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

Joanna A. Chrzanowska; Agnieszka Zubkiewicz-Kucharska; Monika Seifert; Aleksander Całkosiński; Anna Noczyńska

doi:10.5114/pedm.2021.109129

eISSN: 2083-8441
ISSN: 2081-237X

Pediatric Endocrinology Diabetes and Metabolism

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abstract:

Original paper

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

Joanna A. Chrzanowska

¹

,

Agnieszka Zubkiewicz-Kucharska

¹

,

Monika Seifert

¹

,

Aleksander Całkosiński

²

,

Anna Noczyńska

¹

Department of Endocrinology and Diabetology for Children and Adolescents, Wroclaw Medical University, Poland
Student Scientific Club at Department of Endocrinology and Diabetology for Children and Adolescents, Wroclaw Medical University, Poland

Pediatr Endocrinol Diabetes Metab 2021; 27 (4): 272–282

DOI: https://doi.org/10.5114/pedm.2021.109129

Online publish date: 2021/12/30

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AMA

A. Chrzanowska J, Zubkiewicz-Kucharska A, Seifert M, Całkosiński A, Noczyńska A. 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. Pediatric Endocrinology Diabetes and Metabolism. 2021;27(4):272-282. doi:10.5114/pedm.2021.109129.

APA

A. Chrzanowska, J., Zubkiewicz-Kucharska, A., Seifert, M., Całkosiński, A.,  & Noczyńska, A. (2021). 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. Pediatric Endocrinology Diabetes and Metabolism, 27(4), 272-282. https://doi.org/10.5114/pedm.2021.109129

Chicago

A. Chrzanowska, Joanna, Agnieszka Zubkiewicz-Kucharska, Monika Seifert, Aleksander Całkosiński,  and Anna Noczyńska. 2021. "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". Pediatric Endocrinology Diabetes and Metabolism 27 (4): 272-282. doi:10.5114/pedm.2021.109129.

Harvard

A. Chrzanowska, J., Zubkiewicz-Kucharska, A., Seifert, M., Całkosiński, A.,  and Noczyńska, A. (2021). 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. Pediatric Endocrinology Diabetes and Metabolism, 27(4), pp.272-282. https://doi.org/10.5114/pedm.2021.109129

MLA

A. Chrzanowska, Joanna et al. "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." Pediatric Endocrinology Diabetes and Metabolism, vol. 27, no. 4, 2021, pp. 272-282. doi:10.5114/pedm.2021.109129.

Vancouver

A. Chrzanowska J, Zubkiewicz-Kucharska A, Seifert M, Całkosiński A, Noczyńska A. 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. Pediatric Endocrinology Diabetes and Metabolism. 2021;27(4):272-282. doi:10.5114/pedm.2021.109129.

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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.

keywords:

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

Joanna A. Chrzanowska 1 , Agnieszka Zubkiewicz-Kucharska 1 , Monika Seifert 1 , Aleksander Całkosiński 2 , Anna Noczyńska 1

short stature, pituitary stalk interruption syndrome, combined pituitary hormone deficiency

Joanna A. Chrzanowska

¹

,

Agnieszka Zubkiewicz-Kucharska

¹

,

Monika Seifert

¹

,

Aleksander Całkosiński

²

,

Anna Noczyńska

¹