{"entities":{"Q41278":{"pageid":31646,"ns":120,"title":"Item:Q41278","lastrevid":136769,"modified":"2026-08-13T07:23:25Z","type":"item","id":"Q41278","labels":{"mul":{"language":"mul","value":"7A42.2"},"pt-br":{"language":"pt-br","value":"Hipoventila\u00e7\u00e3o central n\u00e3o cong\u00eanita com anormalidades hipotal\u00e2micas"},"en":{"language":"en","value":"Non-congenital central hypoventilation with hypothalamic abnormalities"}},"descriptions":{"pt-br":{"language":"pt-br","value":"Hipoventila\u00e7\u00e3o central n\u00e3o cong\u00eanita com disfun\u00e7\u00e3o hipotal\u00e2mica \u00e9 um transtorno do controle central da ventila\u00e7\u00e3o. Os pacientes usualmente s\u00e3o saud\u00e1veis no in\u00edcio da inf\u00e2ncia (frequentemente at\u00e9 2-3 anos de idade) quando eles desenvolvem hiperfagia e obesidade grave, seguidas por hipoventila\u00e7\u00e3o central, que se apresenta com frequ\u00eancia como insufici\u00eancia respirat\u00f3ria. A disfun\u00e7\u00e3o end\u00f3crina hipotal\u00e2mica pode ser caracterizada por n\u00edveis hormonais aumentados ou diminu\u00eddos e pode incluir um ou mais dos seguintes: diabetes insipidus, hipersecre\u00e7\u00e3o inadequada do horm\u00f4nio antidiur\u00e9tico, puberdade precoce, hipogonadismo, hiperprolactinemia, hipotireoidismo e secre\u00e7\u00e3o diminu\u00edda do horm\u00f4nio do crescimento, ou tumores de origem neural. Altera\u00e7\u00f5es comportamentais e do humor, algumas vezes graves, est\u00e3o frequentemente presentes. Atrasos do desenvolvimento ou caracter\u00edsticas de autismo podem estar presentes, mas muitos pacientes s\u00e3o cognitivamente normais._x000D_ _x000D_ Nota: Um diagn\u00f3stico definitivo requer a evid\u00eancia objetiva baseada na polissonografia com monitoriza\u00e7\u00e3o do di\u00f3xido de carbono (CO2) por medida arterial ou expirat\u00f3ria ou transcut\u00e2nea."},"en":{"language":"en","value":"Non-congenital central hypoventilation with hypothalamic dysfunction is a disorder of central control of ventilation. Patients are usually healthy until early childhood (often 2-3 years of age) when they develop hyperphagia and severe obesity, followed by central hypoventilation, which often presents as respiratory failure. Hypothalamic endocrine dysfunction may be characterised by increased or decreased hormone levels and may include one or more of the following: diabetes insipidus, inappropriate antidiuretic hormone hypersecretion, precocious puberty, hypogonadism, hyperprolactinemia, hypothyroidism, and decreased growth hormone secretion, or tumours of neural origin. Mood and behaviour abnormalities, sometimes severe, are often present. Developmental delay or autistic features may be present, but many patients are cognitively normal. Note: A definitive diagnosis requires objective evidence based on polysomnography with carbon dioxide (CO2) monitoring (by arterial, end-tidal or transcutaneous measures)."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"0b95dc5381243915debeccbfb8b77f60864b153e","datavalue":{"value":"https://id.who.int/icd/entity/1928918452","type":"string"},"datatype":"url"},"type":"statement","id":"Q41278$B0013031-5F67-4752-B0EE-33EE0C94C8E7","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"41e6314fcd724c7b6d405fc2a06296db4fd4d2ad","datavalue":{"value":"CID11:7A42.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q41278$B6881F06-9222-45B7-B0A7-7324876379F7","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"b2fde2688daeb4720c3c0ffae4cb37943f501d04","datavalue":{"value":"dki-india-7A42.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q41278$6651EF3E-F12D-45FB-A882-2465E638E57E","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q41278$1FCBFFD5-FF7B-45EB-8EB9-24B3E3A9B64E","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q41278$C5A934E8-5753-44C9-BC91-910545118B79","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q41278$303A36B3-DB9D-4CD4-87BE-912A44EEC794","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"4edf39454276e1f01ecf2a3245450e8c7bcb733c","datavalue":{"value":{"entity-type":"item","numeric-id":17084,"id":"Q17084"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q41278$46064AC9-B178-4047-AC8C-65F14325F9C6","rank":"normal"}]},"sitelinks":{}}}}