{"id":2541,"date":"2014-08-05T18:08:04","date_gmt":"2014-08-05T18:08:04","guid":{"rendered":"http:\/\/www.sursamedicala.ro\/blog\/\/boala-urinei-cu-miros-de-sirop-de-artar\/"},"modified":"2014-08-05T18:08:04","modified_gmt":"2014-08-05T18:08:04","slug":"boala-urinei-cu-miros-de-sirop-de-artar","status":"publish","type":"post","link":"https:\/\/www.sursamedicala.ro\/blog\/boala-urinei-cu-miros-de-sirop-de-artar\/","title":{"rendered":"BOALA URINEI CU MIROS DE SIROP DE ARTAR"},"content":{"rendered":"<p>BOALA URINEI CU MIROS DE SIROP DE ARTAR \/ m. des urines a odeur de sirop d&#8217;erable \/ maple syrup urine d. B. metabolica cu transmitere autozomal dominanta, cauzata de un deficit al enzimei ce catalizeaza decarboxilarea oxidativa a aminoacizilor cu catena ramificata (leucina, izo-leucina si va-lina), care duce la acumularea acestora si a cetoadzilor omo-logi In sange si urina. Caracteristidle clinice includ coma, con-vulsii, retardare fizica si mentala si un miros caracteristic de ,,sirop de artar&#8221; al urinei si al corpului. Exista patru fenotipuri clinice ale afectiunii: 1) clasic, eel mai sever, cu exitus la scurt timp dupa nastere; 2) intermediar, cu debut mai tardiv; 3) intermitent: 4) legat de tiamina (afinitate scazuta a enzi-melor pentru cofactorul tiamin-pirofosfat). Sin.: boala Menkes, leucinoza. <\/p>\n","protected":false},"excerpt":{"rendered":"<p>BOALA URINEI CU MIROS DE SIROP DE ARTAR \/ m. des urines a odeur de sirop d&#8217;erable \/ maple syrup urine d. B. metabolica cu transmitere autozomal dominanta, cauzata de un deficit al enzimei ce catalizeaza decarboxilarea oxidativa a aminoacizilor cu catena ramificata (leucina, izo-leucina si va-lina), care duce la acumularea acestora si a cetoadzilor [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_bbp_topic_count":0,"_bbp_reply_count":0,"_bbp_total_topic_count":0,"_bbp_total_reply_count":0,"_bbp_voice_count":0,"_bbp_anonymous_reply_count":0,"_bbp_topic_count_hidden":0,"_bbp_reply_count_hidden":0,"_bbp_forum_subforum_count":0,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"tdm_status":"","tdm_grid_status":"","footnotes":""},"categories":[3],"tags":[],"class_list":["post-2541","post","type-post","status-publish","format-standard","category-dictionar-medical"],"_links":{"self":[{"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/posts\/2541","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/comments?post=2541"}],"version-history":[{"count":0,"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/posts\/2541\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/media?parent=2541"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/categories?post=2541"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.sursamedicala.ro\/blog\/wp-json\/wp\/v2\/tags?post=2541"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}