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["Ondine's curse" in adults].
Pneumologie. 1999 Oct; 53 Suppl 2:S91-2.P

Abstract

The term "Ondine's curse" is normally used for congenital central hypoventilation in children. We report on 10 adult patients with this disorder (average age 46 y), who were treated from 1990 to 1996. They were hypercapnic while awake (mean 57 Torr) and during sleep (mean 87 Torr). The CO2-rebreathing response was negative. During exercise test minute ventilation volume did not rise adequately (mean pCO2 : 63 Torr, mean pH: 7.21). Five of these patients underwent emergency intubation before diagnosis, 2 presented with hypoventilation syndrome and 2 with decompensated cor pulmonale, 1 was diagnosed without complaints. 8 patients needed intermittent positive pressure ventilation therapy, 7 of them noninvasive, 1 via tracheal canula. One female patient has already died, the others are under stable clinical condition with the underlying central disorder remaining unchanged. Only 2 patients showed central defects visible in magnetic resonance tomography.

Authors+Show Affiliations

Bullemer F
Klinik für Intensivmedizin und Langzeitbeatmung, Asklepios Fachkliniken München-Gauting.
Heindl S
No affiliation info available
Karg O
No affiliation info available

MeSH

AdultAgedCarbon DioxideExercise TestFemaleHumansHypoventilationIntermittent Positive-Pressure BreathingIntubationMaleMiddle AgedSleepSleep Apnea, CentralWakefulness

Pub Type(s)

English Abstract
Journal Article

Language

ger

PubMed ID

10613051

Citation

Bullemer, F, et al. "["Ondine's Curse" in Adults]." Pneumologie (Stuttgart, Germany), vol. 53 Suppl 2, 1999, pp. S91-2.
Bullemer F, Heindl S, Karg O. ["Ondine's curse" in adults]. Pneumologie. 1999;53 Suppl 2:S91-2.
Bullemer, F., Heindl, S., & Karg, O. (1999). ["Ondine's curse" in adults]. Pneumologie (Stuttgart, Germany), 53 Suppl 2, S91-2.
Bullemer F, Heindl S, Karg O. ["Ondine's Curse" in Adults]. Pneumologie. 1999;53 Suppl 2:S91-2. PubMed PMID: 10613051.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - ["Ondine's curse" in adults]. AU - Bullemer,F, AU - Heindl,S, AU - Karg,O, PY - 1999/12/29/pubmed PY - 1999/12/29/medline PY - 1999/12/29/entrez SP - S91 EP - 2 JF - Pneumologie (Stuttgart, Germany) JO - Pneumologie VL - 53 Suppl 2 N2 - The term "Ondine's curse" is normally used for congenital central hypoventilation in children. We report on 10 adult patients with this disorder (average age 46 y), who were treated from 1990 to 1996. They were hypercapnic while awake (mean 57 Torr) and during sleep (mean 87 Torr). The CO2-rebreathing response was negative. During exercise test minute ventilation volume did not rise adequately (mean pCO2 : 63 Torr, mean pH: 7.21). Five of these patients underwent emergency intubation before diagnosis, 2 presented with hypoventilation syndrome and 2 with decompensated cor pulmonale, 1 was diagnosed without complaints. 8 patients needed intermittent positive pressure ventilation therapy, 7 of them noninvasive, 1 via tracheal canula. One female patient has already died, the others are under stable clinical condition with the underlying central disorder remaining unchanged. Only 2 patients showed central defects visible in magnetic resonance tomography. SN - 0934-8387 UR - https://www.unboundmedicine.com/medline/citation/10613051/["Ondine's_curse"_in_adults]_ DB - PRIME DP - Unbound Medicine ER -