Qee qhov teeb meem ntawm qib caj ces provoke kev ua txhaum ntawm bilirubin metabolism. Vim li no, pigmentary hepatosis tuaj yeem tshwm sim. Cov kab mob no suav hais tias yog qhov txaus ntshai heev. Qhov no yog vim qhov tseeb tias tus neeg mob muaj jaundice, tab sis tsis muaj teeb meem nrog lub siab ntawm tus kab mob, kis kab mob. Kev puas tsuaj ntawm bilirubin metabolism yog txawv, raws li lawv, thiab cov pab pawg neeg tseem ceeb ntawm cov kab mob tau muab faib ua ob peb hom.
Kev piav qhia
Hauv tib neeg lub cev, erythrocytes nyob txog 120 hnub. Tom qab ntawd, lawv sib cais. Ib qho ntawm cov khoom tawg yog bilirubin. Nws yog direct thiab indirect. Cov tshuaj no, tom qab tshem tawm, ua tiav hauv daim siab thiab nkag mus rau hauv cov hnyuv hauv daim ntawv ntawm cov kua tsib. Yog tias ib qho ntawm cov theem raug ua txhaum cai, ib qho tshuaj tsis ua tiav tseem nyob hauv cov ntshav. Vim li no, daim tawv nqaij ntawm tus neeg mob pigmentary hepatosis ua daj.
Ua rau kab mob
Qhov teeb meem piav qhia yog keeb kwm. Nws tshwm sim vim kev ua tsis tiav hauv kev ua ntawm bilirubin, uas tau kis mus rauqib caj ces. Tam sim no, plaub hom teeb meem tau paub. Lawv cuam tshuam nrog cov yam ntxwv etiological:
- Yog tias bilirubin xa mus rau cov ntshav, thiab tsis mus rau cov kua tsib, ces peb tab tom tham txog qhov tshwm sim ntawm Dubin-Johnson syndrome.
- Rotor Syndrome muaj cov ntsiab lus saum toj no, tab sis nws cov tsos mob tsis muaj zog.
- Gilbert's pigmentary hepatosis tshwm sim vim qhov tseeb tias ib tus neeg tsis muaj cov khoom txaus uas tso cai rau koj ntes thiab tshem tawm bilirubin. Daim tawv nqaij hloov daj. Qhov concentration ntawm bilirubin hauv cov ntshav nce.
- Nrog Crigler-Najjar syndrome, ib tus neeg tsis muaj enzyme glucuronyl transferase hauv lub cev. Muaj ntau bilirubin hauv cov ntshav.
Symptomatics
Cov tsos mob ntawm pigmentary hepatosis yog dab tsi, nyob ntawm kev sib pauv ntawm bilirubin. Feem ntau tshwm sim yog mob nyob rau hauv daim siab, yellowness ntawm daim tawv nqaij thiab malfunctions nyob rau hauv zus tau tej cov kua tsib. Xav txog cov tsos mob tus yam ntxwv ntawm txhua hom pathology:
- Thaum tus mob Dubin-Johnson tshwm sim, tus neeg mob yuav muaj sclera daj. Thaum cev xeeb tub lossis thaum noj cov tshuaj tiv thaiv qhov ncauj hauv cov poj niam, qhov kev tshwm sim no tuaj yeem ua rau hnyav dua. Kev tso zis ntawm cov kua tsib cuam tshuam.
- Nrog rotor tus kab mob, yellowness ntawm sclera tshwm sim.
- YJulbert's syndrome yog pigmentary hepatosis, uas tshwm sim los ntawm qhov mob plab hauv plab. Daim tawv nqaij daj thiab daim siab loj.
- Tus kab mob Crigler-Najjar tsis tshua muaj. Feem ntau yog kuaj pom tam sim tom qab yug me nyuam. Muaj jaundice. Yog hais tias pathology yog heev, ces cov teeb meem pib nrogcentral paj hlwb. Qhov no feem ntau ua rau tus neeg mob tuag. Yog tus me nyuam muaj daim ntawv me me, ces daim tawv nqaij tig daj me ntsis.
Hereditary pigmentary hepatosis tuaj yeem tshwm sim nws tus kheej hauv ntau txoj kev. Cia peb saib qee cov tsos mob ntxiv. Kev pw tsaug zog yog cuam tshuam, muaj mob thiab hnyav hauv daim siab, zis tsaus, asthenic syndrome tshwm, tshwm sim los ntawm kiv taub hau thiab qaug zog, qab los noj mov ploj, xeev siab tshwm sim, cov hnyuv pib ua haujlwm tsis zoo, daim tawv nqaij hloov daj thiab pib khaus khaus..
Yuav kom kuaj tau tus kab mob, tseem yuav tsum tau sau cov neeg txheeb ze.
txheej txheem kuaj kab mob
Thaum kuaj pom qhov teeb meem, nws yog ib qho tseem ceeb kom paub qhov tseeb ua rau yellowing ntawm daim tawv nqaij, nrog rau kev paub qhov txawv pigmentary hepatosis los ntawm ntau yam kab mob zoo sib xws. Nws yog ib qho tsim nyog yuav tau kuaj ntshav biochemical. Qhov no yuav ua rau koj nkag siab tias qib ntawm bilirubin hauv cov ntshav yog dab tsi. Koj yuav tsum tau sau anamnesis. Tus neeg yuav tsum tham txog kev ntxhov siab, kev noj haus, cov tsos mob. Kev tshawb nrhiav caj ces yuav tsum tau kawm. Muab qhov zoo sib xws ntawm qhov teeb meem nrog tus kab mob siab viral, kev sim yuav tsum tau ua kom tsis lees paub lossis lees paub nws muaj. Ua tsaug rau ultrasound, koj tuaj yeem nkag siab tias lub siab yog dab tsi, nrog rau seb nws muaj qhov ntev li cas. Qee lub sij hawm ua daim siab biopsy. Qhov no yuav lees paub qhov kev kuaj mob.
Differential diagnostic method
Thaum kuaj mob, nws yog ib qho tseem ceeb kom paub qhov txawv lwm yam teeb meem ntawm kab mob siab pigmentosa. Foto Kev txom nyemqhov teeb meem no, muaj nyob rau hauv tsab xov xwm. Pathology tuaj yeem tsis meej pem nrog cirrhosis ntawm daim siab, kab mob siab kab mob siab (tshwm sim hauv ob qho tib si mob thiab mob ntev), kab mob siab mob siab (hauv daim ntawv mob me). Qee zaum cov tsos mob zoo ib yam li hemolytic anemia.
Kho qhov teeb meem
Vim hais tias tus kab mob tau txais txiaj ntsig, nws tsis tuaj yeem kho kom tiav. Cov neeg mob yuav tsum zam kev tawm dag zog lub cev, kev ntxhov siab, thiab tseem xaiv qhov chaw ua haujlwm zoo. Koj yuav tsum tsis tu ncua ntxiv zaub thiab txiv hmab txiv ntoo tshiab rau kev noj haus kom muab lub cev nrog txhua qhov tshwj xeeb ntawm cov khoom tsim nyog. Txhua xyoo, koj yuav tsum tau kawm cov tshuaj vitamin kho ob zaug. Tshwj xeeb, koj yuav tsum tau xyuam xim rau cov vitamins B, lawv xav tau ntau tshaj li lwm tus.
Kev noj haus 5 yuav tsum ua raws li lub neej. Nws txhais li cas? Koj yuav tsum tso tseg cawv, rog, qaub, kib, nrog rau cov ncuav pastries, kas fes, tshuaj yej. Yog tias tsim nyog, tus kws kho mob tuaj yeem sau tshuaj kho nrog cov tshuaj hepatoprotective. Tus naj npawb ntawm cov kev kawm hauv ib xyoos yuav tsum tsis pub tshaj 4, kav 1 lub hlis.
Kho ib tus mob tshwj xeeb
Kev kho tus kab mob siab pigmented tsis yog nyob ntawm cov tsos mob uas tus neeg muaj, tab sis kuj nyob ntawm hom teeb meem:
- YCrigler-Najjar syndrome. Tus kab mob no yog hom 1 thiab hom 2. Thaum xub thawj, nws yog ib qho tsim nyog yuav tsum tau ua cov ntshav, nrog rau kev kho cov duab. Hom thib ob suav nrog kev siv tshuaj nrog phenobarbital. Phototherapy tsis tshua muaj tshuaj. Yog poj niam muaj jaundiceniam mis, nws yog qhov zoo dua hloov mus rau mis thaum pub mis rau tus menyuam.
- Julbert's syndrome yog pigmentary hepatosis (raws li ICD-10 code E80.4), uas tsis tas yuav kho tshwj xeeb. Feem ntau, nyob rau hauv cov neeg mob, kev ua ntawm bilirubin yog rov qab los tom qab ib pliag ntawm nws tus kheej. Yog tias muaj ntau heev hauv cov ntshav, ces phenobarbital yog tshuaj. Cov kws kho mob paub tseeb tias qhov kev kho zoo li no tsis cuam tshuam rau tib neeg tus mob. Txawm li cas los xij, kev coj ncaj ncees pib deteriorate. Feem ntau, cov neeg mob uas yuav tsum tau noj cov tshuaj no rau lub neej pib mob heev, thiab kev nyuaj siab tshwm sim tawm tsam keeb kwm yav dhau los no.
Nws yuav tsum nco ntsoov tias kev kho mob yuav tsum raug sau tsuas yog tom qab kuaj tau tiav.
Kev txwv hauv Gilbert's syndrome
Vim tias qhov teeb meem feem ntau tsis kho nrog tshuaj, qee qhov kev txwv yuav tsum tau ua. Qhov no yuav tshem tawm cov tsos mob.
Koj tsis tuaj yeem noj ntau yam zaub mov uas ua rau lub plab ntau dhau. Qhov no siv rau cov khoom noj rog, nrog rau cawv. Tsis txhob cia ntev so hauv kev noj mov. Nws kuj ua rau mob plab hnyuv. Nws yog txwv tsis pub ua rau kev ua kis las thiab ua rau koj tus kheej hnyav. Insolation raug txwv. Qhov no yog vim lub fact tias lawv txhim khu cov tawv nqaij laus. Kev kho lub cev ua kom sov hauv daim siab yog txwv tsis pub. Qhov no tuaj yeem ua rau mob hnyav zuj zus. Tus mob no yuav pib mus sai heev.
Yog tias cov lus pom zoo no ua raws, tus neeg mob lub neej yuav tsis zoo zuj zus.
Ursosan
"Ursosan" yog suav tias yog ib qho txiaj ntsig zoo tshaj plaws hauv kev kho cov teeb meem tau piav qhia. Nws tso cai rau koj kom stabilize qhov mob ntawm daim nyias nyias ntawm daim siab. Qhov no yuav txo cov permeability, nrog rau kev cuam tshuam rau lawv los ntawm lwm yam. Cov tshuaj no cuam tshuam rau cov kua tsib uas tso rau hauv txoj hnyuv. Tus nqi ntawm bilirubin hauv cov ntshav txo qis. Cov tshuaj pab tiv thaiv kev puas tsuaj ntawm daim siab hlwb los ntawm kev ua kom tsis muaj zog ntawm cov co toxins. Tso cai rau muaj cov cholesterol plaques kom yaj lawv. Lawv tuaj yeem kho tus kab mob siab C thiab B ntawm hom kab mob, ob qho tib si ntev thiab mob ntawm tus kab mob cuam tshuam nrog cov pob zeb, thiab cholangitis.
Kev tshuaj xyuas zoo txog cov tshuaj no. Nws yog ib qho cuab yeej zoo heev, tsis muaj contraindications thiab ua tau zoo hauv kev kho mob. Feem ntau nws raug sau tseg hauv kev kho mob hepatosis Dubin-Johnson, Rotor, Krieger-Nayar.
Huab cua thiab kev tiv thaiv
Hmoov tsis zoo, vim qhov tseeb tias hepatosis pigmentosa (raws li ICD-10 code K76.9) yog ib hom kab mob, nws tsis tuaj yeem tiv thaiv nws. Qhov kev kwv yees feem ntau yog qhov zoo. Cov neeg mob tuaj yeem ua neej nyob puv npo. Qhov tsuas yog nuance uas tuaj yeem ua rau tsis xis nyob yog xim daj ntawm daim tawv nqaij thiab sclera. Txawm li cas los xij, nws yooj yim rau siv rau nws.
Qhov kev pom tsis zoo tsuas yog nyob rau hauv rooj plaub ntawm Crigler-Najjar hepatosis. Vim yog qhov loj ntawm bilirubin hauv tib neeg, intoxication pib. Qhov no cuam tshuam rau lub cev tag nrho, nrog rau lub hlwb. Feem ntau, cov neeg mob tuag nyob rau hauvthawj theem ntawm qhov teeb meem.
Results
Raws li tau hais los saum no, vim qhov nce qib ntawm bilirubin hauv cov ntshav, yellowing ntawm lub qhov muag thiab lwm yam mucous membranes, tus neeg mob daim tawv nqaij ua tsis xis nyob. Cov txiaj ntsig tuaj yeem sib txawv heev: hnyav, ua rau tuag taus, nruab nrab, uas cov tsos mob tuaj yeem nres ib ntus, thiab mob me, tsis tas yuav tsum tau kho tshwj xeeb. Txawm li cas los xij, nyob rau hauv txhua rooj plaub, hyperbilirubinemia yuav raug suav hais tias yog kev kuaj mob tas mus li.
Cov neeg mob los ntawm Gilbert's syndrome tsis suav tias yog qhov txaus ntshai, tab sis lawv yuav tsum tau kuaj xyuas tas li thiab tswj qib ntawm bilirubin hauv cov ntshav. Kev kho mob yuav ua rau muaj txiaj ntsig zoo tshaj yog tias koj kuaj xyuas sai thiab pib kho raws sijhawm.
