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["Ondine's curse" in adults].
1Klinik für Intensivmedizin und Langzeitbeatmung, Asklepios Fachkliniken München-Gauting.
Pneumologie (Stuttgart, Germany)
|December 29, 1999
Summary
Ondine's curse, or congenital central hypoventilation syndrome, affects adults, causing severe hypercapnia and inadequate ventilation. Many patients require respiratory support, highlighting the disorder
Area of Science:
- Neurology
- Pulmonology
- Genetics
Context:
- Congenital central hypoventilation syndrome (CCHS), commonly known as Ondine's curse, is typically diagnosed in children.
- This study focuses on a cohort of adult patients diagnosed with CCHS, presenting a unique clinical perspective.
Purpose:
- To describe the clinical characteristics, diagnostic challenges, and management of Ondine's curse in adult patients.
- To evaluate the long-term outcomes and treatment efficacy in this adult population.
Summary:
- Ten adult patients (average age 46) with CCHS were retrospectively analyzed.
- Patients exhibited severe hypercapnia (awake and sleep), negative CO2-rebreathing response, and inadequate ventilation during exercise.
- Eight patients required ventilatory support, primarily non-invasive positive pressure ventilation.
Impact:
- Highlights the underdiagnosis and varied presentations of CCHS in adults.
- Underscores the necessity for respiratory support in affected adults.
- Suggests that CCHS can remain clinically stable in adults with appropriate management, despite the persistent central disorder.