Vanishing testes syndrome is often referred to as testicular regression syndrome (TRS) in the recent medical literature. The most characteristic histological findings are presence of a fibrovascular nodule with associated hemosiderin-laden macrophages and dystrophic calcification. Residual testicular tubules are found in less than 10% of cases, with prevalence being unrelated to age at surgery. Presence of seminiferous tubules and viable germ cells in testicular remnant tissue has been reported in some series. TRS theoretically carries a potential for malignant degeneration in the long term and therefore removal of any remnant is a common practice to eliminate this risk. However, no case series has reported germinal dysplasia or intratubular germ cell neoplasia in any of the specimens taken from these patients.Conflict of interest:None declared.

Objective: To compare the prevalence of the metabolic syndrome (MS) in Turkish obese children and adolescents by using three different definitions and to assess the risk factors through a retrospective evaluation of anthropometric and laboratory parameters.Methods: Sixty hundred and fourteen obese patients (307 male, 307 female; mean age: 11.3±2.5 years) were included in the study. Medical history, physical examination, anthropometric measurements, results of biochemical and hormonal assays were obtained from the hospital records. MS was diagnosed according to the modified World Health Organization (WHO), Cook and the International Diabetes Federation (IDF) consensus criteria.Results: The prevalence of MS was found to be 39%, 34% and 33% according to the modified WHO, Cook and the IDF consensus criteria, respectively. MS prevalence in patients aged 12-18 years was significantly higher than that in patients between 7 and 11 years of age (p<0.05). Pubertal patients had a significantly higher MS prevalence than the non-pubertal cases (p<0.05). MS prevalence was also significantly higher in children who had a family history of heart disease, diabetes, obesity and hypertension as well as in those who had not been breast-fed (p<0.05).Conclusion: The use of the modified WHO criteria was found to result in a slightly higher prevalence rate for MS as compared to the other criteria. The prevalence of MS in our study population was higher than that reported in most previous studies in Turkey. A positive family history, puberty and not being breastfed in infancy were shown to be significant risk factors for MS in childhood.The prevalence of MS was found to be 39%, 34% and 33% according to the modified WHO, Cook and the IDF consensus criteria, respectively. MS prevalence in patients aged 12-18 years was significantly higher than that in patients between 7 and 11 years of age (p<0.05). Pubertal patients had a significantly higher MS prevalence than the non-pubertal cases (p<0.05). MS prevalence was also significantly higher in children who had a family history of heart disease, diabetes, obesity and hypertension as well as in those who had not been breast-fed (p<0.05).Conclusion: The use of the modified WHO criteria was found to result in a slightly higher prevalence rate for MS as compared to the other criteria. The prevalence of MS in our study population was higher than that reported in most previous studies in Turkey. A positive family history, puberty and not being breastfed in infancy were shown to be significant risk factors for MS in childhood.Conflict of interest:None declared.

Objective: To investigate the efficacy and safety of n-3 polyunsaturated fatty acids (PUFA) treatment in obese children with nonalcoholic fatty liver disease (NAFLD).Methods:One hundred and eight obese (body mass index (BMI) >95th percentile for age and sex) adolescents with NAFLD were included in the study. Mean age of the subjects was 13.8±3.9 years (9-17 yrs). The diagnosis of NAFLD was based on the presence of liver steatosis with high transaminases. The subjects were randomly divided into two groups. Group 1 (PUFA group, n=52) received a 1000 mg dose of PUFA once daily for 12 months and lifestyle intervention. Group 2 (placebo group, n=56) received a recommended diet plus placebo and lifestyle intervention for 12 months. Insulin resistance was evaluated by homeostasis model assessment of insulin resistance (HOMA-IR) from fasting samples.Results: BMI, fasting insulin levels and HOMA-IR values in both groups decreased significantly at the end of the study. In group 1, 67.8% of the patients had a decrease from baseline in the prevalence of steatosis (p<0.001). Frequency of elevated alanine aminotransferase (ALT) levels (39.2% to 14.2%; p<0.01) and elevated aspartate aminotransferase (AST) levels (25% to 17.8%; p=0.01) decreased significantly in the PUFA group. Following a 12-month diet plus placebo and lifestyle intervention treatment, 40.3% (21) of the patients in the placebo group also showed a decrease in frequency of steatosis (p=0.04) and slight decreases in frequency of elevated ALT levels (38.4% to 28.8%; p=0.01) and AST levels (30.7% to 28.8%; p>0.05).Conclusion: Our results indicated that n-3 PUFA treatment is safe and efficacious in obese children with NAFLD and can improve ultrasonographic findings and the elevated transaminase levels.

Objective:Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods:Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results:The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto’s thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion:This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan.

This large case series underlines the variation in the clinical phenotype of RTHα patients. RTHα should be suspected in subjects when even mild clinical and laboratory features of hypothyroidism are present along with high/high-normal free T3, low/normal free T4, and normal TSH.