Cutar Hirschsprung (HSCR) tana halin gurguwa a cikin ɓangaren babban hanji.

Wannan cututtukan yana bayyana daga haihuwa kuma shine sakamakon rashi na ganglia jijiya (ƙwayoyin da ke haifar da kumburi a kan hanyar jijiya) a bangon hanji.

Ciyar da abinci ta hanyar narkar da abinci har sai an fitar da shi, a mafi yawan lokuta, mai yiwuwa ne godiya ga peristalsis na hanji. Wannan peristalsis wani salo ne na tsokar hanji wanda ke ba da damar ci gaban bolus na abinci tare da narkar da abinci.

A cikin wannan yanayin inda babu raunin jijiyoyin jijiyoyin jiki a cikin babban hanji, jiki baya ba da peristalsis. A wannan ma'anar, an ƙirƙiri fadada hanji da haɓaka ƙarar sa.

Alamu masu alaƙa duk suna da mahimmanci idan yankin na ganglia jijiya yana da girma. (1)

Saboda haka an bayyana wannan cutar ta alamun cututtukan hanji na hanji: toshewar hanji. Yana toshe hanyar wucewa da iskar gas wanda ke haifar da ciwon ciki, colic (ciwon hanji), tashin zuciya, kumburin ciki, da sauransu.

HSCR yana shafar kusan 1 cikin haihuwa 5 a kowace shekara. Siffar da ke shafar ɓangaren hanji na hanji (babban hanji) yafi shafar samari. (000) 'Yan mata sun fi fuskantar ci gaban wannan cuta ta hanyar yaduwa. (2)

Wannan cuta ta fi shafar jarirai da yara ƙanana. (3)

An nuna nau'o'in cutar da dama (2):

-siffar “classic”, ko kuma ana kiranta da “gajeriyar sashi”. Wannan nau'in yafi kowa a cikin marasa lafiya da wannan cutar, har zuwa 80%. Wannan nau'in cutar yana shafar ɓangaren ƙarshe na hanji zuwa sashin dubura;

 -nau'in “dogon-sashi”, wanda ya faɗaɗa zuwa sigmoid colon, yana shafar kusan 15% na marasa lafiya;

- nau'in “jimlar colic”, wanda ke shafar hanji gaba ɗaya, ya shafi 5% na marasa lafiya.

Tsarin jijiyoyin jiki yana sarrafa hanyar wucewa ta hanji. Saboda haka ganglia na jijiya yana cikin hanji yana ba da damar canja wurin bayanai daga kwakwalwa don sarrafa peristalsis na hanji don haka ci gaban abinci tare da narkewar abinci.

Rashin waɗannan nodes, a game da cutar Hirschsprung, yana hana kowane watsa bayanai don haka yana toshe peristalsis na hanji. Abinci baya iya wucewa ta hanji kuma ya ƙare a toshewa a cikin narkar da abinci.

Alamun kamuwa da wannan cuta galibi ana iya ganinsu da wuri tun suna haihuwa. Koyaya, a wasu lokuta, suna iya bayyana bayan shekara ɗaya ko biyu. (3)

Alamomin da ke shafar jarirai da yara galibi:

- matsalolin wucewa;

- rashin iya fitar da meconium (abubuwan farko na jariri) a cikin awanni 48 na farko;

- maƙarƙashiya;

- jaundice;

- amai;

- gudawa;

- ciwon ciki;

- rashin abinci mai gina jiki.

Alamomin da ke shafar manyan yara sune:

- maƙarƙashiya mai tsanani tare da rikitarwa (rashin bunƙasa a tsayi da nauyi);

- rashin abinci mai gina jiki;

- karkacewar ciki;

- zazzabi.

Haka kuma yaron na iya kamuwa da cututtukan hanji, kamar enterocolitis.

Ƙarin abubuwan da ba a saba gani ba na iya kasancewa a bayyane: raunin ji na jijiya (Waardenburg-Shah syndrome), raunin hankali (cutar Mowat-Wilson), tsakiyar alveolar hypoventilation (Haddad syndrome), munanan ƙafa (Bardet-syndrome) Biedl), medullary thyroid cancer (endocrine da yawa) neoplasia type 2B) ko rashin lafiyar chromosomal (Down syndrome). (2)

Cutar Hirschsprung tana faruwa ne ta hanyar rashin daidaituwa a cikin ci gaban tsarin jijiyoyin jini. Aganglionosis ne, watau babu raunin jijiya ganglia (wanda kuma ake kira “sel Cajal”) a cikin hanji. Wannan raunin kumburin kumburin lymph ya fi kasancewa musamman a cikin ɓangaren babban hanji (colon).

A cikin batun da wannan cutar ta shafa, wannan ɓangaren hanji saboda haka yana cikin yanayin tonic da ƙuntatawa na dindindin. Wannan yanayin yana haifar da toshewar hanji. (2)

Duka abubuwan da suka shafi kwayoyin halitta da muhalli sun shiga cikin ci gaban cutar Hirschsprung. (2)

Lallai, an nuna wasu kwayoyin halitta a cikin ci gaban wannan ƙwayar cuta. Cutar polygenetic ce wacce ta shafi musamman kwayoyin halitta:

- Proco-oncogene ret (RET);

-glial cell-samu neutrotrophic factor gene (GDNF);

- nau'in B endothelin gene receptor gene (EDNRB);

- endothelin 3 gene (EDN3);

- gene don endothelin 1 mai canza enzyme 1 (ECE1);

- kwayar halittar kwayar halittar adhesion kwayar halitta L1 (L1CAM).

Kamar yadda aka fada a baya, cutar Hirschsprung ita ce sakamakon raunin ganglia a cikin babban hanji har zuwa dubura, yana hana peristalsis na hanji don haka hawan abinci zuwa wannan matakin.

Wannan rashi na ƙwayoyin Cajal (ganglia jijiya) shine sakamakon rashi a cikin haɓakar waɗannan sel yayin haɓaka tayi. Har yanzu ba a san abubuwan da ke haifar da wannan rashin ci gaban sel ba kafin haihuwa. Duk da haka, an gabatar da yuwuwar alaƙa tsakanin lafiyar gaba ɗaya na mahaifiyar a lokacin da take ciki da kuma rashin irin wannan sel a cikin tayi.

An nuna kwayoyin halittu da yawa a ci gaban cutar. Kasancewar waɗannan kwayoyin halittar na iya zama da yawa a cikin iyali ɗaya. Wani bangare na gado zai kasance a asalin ci gaban wannan cutar.

Bugu da ƙari, wasu cututtukan na iya zama ƙarin haɗarin haɗari dangane da ci gaban cutar Hirschsprung. Musamman yanayin yanayin Down syndrome. (3)

Ana yin ganewar banbanci gwargwadon halayen cututtukan da cutar ke gabatarwa ta hanyar: hanjin hanji, rashin kumburin ciki, kumburin pelvic, da sauransu (2)

An gano mafi yawan cututtukan da ke da alaƙa da cutar ta dubura ta dubura. Wannan biopsy yana nuna kasancewar ko babu jijiyoyin ganglia a cikin babban hanji. Bugu da ƙari, yawan wuce gona da iri na acetylcholine esterase (enzyme da ke ba da damar sanya hydrolyzed cikin acetic acid da choline). (2)

Ana iya yin gwajin barium enema (gwajin X-ray don ganin babban hanji) a cikin ganewar wannan cutar. Wannan hanyar tana ba da damar hango wani yanki mai wucewa na rashin ƙwayoyin jijiya, yana nuna ci gaban cutar Hischsprung. Koyaya, wannan dabarar bincike ba abin dogaro bane 100%. Tabbas, kashi 10 zuwa 15% na cututtukan Hirschsprung ba za a gano su ba bayan wannan yunƙurin bincike. (4)

Mafi mahimmancin maganin cutar shine tiyata. Yana ba da damar kawar da ɓangaren hanji wanda ke da ƙarancin ƙwayoyin jijiya. (4)

Game da lalacewar hanji gaba ɗaya, jujjuyawar mahaifa na iya zama dole. (2)

Bayan wannan, ana iya yin istim ɗin (dabarar tiyata da ke ba da damar yin haɗin gwiwa tsakanin gabobin biyu) don haɗa sashin hanji mai aiki da dubura ko tare da ɓangaren hanji. Wannan stoma na iya zama na dindindin ko na ɗan lokaci dangane da yanayin. (4)

Yin tiyata yana taimakawa rage alamun cutar da ke tattare da cutar. Koyaya, hasashen bai cika ba kuma matsalolin kumburi na iya bayyana kuma su zama masu mutuwa.