Etiology and clinical features in childhood panhypopituitarism


Döğer E., Öner G., Bideci A.

60th Annual Meeting of the European Society for Paediatric Endocrinology, Rome, Italy, 15 - 17 September 2022, vol.95, no.2, pp.361

  • Publication Type: Conference Paper / Summary Text
  • Volume: 95
  • City: Rome
  • Country: Italy
  • Page Numbers: pp.361
  • Gazi University Affiliated: Yes

Abstract

Introduction: Panhypopituitarism is a deficiency of two or more pituitary hormones. Etiological causes of panhypopituitarism include pituitary developmental disorders, traumatic damage, inflammatory and neoplastic diseases affecting the pituitary, and vascular disorders. In this very rare condition, symptoms and signs related to anterior and posterior pituitary hormone deficiency can also be seen, as well as symptoms and findings related to etiological factors causing panhypopituitarism. Aim: In our study, it was aimed to determine the etiological causes and clinical features of the cases followed for panhypopituitarism. Method: The files of the patients with panhypopituitarism who were followed up in Gazi University Faculty of Medicine, Pediatric Endocrinology outpatient clinic between 2009 and 2022 were retrospectively analyzed. Etiological and clinical features of the cases were evaluated. The datas were evaluated with the SPSS 26 program. Results: 55 cases, 21 male (56.4%) and 24 female (43.6%), were included in the study. The age at diagnosis of the cases was 8.4±4.7 (0.1-17.5) years. 71% of the cases were prepubertal. The presenting symptoms were 39.9% vision loss, 38.2% headache, 21.8% polyuria, 18.2% polydipsia, 14.5% short stature, 10.9% thyroid dysfunction, 9.1% hypoglycemia, 5.5% vomiting, 3.6% cholestasis. The etiologic cause was intracranial mass in 71% of the cases. Intracranial masses; It consisted of 43.6% craniopharyngiomas, 18.1% tumors, and 9.1% pituitary adenomas. Central hypothyroidism in 89.1% of cases, central adrenal insufficiency in 78.2%, growth hormone deficiency in 74.5%, diabetes insipidus in 58.2%, hypogonadotropic hypogonadism in 52%, 10.9% of cases low prolactin was detected. In cases of congenital panhypopituitarism, the presenting symptoms were hypoglycemia and/or cholestasis. Genetic mutation was detected in 5 of the cases with pituitary developmental disorder. Discussion and Conclusion: Hypopituitarism in childhood may have either a congenital or an acquired basis. Craniopharyngiomas and intracranial tumors and traumas are among the most common causes of panhypopituitarism in children. Among the rarer causes, genetic disorders leading to deficiency of transcription factors involved in pituitary development are seen. In our study, craniopharyngiomas were the most common cause in the etiology of cases with panhypopituitarism, which was consistent with the literature. The most common reasons for admission were headache and vision loss. Pituitary masses should be kept in mind in cases with headache and visual disturbances in the pediatric age group and the cases should be examined in terms of pituitary insufficiency. Congenital panhypopituitarism should be considered in infants with hypoglycemia and cholestasis.