Celiac disease does not influence markers of ovarian reserve in adolescent girls.

PURPOSE: The aim of the study was to determine whether celiac disease affects ovarian reserve assessed by antral follicle counting, ovarian volume, and anti-müllerian hormone in adolescent patients. METHODS: This case-control multicenter trial was performed from January 1, 2017 to May 31, 2018 and included 45 girls. On days 2-5 of the menstrual cycle, measurements of serum follicle stimulating hormone, luteinizing hormone, estradiol, prolactin, and anti-müllerian hormone were performed. Antral follicle counts and ovarian volumes were determined on the same day. RESULTS: Evaluation was made of 21 (47.7%) celiac patients with a mean age of 15.8 ± 1.3 years, and 24 (52.3%) healthy control subjects with a mean age of 16.2 ± 1.2. There was no difference between the groups in respect of right and left ovarian volumes (p = 0.790 and p = 0.670, respectively). Serum levels of anti-müllerian hormone of the celiac patients and controls were found comparable [(3.7 ± 2.9 (0.5-12) and 3.6 ± 1.8 (1.2-8.1)] ng/mL, respectively, p = 0.915). CONCLUSIONS: Celiac disease may not affect the ovarian reserve determined with established ovarian reserve markers including antral follicle counting, ovarian volume, and anti-müllerian hormone in adolescent patients. TRIAL REGISTRATION: ClinicalTrials.gov identifier (NCT number): NCT04024449 https://clinicaltrials.gov/ct2/show/NCT04024449.

Fecal calprotectin in children with familial Mediterranean fever in the attack-free period.

BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever and serosal inflammation. The aim of this study was to evaluate fecal calprotectin (FC) in children with FMF during the non-attack period. METHODS: A retrospective evaluation was made of the data of a total 66 patients diagnosed with FMF in an attack-free period and without amyloidosis or inflammatory bowel disease (IBD). FC level in the FMF patients was compared with that in the patients with IBD and healthy control subjects. RESULTS: The FMF patients consisted of 37 boys (56.1%) with a mean age of 10.1 ± 3.9 years. Mean FC was 192.5 µg/g (range, 19.5-800 µg/g) in the FMF group, 597.9 µg/g (range, 180-800 µg/g) in the IBD group, and 43.8 µg/g (range, 19.5-144 µg/g) in the control group. The FC level in the children with FMF was higher than in the control group (P < 0.001), and the FC level of the IBD patients was higher than both the FMF and the control groups (P = 0.020, P < 0.001, respectively). CONCLUSIONS: FC was higher in FMF patients compared with healthy children even in the absence of IBD/amyloidosis. Even though colonoscopy is the gold standard in identifying intestinal inflammation in FMF patients, FC, a non-invasive and inexpensive method, can be used for screening. The presence of subclinical intestinal inflammation was also quantitatively identified in children with FMF.

Gallbladder Polyps: Rare Lesions in Childhood.

OBJECTIVE: Unlike adults, gallbladder polyps (GPs) are rare in childhood. The aim of this study was to evaluate patients with a GP diagnosis. METHODS: Patients who were diagnosed with GP via ultrasonography from October 2012 to October 2017 were retrospectively evaluated in terms of sociodemographic characteristics and laboratory findings. RESULTS: The study included 19 patients diagnosed with GP and followed up in our department. The patients comprised 14 (73.6%) girls with a mean age of 13.9 ±â€Š4.1 years and a mean follow-up period of 10.2 ±â€Š5.4 months (range, 3-26 months). The most common presenting symptom of the patients (n = 15, 78.9%) for ultrasonography was abdominal pain without biliary symptoms. Location of the polyps was in the corpus in 55% of patients, and either in the fundus (20%) or the neck of the gallbladder (25%). The average diameter of the polyps was 4.5 ±â€Š1.6 mm (range, 2-9 mm). Multiple polyps were observed in 3 patients. No significant change in the number or size of polyps was noted at the end of the follow-up periods. Cholecystectomy was applied to 1 patient who had >5 polyps with a rapid increase in size, and the pathology report was hamartomatous polyp. There was no remarkable change in the clinical or laboratory findings of other patients during the follow-up period. CONCLUSION: In this study, GPs could be seen in young children as young as 16 months of age and ultrasonography is sufficient for follow-up in stable and asymptomatic patients.

Prevalence of celiac disease among school-age children in Çorum, Turkey.

BACKGROUND/AIMS: Celiac disease (CD) is an autoimmune enteropathy that develops in individuals with genetic susceptibility as a result of a permanent sensitivity to gluten found in grains. The prevalence of CD in Turkey is between 0.3% and 1%. However, the prevalence of CD in Çorum, a city in middle Anatolia in Turkey, is unknown. The purpose of this study was to identify the prevalence of childhood CD in Çorum and to detect patients with silent and atypical CD. MATERIALS AND METHODS: The sample size was calculated using a stratified sampling method, to provide the sample number that would best represent this population. Screenings were conducted using rapid tissue transglutaminase IgA test kits. RESULTS: A total of 1730 students were included in the study; 877 (50.6%) were female. Of students in the city center, 301 (34%) were in primary school, 299 (34%) were in secondary school, and 283 (32%) were in high school. As for towns, 847 (49%) students from 92 schools were included in the study. Eight children had positive screening results; 4 (50%) were female, and the average age was 11.6±3.4 (9-17) years. According to the celiac serology results and endoscopic duodenum biopsies, all children with positive screening results were diagnosed with CD. The prevalence of CD was found to be 0.46% in schoolchildren. CONCLUSION: Various studies in Turkey have reported a prevalence of CD between 0.6% and 0.9%, with 0.47% reported in a multicenter study. The present study identified CD prevalence as 0.46% (1 in 216) among children in Çorum, Turkey.

Efectos de la edad al momento del diagnóstico y cumplimiento de la dieta sobre los parámetros de crecimiento de los pacientes con celiaquía / Effects of age of diagnosis and dietary compliance on growth parameters of patients with celiac disease

Introducción. El objetivo fue evaluar la relación entre edad al diagnóstico y cumplimiento de dieta sin gluten (DSG) y su efecto sobre el crecimiento de niños celiácos y factores que influenciaron el cumplimiento de la DSG. Población y métodos. Se incluyeron pacientes celíacos con seguimiento en nuestro hospital entre enero 2015 a enero 2017. Se los clasificaron según edad al diagnóstico y cumplimiento de la DSG. Se compararon características antropométricas al diagnóstico y durante el seguimiento. Resultados. Participaron 73 pacientes con edad promedio de 10,4 ± 4,5 años; 35 (47,9%), los pacientes de talla baja al diagnóstico; eran mayores (7,8 ± 4,2 años) que los demás (5,1 ± 4,3 años de edad) (p= 0,005). Al diagnóstico, 33 (45,2%) pacientes tenían ≤6 años y 40 (54,8%) tenían >6 años. Los puntajes Z de estatura y peso a la edad >6 años eran significativamente menores que los diagnosticados a ≤6 años, en el diagnóstico (p= 0,01 y 0,04, respectivamente) como en el último control (p= 0,001 y 0,001, respectivamente). Tuvieron cumplimiento riguroso con DSG en 45 (61,6%) pacientes. Al comparar datos antropométricos , el aumento del índice de masa corporal (IMC) y del puntaje Z de peso en el grupo que cumplió la dieta fue significativamente mayor que en el otro grupo.Conclusiones. Demorar el diagnóstico de celiaquía afectó la estatura y peso. El cumplimiento de la DSG mejoró los parámetros de crecimiento, principalmente, el puntaje Z de peso y el IMC.

Introduction. The objective of this study was to evaluate the relation between age at diagnosis and compliance to gluten free diet (GFD) on growth in children with celiac disease and the factors that influenced compliance to GFD. Population and Methods. Celiac disease (CD) patients with villous atrophy followed in our hospital between January 2015 and January 2017, were included. They were classified according to diagnosis age and GFD compliance. Patients' anthropometric characteristics at diagnosis and follow-up were compared. Results. There were 73 patients with 10.4 ± 4.5 years of average age, 35 (47.9%) patients had a short stature at diagnosis, the ages of patients who had short stature (7.8 ± 4.2 years) were higher than those who did not (5.1 ± 4.3 years) (p= 0.005). At diagnosis, 33 (45.2%) patients were aged ≤6 years, 40 (54.8%) were aged >6 years. The height and weight z-scores of patients who were diagnosed at >6 years of age were significantly lower than those who were diagnosed ≤6 years of age both at diagnosis (p= 0.01 and 0.04) and at last control (p= 0.001 and 0.001), respectively. Forty-five (61.6%) patients were fully compliant with GFD. In comparison of anthropometric data in terms of GFD compliance, the increase in BMI and weightz-score in the fully compliant group was found to be significantly higher when compared with the other group. Conclusions. Delay in CD diagnosis negatively affected both the height and weight and other growth parameters. GFD compliance positively affected the patients' all growth parameters, especially weight and BMI z-score.

Retrospective analysis of children with α-1 antitrypsin deficiency.

BACKGROUND: α-1 Antitrypsin (AAT) deficiency is the most frequently occurring genetic liver disorder. The association among classical α-1 antitrypsin deficiency (AATD), chronic liver disease, and cirrhosis is common in adult patients but rare in children. AIM: To assess the clinical characteristics of children with AATD and to compare symptoms between homozygous and heterozygous children. MATERIALS AND METHODS: The study included 20 children who were found to have mutant Pi alleles. AAT phenotyping was conducted on patients with a low serum AAT level. The exclusion criteria included infectious, anatomic, and metabolic conditions. Symptoms on presentation, physical examination findings, laboratory values, liver biopsy results, and follow-up periods were recorded for each patient. RESULTS: The patients included six (30%) girls and 14 (70%) boys, with a mean age of 6.3±5.1 (1-16) years. The PiZZ phenotype was present in eight (40%) and PiMZ in 12 (60%) patients. The most frequent symptom was elevated liver function test results. Three patients were referred with neonatal cholestasis and one with compensated cirrhosis. Eight patients underwent liver biopsy; all patients except one had periodic acid-Schiff-positive diastase-resistant globules in the hepatocytes. The mean follow-up period was 34±33 (12-101) months. At the end of follow-up, all patients with PiZZ were found to have chronic hepatitis, and one with cirrhosis. On the contrary, two patients with PiMZ were found to have chronic hepatitis. CONCLUSION: Children with classical AATD commonly have chronic liver disease. In heterozygous (PiMZ) children with AATD, enzyme levels can normalize with occasional fluctuations, sometimes causing delayed diagnosis.

Ascites and abdominal pseudocyst: two uncommon ventriculoperitoneal shunt complications in two cases.

Ascites and abdominal pseudocysts (APC) are two rare complications that can occur following placement of a ventriculoperitoneal (VP) shunt. Both complications are characterized by abnormal intraperitoneal cerebrospinal fluid (CSF) collections. Although various factors have been implicated, the exact pathogenesis of the two conditions remains elusive. This paper presents two cases of VP shunt placement resulting from hydrocephaly. The first patient presented with generalized ascites and the other with APC, both of whom were six years old. APC and ascites after VP shunt placement are rare and distinct conditions; therefore, they may require different management strategies.

The coexistence of thymic carcinoma and multiple granulomas in a Turkish child.

Thymic carcinoma, which is a thymic epithelial neoplasm with obvious cytologic atypia, is a rare neoplasm. The authors report on a 10-year-old boy who presented with respiratory distress due to bulky anterior mediastinal mass. Histological and immunohistochemical studies confirmed a lymphoepithelioma-like pattern thymic carcinoma. In addition, evaluation of the specimen showed foci of caseation and multiple granulomas with extensive central necrosis within the thymic tissue. The child received chemotherapy, followed by surgery and radiotherapy. To rule out difficulties of tuberculosis he also received antituberculosis therapy. Two years after cessation of treatment, he is still in remission for thymic carcinoma.

The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?

Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old girl with CN and PS. Although some rare associations of renal and pulmonary lesions have been reported, this is the first case report in the English literature that shows the association of CN with PS.