Syndrome Mauriac i roto i te mate huka

Mauriac syndrome - he mate e whanake rite te pōauautanga tika ki te maimoatanga hē o te mate huka i te kuao tau. Hoki te wā tuatahi whakaahuatia te mate i roto i 1930 i te rata French Pierre Mauriac takenga. whakaahuatia e ia te ahua o te pikitia haumanu i roto i nei tamariki e mate ana i te mate huka, me te taiāki therapy mua te inenga he, faaohipa te tahi mau tohu o waho. kite ia e he rite i roto i te ahua, kitea nei i roto i te tupu, te mōmona, te whanaketanga fakasekisualé e pūkeke iti nga tamariki katoa.

Ko te take o te mate

Ko te take matua o te pōauautanga nui he hē therapy huka whai ana. I roto i tenei mate e kore e mahi tika te repe taiaki, e kore whakaputa rawaka nui o taiāki. Nā ki te māuiuitanga puta hē te kūhuka i roto i te pūtau tika ki te meka e aro i te reira i roto i te moni nui i roto i te toto.

Mauriac whanaketanga syndrome i roto i te tūroro ki te mate huka momo mellitus 1 pā ana te ki te maimoatanga takarepa. tamaiti e mate ana hoki te wa roa horopeta iti taiāki faaterehia, ranei whakamahia hua o te kounga iti, me te kino e purea ana, ārahi ki te hē ma'i o te taiāki.

kore auroa o taiāki i roto i te tinana arata'i ki te tukanga e whai ake nei:

• disorders pūkoro tinana, ngā te whakararuraru o warowaihā pākia.
• Te whakanui ake i te rahi o te ate, me tona e hōloa fatty tika ki nui haere raru glycogen.
• Huringa i roto i te hanganga toto - hua i roto i te toto kūhuka, cholesterol, waikawa ngakongako.

Ki te ko tenei e kore tukanga pathological katoa i roto i te tinana i roto i Mauriac syndrome, ano tūroro pediatric wheako nga ine e whai ake:

• production iti o hormones nui - cortisol, taiki, glucagon, a kia rite ki te putanga o te takahi o te tukanga tupu.
• Rärangi o pūmua, me te taonga konupūmā putanga me te ūkuikui i wheua, pae hopea ārahi ki te whanaketanga o te osteoporosis me etahi atrophy uaua.
• Ngoikore absorption o huaora i roto i te piro.

te tikanga faaite te mate ano i waenganui o nga tau o 15-18 tau, engari haamata te tukanga aueue nui mua. I reira, mo te maimoatanga o te mate huka wawe whakamahi taka hou, me te ata pure, e tata faaore te whanaketanga o Mauriac syndrome.

tohutanga o te mate

he maha o ngā pānga Mauriac Syndrome mate huka kua:

• Ko te tamaiti ërä i muri i roto i te whanaketanga me te tupu inhibition. E mea pinepine i reira he mōmona, me te poto roa. I roto i tenei take, kia ngenge te tamaiti mate i muri i ō rātou hoa i roto i te tupu i te 10-30 henimita.
• Ko te takamuri i roto i te whanaketanga fakasekisualé (underdeveloped āhuatanga fakasekisualé me te ngaro o te paheke i roto i ngā kōtiro).
• pūhuruhurutanga tautohetia ana.
• Mōmona, rawa i roto i te mata, me te tinana o runga ki waewae angiangi. He tino rite tamariki mate, i te "heitiki-āhua" i mata tahi e te tahi o, kaki poto, ngako i roto i nga ringa, pokohiwi, puku. Ko te tino kikokore te wahi o raro o te tinana.
• ate nui haere tahi tohanga venous awhio.
• Osteoporosis (tērā iho te whanaketanga o te wheua kiko).
• mate kanohi, tae atu ki ngā mate o te etine, a muri te whanaketanga o awhiowhio wai.

Ko te syndrome tino noa Mauriac i tamariki, whakaatu ana te whakaahua tamaiti mate he aha tohu i te mate.

tātaritanga o te mate

whakaturia te tātaritanga o "syndrome Mauriac" te i te tohu waho kitea o te mate, pērā i: te kore o te teitei tau mo, te aroaro o te mōmona, rawa i roto i te mata o te pangore fakasekisualé, he mārama hua i roto i te ate.

Hei whakaū i te tātaritanga kawea i roto i whakamātautau toto, i hua i roto i te mau faaiteraa e whai ake nei ko:

• taumata kūhuka iu, kia whakaatu hūpeke tamau i roto i te nui, ka ki raro.
• lipids toto nui (hyperlipidemia).
• He hua nui i roto i ngā taumata toto cholesterol (hypercholesterolemia).

I roto i ētahi wā, ia tuatapapa atu â ate unuhanga mo tenei whakaaro, ki te kawe i roto i te kawenga ngako

te mate

Hoki te kuao tinana ko tino uaua te mate. Ka rite ki te ahua nui o te mate huka, he uaua ki te utu mo te. Pinepine ai acidosis me pōroa hyperglycemic.

Puta Mauriac syndrome i nui no o te tua auau o ngā mate hopuhopu, me te whakaora tukanga matüora matatini me te roa i roto i te tinana.

Mauriac syndrome me Nobekura: ritenga me ngā rerekētanga

Mauriac syndrome a maha Nobekura whanake i roto i te tamarikitanga, e rua mate ko te pōauautanga nui o te mate huka e puta i runga i te whakamārama o te maimoatanga tano ore. syndromes rua i rite tohumate, ngā ratou retardation tupu me te whanaketanga tahae, he tautohetia ana te taiohitanga, fatty ate. I roto i tenei take, i te rerekētanga matua Nobekura syndrome ko te kore o te ngako tinana te taikaha. I roto i tenei take, te maimoatanga o syndromes e rua whāinga ki te wharanga te maimoatanga o te mate huka.

Mauriac maimoatanga syndrome

e kore e maimoatanga Direct o te syndrome rite tenei te tīariari. therapy katoa e hāngai ana ki te whakatutuki i te take pakiaka o te mate me te pōauautanga meinga e ratou. Ki te mahi i tenei, whakaritea te manawanui kei ki te therapy taiāki tika i roto i te inenga tika me te kounga ki taero hou.

Kia kore ai lipodystrophy tū he maha o ngā tukanga ārai mō tenei whakaaro i whakaritea kia rite ki te mirimiri, i tua me ngā tūtohi mō ngā werohanga taiāki. Ka taea te whakaritea kai motuhake i roto i te tua ki te maimoatanga, kua kapea ki waho i te kai kai katoa kei roto ngako kararehe. I roto i tenei take, hāngai te kai i runga i ngā kai pūmua me te warowaihā.

Kei te hangaia hoki therapy Medical ki te utu whakawhanake i runga i te whakamārama o te pōauautanga mate whāriki mō tenei naznachetsya manawanui:

• Urunga gepatoprotektorov ki whakahokia pūtau ate.
• huaora B ki te whakahoki pākia noa.
• tarukino Matatini hāngai Whakapūnoa nga taumata o lipids me cholesterol i roto i te toto.
• taero pūtaiaki ki te tīmata te tipu o te tinana.
• taero hormonal ki te whakahokia mai ngā mahi fakasekisualé nui.

Ārai me te waitohunga

Kia kore ai tenei pōauautanga kino o mate huka rite Mauriac syndrome, he mea e tika ana ki te kawe i te maimoatanga tika o te mate e whāriki ana, me te ārai i taiāki hē.

A, no te Kua whakawhanakehia maimoatanga ngā kē te syndrome waitohunga pai nui drugs hou, engari ki te katoa tūtohutanga a te tākuta. Ko te inenga tika o te taiāki huka, me te whakahoki taumahinga o whēkau katoa pāngia e taea ki te hoki mai te hauora o te tamaiti, e rua waho, me te ā.