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Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children

INTRODUCTION: The pituitary stalk interruption syndrome (PSIS) is one of the complex –forms of congenital pituitary insufficiency. Symptoms resulting from insufficiency of the pituitary gland, in spite of the inborn character of the disease, may appear at various stages of life. The aim of this pape...

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Autores principales: Chrzanowska, Joanna A., Zubkiewicz-Kucharska, Agnieszka, Seifert, Monika, Całkosiński, Aleksander, Noczyńska, Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10226364/
https://www.ncbi.nlm.nih.gov/pubmed/35114769
http://dx.doi.org/10.5114/pedm.2021.109129
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author Chrzanowska, Joanna A.
Zubkiewicz-Kucharska, Agnieszka
Seifert, Monika
Całkosiński, Aleksander
Noczyńska, Anna
author_facet Chrzanowska, Joanna A.
Zubkiewicz-Kucharska, Agnieszka
Seifert, Monika
Całkosiński, Aleksander
Noczyńska, Anna
author_sort Chrzanowska, Joanna A.
collection PubMed
description INTRODUCTION: The pituitary stalk interruption syndrome (PSIS) is one of the complex –forms of congenital pituitary insufficiency. Symptoms resulting from insufficiency of the pituitary gland, in spite of the inborn character of the disease, may appear at various stages of life. The aim of this paper was to present clinical presentation in 31 patients with PSIS confirmed radiologically. RESULTS: In the whole study population during first examination 25.8% children were diagnosed with combined pituitary hormone deficiency (CPHD). During the endocrinological observation (median follow-up 5.1 years, range 0.513.2) of the above-mentioned group 74.2% subjects were diagnosed with CPHD, while 25.8% patients with isolated growth hormone deficiency (GHD). Two children with initially short stature were confirmed with GHD. As a result of the parents' decision, growth hormone therapy was either not started or discontinued. During further follow-up, however, the children achieved normal height. CONCLUSIONS: Children with PSIS present a diverse clinical picture and should be observed because of the risk of further pituitary disorders. In the differential diagnosis of hypoglycemia in the neonatal period and in infancy, hypopituitarism should be considered. The phenomenon of normal growth in patients with confirmed growth hormone deficiency has been observed, although is not fully understood.
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spelling pubmed-102263642023-06-05 Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children Chrzanowska, Joanna A. Zubkiewicz-Kucharska, Agnieszka Seifert, Monika Całkosiński, Aleksander Noczyńska, Anna Pediatr Endocrinol Diabetes Metab Original paper | Praca oryginalna INTRODUCTION: The pituitary stalk interruption syndrome (PSIS) is one of the complex –forms of congenital pituitary insufficiency. Symptoms resulting from insufficiency of the pituitary gland, in spite of the inborn character of the disease, may appear at various stages of life. The aim of this paper was to present clinical presentation in 31 patients with PSIS confirmed radiologically. RESULTS: In the whole study population during first examination 25.8% children were diagnosed with combined pituitary hormone deficiency (CPHD). During the endocrinological observation (median follow-up 5.1 years, range 0.513.2) of the above-mentioned group 74.2% subjects were diagnosed with CPHD, while 25.8% patients with isolated growth hormone deficiency (GHD). Two children with initially short stature were confirmed with GHD. As a result of the parents' decision, growth hormone therapy was either not started or discontinued. During further follow-up, however, the children achieved normal height. CONCLUSIONS: Children with PSIS present a diverse clinical picture and should be observed because of the risk of further pituitary disorders. In the differential diagnosis of hypoglycemia in the neonatal period and in infancy, hypopituitarism should be considered. The phenomenon of normal growth in patients with confirmed growth hormone deficiency has been observed, although is not fully understood. Termedia Publishing House 2021-12-30 2021-12 /pmc/articles/PMC10226364/ /pubmed/35114769 http://dx.doi.org/10.5114/pedm.2021.109129 Text en Copyright © Polish Society of Pediatric Endocrinology and Diabetes https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs (CC BY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) ), allowing third parties to download and share its works but not commercially purposes or to create derivative works.
spellingShingle Original paper | Praca oryginalna
Chrzanowska, Joanna A.
Zubkiewicz-Kucharska, Agnieszka
Seifert, Monika
Całkosiński, Aleksander
Noczyńska, Anna
Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title_full Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title_fullStr Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title_full_unstemmed Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title_short Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children
title_sort clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. the unexpected growth without growth hormone in 2 children
topic Original paper | Praca oryginalna
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10226364/
https://www.ncbi.nlm.nih.gov/pubmed/35114769
http://dx.doi.org/10.5114/pedm.2021.109129
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