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Sleep‐disordered breathing, respiratory patterns during wakefulness and functional capacity in pediatric patients with rapid‐onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation syndrome
Author(s) -
Selvadurai Sarah,
Benzon David,
Voutsas Giorge,
Hamilton Jill,
Yeh Ann,
Cifra Barbara,
Narang Indra
Publication year - 2021
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.25199
Subject(s) - medicine , polysomnography , hypoventilation , apnea , obstructive sleep apnea , body mass index , control of respiration , interquartile range , cardiorespiratory fitness , hypopnea , anesthesia , cardiology , respiratory system
Objective To characterize the clinical presentation of sleep‐disordered breathing and respiratory patterns at rest and during a 6‐min walk test (6MWT) in children with rapid‐onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome. Methods Retrospective study of children with ROHHAD who had a diagnostic baseline polysomnography, daytime cardiorespiratory monitoring at rest and a 6MWT. Polysomnography data were also compared with body mass index‐, age‐, and sex‐matched controls. Results Of the eight children with ROHHAD, all eight (100%) had obstructive sleep apnea (OSA) and 2/8 (25%) had nocturnal hypoventilation (NH) on their baseline polysomnography. Comparing the ROHHAD group to the control group, there were no significant differences in the median (interquartile range [IQR]) obstructive apnea‐hypopnea index (11.1 [4.3–58.4] vs. 14.4 [10.3–23.3] events/h, respectively; p = .78). However, children with ROHHAD showed a significantly higher desaturation index compared to the control group (37.9 [13.7–59.8] vs. 14.7 [4.3–27.6] events/h; p = .05). While awake at rest, some children with ROHHAD experienced significant desaturations associated with central pauses. During the 6MWT, no significant desaturations were observed, but two children showed moderate functional limitation. Conclusions Among children with ROHHAD, respiratory instability may be demonstrated by a significant number and severity of oxygen desaturations during sleep in the presence of OSA, with or without NH, and oxygen desaturations with central pauses at rest during wakefulness. Interestingly, during daily activities that require submaximal effort, children may not experience oxygen desaturations. Early recognition of respiratory abnormalities and targeted therapeutic interventions are important to limit associated morbidity and mortality in ROHHAD.