{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T13:02:48Z","timestamp":1791118968359,"version":"4.1.0"},"reference-count":2,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[1991,7,1]],"date-time":"1991-07-01T00:00:00Z","timestamp":678326400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1991,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Children with congenital central hypoventilation syndrome (CCHS) have abnormal ventilatory responses to metabolic stimuli. As there is a genetically determined component of chemoreceptor sensitivity, parents and siblings of children with CCHS may also have blunted ventilatory responses to hypercapnea and hypoxia. To test this, we studied hypercapnic ventilatory responses and hypoxlc ventilatory responses in six mothers, four fathers, and five siblings (6 to 49 yr of age) of seven children with CCHS and compared them with 15 age- and sex-matched control subjects (5 to 47 yr of age). Pulmonary function tests were not different between relatives of children with CCHS and control subjects. To measure hypercapnic ventilatory responses, subjects rebreathed 5% CO2\/95% O2 until Pa \u00a0CO2 reached 60 to 70 mm Hg. To measure hypoxic ventilatory responses (L\/min\/% SaO2), subjects rebreathed 14% O2\/7% CO2\/balance N2 at mixed venous Pco \u00a02 until SaO2 fell to 75%. All tests were completed in less than 4 min. Instantaneous minute ventilation, mean inspiratory flow (tidal volume\/Inspiratory time), and respiratory timing (inspiratory timing\/total respiratory cycle timing) were calculated on a breath-by-breath basis. Hypercapnic ventilatory responses were 1.97 \u00b1 0.32 L\/min\/mm Hg Pa \u00a0CO2 in children with CCHS relatives and 2.23 \u00b1 0.23 L\/min\/mm Hg Pa \u00a0CO2 in control subjects. Hypoxic ventilatory responses were \u22121.99 \u00b1 0.37 L\/min\/% SaO2 in the relatives and \u22121.54 \u00b1 0.25 L\/min\/% SaO2 in the control subjects. There was no significant difference between the relatives and the control subjects. We conclude that parents and siblings of children with CCHS do not have subclinical abnormalities of ventilatory control. We speculate that CCHS is not genetically determined.<\/jats:p>","DOI":"10.1164\/ajrccm\/144.1.136","type":"journal-article","created":{"date-parts":[[2011,8,3]],"date-time":"2011-08-03T16:14:36Z","timestamp":1312388076000},"page":"136-140","source":"Crossref","is-referenced-by-count":22,"title":["Hypercapnic and Hypoxic Ventilatory Responses in Parents and Siblings of Children with Congenital Central Hypoventilation Syndrome"],"prefix":"10.1093","volume":"144","author":[{"given":"Carole L.","family":"Marcus","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Floyd R.","family":"Livingston","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah E.","family":"Wood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas G.","family":"Keens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[1991,7,1]]},"reference":[{"key":"p_3","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1542\/peds.66.3.425","volume":"66","year":"1980","journal-title":"Pediatrics"},{"key":"p_33","first-page":"120A","volume":"28","year":"1980","journal-title":"Coo Res"}],"container-title":["American Review of Respiratory Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/144\/1\/136\/67644076\/ajrccm_144_1_136.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ajrccm\/article-pdf\/144\/1\/136\/67644076\/ajrccm_144_1_136.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T08:37:13Z","timestamp":1774859833000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ajrccm\/article\/144\/1\/136\/8557451"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1991,7,1]]},"references-count":2,"journal-issue":{"issue":"1","published-online":{"date-parts":[[1991,7,1]]},"published-print":{"date-parts":[[1991,7,1]]}},"URL":"https:\/\/doi.org\/10.1164\/ajrccm\/144.1.136","relation":{},"ISSN":["0003-0805"],"issn-type":[{"value":"0003-0805","type":"print"}],"subject":[],"published-other":{"date-parts":[[1991,7]]},"published":{"date-parts":[[1991,7,1]]}}}