Improved Behavior and Neuropsychological Function in Children With ROHHAD After High-Dose Cyclophosphamide.

Autor:	Jacobson LA; Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland; and Department of Psychiatry and Behavioral Sciences, and jacobson@kennedykrieger.org., Rane S; Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland; and., McReynolds LJ; Division of Pediatric Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland., Steppan DA; Division of Pediatric Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland., Chen AR; Division of Pediatric Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland., Paz-Priel I; Division of Pediatric Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Jazyk:	angličtina
Zdroj:	Pediatrics [Pediatrics] 2016 Jul; Vol. 138 (1). Date of Electronic Publication: 2016 Jun 16.
DOI:	10.1542/peds.2015-1080
Abstrakt:	Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare, generally progressive, and potentially fatal syndrome of unclear etiology. The syndrome is characterized by normal development followed by a sudden, rapid hyperphagic weight gain beginning during the preschool period, hypothalamic dysfunction, and central hypoventilation, and is often accompanied by personality changes and developmental regression, leading to substantial morbidity and mortality. We describe 2 children who had symptomatic and neuropsychological improvement after high-dose cyclophosphamide treatment. Our experience supports an autoimmune pathogenesis and provides the first neuropsychological profile of patients with rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. (Copyright © 2016 by the American Academy of Pediatrics.)
Databáze:	MEDLINE
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