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Vol. 71, No. 2, 2004
Issue release date: March–April 2004
Section title: Case Report
Respiration 2004;71:195–198
(DOI:10.1159/000076685)

Long-Term Survival of a Patient with Congenital Central Hypoventilation Syndrome despite the Lack of Continuous Ventilatory Support

Windisch W. · Hennings E. · Storre J.H. · Matthys H. · Sorichter S.
Department of Pneumology, University Hospital Freiburg, Freiburg, Germany

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Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 7/1/2002
Accepted: 5/12/2003
Published online: 3/22/2004
Issue release date: March–April 2004

Number of Print Pages: 4
Number of Figures: 1
Number of Tables: 0

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: http://www.karger.com/RES

Abstract

Untreated idiopathic congenital central hypoventilation syndrome (CCHS) is thought to cause infant death within 1–2 months. Here we present an adult patient with CCHS who survived without continuous ventilatory support, despite hypoventilation from early childhood onward. The diagnosis was confirmed at the age of 22 years, when the patient presented with hypoventilation during the night (PaCO2 60 mm Hg, PaO2 56 mm Hg, pH 7.32 HCO3 30 mmol/l) but hyperventilation when awake (PaCO2 26 mm Hg, PaO2 81 mm Hg, pH 7.56, HCO3 23 mmol/l). The maximal hematocrit was 77%. Despite mental retardation, noninvasive positive pressure ventilation (NPPV) could be successfully established. NPPV-supported ventilation during the night (PaCO2 36, PaO2 84 mm Hg, pH 7.47, HCO3 25 mmol/l) reduced hematocrit values (40.6 to 36.8%) over a period of 4 years. In conclusion, long-term survival with CCHS is possible without continuous ventilatory support. Spontaneous improvement of hypoventilation during sleep throughout childhood is possible and hyperventilation during wakefulness may occur in patients with CCHS. CCHS can be managed with NPPV despite mental retardation, even over a long-term period.

© 2004 S. Karger AG, Basel


  

Author Contacts

Wolfram Windisch, MD
Department of Pneumology, University Medical Clinic Freiburg
Killianstrasse 5
DE–79106 Freiburg (Germany)
Tel. +49 761 270 3706, Fax +49 761 270 3704, E-Mail windisch@med1.ukl.uni-freiburg.de

  

Article Information

Received: July 1, 2002
Accepted after revision: May 12, 2003
Number of Print Pages : 4
Number of Figures : 1, Number of Tables : 0, Number of References : 13

  

Publication Details

Respiration (International Review of Thoracic Diseases)
Founded 1944 as ‘Schweizerische Zeitschrift für Tuberkulose und Pneumonologie’ by E. Bachmann, M. Gilbert, F. Häberlin, W. Löffler, P. Steiner and E. Uehlinger, continued 1962–1967 as ‘Medicina Thoracalis’ as of 1968 as ‘Respiration’, H. Herzog (1962–1997)
Official Journal of the European Association for Bronchology and Interventional Pulmonology

Vol. 71, No. 2, Year 2004 (Cover Date: March-April 2004)

Journal Editor: C.T. Bolliger, Cape Town
ISSN: 0025–7931 (print), 1423–0356 (Online)

For additional information: http://www.karger.com/journals/res


Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 7/1/2002
Accepted: 5/12/2003
Published online: 3/22/2004
Issue release date: March–April 2004

Number of Print Pages: 4
Number of Figures: 1
Number of Tables: 0

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: http://www.karger.com/RES


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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