Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency

Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency...

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Main Authors: Kyung Mi Jang, Cheol Woo Ko
Format: Article
Language:English
Published: Korean Society of Pediatric Endocrinology 2017-09-01
Series:Annals of Pediatric Endocrinology & Metabolism
Subjects:
Online Access:http://e-apem.org/upload/pdf/apem-2017-22-3-208.pdf
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author Kyung Mi Jang
Cheol Woo Ko
author_facet Kyung Mi Jang
Cheol Woo Ko
author_sort Kyung Mi Jang
collection DOAJ
description Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.
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spelling doaj.art-49c19b5bb43742aabe09b318ec61c2172022-12-21T21:58:26ZengKorean Society of Pediatric EndocrinologyAnnals of Pediatric Endocrinology & Metabolism2287-10122287-12922017-09-0122320821210.6065/apem.2017.22.3.208704Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiencyKyung Mi Jang0Cheol Woo Ko1 Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea Department of Pediatric Endocrinology, Kyungpook National University Children’s Hospital, Daegu, KoreaPituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.http://e-apem.org/upload/pdf/apem-2017-22-3-208.pdfPituitary stalk interruption syndromeHyponatremiaHypopituitarismInappropriate ADH syndromeAdrenal insufficiency
spellingShingle Kyung Mi Jang
Cheol Woo Ko
Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
Annals of Pediatric Endocrinology & Metabolism
Pituitary stalk interruption syndrome
Hyponatremia
Hypopituitarism
Inappropriate ADH syndrome
Adrenal insufficiency
title Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
title_full Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
title_fullStr Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
title_full_unstemmed Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
title_short Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
title_sort delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency
topic Pituitary stalk interruption syndrome
Hyponatremia
Hypopituitarism
Inappropriate ADH syndrome
Adrenal insufficiency
url http://e-apem.org/upload/pdf/apem-2017-22-3-208.pdf
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AT cheolwooko delayeddiagnosisofpituitarystalkinterruptionsyndromewithsevererecurrenthyponatremiacausedbyadrenalinsufficiency