Renal Anomalies Associated with Ectopic Neurohypophysis

Objective: Although the etiology of ectopic neurohypophysis that leads to pituitary hormone deficiencies is not yet clearly understood, birth trauma or genetic factors have been considered responsible. Concurrent cranial and extracranial congenital anomalies have been reported in such cases. The aim of the present study was to investigate the frequency of renal anomalies in nonsyndromic cases with ectopic neurohypophysis

Is Dietary Macronutrient Distribution Related to Serum Lipid Profiles in Children and Adolescents with Type 1 Diabetes?

Aim:The aim of this study was to evaluate the dietary macronutrient distribution affects on serum lipid profiles in type 1 diabetes mellitus (T1D).Materials and Methods:This cross-sectional study included 82 children and adolescents between the ages of 2 and 18 years with a diabetes age of ≥1 year. Dietary intake was evaluated by 3-day food diaries, including three consecutive days (two weekdays and one weekend day).Results:The mean age of the 82 patients with diabetes was 11.6±4.3 years (range: 2-18 years) (45.1% female), the median diabetes duration was 3.4 (2.9) years, the mean HbA1c level was 7.0±1.4%, and mean body mass index standard deviation score was 0.2±1.1. The median distribution of energy from carbohydrates, protein and fat in the total energy intake was 50.0% (6.2), 17.4% (2.7) and 32.5% (5.1), respectively. Dietary fiber intake was inadequate in 64 (77.9%) participants, while for 77 participants (93.9%), saturated fatty acid intake was above the recommended intake. For children and adolescents with T1D, mean serum cholesterol, triglycerides, low-density lipoprotein, and high-density lipoprotein levels were 162.9±33.4 mg/dL, 86.2±49 mg/dL, 87.9±29.2 mg/dL, and 60.7±17.9 mg/dL, respectively.Conclusion:To maintain healthy eating, consuming foods high in saturated fat should be limited, and children and adolescents with T1D should be supported by their family and healthcare professionals in the consumption of diets high in fiber

A Randomized Clinical Trial Comparing Breakfast and Bedtime Administration of Insulin Glargine in Children and Adolescents with Type 1 Diabetes

Background: Insulin glargine provides effective glycemic control when administered at bedtime in adults

Clinical Course of Hashimoto's Thyroiditis and Effects of Levothyroxine Therapy on the Clinical Course of the Disease in Children and Adolescents

Objective: The aim of this study was to evaluate the clinical course of Hashimoto’s thyroiditis (HT) in children and adolescents and the effects of levothyroxine therapy on the clinical course and laboratory findings

Ghrelin Levels in Children with Constitutional Delay of Growth and Puberty

Objective: In this study, we aimed to show the role of ghrelin in growth delay in children with constitutional delay of growth and puberty (CDGP)

Bannayan-Riley-Ruvalcaba Syndrome in a Case Evaluated Due to Multinodular Goiter

Bannayan-Rıley-Ruvalcaba syndrome (BRRS) is characterized by macrocephaly, pigmented macules on the glans penis and benign mesodermal hamartomas. 9.6-year-old boy was referred to the pediatric surgeon following an observation of a subcutaneous lipomatous lesion and numerous nodules in the thyroid gland via ultrasonography performed due to swelling in the neck first noticed approximately 3 months previously. Thyroid ultrasonography revealed numerous nodules with distinct margins in both lobes of the thyroid gland, some exhibiting calcification and others hypoechoic areas, and a total thyroidectomy was performed due to a suspicion of malignity. After surgery, the patient was referred to the Pediatric Endocrinology Department. On physical examination, his weight was 30 kg [standard deviation score (SDS): -0.38], height 140 cm (SDS: 0.71) and head circumference 59.5 cm (SDS: +3.21). Pubic hair was Tanner stage 2, bilateral testes 3+3 mL palpable. There was multiple hyperpigmented lesions on the penile skin. His past medical history revealed that pubic hair development was reported at the age of 8 years. Laboratory examinations revealed a 17-OH progesterone level of 4.8 ng/mL, bone age compatible with 8 years. P. V281L heterozygous mutation was determined via CYP21A2 mutation screening performed for non-classic congenital adrenal hyperplasia. BRRS was primarily suspected in this case of macrocephaly, lipomatous lesions and pigmented macular lesions on the penis. Heterozygous p.C136R mutation was determined via PTEN mutation scanning

Clinical characteristics and growth hormone treatment in patients with prader-willi syndrome

Objective: To investigate clinical characteristics and response to growth hormone (GH) treatment in patients with Prader-Willi syndrome (PWS) in Turkey. Methods: The data of 52 PWS patients from ten centers was retrospectively analyzed. A nation-wide, web-based data system was used for data collection. Demographic, clinical, genetic, and laboratory data and follow-up information of the patients were evaluated. Results: The median age of patients at presentation was 1.5 years, and 50% were females. Genetic analysis showed microdeletion in 69.2%, uniparental disomy in 11.5%, imprinting defect in 1.9% and methylation abnormality in 17.3%. Hypotonia (55.7%), feeding difficulties (36.5%) and obesity (30.7%) were the most common complaints. Cryptorchidism and micropenis were present in 69.2% and 15.3% of males, respectively. At presentation, 25% had short stature, 44.2% were obese, 9.6% were overweight and 17.3% were underweight. Median age of obese patients was significantly higher than underweight patients. Central hypothyroidism and adrenal insufficiency were present in 30.7% and 4.7%, respectively. Hypogonadism was present in 75% at normal age of puberty. GH treatment was started in 40% at a mean age of 4.7±2.7 years. After two years of GH treatment, a significant increase in height SDS was observed. However, body mass index (BMI) standard deviation (SDS) remained unchanged. Conclusion: The most frequent complaints were hypotonia and feeding difficulty at first presentation. Obesity was the initial finding in 44.2%. GH treatment was started in less than half of the patients. While GH treatment significantly increased height SDS, BMI SDS remained unchanged, possibly due to the relatively older age at GH start. Keywords: Prader-Willi syndrome, endocrine dysfunction, growth hormone treatment, body compositio

Obez gençlerde yeme tutumlarının değerlendirilmesi

Obesity is an important health problem. There are limited number of studies about eating disorders in obese children and these studies are generally focused to binge eating disorders. the aim of this study is to investigate the eating attitudes of obese adolescents. the study sample consisted of a clinical group of 30 obese adolescents, a non-clinical obese group of 30 obese adolescents and control group of non-obese adolescents. Psychological assessments were administered using a non-structured psychiatric interview and Eating Attitude Test (EAT). There was no difference between the groups according to gender and age. the mean age of the adolescents was 13.8+1.3. 53.3% were girls and 46.7% were boys. the mean total scores of EAT of the obese groups were significantly higher than the non obese group. the mean total score of EAT of the clinically obese group was significantly higher than the non-clinically obese group. These results suggest that obese adolescents reveal a higher ratio of eating attitudes than non obese adolescents. Findings provided that clinically obese group has more eating problems than non-clinically obese patients.Çocuk ve gençlerde görülme sıklığı gittikçe artan, obez hastalarda, yeme bozukluklarını araştıran çalışmaların sayısı oldukça kısıtlı olup bu konuda en çok vurgulanan tıkınırcasına yeme bozukluğudur. Bu çalışmanın amacı obez gençlerde yeme tutumlarının araştırılmasıdır. Ekzojen obezite tanısı konmuş 12-15 yaş arasında 60 (otuz tanesi tedavi için hastaneye başvurmuş, otuz tanesi toplum ömekleminden seçilmiş) ve kontrol amacıyla normal kiloda 30 genç çalışma kapsamına alınmıştır. Hastaların psikiyatrik muayeneleri yapılmış ve yeme tutum testi verilmiştir. Gruplar arasında yaş ve cinsiyet açısından bir fark olmayıp, yaş ortalaması 13.8 ± 1.3'dür. Olguların %53.3'ü kız, %46,7'si erkektir. Obez gençlerde yeme tutumu testi puanı kontrol grubundan daha yüksek bulunmuştur (f=10.9, p=0.0001). Psikiyatrik muayene sonucunda obezlerde %6.7 oranında tıkınırcasına yeme bozukluğuna rastlanmıştır. Obez gereçlerde yeme tutumu sorunlarına daha fazla rastlanması ve tıkınırcasına yeme bozukluğu görülme oranlarının toplum ömekleminden yüksek oranda olması, bu grupta yeme bozukluklarının araştırması ve bu alanda psikiyatrik destek verilmesinin önemini göstermiştir