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It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients' families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors' own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD.

Background: Nijmegen breakage syndrome (NBS) is an autosomal recessive DNA repair disorder that manifests through increased genomic instability, malignancy, and cellular and humoral immunodeficiencies. The prognosis for NBS patients is poor due to their increased susceptibility to fatal infections and lymphoproliferative malignancies. Currently, there is no specific treatment for NBS, though allogeneic hematopoietic stem cell transplantation (HSCT) has been performed and documented as case series to demonstrate the utility of transplantation. Methods: A 14‐year‐old girl with NBS and haploidentical HSCT from her older brother due to recurrent lung infection was referred for liver transplantation (LT) due to liver cirrhosis, hepatopulmonary syndrome (HPS), and suspicion of liver malignancy. It was decided to perform LT using the living donor who had previously donated for HSCT. Results: Living donor left lobe LT was successfully performed from her brother. The patient experienced no complications in the early postoperative period and was discharged on the seventh postoperative day. Pathological examination of extracted liver has shown "intermediate cell carcinoma" in two foci. After 1 year LT, the patient has had an uneventful course in terms of LT complications and infection, with minimal immunosuppression. Conclusions: NBS patients have an increased prevalence of malignancies, including primary hepatic malignancy, but most are managed medically or with limited resections. Transplantation in these patients can be curative for hepatic malignancy with a favorable safety profile. [ABSTRACT FROM AUTHOR]

DOI: 10.26650/eor.2018.525Purpose To examine dental hard and soft tissue changes of coeliac children in order to increase the awareness of the pediatric dentists in prediagnosis of especially undiagnosed coeliac disease. Materials and methods Sixty children, 28 (46.7%) boys and 32 (53.3%) girls whose ages were between 6 to 16 years were included in the present study. Thirty children who had undergone endoscopy and diagnosed with the coeliac disease in the Şişli Hamidiye Etfal Hospital, İstanbul, Turkey, formed the study group. Also, thirty children clinically suspected of having the coeliac disease with the same gastrointestinal complaints had undergone endoscopy and proven not coeliac were chosen as the control group. Oral examination involved assessment of dentition and specific and unspecific dental enamel defects. Also, soft tissue lesions, clinical delay of the dental eruption, salivary flow rate, pH, and buffering capacity were examined. Results Twenty coeliac patients had enamel defects, however none in the control subjects. In the coeliac group, all enamel defects were diagnosed in permanent teeth and as specific in all children. Grade I dental enamel defects found mainly in the incisors. The clinical delayed eruption was observed in 10 (33.3%) of 30 coeliac children and none of the children in the control group. While the level of DMFT/S numbers and stimulated salivary flow rate were found significantly lower in the coeliac group, pH was found significantly higher. Conclusion Oral cavity may be involved in coeliac disease and pediatric dentists can play an important role in the early diagnosis of the coeliac disease.

Purpose To examine dental hard and soft tissue changes of coeliac children in order to increase the awareness of the pediatric dentists in prediagnosis of especially undiagnosed coeliac disease. Materials and methods Sixty children, 28 (46.7%) boys and 32 (53.3%) girls whose ages were between 6 to 16 years were included in the present study. Thirty children who had undergone endoscopy and diagnosed with the coeliac disease in the Şişli Hamidiye Etfal Hospital, İstanbul, Turkey, formed the study group. Also, thirty children clinically suspected of having the coeliac disease with the same gastrointestinal complaints had undergone endoscopy and proven not coeliac were chosen as the control group. Oral examination involved assessment of dentition and specific and unspecific dental enamel defects. Also, soft tissue lesions, clinical delay of the dental eruption, salivary flow rate, pH, and buffering capacity were examined. Results Twenty coeliac patients had enamel defects, however none in the control subjects. In the coeliac group, all enamel defects were diagnosed in permanent teeth and as specific in all children. Grade I dental enamel defects found mainly in the incisors. The clinical delayed eruption was observed in 10 (33.3%) of 30 coeliac children and none of the children in the control group. While the level of DMFT/S numbers and stimulated salivary flow rate were found significantly lower in the coeliac group, pH was found significantly higher. Conclusion Oral cavity may be involved in coeliac disease and pediatric dentists can play an important role in the early diagnosis of the coeliac disease.

It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients’ families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors’ own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD. [ABSTRACT FROM AUTHOR]

Purpose To examine dental hard and soft tissue changes of coeliac children in order to increase the awareness of the pediatric dentists in prediagnosis of especially undiagnosed coeliac disease. Materials and methods Sixty children, 28 (46.7%) boys and 32 (53.3%) girls whose ages were between 6 to 16 years were included in the present study. Thirty children who had undergone endoscopy and diagnosed with the coeliac disease in the Şişli Hamidiye Etfal Hospital, İstanbul, Turkey, formed the study group. Also, thirty children clinically suspected of having the coeliac disease with the same gastrointestinal complaints had undergone endoscopy and proven not coeliac were chosen as the control group. Oral examination involved assessment of dentition and specific and unspecific dental enamel defects. Also, soft tissue lesions, clinical delay of the dental eruption, salivary flow rate, pH, and buffering capacity were examined. Results Twenty coeliac patients had enamel defects, however none in the control subjects. In the coeliac group, all enamel defects were diagnosed in permanent teeth and as specific in all children. Grade I dental enamel defects found mainly in the incisors. The clinical delayed eruption was observed in 10 (33.3%) of 30 coeliac children and none of the children in the control group. While the level of DMFT/S numbers and stimulated salivary flow rate were found significantly lower in the coeliac group, pH was found significantly higher. Conclusion Oral cavity may be involved in coeliac disease and pediatric dentists can play an important role in the early diagnosis of the coeliac disease. [ABSTRACT FROM AUTHOR]

Background: Abdominal tuberculosis, the sixth commonest extrapulmanory tuberculosis, is easily misdiagnosed due to nonspecific gastrointestinal symptoms. The diagnosis is highly dependent on clinician’s suspicion of the disease; therefore to increase awareness we retrospectively reviewed clinical features of eight children in a four year-period. Methods: The medical records of children with abdominal TB were retrospectively reviewed. The diagnosis of abdominal TB was defined as Mycobacterium tuberculosis infection of gastrointestinal tract along with peritoneal or solid organ involvement. Results: There were six girls and two boys with a mean age of 13.6 ± 2.8 years (range, 7 - 16 years). Abdominal pain and weight loss were common complaints in all patients (100%) at presentation; fever was present in four (50%) patients and abdominal distension in two (25%) patients. Mean duration of symptoms before diagnosis was 2.5 ± 1 months. Laparotomy performed in six patients, and the peritoneum was the most common infection site (in 5 patients). Chest X-rays of 7 patients indicated lung involvement. The most common abdominal computed tomography and ultrasonography finding was ascites. Bowel wall thickening was recorded in 2 patients and ileal thickening in 1 patient. Inguinal lymphadenopathy and multiple mesenteric lymphadenitis were present as single cases. Conclusions: The abdominal tuberculosis should be suspected in children with ongoing abdominal pain, fever, and abdominal distension, laparoscopy or laparotomy could be useful in the differential diagnosis and utilizing imaging techniques, invasive methods with clinical suspicion may prevent delay of the diagnosis. [ABSTRACT FROM AUTHOR]

Background: Celiac disease is an autoimmune disorder characterized by damage to the small intestinal mucosa following the intake of gluten-containing foods in genetically predisposed individuals. Studies suggest that celiac disease is associated with asthma and allergic rhinitis. Objectives: The aim of this study was to examine the prevalence of asthma and allergic rhinitis in children with celiac disease compared to healthy controls. Methods: This study was conducted in 53 celiac children and 80 children with nonspecific abdominal pain as a control group; all participants were between 6 - 19 years. The children completed the ISAAC questionnaire, and spirometry was performed in each participant. Results: The prevalence rates of asthma symptoms and physician-diagnosed asthma were similar in both groups (30% and 19%, respectively, in the celiac group and 17.5% and 22.5%, respectively, in the control group) (P > 0.05). Six (11%) patients with celiac disease and seven (9%) children in the control group showed obstructive changes on pulmonary function tests. The prevalence rates of allergic rhinitis symptoms and physician-diagnosed allergic rhinitis were also similar in both groups (36% and 9%, respectively, in the celiac group, and 34% and 9%, respectively, in the control group) (P > 0.05). Conclusions: The prevalence rates of asthma and allergic rhinitis in patients with celiac disease were not significantly higher when compared to the healthy controls. [ABSTRACT FROM AUTHOR]

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/κ 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., Competing Interests: Conflict-of-interest statement: There are no potential conflicts of interest for any of the authors.