachycardia. Initial laboratory findings revealed lower hemoglobin, RBC and albumin levels with higher WBC and BUN levels. They need significantly more nasogastric tube placement and PPI and H2 blocker treatment. Final diagnosis included more gastritis and peptic ulcers. These patients have less hematochezia, less lower gastrointestinal bleeding and less commonly diagnosed as acute gastroenteritis or Mallory Weiss tear as a final diagnosis. Conclusions Pediatric emergency physicians should be aware of clinical and laboratory parameters of patients with clinically significant GIS bleeding to predict which patients are under risk of life threatening blood loss. Patients who have melena, pallor, tachycardia, anemia and uremia at presentation are more prone to have significant GIS bleeding.
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- 2018
24. Index of SuspicionCase 1: Malaise, Ataxic Gait, and Somnolence in a 2-Year-Old Boy With a History of Neonatal CholestasisCase 2: Failure to Thrive and Shaking Episodes in 1-Year-Old BoyCase 3: Jaundice in a 7-Year-Old Girl
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Nader Nakhleh, Burcu Berberoglu-Ates, Hayriye Hizarcioglu-Gulsen, Inci Nur Saltik-Temizel, Sudipta Misra, Richard Sultan, Harmanpreet S. Chawla, Cathleen Ballance, Fabian Ngido, Srividya Naganathan, Gulin Hizal, Paul Schwartzberg, Bora Peynircioglu, Yusuf Aydemir, Beth Loveridge-Lenza, and Safak Gucer
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Pediatrics ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Glasgow Coma Scale ,Physical examination ,Jaundice ,Malaise ,Pediatrics, Perinatology and Child Health ,Failure to thrive ,Medicine ,Medical history ,Neonatal cholestasis ,medicine.symptom ,Family history ,business - Abstract
We invite readers to contribute case presentations and discussions. Please use the Submit and Track My Manuscript link on the Pediatrics in Review homepage: http://pedsinreview.aappublications.org. A 2-year-old boy presents to the emergency department with a 24-hour history of progressive malaise, ataxic gait, and somnolence. He has had bilious vomiting 4 to 5 times a day unrelated to food consumption and constipation for nearly a week. His mother states that he has had no fever, convulsions, diarrhea, jaundice, or symptoms of viral upper respiratory tract infection. There is no history of trauma, exposure to toxic substances, or drug use. He was born from a consanguineous marriage. His medical history is significant for cholestasis that developed during his second month after birth. There is no family history of neonatal cholestasis. On physical examination, the boy has normal vital signs. He has incomprehensible speech, makes purposeless movement in reaction to stimuli, and has hyperactive deep tendon reflexes. His Glasgow Coma Scale score is 9. There are no signs of meningeal irritation and no focal neurologic deficits. The rest of his findings on physical examination are normal. Laboratory studies reveal a normal complete blood cell count, serum electrolyte levels, and blood gas test results. He has mildly elevated aminotransferase levels (alanine aminotransferase [ALT], 85 U/L; aspartate aminotransferase [AST], 131 U/L; and γ-glutamyl transferase, 8.6 U/L). The direct bilirubin level is 0.29 mg/dL (5.0 μmol/L), total bilirubin level is 1.05 mg/dL (18.0 μmol/L), serum glucose level is 101 mg/dL (5.6 mmol/L), albumin level is 3.5 g/dL (35 g/L), and international normalized ratio (INR) is 1.73 (reference range, 0.86–1.20). Urine toxicology screening results are negative. Cerebrospinal fluid analysis results are normal. However, his blood ammonia level is elevated at 398 μg/dL (reference range, 15–45 μg/dL). Additional medical history and imaging studies reveal the diagnosis. Review …
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- 2014
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25. Intractable Diarrhea of Infancy
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Hülya Demir, Aysel Yüce, Hayriye Hizarcioglu-Gulsen, Hasan Özen, Figen Gürakan, and Inci Nur Saltik-Temizel
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Diarrhea ,Male ,Short Bowel Syndrome ,medicine.medical_specialty ,Allergy ,Malabsorption ,Adolescent ,Cystic Fibrosis ,Cystic fibrosis ,Gastroenterology ,Congenital Disorders of Glycosylation ,Crohn Disease ,Malabsorption Syndromes ,Mucolipidoses ,Internal medicine ,Dietary Carbohydrates ,medicine ,Humans ,Child ,Retrospective Studies ,Inflammation ,Microvilli ,business.industry ,Intestinal Pseudo-Obstruction ,Infant, Newborn ,Infant ,Retrospective cohort study ,Length of Stay ,medicine.disease ,Short bowel syndrome ,Enterocytes ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Etiology ,Female ,Calprotectin ,medicine.symptom ,business ,Leukocyte L1 Antigen Complex ,Food Hypersensitivity - Abstract
Objectives Intractable diarrhea of infancy (IDI), a group of prolonged diarrheal disorders, is difficult to diagnose and manage. We documented general features of patients and the causes of IDI. Methods The present retrospective study included 60 hospitalized patients with IDI ages 0 to 24 months during January 2000 to December 2010. Detailed history, laboratory and endoscopic findings, diagnoses, and clinical courses were reviewed. Descriptive analyses were used for statistical evaluation. Results The male/female ratio was 1.4. The median age at onset of diarrhea was 12 days. A total of 70% and 11% of patients were severely and moderately malnourished, respectively. Carbohydrate malabsorption (CM) and food allergies (n = 11, 18% for both) were the most frequent causes. A total of 16 of the patients (27%) did not have a specific diagnosis. The other diagnoses were infections (n = 5), immune-mediated disorders (IMD) (n = 6), congenital enterocyte defects (CED) (n = 3, 5%), short bowel syndrome (n = 2), cystic fibrosis (n = 2), intestinal pseudoobstruction (n = 1), congenital disorder of glycosylation (n = 1), abetalipoproteinemia (n = 1), and proprotein convertase (PC) 1 deficiency (n = 1). Stool calprotectin level was high in 10 of 19 patients with Crohn disease (n = 3, mean 1116 ± 851 mg/L), food allergy (n = 4, mean 516 ± 288 mg/L), and undefined etiology (n = 3, mean 616 ± 780 mg/L). The mean duration of hospitalization was 76 days. Conclusions IDI is a heterogeneous group of diarrheal disorders. The most frequent causes were CM and food allergies in our study. Because high levels of calprotectin support inflammation, calprotectin levels may help to discriminate CED and inflammatory causes of IDI.
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- 2014
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26. Familial psychological factors are associated with encopresis
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Ayşe Zeki, Inci Nur Saltik Temizel, S. Ebru Çengel Kültür, Devrim Akdemir, and Gülser Şenses Dinç
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Encopresis ,business.industry ,Family functioning ,Psychological intervention ,medicine.disease_cause ,Treatment efficacy ,General family ,Pediatrics, Perinatology and Child Health ,Parenting styles ,Medicine ,Psychological stress ,medicine.symptom ,business ,Clinical psychology - Abstract
Background The aim of this study was to assess maternal psychiatric symptoms, family functioning and parenting styles in children with encopresis. Methods Forty-one children with encopresis were compared to 29 children without any psychiatric disorder. Results Higher maternal psychiatric symptoms were found in children with encopresis. The general family functioning and strictness/supervision in parenting were significant predictors of encopresis. Conclusions Family functioning may be screened in children with encopresis, especially when standard interventions have had limited success. Identification and treatment of familial factors may enhance the treatment efficacy in encopresis.
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- 2014
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27. Long-term visceral and hematologic outcomes of enzyme replacement therapy in a pediatric cohort of type 1 and type 3 Gaucher disease: A single center experience
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Asuman Nur Karhan, Serap Emre, Aysel Yüce, Ersin Gümüş, Inci Nur Saltik Temizel, Hasan Özen, and Hülya Demir
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Pediatrics ,medicine.medical_specialty ,business.industry ,Endocrinology, Diabetes and Metabolism ,Disease ,Enzyme replacement therapy ,Single Center ,Biochemistry ,Term (time) ,Endocrinology ,Cohort ,Genetics ,medicine ,business ,Molecular Biology - Published
- 2019
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28. Is Prolonged Copper Restriction Needed in Pediatric Wilson’s Disease?
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Hayriye Hizarcıoğlu-Gülşen, Pınar Şimşek Onat, Damla Yıldırım, Duygu Demirtaş, Meryem S. Boyraz, Mehmet A. Göktaş, Hülya Demir, Hasan Özen, and İnci Nur Saltık-Temizel
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Diseases of the digestive system. Gastroenterology ,RC799-869 - Published
- 2023
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29. Comparison of multichannel intraluminal impedance-pH monitoring and reflux scintigraphy in pediatric patients with suspected gastroesophageal reflux
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Biray Caner, Nuray Uslu Kızılkan, Murat Bozkurt, Hülya Demir, Aysel Yüce, Inci Nur Saltik Temizel, Hasan Özen, Kızılkan, Nuray Uslu, Bozkurt, Murat Fani, Temizel, İnci Nur Saltık, Demir, Hülya, Yüce, Aysel, Caner, Biray, Özen, Hasan, School of Medicine, and Department of Pediatric Gastroenterology
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Male ,Medicine ,Gastroenterology and hepatology ,Supine position ,Time Factors ,Clinical Trials Study ,Gastroesophageal reflux disease ,Children ,Multichannel intraluminal impedance ,Esophageal Ph monitoring ,Scintigraphy ,0302 clinical medicine ,Electric Impedance ,Supine Position ,Radionuclide imaging ,Child ,reproductive and urinary physiology ,medicine.diagnostic_test ,digestive, oral, and skin physiology ,Gastroenterology ,Age Factors ,General Medicine ,humanities ,3. Good health ,Predictive value of tests ,Child, Preschool ,Gastroesophageal Reflux ,030211 gastroenterology & hepatology ,Female ,Radiology ,Esophageal pH monitoring ,Impedance–pH monitoring ,medicine.medical_specialty ,Esophageal pH Monitoring ,Adolescent ,Patient positioning ,Patient Positioning ,03 medical and health sciences ,Predictive Value of Tests ,030225 pediatrics ,otorhinolaryngologic diseases ,Humans ,Radionuclide Imaging ,business.industry ,urogenital system ,Reflux ,equipment and supplies ,digestive system diseases ,Radiopharmaceuticals ,business - Abstract
AIM To evaluate the agreement of multichannel intraluminal impedance-pH monitoring (MII-pHM) and gastroesophageal reflux scintigraphy (GES) for the diagnosis of gastroesophageal reflux disease. METHODS Seventy-five consecutive patients with suspected gastroesophageal reflux disease (GERD) underwent 24-h combined MII-pHM recording and one hour radionuclide scintigraphy during the course of the MII-pHM study. Catheters with 6 impedance channels and 1 pH sensor were placed transnasally. Impedance and pH data analysis were performed automatically and manually. For impedance monitoring, reflux was defined as a retrograde 50% drop in impedance, starting distally and propagating retrogradely to at least the next two more proximal measuring channels. Reflux index (RI, percentage of the entire record that esophageal pH is < 4.0) greater than 4.2% for pHM and number of refluxes more than 50 for 24 h for MII were accepted as positive test results. At scintigraphy, 240 frames of 15 seconds duration were acquired in the supine position. Gastroesophageal reflux was defined as at least one reflux episode in the esophagus. After scintigraphic evaluation, impedance-pH recordings and scintigraphic images were evaluated together and agreement between tests were evaluated with Cohen's kappa. RESULTS Sufficient data was obtained from 60 (80%) patients (34 male, 56.7%) with a mean age of 8.7 +/- 3.7 years (range: 2.5-17.3 years; median: 8.5 years). Chronic cough, nausea, regurgitation and vomiting were the most frequent symptoms. The mean time for recording of MII-pHM was 22.8 +/- 2.4 h (range: 16-30 h; median: 22.7 h). At least one test was positive in 57 (95%) patients. According to diagnostic criteria, GERD was diagnosed in 34 (57.7%), 44 (73.3%), 47 (78.3%) and 51 (85%) patients by means of pHM, MII, GES and MII-pHM, respectively. The observed percentage agreements/kappa values for GES and pHM, GES and MII, GES and MII-pHM, and MII and pHM are 48.3%/-0.118; 61.7%/-0.042; 73.3%/0.116 and 60%/0.147, respectively. There was no or slight agreement between GES and pHM alone, MII alone or MII-pHM. pH monitoring alone missed 17 patients compared to combined MII-pHM. The addition of MII to pH monitoring increased the diagnosis rate by 50%. CONCLUSION No or slight agreement was found among pH monitoring, MII monitoring, MII-pH monitoring and GES for the diagnosis of gastroesophageal reflux disease., Scientific and Technological Research Council of Turkey (TÜBİTAK)
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- 2016
30. Acute liver failure in children: 20-year experience
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Figen Gürakan, Hasan Özen, Yusuf Usta, Aysel Yüce, Hülya Demir, Inci Nur Saltik Temizel, Nuray Uslu, and Zeren Baris
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medicine.medical_specialty ,Adolescent ,Turkey ,medicine.medical_treatment ,Encephalopathy ,Jaundice ,Liver transplantation ,Hepatolenticular Degeneration ,Risk Factors ,Internal medicine ,medicine ,Edema ,Humans ,International Normalized Ratio ,Child ,Retrospective Studies ,Prothrombin time ,medicine.diagnostic_test ,business.industry ,Mortality rate ,Infant, Newborn ,Gastroenterology ,Ascites ,Infant ,Hepatitis A ,Bilirubin ,Retrospective cohort study ,Liver Failure, Acute ,Hospitals, Pediatric ,medicine.disease ,Liver Transplantation ,Transplantation ,Child, Preschool ,Splenomegaly ,Prothrombin Time ,Etiology ,Gastrointestinal Hemorrhage ,business ,Hepatomegaly - Abstract
Background/aims We aimed to determine the causes, demographic findings, clinical status, outcomes, and prognostic risk factors of patients with acute liver failure admitted to Hacettepe University Children's Hospital between October 1987-October 2006. Methods This retrospective case study included 74 patients with acute liver failure according to the Pediatric Acute Liver Failure Study Group definition. Results The etiology of acute liver failure was metabolic in 26 (35.1%) and infectious in 21 (28.4%) patients. Sixteen (21.6%) patients had indeterminate causes. Wilson's disease (16/26 patients, 61.5%) was the most frequent metabolic disease, while hepatitis A (14/21 patients, 66.7%) was the most frequent infectious agent. Neurologic functions were normal in 21 (28.4%) patients. Forty-nine (66.2%) patients died and 24 (32.4%) recovered. Two patients underwent liver transplantation. The mortality rate was 82.9% for patients who were not transplanted but fulfilled King's College Hospital criteria and 45.4% for patients who were not suitable for transplantation. This difference was statistically significant (p=0.001). Total bilirubin >5.35 mg/dl, international normalized ratio (INR) >3.66 and prothrombin time >23.5 seconds were shown to be the risk factors to predict death. Conclusions Metabolic and infectious etiologies were responsible for most of the acute liver failure cases. Clinical encephalopathy may not be present in children.
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- 2012
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31. Responsiveness of children with celiac disease to different hepatitis B vaccination protocols
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Inci Nur Saltik-Temizel, Aysel Yüce, Necati Balamtekin, Hülya Demir, Nuray Uslu, and Gokhan Baysoy
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Male ,medicine.medical_specialty ,Hepatitis B vaccine ,First year of life ,Disease ,medicine.disease_cause ,Internal medicine ,medicine ,Humans ,Hepatitis B Vaccines ,Hepatitis B Antibodies ,Hepatitis B virus ,Hepatitis B Surface Antigens ,Immunization Programs ,business.industry ,Significant difference ,Gastroenterology ,Infant ,Hepatitis B ,Vaccination ,Celiac Disease ,Immunization ,Hepatitis b vaccination ,Immunology ,Female ,business - Abstract
BACKGROUND/AIMS We aimed to compare the response rates to hepatitis B virus vaccination in the first year of life, using two different immunization protocols, in children with celiac disease. METHODS Study Group 1 included patients with celiac disease who received 10 μg of hepatitis B vaccine intramuscularly at birth (0), 2 and 9-12 months of life. Group 2 included those who received hepatitis B vaccine at 0, 1 and 6 months of life. Healthy children were divided into two control groups according to the above schedules. RESULTS The total study group included 64 patients and 49 healthy controls. Celiac patients were found to have lower response rates with respect to controls (78.1% vs. 95.9%, respectively). The difference in response rates in the two patient groups was not statistically significant. CONCLUSIONS The response rates of celiac patients to the two different hepatitis B vaccination schedules showed no statistically significant difference.
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- 2011
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32. The Association of Inflammatory Bowel Disease and Mediterranean Fever Gene (MEFV) Mutations in Turkish Children
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Inci Nur Saltik-Temizel, Seza Ozen, Nesrin Besbas, Nuray Uslu, Hülya Demir, Yusuf Usta, Engin Yilmaz, Figen Gürakan, Aysel Yüce, and Hasan Özen
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Male ,medicine.medical_specialty ,Adolescent ,Turkey ,Physiology ,DNA Mutational Analysis ,Population ,Familial Mediterranean fever ,Disease ,Severity of Illness Index ,Inflammatory bowel disease ,Gastroenterology ,Consanguinity ,Crohn Disease ,Seroepidemiologic Studies ,Internal medicine ,Severity of illness ,Prevalence ,medicine ,Humans ,Genetic Predisposition to Disease ,Child ,education ,education.field_of_study ,business.industry ,Infant ,Pyrin ,Hepatology ,medicine.disease ,MEFV ,Ulcerative colitis ,Familial Mediterranean Fever ,Cytoskeletal Proteins ,Child, Preschool ,Mutation ,Colitis, Ulcerative ,Female ,business - Abstract
Familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD) concordance has been investigated in a few studies. We investigated MEFV mutations and prevalence of FMF disease in Turkish children with IBD and their relationship with the disease severity. Sixteen patients with ulcerative colitis (UC), 14 with Crohn’s disease (CD) and three with indeterminate colitis (IC) were enrolled in the study (median age 13 years, range 0.6–16 years, n = 19 boys). Demographic, clinical and laboratory characteristics of the patients were evaluated as well as the parameters of disease severity. All patients were screened for 12 common MEFV mutations. MEFV mutations were detected in 17 of 66 (25.7%) alleles. Seven patients (four patients with CD, two with IC, and one with UC) were also diagnosed as FMF. FMF disease was found in seven of all IBD patients (21.2%) and four of them had CD. M694V was the leading mutation, and as a disease-causing mutation, it was found to be significantly more frequent in CD patients than UC patients (Fisher’s exact test P = 0.03). Demographics, laboratory evaluations, growth parameters, extraintestinal manifestations, and treatment with immunosuppressive agents other than steroids were comparable between the patients with and without FMF in most aspects. Although this is a small cohort, disease-causing MEFV mutations and FMF disease rate were increased among our patients with IBD. The increase was prominent among CD patients, whereas in UC the rate was similar to the Turkish healthy control population.
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- 2010
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33. Evaluation Of Central Nervous System In Patients With Glycogen Storage Disease Type 1A
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Hasan Özen, Yusuf Aydemir, Dilek Yalnizoglu, Meral Topçu, Inci Nur Saltik Temizel, Hülya Demir, Aysel Yüce, Kader Karli Oguz, Figen Gürakan, and Çocuk Sağlığı ve Hastalıkları
- Subjects
Central Nervous System ,Male ,0301 basic medicine ,medicine.medical_specialty ,Adolescent ,Central nervous system ,Neurological examination ,Glycogen Storage Disease Type I ,Hypoglycemia ,Electroencephalography ,Pediatrics ,03 medical and health sciences ,Central Nervous System Diseases ,Internal medicine ,Evoked Potentials, Auditory, Brain Stem ,medicine ,Humans ,Glycogen storage disease ,Child ,medicine.diagnostic_test ,business.industry ,Infant ,Magnetic resonance imaging ,medicine.disease ,Magnetic Resonance Imaging ,030104 developmental biology ,medicine.anatomical_structure ,Child, Preschool ,Metabolic control analysis ,Pediatrics, Perinatology and Child Health ,Cardiology ,Evoked Potentials, Visual ,Female ,Brainstem ,business - Abstract
We aimed to evaluate structure and functions of central nervous system (CNS) in children with glycogen storage disease (GSD) type 1a. Neurological examination, psychometric tests, electroencephalography (EEG), magnetic resonance imaging (MRI), visual evoked potentials (VEP) and brainstem auditory evoked potentials (BAEP) were performed. The results were compared between patients with good and poor metabolic control and healthy children. Twenty-three patients with GSD type 1a were studied. Twelve patients were in poor metabolic control group and 11 patients in good metabolic control group. Five patients had intellectual disability, 10 had EEG abnormalities, seven had abnormal VEP and two had abnormal BAEP results. MRI was abnormal in five patients. There was significant correlation between the number of hypoglycemic attacks and MRI abnormalities. Central nervous system may be affected in GSD type 1a even in patients with normal neurologic examination. Accumulation of abnormal results in patients with poor metabolic control supports the importance of metabolic control in GSD type 1a.
- Published
- 2016
34. Withdrawn: Risk factors associated with clinically significant gastrointestinal bleeding in pediatric emergency department
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Hülya Demir, Inci Nur Saltik-Temizel, Ozlem Teksam, Hayriye Hızarcıoğlu Gülşen, Burcu Berberoğlu Ates, and Ayse Gultekingil Keser
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medicine.medical_specialty ,Gastrointestinal bleeding ,Lower gastrointestinal bleeding ,business.industry ,Anemia ,Peptic ,General Medicine ,medicine.disease ,Pallor ,Uremia ,Surgery ,Melena ,Internal medicine ,Emergency Medicine ,medicine ,Gastritis ,medicine.symptom ,business - Abstract
Introduction Gastrointestinal bleeding is a common problem in pediatric emergency department(PED). Some of these patients can lose significant amount of blood which may lead to shock. The aim of this study is to determine the risk factors predicting clinically significant gastrointestinal(GIS) bleeding in patients presenting to PED. Methods This study was performed prospectively from January 1st 2013 to December 31th 2013 in patients with upper or lower GIS bleeding. Clinically significant GIS bleeding was defined as more than 2 gr/dL hemoglobin decrease at any time during observation in PED, need for erythrocyte tranfusion or need for rapid endoscopic evaluation. Results 105 patients were enrolled, 81 of which were eligible for the study. Twenty two patients (26,8%) had clinically significant GIS bleeding. These patients have significantly more commonly have upper GI bleeding and symptoms of melena, pallor and tachycardia. Initial laboratory findings revealed lower hemoglobin, RBC and albumin levels with higher WBC and BUN levels. They need significantly more nasogastric tube placement and PPI and H2 blocker treatment. Final diagnosis included more gastritis and peptic ulcers. These patients have less hematochesia, less lower gastrointestinal bleeding and less commonly diagnosed as acute gastroenteritis or Mallory Weiss tear as a final diagnosis. Conclusions Pediatric emergency physicians should be aware of clinical and laboratory parameters of patients with clinically significant GIS bleeding to predict which patients are under risk of life threatening blood loss. Patients who have melena, pallor, tachycardia, anemia and uremia at presentation are more prone to have significant GIS bleeding.
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- 2015
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35. Detection of parasites in children with chronic diarrhea
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Salih, Maçin, Filiz, Kaya, Deniz, Çağdaş, Hayriye, Hizarcioglu-Gulsen, Inci Nur, Saltik-Temizel, İlhan, Tezcan, Hülya, Demir, Sibel, Ergüven, and Yakut, Akyön
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Diarrhea ,Male ,Turkey ,Incidence ,Feces ,Child, Preschool ,Chronic Disease ,Animals ,Humans ,Female ,Parasites ,Intestinal Diseases, Parasitic ,Child ,Retrospective Studies - Abstract
The aim of this study was to investigate the frequency of intestinal parasites in patients with chronic diarrhea and clarify the importance of these parasitic pathogens in such cases. A total of 60 pediatric patients with chronic diarrhea between June 2012 and October 2014 were enrolled in the study. Out of 60 stool samples, five were positive for Giardia lamblia, two, Dientamoeba fragilis, and one, Blastocystis hominis. One stool sample was positive for Entamoeba hartmanni and B. hominis, another one was positive for G. lamblia and B. hominis, another, G. lamblia and E. hartmanni and one sample was positive for Enterobius vermicularis, D. fragilis and B. hominis together. Parasitic infection, which decreases quality of life and increases susceptibility to other infections, should not be neglected, particularly in patients with chronic diarrhea. Accurate diagnosis decreases morbidity and mortality in patients with parasite infection.
- Published
- 2015
36. Bone mineral density in children with cirrhosis
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Nurten Koçak, Yusuf Usta, Inci Nur Saltik-Temizel, Hülya Demir, Nuray Uslu, Hasan Özen, Figen Gürakan, and Aysel Yüce
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Liver Cirrhosis ,Male ,musculoskeletal diseases ,medicine.medical_specialty ,Cirrhosis ,Adolescent ,Bone density ,Biopsy ,Osteoporosis ,Lumbar vertebrae ,Severity of Illness Index ,Gastroenterology ,Absorptiometry, Photon ,Bone Density ,Risk Factors ,Internal medicine ,Humans ,Medicine ,Child ,Retrospective Studies ,Bone mineral ,Lumbar Vertebrae ,business.industry ,musculoskeletal, neural, and ocular physiology ,Confounding ,Hepatology ,musculoskeletal system ,medicine.disease ,Osteopenia ,medicine.anatomical_structure ,Child, Preschool ,Female ,Radiology ,business ,Follow-Up Studies - Abstract
Despite the clinical importance of osteoporosis in individuals with cirrhosis, little is known about it, especially in children. We evaluated the bone mineral density (BMD) and bone mineral content (BMC) of children with cirrhosis.Forty children with cirrhosis (mean age, 10.4 +/- 3.9 years) were involved. BMD and BMC were measured by dual energy X-ray absorptiometry at lumbar vertebrae 1-4, and the results were compared with those of 62 healthy age- and sex-matched children.The mean lumbar spine BMD of patients with cirrhosis was 0.482 +/- 0.107 g/cm(2) and that of the controls was 0.687 +/- 0.172 g/cm(2) (P0.0001). The mean lumbar spine BMC of patients with cirrhosis was 20.008 +/- 8.409 g and that of controls was 32.859 +/- 14.665 g (P0.0001). After the confounding variables (weight, height, and pubertal stage) were controlled for, the difference in BMD and BMC values between patients with cirrhosis and healthy controls was significant (0.535 +/- 0.061 g/cm(2) vs 0.653 +/- 0.048 g/cm(2), and 24.515 +/- 5.052 g vs 29.952 +/- 3.971 g, respectively).Because of the significant difference in BMD and BMC values between our patients with cirrhosis and healthy controls, patients with cirrhosis should be evaluated for osteopenia.
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- 2006
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37. Cirrhosis in Children With Celiac Disease
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Hülya Demir, Nurten Koçak, Inci Nur Saltik-Temizel, Hasan Özen, Figen Gürakan, Gülsev Kale, Aysel Yüce, and Melda Çağlar
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Liver Cirrhosis ,Male ,medicine.medical_specialty ,Cirrhosis ,Adolescent ,Biopsy ,Disease ,Chronic liver disease ,Severity of Illness Index ,Gastroenterology ,Coeliac disease ,Liver disease ,Internal medicine ,Intestine, Small ,medicine ,Humans ,Aspartate Aminotransferases ,Child ,Ultrasonography ,Hepatitis ,business.industry ,Alanine Transaminase ,medicine.disease ,Antibodies, Anti-Idiotypic ,Immunoglobulin A ,Celiac Disease ,Liver ,Immunoglobulin G ,Disease Progression ,Etiology ,Female ,Differential diagnosis ,business ,Biomarkers ,Follow-Up Studies - Abstract
Background: Liver involvement represents an extra-intestinal feature of celiac disease (CD) and shows a clinical spectrum varying from nonspecific reactive hepatitis to cirrhosis. Here we report the association of cirrhosis with CD in 5 children. Patients and Methods: The mean age of the patients was 9.4 ± 2.8 years. Viral, metabolic, and autoimmune etiology of liver disease was ruled out. Intestinal and liver biopsies were performed to confirm the histologic diagnosis in all subjects. Results: Three of the patients had chronic diarrhea and hepato-splenomegaly in whom diagnoses of CD and cirrhosis were established at presentation simultaneously. In the other 2 patients, CD was diagnosed following an initial diagnosis of cirrhosis. At diagnosis, alanine aminotransferase (range, 64-271 IU/L) and aspartate aminotransferase (range, 90-225 IU/L) values were elevated. After 1 to 5 years of a gluten-free diet (GFD), normalization of serum aminotransferase levels and clinical improvement were observed in 3 patients with strict GFD. The other 2 patients without improvement of the liver disease had poor dietary compliance. Conclusion: CD may be associated with severe hepatic damage in children and strict GFD may have beneficial effect on the course of liver disease. Serologic screening of CD should be included in differential diagnosis of chronic liver disease of unknown origin.
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- 2005
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38. Index of suspicion
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Hayriye, Hızarcioglu-Gulsen, Yusuf, Aydemir, Safak, Gucer, Burcu, Berberoglu-Ates, Gulin, Hizal, Bora, Peynircioglu, Inci Nur, Saltik-Temizel, Harmanpreet, Chawla, Paul, Schwartzberg, Nader, Nakhleh, Beth, Loveridge-Lenza, Cathleen, Ballance, Richard, Sultan, Srividya, Naganathan, Fabian, Ngido, and Sudipta, Misra
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Male ,Cholestasis ,Cystic Fibrosis ,Portal Vein ,Vascular Malformations ,Infant ,Jaundice ,Disorders of Excessive Somnolence ,Failure to Thrive ,Diagnosis, Differential ,Hepatitis, Autoimmune ,Child, Preschool ,Humans ,Ataxia ,Female ,Child ,Fatigue - Published
- 2014
39. Serum selenium concentrations in cirrhotic children
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Figen Gürakan, Inci Nur Saltik-Temizel, Nuray Uslu, Hasan Özen, Hülya Demir, and Aysel Yüce
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Liver Cirrhosis ,Male ,inorganic chemicals ,medicine.medical_specialty ,Cirrhosis ,Adolescent ,Aspartate aminotransferase level ,chemistry.chemical_element ,Serum selenium ,Gastroenterology ,Selenium ,Plasma selenium ,Internal medicine ,Humans ,Medicine ,Child ,medicine.diagnostic_test ,business.industry ,food and beverages ,medicine.disease ,chemistry ,Child, Preschool ,Female ,business ,Liver function tests - Abstract
Selenium is an essential trace element for humans. Plasma selenium concentration is decreased in adults with cirrhosis. We aimed to investigate the serum selenium concentration in cirrhotic children.The serum selenium concentration of 38 patients was determined by spectrofluorometric method. The results of the patients were compared with those of 41 age- and gender-matched healthy children. Correlations between the liver function tests, Child classes and serum selenium concentrations in cirrhotic children were also investigated.The mean serum selenium concentration in cirrhotic children was significantly lower than that of controls (42.4 ± 8.2 μg/L vs 64.4 ± 16.9 μg/L, p0.05). There was no significant difference between the serum selenium concentrations of cirrhotic children who were in Child-Pugh class A versus B+C (p0.05). Except for serum aspartate aminotransferase level (Pearson coefficient = -0.34), there was no correlation between serum selenium concentration and liver function tests in cirrhotic children.Serum selenium concentration in cirrhotic children was found to be low; supportive selenium administration may be beneficial in cirrhotic children in appropriate cases.
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- 2010
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40. Safety of ribavirin in adolescent thalassemic patients with chronic hepatitis C virus infection
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Hülya Demir, Gökhan Baysoy, Aysel Yüce, Nuray Uslu, and Inci Nur Saltik Temizel
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Male ,medicine.medical_specialty ,Adolescent ,Thalassemia ,Hepacivirus ,Hepatitis C virus ,medicine.disease_cause ,Antiviral Agents ,chemistry.chemical_compound ,Flaviviridae ,Pegylated interferon ,Virology ,Internal medicine ,Ribavirin ,Humans ,Medicine ,biology ,business.industry ,virus diseases ,Hepatitis C ,Hepatitis C, Chronic ,medicine.disease ,biology.organism_classification ,digestive system diseases ,Infectious Diseases ,chemistry ,Ferritins ,Immunology ,RNA, Viral ,Female ,business ,Viral load ,medicine.drug - Abstract
Hepatitis C virus (HCV) infection is still a common and imporant problem in thalassemic patients.1 There have been important mprovements in the treatment of chronic HCV infection especially n the last decade. Combination of pegylated interferon (peg-IFN) lfa-2 and ribavirin seems to improve the success of treatment esponse and currently is the recommended therapy for adults.2,3 owever, studies about the treatment of adolescents with thaassemia and HCV infection are limited and are concerned with the dverse effects of the treatment.1,4 We herein present two adoescents with -thalassemia major and chronic HCV infection who ere treated with peg-IFN and ribavirin and discuss the safety of he treatment.
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- 2010
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41. Pityriasis Lichenoides Chronica: An Association with Autoimmune Hepatitis?
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A. Yüce, Sibel Ersoy-Evans, Inci Nur Saltik-Temizel, and H. Demir
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Autoimmune disease ,Hepatitis ,business.industry ,Pityriasis lichenoides ,Dermatology ,Autoimmune hepatitis ,medicine.disease ,Chronic hepatitis ,Parapsoriasis guttata ,Pityriasis lichenoides chronica ,Immunopathology ,Pediatrics, Perinatology and Child Health ,Immunology ,medicine ,business - Abstract
Pityriasis lichenoides is an uncommon, benign skin disorder with two major variants: acute and chronic. Autoimmune hepatitis comprises heterogeneous forms of chronic hepatitis that are generally progressive and often fluctuating. Two major forms of this entity are recognized. Herein, we report the concurrence of pityriasis lichenoides chronica with type I autoimmune hepatitis in a child, which, to the best of our knowledge, has not been previously reported. Although it is hard to say whether an etiologic relationship or coincidental coexistence occurred between the two entities in our patient, some common mechanisms may be involved in both diseases.
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- 2007
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42. Bone mineralization in children with Wilson's disease
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Ahmet Çetinkaya, Inci Nur Saltik-Temizel, Hülya Demir, Hasan Özen, Figen Gürakan, and Aysel Yüce
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musculoskeletal diseases ,Liver Cirrhosis ,Male ,medicine.medical_specialty ,Cirrhosis ,Bone disease ,Bone density ,Adolescent ,Cross-sectional study ,Hypercalciuria ,Gastroenterology ,Pubertal stage ,Absorptiometry, Photon ,Calcification, Physiologic ,Hepatolenticular Degeneration ,Bone Density ,Internal medicine ,Medicine ,Humans ,Child ,Lumbar Vertebrae ,business.industry ,musculoskeletal, neural, and ocular physiology ,musculoskeletal system ,medicine.disease ,Urinary calcium ,Wilson's disease ,Zinc ,Cross-Sectional Studies ,Child, Preschool ,Calcium ,Female ,business ,Biomarkers - Abstract
The goal of this study was to determine bone mineralization in children with Wilson's disease (WD).Twenty-seven patients (16 males) and two age- and gender-matched healthy children for each patient were enrolled in the study. Bone mineral content (BMC, grams) and density (BMD, g/cm(2)) at lumbar 1-4 vertebrae were measured by dual-energy X-ray absorptiometry. Urinary calcium excretion was calculated in 19 patients. The effect of cirrhosis and hypercalciuria on BMC and BMD was also evaluated in WD patients.There was no statistically significant difference between patients and healthy controls regarding mean BMC (33.0 ± 13.9 vs. 35.8 ± 13.8 g) (p = 0.940) and mean BMD values (0.66 ± 0.16 vs. 0.71 ± 0.18 g/cm(2)) (p = 0.269), respectively. Nine (47.4 %) patients had hypercalciuria. Hypercalciuric patients had statistically significant lower BMC and BMD values than those without hypercalciuria. A significant difference continued to be present after age, weight, height, and pubertal stage adjustment was done, but disappeared after weight, height, follow up duration, and pubertal stage adjustment was done. The presence of cirrhosis did not affect BMC and BMD significantly in WD patients.BMC and BMD in children with WD were normal. The presence of hypercalciuria but not cirrhosis may affect BMC and BMD negatively in the patients.
- Published
- 2014
43. Does nissen fundoplication improve deglutition in children?
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Feridun Cahit Tanyel, Tutku Soyer, Inci Nur Saltik-Temizel, Sule Yalcin, Hülya Demir, Numan Demir, and Asuman Nur Karhan
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Male ,medicine.medical_specialty ,medicine.medical_treatment ,Fundoplication ,Nissen fundoplication ,Gastroenterology ,Postoperative Complications ,Swallowing ,Internal medicine ,medicine ,Postoperative infection ,Humans ,Postoperative Period ,Prospective Studies ,Child ,Prospective cohort study ,Medical treatment ,business.industry ,Reflux ,Infant ,Dysphagia ,Deglutition ,Treatment Outcome ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Gastroesophageal Reflux ,Vomiting ,Female ,Laparoscopy ,medicine.symptom ,Deglutition Disorders ,business - Abstract
Soyer T, Yalçın Ş, Demir N, Karhan AN, Saltık-Temizel İN, Demir H, Tanyel FC. Does Nissen fundoplication improve deglutition in children? Turk J Pediatr 2017; 59: 28-34. A prospective study was performed to evaluate the effect of Nissen fundoplication (NF) on deglutition in children. Children who underwent NF between 2011-2015 were evaluated for demographic features, clinical findings, diagnostic methods for gastroesophageal reflux (GER) and indications for NF. Penetration aspiration scale (PAS), functional oral intake scale (FOIS) and esophageal functions were evaluated by videoflouroscopy (VFS). Preoperative and postoperative VFS findings were compared to evaluate the effect of NF on clinical findings and deglutition. Twenty-three children with a mean age of 5.08 ± 3.7 years were included. Female to male ratio was 15:8. Recurrent respiratory infections (RTI) (n: 14, 60.8%), swallowing dysfunction (n:13, 56.5%) and vomiting (n:10, 43.4%) were the most common symptoms. Preoperatively GER was diagnosed with barium swallowing study (BSS) contrast graphs (n:20, 87%) and with 24-hour esophageal pH monitorization (n:8, 34.8%). In 39.1% of patients, medical treatment for GER was used with a mean duration of 8 ± 5.8 months. Indications for NF were swallowing dysfunction (n: 18, 78%), GER complications (n:6, 26%), associated anatomical problems (n:4, 17.3%) and unresponsiveness to medical treatment (n: 3, 13%). Postoperative barium swallowing study and 24-hour esophageal pH monitorization showed no GER after NF in 95% of patients. Number of RTI were significantly decreased after NF (preoperative vs postoperative infection rate 4.21 vs 1.6 respectively, p0.05). VFS findings showed that PAS was significantly decreased after NF during both liquid and semi-liquid swallowing (p0.05). After NF, upper esophageal opening (UEO) was decreased when compared to preoperative VFS findings (p0.05 Esophageal cleaning, esophageal motility, esophageal backflow and lower esophageal sphincter narrowing did not alter after NF (p0.05). FOIS were significantly improved after NF (p0.05). VFS findings showed that penetration and aspiration were significantly decreased after NF and children had less RTI. Although, esophageal motility evaluated by VFS did not changed after NF, functional oral intake significantly improved in children.
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- 2017
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44. Polyposis deserves a perfect physical examination for final diagnosis: bannayan-riley-ruvalcaba syndrome
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Inci Nur Saltik-Temizel, Esra Kılıç, Gülen Eda Utine, Elena Dominguez-Garrido, Hayriye Hizarcioglu-Gulsen, Yusuf Aydemir, and İç Hastalıkları
- Subjects
Male ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,Lipomatosis ,Physical examination ,030105 genetics & heredity ,Pediatrics ,03 medical and health sciences ,Polyps ,0302 clinical medicine ,Bannayan–Riley–Ruvalcaba syndrome ,medicine ,Humans ,PTEN ,Child ,Physical Examination ,Gastrointestinal Neoplasms ,medicine.diagnostic_test ,biology ,business.industry ,Juvenile Polyp ,PTEN Phosphohydrolase ,Macrocephaly ,medicine.disease ,Hematochezia ,medicine.anatomical_structure ,Mutation ,Pediatrics, Perinatology and Child Health ,biology.protein ,030211 gastroenterology & hepatology ,medicine.symptom ,Hamartoma Syndrome, Multiple ,business ,Penis - Abstract
Hızarcıoğlu-Gülşen H, Kılıç E, Dominguez-Garrido E, Aydemir Y, Utine GE, Saltık-Temizel İN. Polyposis deserves a perfect physical examination for final diagnosis: Bannayan-Riley-Ruvalcaba syndrome. Turk J Pediatr 2017; 59: 80-83. Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare autosomal dominant inherited polyposis syndrome characterized by macrocephaly, lipomatosis, hemangiomatosis, intestinal polyposis and pigmented macules on penis. The mutation of the PTEN gene that is responsible for controlling cellular proliferation, migration and apoptosis clarifies the reason of tissue overgrowth in BRRS. Gastrointestinal tract involvement is seen 35-45% of the patients. Histologic features of polyps in BRRS resemble juvenile polyps. In this report, we describe a boy presenting with hematochezia and aggressive polyposis and finally was diagnosed as BRRS due to extra intestinal findings.
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- 2017
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45. Fecal calprotectin concentration is increased in children with celiac disease: relation with histopathological findings
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Necati BALAMTEKIN, Hulya DEMIR, Gokhan BAYSOY, Nuray USLU, Diclehan ORHAN, Zuhal AKCOREN, Hasan OZEN, Figen GURAKAN, Inci Nur SALTIK TEMIZEL, and Aysel YUCE
- Subjects
Male ,medicine.medical_specialty ,Pathology ,Biopsy ,Diet adherence ,Endomysial antibody ,Newly diagnosed ,Gastroenterology ,Diagnosis, Differential ,Feces ,Internal medicine ,medicine ,Humans ,In patient ,Intestinal Mucosa ,Child ,Retrospective Studies ,business.industry ,Gastrointestinal inflammation ,Celiac Disease ,Female ,Neutrophilic infiltration ,Calprotectin ,business ,Leukocyte L1 Antigen Complex - Abstract
Amac: Bu cal›flman›n amac› yeni tan› ve diet alt›ndaki colyak hastalar› ile sal›kl› cocuklardaki fekal kalprotektin miktar›n› kar- fl›laflt›rmakt›r. Ayr›ca histopatolojik evre (Marsh evresi) ve notrofilik infiltrasyon derecesi ile de fekal kalprotektin oran› karfl›laflt›- r›ld›. Gerec ve Yontem: Cal›flma, yeni tan›, glutensiz diet alt›ndaki hastalar ve sal›kl› kontrol grubu olmak uzeri 3 grubu icer- mektedir. Ayr›ca cal›flma boyunca glutensiz diet ile serolojisi negatifleflen 9 hastada fekal kalprotektin miktar› tekrar cal›fl›ld›. Bulgular: Fekal kalprotektin oran› yeni tan› colyak hastalar›nda (n=31), diet alt›ndaki hastalara (n=33) ve sal›kl› kontrol gru- buna (n=34) gore yuksek saptand› (s›ras›yla 117,2 μg/g (3.2-306), 3.7 μg/g (0.5-58.2) ve 9.6 μg/g (1-70), p
- Published
- 2012
46. Outcome of enzyme replacement therapy in Turkish patients with Gaucher disease: does late intervention affect the response?
- Author
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Zeynep, Arikan-Ayyildiz, Aysel, Yüce, Serap, Emre, Gökhan, Baysoy, Inci Nur, Saltik-Temizel, and Figen, Gürakan
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Male ,Gaucher Disease ,Treatment Outcome ,Turkey ,Child, Preschool ,Glucosylceramidase ,Humans ,Infant ,Enzyme Replacement Therapy ,Female ,Child - Abstract
We aimed to evaluate the outcome of enzyme replacement therapy (ERT) in Turkish Gaucher patients since it first became available in our country. Eleven patients with type I and one patient with type III Gaucher disease (GD) received therapy as 30-60 U/kg and 120 U/kg every two weeks, respectively, for at least six months, starting a mean period of 4.2 years after the diagnosis. Assessment of response included serial measurements of hematological and biochemical parameters and liver and spleen volumes. Symptoms and signs of bone disease, growth and severity scores were also evaluated. ERT in Turkish patients led to marked improvement in hematological parameters and organomegaly in the majority of them. Patients with growth failure demonstrated catch-up growth. Progression of bone disease was not observed except in two patients who experienced a delay of 15 and 8.6 years, respectively, between the diagnosis and the start of ERT.
- Published
- 2012
47. The presentation of celiac disease in 220 Turkish children
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Necati, Balamtekin, Nuray, Uslu, Gökhan, Baysoy, Yusuf, Usta, Hülya, Demir, Inci Nur, Saltik-Temizel, Hasan, Ozen, Figen, Gürakan, and Aysel, Yüce
- Subjects
Male ,Celiac Disease ,Turkey ,Child, Preschool ,Humans ,Female ,Child - Abstract
The aim of this study was to investigate the presentation pattern of newly diagnosed celiac disease (CD) in Turkish children in the last eight years. Two hundred twenty patients with newly diagnosed CD were included. The medical records of all the patients between January 2000 and October 2008 were reviewed. The clinical spectrum was divided into three categories according to the main symptoms that led to the diagnosis: gastrointestinal presentation, non-gastrointestinal presentation, and silent cases. The mean age of the patients was 7.2 +/- 4.3 years at diagnosis. According to the presenting signs, the patients were defined as gastrointestinal presentation (129 patients, 58.6%), non-gastrointestinal presentation (76 patients, 34.6%) and silent cases (15 patients, 6.8%). This study showed that the number/percentage of CD cases who presented with non-gastrointestinal symptoms/conditions, so-called "non-gastrointestinal presentation", have been increasing in the last eight years.
- Published
- 2010
48. A Rare Cause Of Carpal Tunnel Syndrome In Childhood: Benign Recurrent Intrahepatic Cholestasis
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Inci Nur Saltik Temizel, Nuray Uslu, Çağrı Mesut Temuçin, Necati Balamtekin, Hülya Demir, and Nöroloji
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child ,medicine.medical_specialty ,Cholestyramine ,benign recurrent intrahepatic cholestasis ,business.industry ,Benign Recurrent Intrahepatic Cholestasis ,carpal tunnel syndrome ,Muscle weakness ,General Medicine ,Jaundice ,medicine.disease ,Asymptomatic ,Ursodeoxycholic acid ,Surgery ,body regions ,Cholestasis ,General & Internal Medicine ,Medicine ,medicine.symptom ,business ,Carpal tunnel syndrome ,medicine.drug - Abstract
Carpal tunnel syndrome and benign recurrent intrahepatic cholestasis are rare conditions in childhood. Benign intrahepatic cholestasis is characterized by repeated self-limited attacks of cholestasis that can start at any age and last from weeks to months. The patients are asymptomatic between these attacks. We report a 16 year-old male patient with benign recurrent intrahepatic cholestasis who developed carpal tunnel syndrome during a cholestatic attack. He was admitted with complaints of jaundice, pruritus and pain, tingling and muscle weakness in both hands for 15 days. Nerve conduction studies revealed findings compatible with carpal tunnel syndrome. He was started on ursodeoxycholic acid, fat soluble vitamins and cholestyramine and cholestasis regressed after four weeks of therapy. With the improvement of cholestasis, the symptoms of carpal tunnel syndrome also disappeared. In conclusion, benign recurrent intrahepatic cholestasis can be a rare cause of carpal tunnel syndrome in childhood. We also advocate treating the underlying disease as an appropriate conservative treatment before surgery.
- Published
- 2010
49. Ineffectiveness Of Infliximab Therapy In Severe Infantile Crohn'S Disease
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Inci Nur Saltik-Temizel, Yusuf Usta, Aysel Yüce, Hülya Demir, Hasan Özen, Figen Gürakan, Nuray Uslu, and İç Hastalıkları
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Male ,medicine.medical_specialty ,Anti-Inflammatory Agents ,Disease ,Gastroenterology ,Inflammatory bowel disease ,Crohn Disease ,Internal medicine ,medicine ,Humans ,Treatment Failure ,Immunodeficiency ,Crohn's disease ,biology ,Gastroenterology & Hepatology ,business.industry ,Tumor Necrosis Factor-alpha ,Antibodies, Monoclonal ,Infant ,General Medicine ,medicine.disease ,Infliximab ,digestive system diseases ,Immunology ,Monoclonal ,biology.protein ,Tumor necrosis factor alpha ,Female ,Antibody ,business ,medicine.drug - Abstract
Crohn's disease is extremely rare in infancy and can be present in severe forms. Infants with Crohn's disease might require intensive immunosuppressive therapy. Infliximab is a chimeric mouse/human monoclonal IgG1 antibody against tumor necrosis factor-a, and completely neutralizes its biologic activity. Though widely used in the treatment of pediatric Crohn's disease, there are few data regarding its applicability in infancy. We therefore report herein our experiences with infliximab therapy in two infantile patients with Crohn's disease who were resistant to conventional therapies; one patient showed a partial response while there was no response in the second. We were unable to achieve satisfactory results from infliximab therapy. It remains to be determined whether inflammatory bowel disease starting in infancy represents a separate pathogenetic subgroup and whether the inflammatory bowel disease diagnosis should follow the exclusion of an immunodeficiency state. Studies in larger series are needed to further clarify the efficacy, safety and timing of infliximab therapy for infantile Crohn's disease patients. (C) 2009 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.
- Published
- 2010
50. Hemophagocytic Syndrome In A Child With Severe Crohn'S Disease And Familial Mediterranean Fever
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Hasan Özen, Figen Gürakan, Gunay Balta, Inci Nur Saltik-Temizel, Nuray Uslu, Aysel Yüce, and Hülya Demir
- Subjects
Male ,Familial Mediterranean fever ,Disease ,Inflammatory bowel disease ,Organomegaly ,Lymphohistiocytosis, Hemophagocytic ,Fatal Outcome ,Crohn Disease ,hemic and lymphatic diseases ,medicine ,Humans ,Child ,Histiocyte ,Immunosuppression Therapy ,Cytopenia ,Crohn's disease ,Hemophagocytic lymphohistiocytosis ,business.industry ,Gastroenterology ,General Medicine ,medicine.disease ,Familial Mediterranean Fever ,Immunology ,medicine.symptom ,business ,Immunosuppressive Agents - Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, severe condition of hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Here we report a fatal hemophagocytic syndrome in a 11-year-old boy with a diagnosis of both Crohn's disease receiving immunosuppressive therapy and familial Mediterrenean fever. It is important to evaluate the patients with inflammatory bowel disease receiving immunosuppressive therapy presenting with unexplained fever, cytopenia, progression of organomegaly and biochemical changes for the investigation of HLH for diagnosis and treatment. (C) 2009 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.
- Published
- 2010
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