Comprehensive Insights Into Pediatric Craniopharyngioma: Endocrine and Metabolic Profiles, Treatment Challenges, and Long-term Outcomes from a Multicenter Study

Objective: 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. 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.

Evaluation of quality of life in children with precocious puberty.

OBJECTIVE: This study aimed to investigate the quality of life of patients with central precocious puberty (CPP) who required treatment and premature thelarche (PT) followed up without treatment and to compare the groups with and without treatment among themselves and with healthy children. DESIGN, PATIENTS AND MEASUREMENT: This study is designed as a case-control study. A total of 193 children including 59 children with CPP, 53 children with PT, 81 healthy children and their parents were included in the study. A questionnaire was applied to evaluate the sociodemographic characteristics that would affect the quality of life. The 'Pediatric Quality of Life Inventory (PedsQL)' was used to assess the quality of life. RESULTS: The PedsQL total scale score was 78.10 ± 17.13, 79.35 ± 11.54 and 79.52 ± 14.65, the psychosocial health summary score was 78.86 ± 16.83, 79.40 ± 12.54 and 79.94 ± 14.94 and physical health summary score was 75.81 ± 20.69, 79.41 ± 15.04 and 78.25 ± 17.52 in CPP, PT and control groups, respectively; however, there was no statistical difference (p > .05). In the scale administered to the parents, scores were similar in the three groups. No difference was found between CPP, PT and control groups in terms of sociodemographic data in the study (p > .05). CONCLUSION: Unlike previous studies, in this study the effects of sociodemographic characteristics and whether treatment was initiated or not on quality of life were investigated. Although the scale scores of the CPP group were lower than the PT and control group, there was no statistically significant difference, indicating that quality of life was not negatively affected in the CPP group receiving treatment.

A Challenging Case of Ectopic ACTH Syndrome with Bronchial Carcinoid and Literature Review.

Here we report an adolescent boy diagnosed with ectopic ACTH (Adrenocorticotropin hormone) syndrome (EAS) caused by atypical bronchial carcinoid. The patient was evaluated multidisciplinaryly: he had surgery and took chemotherapy and radiotherapy treatments afterward. The patient is still under our follow-up. Until today eighteen pediatric and adolescent patients with EAS because of bronchial carcinoid tumors were reported in 13 case reports and literature reviews. Ectopic ACTH syndrome caused by bronchial carcinoids is very rare in children and adolescents. Careful diagnostic evaluation and rapid treatment should be started immediately. Although complete remission is possible in bronchial carcinoids, atypical carcinoids have a more aggressive nature. A multidisciplinary approach and follow-up will improve quality of life and survival.

Approach to Rickets: Is It Calciopenic or Phosphopenic?

Rickets is a childhood disorder of decreased mineralization of bone tissue. It is either calciopenic or phosphopenic, according to the deficient mineral. Calcium, phosphate, and vitamin D metabolism should be known to understand the pathophysiology of rickets. A deficiency of calcium or vitamin D can be caused by several conditions. These conditions lead to defective osteoid mineralization, impaired chondrocyte differentiation, and apoptosis in the growth plate, resulting in clinical and radiological findings of rickets. Rickets developing as a result of vitamin D deficiency is the most frequently encountered form. Vitamin D-dependent rickets classification is made according to genetic abnormalities of enzymes that are involved in vitamin D metabolism. Phosphopenic rickets is divided mainly into 2 categories that are FGF23 related or not. A systemic approach that includes a detailed history, physical examination, and laboratory evaluation is required when performing a diagnostic evaluation. Vitamin D and calcium supplementation should be used to treat nutritional rickets. To prevent rickets and its morbidities, vitamin D prophylaxis in the newborn period is suggested. High dose of vitamin D3, 1.25(OH)2D, and calcium are treatment choices in vitamin D-dependent rickets according to its subgroup. If conventional treatment consisting of phosphate and calcitriol is ineffective in the treatment of phosphopenic rickets, Burosumab is the new treatment option.

Clinical and demographic characteristics of children and adolescents with acute vertigo symptoms: A cross-sectional study

Background/aim: Vertigo is one of the rarely diagnosed disorders during childhood due to insufficient description of the children regarding their experiences to the physicians. The clinical features of children and adolescents admitted by acute vertigo symptoms were investigated to elaborate the subject retrospectively. Materials and methods: BetweenJanuary 2017­July 2019, records of cases admitted with acute vertigo complaints to pediatric neurology were retrospectively examined. Results: Of 761 patients, mean age was 13.8 years, 64% (n = 487) were women, 22.6% (n = 172) of which were children (1­11 years). A total of 37.3% of the cases (n = 284) had unknown etiology of acute vertigo symptoms, 39.6% (n = 301) had acute vertigo, and 23.1% (n = 176) were considered with no organicity problems but a group of the families stopped cooperating to the full extent in the study. Among all the patients, 25.6% (195/761) had paroxymal vertigo, 6.8% (52/761) had migraine-associated vertigo, 4.5% (34/761) had psychogenic vertigo, and 2.6% (20/761) had epileptic vertigo. Epileptic vertigo was significantly higher in younger children (mean age = 10.6, F(3) = 8874, P < .001), and the ratio of its occurence was also higher among children (60%, χ2 (3) = 20.347, P < .001). Conclusion: Vertigo complaints are 1.7 times more common among the girls. Epileptic vertigo is significantly higher among the children. Among younger children, it seems important to consider epilepsy when vertigo emerged.