Mate o Hirschsprung

e pā ana te mate o Hirschsprung ki te malformation o te hawhe maui o te kopirua. Kei te āhuatanga i te reira e te ngaro o ngā pūtau ganglion i roto i te submucosal me plexus intramuscular. puta mate o Hirschsprung i ngā tamariki i roto i te ōwehenga o te 1: 5000 wā. I roto i te taiohi, kua kitea te reira nui iti pinepine. Boys mamae rima nga wa atu pinepine atu i te mau tamahine. Hoki tenei huru pathological āhuatanga e tupu te utuafare,. I roto i ētahi wā, e pā ana te mate e ki te ahua o Down syndrome. I roto i te pakeke puta mate a Hirschsprung ka te huru o localization i roto i te rohe anorectal. I roto i ngā take kino, e taea wherahia ana ki te distal whēkau iti.

I roto i te pathogenesis o te mate e whai wāhi nui te kore o mana o te wehenga parasympathetic o te pūnaha io. Ko te hua o tenei huru ko te roa ā-tōu sphincter whakangā, e arata'i ki te whakaiti tūturu i roto i te mate hukihuki, inhibition o ioio, me te roha o te hypertrophy. Hauora faaite te mate he tu'unga mohiotia e te rite megacolon.

Ka taea e te mate o Hirschsprung tupu i roto i te utua, decompensated subcompensated me te āhua. Ko te pakeke o te huru pathological tei runga i anglionarnogo roa wahi. whakakitea puka Decompensated te tohu nui o te ileus.

āhuatanga āhuatanga o te mate he mamae kaha, bloating, ngaehe puku, kōroke. Roa o te heamana he akoranga ma'i (i roto i ētahi wā ki runga ki te marama). Bloating Ko hoki he āhuatanga tonu o te mate, he taimaha, me te taea meinga uaua manawa tamaiti. Bloating tonu ara i muri i te kaupapa whēkau ki te awhina o enemas. Ka taea te huaina tona nā te ahua o te mamae kaha. Kua piri ana te tohu matua o te mate whakapairuaki, ruaki, ngoikore, hiahia ino. Fifi o tenei huru pathological he anemia, deformation o koki raina. Ko te aroaro o te mumura, ulceration, kia hoatu goiter ara ki te korere tamau e meinga te rohirohi, āhua collaptoid.

tīmata te mate tātaritanga Hirschsprung ki te tirohanga ataata. hua i tohua i roto i te puku e tika ana ki te puku tamau, contractions peristaltic o te piro pānga, kumete matotoru. whakaritea hukihuki huri o te sphincter whero e whakamātautau mamati. tika whakarato Fluoroscopy he hua tika tika. Tohua te huringa i roto i te tauira intestinal tika ki te aroaro o tino moni o hau e meinga tukuna ai. I roto i He colonoscopy atu tikanga mauhaa e kua whakamahia mo te tātaritanga o ngā āhuatanga pathological, pārana werowero. whakamahia te whakamutunga e ki te whakatau i te whānuitanga me te localization o te constriction diameter rohe suprastenoticheskoy kopirua.

I roto i te tikanga ki te manatoko i te tātaritanga whakamahia te unuhanga transanal. Ko te āhuatanga e haapapu mate Hirschsprung whakaiti ganglia neuronal plexus intermuscular. I roto i te mahi, āwhina tenei tikanga ki te whakatau i te taumata o te resection.

Treatment o te kāwanatanga mate tei runga i te pakeke o tona akoranga. Ka taea te whakakorea puka utua e tikanga momo o therapy, i roto i te tahi atu te wā e rapu wawaotanga hāparapara akiaki, i te mea ki te tangohia kolonostomy, te tango i aganglionarnoy rohe, decompensated, overextension o te whēkau nui. Te tahuti nei i muri i maimoatanga whakahaere mai ki runga ki te wha ōrau.