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Published on: April 7, 2021
Abnormal control of ventilation in adolescents with myelodysplasia
S Swaminathan1, J Y Paton, S L Ward
1Division of Neonatology and Pediatric Pulmonology, Childrens Hospital of Los Angeles, CA 90027.
Insights
Adolescents with myelomeningocele show persistent abnormal ventilatory control, specifically a reduced hypercapnic ventilatory response. This suggests lasting effects of the condition on breathing regulation.
Area of Science:
- Neurology
- Pulmonology
- Pediatrics
Background:
- Infants with myelomeningocele exhibit ventilatory control abnormalities.
- It is unknown if these respiratory control issues persist into adulthood.
Purpose of the Study:
- To investigate persistent ventilatory control abnormalities in adolescents and young adults with myelomeningocele.
- To compare respiratory responses between myelomeningocele patients and healthy controls.
Main Methods:
- Studied 14 patients with myelomeningocele and Arnold-Chiari malformation (mean age 18 years).
- Compared them with 14 age-matched healthy control subjects.
- Assessed pulmonary function, ventilatory muscle strength, and hypercapnic/hypoxic ventilatory responses.
Main Results:
- No significant differences in pulmonary function or muscle strength were found.
- Patients with myelomeningocele had significantly lower hypercapnic ventilatory responses compared to controls (1.98 vs. 3.33 L/min/mmHg).
- Hypoxic ventilatory responses and their correlation with hypercapnic responses differed between groups.
Conclusions:
- Adolescents and young adults with myelomeningocele retain abnormalities in ventilatory control.
- The Arnold-Chiari malformation may impair central chemosensitivity and respiratory control integration.
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.
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