Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.

Abstract:

:Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome. Management usually includes resection of the primary tumor with or without additional medical treatment, but manifestations may persist after resolution of hypercortisolemia.

journal_name

Clin Perinatol

journal_title

Clinics in perinatology

authors

Tatsi C,Stratakis CA

doi

10.1016/j.clp.2017.10.002

subject

Has Abstract

pub_date

2018-03-01 00:00:00

pages

103-118

issue

1

eissn

0095-5108

issn

1557-9840

pii

S0095-5108(17)30102-1

journal_volume

45

pub_type

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