{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T08:01:06Z","timestamp":1783065666529,"version":"3.54.6"},"reference-count":21,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,1,12]],"date-time":"2018-01-12T00:00:00Z","timestamp":1515715200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100001032","name":"Chicago Community Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001032","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To investigate neurocognitive deficits in children with Congenital Central Hypoventilation Syndrome (CCHS) by comparing them to their parents, since parents comprise a particularly suitable control group matched on disease\u2010extrinsic factors that can influence neurocognitive functioning. We compared CCHS patients to their parents and to population norms, hypothesizing that they would obtain lower intelligence test scores than both groups. We also compared patient\u2010parent differences against patient\u2010normative differences, to determine whether the two analytic approaches would yield different results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We administered an intelligence screening, the Shipley\u20102, to 21 school\u2010aged patients (age 14.2\u2009\u00b1\u20095.5 years) with <jats:italic>PHOX2B<\/jats:italic> mutation\u2010confirmed CCHS and their parents. Patients also received detailed clinical intellectual assessments using the Wechsler scales.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>CCHS patients scored significantly below parents on Shipley\u20102 indices of intelligence, vocabulary, and abstraction, with a trend for perceptual reasoning. The CCHS patients scored significantly below population norms on indices of abstraction and perceptual reasoning. Patient\u2010parent differences were significantly larger than patient\u2010normative differences for vocabulary scores. CCHS patients scored significantly below population norms on Wechsler indices of intelligence, perceptual reasoning, working memory, and processing speed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>CCHS may affect a broader range of cognitive abilities than previous research based on comparisons to population norms has indicated. Comparisons of CCHS children to their parents reveal deficits of vocabulary and abstract reasoning which have not been previously identified. A full understanding of the neurocognitive impact of CCHS requires comparisons between patients and other individuals such as friends, parents, or siblings who closely resemble them on disease\u2010extrinsic characteristics.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.23939","type":"journal-article","created":{"date-parts":[[2018,1,12]],"date-time":"2018-01-12T09:17:11Z","timestamp":1515748631000},"page":"492-497","source":"Crossref","is-referenced-by-count":18,"title":["Congenital central hypoventilation syndrome: Broader cognitive deficits revealed by parent controls"],"prefix":"10.1002","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3560-7323","authenticated-orcid":false,"given":"Frank A.","family":"Zelko","sequence":"first","affiliation":[{"name":"Department of Child and Adolescent Psychiatry Ann &amp; Robert H. Lurie Children's Hospital of Chicago Chicago Illinois"},{"name":"Northwestern University Feinberg School of Medicine Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tracey M.","family":"Stewart","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Center for Autonomic Medicine in Pediatrics (CAMP) The Stanley Manne Children's Research Institute Ann &amp; Robert H. Lurie Children's Hospital of Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cindy D.","family":"Brogadir","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Center for Autonomic Medicine in Pediatrics (CAMP) The Stanley Manne Children's Research Institute Ann &amp; Robert H. Lurie Children's Hospital of Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Casey M.","family":"Rand","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Center for Autonomic Medicine in Pediatrics (CAMP) The Stanley Manne Children's Research Institute Ann &amp; Robert H. Lurie Children's Hospital of Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debra E.","family":"Weese\u2010Mayer","sequence":"additional","affiliation":[{"name":"Northwestern University Feinberg School of Medicine Chicago Illinois"},{"name":"Department of Pediatrics Center for Autonomic Medicine in Pediatrics (CAMP) The Stanley Manne Children's Research Institute Ann &amp; Robert H. Lurie Children's Hospital of Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,1,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1130"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.20527"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200602-305OC"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/pr.2016.196"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21170"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80902-3"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)83038-0"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.15-0402"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1076\/clin.15.4.446.1879"},{"key":"e_1_2_8_11_1","volume-title":"Shipley\u20102 Manual","author":"Shipley W","year":"2009"},{"key":"e_1_2_8_12_1","volume-title":"Wechsler Abbreviated Scale of Intelligence (2nd ed.)","author":"Wechsler D","year":"2011"},{"key":"e_1_2_8_13_1","volume-title":"Wechsler Intelligence Scale for Children\u2014Fourth Edition: Administration and Scoring Manual","author":"Wechsler D","year":"2003"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200807-1069ST"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1037\/a0024338"},{"key":"e_1_2_8_16_1","first-page":"153","volume-title":"Statistical Abstract of the United States: 2012 (131st Edition)","author":"Bureau USC","year":"2011"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2015.65"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-015-1441-0"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1203\/PDR.0b013e31817da10a"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2010.02.078"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2015.00415"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.20565"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.23939","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.23939","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T12:07:34Z","timestamp":1695557254000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.23939"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,1,12]]},"references-count":21,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,4]]}},"alternative-id":["10.1002\/ppul.23939"],"URL":"https:\/\/doi.org\/10.1002\/ppul.23939","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,1,12]]}}}