I-ICP ichongwa njani kwaye iphathwa njani na?
I-cholestasis ye-Intrahepatic yokukhulelwa (ICP) yeso sibini esona sizathu esiqhelekileyo se-jaundice ekukhulelweni. Kwakhona kungabizwa ngokuba yi-cholestasis engumqobo. Le meko ibandakanya ukwakheka kwe-bile acids egazini kunye nesikhumba okubangela ukuba kube nzima. Kucatshangelwa ukuba kubangelwa udibaniso lwe-hormonal, i-genetic, kunye neemeko zendalo, kwaye ngokuqhelekileyo, kwenzeka kwi-trimester yesithathu yokukhulelwa.
Ukukhula
Ubuninzi be-ICP buhluka kakhulu kwilizwe elizwe. E-United States, eSwitzerland, naseFransi ICP ivela ngo-1 kwi-100 ukuya kwi-1 kwi-1000 yokukhulelwa. Nangona kunjalo, kuxhaphake kakhulu kubantu bezinye iintlanga. Kwisizwe sabantu baseChile, iziganeko ziyi-16 ekhulwini, kodwa ziphakamileyo ezingama-28 kwi-Aracucanos Indiya. Engaqhelekanga kunaseChile, imeko ixhaphakile kwi-Asia yaseMzantsi, ezinye iindawo zaseMzantsi Melika kunye namazwe aseScandinavia kunase-United States.
Kubonakala
Uphawu oluqhelekileyo lwe-cholestasis ye-intrahepatic yokukhulelwa lubuhlungu obuya kukhula kwi-trimester yesithathu yokukhulelwa. Ukubetha, okuqhelekileyo kunzima kwaye kukhange kakhulu ebusuku, ngokuqhelekileyo kuqala kwiintendelezo kunye neengqimba, kwaye kwandula kwandiswe kuwo wonke umzimba. Ukukhawuleza kwe-ICP kubangelwa ukukhanda isikhumba esiqhenqileyo.
I-jaundice, ukugqithisa okwesibhakabhaka kwesikhumba kunye nabamhlophe bamehlo kwenzeka kwi-10 ekhulwini ukuya kuma-15 ekhulwini kwabasetyhini abanesifo.
Ukugqithisa ngokuqhelekileyo kubonakala iiveki ezimbini ukuya kwezine emva kokuqala kokubamba. Emva kokunikezelwa, kokubambisa kunye ne-jaundice kusombulula ngokukhawuleza.
Emva kokuba ibhinqa lenze i-ICP ngokukhulelwa okukodwa, ithuba lokuba liza kuphinda likhulelwe li-45 ukuya kuma-70 ekhulwini.
Iimpawu kunye neeMpawu
Ukongeza kokubamba kakhulu, iimpawu kunye neempawu ze-cholestasis ye-intrahepatic yokukhulelwa zingabandakanya:
- Jaundice
- I-Rash ngenxa yokutshitshiswa (ukukhutshwa)
- Ukulahlekelwa ngumdla (i-anorexia)
- Ukukhathala
- Imibala ekhanyayo kunye nezihlalo ezityebileyo ezijikelezayo (i-steatorrhea)
- Umchamo omnyama
- Ubuhlungu kwi-quadrant ephezulu kunye nommandla wesibini wesisu
- Ukuxinezeleka
- I sizathu
Izizathu
I-intrahepatic cholestasis yokukhulelwa kucingelwa ukuba yimbangela yentlangano ye-hormonal, yendalo, kunye nemfuza.
I-Hormonally, amanqanaba aphezulu e-estrogen ahlobene nokukhulelwa yimbangela ebalulekileyo. I-cholestasis ye-Intrahepatic yokukhulelwa ibangelwa ukuphazamiseka kwesibindi se-bindi esibindi. IiHormone eziveliswe xa ukhulelwa zichaphazela i-gallbladder (umzekelo, umngcipheko we-gallstones ukwandiswa ngokukhulelwa.) Umsebenzi we gallbladder kukusebenza njengendlu yokugcina i-bile eveliswa esibindi. I-Bile, isetyenziselwa ukuphula amafutha kwindawo yokutya. Xa i-buct ye-bile ivinjiwe, i-bile acids isiphakamiso kwisibindi. Njengoko inqanaba le-bile kwisibindi landa, liye lafikelela kwigazi. Zizo zi-acid acids ezifaka igazi kunye kwaye zifakwe elukhumbeni ezibangela ukuba zibuhlungu kakhulu. I-Estrogen iphazamisa ukukhutshwa kwe-bile kwisibindi kunye neprogesterone iphazamisa ukuvunyelwa kwe-estrogen kwisibindi.
Amanqanaba eHormone njenge-estrogen kunye ne-progesterone zi malunga namawaka angama-1000 aphakamileyo ngexesha lokukhulelwa kunokuba umfazi engakhulelwe.
Iimbangela ze-Genetic zidlala indima, yaye eso sifo sixhaphake kakhulu kwiintsapho. Ezinye izigulo zentsholongwane nazo zidibene nomngcipheko ophezulu. Amaphesenti angama-15 amabhinqa abane-ICP abonakala enokutshintshisana (okwenziwe utshintsho oluhlukeneyo) kwi-adenosine triphosphate ephothiweyo ikhaseteti, indlu encinane B, ilungu 4 (i-ABCB4 / abcb4) yemfuza (ikwabizwa ngokuba yiprotheni engapheliyo ye-3 (MDR3).
Imiba yendalo iyabonakala inendima ethile, kunye nemeko eqhelekileyo ebusika kwaye ihambelana nokusilela kwe-mineral selenium.
Izinto zobungozi
Kukho iimeko ezininzi eziphakamisa umngcipheko wokuphuhlisa i-ICP. Kubalulekile ukuqaphela ukuba ezi zinto azibangeli, kodwa zihambelana nomngcipheko ophezulu ukuba imeko iya kwenzeka. Ezinye izinto ezinobungozi ziquka:
- Imbali yomntu we-ICP (njengoko kuphawuliwe kwangaphambili, imeko ibuyeka emva kokukhulelwa okulandelayo malunga nesiqingatha sexesha)
- Imbali yentsapho ye-ICP (abafazi abanomama okanye udade oye waba ne-ICP basengozini enkulu yokuphuhlisa imeko
- Imbali yentsapho yokuphuhliswa kwamatye ngelixa ithatha i-contraceptive ngomlomo
- Ubume belizwe - Njengoko kuphawuliwe ngaphambili, imeko ixhaphake kakhulu kwezinye iindawo zehlabathi, njengeChile
- Umdala osekhulile
- Ukwahlula (kuba nabantwana abaninzi)
- Iindidi ezininzi - i-ICP iphindwe ngokuphindwe ezintlanu ngokuphindaphindiweyo kwezibini ezikhulelweyo kunezo zokukhulelwa kwabantwana abangabodwa
- Imbali ebalulekileyo yokusetyenziswa komlomo ngomlomo
- Abasetyhini abanomdla kwi-estrogen (njengaleyo abaye bafumana iingxaki ngokuthatha izithintelo zomlomo ngexesha elidlulileyo) kubonakala ngathi bayingozi
- I-ICP ixhaphake kakhulu kwiinyanga zasebusika
Ukuxilongwa
Ukuxilongwa kwe-ICP ngokuqhelekileyo kusekelwe kwimbali echanekileyo kunye nomzimba, kunye neemvavanyo zegazi ezibonisa indawo ephakamileyo ye-salt salts kunye ne-enzyme ezithile zesibindi (iimvavanyo zomsebenzi wesibindi). Ubungqina bokubamba ngaphandle kokugqithisa okuphambili kukunceda ukuqinisekisa ukuxilongwa. I-biopsy yesibindi okanye i-ultrasound ayifanelekanga ukuba iqinisekise ukuxilongwa. Ngokubanzi, i-ICP ngokuyinhloko isifo sokuxilongwa (ngaphandle kokunye okubangela i-jaundice kunye nokubetha ngexesha lokukhulelwa.)
Ukujonga iimvavanyo ezithile zebhabhi, i-serum bile acids ihlala iphakamileyo kune-10 (kwaye ingaba iphakamileyo njengama-40.) Izilingo zomsebenzi wesibindi zivame kakhulu. I-Serum bilirubin ivame ukuphakanyiswa, kodwa ngokuphindaphindiweyo ingaphantsi kwemihlanu. Amabhanki nawo angabonisa izinga eliphezulu le-aclic acid, i-chenoeoxycholic acid kunye ne-alkaline phosphatase.
Ezinye izizathu ze-Jaundice ekukhulelweni
I-ICP ngokuyininzi i-diagnosis-excluding-ithetha ukuba ukuxilongwa ngenye indlela kwenziwa ngokungabandakanyi ezinye izizathu ezibangelwa i-jaundice kunye nokubetha. Ezinye izimo ezinokulinganisa ezinye iimpawu ze-ICP ziquka:
- Isibindi esinamafutha aphezulu sokukhulelwa
- I-HEELP syndrome kunye nesifo sesibindi esandulela eclamptic
- Ezinye izifo zesikhumba zokukhulelwa (ezinokubangela ukugqithisa kunye nokubetha kodwa kungekhona iimvavanyo zesibindi ezingavamile okanye i-jaundice)
- Gallstones
- Izimo ezingabandakanywa nokukhulelwa ezibandakanya i-virus ye-hepatitis, i-hepatitis, kunye nesifo esingapheliyo sesibindi.
Iingxaki kuMama
Iingxaki ze-ICP, ngaphandle kokubamba okunokuba nzima kakhulu, ngokuqhelekileyo kunzima kakhulu kumntwana kunomama. Izifo ezithintekayo kwi-urinary ziqhelekileyo kubasetyhini abane-ICP kunabesetyhini abakhulelweyo abangenayo. Ukongezelela, ukungabikho kwe-vitamin K kunokuphumela emva kwekhosi yexesha elide le-ICP, leyo leyo ingakhokelela kwiingxaki zokuphuma kwegazi.
Iingxaki zeNtsana
I-ICP ingaba yinto enzima kakhulu kubantwana, okubangelwa ukuhanjiswa kwangaphambili kunye nokufa kwangaphakathi kwegazi (iintsana ezingaphange zizalwe.) Ngombulelo, unyango olutsha olumama kunye ne-ICP kunye nokubeka iliso ngokugqithiseleyo iintsana luye lwabangelwa ubunzima obuncinane kubantwana kunakudala.
Isibindi somntwana onempilo sinokukwazi ukukhupha i-bile acids egazini. Umntwana ngokuqhelekileyo kufuneka athembele kwisibindi sokubeletha ukwenza lo msebenzi. Ngako oko, amanqanaba aphakamileyo obuninzi bebele abangela uxinzelelo kwisibindi sesisu. Ulawulo lo mngcipheko luxutyushwa ngezantsi.
I-cholestasis ye-Intrahepatic yokukhulelwa ikhulisa umngcipheko kwintsana ye-meconium ephahleni ngexesha lokunikezelwa, ukuhanjiswa kwangaphambili, kunye nokufa kwangaphakathi kwe- intrauterine . Abafazi kunye ne-ICP kufuneka bahlolwe ngokusondeleyo, kwaye kufuneka kuqwalaselwe ingqalelo ekunciphiseni abasebenzi ngokukhawuleza xa kukhula ukukhula kwemiphunga.
Unyango
Ngenxa yeengxaki ezibangelwa umntwana, unyango lwe-ICP kufuneka luqale ngokukhawuleza emva kokuba isifo sifumaneka. Iindlela zonyango ziquka zombini ezo zenzelwe ukuqeda i-bile acid kunye nezindlela zokuxhasa ukulawula iimpawu. Ukongezelela, ukujonga esondeleyo umntwana kubalulekile.
Okona unyango olungcono kunye "nomgangatho wokunyamekela" we-cholestasis ye-intrahepatic yokukhulelwa ngu- ursodeoxycholic acid okanye i-UDCA . La mayeza athatyathwa ngokukhawuleza kwaye aqhubeke ngokuziswa. Ngokuphambene nezo kunyango zangaphambili, ii-UDCS zibonakala ziphucula kakhulu iziphumo zomama nomntwana kunye ne-ICP. Akuqinisekanga ukuba lisebenza njani leyeza.
Ngokusetyenziswa kwe-UDCA, ukubetha kuphucula kubafazi abathathu kwabasetyhini kwaye kungabangela ukunyamalala ngokupheleleyo kwimeko ukuya kuma-25 ekhulwini. Ekubeni oomama bahlala bexhalabele kakhulu ngentsana yabo kunabo, iziphumo ekusebenziseni le nyango ziyakhuthaza. Abafazi abakhulelweyo baphathwe ne-UDCA banokuzalwa okungaphantsi kwezibeleko , iintsana azikwazi ukufumana uxinzelelo lwe-fetal okanye ukuphefumula kwesifo sokugula , kwaye akunakwenzeka ukuba zivunyelwe ukuba zivunywe kwi-unit health care unit. Iintsana ezinomama oye waphathwa ngayo ne-UDCA nazo zivame ukuzalwa kamva-kwixesha eliphambili kakhulu lokunyuka kwamantombazana kunabantwana abanomama abangaphathwa.
Olunye unyango oluye lwasetyenziswa ngenxa yemiphumo yabo kwi-secretion ye-bile, nangona kungasebenzi ngaphantsi, kuquka uS-adenosylmethionine (SAMe) kunye ne-cholestyramine. I-Cholestyramine, ngokukodwa, ibonakala ingaphumeleli, kwaye ingaba yingozi nakakhulu amanqanaba aphantsi e-vitamin K afunyenweyo. I-oral stroids oral dose efana ne-dexamethasone ingaba yonyango oluya ku-ICP.
Ukutshiza kwe-ICP kungaphathwa nge-emollients, antihistamines, iibhotile ezithunga, i-primrose ioli kunye nemveliso echasayo njengeSarna. Nazi ezinye iingcebiso malunga nendlela yokubeka ukuyeka, kodwa abo abangabhekana neengxaki kufuneka baqaphele ukuba ukukhangela okubangelwa yilo mqathango akuyona into eqhelekileyo yokubamba. Abanye abantu baye bathetha ukuba banokukwazi ukujamelana nokubuhlungu kunolu hlobo lokubamba, kwaye abanye abantu baye bazama ukuzibulala. Ukuba umhlobo wakho uhlangabezana ne-ICP, mxhase nayiphi na indlela onokuyenza.
Ukulawula Ukukhulelwa
Inxalenye ebaluleke kakhulu yokulawula umntwana onoMama ene-ICP kukucwangcisa ukuhanjiswa ngokukhawuleza xa ukukhula kwemiphunga ye-fetal ibhalwe. Ngokomlando, eli xesha liye laqwalaselwa ngeeveki ezingama-37, kodwa ngokufumaneka kwale-UDCA, ukukhulelwa okunye kuye kwavunyelwa ukuba kuqhubele phambili kunoko.
Ngaphambi kokunikezelwa, kunconywa ukuba oomama banokuphindwa kabili kwiintsuku zokuvavanya ingcinezelo yentsana . Ukuva malunga nomngcipheko wezilwanyana ezinokutshatyalaliswa kunokubangela uxhalabhiso ngokupheleleyo kwabasetyhini abajongene nale meko. Ngombulelo, banokuyifumana isiqiniseko kwinto yokuba ukufa kwe-fetal ehambelana ne-ICP ayinqabile phambi kweeyonto ezingama-36.
Kwezinye iinjongo, iziganeko ze- meconium ukukhangela ngexesha lokunikezelwa liphakanyisiwe, ngoko ke ukuhanjiswa kufuneka kuqhutyelwe kwimeko apho umgqirha afikelele ukulungelelanisa nayiphi na into ayenokuyimfuneko ukukhusela isifiso (gcina umntwana ukuba angene kwi-meconium) enokuthi bangela i-meconium aspiration syndrome.
ICP kunye ne-Hepatitis C
Iingcali zenzululwazi aziqinisekanga ngokubaluleka okubalulekileyo, kodwa abafazi abanesifo esingapheliyo sesifo se-hepatitis C banokuthi bavelise i-ICP, kwaye abafazi abaye bafumana i-ICP banokufumaneka ukuba banokusuleleka kwizifo ezingapheliyo ze-hepatitis C. Abo bafumana i-ICP banqwenela ukuthetha noogqirha babo malunga nokuhlolwa kwe-hepatitis C.
Ukuphila Nge-ICP
Ukuba ufumene uvavanyo lwe-ICP usenokwesaba-zombini ngenxa yakho nakwintsana yakho. Ngombulelo, unyango lwalo mqathango luye lwaphucula ngokuphawulekayo, ukunciphisa ingozi kumama kunye nosana. Ukongezelela, ukubeka iliso kwintsana kunciphise ingozi yokuxakeka kwengxaki efana nokuzalwa komntwana, ngo-2016 ukufundwa kungabikho ukubeleka phakathi kweqela labasetyhini abaye baphathwa kwaye bavavanyiswa emva kokuxilongwa kwabo.
Unyango wenza ukuba kunokwenzeka ukulibaziseka ukuhanjiswa de kube umntwana ekhulile ukuya kwindawo apho ukukhathazeka okuphefumulayo akukhathalelekanga.
Sekunjalo, khumbula ukuba nayiphi na inkathazo yokukhulelwa kuyingozi. Cela kwaye wamukele uncedo. Abanye abantu bakuthola kukunceda ukufikelela kumaqela enkxaso kwaye uthethe nabanye abafazi abaye bahlala nale meko. Igama lokulumkisa lenzelwe ukuba ukwenza oku, nangona kunjalo. Impumelelo enkulu yentsholongwane kunye nenkqubela phambili kwonyango iyakutshanje-kwaye abo unokuxoxa nabo abajamelana nesi sifo nokuba unyaka okanye udlulileyo kunokwenzeka ukuba babejamelene neziphumo ezahlukileyo.
Imithombo:
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