Congenital Central Hypoventilation Syndrome and Disorders of Control of Ventilation.

Autor: Kasi AS; Division of Pediatric Pulmonology, Emory University, Children's Healthcare of Atlanta, 1400 Tullie Road Northeast, Atlanta, GA 30329, USA. Electronic address: ajay.kasi@emory.edu., Perez IA; Division of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, Keck School of Medicine of the University of Southern California, 4650 Sunset Boulevard, MS #83, Los Angeles, CA 90027, USA. Electronic address: iaperez@chla.usc.edu.
Jazyk: angličtina
Zdroj: Clinics in chest medicine [Clin Chest Med] 2024 Sep; Vol. 45 (3), pp. 663-673.
DOI: 10.1016/j.ccm.2024.02.018
Abstrakt: Congenital disorders of ventilatory control typically manifest as central apneas, periodic breathing, and hypoventilation in the neonatal period, but some may present at a later age. Obstructive apneas may be the initial presentation, and some may have associated autonomic nervous system dysfunction. Individuals with these disorders can have absent or impaired ventilatory and arousal responses to hypoxemia and hypercapnia. This article discusses the presentation, pathophysiology, evaluation, and management of congenital central hypoventilation syndrome, rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome, Prader-Willi syndrome, and myelomeningocele.
Competing Interests: Disclosure The authors report no conflicts of interest.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE