Isifo sikaHirschsprung

Izifo Hirschsprung zibhekisela ezingxenyeni ezingezansi kwesibindi esikhulu. Kubonakala ngokungabi khona kwamangqamuzana e-ganglion ku-pmusus submucosal ne-intermuscular. Izifo Hirschsprung ezinganeni zenzeka ngokwesilinganiso samacala angu-1: 5000. Entsha, itholakala kaningi kakhulu. Abafana badla kaningi kaningi ngaphezu kwamantombazane. Ngenxa yalesi sifo esibucayi sibonakala ngemvelo yomndeni wefa. Kwezinye izimo, lesi sifo sihlobene nokuvela kwe-Down syndrome. Kubantu abadala, isifo sikaHirschsprung senziwa nesimo sokuhlala endaweni yangasese. Ezimweni ezimbi, zingasakazeka engxenyeni yokuhlukanisa emathunjini amancane.

Ku-pathogenesis yesifo, indima ebalulekile idlala ngokungabi nethonya lomnyango we-parasympathetic wesimiso sezinzwa. Umphumela walesi simo ukulibaziseka ekuphumuleni kwe-sphincter yangaphakathi ye-rectum, okuholela ekuqhumeni okuphakade kwe-spastic, ukucindezelwa kwe-peristalsis, ukunwetshwa nokuxhunywa kwe-hypertrophy. Ngokwemithi, lesi sifo sibonakala esimweni esibizwa ngokuthi i-megacolon.

Isifo se-Hirshprunga singase senzeke kwifomu ekhokhelwa, ekhokhelwa futhi ekhokhisiwe. Ubukhulu besimo se-pathological ngqo kuxhomeke ebude be-anglionic site. Ifomu elikhokhelwayo liboniswa izimpawu zokuvimbela amathumbu emathunjini.

Izimpawu zesifo yizobuhlungu obukhulu, ukuvuvukala, ukuqhuma esiswini, ukuqothulwa. Ukulibaziseka kwe-stool kuyinto engapheli (ngezinye izikhathi ifika ngenyanga). Ukubhubhisa kuyisibonakaliso esiqhubekayo sesifo, sinzima futhi kungabangela ubunzima bokuphefumula ingane. Ukuvimbela kubamba ngisho nangemva kokukhipha amathumbu nge-enemas. Umphumela walo ungabizwa ngokuthi ukuvela kobuhlungu obukhulu. Ukudabuka, ukuhlanza, ubuthakathaka, ukuwohloka kwesidlo kuhlanganiswa nezibonakaliso eziyinhloko zesifo. Izinkinga zalesi sifo sezinkinga ziyi-anemia, ukuguqulwa kwamakhonksi okumbambo. Ukuba khona kwezinqubo zokuvuvukala, izilonda, i-dysbiosis kungabangela ukuvela kwesifo sohudo oluqhubekayo, okubangela ukukhathala, isimo se-collapoid.

Ukuxilongwa kwesifo sikaHirschsprung kuqala ngokuhlolwa kwangaphandle. Kukhona ukwanda kwesisu ngenxa yokuzikhukhumeza okuqhubekayo, ukuphazamiseka kokuphazamiseka kwesibindi esithintekile, izixuku ezinde kakhulu. Ukuguqulwa kwesivumelwano se-sphincter esilwe kunqunywa ukucwaninga ngomunwe. I-X-ray endaweni yokuma ikuvumela ukuba uthole imiphumela enembile. Kukhona ushintsho emaphethini emathumbu ngenxa yobuningi bezinombolo zegesi ezibangela ukuvinjelwa. Ezinye izindlela zokwenza izinto ezisetshenziselwa ukuxilonga lesi simo se-pathological colonoscopy, irrigoscopy. Lezi zinsuku zisetshenziselwa ukucacisa izinga kanye nokwehlukaniswa kwendawo yokunciphisa, ububanzi bendawo ephezulu yekoloni.

Ukuze uqinisekise ukuxilongwa, i-biopsy transanal isetshenziswa. Ukunciphisa i-nerve ganglia ye-plexus ye-intermuscular isibonakaliso esiqinisekisa isifo sikaHirschsprung. Ngesikhathi sokusebenza, le ndlela isisiza ukunquma izinga lokuthengiswa.

Ukwelashwa kwalesi sifo sokugula kuncike ebukhulu besigaba sawo. Amafomu ahlawulisiwe angaqedwa ngezindlela zokwelashwa ezizimele, kwezinye izimo ukuhlinzwa okuphuthumayo okudingekayo, okubandakanya ukusebenzisa i-colonostomy, ukususwa kwendawo ye-aganglionic, ukukhokhelwa ngokweqile, izingxenyana ezithintekayo kakhulu emathunjini amakhulu. Ukufa ngemva kokwelapha kufinyelela kumaphesenti amane.