60th Annual Meeting of the European Society for Paediatric Endocrinology, Rome, İtalya, 15 - 17 Eylül 2022, cilt.95, sa.2, ss.361
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.