Search

INTRODUCTION: Craniopharyngiomas (CPG) have complex treatment challenges due to their proximity to vital structures, surgical and radiotherapeutic complexities, and the tendency for recurrence. The aim of this study was to identify the prevalence of endocrine and metabolic comorbidities observed during initial diagnosis and long-term follow-up in a nationwide cohort of pediatric CPG patients. A further aim was to highlight the difficulties associated with CPG management. METHODS: Sixteen centers entered CPG patients into the ÇEDD NET data system. The clinical and laboratory characteristics at presentation, administered treatments, accompanying endocrine, metabolic, and other system involvements, and the patient’s follow-up features were evaluated. RESULTS: Of the 152 evaluated patients, 64 (42.1%) were female. At presentation, the mean age was 9.1±3.67, ranging from 1.46 to 16.92, years. The most common complaints at presentation were headache (68.4%), vision problems (42%), short stature (15%), and nausea and vomiting (7%). The surgical procedures were gross total resection (GTR) in 97 (63.8%) and subtotal resection in 55 (36.2%). Radiotherapy (RT) was initiated in 11.8% of the patients. Histopathological examination reported 92% were adamantinamatous type and 8% were papillary type. Postoperatively, hormone abnormalities consisted of thyroid-stimulating hormone (92.1%), adrenocorticotropic hormone (81%), antidiuretic hormone (79%), growth hormone (65.1%), and gonadotropin (43.4%) deficiencies. Recombinant growth hormone treatment (rhGH) was initiated in 27 (17.8%). The study showed hesitancy among physicians regarding rhGH. The median survival without relapse was 2.2 years. Median (range) time of relapse was 1.82 (0.13-10.35) years. Relapse was related to longer followups and reduced GTR rates. The median follow-up time was 3.13 years. Among the last follow-up visits, the prevalence of obesity was 38%, but of these, 46.5% were already obese at diagnosis. However, 20% who were not obese at baseline became obese on follow-up. Permanent visual impairment was observed in 26 (17.1%), neurological deficits in 13 (8.5%) and diabetes mellitus in 5 (3.3%) patients. DISCUSSION AND CONCLUSION: Recurrence was predominantly due to incomplete resection and the low rate of postoperative RT. Challenges emerged for multidisciplinary regular follow ups. It is suggested that early interventions, such as dietary restrictions and increased exercise to prevent obesity, be implemented.

Aim:Congenital hypothyroidism (CH) is one of the most common endocrinological problems in the neonatal period. CH, which occurs in 1:3,000-4,000 births, is a preventable cause of mental retardation. In the literature, the most common cause of CH is dysgenesis of the thyroid gland, followed by thyroid dyshormonogenesis. We aimed to determine the prevalence of permanent (P-CH) and transient (T-CH) CH in cases followed up with a diagnosis of CH and to identify a prediction method for persistency.Materials and Methods:We retrospectively analyzed the medical records of 105 children with CH. TSH levels in a screening program, TSH and fT4 levels of the first venous sample, 10-15th day of treatment, monthly in the first 6 months, 2-3 monthly until 18th months, and 6-monthly until 3 years of age were recorded. L-T4 doses (per microgram per body weight) in each visit were also recorded.Results:From the 105 children (58 males, 47 females) enrolled in this study, 38 (36.2 %) were referred from the National Newborn Screening Program. Treatment was discontinued during the 3rd year follow-up period in 44 (41.9 %) of the cases with normal thyroid gland morphology. L-thyroxine (L-T4) therapy was discontinued at a median age of 1.9+-1.08 years.TSH levels at the time of diagnosis were higher in the P-CH group, but this difference was not statistically significant (p=0.165). At the sixth month, first and second years of follow-up, L-T4 doses were significantly higher in the P-CH than in the T-CH group (p

Apricot kernels contain a significant amount of amygdaline, one of the cyanogenic glycosides. In Turkey, most cases of cyanide intoxication in children are related to ingestion of apricot kernels. In this manuscript, we present two pediatric cases of cyanide intoxication presenting with vomiting and loss of consciousness after ingestion of unknown amount of fresh apricot kernels. Two patients were discharged with cure after supportive treatment without the need for cyanide antidote administration. Our report of two cases aims to raise awareness amongst the general population and health workers of the potential risk of cyanide poisoning associated with the consumption of apricot kernels. Cyanide poisoning should be kept in mind for patients with sudden loss of consciousness, high anion gap metabolic acidosis and lactic acidosis.

(1) Background: This study aimed to determine whether standard-dose vitamin D supplementation could provide adequate levels in exclusively breastfed infants born with different cord 25(OH)D levels and to evaluate related neonatal morbidities. (2) Methods: A prospective cross-sectional study was designed in term infants. Three groups were formed based on cord 25(OH)D levels; Group1 Deficiency:25(OH)D < 12 ng/mL, Group2 Insufficiency:25(OH)D = 12–19 ng/mL, and Group3 Optimum:25(OH)D = 20–100 ng/mL. Cord and 1st month 25(OH)D levels, after receiving standard-dose vitamin D prophylaxis, neonatal outcomes and anthropometric measurements were compared. (3) Results: The study involved 86 infants. Group1 Deficiency had also significantly lower 25(OH)D levels at 1st month compared to the others (p < 0.001). There was a significant positive correlation between cord and 1st month 25(OH)D levels (r = 0.78, p < 0.001). Despite the fact that the mother’s age and clothing style were similar, Group1 Deficiency mothers had higher parity numbers and used less vitamin D during pregnancy (p = 0.03, p = 0.04). Neonatal outcomes analysis revealed that newborns in Group1 Deficiency experienced more respiratory distress, transient tachypnea, and early-onset sepsis, as well as more and longer hospital stays in the first-month of life (p < 0.05). (4) Conclusions: Infants with low cord 25(OH)D levels had also lower 25(OH)D values in the 1st month of life and experienced higher rates of neonatal morbidities. Given its numerous biological activities and impact on neonatal morbidities, determining an individualized dose of vitamin D supplementation may be more accurate than using the standard approach.