Izifo Hirschsprung - kanjani ukulungisa pathology?

Uma ukuthuthukiswa kwe-fetal kungalungile, i-fetus inamaphutha kwisakhiwo sezitho zangaphakathi. Enye yalezi zinguquko i-Hirschsprung syndrome (i-megacolon noma i-aganglion). Lesi sifo senzeke kwenye yezingane ezingu-5000, ikakhulukazi amadoda.

Isifo sikaHirschsprung - siyini?

I-motility yamathumbu kanye nokudlulelwa kwesikhashana kwamagqabha kulawulwa ngamaseli akhethekile. I-megacolon engumgogodla ibonakala ngokuntuleka kwabo noma ukuntuleka kwayo, ngenxa yokuthi ukukhishwa kwamagqabha okugcoba kuncike kakhulu. Ngenxa yalokho, inqwaba yezinhlanzi iqoqa emathunjini amakhulu. Lokhu kuholela ekwandiseni nasekuqiniseni isitho.

Isifo sikaHirschsprung ezinganeni - izimpawu

Isithombe sesitholampilo sincike ekutheni indawo yangakini kanye nezinga lokungenwa kwamathumbu. Isifo sikaHirschsprung ezinsaneni sibonakala ngesimo sezimpawu ezilandelayo:

• ukungabikho kwamanzi okuqala (meconium);
• i-gassing emakwe;
• flatulence ;
• ukuhlanza, ngezinye izikhathi nge-bile;
• ukuqotshwa isikhathi eside;
• shintsha esimweni sesisu;
• umbilicus ephikisiwe noma ephansi;
• okungavamile ukuhuda.

Izifo Hirschsprung ezinganeni ezindala zihambisana nezimpawu ezengeziwe:

• ukungenakwenzeka kokuhamba kwamathumbu ngaphandle kwe-enema;
• ubuhlungu besisu;
• ukudakwa komzimba;
• ukulahla ekuthuthukiseni ngokomzimba;
• i-heterochromy eyingxenye;
• ukutholakala kwezinhlanzi ezinzima ("amatshe emathumbu");
• i-anemia ;
• ukuchithwa kwesifuba;
• i-hypotrophy;
• i-enterocolitis;
• Ukwandiswa kwamathumbu amakhulu;
• amandla okuthuthukisa ubhononi kanye ne-pneumonia;
• ukuhlanza nokuhuda ngemva kokuqotshwa isikhathi eside.

Izifo Hirschsprung ezinganeni - ukuxilongwa

Ukuze aqinisekise izinsolo ze-pathology echazwe, udokotela kuqala uhlola ngokucophelela isiguli esincane. Isifo sikaHirschsprung ezinsana singabonakala esidlangalaleni ngisho nokuzwakala, ukuzwa isisu esisendaweni yomathumbu amakhulu. Inendima ebalulekile idlalwe ukubuza abazali kwabazali. Uma umndeni unecala le-anomaly embuzweni, amathuba okuthi kwenzeke kulo mntwana akhuliswa kakhulu (isici esiyinzalo).

Ukuhlola okulinganayo kwengane enezifo zeHirschsprung

Indlela eyinhloko yokuxilonga yalesi sifo ukuhlolwa kokubukwa kanye nomunwe we-rectum (sigmoidoscopy). Isifo sikaHirschsprung sihambisana nephimbo eliphakeme le-sphincter kanye nendawo engenalutho phambi kokuvula kwesilwane, noma ngabe ukuthuthwa akuzange kwenzeke izinsuku ezimbalwa. Ukuze uthole olunye ulwazi, ucwaningo lwe-hardware lunikezwa. Izifo Hirschsprung - Ukuxilongwa:

• I-irrigoscopy ngokusungulwa kwengxube ye-barium;
• ikholonoscopy;
• i-manometry ye-anorectal;
• i-biopsy ngokuhlaziywa kwe-histological elandelayo.

Isifo se-Hirschsprung - X-ray

Uhlobo lokuhlola oluphoqelekile ekutholeni isifo esichazwe yilesi sifundo. I-radiography isiza ukuthola ukuthi impela i-megacolon (isifo se-Hirschsprung) sendawo, ukuthola ukuthi ubukhulu be-pathology buyini. Ukuhlola ikhwalithi ye-motility yamathumbu, kunconywa ukwethula ukulinganisa okuphakathi kanye ne-ultrasound eyengeziwe.

Ukwelashwa kwesifo seHirschsprung ezinganeni

Indlela kuphela ephumelelayo yokwelapha i-anomaly ekhonjisiwe kungenela ukungenela. Indlela yokugcinwa kwemvelo iyadingeka kuphela ngaphambi kokusebenza. Kuyasiza ukunciphisa nokunciphisa isifo sikaHirschsprung - ukwelashwa kuhilela ukuqeda izimpawu ze-pathology, normalizing the excretion of fecal nodokotela kanye nokubuyisela imithi yomzimba. Ngemuva kokwelashwa okubalulekile kubalulekile ukuqinisekisa ukuthi ingane inakekelwa kahle futhi ilandele ukunamathela ekudleni okukhethekile.

Isifo sikaHirschsprung ezinganeni - izincomo zomtholampilo

Uma ukuxilongwa kuqinisekisiwe futhi udokotela usemise usuku lokusebenza, kubalulekile ukulungiselela inqubo. I-Megacolon ezinganeni kuhilela izinyathelo zokwelapha ezilandelayo:

1. Ukuqedwa kwamathumbu emathunjini. Ukufakwa njalo kwe-siphon enemas ngokusungulwa kwesisombululo esifudumele se-sodium chloride concentration ye-0.9%.
2. Ukubuyiselwa kwe-microflora yamathumbu. Izingane kufanele zithathe izidakamizwa ezikhuthaza umsebenzi obalulekile wama-bacteria anenzuzo ( ama-probiotics ).
3. Ukuvikela i-immunity. Ingane imisiwe amavithamini - E, C, B6, B12.
4. Ukukhishwa kwezinyosi ngcono. Ukwenza kube njalo ukuthungwa kwamathumbu kusiza ukugaya isisu kanye nokuzivocavoca.
5. Ukuvimbela ukuqotshwa. Vimbela ukujula kwamanqamu ngokudla okunothile nge-fibre ngokuhambisana nokusetshenziswa kwamanzi ahlanzekile.
6. Ukubuyiselwa kwezinqubo ze-metabolic. Ngenkinga yokudla okunzima, izixazululo zombuso we-electrolyte, i-glucose nama-protein amalungiselelo anconywa.

Ukuhlinza izifo zeHirschsprung

Kunezinketho eziningi zokungenela okuhlinzekwa ukuqeda lesi sifo esibucayi. Zonke zazo zihlanganisa ukungahleleki kwezingosi ezonakalisiwe zomugqa oqondile nomathumbu we-sigmoid nokubeka phakathi kwezicubu ezinempilo ze-anastomosis. Kusencane, ukuhlinzwa kwaDuhamel kwenziwa ngokuyinhloko esibhedlela saseHirschsprung, kodwa kunezinye izinhlobo zezinqubo eziphumelelayo neziphephile:

• I-Rectosigmoidectomy ngokweSvenson-Hyatt-Isakov;
• i-anastomosis ejulile e-anastomosis ngokusho kukaRebein;
• Operation Soave.

Izindlela ezifakwe ohlwini zifana cishe ngokuhambisana nokusebenza, ngakho-ke ukukhetha uhlobo lokuhlinzeka ngokuhlinzwa kwenziwa ngumchwepheshe. Ebeka ukuxhashazwa okuthile okudlulele, udokotela uqondiswa yisikhathi nesimo sesiguli esincane, uhlola ukuthi ngokushesha isifo sikaHirschsprung sithuthuka kanjani. Kubalulekile ukuba abazali bathole udokotela ohlinzayo owaziwayo, yonke le misebenzi ibonakala inqubo eyinkimbinkimbi yokuqaliswa.

Izifo Hirschsprung - ukudla

Ukuze kunqunywe izimpawu ze-pathology echazwe, kuphakanyiswa ukudla okukhethekile okwe-laxative. Ukudla okunomdlavuza weHirschsprung kuhilela ukusetshenziswa kwemikhiqizo elulaza isitoreji futhi ivuselele umlotha wamathumbu:

• i-decoction ye-bran noma i-dill (yezinsana);
• Ama-aprikoti omisiwe, umlimi otholwa emculweni, ikakhulukazi uhlaziya ngokusisekelo kwawo;
• ithanga;
• ukunye okufana nokolweni, i-oats, i-porridge yengqolowa;
• usuku olulodwa kefir;
• amabhontshisi;
• ukhukhamba;
• i-yoghurt;
• isobho semifino;
• iziphazamisi ze-steam kanye ne-soufflé ezivela ekudleni okunamafutha aphansi;
• amafutha yemifino;
• amajikijolo amasha, izithelo nemifino.

Kusukela ekudleni, kumele sikhiphe konke okuqinisa nokukhipha izinqubo zokuvutshelwa emathunjini:

• amapheya;
• irayisi, i-semolina iphariji;
• isinkwa esisha esimhlophe nesinkwa;
• imikhiqizo yokubhema;
• i-bun ne-pastry;
• i-cocoa neshokoledi;
• imbewu;
• iklabishi;
• inyama enamafutha;
• ama-broth acebile kanye nesobho;
• amazambane (ngobuningi obulinganiselwe);
• i-pasta;
• i-cherry;
• amaswidi nama-creams amafutha;
• ubhontshisi;
• ubisi lonke.

Isifo sikaHirschsprung ezinganeni - imiphumela

Ngaphandle kokwelashwa okulungile nesikhathi esifanele, i-anomaly ecatshangelwe ingase iphele kabi, ikakhulukazi uma itholakale ukuthi isanda kuzalwa. Ezingama-80% zamacala anjalo, isifo sikaHarald Hirschsprung siholela ekufeni kwengane. Lapho ukuhlinzwa kwenziwa ngesikhathi futhi ngendlela efanele, izibikezelo zithuthukiswa kakhulu, cishe amaphesenti angaba ngu-90 eziguli abuyele ezingeni eliyanelisayo.

Ezinye izinkinga ezivamile zesifo sikaHirschsprung:

• i-enterocolitis enzima;
• i-anemia;
• ukuvimbela emathunjini ;
• ukuphuza ngokweqile;
• i-peritonitis;
• umkhuhlane;
• ukuphuma kwamathumbu emathunjini;
• i-fistula;
• i-stenosis;
• ukungathandeki kocingo.