Literature DB >> 16622308

Untreated hypopituitarism due to absence of the pituitary stalk with normal adult height: report of two cases.

Leda Papastathopoulou1, Marinella Tzanela, Vania Vlassopoulou, Dimitra Vassiliadi, Nikolaos Thalassinos.   

Abstract

Patients with congenital multiple pituitary hormones deficiency (MPHD) occasionally present with pituitary stalk interruption and ectopic posterior lobe on magnetic resonance imaging (MRI). Very rarely normal adult height despite growth hormone deficiency (GHD) has been described in these patients. We report two patients with evidence of congenital MPHD, who remained untreated until adulthood. They both failed to develop spontaneous puberty, and they demonstrated very low growth velocity until adulthood when they continued to grow, with a final height of 176 and 169 cm when they sought medical attention in our department at the age of 45 and 33 yr, respectively. At that time a hypoplastic pituitary, absence of pituitary stalk, and ectopic posterior pituitary lobe were found on MRI, and the laboratory investigations, including dynamic tests for pituitary hormone reserve, revealed MPHD with severe GHD. In conclusion, these cases illustrate that very rarely patients with untreated MPHD can reach normal adult height. Some postulations about the pathophysiology of this phenomenon are discussed.

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Year:  2006        PMID: 16622308     DOI: 10.1385/endo:29:1:175

Source DB:  PubMed          Journal:  Endocrine        ISSN: 1355-008X            Impact factor:   3.633


  30 in total

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Journal:  J Clin Endocrinol Metab       Date:  2000-11       Impact factor: 5.958

2.  Pituitary magnetic resonance imaging and function in patients with growth hormone deficiency with and without mutations in GHRH-R, GH-1, or PROP-1 genes.

Authors:  Maria Geralda F Osorio; Suemi Marui; Alexander A L Jorge; Ana C Latronico; Leonard S S Lo; Claudia C Leite; Vivian Estefan; Berenice B Mendonca; Ivo J P Arnhold
Journal:  J Clin Endocrinol Metab       Date:  2002-11       Impact factor: 5.958

Review 3.  The growth without growth hormone syndrome.

Authors:  M E Geffner
Journal:  Endocrinol Metab Clin North Am       Date:  1996-09       Impact factor: 4.741

4.  A study of the growth-promoting and metabolic effects of growth hormone (GH) in a patient with the "growth without GH" syndrome.

Authors:  M Pavlou; A Tsatsoulis; Z Efstathiadou; S Bitsis; Z L Papadopoulou
Journal:  Growth Horm IGF Res       Date:  2001-08       Impact factor: 2.372

5.  Near-normal linear growth in the setting of markedly reduced growth hormone and IGF-1. A case report.

Authors:  M Murashita; T Tajima; J Nakae; N Shinohara; M E Geffner; K Fujieda
Journal:  Horm Res       Date:  1999

6.  Molecular analysis of LHX3 and PROP-1 in pituitary hormone deficiency patients with posterior pituitary ectopia.

Authors:  K W Sloop; E C Walvoord; A D Showalter; O H Pescovitz; S J Rhodes
Journal:  J Clin Endocrinol Metab       Date:  2000-08       Impact factor: 5.958

7.  Dynamic MRI in the congenital agenesis of the neural pituitary stalk syndrome: the role of the vascular pituitary stalk in predicting residual anterior pituitary function.

Authors:  M Maghnie; E Genovese; A Villa; L Spagnolo; R Campan; F Severi
Journal:  Clin Endocrinol (Oxf)       Date:  1996-09       Impact factor: 3.478

8.  Normal growth in a patient with septo-optic dysplasia despite both growth hormone and IGF-I deficiency.

Authors:  A Bereket; C H Lang; M E Geffner; T A Wilson
Journal:  J Pediatr Endocrinol Metab       Date:  1998 Jan-Feb       Impact factor: 1.634

9.  Adult height exceeding target height in a patient with congenital panhypopituitarism diagnosed after the age of 25 years.

Authors:  T Arrigo; G Crisafulli; A Salamone; D Cucinotta; F De Luca
Journal:  J Pediatr Endocrinol       Date:  1994 Jul-Sep

10.  Congenital panhypopituitarism of late onset.

Authors:  P Navarro; I Halperin; C Rodríguez; J M González; J Vidal; E Vilardell
Journal:  J Endocrinol Invest       Date:  1994-05       Impact factor: 4.256

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  3 in total

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Journal:  Pituitary       Date:  2012-09       Impact factor: 4.107

2.  Pituitary stalk interruption syndrome presenting as short stature: a case report.

Authors:  Nanik Ram; Syed Ahsan Ali; Syed Zubair Hussain
Journal:  J Med Case Rep       Date:  2014-12-19

3.  High prevalence of PROP1 gene mutations in Hungarian patients with childhood-onset combined anterior pituitary hormone deficiency.

Authors:  Zita Halász; Judit Toke; Attila Patócs; Rita Bertalan; Zsófia Tömböl; Agnes Sallai; Eva Hosszú; Agota Muzsnai; László Kovács; János Sólyom; György Fekete; Károly Rácz
Journal:  Endocrine       Date:  2006-12       Impact factor: 3.925

  3 in total

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