Ubuhlungu lukaHirschsprung: Izimpawu, Izimbangela, Nokwelapha

Ubuhlungu lukaHirschsprung yiphutha lokuzalwa elithinta i-large intestine (ikholoni) futhi lenze kube nzima kubantwana ukuba ne-bowel movements. Lesi simo senzeka lapho ama-nerve cells athile asiza ama-intestines ukususa ukhukhwini ephucelwe ezingxenyeni zekoloni.

Cishe ingane eyi-1 ku-5,000 izalwa naleli phutha, futhi livamile kubafana kunamantombazane. Izindaba ezinhle ukuthi ngokwelashwa okulungile, abantwana abane-Hirschsprung's disease bangaphila impilo enempilo, evamile.

Iyini i-Hirschsprung's Disease?

Ubuhlungu lukaHirschsprung buvela lapho ama-nerve cells abizwa ngama-ganglion cells engaqali kahle odongeni lwekoloni. La ma-nerve cells akhethekile atshela imisipha ye-intestinal ukuthi iphumule nini futhi ihlangane ukuhambisa ukhukhwini.

Ngaphandle kwala ma-nerve cells, ingxenye ethintekile yekholoni ayikwazi ukususa ukhukhwini ngokujwayelekile. Cabanga ngayo njengophahla lokulinywa olune-kink kulo - konke kuya kubuyela emuva endaweni evaliwe.

Lesi simo siqala njalo e-anus siye phezulu e-koloni. Ezimweni eziningi, kuphela ingxenye engezansi yekholoni ethintekile, kodwa ngezinye izikhathi ingase ihlanganise izingxenye ezinde ze-intestine.

Ziyini Izimpawu zobuhlungu lukaHirschsprung?

Izimpawu zobuhlungu lukaHirschsprung zivame ukuvela emasontweni ambalwa okuqala okuphila, yize ngezinye izikhathi zingabonakali kuze kube kamuva ebuntwaneni. Ingane ngayinye ihlukile, kodwa kunezibonakaliso ezivamile okufanele uzibheke.

Ezinganeni ezisanda kuzalwa, izibonakaliso ezibaluleke kakhulu zihlanganisa:

• Ukungaqali ukukhishwa kwe-stool yokuqala (meconium) phakathi kwamahora angu-48 ngemuva kokuzalwa
• Ukugabha okuluhlaza noma olu-brown
• Isisu esikhulu esizizwa siqine
• Igesi kodwa akukho bowel movements
• Ubunzima bokudla nokukhula okubi kwesisindo
• I-bowel movements ezivuthayo ngemuva kokubeka umunwe noma i-thermometer e-rectum

Lezi zimpawu zokuqala zenzeka ngoba ukhukhwini awukwazi ukuhamba ngokujwayelekile ngengxenye ethintekile yekholoni. Ukubuyela emuva kubangela ukucindezela okungakhululekile futhi kuvimba ukudla okujwayelekile nokukhula.

Ezinganeni ezindala kakhulu nabantwana, ungase ubone:

• I-constipation eqhubekayo engathuthuki ngokwelashwa okujwayelekile
• Ukwehluleka kokukhula kwesisindo ngesivinini esijwayelekile
• Ukukhathala nokuntuleka kwamandla
• Isisu esikhulu
• I-bowel movements ezinkulu, ezingavamile

Abanye abantwana abanezimpawu ezincane zalesi simo bangase bangabonisi izimpawu kuze kube yilapho beyizinsana noma badala. Lezi zimo zingaba nzima ukuzithola ngoba izimpawu zingase zibonakale njenge-constipation evamile ekuqaleni.

Yini ebangela ubuhlungu lukaHirschsprung?

Ubuhlungu lukaHirschsprung buvela ngesikhathi sokukhulelwa ekuqaleni lapho ingane ithuthukisa esizalweni. Imbangela eqondile ayiqondakali ngokuphelele, kodwa siyazi ukuthi ihilela izinkinga zendlela ama-nerve cells athile akhula futhi ahambela khona.

Phakathi nezinyanga ezimbalwa zokuqala zokukhulelwa, ama-nerve cells akhethekile aqala ohlelweni lokugaya lokukhula kwengane bese kancane kancane eya e-anus. Ezinganeni ezine-Hirschsprung's disease, la ma-nerve cells ayeka ukuhamba ngaphambi kokuba afike endaweni yabo yokugcina.

Lesi simo kubonakala sengathi sine-genetic component, okusho ukuthi singadluliselwa emindenini. Uma ingane enye ine-Hirschsprung's disease, abazalwane banethuba elingu-3-12% lokuba nayo, kuye ngezici ezahlukene.

Ama-gene amaningana ahlanganiswe naleli phutha, i-RET gene ihileleke kakhulu. Kodwa-ke, ukuba nalezi zinguquko ze-genetic akuqinisekisi ukuthi ingane izophuthuma lesi sifo - kukhuphula nje amathuba.

Ziyini Izinhlobo zobuhlungu lukaHirschsprung?

Odokotela bahlukanisa ubuhlungu lukaHirschsprung ngokusekelwe ekutheni ikholoni elingakanani elithintekile ngama-nerve cells asweleke. Ukuqonda uhlobo kusiza ekutholeni indlela yokwelapha engcono kakhulu.

I-short-segment disease uhlobo oluvamile kakhulu, oluthinta cishe ama-80% ezimo. Kulolu hlobo, kuphela ingxenye engezansi yekholoni (i-rectum ne-sigmoid colon) engenama-nerve cells. Abantwana abalolu hlobo bavame ukuba nezimpawu ezincane futhi ngokuvamile benza kahle kakhulu ngemuva kokwelashwa.

I-long-segment disease ithonya ingxenye enkulu yekholoni futhi ivela cishe ku-20% yezimo. Lolu hlobo luvamise ukubangela izimpawu ezinzima kakhulu futhi lungase lidingi ukwelashwa okuyinkimbinkimbi. Abanye abantwana bangase badinge ukuhlinzwa okuningi noma babe nezinkinga zokugaya eziqhubekayo.

Ezimweni ezingavamile, lesi simo singathinta ikholoni lonke noma siye siye e-small intestine. Lezi zimo zidinga ukunakekelwa okukhethekile futhi ngokuvamile zihilela ukusebenza neqembu lochwepheshe bezingane.

Nini Okufanele Ubheke Udokotela Ngobuhlungu lukaHirschsprung?

Kufanele uxhumane nodokotela wengane yakho ngokushesha uma ingane yakho esanda kuzalwa ingaqali ukukhishwa kwe-stool yokuqala phakathi kwamahora angu-48 ngemuva kokuzalwa. Lena enye yezimpawu zokuqala ezibaluleke kakhulu.

Ezinye izizathu ezibalulekile zokufuna ukunakekelwa kwezokwelapha zihlanganisa ukugabha okuluhlaza noma olu-brown, isisu esikhulu futhi esiqine, noma izibonakaliso zokuthi ingane yakho ayidli kahle futhi ibonakala ingakhululekile. Lezi zimpawu zingabonisa ukuphahlazeka okuthile okudingayo ukwelashwa ngokushesha.

Kubantwana abancane kakhulu nabantwana, i-constipation eqhubekayo engaphenduli ekuchithweni kokudla noma ekuwelapheni okuthambile kufanele ibe yivizithi yodokotela. Uma ingane yakho inezinto ezincane kunokuthi ezintathu ze-bowel movements ngesonto noma ibonakala icindezela kakhulu, kufanele ukuxoxe nodokotela wakho wezingane.

Ungalindi uma ubona ukuthi ingane yakho ayikhula noma ayithuthuki ngesisindo njengoba kulindelekile, ikakhulu uma lokhu kwenzeka kanye nezimpawu zokugaya. Ukuxilongwa kwasekuqaleni nokwelashwa kungavimbela izinkinga futhi kusize ingane yakho ikhule.

Ziyini Izici Zengozi zobuhlungu lukaHirschsprung?

Izici eziningana zingandisa amathuba okuba ingane izalwe ine-Hirschsprung's disease. Ukuqonda lezi zici zengozi kungasiza imindeni nodoctor ukuba baqaphele izibonakaliso zokuqala.

Ukuba owesilisa iyona nto enkulu yengozi - abafana banethuba elingu-4 lokuthola lesi simo kunamantombazane. Isizathu salesi sihlukaniso sobulili asiqondakali ngokuphelele, kodwa sibonakala njalo ekuhlaleni okuhlukene.

Umlando womndeni udlala indima ebalulekile. Ukuba nomzali noma umzalwane one-Hirschsprung's disease kukhuphula ingozi kakhulu. Ingozi iphakeme uma ilungu lomndeni elithintekile lingowesifazane noma line-long-segment type yesifo.

Ezinye izimo ze-genetic zihlanganiswe ne-Hirschsprung's disease, kuhlanganise i-Down syndrome, evela cishe ku-2-10% yabantwana abalesi simo se-intestinal. Ezinye izifo ze-genetic njenge-Waardenburg syndrome ne-congenital central hypoventilation syndrome nazo zithwala ingozi ephakeme.

Ezinye izinguquko ze-genetic ezingavamile zingenza imindeni ibe sengozini kakhulu, yize lezi zihlanganisa kuphela ingxenye encane yezimo. Abantwana abaningi abane-Hirschsprung's disease abanazo lezi zinguquko ze-genetic.

Ziyini Izinkinga Ezingase Zenzeke Ngobuhlungu lukaHirschsprung?

Nakuba ubuhlungu lukaHirschsprung bulapheka kakhulu, bungase buhole ezingxeni ezinzima uma lungaxilongwa futhi lungalawulwa kahle. Ukwazi lezi zinkinga ezingase zenzeke kusiza ekuqinisekiseni ukwelashwa okusheshayo.

Inkinga enzima kakhulu yisimo esibizwa nge-enterocolitis, okuwukuvuvukala kwama-intestines. Lokhu kungenzeka ngaphambi noma ngemuva kokuhlinzwa futhi kudinga ukunakekelwa kwezokwelapha ngokushesha. Izibonakaliso zihlanganisa ukushisa, i-diarrhea evuthayo, ukugabha, nesisu esikhulu.

I-toxic megacolon enye inkinga enzima lapho ikholoni liba likhulu futhi liqine kakhulu. Lesi simo esiyingozi esiyingozi singabangela ukuba udonga lwe-intestinal luqhekeke uma lungelaphiwe ngokushesha ngama-antibiotics futhi ngezinye izikhathi kwenziwe ukuhlinzwa okusheshayo.

Izinkinga zokukhula nokudla zingase zivele lapho abantwana bengakwazi ukuthatha izondlo kahle ngenxa yezinkinga zokugaya eziqhubekayo. Abanye abantwana bangase badinge ukusekelwa kokudla okukhethekile noma izithasiselo ukuze basize ekukhuleni kwabo ngokujwayelekile.

Ngemuva kokuhlinzwa, abanye abantwana bangase babe nezinkinga eziqhubekayo njenge-constipation eqhubekayo, ukungcola, noma ubunzima bokufunda ukuya esitulweni. Kodwa-ke, ngesineke nokukhathalela okulandelayo okulungile, izinkinga eziningi zithuthuka kakhulu ngokuhamba kwesikhathi.

Ukuze kuvinjelwe izinkinga, kubalulekile ukuxilongwa kwasekuqaleni, ukwelashwa okuhlinzwayo okufanele, nokulandelwa njalo neqembu lokukhathalela impilo lengane yakho. Abantwana abaningi abamukela ukunakekelwa okulungile baqhubeka bephila impilo evamile, enempilo.

Ubuhlungu lukaHirschsprung buxilongwa kanjani?

Ukuxilonga ubuhlungu lukaHirschsprung kuhilela ukuhlolwa okuningi okusiza odokotela ukubona ukuthi ikholoni isebenza kahle kangakanani futhi uma ama-nerve cells akhona. Inqubo ivame ukuba lula, yize kungase kudingeke ukuhlolwa okumbalwa ukuze uthole isithombe esiphelele.

Udokotela wakho uzoqala ngokuhlolwa komzimba nomlando wezokwelapha ophelele. Bazokuhlanganisa nge-bowel movements, izindlela zokudla, nanoma yiziphi izimpawu ozibonile. Bazophinde bazizwe isisu lengane yakho ukuze bahlole ukukhulu noma izindawo ezibuhlungu.

I-barium enema ngokuvamile iyona yokuqala yokuhlola izithombe eyenziwayo. Ingane yakho iphuza noma yamukela i-liquid ekhethekile ebonakala kuma-X-rays, ivumela odokotela ukubona ukuma nokusebenza kwekoloni. Ebuhlungwini lukaHirschsprung, lo mkhuba ngokuvamile ubonisa indawo ecindezelekile elandelelwa yingxenye ebanzi ngaphezulu kwayo.

Ukuxilongwa okuqinisekisiwe kuvela e-rectal biopsy, lapho odokotela besusa ingxenye encane yethishu odongeni lwe-rectal ukuze bahlole ngaphansi komkhiqizo. Lo mkhuba ungabonisa ngokuqinisekile uma ama-nerve cells akhona noma asweleke.

Ngezinye izikhathi odokotela basebenzisa i-anorectal manometry, ukuhlolwa okuqapha ukucindezela nokusebenza kwemisipha e-rectum nase-anus. Lokhu kungasiza ekutholeni imiphumela yemisipha engavamile ephawula ubuhlungu lukaHirschsprung.

Iyini Ukwelashwa kobuhlungu lukaHirschsprung?

Ukwelashwa okuyinhloko lobuhlungu lukaHirschsprung ukuhlinzwa kokususa ingxenye yekholoni engenama-nerve cells nokuxhuma izingxenye ezinempilo. Nakuba lokhu kungase kuzwakale kukhulu, le mihlangano iphumelela kakhulu futhi isize abantwana babuyele ekusebenzeni okujwayelekile kwe-bowel.

Abantwana abaningi bane-procedure ebizwa nge-“pull-through”, lapho ochwepheshe bokuhlinzwa besusa ingxenye ethintekile yekholoni bese bedonsa ingxenye enempilo phansi ukuze ixhume ne-anus. Lokhu ngokuvamile kungenziwa ngokuhlinzwa okukodwa, ikakhulukazi nge-short-segment disease.

Abanye abantwana, ikakhulu labo abanesifo esibanzi noma izinkinga, bangase badinge i-colostomy yesikhashana kuqala. Lokhu kudala ukwephulwa odongeni lwesisu lapho ukhukhwini ungaphuma khona e-collection bag, kunika i-lower intestine isikhathi sokuphumula nokuphola ngaphambi kokuhlinzwa okuyinhloko.

Ukuhlinzwa ngokuvamile kwenziwa ochwepheshe bokuhlinzwa bezingane abakhethekile kulezi zinhlobo zokuhlinzwa. Imikhuba eminingi ingenziwa ngezindlela ezingena kakhulu, okusho ukunqunywa okuncane nokubuyela esimweni esisheshayo.

Ngemuva kokuhlinzwa, abantwana abaningi kancane kancane babuyela ekusebenzeni okujwayelekile kwe-bowel, yize kungase kudingeke izinyanga ezimbalwa ukuze konke kuhlale ku-pattern ejwayelekile. Iqembu lakho lokuhlinzwa lizonikeza imiyalo eminingilizi yokunakekelwa ngemuva kokuhlinzwa nokulandelwa.

Ungayiphatha Kanjani Ukukhathalela Ekhaya Ngesikhathi Sokubuyela Esimweni?

Ukukhathalela ingane yakho ekhaya ngemuva kokuhlinzwa kuhilela ukulandela imiyalo yeqembu lakho lezokwelapha ngokucophelela nokubheka izibonakaliso zokwelapha noma izinkinga ezingase zenzeke. Imindeni eminingi ithola ukuthi ngokulungiselela okulungile, ukubuyela ekhaya kuhamba kahle kakhulu.

Gcina indawo yokuhlinzwa ihlanzekile futhi yomile ngokuvumelana nemiyalo yochwepheshe bakho bokuhlinzwa. Cishe uzodinga ukushintsha izembatho njalo futhi uqaphele izibonakaliso zokutheleleka njengokukhuluka kobomvu, ukukhuluka, noma ukukhishwa. Ungangabazi ukubiza udokotela uma uneminye imibuzo.

Ukudla kungase kudingeke kulungiswe ekuqaleni, ikakhulu uma ingane yakho yayine-colostomy. Iqembu lakho lokukhathalela impilo lizonikeza imiyalo ethile mayelana nokudla okumele ukunikwe nokuthi nini kufanele uthuthukise ukudla. Qala kancane futhi uqaphele ukuthi ingane yakho ibekezelela kanjani ukudla okuhlukene.

Ukuphathwa kobuhlungu kubalulekile ngenxa yokududuza nokuphola. Nikeza imithi ngokuqondile njengoba kulotshiwe futhi ungasidluli isithombe ngisho noma ingane yakho ibonakala ikhululekile. Ukuba nokulungiswa okuqhubekayo kokulawula ubuhlungu kusiza abantwana ukuba baphole ngokushesha.

Qaphela izibonakaliso zezinkinga njengokushisa, ukugabha okuqhubekayo, noma izinguquko e-bowel movements ezikukhathazayo. Iqembu lakho lokuhlinzwa lizonika uhlu lwezimpawu zezesawoti nolwazi loxhumana nabo oluphuthumayo.

Khumbula ukuthi ingane ngayinye iphala ngesivinini sayo. Abanye bangase babuyele emisebenzini evamile phakathi kwamasonto ambalwa, kuyilapho abanye bedinga isikhathi esiningi. Landela ingane yakho futhi ungasheshisi inqubo yokubuyela esimweni.

Kufanele Uzilungiselele Kanjani I-Appointment Yakho Yodokotela?

Ukulungiselela i-appointment yakho kusiza ekuqinisekiseni ukuthi uthola ulwazi oluwusizo kakhulu futhi wenze izinqumo ezinhle kakhulu ngokunakekelwa kwengane yakho. Ukulungiselela kancane ngaphambi kwesikhathi kungenza umehluko omkhulu ekutheni i-visit yakho isebenze kangakanani.

Gcina irekhodi eliphelele le-bowel movements yengane yakho, kuhlanganise ubukhulu, ubuqiniso, nanoma yiziphi izindlela ozibonile. Landela nezindlela zokudla, izinguquko zesindo, nanoma yiziphi izimpawu njengokugabha noma ubuhlungu besisu. Lolu lwazi lusiza odokotela ukuqonda ngokuqondile ukuthi kwenzekani.

Bhala yonke imibuzo yakho ngaphambi kwe-appointment ukuze ungakhohlwa lutho olubalulekile. Faka imibuzo mayelana nokuxilongwa, izinketho zokwelapha, okumele ukulindele ngesikhathi sokubuyela esimweni, nanoma yiziphi izinkathazo mayelana nemiphumela yesikhathi eside.

Letha uhlu oluphelele lanoma iyiphi imithi noma izithasiselo ingane yakho eziyithathayo, kuhlanganise ne-dosages nokuthi zinikezwa kaningi kangakanani. Khumbula nanoma yiziphi izifo zokuzalwa noma izimpendulo zangaphambili emithi.

Uma kungenzeka, letha omunye umuntu womndeni noma umngani ukuze asekele, ikakhulu uma uxoxa ngokuhlinzwa noma izinhlelo zokwelapha ezinzima. Ukuba nomunye umuntu ohlala elalele kungakusiza ukuba ukhumbule ulwazi futhi unikeze ukwesekwa ngokomzwelo.

Ungesabi ukucela ulwazi olubhaliwe noma izinsiza mayelana nesimo sengane yakho. Imindeni eminingi ithola ukuba usizo ukuba izinto ezibhaliwe ezingabuka ekhaya lapho zingazizwa zicindezelekile.

Iyini into ebaluleke kakhulu mayelana nobuhlungu lukaHirschsprung?

Ubuhlungu lukaHirschsprung yiphutha lokuzalwa elilapheka elithinta ikhono lekoloni lokuhambisa ukhukhwini ngokujwayelekile. Nakuba ukuxilongwa kungase kuzwakale kukhulu ekuqaleni, iningi labantwana abamukela ukwelashwa okufanele baqhubeka bephila impilo evamile, enempilo ngokuphelele.

Ukuqaphela kwasekuqaleni nokwelashwa kubalulekile ngenxa yemiphumela engcono kakhulu. Uma ubona izibonakaliso njengokudelela kwe-bowel movements yokuqala ezinganeni ezisanda kuzalwa noma i-constipation eqhubekayo ezinganeni ezindala, ungangabazi ukuxoxa ngalezi zinkathazo nodokotela wakho wezingane.

Ukuhlinzwa kuphumelela kakhulu ekwelapheni lesi simo, futhi abantwana abaningi babona ukuthuthuka okukhulu ezimpawu zabo ngemuva kwalokho. Nakuba ukubuyela esimweni kudinga isikhathi nesineke, imindeni ngokuvamile ithola ukuthi impilo ibuyela esimweni esijwayelekile phakathi nezinyanga ezimbalwa zokwelashwa.

Khumbula ukuthi ukuba nobuhlungu lukaHirschsprung akulinganiselwe yilokho ingane yakho engakukwazi ukukufinyelela ekuphileni. Ngokunakekelwa kwezokwelapha okufanele nokusekela kwakho okuthandekayo, abantwana abalesi simo bahlanganyela ngokugcwele esikoleni, ezemidlalo, nakuyo yonke imisebenzi eyenza ubuntwana bube okhethekile.

Imibuzo Evamile Ebuzwayo Mayelana nobuhlungu lukaHirschsprung

Ingane yami izokwazi ukulawula i-bowel movements yayo ngokujwayelekile ngemuva kokuhlinzwa?

Abantwana abaningi bathuthukisa ukulawula okujwayelekile kwe-bowel ngemuva kokuhlinzwa, yize kungase kudingeke izinyanga ezimbalwa kuya konyaka ukuze konke kuhlale ngokuphelele. Abanye abantwana bangase badinge usizo olwengeziwe ekufundiseni ukuya esitulweni noma ekulawuleni izigameko ezingavamile, kodwa iningi lifinyelela ekulawuleni okuhle ngokuhamba kwesikhathi. Iqembu lakho lokuhlinzwa lizohlangana nawe ukuze lixazulule noma yiziphi izinkathazo eziqhubekayo futhi linikeze amasu okusiza ingane yakho iphumelele.

Ubuhlungu lukaHirschsprung bungavikeleka?

Okwamanje ayikho indlela yokuvimbela ubuhlungu lukaHirschsprung ngoba kuyisimo sokuthuthuka esenzeka ngesikhathi sokukhulelwa. Kodwa-ke, uma unemlando womndeni wesimo, ukwelulekwa kwe-genetic ngaphambi kokukhulelwa kungakusiza ukuqonda izici zengozi zomndeni wakho. Into ebaluleke kakhulu ukuqaphela kwasekuqaleni nokwelashwa uma ingane izalwa.

Ukuhlinzwa kuthatha isikhathi esingakanani futhi ukubuyela esimweni kunjani?

Ukuhlinzwa kwe-pull-through ngokuvamile kuthatha amahora angu-2-4, kuye ngokuthi ikholoni elingakanani elithintekile. Abantwana abaningi bahlala esibhedlela izinsuku ezingu-3-7 ngemuva kokuhlinzwa. Ukubuyela esimweni ngokuphelele ngokuvamile kuthatha amasonto ambalwa kuya ezinyangeni ezimbalwa, lapho ingane yakho izobuyela kancane kancane emisebenzini evamile nasekudleni. Iqembu lakho lokuhlinzwa lizonikeza isimiso esiningilizi nesikhathi sokubuyela esimweni.

Ingane yami izodinga ukulinganiselwa kokudla okukhethekile ngemuva kokwelashwa?

Abantwana abaningi bangabuyela ekudleni okujwayelekile ngokuphelele ngemuva kokuhlinzwa nokubuyela esimweni. Ekuqaleni, udokotela wakho angase akukhuthaze ukuqala ngokudla okulula ukukugaya bese kancane kancane uthuthukisa ukudla okuningi. Abanye abantwana bazuza ngokudla ukudla okuncane, okuvamile noma ukufaka okuningi kwefayibha ekudleni kwabo ukuze basekele i-bowel movements enempilo, kodwa lezi azikho izikhawu eziqinile.

Ziyini izindlela zokuthi abantwana bakusasa babe nobuhlungu lukaHirschsprung?

Uma une-child enye ene-Hirschsprung's disease, ingozi yabantwana bakusasa iphakeme kunabantu abavamile kodwa isancane. Ingozi eqondile ihlanganisa izici njengobudoda bengane ethintekile nobukhulu besifo sabo, kodwa ngokuvamile iphakathi kuka-3-12%. Udokotela wakho anganikeza ulwazi oluqondile lwegozi ngokusekelwe esimweni somndeni wakho futhi angase akukhuthaze ukwelulekwa kwe-genetic.