Pituitary Stalk Interruption Syndrome from Infancy to Adulthood: Clinical, Hormonal, and Radiological Assessment According to the Initial PresentationReport as inadecuate




Pituitary Stalk Interruption Syndrome from Infancy to Adulthood: Clinical, Hormonal, and Radiological Assessment According to the Initial Presentation - Download this document for free, or read online. Document in PDF available to download.

Background

Patients with pituitary stalk interruption syndrome PSIS are initially referred for hypoglycemia during the neonatal period or growth retardation during childhood. PSIS is either isolated nonsyndromic or associated with extra-pituitary malformations syndromic.

Objective

To compare baseline characteristics and long-term evolution in patients with PSIS according to the initial presentation.

Study Design

Sixty-seven patients with PSIS were included. Data from subgroups were compared: neonates n = 10 versus growth retardation patients n = 47, and syndromic n = 32 versus nonsyndromic patients n = 35.

Results

Neonates displayed a more severe hormonal and radiological phenotype than children referred for growth retardation, with a higher incidence of multiple hormonal deficiencies 100% versus 34%; P = 0.0005 and a nonvisible anterior pituitary lobe 33% versus 2%; P = 0.0017. Regular follow-up of growth might have allowed earlier diagnosis in the children with growth retardation, as decreased growth velocity and growth retardation were present respectively 3 and 2 years before referral. We documented a progressive worsening of endocrine impairment throughout childhood in these patients. Presence of extra-pituitary malformations found in 48% was not associated with more severe hormonal and radiological characteristics. Growth under GH treatment was similar in the patient groups and did not vary according to the pituitary MRI findings.

Conclusions

PSIS diagnosed in the neonatal period has a particularly severe hormonal and radiological phenotype. The progressive worsening of endocrine impairment throughout childhood justifies periodic follow-up to check for additional hormonal deficiencies.



Author: Céline Bar, Charline Zadro, Gwenaelle Diene, Isabelle Oliver, Catherine Pienkowski, Béatrice Jouret, Audrey Cartault, Zeina Aja

Source: http://plos.srce.hr/



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