Hirschsprung chirwere (HSCR) inoratidzirwa nekuoma mitezo muchikamu chekupedzisira chemuura mukuru.

Ichi chirwere chinoratidzika kubva pakuzvarwa uye mhedzisiro yekushaikwa kwenzara ganglia (maseru anogadzira bhuru panzira yetsinga) mumadziro emukati.

Kumedza kwechikafu kuburikidza nemudumbu wekugaya kusvika waburitswa, muchikamu chikuru, zvinokwanisika nekuda kwematumbu peristalsis. Iyi peristalsis igadziriro yezvidimbu zvemuviri wematumbo inobvumira kufambira mberi kweiyo bolus yechikafu padanho rekugaya.

Mune ino mamiriro apo pane kusavapo kweiyo nerve ganglia mumatumbo makuru, peristalsis haichapiwi nemuviri. Mupfungwa iyi, kuwedzera kwemukati uye kuwedzera kwehuwandu hwayo kunoitwa.

Zviratidzo zvinosanganisirwa izvi zvakanyanya kukosha kana nzvimbo yenzara ganglia yakakura. (1)

Ichi chirwere saka chinotsanangurwa neatypical matumbo zviratidzo: hutumbo kuvharika. Iko kuvharika kwekufamba uye gasi inotungamira kune kurwadziwa mudumbu, colic (ura hwechirwere), kuda kurutsa, kuzvimba, nezvimwewo.

HSCR inobata ingangoita 1 muvashanu vanozvarwa pagore. Iyo fomu inokanganisa iyo yekupedzisira chikamu chekoloni (hombe ura) inonyanya kubata vakomana. (5) Vasikana vanonyanya kutarisana nekuvandudzwa kwechirwere ichi nenzira yakapararira. (000)

Ichi chirwere chinonyanya kubata vacheche nevana vadiki. (3)

Mhando dzinoverengeka dzechirwere dzakaratidzirwa (2):

- iyo "yechinyakare" chimiro, kana inonziwo "pfupi-chidimbu chimiro". Iyi fomu inowanzoitika kune varwere vane chirwere ichi, kusvika ku80%. Iyi fomu yechirwere inokanganisa chikamu chekupedzisira chekoloni kune chikamu chechikamu;

 - iyo "refu-chikamu" fomu, iyo inosvika kune sigmoid colon, inokanganisa vangangoita 15% yevarwere;

- iyo "yakazara colic" fomu, inokanganisa iyo colon seyakazara, ine chekuita ne5% yevarwere.

Kufamba kwemukati kunodzorwa nehurongwa hwemitsipa. Tsinga ganglia saka iri mumatumbu ichibvumira kuendeswa kweruzivo kubva kuuropi kwekutonga kwematumbo peristalsis uye nekudaro kufambira mberi kwechikafu padanho rekugaya.

Kushaikwa kwenzvimbo idzi, mune chirwere cheHirschsprung, kunodzivirira chero kufambiswa kweruzivo uye nekudaro kunovharira hutumbu peristalsis. Chikafu hachichakwanise kupfuura nepamatumbo uye chinoguma chavharika mudumbu.

Zviratidzo zvechirwere ichi zvinowanzoonekwa nekukurumidza kwazvo pakuzvarwa. Zvisinei, pane dzimwe nguva, vanogona kuonekwa mushure megore rimwe kana maviri. (3)

Zviratidzo zvinobata vacheche uye vana zvinonyanya:

- matambudziko ekufambisa;

- kutadza kudzinga meconium (tsvina yekutanga yemwana achangozvarwa) mukati memaawa makumi mana nemasere;

- kupatirwa;

- jaundice;

- kurutsa;

- manyoka;

- marwadzo emudumbu;

- kushaya chikafu.

Zviratidzo zvinobata vana vakura ndezvi:

- kuvharirwa kwakanyanya nematambudziko (kutadza kukura pakureba nekurema);

- kudya kwakashata;

- dumbu distension;

- fivha.

Mwana anogona zvakare kukura hutachiona hutachiona, senge enterocolitis.

Zvimwe zvakasarudzika zvinogona zvakare kuoneka: sensorineural kunzwa kurasikirwa (Waardenburg-Shah syndrome), hurema hwehungwaru (Mowat-Wilson syndrome), central alveolar hypoventilation (Haddad syndrome), kukanganisika kwemaoko (Bardet-syndrome) Biedl), medullary thyroid cancer (yakawanda endocrine neoplasia mhando 2B) kana chromosomal zvisizvo (Down syndrome). (2)

Chirwere cheHirschsprung chinokonzereswa neyakajairika mukuvandudzwa kweiyo enteric nervous system. Iyo aganglionosis, kureva kusavapo kwenzara ganglia (inonziwo "Cajal masero") mumatumbu. Iyi lymph node kushomeka inonyanya kuwanikwa munzvimbo yekupedzisira yeura hukuru (colon).

Mune chinyorwa chakakanganiswa nechirwere ichi, chikamu chemukati ichi saka chinoramba chiri muchimiro che tonic uye chisingaperi chisingaperi. Iyi mamiriro anotungamira kumatumbu emukati. (2)

Zvese zviri zviviri magene uye zvakatipoteredza zvinhu zvakabatanidzwa mukukudziridzwa kwechirwere cheHirschsprung. (2)

Chokwadi, mamwe majeni akaratidzirwa mukukudziridzwa kweichi pathogenesis. Icho chirwere che polygenetic chinonetsekana kunyanya magene.

- Proco-oncogene ret (RET);

- iyo glial cell-yakatorwa neutrotrophic factor gene (GDNF);

- mhando B endothelin receptor gene (EDNRB);

- iyo endothelin 3 geni (EDN3);

- iyo geni ye endothelin 1 inoshandura enzyme 1 (ECE1);

- iyo geni yesero yekunamatira mamorekuru L1 (L1CAM).

Sezvambotaurwa, Hirschsprung chirwere ndicho chikonzero chekushayikwa kwenzara ganglia mumatumbo makuru kusvika kune anus, kudzivirira ura peristalsis uye nekudaro kukwira kwechikafu kusvika padanho iri.

Uku kushomeka kwemaCajal maseru (nerve ganglia) ndiwo mhedzisiro yekushomeka mukukura kwemasero aya panguva yekukura kwechi fetus. Zvinokonzera kushomeka kwemasero kukura asati aberekwa hazvisati zvazivikanwa. Zvakangodaro, mukana wehukama pakati pehutano hwakazara hwaamai panguva yekuzvitakura kwake uye kusavapo kwerudzi urwu rwemaseru ari muchibereko kwakaiswa kumberi.

Magen'a mazhinji akaratidzirwa mukukura kwechirwere. Kuvapo kwemajini aya kunogona kuitika mukati memhuri imwechete. Chikamu chekugara nhaka chinobva chave panotangira kukura kwechirwere ichi.

Uye zvakare, mamwe maratifomu anogona zvakare kunge ari emwezve njodzi mhedzisiro yekukura kwechirwere cheHirschsprung. Izvi zvinonyanya kuitika ne Down's syndrome. (3)

Iyo kusiyanisa kuongororwa kunoitwa zvinoenderana nehunhu hunoratidza chirwere chinounzwa nemusoro wenyaya: kuvharika kwematumbo, anorectal stenosis, tumarara twepamuviri, nezvimwe (2)

Iko kuongororwa kazhinji kunosanganisirwa nechirwere kunoitwa kuburikidza nerevhuru biopsy. Iyi biopsy inoratidza kuvapo kana kusavapo kwenzara ganglia mumatumbo makuru. Uye zvakare, kuwedzera kwekuwedzera kweacetylcholine esterase (enzyme inobvumira acetylcholine kuve hydrolyzed muacetic acid uye choline). (2)

Barium enema (X-ray kuongororwa kuti ufunge ura hukuru) inogona zvakare kuitwa mukuongororwa kwechirwere ichi. Iyi nzira inoita kuti zvikwanise kufungidzira nzvimbo inopfuura yekushaikwa kwemasero etsinga, zvichiratidza kukura kwechirwere cheHischsprung. Zvisinei, iyi nzira yekuongorora haisi 100% yakavimbika. Chokwadi, gumi kusvika gumi neshanu muzana ezviitiko zvehirschsprung chirwere hazvaizoonekwa mushure mekuyedza kuyedza. (10)

Mushonga wakanyanya kukosha wechirwere kuvhiyiwa. Iyo inobvumira kubviswa kwechikamu chemuura kushomeka kwemaseru etsinga. (4)

Muchiitiko chekukanganisa kwakazara kukoloni, kuisirwa koloni kunogona kudikanwa. (2)

Kutevera izvi, ostomy (yekuvhiya nzira inobvumidza kuita kubatana pakati penhengo mbiri) inogona kuitwa kuitira kubatanidza chikamu chakaitwa chemuura neiyo anus kana nechikamu chepamusoro chemuura. Iyi stoma inogona kuve yekusingaperi kana yenguva pfupi zvichienderana nenyaya yacho. (4)

Kuvhiya kunobatsira kudzikisa zviratidzo zvine hukama nechirwere ichi. Nekudaro, kufungidzira hakuna kukwana uye kunetsekana kusagadzikana kunogona kuoneka uye kuuraya.