daj ntawm cov mucous daim nyias nyias, qhov muag sclera, thiab daim tawv nqaij yuav tsum ceeb toom rau txhua tus neeg. Txhua tus paub tias cov tsos mob zoo li no qhia txog qee yam kev mob hauv kev ua haujlwm ntawm lub cev tseem ceeb xws li daim siab. Cov kab mob no yuav tsum tau tswj los ntawm kws kho mob. Nws yuav ua qhov kev kuaj mob kom raug thiab sau cov kev kho mob uas tsim nyog. Nrog rau kev nce qib ntawm bilirubin, raws li txoj cai, jaundice tshwm. Benign hyperbilirubinemia kuj muaj cov tsos mob zoo sib xws. Hauv tsab xov xwm, peb yuav tshuaj xyuas kom meej ntxiv tias nws yog tus kab mob dab tsi, nws yog vim li cas thiab txoj kev kho mob dab tsi.
Kev txhais ntawm benign hyperbilirubinemia
Nyob rau hauv cov ntsiab lus, bilirubin yog cov kua tsib xim, nws muaj xim liab-daj. Cov tshuaj no yog tsim los ntawm cov qe ntshav liab hauv hemoglobin, uas lwj vim qhov kev hloov pauv hauv cov hlwb ntawm daim siab, tus po, cov ntaub so ntswg thiab cov pob txha.
Benign hyperbilirubinemia yog ib qho kab mob ywj pheej uas suav nrogHepatosis pigmentosa, tsev neeg choleemia simplex, juvenile jaundice, non-hemolytic familial jaundice, kab mob siab tsis ua haujlwm, khaws cia daj daj thiab ua haujlwm hyperbilirubinemia. Tus kab mob no tshwm sim los ntawm kev sib quas ntus lossis mob jaundice, pom tseeb ua txhaum ntawm lub siab ua haujlwm thiab nws cov qauv yam tsis muaj kev ua txhaum cai. Tib lub sijhawm, tsis muaj cov tsos mob pom tseeb ntawm cholestasis thiab nce hemolysis.
Benign hyperbilirubinemia (ICD code 10: E 80 - cov kab mob sib kis ntawm bilirubin thiab porphin metabolism) kuj muaj cov lej hauv qab no E 80.4, E 80.5, E 80.6, E 80. Gilbert's syndromes, Crigler's syndromes, lwm yam kab mob. Raws li - Dubin-Johnson syndromes thiab Rotor syndromes, ib qho kev tsis meej ntawm bilirubin metabolism.
Kev tshwm sim
Benign hyperbilirubinemia nyob rau hauv cov neeg laus feem ntau yog cov kab mob uas muaj tsev neeg tus yam ntxwv, lawv kis tau los ntawm hom tseem ceeb. Qhov no tau lees paub los ntawm kev kho mob.
Muaj kab mob siab tom qab hyperbilirubinemia - tshwm sim los ntawm tus kab mob siab mob siab. Tsis tas li ntawd, qhov ua rau ntawm tus kab mob tuaj yeem kis tau tus kab mob mononucleosis, tom qab rov zoo, cov neeg mob yuav pom cov tsos mob ntawm hyperbilirubinemia.
Qhov ua rau tus kabmob yog qhov ua tsis tiav hauv cov metabolism hauv bilirubin. Nyob rau hauv cov ntshav, cov tshuaj no nce, los yog muaj kev ua txhaum ntawm nws capture los yog hloov mus rau hauv daim siab hlwb los ntawm plasma.
Lub xeev no kuj ua tau thaummuaj kev ua txhaum ntawm cov txheej txheem khi ntawm bilirubin thiab glucuropic acid, qhov no tau piav qhia los ntawm qhov tsis muaj peev xwm mus tas li lossis ib ntus ntawm cov enzyme xws li glucuronyltransferase. Hauv Rotor thiab Dubin-Johnson syndromes, cov ntshav bilirubin nce siab vim qhov ua tsis taus ntawm cov xim rau hauv cov kua tsib los ntawm daim nyias nyias ntawm lub hepatocyte.
Txoj kev sib cav
Benign hyperbilirubinemia, qhov kev kuaj mob uas lees paub qhov tseeb tias nws feem ntau kuaj pom thaum hluas, tuaj yeem qhia nws cov tsos mob rau ntau xyoo thiab txawm tias lub neej. Hauv cov txiv neej, tus kab mob no tau kuaj pom ntau dua li cov poj niam.
Qhov kev tshwm sim classic ntawm tus kab mob yog yellowing ntawm sclera, icteric xim ntawm daim tawv nqaij yuav tshwm sim nyob rau hauv qee kis, tsis tas li. Kev tshwm sim ntawm hyperbilirubinemia feem ntau cuam tshuam, qee zaus lawv nyob ruaj khov, tsis dhau.
Npaj daj daj tuaj yeem tshwm sim los ntawm cov hauv qab no:
- pronounced lub cev los yog nervous fatigue;
- kev kis kab mob ntau ntxiv, qhov txhab ntawm cov kab mob biliary;
- drug resistance;
- caws;
- ntau yam kev cuam tshuam kev phais;
- haus cawv.
Symptoms of disease
Dhau li ntawm qhov tseeb tias sclera thiab tawv nqaij tig daj, cov neeg mob hnov qhov hnyav hauv txoj cai hypochondrium. MuajThaum cov tsos mob dyspeptic cuam tshuam yog xeev siab, ntuav, quav quav, tsis qab los noj mov, nce roj tsim hauv cov hnyuv.
Kev tshwm sim ntawm hyperbilirubinemia tuaj yeem ua rau pom cov tsos mob asthenovegetative, uas tshwm sim raws li kev nyuaj siab, tsis muaj zog, thiab qaug zog sai. Ntawm kev ntsuam xyuas, ua ntej ntawm tag nrho cov, tus kws kho mob saib xyuas cov yellowed sclera thiab cov dull yellow tint ntawm tus neeg mob daim tawv nqaij. Qee zaum, daim tawv nqaij tsis tig daj. Lub siab yog palpated raws ntug ntawm tus nqi qis, thiab tej zaum yuav tsis hnov. Muaj me ntsis nce qhov loj ntawm lub cev, daim siab ua mos, tus neeg mob ntsib qhov mob thaum palpation. Tus spleen tsis loj tuaj. Kev zam yog cov xwm txheej thaum benign hyperbilirubinemia tshwm sim los ntawm kab mob siab. Posthepatitis hyperbilirubinemia kuj tuaj yeem tshwm sim tom qab kis kab mob - mononucleosis.
Benign Hyperbilirubinemia Syndrome
Benign hyperbilirubinemia hauv kev kho mob suav nrog xya tus mob congenital:
- Crigler-Najjar syndromes 1 thiab 2 hom;
- Dubin-Johnson Syndromes;
- Gilbert's syndromes;
- Rotor Syndrome;
- Byler's disease (tsis tshua muaj);
- Lucy-Driscoll syndrome (tsis tshua muaj);
- Tsev neeg benign muaj hnub nyoog ntsig txog cholestasis - benign hyperbilirubinemia (tsis tshua muaj).
Tag nrho cov kab mob saum toj no tshwm sim hauvvim yog kev puas tsuaj bilirubin metabolism, yog tias qib ntawm unconjugated bilirubin nyob rau hauv cov ntshav, uas accumulates nyob rau hauv cov ntaub so ntswg, yog nce. Conjugation ntawm bilirubin plays lub luag haujlwm tseem ceeb hauv lub cev, cov tshuaj lom bilirubin tau hloov mus rau hauv cov tshuaj lom neeg tsawg, mus rau hauv diglucoronide - cov khoom sib xyaw ua ke (conjugated bilirubin). Daim ntawv pub dawb ntawm bilirubin yooj yim nkag mus rau hauv cov ntaub so ntswg elastic, lingers hauv mucous daim nyias nyias, daim tawv nqaij, thiab ntawm phab ntsa ntawm cov hlab ntsha, thaum ua rau jaundice.
Crigler-Najjar Syndrome
Tus kws kho mob Asmeskas cov kws kho mob V. Nayyar thiab J. Krigler tau txheeb xyuas tus mob tshiab rov qab rau xyoo 1952 thiab piav qhia meej. Nws tau hu ua Crigler-Najjar type 1 syndrome. Qhov no congenital pathology muaj ib tug autosomal recensive hom ntawm qub txeeg qub teg. Kev loj hlob ntawm tus mob tshwm sim tam sim ntawd hauv thawj teev tom qab yug me nyuam. Cov tsos mob no muaj sib npaug ntawm cov ntxhais thiab cov tub.
Lub pathogenesis ntawm tus kab mob yog vim tsis tiav ntawm xws li ib tug enzyme li UDFGT (lub enzyme urndine-5-diphosphate glucuronyltransferase). Nrog rau hom 1 ntawm tus mob no, UDFGT tsis tuaj yeem ua tiav, dawb bilirubin nce nrawm, cov nqi nce mus txog 200 μmol / l thiab ntau dua. Tom qab yug me nyuam, thawj hnub, permeability ntawm cov ntshav-hlwb barrier yog siab. Nyob rau hauv lub hlwb (grey teeb meem) yog tsub zuj zuj ntawm cov xim, daj nuclear jaundice tsim. Kev sim nrog cordiamine hauv kev kuaj mob ntawm benign hyperbilirubinemia, nrog phenobarbital - tsis zoo.
Bilirubin encephalopathy ua rau kev txhim kho ntawm nystagmus, cov leeg mob siab, atheretosis,opisthotonus, clonic thiab tonic convulsions. Kev kwv yees ntawm tus kab mob yog qhov tsis zoo. Thaum tsis muaj kev kho mob hnyav, qhov tshwm sim tuag tau tshwm sim nyob rau thawj hnub. Lub siab tsis hloov ntawm kev txiav txim.
YDabin-Johnson Syndrome
Dubin-Johnson Benign Hyperbilirubinemia Syndrome tau piav qhia thawj zaug hauv xyoo 1954. Feem ntau tus kab mob no tshwm sim ntawm cov neeg nyob hauv Middle East. Hauv cov txiv neej hnub nyoog qis dua 25 xyoos, nws tshwm sim hauv 0.2-1% ntawm cov neeg mob. Cov cuab yeej cuab tam tshwm sim nyob rau hauv ib qho autosomal dominant yam. Cov kab mob no muaj cov kab mob pathogenesis uas cuam tshuam nrog kev cuam tshuam ntawm kev thauj mus los ntawm bilirubin rau hauv hepatocyte, thiab los ntawm nws vim qhov tsis ua haujlwm ntawm kev thauj mus los ntawm ATP-dependent membrane system ntawm cov hlwb. Raws li qhov tshwm sim, qhov ntws ntawm bilirubin mus rau hauv cov kua tsib cuam tshuam, muaj cov reflux ntawm bilirubin rau hauv cov ntshav los ntawm hepatocyte. Qhov no tau lees paub los ntawm qhov siab tshaj plaws hauv cov ntshav ntawm cov dye tom qab ob teev thaum ua kev sim siv bromsulfalein.
Morphological yam ntxwv feature - chocolate-xim daim siab, qhov chaw tsub zuj zuj ntawm coarse granular pigment yog siab. Cov tsos mob ntawm tus kab mob: jaundice tsis tu ncua, rov tshwm sim ntawm daim tawv nqaij khaus, mob ntawm sab xis nyob rau hauv lub hypochondrium, asthenic tsos mob, dyspepsia, loj ntawm tus po thiab daim siab. Tus kab mob tuaj yeem pib thaum twg los tau. Muaj kev pheej hmoo tom qab siv cov tshuaj tiv thaiv hormonal ntev, nrog rau thaum cev xeeb tub.
Tshuaj xyuas tus kab mob raws li bromsulfaleickuaj, nrog cholecystography nrog ncua kev tso tawm ntawm tus neeg sawv cev sib txawv rau hauv cov kua tsib, thaum tsis muaj qhov sib txawv hauv lub gallbladder. Cordiamin tsis siv rau hauv kev kuaj mob ntawm benign hyperbilirubinemia hauv qhov no.
Tag nrho bilirubin tsis pub tshaj 100 µmol / L, pub dawb thiab conjugated bilirubin muaj qhov sib piv ntawm 50/50.
Kev kho rau tus mob no tsis tau tsim. Tus mob no tsis cuam tshuam rau lub neej expectancy, tab sis lub neej zoo nrog cov kab mob no zuj zus.
Benign hyperbilirubinemia - Gilbert's syndrome
Tus kab mob no tshwm sim ntau tshaj, peb yuav qhia koj ntxiv txog nws. Tus kab mob no kis tau los ntawm niam txiv mus rau cov me nyuam, yog txuam nrog ib tug tsis xws luag nyob rau hauv lub gene uas koom nyob rau hauv lub metabolism ntawm bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) tsis muaj dab tsi ntau tshaj li Gilbert's syndrome.
Bilirubin yog ib qho ntawm cov kua tsib tseem ceeb pigments, ib qho khoom nruab nrab ntawm kev tawg ntawm hemoglobin, uas koom nrog hauv kev thauj cov pa oxygen.
nce qib ntawm bilirubin (los ntawm 80-100 µmol / l), qhov tseem ceeb ntawm bilirubin tsis cuam tshuam nrog cov ntshav protein (indirect) ua rau muaj qhov tshwm sim ntawm qhov tshwm sim ntawm jaundice (mucous daim nyias nyias, sclera, tawv nqaij). Nyob rau tib lub sijhawm, kev kuaj mob siab, lwm yam ntsuas tseem nyob li qub. Hauv cov txiv neej, Gilbert's syndrome yog 2-3 zaug ntau dua li cov poj niam. Nws tuaj yeem tshwm sim thawj zaug thaum muaj hnub nyoog peb thiab kaum peb. Feem ntau, tus kab mob no nrog ib tug neeg tag nrho nws lub neej. Gilbert's syndrome muaj xws li fermentopathic benign hyperbilirubinemia (pigmented hepatoses). Lawv tshwm sim, raws li txoj cai, vim qhov tsis ncaj (tsis yog-conjugated) feem ntawm cov kua tsib xim. Qhov no yog vim muaj cov kab mob hauv lub siab. Cov chav kawm yog benign - tag nrho cov kev hloov pauv hauv daim siab, hais tias hemolysis tsis tshwm sim.
Cov tsos mob ntawm Gilbert's syndrome
Gilbert's syndrome tsis muaj cov tsos mob tshwm sim, pib nrog qhov tshwm sim tsawg. Qee tus kws kho mob tsis xav tias tus mob no yog kab mob, tab sis xa mus rau cov yam ntxwv ntawm lub cev.
Qhov tsuas yog tshwm sim feem ntau yog qhov ntsuas nruab nrab ntawm jaundice nrog staining ntawm mucous daim nyias nyias, tawv nqaij, qhov muag sclera. Lwm cov tsos mob yog me me los yog tsis muaj nyob. Tej zaum cov tsos mob ntawm lub paj hlwb tsawg:
- tsis muaj zog;
- kiv taub hau;
- faus;
- pw tsis tsaug zog;
- insomnia.
Txawm tias tsis tshua muaj cov tsos mob ntawm Gilbert's syndrome yog digestive disorders (dyspepsia):
- tsis lossis tsis qab los;
- iab tom qab noj;
- siab kub;
- iab saj hauv qhov ncauj; tsis tshua ntuav, xeev siab;
- xav hnyav, plab puv;
- quav quav (cem quav lossis raws plab);
- mob nyob rau hauv txoj cai hypochondrium ntawm ib tug npub aching cim. Tej zaum yuav tshwm sim nrog kev ua yuam kev hauv kev noj haus, tom qab kev tsim txom ntawm ntsim, nrog rau cov khoom noj muaj roj;
- yuav ntsib daim siab o.
YBenign hyperbilirubinemia kho
Yog tias tsis muaj cov kab mob sib kis ntawm txoj hnyuv, thaum lub sijhawm tshem tawm, tus kws kho mob tau sau ntawv noj zaub mov 15. Nyob rau lub sijhawm mob hnyav, yog tias muaj cov kab mob sib xyaw ua ke ntawm lub gallbladder, kev noj zaub mov No 5 raug sau tseg. Tsis tas yuav kho mob siab tshwj xeeb rau cov neeg mob.
Pab tau rau cov xwm txheej no, kho cov tshuaj vitamin, siv cov tshuaj choleretic. Cov neeg mob tsis tas yuav muaj kev kho mob tshwj xeeb spa ib yam nkaus.cov txheej txheem hluav taws xob lossis cua sov hauv thaj tsam ntawm daim siab yuav tsis tsuas yog tsis muaj txiaj ntsig, tab sis kuj yuav muaj kev phom sij. Kev kwv yees ntawm tus kab mob yog qhov zoo heev. Cov neeg mob tseem ua haujlwm tau zoo, tab sis yuav tsum txo qis kev ntxhov siab thiab lub cev.
Gilbert's benign hyperbilirubinemia kuj tsis tas yuav kho tshwj xeeb. Cov neeg mob yuav tsum ua raws li qee cov lus pom zoo kom tsis txhob ua rau qhov tshwm sim ntawm tus kab mob hnyav dua.
Table 5.
- Tso cai siv: tshuaj yej tsis muaj zog, compote, tsev cheese tsis muaj rog, qhob cij nplej, zaub kua zaub, nqaij nyuj muaj roj tsawg, cov nplej zom, nqaij qaib, txiv hmab txiv ntoo tsis muaj kua qaub.
- Forbidden noj: lard, tshiab pastry, spinach, sorrel, fatty nqaij, mustard, fatty ntses, ice cream, kua txob, cawv, dub kas fes.
- Ua raws li tsoomfwv - kev tawm dag zog lub cev hnyav tsis suav nrog tag nrho. Kev siv cov tshuaj kws kho mob: tshuaj tua kab mob, tshuaj tua kab mob, tshuaj anabolic steroids, yog tias tsim nyog - analogues ntawm kev sib deevCov tshuaj hormones uas siv los kho cov tshuaj hormonal cuam tshuam, nrog rau cov neeg ncaws pob - txhawm rau txhim kho kev ua kis las.
- Txhob haus luam yeeb, haus cawv.
Yog cov tsos mob ntawm jaundice tshwm sim, tus kws kho mob yuav sau ntau yam tshuaj.
- Barbiturate pab pawg - cov tshuaj tiv thaiv kab mob txo qis bilirubin zoo.
- Cholagogues.
- txhais tau tias cuam tshuam rau kev ua haujlwm ntawm lub gallbladder, nrog rau nws cov ducts. Tiv thaiv kev txhim kho ntawm cholelithiasis, cholecystitis.
- Hepatoprotectors (cov tshuaj tiv thaiv, tiv thaiv daim siab hlwb los ntawm kev puas tsuaj).
- Enterosorbents. Cov tshuaj uas txhim kho kev tso tawm ntawm bilirubin los ntawm txoj hnyuv.
- Digestive enzymes raug sau rau cov kab mob dyspeptic (ntuav, xeev siab, tsim roj) - pab zom zaub mov.
- Phototherapy - raug lub teeb los ntawm lub teeb xiav ua rau kev puas tsuaj ntawm cov bilirubin ruaj khov hauv cov ntaub so ntswg. Kev tiv thaiv qhov muag yog qhov tseem ceeb los tiv thaiv qhov muag kub.
Thaum ua raws li cov lus pom zoo no, cov neeg mob muaj qib bilirubin ib txwm muaj, cov tsos mob ntawm tus kab mob tshwm sim tsawg dua.
