Isifo Hirschsprung kaThixo

isifo Hirschsprung ithi kukho malformation wesiqingatha lasekhohlo ikholoni. Lubonakala kukungabikho iiseli ganglion kwi submucosal kunye plexus intramuscular. isifo Hirschsprung lenzeka khona abantwana umlinganiselo we-1: 5000 amatyala. Xa abakwishumi elivisayo, ukuba ifumaneka phi alufane. Boys ubunzima izihlandlo ezihlanu kaninzi ngaphezu amantombazana. Ngenxa yale meko zophendlo lwezifo luphawulwa ilifa kwiintsapho. Kwezinye iimeko, esi sifo lu dityanisiwe inkangeleko ka-Down syndrome. Xa abadala isifo Hirschsprung lenzeka xa imeko zalapha kummandla anorectal. Kwiimeko kakhulu, oku kungawunwenisa kwi distal emathunjini amancinci.

Xa Pathogenesis esi sifo ludlala indima ebalulekileyo ukungabikho yempembelelo kwisahlulo parasympathetic-luvo. Isiphumo sale meko Ukubambezela lwangaphakathi ezinqeni sphincter ukuphumla, nto leyo ekhokelela ekubeni unciphiso esisigxina yiSpasticity, inhibition of peristalsis, kunye ukwandiswa hypertrophy. Ngokwezonyango esi sifo ibonisa imeko eyaziwa ngokuba megacolon.

isifo Hirschsprung kunokuba kwenzeka babuyekezwe, decompensated subcompensated nemilo. Ubungqongqo imeko zophendlo lwezifo luxhomekeke anglionarnogo ubude isabelo. ifomu Decompensated kubonakala iimpawu okunzima ileus.

yeempawu zesifo intlungu obukhulu, ukuqumba, ukuduma isisu, ukuqhina kwesisu. Ukulibaziseka kwesitulo unalo ikhondo ezingapheliyo (kwezinye iimeko ukuya ngenyanga). Nokuqunjelwa kwakhona inkalo lomgadi sifo, unzima kwaye kunokubangela kunzima nokuphefumla umntwana. Nokuqunjelwa uhlala kude emva intshukumo wamathumbu ngoncedo enemas. ngenxa yayo kubizwa ngokuba imbonakalo intlungu obukhulu. Iimpawu eziphambili zale sifo ziqhotyoshelwe isicaphucaphu, ukugabha, ubuthathaka, inkanuko kubola. Iingxaki kule meko lwezidumbu zezi anaemia, deformation-engile emgceni. Ubukho nangokurhala, izilonda, goiter enokwenza ukuba lurhudo ezingisayo ezibangela ukudinwa, chaza collaptoid.

isifo Uxilongo Hirschsprung luqala uhlolo olubonakalayo. ukwanda okuphawulekayo esiswini ngenxa ukudumba rhoqo, izishunqulelo peristaltic yethumbu ochaphazelekayo, esitulweni zamafu. Ukutshintsha yindibanisela le sphincter ezimpundu ugqitywa iimviwo yedijithali. Fluoroscopy tye inika iziphumo echanileyo. Iphawulwe utshintsho pattern wamathumbu ngenxa yobukho ubeka imali ebonakalayo ngeegesi ingenza kuvaleke. Kwezinye iindlela nezixhobo ezisetyenziswa ngenxa uxilongo kweemeko lwezidumbu ezi colonoscopy, barium enema. Le yokugqibela isetyenziselwa ukugqiba ubungakanani kunye zalapha ekhaya kwabo constriction ubukhulu kummandla suprastenoticheskoy wamathumbu.

Ukuze uqinisekise uxilongo iyasetyenziswa biopsy transanal. Ukunciphisa ganglia neuronal plexus intermuscular ngumsebenzi engqina isifo Hirschsprung. Ngexesha lo msebenzi, le ndlela kunceda ukuqinisekisa inqanaba resection.

Unyango bakarhulumente sifo kuxhomekeke ubuzaza yawo. Iifomu ingahlawulwa lupheliswe ngeendlela angqingqwa unyango, kwezinye iimeko zifuna ungenelelo engxamisekileyo zotyando, nto leyo ukumisela kolonostomy, ukususa aganglionarnoy ndawo, decompensated, overextension yethumbu enkulu. Mortality emva unyango olwenziwe unyuka ukuya ekhulwini ezine.