title: Abnormal control of ventilation in adolescents with myelodysplasia.
abstract: Infants with myelomeningocele have abnormalities in ventilatory control. To determine whether these persist into later life, we studied 14 patients with myelomeningocele and Arnold-Chiari malformation (age 18.0 +/- 0.8 (SE) years), and compared them with 14 control subjects (age 24.0 +/- 0.9 years). Pulmonary function and ventilatory muscle strength did not differ between patients with myelomeningocele and control subjects. Hypercapnic ventilatory responses were significantly lower in the group with myelomeningocele (1.98 L/min/mm Hg) compared with control values (3.33 L/min/mm Hg; p less than 0.01). Hypoxic ventilatory responses (-1.4 L/min/%oxygen saturation of hemoglobin in arterial blood) were not significantly different from control values (-2.14 L/min/%oxygen saturation). In control subjects the hypercapnic and hypoxic ventilatory responses were highly correlated with each other within subjects (r = 0.84; p less than 0.002) but not in those with myelomeningocele (r = 0.34; not significant). We concluded that adolescents and young adults with myelomeningocele have abnormalities in control of ventilation during sleep and wakefulness. We speculate that the Arnold-Chiari malformation interferes with central chemosensitivity (hypercapnic ventilatory response) and central integration of chemoreceptor output.
authors.affiliation: Division of Neonatology and Pediatric Pulmonology, Childrens Hospital of Los Angeles, CA 90027.
authors.forename: S
authors.initials: S
authors.lastname: Swaminathan
authors.forename: J Y
authors.initials: JY
authors.lastname: Paton
authors.forename: S L
authors.initials: SL
authors.lastname: Ward
authors.forename: R A
authors.initials: RA
authors.lastname: Jacobs
authors.forename: C W
authors.initials: CW
authors.lastname: Sargent
authors.forename: T G
authors.initials: TG
authors.lastname: Keens
mesh_terms: D000293:Adolescent; D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications*; D005260:Female; D006801:Humans; D006935:Hypercapnia / Q000209:etiology*; D000860:Hypoxia / Q000209:etiology*; D008297:Male; D009436:Neural Tube Defects / Q000150:complications*; D012129:Respiratory Function Tests