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2015 ; 10
(11
): e0142354
Nephropedia Template TP
gab.com Text
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English Wikipedia
Pituitary Stalk Interruption Syndrome from Infancy to Adulthood: Clinical,
Hormonal, and Radiological Assessment According to the Initial Presentation
#MMPMID26562670
Bar C
; Zadro C
; Diene G
; Oliver I
; Pienkowski C
; Jouret B
; Cartault A
; Ajaltouni Z
; Salles JP
; Sevely A
; Tauber M
; Edouard T
PLoS One
2015[]; 10
(11
): e0142354
PMID26562670
show ga
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.