Human chorionic gonadotrophin secreting adrenocortical neoplasm presenting with peripheral precocious puberty in an infant.

Adrenocortical tumor (ACT) is a rare malignant tumor which usually present with Cushing syndrome and virilization. Paraneoplastic syndromes (PNS) due to neoplasms can occur with peptides or cytokines secreted by the tumor. Here, we report a 13-month-old-male presented with severe masculinization. He had signs of precocious puberty with enlarged testicles, very high testosterone levels but low levels of gonadotrophins, and elevated ß-hCG. He underwent a left nephrectomy. The histopathological evaluation revealed a diagnosis of adrenocortical neoplasm. The levels of androgens and ß-hCG normalized after the resection of tumor, and the clinical findings improved within few months. We report the first pediatric patient with peripheral precocious puberty due to an ACT that secretes ß-hCG as PNS. A ß-hCG secreting ACT can cause severe virilization due to increased gonadal androgens stimulated by ß-hCG as well as due to increased adrenal androgens from the tumor.

Familial early-onset obesity in Turkish children: variants and polymorphisms in the melanocortin-4 receptor (MC4R) gene.

OBJECTIVES: Genetic factors have a key role in childhood obesity with higher rates in children than adults. Among the monogenic types of non-syndromic obesity, melanocortin-4 receptor (MC4R) deficiency is the most frequent cause. Beside pathogenic variants, single-nucleotide polymorphisms in MC4R gene are also associated with lower energy expenditure. The aim of this study was to estimate the frequency of MC4R variants and polymorphisms in a cohort of Turkish children and adolescents with severe early-onset obesity, and to understand the clinical features of patients. METHODS: Patients, 1-17 years of age, with the onset of obesity before 10 years of age and a body mass index (BMI) standard deviation score (SDS) of >2.3, and who had a family history of early-onset obesity in at least one of their first-degree relatives were included in the study. Beside routine blood tests genetic analyses for MC4R gene were performed. RESULTS: Analyses of MC4R revealed previously known variations in three (3.5%) patients, and pathogenic polymorphisms related with obesity in four (4.7%) patients. BMI SDS values were between 2.8 and 5.5 SDS in the pathogenic variant carrier group, and 2.8-4.9 SDS in the polymorphism group. Mean BMI SDS in variant-negative group was 3.4 ± 0.82. CONCLUSIONS: Investigation of the MC4R in individuals with early-onset obesity and presence of obesity first-degree relatives is important. Hypertension is a rare comorbidity compared to other causes. Contrary to studies reporting that insulin resistance was absent or very rare, we found it as a frequent finding in both pathogenic variants and polymorphisms of MC4R.

Pediatricians' attitudes in management of acute otitis media and ear pain in Turkey.

OBJECTIVES: Acute otitis media (AOM) is predominantly a disease of childhood and one of the common reasons for prescribing antibiotics. Ear pain is the main symptom of AOM, with the result that parents frequently seek immediate medical assistance for their children. Antibiotic therapy for AOM does not provide symptomatic relief in the first 24 hours, and analgesics are commonly recommended for relieving the pain associated with AOM. The aims of the present study were to assess pediatricians' attitudes toward AOM and ear pain management in Turkey. METHODS: This multicenter descriptive questionnaire study was conducted in 20 centers from different geographic locations in Turkey, with 977 pediatricians, between June 2015 and December 2016. The questionnaire comprised 20 questions focusing on the pediatricians' sociodemographic variables, experiences, and treatment related to AOM and ear pain. RESULTS: Of the pediatricians, 58.2% were residents, 36.5% were specialists, and 4.3% were lecturers. Most participants were working in a university hospital (54.8%) or education and research hospital (32.2%). In general daily practice, the AOM diagnosis rates were between 6% and 20% in outpatient clinics, and 52.3% of the participants stated the patients complained about ear pain in pediatric clinics. The watchful waiting (WW) rate, as opposed to immediate antibiotic treatment, was 39.8% for all the pediatricians. The pediatric residents used the WW strategy less than the specialists and lecturers did (p = 0.004). The rates of the WW strategy were higher in outpatient clinics where AOM was commonly diagnosed (p < 0.001). The most common antibiotic prescribed for AOM was amoxicillin clavulanate (76.7%). The mean recommended treatment period for AOM was 9.3 ± 2.2 days. The choices for systemic ear pain treatment were acetaminophen (26.8%), ibuprofen (29.4%), and alternating between ibuprofen and acetaminophen (43.9%). Moreover, 34.6% of the participants recommended topical agents for otalgia. Topical agents were more commonly recommended by the pediatric residents than specialists or lecturers (p < 0.001). Finally, 58.3% of pediatricians had experiences of the parents' usage of a variety of herbal and folk remedies, such as breast milk or olive oil, for their children's ear pain. CONCLUSION: Amoxicillin clavulanate was the most frequently prescribed antibiotic for AOM. WW was approved by the pediatricians, and having more AOM patients was a significant factor in the physicians' choice of WW; nevertheless, the WW rate was poor. Implementation of educational intervention strategies will help pediatricians in improving their compliance with evidence-based guidelines for AOM treatment. Otalgia is taken seriously by parents and pediatricians, and otalgia treatment seems to be well accepted in Turkey for providing symptomatic relief and enhancing the patients' quality of life.