title: [Continuous ambulatory monitoring in quality control of home therapy of congenital central hypoventilation syndrome (CCHS)].
abstract: 4 children with congenital central hypoventilation syndrome (CCHS) now aged 6 to 9 years were studied for 1 to 8 years. In all patients CO2-response is missing, hypoxic drive is maintained. All patients required mechanical ventilation after birth. 1 patient is supported by controlled oxygen therapy during sleep since 9 months of age. 2 patients are IPPV-ventilated during sleep. 1 patient is pressure control ventilated with an oro-nasal mask since 6 years of age. All children showed phases of hypo- and hyperventilation (max. pCO2 107 mm Hg) depending on vigilance with respiratory acidosis in awake state and during sleep. These findings required ambulatory monitoring of home-therapy by a professional guard and continuous recording of pCO2 and pulseoximetry. These longtime data (max. pCO2 72 mm Hg) show that ambulatory monitoring and control of therapy is able to avoid extreme variation of blood gases and to stabilize acid-base regulation during sleeptime in patients with CCHS.
authors.affiliation: Abteilung für angewandte Physiologie, Ruhr-Universität, Bochum, Deutschland.
authors.forename: C
authors.initials: C
authors.lastname: Schäfer
authors.forename: T
authors.initials: T
authors.lastname: Schäfer
authors.forename: G L
authors.initials: GL
authors.lastname: Wölfle
authors.forename: M E
authors.initials: ME
authors.lastname: Schläfke
mesh_terms: D002245:Carbon Dioxide / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006699:Home Care Services; D006801:Humans; D007223:Infant; D008297:Male; D010100:Oxygen / Q000097:blood; D017286:Polysomnography; D011785:Quality Assurance, Health Care; D012891:Sleep Apnea Syndromes / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D016896:Treatment Outcome; D012122:Ventilators, Mechanical
chemical_list: D002245:Carbon Dioxide; D010100:Oxygen