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Juvenile dermatomyositis (JDM) is an inflammatory myopathy which causes severe morbidity and high mortality if untreated. In this study, we aimed to define the T-helper cell profile in the muscle biopsies of JDM patients. Muscle biopsies of twenty-six patients (50% female) were included in the study. Immunohistochemical expression of CD3, CD20, CD138, CD68, IL-17, Foxp3, IFN-ɣ, IFN-alpha and IL-4 was studied and muscle biopsies were scored using the JDM muscle biopsy scoring tool. Inflammatory cells were in small clusters in perimysium and perivascular area or scattered throughout the endomysium in most biopsies; however in 2 biopsies, lymphoid follicle-like big clusters were observed, and in one, there was a very dense and diffuse inflammatory infiltration nearly destroying all the muscle architecture. Seventy-three per cent of the biopsies had T cells, 88% had B cells, 57% had plasma cells, and all had macrophages. As for T-helper cell subtypes, 80% of the biopsies were Th1 positive, 92% Th17 positive and 30% Treg positive. No IL-4 positive inflammatory cell was detected, and only 2 biopsies showed IFN-alpha positivity. The mean JDM biopsy score was 17.6, meaning moderate to severe muscular involvement. Visual analogue score of the pathologist was strongly correlated with histopathological features. B cells, macrophages, plasma cells and T cells constitute the inflammatory milieu of the JDM muscle biopsies. As for T cells, JDM is a disease mainly related with Th1 and Th17 T-helper cell subtypes and to some extend Treg. Th2 cells are not involved in the pathogenesis.

Diffuse alveolar damage (DAD) is one of the pathological hallmarks of acute respiratory distress syndrome (ARDS). We aimed to compare pathological findings of DAD with clinical ARDS criteria. We re-evaluated 20 patients whose clinical autopsy revealed DAD. Total 11/20 patients with DAD (55%) met the 1994 American–European Consensus Conference and 7/17 (41%) met the 2012 Berlin clinical criteria. DAD showed only moderate correlation with current clinical ARDS definition. Oxygenation index (OI), seems to be the most valuable tool in predicting pulmonary damage severity, though OI is not listed in either of the previous definitions. We support the recommended use of OI by 2015 consensus conference.

Background Alpha-dystroglycan (αDG) is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin globular domains and certain arenaviruses. An important enzyme, known as Like-acetylglucosaminyltransferase (LARGE), has been shown to transfer repeating units of -glucuronic acid-β1,3-xylose-α1,3- (matriglycan) to αDG that is required for functional receptor as an extracellular matrix protein scaffold. The reduction in the amount of LARGE-dependent matriglycan result in heterogeneous forms of dystroglycanopathy that is associated with hypoglycosylation of αDG and a consequent lack of ligand-binding activity. Our aim was to investigate whether LARGE expression showed correlation with glycosylation of αDG and histopathological parameters in different types of muscular dystrophies, except for dystroglycanopathies. Methods The expression level of LARGE and glycosylation status of αDG were examined in skeletal muscle biopsies from 26 patients with various forms of muscular dystrophy [Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), sarcoglycanopathy, dysferlinopathy, calpainopathy, and merosin and collagen VI deficient congenital muscular dystrophies (CMDs)] and correlation of results with different histopathological features was investigated. Results Despite the fact that these diseases are not caused by defects of glycosyltransferases, decreased expression of LARGE was detected in many patient samples, partly correlating with the type of muscular dystrophy. Although immunolabelling of fully glycosylated αDG with VIA4–1 was reduced in dystrophinopathy patients, no significant relationship between reduction of LARGE expression and αDG hypoglycosylation was detected. Also, Merosin deficient CMD patients showed normal immunostaining with αDG despite severe reduction of LARGE expression. Conclusions Our data shows that it is not always possible to correlate LARGE expression and αDG glycosylation in different types of muscular dystrophies and suggests that there might be differences in αDG processing by LARGE which could be regulated under different pathological conditions.

Munchausen syndrome by proxy (MSBP) is a rare form of child abuse in which a caregiver deliberately exaggerates, fabricates, and/or induces health problems in those in their care. The condition is extremely difficult to characterize and diagnose, as it manifests in many forms, often confusing the medical team. Insufficient knowledge regarding the syndrome and rare consideration of the differential diagnosis results in failure to recognize the problem. Literature suggests that an increase in physician awareness of the disorder can prevent or reduce its morbidity and mortality. For this reason, we believe that case series can help increase current knowledge on epidemiology, aetiology, diagnostic criteria, advised management of MSBP, and the psychological portrait of the perpetrator. Additionally, no data from Turkey has previously been presented on this issue. This study aimed to report characteristics of MSPB victims and alleged perpetrator, which were diagnosed and managed by a multidisciplinary team.

Purpose – The purpose of this paper is to demonstrate burnout syndrome among internal medicine and pediatrics residents in a country that does not have the working time directive (WTD) and also to determine the risk factors and consequent impact on efficient functioning in clinical areas. Design/methodology/approach – A 57-item questionnaire was given to internal medicine and pediatrics residents. Responses from 22 pediatrics and 33 internal medicine residents were evaluated. Findings – Demographic findings, burnout scores, having hobbies, social activities and reading books unrelated to medicine were similar between the two groups. Six pediatrics residents (27.3 per cent) and 11 (33.3 per cent) internal medicine residents met the criteria for clinically significant burnout. Personal accomplishment scores and reading books unrelated to medicine were found to be related to burnout. Originality/value – Burnout is a syndrome characterized by depersonalization, emotional exhaustion and a low sense of personal accomplishment. It is important to document burnout in countries where WTDs are not implemented. Further studies might demonstrate burnout's effect on patient safety, service quality and physician's performance.

Anaplastic large cell lymphoma (ALCL) tends to have frequent relapse and good response to salvage chemotherapy. The frequency of ALCL among 1486 Non-Hodgkin's lymphoma (NHL) cases followed-up since 1972 was 1.5%, however, the percentage was 9.3% in cases diagnosed after 2000. Event-free survival (EFS) and overall survival (OS) rates for 23 children were 32.2% and 72.8% at 3 years, respectively. Disseminated diseases, no response to first line treatment, anaplastic lymphoma kinase (ALK) negativity were found as significant predictors on survival of ALCL. The proper diagnosis and early referral is essential in these children for a better survival rate. The children with ALK negative status should be monitored carefully because of the poor prognostic factors, and treated differently. The survival rates in this study are need of further improvement since the survival rates with current protocols are achievable at a level more than 80%. This is mainly related with late referral of those children with advanced disease.

Amaç: İdrar yolu enfeksiyonu tedavisinde uygun antibiyotik kullanımı gelecekte oluşabilecek antibiyotik direncine ve böbrekte parankimal hasar reflü nefropatisi gibi komplikasyonlara karşı koruma sağlayacaktır Burada Konya bölgesinde toplumdan kazanılmış idrar yolu enfeksiyonu etkenlerini ve zaman içindeki antibiyotik direnci oranlarındaki değişimi göstermek amaçlanmıştır.Gereç ve Yöntem: Temmuz 2003 Ocak 2010 döneminde idrar kültüründe üreme belirlenen 0 17 yaş aralığındaki hastalar araştırmaya alındı Toplam 1742 hastadan 2544 kültür üremesi elde edildi.Bulgular: Üreme olan hastaların 57 6 rsquo;sını kızlar oluşturmaktaydı Kız ve erkek hastalarda enfeksiyon etkeni olarak sırasıyla E coli 76 1 41 9 Klebsiella spp 13 7 ndash;24 3 Proteus spp 6 9 28 4 ve diğerleri 3 4 5 2 oranında saptandı E coli Klebsiella ve Proteus türlerinin en duyarlı olduğu antibiyotikler karbapenemler aminoglikozitler kinolonlar ve üçüncü kuşak sefalosporinler olarak belirlendi E coli ve Proteus gruplarında yüksek trimetoprim sulfametoksazol TMP SM direnci 48 2 48 5 E coli ve Klebsiella gruplarında ise yüksek ampisilin direnci 68 9 88 2 dikkat çekiciydi Her üç mikroorganizma için 100 rsquo;e yakın amikasin duyarlılığı saptandı 2003 2006 ve 2006 2010 yılları arasında antibiyotik duyarlılıkları karşılaştırıldığında mikroorganizmaların birçok antibiyotiğe karşı direnç oranlarının anlamlı düzeyde arttığı görüldü. Çıkarımlar: İdrar yolu enfeksiyonuna neden olan mikroorganizmaların aminoglikozit ve üçüncü kuşak sefalosporinlere duyarlı olduğu görülmüştür İdrar yolu enfeksiyonu tedavisinde yüksek direnç gelişimi nedeniyle TMP SM ve ampisilin etkeni bilinmeden yapılan tedavide ilk seçenek olmamalıdır., Aim: Glazmann thrombasthenia is a rare autosomal recessive disease characterized by a defect in platelet aggregation Here we report the management of children with Glazmann thrombasthenia followed up at Cerrahpasa Medical Faculty Pediatric Hematology Department Material and Method: The files of nineteen patients 42 girls 58 boys; median age: 10 months were retrospectively reviewed Results: The median age of onset of bleeding symptoms was 9 months 2 weeks 24 months All patients presented with easy bruising and mucosal bleeding Fourteen patients rsquo; parents were consanguineous In 15 patients flow cytometry was performed According to this seven patients had type I six patients had type II and two patients had type III disease Nine patients were treated with thrombocyte transfusion tranexamic acid recombinant active factor VII and fibrin glue as a single or combined therapy; none of them had a major bleeding complication Conclusions: Bleeding control during invasive procedures may be challenging in children with Glazmann thrombasthenia; local treatments desmopressin steroid and antifibrinolytics may be used with success Turk Arch Ped 2012; 47: 104 6

Congenital muscular dystrophy is a heterogeneous group of inherited muscle diseases characterized clinically by muscle weakness and hypotonia in early infancy. A number of genes harboring causative mutations have been identified, but several cases of congenital muscular dystrophy remain molecularly unresolved. We examined 15 individuals with a congenital muscular dystrophy characterized by early-onset muscle wasting, mental retardation, and peculiar enlarged mitochondria that are prevalent toward the periphery of the fibers but are sparse in the center on muscle biopsy, and we have identified homozygous or compound heterozygous mutations in the gene encoding choline kinase beta (CHKB). This is the first enzymatic step in a biosynthetic pathway for phosphatidylcholine, the most abundant phospholipid in eukaryotes. In muscle of three affected individuals with nonsense mutations, choline kinase activities were undetectable, and phosphatidylcholine levels were decreased. We identified the human disease caused by disruption of a phospholipid de novo biosynthetic pathway, demonstrating the pivotal role of phosphatidylcholine in muscle and brain.

Giant cell hepatitis associated with direct Coombs' test-positive hemolytic anemia is a rare condition of childhood and the pathogenesis remains unclear. An autoimmune activation and loss of self-tolerance in these patients may be the underlying pathology related to the response of some of the patients to immunosuppressive treatment. Herein, we report the clinical presentation and course of three consecutive patients with this rare condition. We conclude that serum ferritin at diagnosis may be used for prediction of the outcome.Direk Coombs’ testi pozitif hemolitik anemi ve dev hücreli hepatit birlikteliği çocukluk çağında patogenezi tam olarak aydınlatılmamış nadir bir durumdur. Bu hastaların bir kısmının immünosupresif tedaviye cevap veriyor olması otoimmün aktivasyon ya da kendi antijenlerine karşı tolerans kaybının patogenezde sorumlu olabileceğini düşündürmektedir. Bu makalede bu nadir duruma sahip üç hastanın başvuru özellikleri ve takibi sunulmuştur. Başvuru anındaki serum ferritin değerinin hastanın prognozunu belirlemede kullanılabileceği önerilmiştir.

melikoglu, mustafa/0000-0001-9646-3787; Aydin, Burca/0000-0002-6013-6271 WOS: 000278808800001 PubMed: 20367260 Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Isiyel E, Teksam O, Foto-Ozdemir D, Ozmert E, Tumer AR, Kale G. Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department. Turk J Pediatr 2018; 60: 625-632. Most cases of physical abuse and neglect refer to pediatric emergency clinics, and these patients are diagnosed as only home accidents. Cases that cannot be diagnosed and managed correctly are again exposed to severe trauma and consequently, they may die. The aim of this study is to evaluate the physical abuse and neglect in children younger than three years of age, admitted to the pediatric emergency department with the complaints of trauma, burn, drug poisoning and/or caustic ingestion. This prospective study included 132 patients who were admitted to the pediatric emergency department. Children were classified into three groups as `no abuse`, `suspected abuse` and `abused` after being evaluated with a standard form. Additionally, suspected abuse and abused cases were evaluated once again by `The Team of Child Abuse and Neglect Evaluation, Research and Treatment` in Hacettepe University, Ihsan Dogramaci Children's Hospital. The frequency of the suspected abuse or abused cases in all burn, trauma and poisoning cases was found to be 7.5%. It was noticed that 65 of the patients (49.2%) were physically neglected considering the mechanism of occurrence of events. Unplanned pregnancy, absence of prenatal follow-up, high number of siblings, previous physical abuse in the family, absence of witness during the event, and hospitalization were statistically significant differences between no abuse and suspected abuse or abused cases (p < 0.05). Moreover, age group and income were significant risk factors in the logistic regression model for the patients who were thought to be suspected abuse/abused in univariate analyses (age group: Odds ratio (OR) 0.279, 95% confidence interval (CI) 0.085-0.723, p=0.0049; income: OR 2.323, 95% CI 1.052-6.198, p=0.0345). In conclusion, most cases of physical abuse and neglect are referred to the pediatric emergency clinics, and these patients are misdiagnosed as home accidents. The physicians working in the emergency department should be informed and trained in recognizing the cases of abuse and neglect, making the differential diagnosis, identifying the high-risk families and appropriate physical and psychological treatment for the abused and neglected children.

Aim Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT. Patients and Methods Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined. Results All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03–14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002). Conclusions Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done. Pediatr Blood Cancer 2009;54:885–889 © 2009 Wiley-Liss, Inc.

OBJECTIVE AND METHOD The survival of the patients with neuroblastoma has improved in last few decades. But still it depends on various clinical and biological factors. To assess the clinical features and trends in survival, the data for 500 newly diagnosed patients between January 1972 and December 2004 from a single center were retrospectively analyzed. RESULTS Histopathologic subtypes were neuroblastoma (NBL) in 462 patients (92.4%) and ganglioneuroblastoma in 38 patients (7.6%). The median age was 2.9 years and Male/Female ratio was 1.3/1. Primary tumor sites were abdomen, thorax, pelvis, neck, and others with the frequency of 72.2%, 14.9%, 3.8%, 3.2%, and 5.9%, respectively. There were 30, 49, 133, 257, 31 patients with stage 1, 2, 3, 4, 4S disease and their 10-year survival rates were 100%, 75.8%, 34.1%, 6.5%, and 59.4%, respectively. The outcome has significantly improved according to 10-year periods. The 5-year overall survival rates were 14%, 26.1%, 39.2%, and 52.4% for the years of 1970s, 1980s, 1990s, and after 2000. Surgical procedure involving total or near total tumor removal improved the survival (P=0.002). Both 5-year overall survival and event free survival rates were higher when partial resection was performed, especially in stage 3 disease (P=0.002 and P=0.02). In multivariate analysis, age above 18 months at diagnosis (P=0.01), stage 4 disease (P

The aim of this study was to investigate the effects of antenatal steroid treatment and/or postnatal surfactant replacement therapy on the incidence and extent of selected histopathological findings. Seventy complete autopsies were reviewed, and only lung tissues were examined and graded. Infants were divided into treatment and control groups as follows: group 1: surfactant-treated infants (n=15); group 2: infants whose mothers were given steroid treatment (n=16); group 3: surfactant-treated infants whose mothers were given steroid treatment (n=10). The control group included 29 patients not treated with surfactant and steroid. The overall incidence and severity of hyaline membrane and pulmonary hemorrhage were similar in each treatment group when compared to the control group. However, when the treatment groups were compared with each other, the incidence of severe hyaline membrane was more common in group 1 than in group 3. A significant reduction in severe hyaline membrane was associated with combined surfactant and antenatal steroid therapy. However, the cause for the similar incidence of selected histopathological findings in the treatment groups and the control group may be linked to oxygen toxicity due to insufficient antioxidant capacity in premature infants and barotrauma from mechanical ventilation.

Cross syndrome is a rare syndrome characterized by ocular and cutaneous hypopigmentation and neurological manifestations. A few reports have been published since 1967. In this report, we present a new case of Cross syndrome with additional findings such as urinary tract abnormality, bilateral inguinal hernia, focal interventricular septal hypertrophy of the heart and vacuolization of myeloid series cells and distinct ultrastructural features of the skin.

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing, periosteal new bone formation and polyarthritis. The pathogenesis of clubbing involves an increased expression of platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) from the digitally lodged platelet clumps, which bypass the pulmonary capillary network as a result of various systemic disorders. Intrathoracic neoplasms are rare causes of HOA in children. We report here a 14-year-old boy with digital clubbing, who eventually received the diagnosis of intrathoracic Hodgkin lymphoma (HL) and HOA. Eight cases previously reported with these two diagnoses are reviewed to emphasize the prognostic significance of HOA in childhood HL. Conclusion: Given the pathogenesis of clubbing and the prognostic significance of HOA, intrathoracic disease should be considered when HOA is detected in a child with a known or suspected malignant disease, and the occurrence of HOA during follow-up should alert the physicians for possible recurrence of the neoplastic disease or intrathoracic involvement.

This study aims to define the placental findings in intrauterine growth restriction (IUGR). The study group consisted of 40 neonates without IUGR and 24 neonates with IUGR, including their placentas. The cases were separated into three groups according to gestational age. Group A (n=21): 37 ≤ weeks, Group B (n=27): 32≤ -37 weeks, Group C (n=16):32 weeks. Each group had two subgroups, IUGR and non-IUGR. The placentas from the non-IUGR subgroup were heavier than the IUGR subgroup placentas in the term group (p0.05). Infarct (over 5%), increased syncytial knots and histiocytic intervillositis were more common in the IUGR cases (p0.05). This study shows that chronic patterns of injury are significantly increased in placentas from pregnancies complicated by IUGR. If the injury in the placenta is acute or mild, fetal adaptation can compensate and prevent fetal growth restriction.

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.

Acute proliferative glomerulonephritis is characterized by glomerular hypercellularity that can be caused by many different etiologies and pathogenetic mechanisms. A balance between cell birth by mitosis and cell death by apoptosis is crucial. In this study, apoptosis and the regenerative activity (Ki67/apoptosis index) were investigated in acute proliferative glomerulonephritis. Thirty-five children with biopsy-proven acute proliferative glomerulonephritis and five controls with MCD were studied retrospectively. According to the clinical outcome, patients were divided into 2 groups: group 1 (n =21) were patients with normal renal functions at follow-up; group 2 (n =8) were patients with end-stage renal failure or those who died. Immunohistochemical staining of proliferating cells (Ki67) was done. In situ end labeling of DNA was used to evaluate apoptosis. Glomerular cell apoptosis was 45% in the patients with acute proliferative glomerulonephritis and 3% in controls ( p0.001). Apoptotic cells were identified in the tubulointerstitial compartment with higher and heavier immunostaining in patients than controls (p =0.001). Tubular proliferative index (= tubular proliferation/tubular apoptosis ratio) was significantly higher in group 1 patients than in group 2 patients (2.03+/-2% versus 0.32+/-0.6%, p =0.002). Tubulointerstitial regenerative ratio (=tubular proliferation/interstitial proliferation ratio) was significantly higher in controls than in patients (3.4+/-1.9 versus 1.52+/-0.8, p =0.01). In addition, it was significantly increased in group 1 patients when compared with those in group 2 patients (1.89+/-0.8 versus 0.73+/-0.2, p =0.001). Since 17 patients presented with postinfectious proliferative glomerulonephritis, which is known to exhibit better course, we also evaluated those parameters in patients with postinfectious proliferative glomerulonephritis separately. We found statistically significant differences only in the tubulointerstitial regenerative ratio, which was higher in postinfectious cases when compared with those in other cases [1.60 interquartile range (IQR) 1.54 versus 1.22 IQR 1.26, respectively, p =0.003]. In conclusion, tubular proliferative index and tubulointerstitial regenerative ratio might be useful parameters for predicting final functional outcome in acute proliferative glomerulonephritis. Further studies, however, are still needed to clarify the importance of these histopathological parameters.

Objective: To evaluate renal involvement in childhood lymphoma and define its prognostic effects. Patients and Methods: One hundred and four patients with non-Hodgkin’s lymphoma and renal involvement on admission to a single center between 1972 and 2003 were evaluated retrospectively. Blood urea nitrogen, serum creatinine, uric acid, electrolytes, and lactate dehydrogenase levels, as well as urinalysis, were evaluated. One or more of the following imaging methods were performed: intravenous urogram, ultrasound, computed tomography, and magnetic resonance imaging. The χ2 test was used to compare the groups. The Kaplan-Meier survival method was used to calculate survival rates, and the log-rank test was used to compare groups with respect to survival. Survival rates were also compared in two different time periods (before 1991 and after 1991). Results: There were 76 boys and 28 girls with a median age of 6 (0.9–16) years. The renal infiltration pattern was nodular in 62 patients (59.6%) and diffuse in 40 patients (38.5%). Two patients had tumoral masses that originated from their kidneys (1.9%). Renal involvement was bilateral in 75 patients (72.1%); the remaining 29 patients had unilateral involvement. The overall survival rate was 42.5% with a median follow-up of 64 months. The factors that had a statistically significant impact on survival were high creatinine (p = 0.00001) and blood urea nitrogen levels (p = 0.0001), the onset of tumor lysis syndrome (p = 0.01), and the need for dialysis (p = 0.009). The survival rate was higher in the time period after 1991 (p = 0.01). Conclusion: Impaired renal function is a poor prognostic factor for non-Hodgkin’s lymphoma. Renal function should therefore be monitored closely. Renal dysfunction caused by direct tumoral involvement may complicate therapy and shorten survival.

Prenatal diagnosis of congenital atresia of the larynx is difficult but is possible by the findings of increased lung echogenicity and size coexisting with fetal ascites in ultrasonography. Sonographic findings may not always be typical. We report on a case of congenital laryngeal atresia diagnosed prenatally by the findings of fetal hydrops and hyperechogenic lungs. Our case presented with oligohydramnios. We also review syndromes that demonstrate laryngeal anomalies.

Cardiopathy is an expected finding in X-linked Duchenne and Becker muscular dystrophies. This holds true for some other forms such as autosomal recessive limb-girdle dystrophies. However, data on early-onset and usually severe congenital muscular dystrophies are limited. The purpose of this study was to investigate the presence of cardiac involvement in children with merosin-positive congenital muscular dystrophy. A total of 42 patients and 22 healthy subjects were evaluated by M-mode, 2D, and Doppler echocardiography. Cardiac anatomy, left ventricular dimensions, wall thickness and systolic and diastolic functions were investigated in patients and compared with those of healthy control subjects. Mean left ventricular ejection fraction and shortening fraction were significantly lower in the patient group (P

Introduction: The structure called the residual gubernaculum (G) has been evaluated to define its origin. Materials and Methods: Girls operated for inguinal hernia (n = 7) and boys operated for undescended testis (n = 12), inguinal hernia (n = 7), hydrocele of the cord (n = 1), and ectopic (n = 1) and entrapped (n = 1) testes were evaluated. Attachments of the structures coursing distally from the sacs were identified, and they were removed en block. Three samples, first from the distal part of the sac with the beginning of the fibrous structure, second from the midportion, and third from the most distal part, were prepared and stained with hematoxylin-eosin, trichrome and elastic van Gieson. Histologic structures within the samples were determined and compared with Mann-Whitney U test. Results and Conclusions: Structures called the residual G differed from the walls of accompanying sacs by only the absence of mesothelial lining. Therefore, the structure called the residual G in boys with an undescended testis and the round ligament in girls actually represent the obliterated processus vaginalis.

Immunoglobulin A vasculitis (IgA-V), formerly known as Henoch-Schönlein purpura (HSP), is the most common small vessel vasculitis in children. In recent years, the role of T cells in the pathogenesis of HSP/IgA-V has become a focus of research.Renal biopsy specimens from 22 pediatric patients diagnosed with Henoch-Schönlein nephritis (patient group) were compared to normal renal tissue in nephrectomy specimens from 20 pediatric patients diagnosed with Wilms tumor (control group). All renal specimens were scored according to International Study of Kidney Disease in Children (ISKDC) and Oxford classification. Immunohistochemical analyses of interferon-gamma (IFN-γ), interleukin (IL)-4, IL-17 and FOXP3 expression were performed.All glomeruli and tubules of the HSP/IgA-V patients showed significantly higher IFN-γ and IL-17 expression than those of the control group. Glomerular IFN-γ and IL-17 staining grades correlated with the urinary protein/creatinine ratio (r = 0.62, p = 0.02 and r = 0.507, p = 0.016, respectively). IL-17 expression also correlated with the percentage of crescents (r = 0.518, p = 0.014). IL-4 staining was present in only nine of the 22 patient biopsies and did not correlate with any of the parameters studied. Interstitial areas of patient biopsies had more FOXP3+ cells/μm(2) than those of the control group (p 0.001), but differences in glomerular and tubular FOXP3+ levels (cells/μm(2)) between the two groups were not statistically different. The ISKDC and Oxford scores did not correlate with any parameter studied. However, endocapillary hypercellularity did correlate with IFN-γ expression.Based on these results, we conclude that IFN-γ and IL-17 contribute to HSP/IgA-V in children.

We aimed to review our experience with the clinical characteristics and outcome in childhood liver tumors. We investigated the clinical, laboratory and pathological characteristics, treatments and outcome in hepatoblastomas (HBL) and hepatocellular carcinomas (HCC). We identified 91 HBL and 42 HCC cases. Distant metastases were detected in 16% of HBLs and 22% of HCCs. PRETEXT stages were I/II in 34% and III/IV in 66% of HBLs and I/II in 16% and III/IV in 84% of HCCs. Most cases received cisplatin + doxorubicin chemotherapy. At a median of 58 months, 90 cases had died, 28 were alive, and 15 were lost to follow-up. Five-year survival rates were 32.4% for all HBLs and 15.6% for HCCs. Five-year survival rates were 47% in HBLs and 22.8% in HCCs diagnosed after 1990. In HBLs, distant metastases and absence of chemotherapy response indicated poor prognosis. Prognosis for childhood liver tumors has improved over the last two decades with preoperative chemotherapy with cisplatin + doxorubicin. Surgical resectability is important for cure. For HCC, more effective chemotherapy approaches are essential.

Limb-girdle muscular dystrophies constitute a broad range of clinical and genetic entities. We have evaluated 38 autosomal recessive limb-girdle muscular dystrophy (LGMD2) families by linkage analysis for the known loci of LGMD2A-F and protein studies using immunofluorescence and western blotting of the sarcoglycan complex. One index case in each family was investigated thoroughly. The age of onset and the current ages were between 11/2 and 15 years and 6 and 36 years, respectively. The classification of families was as follows: calpainopathy 7, dysferlinopathy 3, α sarcoglycan deficiency 2, β sarcoglycan deficiency 7, γ sarcoglycan deficiency 5, δ sarcoglycan deficiency 1, and merosinopathy 2. There were two families showing an Emery-Dreifuss phenotype and nine showing no linkage to the LGMD2A-F loci, and they had preserved sarcoglycans. γ sarcoglycan deficiency seems to be the most severe group as a whole, whereas dysferlinopathy is the mildest. Interfamilial variation was not uncommon. Cardiomyopathy was not present in any of the families. In sarcoglycan deficiencies, sarcoglycans other than the primary ones may also be considerably reduced; however, this may not be reflected in the phenotype. Many cases of primary γ sarcoglycan deficiency showed normal or only mildly abnormal δ sarcoglycan staining. Keywords: limb-girdle muscular dystrophy; genetic linkage analysis; sarcolemmal complex proteins

Although whole bowel irrigation (WBI) is a widely used method of bowel preparation in daily surgical practice, almost nothing is known about the histopathologic alterations caused by WBI and whether these differences have any detrimental effect on the outcome of gastrointestinal surgical procedures. Therefore, an experimental study has been conducted to evaluate and compare the effects of WBI with various solutions on the histology of gastrointestinal tract.During the experimental procedures animals were divided into 4 groups consisting of 8 animals each as follows: group A, WBI performed by using isotonic saline solution; group B, WBI performed by using an isoosmolar solution containing polyethylene glycol (PEG); group C, WBI performed by using Lactated Ringer's solution; group D, Animals that were not irrigated but sham operations that were performed served as controls. Four hours after WBI the animals underwent laparotomy and a segment of transverse colon with intact vascular peduncle was prepared. After waiting for 30 minutes, specimens from duodenum, small intestine, large bowel, colonic segment, and liver were obtained from each animal. Histopathologically, all of the specimens were evaluated and graded by 3 parameters including congestion, edema, and inflammation.Although varying degrees of congestion, edema, and inflammation were encountered from all of the specimens of group A, B, and C, only slight congestion was noted in all specimens of group D. The difference between group D and other groups was statistically very significant (P.001). When the sections from duodenums of groups were evaluated, the degree of congestion, edema, and inflammation were found to be moderate in group B, mild-moderate in group A, and mild in group C. Histopathologic examinations of specimens of the small, large bowel, and isolated colonic segment showed severe congestion, edema, and inflammation in group A, moderate-mild in group B, and mild in group C. The difference between A and B, A and C, and A and D was statistically significant (P.01). Although severe congestion was encountered in liver specimens of group A, only mild congestion was encountered in groups B and C (P= .0001). The matched durations of irrigations and total volume of irrigation solutions were found to be not related with the difference in histopathologic findings.WBI has induced varying degrees of histopathologic alterations from mild to severe in the rat gastrointestinal tract. Lactated Ringer's solution and PEG solution have induced the least alterations. Therefore, WBI with Lactated Ringer's solution and PEG solution seem to be safe alternatives of mechanical bowel preparation before elective large bowel surgery. Because saline solution has caused detrimental alterations in distal gastrointestinal tract histology, WBI with saline solution seems to be unadvisable.

Summary The structures of sacs from boys and girls with inguinal hernia, and from boys with undescended testis or hydrocele were evaluated and compared. Particular attention was directed to determine if a clue helpful in describing the mechanism of obliteration of processus vaginalis (PV) exists. Sacs from boys (n: 8) and girls (n: 11) with inguinal hernia, and sacs from boys with undescended testis (n: 11) and hydrocele (n: 10) were obtained and stained with hematoxylin-eosin, trichrome and elastic van Gieson. The histologic structures of each group of samples were determined and compared. Smooth muscle was presented as a layer within the sacs of girls and boys with inguinal hernia. However, smooth muscle bundles were sparsely presented in sacs associated with undescended testis and hydrocele. Myofibroblasts were commonly encountered in sacs associated with inguinal hernia. The smooth muscle was invariably presented in sacs that contained myofibroblasts. While sacs from boys only had smooth muscle, sacs from girls also had striated muscle. Since the muscle components of sacs vary, PV is sexually dimorphic. Persistence of PV seems to be associated with the presence of smooth muscle and myofibroblasts within the sac wall. Myofibroblasts may have originated from the smooth muscle, and reflect the attempts at obliteration of PV.

Background/Purpose: Entamoeba hystolytica (EH) is an enteric protozoan that may cause serious complications that require operative management in children. A retrospective clinical trial was performed to find out the incidence and outcome of complicated intestinal amebiasis (IA) by means of surgically resected specimens in children. Methods: The histopathologic evaluation of 554 intestinal specimens (including appendix and polyp) of 482 patients between 1980 and 1997, inclusive, were reviewed. Intestinal biopsy specimens taken from successfully medically treated patients with uncomplicated IA were excluded. Results: Complicated IA was noted in 18 children (3.7%) consisting of 7 girls and 11 boys with a mean age of 9.2 ± 2.3 years. EH was present in the (1) appendices of 4 patients with perforated appendicitis and 3 patients with normal appendix who underwent negative exploration; (2) juvenile polyps of 4 patients presenting with hematochesia; (3) colonic wall of 1 patient presenting with intussusception and treated by resection and anastomosis; (4) cecum of 1 patient presenting with right lower quadrant mass and underwent exploration with the presumptive diagnosis of lymphoma; (5) colonic wall of 5 patients who underwent ileostomy followed by subtotal colectomy and endorectal pull-through with the presumptive diagnosis of chronic inflammatory bowel disease (n = 3), toxic megacolon + peritonitis (n = 1), and total colonic polyposis (n = 1). All above-mentioned patients are alive and free of symptoms. Conclusions: The diagnosis of IA should be considered in a very wide spectrum of clinical appearances. IA may be associated with colonic polyps and perforated appendicitis, may act as a leading point for intussusception, and may mimic the clinical picture of appendicitis and lymphoma resulting in negative explorations. The diagnosis and treatment of complicated IA in patients who received a misdiagnosis of inflammatory bowel disease is a great challenge and requires major and emergency surgery.

A 5-year-old boy was admitted to the hospital with complaints of abdominal pain, weight loss (2 kg lost in 2 months) and sweating lasting for 2 months. Past medical history was unremarkable. On physical examination his body temperature was 36.8°C (axillary), and the rest of the physical examination was unremarkable. The peripheral blood count showed a hemoglobin level of 14.2 g/dl, a hematocrit value of 41.7%, a platelet count of 318,000/ mm and a white blood cell count of 5,900/mm with normal differential. No atypical cells were seen in his peripheral blood smear. The erythrocyte sedimentation rate was 31 mm/h, and the serum biochemistries were normal except for a temporary increase in transaminases. Creactive protein was 64.2 mg/l (N: 0–10). Levels of urinary catecholamine metabolites, α-fetoprotein, and β-human chorionic gonadotropin were normal. Bone marrow aspiration revealed hypercellularity, but no malignant infiltration was seen. Abdominal computed tomography and magnetic resonance imaging studies showed a retroperitoneal pararenal soft tissue mass extending to the pre-aortic area and occupying mainly the left surrenal region (Fig. 1). The adrenal gland could not be discerned in the mass. The lesion consisted of cystic and solid components. There were also a few lymph nodes varying from 0.5 cm to 1 cm in size in the para-aortic region. At laparotomy, a 6 cm×5 cm×4 cm retroperitoneal mass was found in the left suprarenal region.

This case report describes congenital nephrotic syndrome in a 2-month-old girl associated with cytomegalovirus infection. Histological examination on renal biopsy showed diffuse mesangial sclerosis and cytomegalic inclusion bodies in the tubular cells and in some glomeruli. Cytomegalovirus (CMV) polymerase chain reaction (PCR) titer in serum was high. Remission of pulmonary and renal symptoms was achieved with ganciclovir in 3 weeks. No recurrence of proteinuria was observed during the follow-up period of 14 months. These finding suggested a causal relationship between congenital nephrotic syndrome and cytomegalovirus infection.

In familial Mediterranean fever (FMF), a genetically inherited disease characterized by fever and serositis, renal involvement is mainly AA amyloidosis. We report a patient with FMF who developed mesangial proliferative glomerulonephritis; presumably in response to colchicine treatment, the activity of the disease decreased and renal function tests and urinary findings normalized. This report emphasizes the concurrent existence of mesangial proliferative glomerulonephritis with FMF in the absence of renal amyloidosis. Due to increased inflammatory response observed in FMF, immunologic glomerular injury, a common cause of glomerulonephritis, may occur more frequently in patients with FMF.

While many physicians are familiar with the sexual or physical abuse of children, there is little awareness about Munchausen syndrome by proxy (MSBP). As case reports of MSBP increase, awareness among physicians is thought to increase as well. We thus present herein a 16-month-old girl who admitted to Hacettepe University İhsan Doğramacı Children's Hospital with the complaint of seizure, recurrent apnea and thigh abscess, who was later diagnosed as MSBP. The case was being followed by the Child Protection Team of the hospital (Haceteppe University Child Protection Unit [HU-CPU]). HU-CPU contributed to the early detection of this case and protected the child from a possible fatal outcome. The mother was confronted for MSBP and refused to take responsibility for her child's symptoms. As seen in this case, when MSBP is suspected, psychiatric evaluation of the mother, evaluation of the mother-child interaction and collection of a detailed family and social history can have a positive impact on the prognosis in these cases. This case report underlines the importance of multidisciplinary team work to share the responsibility and reduce the burden during the treatment process of these difficult and complicated cases.

Alpha-fetoprotein (AFP) is used as a tumor marker for hepatocellular carcinoma, hepatoblastoma and germ cell tumors. It may also be elevated in infants with some hepatobiliary disorders. The mechanism of AFP elevation in neonatal cholestasis is not known. We retrospectively evaluated serum AFP levels in 53 infants with neonatal cholestasis. Thirty patients (56.6%) had elevated AFP, and the ratio of patients with elevated AFP was significantly high in both the metabolic diseases and idiopathic neonatal hepatitis groups (p=0.021). Serum aspartate aminotransferase (AST) levels increased significantly in patients with elevated AFP (p=0.004). Steatosis was the distinctive histopathological finding of the patients with high AFP. The patients with steatosis had significantly higher standard deviation (SD) score of AFP than the patients without steatosis (p=0.001). We have shown AFP elevation in neonatal cholestasis due to metabolic disorders and idiopathic neonatal hepatitis and its association with steatosis and AST elevation.

We studied the cytokines secreted by the inflammatory T cell subgroups (IFN-γ and IL-17) and FOXP3 expression in lupus nephritis (LN) and analyzed associations with clinical and histopathological parameters.Renal tissue samples of 39 LN patients were studied. Immunohistochemical staining was carried out with antibodies against IFN-γ, IL-17, and FOXP3.Both IFN-γ (+) and IL-17+ cells were statistically higher in LN tissues when compared with controls (p 0.01). The cells in the tubulointerstitium were CD3 + CD4+ displaying a Th1 and Th17 phenotype, whereas the less intense population in the glomeruli was CD3-CD4-. Interstitial CD3 + CD4+ FOXP3+ cells were also significantly higher in LN biopsies than in control tissues (p 0.01). IFN-γ (+) and IL-17+ cells were more intense among class IV LN as compared to class II, III LN (p 0.01 and p = 0.001, respectively). Subsequently, when IL-17 and IFN-γ staining was compared between the proliferative LN classes, class III and IV patients had more intense staining compared to class II (all p 0.05). IFN-γ immunostaining correlated positively with serum creatinine and negatively with albumin levels and glomerular filtration rate (GFR). IL-17 immunostaining correlated with proteinuria, requirement for pulse steroids, and SLEDAI renal score, and negatively with GFR. Furthermore, glomerular and interstitial IL-17 and IFN-γ stainings were significantly associated with various parameters of histological activity (p 0.05).We suggest that IFN-gamma and IL-17 could have a role in the pathogenesis and progression of LN. The Th1 and Th17 cells may be imperative in the severity of LN. Recognizing the complexity of the immune pathways involved in lupus reminds us that targeting B cells only may not suffice to control the progression of the inflammation.

This study aimed to evaluate the diagnosis, treatment and follow-up of child abuse and neglect (CAN) cases. Subjects were 102 cases that were clinically assessed by the multidisciplinary hospital team. Early and late childhood cases (according to the age at first abuse) were compared by means of characteristics of abuse. Among the 102 subjects, 64 were female and 38 were male (mean age: 8.68 years). Being abused by someone within the family was found to be significantly higher in preschool children compared to children in late childhood. 27.5% (n: 28) of the cases concomitantly had low socioeconomic and educational level and high parental psychological problems and 64.8% had psychiatric problems. A limited number of studies have compared characteristics of early and late childhood abuse considering the age of onset of maltreatment. Consistent with the literature, our study showed that early age of onset of maltreatment is a poor prognostic factor in many ways.

This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions.The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out. Excluding epithelial tumors of the skin, leukemia, lymphoreticular system neoplasias, metastatic tumors, and hamartomas, 1362 solid tumors in 1358 patients were classified according to age, sex, localization, histopathology and clinical behavior.The male/female ratio was 0.9; 14.8% (201) of the patients belonged to 0-1 year age group, 20.7% (281) to 2-4 years, 25.9% (352) to 5-10 years, 22.2% (301) to 11-14 years, and 16.4% (223) to 15-18 years. Among all tumors, 708 (52.0%) were malignant, 645 (47.4%) benign tumors, 2 (0.1%) borderline tumors, and 2 (0.1%) unknown behavioral tumors. Malignant tumors were found in 50.2% (357) of female patients and in 54.0% (349) of male patients. A balanced distribution between benign and malignant entities among children under 18 years was observed. Comparison between the age groups revealed malignant cases outnumbered benign cases under 4 years of age while benign tumor numbers increased after 10 years of age. The most common entities in the malignant group were of sympathetic nervous system origin, while soft tissue tumors far outnumbered the others in the benign group.We conclude that the cancer patterns of children in the Ankara region mostly resemble with those of the western population. This study provides useful information on the diagnosis of solid tumors in children and highlights variations in cancer incidence in different age groups.

Methods All children [n: 422; mean age 7.90 ± 3.95 (range: 4 mo.18 years); 48.2% female] presenting to the outpatient clinic for the first time due to pain, swelling or limitation of movement attributed to musculoskeletal system in a 6 month period were enrolled. Demographical features, duration, and type of complaints, physical signs on initial presentation and laboratory findings [a complete blood count, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)] were recorded.

Nemaline myopathy (NEM) is a common congenital myopathy. At the very severe end of the NEM clinical spectrum are genetically unresolved cases of autosomal-recessive fetal akinesia sequence. We studied a multinational cohort of 143 severe-NEM-affected families lacking genetic diagnosis. We performed whole-exome sequencing of six families and targeted gene sequencing of additional families. We identified 19 mutations in KLHL40 (kelch-like family member 40) in 28 apparently unrelated NEM kindreds of various ethnicities. Accounting for up to 28% of the tested individuals in the Japanese cohort, KLHL40 mutations were found to be the most common cause of this severe form of NEM. Clinical features of affected individuals were severe and distinctive and included fetal akinesia or hypokinesia and contractures, fractures, respiratory failure, and swallowing difficulties at birth. Molecular modeling suggested that the missense substitutions would destabilize the protein. Protein studies showed that KLHL40 is a striated-muscle-specific protein that is absent in KLHL40-associated NEM skeletal muscle. In zebrafish, klhl40a and klhl40b expression is largely confined to the myotome and skeletal muscle, and knockdown of these isoforms results in disruption of muscle structure and loss of movement. We identified KLHL40 mutations as a frequent cause of severe autosomal-recessive NEM and showed that it plays a key role in muscle development and function. Screening of KLHL40 should be a priority in individuals who are affected by autosomal-recessive NEM and who present with prenatal symptoms and/or contractures and in all Japanese individuals with severe NEM.

Gastrointestinal stromal tumor is a rare pathology in childhood. It may occur anywhere along the alimentary tract and represents with a wide spectrum of signs and symptoms according to location. An infant with malignant colonic stromal tumor that has presented with a huge abdominal mass and lower gastrointestinal bleeding is reported to discuss the difficulties in diagnosis and differential diagnosis of this unusual tumor.

By comparing our data for the period 1985-2008 with findings from a previous report covering the period 1975-1984, we aimed to share our experience with poisoning cases in order to contribute toward its prevention, diagnosis and treatment. The records of patients admitted to the Pediatric Intensive Care Unit with acute poisoning between November 1985 and October 2008 were evaluated retrospectively. The records of 2251 patients with acute poisoning could be retrieved. Poisoning mostly occurred in the home (92%), via the oral route (92.5%) and by a single intoxicant (81.3%). Two distinct peaks were observed: in boys between 1-5 years of age and in girls between 13-16 years of age. It was noted that 67.4% of poisoning cases were accidental, whereas 25.9% were suicidal and 6.7% were a result of a therapeutic error. Nearly two-thirds (64%) of cases were drug-related, while 36% were non-drug-related. Analgesics-antipyretics ranked first among the drug-related cases, whereas ingestion of a corrosive substance was most common among cases with non-drug poisoning. Colchicine was associated with the highest fatality, while among the causes of non-drug poisoning, carbon monoxide was the deadliest. The overall mortality rate in this study was 1.9%. Mortality from non-drug poisoning was higher than from drug-related causes (3.9% vs. 1.3%). Almost all cases of poisoning below the age of 6 years are potentially preventable. The results of this study highlight the need for reforms in industrial and health policies, with the aim of increasing awareness regarding potential toxins, appropriate storage of potential toxins, and general precautions to promote safety in the home.

Kawasaki disease (KD) is an acute, systemic and self-limited vasculitis that is complicated with the development of coronary artery (CA) aneurysms. We present the clinical features of Turkish KD patients from a tertiary referral center. When 33 KD patients were assessed, a number of features stood out as differing from the expected, for example, periungual peeling 7.5 +/- 7.5 days after fever onset--42.4% of patients had periungual peeling within 14 days after fever onset. CA involvement was detected at an average of 12.3 +/- 7.9 days after fever onset. Fifty percent of the patients had been diagnosed to have CA involvement within eight days after the onset of fever. The performance of criteria suggested by American Heart Association was satisfactory, with 19 of 29 patients (65.5%) having three or more of the required laboratory features (sensitivity 65.5%). We believe Turkish patients may present differences in the course of KD.

Papillary thyroid carcinoma has a favorable outcome in children. Recent experience with young children cases with early recurrences, after the total thyroidectomy and excision of palpable lymph nodes, in contrast to usual course of disease in adolescent cases, prompted us to review our experience on papillary carcinoma surgery.Sixteen children who underwent surgery for papillary carcinoma between 1997 and 2010 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, past medical history, results of laboratory tests, imaging findings, aspiration biopsy, extent of disease, details of surgery, complications and postoperative course were noted.Male to female ratio was 3:1. The mean age was 10.9 years (range, 5-16). The presenting symptom was palpable swelling in the neck (n = 12) or asymptomatic nodule was detected incidentally (n = 4). Physical examination findings were palpable nodule (n = 7), lymphadenopathy (n = 6) or both (n = 4). Thyroid ultrasound (US) revealed nodule (n = 9), heterogenisity of the parenchyma and enlargement of thyroid (n = 9), and microcalcifications (n = 5). Fine-needle aspiration biopsy was performed in 12 patients and was suggestive for malignancy in most cases (91%). Pulmonary metastasis was detected at presentation in two patients and appeared after thyroidectomy in one patient. Complementary thyroidectomy was performed in nine patients because the total thyroidectomy with or without cervical lymph node dissection was the treatment of choice (n = 15). Iodine scan was performed 4 weeks later following thyroidectomy. Radioactive iodine ablation (RIA) therapy was given to 11 patients who had residual or recurrent disease. Postoperative complications were permanent hypoparathyroidism (n = 4), recurrent laryngeal nerve injury (n = 3), lymphorea (n = 1) and Horner's syndrome (n = 1).Total thyroidectomy and excision of affected lymph nodes is the current mode of surgical management for thyroid papillary carcinoma in children. However, especially in young children (10 years), modified lymph node dissection should be added to total thyroidectomy in order to avoid leaving the residual tumor foci which can impair the efficacy of the ablation therapy.

Objective: The aim of this study was to evaluate distinguishing the right/left side of the fetus, cardiac axis and position according to the standardized Cordes technique in 20 cases with cardiac malposition. Methods: We studied retrospectively 1536 cases whose fetal echocardiographic examinations were performed between 1999 and 2006 in prenatal cardiology unit. Among these, cardiac malpositions were determined in 20 cases. The cardiac axis and position were determined according to the Cordes technique. All cases were followed-up by serial fetal echocardiograms until birth or intrauterine death occurred. In cases of intrauterine death, an autopsy was performed. After birth, physical and echocardiographic examinations were done and prenatal and postnatal diagnoses were compared. Results: Of 1536 fetal echocardiograms performed, 144 revealed congenital heart diseases (9.4%), among these cases 20 were diagnosed with cardiac malposition. Of cases with cardiac malposition, 16 had congenital heart disease, and four had extracardiac malformation. There were six cases of isolated dextrocardia, three cases of situs inversus totalis, six cases of situs ambiguous, and one case of situs inversus with isolated levocardia. Of four cases with extracardiac malformations, two cases had mesoposition, one had dextroposition, and one had extreme levoposition. In six cases the autopsy findings were the same as that their prenatal echocardiographic findings. When postnatal echocardiographic results of the remaining cases with cardiac malposition due to congenital heart disease were compared with prenatal diagnoses, the same echocardiographic findings were verified. Conclusion: The fetal right/left axis must be determined correctly for the accurate diagnosis of cardiac malpositions. Therefore, we recommend that Cordes technique provides a simple and reliable determination of the fetal right/left axis and fetal situs. Bu çalışmada, standart Cordes tekniğine göre fetüsün sağ/sol tarafının, kalbin aksının ve pozisyonunun ayrımı ve bu teknikle kardiyak malpozisyon tanısı konulan 20 olgunun değerlendirilmesi amaçlandı. Yöntemler: Çalışmamızda, 1999-2006 yılları arasında prenatal Kardiyoloji ünitesinde fetal ekokardiyografi yapılan 1536 olguyu retrospektif olarak değerlendirildi. Bu olguların 20’sinde kardiyak malpozisyon saptandı. Kalbin aksı ve pozisyonu Cordes tekniğe göre saptandı. Tüm olgular seri fetal ekokardiyografik çalışmalar ile doğuma veya intrauterinde ölüm meydana gelene kadar izlendi. İntrauterin ölen olgulara otopsi yapıldı. Doğumdan sonra fizik muayene ve ekokardiyografik değerlendirme yapıldı, prenatal ve postnatal tanılar karşılaştırıldı. Bulgular: Fetal ekokardiyografi yapılan 1536 olgunun 144’ ünde konjenital kalp hastalığı saptandı ve bu olguların 20’sine kardiyak malpozisyon tanısı konuldu. Kardiyak malpozisyonlu olguların 16’sında konjenital kalp hastalığı ve 4’ünde kalp dışı nedenlere bağlı malpozisyon mevcuttu. Altı olguda izole dekstrokardi, 3 olguda situs inversus totalis, 6 olguda situs ambigus ve 1 olguda izole levokardi ile birlikte situs inversus saptandı. Kalp dışı nedenlere bağlı malpozisyonlu 4 olgunun 2’sinde mezopozisyon, 1’inde dekstropozisyon ve 1’inde ileri levopozisyon mevcuttu. Altı olguya yapılan otopsi bulguları prenatal ekokardiyografik bulgular ile benzerdi. Konjenital kalp hastalığına bağlı kardiyak malpozisyonlu diğer olguların doğum sonrası ekokardiyografik sonuçları prenatal tanılarıyla karşılaştırıldığında aynı sonuçlar elde edildi. Sonuç: Kardiyak malpozisyonun doğru tanısı için fetüsün sağ/sol aksı doğru saptanmalıdır. Bu nedenle fetal situs ve fetüsün sağ/sol aksının saptanmasında kolay ve güvenilir bir teknik olan Cordes tekniğini öneriyoruz.

Pleuropulmonary blastoma is an uncommon primary malignant tumor of the lung. Surgery is the most important part of the treatment. Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass. The patient underwent surgical resection of the mass. Pathologic diagnosis was type II pleuropulmonary blastoma. Postoperative thoracic CT showed nodular residual densities so the patient was given adjuvant combination chemotherapy with cisplatin and etoposide. He has been disease free for 12 months.

Carbon monoxide (CO)-induced cardiotoxicity has been investigated infrequently in children and reports of its cardiovascular effects are limited to isolated case reports. Our aims were to describe acute cardiac effects and associated factors with myocardial injury in children with CO poisoning.We reviewed the medical records of children below 17 years of age who were diagnosed with CO poisoning at pediatric emergency department between July 2004 and June 2007. Patients who had carboxyhemoglobin level at least 10% were included. Myocardial injury was defined as elevated cardiac biomarkers (creatine kinase-MB or troponin-t). Carboxyhemoglobin level, electrocardiogram (ECG) findings, cardiac biomarkers, and echocardiograph results were recorded for each patient.Cardiac biomarkers were drawn in 107 patients, of which 16 patients (15%) had cardiac biomarkers confirmed diagnosis of myocardial injury. Sinus tachycardia was present in 32% of patients on baseline ECG. None of the patients had ischemic changes on ECG. Echocardiograph was performed in 27 patients (25% of patients with biomarkers drawn), of which nine patients had low ejection fraction and abnormal left ventricular function. Determinators of myocardial injury included a Glasgow Coma Scale (GCS) scoreor=14 and hypotension (for GCS scoreor=14 and hypotension, relative risk: 90 and 95% confidence interval: 9.9-813.Myocardial injury may exist in children with CO poisoning without abnormal ECG findings. GCS scoreor=14 and hypotension were associate factors with myocardial injury.

Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT.Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined.All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002).Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.