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

Author

Zeynep Şıklar, Elif Özsu, Sirmen Kızılcan Çetin, Samim Özen, Filiz Çizmecioğlu-jones, Hanife Gül Balkı, Zehra Aycan, Damla Gökşen, Fatih Kilci, Sema Nilay Abseyi, Ummahan Tercan, Gözde Gürpınar, Şükran Poyrazoğlu, Feyza Darendeliler, Korcan Demir, Özge Besci, İlker Tolga Özgen, Semra Bahar Akın, Zümrüt Kocabey Sütçü, Emel Hatun Aykaç Kaplan, Emine Çamtosun, İsmail Dündar, Elif Sağsak, Hüseyin Anıl Korkmaz, Ahmet Anık, Gül Yeşiltepe Mutlu, Bahar Özcabı, Ahmet Uçar, Aydilek Dağdeviren Çakır, Beray Selver Eklioğlu, Birgül Kırel, and Merih Berberoğlu

Subjects
craniopharyngioma, pituitary, dysfunction, Pediatrics, RJ1-570, Diseases of the endocrine glands. Clinical endocrinology, RC648-665
Abstract

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.

Published
2024
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2. Prediction of Transient or Permanent Congenital Hypothyroidism

Author

Ferda Evin, Hanife Gül Balkı, Aysun Ata, Eren Er, Zeynep Vatansever, Samim Özen, Damla Gökşen, and Şükran Darcan

Subjects
Congenital hypothyroidism, dyshormonogenesis, screening, Diagnosis, transient hypothyroidism, thyroid dysgenesis, permanent hypothyroidism
Abstract

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

Published
2022

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