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Cholestasis Reveals Severe Cortisol Deficiency in Neonatal Pituitary Stalk Interruption Syndrome
- Source :
- PLoS ONE, Vol 11, Iss 2, p e0147750 (2016), PLoS ONE
- Publication Year :
- 2016
- Publisher :
- Public Library of Science (PLoS), 2016.
-
Abstract
- Objectives Cholestasis has been reported during the course of congenital hypothalamic-pituitary deficiency, but crucial information is lacking regarding both its origin and prognosis. We aimed to characterize the course of cholestasis and factors contributing to it in patients with deficiency due to pituitary stalk interruption syndrome (PSIS). Methods We conducted a retrospective single-center, case-cohort study including 16 patients with PSIS diagnosed before one year of age. We collected clinical and biological parameters from medical records and compared the characteristics of the endocrine syndrome in PSIS patients with and without cholestasis. Results 5/16 patients had cholestasis, all with a neonatal onset and multiple hypothalamic-pituitary deficiency. Patients with cholestasis presented with lower Apgar score and higher rate of ophthalmic malformations: 3/5 vs 1/11, p = 0.03 and 5/5 vs 4/11, p = 0.02, respectively. The plasma cortisol level was strongly decreased in patients with cholestasis: 12.4 ng/mL (8–15 ng/mL) vs 79.4 ng/mL (10–210 ng/mL), p = 0.04. Cholestasis resolved within 9 months following hormone supplementation. No development of chronic liver disease was observed during a median follow-up of 9.4 years (range, 1.3–13.3 years). Conclusions Cholestasis is a frequent symptom at presentation of PSIS during the neonatal period that may help earlier diagnosis and that indicates a profound cortisol deficiency.
- Subjects :
- Pituitary gland
Hydrocortisone
Physiology
lcsh:Medicine
Growth hormone
Biochemistry
Nervous System
Pediatrics
Families
0302 clinical medicine
Risk Factors
Cryptorchidism
Medicine and Health Sciences
Morphogenesis
Bile
Lipid Hormones
Gamma-glutamyltransferase
lcsh:Science
Children
Cholestasis
Multidisciplinary
biology
Syndrome
gamma-Glutamyltransferase
Body Fluids
Treatment Outcome
medicine.anatomical_structure
Pituitary Gland
Anatomy
Research Article
medicine.drug
medicine.medical_specialty
Pituitary Stalk Interruption Syndrome
Endocrine System
030209 endocrinology & metabolism
Hypoglycemia
Bile Acids and Salts
03 medical and health sciences
Diagnostic Medicine
030225 pediatrics
Internal medicine
Congenital Disorders
medicine
Humans
In patient
Birth Defects
Steroid Hormones
business.industry
lcsh:R
Infant, Newborn
Infant
Biology and Life Sciences
Bilirubin
medicine.disease
Hormones
Neuroanatomy
Endocrinology
Age Groups
Metabolic Disorders
People and Places
biology.protein
Population Groupings
lcsh:Q
business
Neuroscience
Developmental Biology
Subjects
Details
- Language :
- English
- ISSN :
- 19326203
- Volume :
- 11
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- PLoS ONE
- Accession number :
- edsair.doi.dedup.....81c448ee676f7e692de7c86853c1316f