Intrahepatic Cholestasis yeKuberekwa Zviratidzo nezviratidzo

ICP Inozivikanwa Sei uye Inoitwa Sei?

Intrahepatic cholestasis yekuzvitakura (ICP) ndiyo yechipiri inowanzokonzera chirwere chendangwana muhutumbi. Inogonawo kunzi "obstetric cholestasis". Mamiriro acho ezvinhu anosanganisira kuvaka-up ye bile acid muropa nerukanda izvo zvinokonzera kupisa kwakanyanya. Inofungidzirwa kuti inokonzerwa nekubatanidzwa kwemasimoni, mararamiro, uye zvakatipoteredza, uye kazhinji, inowanikwa mumatatu matatu ekuzvitakura.

Kuwanda

Kuwanda kweCIP kwakasiyana zvikuru kubva munyika nemunyika. MuUnited States, Switzerland, neFrance ICP inowanikwa munenge 1 mu100 kusvika 1 mu 1000 pamuviri. Zvisinei, yakawanda zvikuru pakati pevanhu vemamwe madzinza. Munharaunda yeChile yakawanda, chiitiko chacho chinenge 16 muzana, asi iyo inopfuura 28 muzana pakati peAracucanos Indians. Zvisingatauriki kudarika muChile, mamiriro acho anowanikwa muSouth Asia, dzimwe nzvimbo dzeSouth America, uye nyika dzeScandinavia kudarika muUnited States.

Kuonekwa

Chiratidzo chinowanzozivikanwa che intrahepatic cholestasis yekudzivirira chiri kutema izvo zvinowanzoitika mumatatu matatu ekuzvitakura. Kutema, kunowanzova kwakanyanya uye kwakaipisisa usiku, kazhinji kunotanga pamiti yemichindwe nemaruva, uye zvinopararira kune imwe yose yemuviri. Kukwezva kwe ICP kunokonzerwa nokucheka ganda rakanyanyisa.

Jaundice, kuputika kwechivara kweganda uye vatsvene vemaziso anoitika mu10 kubva muzana kusvika ku15 muzana yevakadzi vane chirwere.

Iro rinowanzosvikika rinoonekwa mavhiki maviri kana mana mushure mokutanga kwekuchenesa. Mushure mekusununguka, zvose zvinosvibisa uye jaundice zvinogadzirisa zvishoma nezvishoma.

Mushure mekunge mukadzi atanga ICP mune imwe pamuviri, mukana wekuti uchadzoka mumubhedha unotevera unenge 45 kusvika ku70 muzana.

Maererano nezviratidzo nezviratidzo

Mukuwedzera pakunetsa kukuru, zviratidzo nezviratidzo zve intrahepatic cholestasis yekuzvitora zvinogona kusanganisira:

Zvinokonzera

Intrahepatic cholestasis yekuzvitakura inofungidzirwa kuti inokonzerwa nekubatana kwehomoni, yezvakatipoteredza, uye zvizvarwa zvinokonzera.

Hormonally, yepamusoro yeserogen mazinga anobatwa nehutano ndeimwe chikonzero chinokosha. Intrahepatic cholestasis yekudzivirira inokonzerwa nekusawirirana kwebivha secretion pachiropa. Mahomoni anobudiswa mukuzvitakura anokonzera gallbladder (somuenzaniso, njodzi yeguruva inowedzera nehutachiwana.) Basa reguruvladder nderokuchengetedza imba yekuchengetedza bile iyo inobudiswa muchiropa. Bira, zvakare, rinoshandiswa kuputsa mafuta mumugwagwa wekudya. Apo bhati duct yakavharwa, bile acid back back in the liver. Sezvo uwandu hweB bile muropa hunowedzera, hunoputika muropa remvura. Ndizvo bile acids iyo inopinda muropa uye inoiswa mucheche inokonzera kupisa kwakanyanya. Estrogen inopesana nekubviswa kwebibi kubva muchiropa uye progesterone kunopesana nekubvumirana kweesrogen kubva pachiropa.

Utachiona hwemahomoni akadai saestrogen uye progesterone inenge chikamu chikamu chakapamhi panguva yekuzvitakura kupfuura apo mukadzi asina pamuviri.

Genetic inokonzera kuita basa, uye chirwere chinowanzomhanya mumhuri. Zvimwe zvipembenene zvemashoko zvakabatanawo nehuwandu hwehutano. Inenge 15 kubva muzana yevakadzi vane ICP inoita seine kuchinja (chaizvoizvo kuchinja kwakasiyana-siyana) mune adenosine triphosphate binding cassette, subfamily B, nhengo 4 (ABCB4 / abcb4) gene (inonziwo zvinodhaka zvinopesana nezvinokonzerwa nemishonga 3 (MDR3).

Zvimwe zvinhu zvinokonzerwa nehutachiona zvinoitawo seine mamwe mabasa, pamwe nemamiriro ezvinhu anonyanya kuwanwa munguva yechando uyewo inobatanidza nekushayikwa kwemaminerari selenium.

Risk Factors

Pane mamiriro ezvinhu akawanda anosimudza dambudziko rekuvaka ICP. Zvakakosha kucherechedza kuti izvi hazvisi izvo zvinokonzera, asi zvinongobatanidza nehuwandu hwehutano hwekuti chiitiko chichaitika. Zvimwe zvinokonzera ngozi zvinosanganisira:

Kuziva

Kuongororwa kweCPP kunowanzoenderana nekanyatsocherechedza uye muviri, pamwe neropa rekuyera rinoratidza huwandu hwakareba hwebe salt uye mamwe mavhinga eropa (chiropa function test). Kuvapo kwekuchenesa pasina kubhururuka kunobatsirawo kusimbisa kuongororwa. Chiropa chinoputika kana kuti ultrasound hachidikanwi kudiwa kuti chigadziriswe. Kakawanda, ICP inonyanya kuongororwa kwekuregererwa (kusabatanidzwa zvimwe zvinokonzera kukonzera jaundice uye kubvisa panguva yekuzvitakura.)

Kutarisa pane imwe nzira yekuongorora, serum bile acids inowanzova yakakura kupfuura 10 (uye inogona kunge yakareba se40.) Chiropa chekuita zvinowanzoswedzerwa zvakanyanya. Serum bilirubin inowanzokwidziridzwa, asi kazhinji isiri pasi pechishanu. Labs zvakare inogona kuratidza huwandu hunowedzera hweliclic acid, chenoeoxycholic acid, uye alkaline phosphatase.

Zvimwe Zvinokonzera Jaundice Mukubata

ICP inonyanya kuongororwa-kureva kuti kuongororwa kunowanzoitwa nekusaisa zvimwe zvinokonzera kukonzera jaundice uye kubvisa. Mimwe mamiriro ezvinhu anogona kuenzanisa zvimwe zviratidzo zve ICP dzinosanganisira:

Matambudziko kune Amai

Izvo zvinetso zve ICP, kunze kwekuchenesa izvo zvinogona kuva zvakanyanya, zvinowanzosanyanya kuderera kumucheche kupfuura amai. Urinary tract infections inowanzoonekwa kune vakadzi vane ICP kupfuura vakadzi vasingatauri vane pamuviri. Mukuwedzera, vitamin K inoshaya inogona kuguma mushure mekufamba kwenguva yakareba ye ICP, iyo inogonawo, inokonzera matambudziko ekubuda ropa.

Zvinetso zveMucheche

ICP inogona kuva yakakomba zvikuru kumucheche, zvichikonzera kufa kwepakutanga uye intrauterine kufa (vana vasati vaberekwa.) Ndinofara, mushonga mutsva kuvanaamai vane ICP uye kunyatsongwarira kwevana vakaguma nehuwandu hwakaoma hwemucheche kupfuura zvekare.

Chiropa chemucheche ane utano ine simba rakakwana rokubvisa bile acids kubva muropa. Iyo fetus inowanzofanira kuvimba nechiropa chekuberekwa kuita basa iri. Nokudaro, huwandu hwakakosha hwebumbi hwemamai hunokonzera kushungurudzika muropa re fetal. Kutungamirirwa kwezviitiko izvi zvinokurukurwa pasi apa.

Intrahepatic cholestasis yekuzvitakura inowedzera ngozi kune mwana we meconium kudonha panguva yekubereka, kutangira kusununguka, uye intrauterine kufa . Vakadzi vane ICP vanofanira kunyatsoongororwa, uye kunyatsofungisisa kunofanira kupiwa kukurudzira vashandi mushure mekunge fetal lungurudza yakasimbiswa.

Kurapa

Pamusana pezvimwe zvinetso zvemucheche, kurapwa kwe ICP kunofanira kutanga pakarepo mushure mekunge chirwere chacho chinowanikwa. Kurapa nzira dzinosanganisira zvose izvo zvakagadzirirwa kubvisa bile acid uye nzira dzinotsigira kuti dzidzora zviratidzo. Mukuwedzera, kutarisa pedyo kwecheche kunokosha.

Iyo yakanakisisa kurapwa uye "muchengeti wekutarisira" ye intrahepatic cholestasis yekuzvitakura ursodeoxycholic acid kana UDCA . Iyi mishonga inowanzotangwa pakarepo uye inopfuurira kuburikidza nekuberekwa. Kusiyana nemarapirwo apfuura, UDCS inoratidzika kuti inonyanya kukosha migumisiro yemadzimai nemwana ne ICP. Hazvinyatsoziva kuti mushonga uyu unoshanda sei.

Nekushandiswa kweUDCA, kuchenesa kunowedzera mumadzimai matatu kubva kuvakadzi uye kunogona kuita kuti kupera zvachose kwechiitiko ichi kusvika 25 muzana. Sezvo vanaamai vanowanzonyanya kunetseka pamusoro pemucheche wavo kupfuura ivo, zvinoguma nekushandiswa kwechirapa ichi zvinogona kusimbisa. Vakadzi vane pamuviri vanobatwa neUDCA vane vana vashoma vasati vaberekwa , vana vacho havasati vava nehutano hwemucheche kana kupemha kunetseka , uye havawanzodi kuti vabvumirwe nevana vasati vakura. Vacheche vanaamai vakabatwa neUDCA vanoitawo kuberekwa gare gare-panguva yakanyanya kufema kune vakomana vane vanaamai vasina kubatwa.

Mimwe mishonga yakave yakashandiswa nemhaka yemigumisiro yavo yakavhara, kunyange isiri yashanda, inosanganisira S-adenosylmethionine (SAMe) uye cholestyramine. Cholestyramine, kunyanya, inoratidzika kunge isina kusimba, uye inogonawo kupisa pasi mavhamisiki ediki eVitamin K anowanzowanikwa. Mvura yakanyanya steroid yemuromo yakadai sa dexamethasone inogonawo kuva yakakosha kurapa ICP.

Kuve kwe ICP kunogona kurapwa ne emollients, antihistamines, zvinotonhora mabhati, mafuta ekutanga, uye anti-itching michina yakadai seSarna. Heano mamwe mazano ekuti iwe ungaisa sei kumira, asi avo vasina kurwara nehutano vanofanira kuziva kuti kubvumira kunoguma kubva mumamiriro ezvinhu aya hakusi kuchenesa. Vamwe vanhu vakataura kuti vanganyanya kukurira kurwadziwa kupfuura rudzi urwu rwekushaya, uye vamwe vanhu vakatotanga kuzviuraya. Kana mudiwa wako ari kurwisana neI ICP, mumutsigire nenzira ipi zvayo yamunogona.

Kutarisira Kubata

Chikamu chakakosha chekutarisira mwana ane amai vane ICP ndechokugadzirira kupa nekukurumidza kana kukura kwemapapu emucheche kwakanyorwa. Nhoroondo, nguva ino yave ichitarirwa mavhiki makumi matatu, asi nekuwanikwa ikozvino kweUDCA, vamwe pamuviri vakabvumirwa kufambira mberi kwenguva yakareba kupfuura iyi.

Isati yasvika, inokurudzirwa kuti vanaamai vane kechipiri vhiki nevhiki mushure mekutadza kusinganzwisisiki . Kunzwa pamusoro pengozi yekuberekwa kunogona kuva kushungurudzika zvachose kuvhiringidzi vevakadzi vari kurarama nemamiriro acho ezvinhu. Ndinofara, vanogona kutora humwe husimbiso muchokwadi chokuti fetal death inobatana ne ICP inowanikwa vasati vasvika mavhiki makumi matatu nemaviri.

Mune zvimwe zvidzidzo, kuitika kwemeconium kudonha panguva yekubereka kwave kuchikwidziridzwa, saka kutora kunofanira kuitika mune imwe nzvimbo iyo chigadziri chakagadzirira kupinda chero chero chipi zvacho chaanoda kuti chidzivirire aspiration (chengetedza mwana kubva pakutsvaga meconium) iyo inogona kukonzera meconium aspiration syndrome.

ICP uye Hepatitis C

Masayendisiti haazive kuti zvakakosha zvakadini, asi vakadzi vane chirwere chisingaperi chekushaya chipatara C zvinowanzoita kuti vawane ICP, uye vakadzi vane ruzivo rwe ICP vanowanzoonekwa kuti vane zvirwere zvisingaperi zvehutachipatachi C. Vaya vanoona ICP vangada kutaura nevanachiremba nezvekuongorora kwehepatitis C.

Kurarama ne ICP

Kana iwe wakamboongororwa ne ICP iwe unogona kunge uchitya-zvose nekuda kwako uye zvemucheche wako. Ndinofara, kurapwa kwechiitiko ichi kwakagadzirisa zvinoshamisa, kuderedza ngozi kune vose amai nechecheche. Mukuwedzera, kunyatsochenjerera kwevana vakaderedza njodzi yezvinetso zvinoshungurudza zvakadai sekuberekwa, muna 2016 kuongororwa kutsvaga kusina kuberekwa pakati peboka revakadzi vakarapwa uye kunyatsoongororwa mushure mekutsvaga kwavo.

Kurapa kuri kuitawo kuti zvibvirire kunonoka kutumira kusvika mwana anenge achikwanisa kukura kusvika pane imwe nzvimbo iyo kufema kunetseka hakusi kunetseka.

Kunyange zvakadaro, ramba uchiyeuka kuti chero chinetso chekuzvitakura chinoshungurudza. Kumbira uye kubvuma rubatsiro. Vamwe vanhu vanoona zvichibatsira kuwana mapoka ekutsigira uye kutaura kune vamwe vakadzi vakararama nemamiriro ezvinhu. Shoko rekuchenjerera riri mukutenda kana ukaita izvi, zvakadaro. Zvakawanda zvekubudirira kwechangobva kuitwa uye kufambira mberi mukurapa kwave ikozvino-uye avo vamunogona kutaurirana nevaya vakatarisana nechirwere kunyange gore kana kupfuura yakapfuura vangangodaro vakatarisana nemitemo yakasiyana.

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