Ukuba uphuhliso lomntwana aluhambanga kakuhle, umntwana lowo unesiphumo kwisakhiwo sezitho zangaphakathi. Enye yezi nguqulelo nguHirschsprung syndrome (megacolon okanye aganglion). Esi sifo senzeke kwenye yezingane ezingama-5000, ngokuyininzi kwindoda.
Isifo sikaHirschsprung - yintoni na?
I-intestinal motility kunye ne-periodic excretion yamagqabi alawulwa ngamaseli akhethekileyo. I-megacolon yamaKhanjethi ibonakaliswa ukungabikho kwabo okanye ukungabikho, ngenxa yokuba ukufuduka kwamagqabi kunqandwa kakhulu. Ngenxa yoko, inani elikhulu leemfesi liqokelela emathunjini amakhulu. Oku kubangela ukwandiswa kunye nokwandiswa kwelungu.
Isifo sikaHirschsprung kubantwana - iimpawu
Umfanekiso wekliniki uxhomekeke kakhulu kwiindawo ezikhoyo kunye neqondo lokungabikho kwamathumbu emathumbu. Isifo sikaHirschsprung kwiintsana sibonakala ngolu hlobo lweempawu ezilandelayo:
- ukungabikho kwamanzi okuqala (meconium);
- kuphawulwa;
- ukuguquka ;
- ukuhlanza, maxa wambi ube yi-bile;
- ukuqhenyiswa ixesha elide;
- utshintshe ngohlobo lwesisu;
- umbilicus ophantsi okanye ophantsi;
- nqabile i diarrhea.
Izifo eHirschsprung kubantwana abadala zihamba kunye neempawu ezongezelelweyo:
- ukungenakwenzeka kweentshukumo zokuhamba ngaphandle kwe-enema;
- intlungu yesisu;
- ukuxiliswa komzimba;
- ukuguga ekuphuculeni ngokomzimba;
- heterochromy;
- ubukho beentsimbi ezinzima ("amatye emathumbu");
- ukukhulelwa kwegazi ;
- ukuguquka kwesifuba;
- hypotrophy;
- enterocolitis;
- Ukwandiswa kwamathumbu amakhulu;
- ukukhusela ukuvelisa i-bronchitis kunye ne-pneumonia;
- ukuhlanza kunye nesifo sohudo emva kokuqhawula ixesha elide.
Izifo eHirschsprung kubantwana - ukuxilongwa
Ukuqinisekisa ukusola kwe-pathology echazwe, ugqirha kuqala uhlola ngesigxina isiguli esincinane. Isifo sikaHirschsprung kwiintsana ezinokuzalwa singabonakaliswa kwangaphambili, nokuba sisisu kwisimo semathumbu amakhulu. Inendima ebalulekileyo idlalwe ngabazali bemibuzo yomntwana. Ukuba intsapho yayineziganeko zokungabikho kombuzo, umonakalo wokubonakala kwayo kwintsana inyuka kakhulu (inzala).
Uviwo oluqhelekileyo lomntwana kunye nezifo zeHirschsprung
Indlela eyona nto yokuxilonga kwesi sifo kuboniswa kunye nokuhlolwa komnwe kwirectum (sigmoidoscopy). Isifo sikaHirschsprung sihamba kunye neetoni eziphezulu ze-sphincter kunye nesithuba esingenalutho phambi kokuvula kwesilwane, nangona ukukhutshwa kungazange kwenzeke iintsuku eziliqela. Ukufumana ulwazi oluninzi, uphando lweehadi lunikezelwa. Izifo Hirschsprung - Ukuchonga:
- I-Irrigoscopy ngokusungulwa komxube we-barium;
- colonoscopy;
- manometry;
- i-biopsy kunye nohlalutyo lwe-histological.
Isifo seHirschsprung - X-ray
Uhlobo loviwo oluyimfuneko ekuxilongweni kwezifo ezichazwe yintsebenzo yolu vavanyo. Izahlulo-mzo zinceda ukufumana ngokucacileyo apho i-megacolon (isifo seHirschsprung) sendawo, ukufumanisa ubunzima be-pathology. Ukuvavanya umgangatho we-intestinal motility, kucetyiswa ukuba uqalise umgangatho ophakathi kunye nolongezelelweyo lwe-ultrasound.
Ukunyangwa kwesifo seHirschsprung kubantwana
Inye indlela efanelekileyo yokuphatha umonakalo owenziwe ngongenelelo lophuhliso. Inkqubo yokugcinwa kwemilingo iyadingeka kuphela ekuhambeni kokusebenza. Unceda ukunciphisa nokunciphisa isifo sengqondo sikaHirschsprung kubandakanya ukuphelisa iimpawu zentsholongwane, ukulungelelanisa i-excletion ye-fecal kunye nokubuyiselwa kwamathumbu emathumbu. Emva kokunyangwa ngonyango kubalulekile ukuqinisekisa ukuba umntwana unonophelo olufanelekileyo kwaye alandele ukuthobela ukutya okukhethekileyo.
Isifo sikaHirschsprung kubantwana - iingcebiso zekliniki
Xa ukuxilongwa kugqityiwe kwaye ugqirha umisele umhla wokusebenza, kuyimfuneko ukulungiselela inkqubo. I-Megacolon kubantwana iquka oku:
- Ukupheliswa kwamathumbu emathunjini. Ukufakela i siphon ngokufakela ngokusungulwa kwesisombululo esifudumele se-sodium chloride ye-0.9%.
- Ukubuyiselwa kwe-microestyle yamathumbu. Ingane kufuneka ithathe iziyobisi ezikhuthaza umsebenzi obalulekileyo weebhaktheriya ezinobuncedo ( probiotics ).
- Ukuxhaswa kwe-immunity. Umntwana umiselwe amavithamini-E, C, B6, B12.
- Ukuphuculwa kwamatye okuphucula. Ukuqhelanisa ukukhutshwa kwamathumbu kunceda ukuhlalisa isisu kunye ne-gymnastics.
- Ukuthintela ukuqothulwa. Ukuthintela ukunyuka kwezilwanyana kunye nokutya okucetyiswayo ngefayibha ngokuhambisana nokusetyenziswa kwamanzi acocekileyo.
- Ukubuyiselwa kweenkqubo zokuxilongwa. Ngenxa yokuphazamiseka okukhulu kokutya, iisombululo ze-electrolyte ezingenayo i-electrolyte, i-glucose kunye nokulungiselela amalungiselelo eeprotheni.
Ugqirha lwezifo zeHirschsprung
Kukho iindlela ezininzi zokungenelela ukuphazamiseka kwesi sifo. Zonke ziquka ukucocwa kweendawo ezonakaliswe kumgca ochanekileyo kunye nesisu esiswini kunye nokumiselwa phakathi kweethambo ezinempilo ze-anastomosis. Ukususela ebusaneni, utyando lukaDuyel lwenziwa ngokusisiseko kwisifo sikaHirschsprung, kodwa kukho ezinye iintlobo zenkqubo efanelekileyo nekhuselekileyo:
- I-Rectosigmoidectomy ngokweSvenson-Hyatt-Isakov;
- i-anastomosis yangaphakathi e-anastomosis ngokubhekiselele kuRebein;
- U msebenzi.
Iindlela ezidwelisweyo zifana ngokulinganayo ngokubhekiselele ekusebenzeni, ngoko ukhetho lohlobo lokungenelela ngophando luyenziwa ngutitshala. Ukunikezela ngokunyanzeliswa komntu othile, ugqirha ukhokelwa ngumdala kunye nesimo sesigulane esincinci, sivavanya ukuba isifo sikaHirschsprung siphuthuma kangakanani. Kubalulekile ukuba abazali bafumane ugqirha onogqirha, yonke le misebenzi ibonakaliswe yinkqubo enzima yokuphunyezwa.
Izifo Hirschsprung - ukutya
Ukunciphisa iimpawu ze-pathology ezichazwe, kutyalwa ukutya okukhethekileyo kwe-laxative. Isondlo sesifo seHirschsprung siquka ukusetyenziswa kweemveliso ezithambisa isisitye kunye nokukhuthaza intlungu yamathumbu:
- i-decoction ye-bran okanye idill (kubantwana);
- ama-aprikoti omisiwe, i-prunes, ngokukodwa ixhomekeke kwisiseko sabo;
- ithanga;
- i-buckwheat, i-oats, iphareji yengqolowa;
- Ngenye imini kefir;
- beet;
- ukhukhamba;
- yoghurt;
- isobho;
- i-cutlets ye-steam kunye ne-soufflé kwi-fat-fat meat diet;
- iifuno zemifuno;
- amajikijolo amnandi, izithelo kunye nemifuno.
Ukususela ekudleni, kufuneka sikhankanye yonke into eqinisa kwaye ibangele iinkqubo zokuvumba emathumbu:
- peyari;
- irayisi, i-semolina iphariji;
- isonka esimhlophe kunye nesonka;
- iimveliso zokutshaya;
- bun and pastry;
- i-cocoa kunye nechocolatelethi;
- imbewu;
- iklabishi;
- inyama enamafutha;
- izithambiso ezizityebi kunye nesobho;
- amazambane (ngamanani amaninzi);
- ipasta;
- cherry;
- iipesele ngeekremu zeoli;
- imbotyi;
- ubisi bonke.
Isifo sikaHirschsprung kubantwana - imiphumela
Ngaphandle kolwaphulo oluchanekileyo kunye nexesha elifanelekileyo, i-anomaly ingqalelo ingaphelelwa kakubi, ingakumbi ukuba ifunyanwe kwintsana. Kwi-80% yeemeko ezinjalo, isifo sikaHarald Hirschsprung sikhokelela ekufeni komntwana. Xa utyando lwaluqhutywa ngexesha kunye nekhwalithi, izibikezelo ziphucuke kakhulu, malunga ne-90% yezigulane ezincinci zibuyela kwizinga elonelisayo.
Ezinye iingxaki eziqhelekileyo zesifo sikaHirschsprung:
- enterocolitis;
- ukukhulelwa kwegazi;
- isithintelo esiswini ;
- utywala;
- peritonitis;
- umkhuhlane;
- ukuphuma kwamathumbu emathunjini;
- i fistula;
- stenosis;
- ukungabikho komzimba.