{"entities":{"Q40047":{"pageid":30415,"ns":120,"title":"Item:Q40047","lastrevid":126126,"modified":"2026-08-13T05:42:15Z","type":"item","id":"Q40047","labels":{"mul":{"language":"mul","value":"5C61.2"},"pt-br":{"language":"pt-br","value":"Defici\u00eancia cong\u00eanita de sacarase-isomaltase"},"en":{"language":"en","value":"Congenital sucrase-isomaltase deficiency"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A defici\u00eancia cong\u00eanita de sacarase-isomaltase \u00e9 um dist\u00farbio de intoler\u00e2ncia a carboidratos, caracterizado pela m\u00e1 absor\u00e7\u00e3o de oligossacar\u00eddeos e dissacar\u00eddeos. Ela \u00e9 transmitida como um tra\u00e7o autoss\u00f4mico recessivo e \u00e9 causada por muta\u00e7\u00f5es no complexo de membrana da borda em escova sacarase-isomaltase, que \u00e9 necess\u00e1rio para a quebra de sacarose e amido em monossacar\u00eddeos. A defici\u00eancia de sacarase-isomaltase resulta em um ac\u00famulo de dissacar\u00eddeos no l\u00famen, causando diarreia osm\u00f3tica. O progn\u00f3stico para os pacientes \u00e9 bom, pois a intoler\u00e2ncia ao amido geralmente se resolve durante os primeiros anos de vida e a intoler\u00e2ncia \u00e0 sacarose geralmente melhora com a idade."},"en":{"language":"en","value":"Congenital sucrase-isomaltase deficiency (CSID) is a carbohydrate intolerance disorder characterised by malabsorption of oligosaccharides and disaccharides. CSID is transmitted as an autosomal recessive trait and is caused by mutations in the brush-border membrane complex sucrase-isomaltase (SI), which is required for the breakdown of sucrose and starch into monosaccharides. The SI deficiency results in an accumulation of disaccharides in the lumen, causing osmotic diarrhoea. The prognosis for patients is good as the starch intolerance usually resolves during the first few years of life and sucrose intolerance usually improves with age."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"d023ed9b7c4b94c5ba5237827847b921164cb610","datavalue":{"value":"https://id.who.int/icd/entity/1817406536","type":"string"},"datatype":"url"},"type":"statement","id":"Q40047$D803CD42-2FED-4059-954B-92AFC1B562AE","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"8b04faca9ea2d7da5ace468a3f0c33930145cae9","datavalue":{"value":"CID11:5C61.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q40047$C8A12161-9394-407C-864C-43588D28817C","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"05604dad34d494ab9b0a64205275f72d3ed44148","datavalue":{"value":"dki-india-5C61.2","type":"string"},"datatype":"string"},"type":"statement","id":"Q40047$7D06B5F5-20D1-40E5-A7FA-3409B4D0D782","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q40047$6C372667-5B2D-4CCC-A5B0-875256C6ABC1","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":"Q40047$7E91E54A-595E-4F4E-8570-6F9C95721D0D","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":"Q40047$261DCF09-ACFF-43F8-BD0F-A64EAE3FA55C","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"2f566274688c139127861bb92a9a5893fe9f6162","datavalue":{"value":{"entity-type":"item","numeric-id":15605,"id":"Q15605"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q40047$B59EA403-7504-423D-8785-931C00966897","rank":"normal"}]},"sitelinks":{}}}}