Benign hyperbilirubinemia: mob thiab kev kho mob

Daj txheej week, qhov muag sclera thiab daim tawv nqaij yuav tsum tau alerted ntawm txhua tus neeg. Sawv daws yeej paub hais tias cov tsos mob taw tes rau tej yam irregularities nyob rau hauv lub chaw ua hauj lwm ntawm xws li ib qho tseem ceeb lub cev raws li lub siab. Cov ailments yuav tsum tau tswj los ntawm ib tug kws kho mob. Nws yuav muab tso rau lub tseeb mob thiab muab cov kev tsim nyog kho mob. Thaum feem ntau ntawm cov bilirubin feem ntau tshwm yellowness. Benign hyperbilirubinemia kuj muaj ntsis zoo ib cov tsos mob. Cov tsab xov xwm tsom xam nyob rau hauv ntau yam dab tsi tus kab mob no yog, dab tsi yog nws ua thiab kev kho mob.

Ntsiab Txhais ntawm Kev benign hyperbilirubinemia

Nyob rau hauv essence bilirubin - ib tug kua tsib xim, nws muaj ib tug yam ntxwv reddish-daj xim. Ua los ntawm no substance nyob rau hauv hemoglobin los ntawm erythrocytes uas tawg ua me me vim muaj ib tug involutive kev hloov ntawm mob hlwb, tus po, connective ntaub so ntswg, thiab hlwb pob txha.

Benign hyperbilirubinemia yog ywj siab kab mob muaj xws li xim steatosis, yooj yim Holem tsev neeg, cov hluas sib quas ntus daj ntseg, uas tsis yog-hemolytic daj tsev neeg, kev cai siab kawg, tej distention daj ntseg thiab hyperbilirubinemia. Tus kab mob yog manifested promezhayuscheysya los yog mob obstructive daj ntseg, qhia tib neeg lub siab thiab nws cov qauv tsis tseem ceeb cuam tshuam. Tsis thiab nyob rau tib lub sij hawm cuab tsos mob ntawm cholestasis thiab muaj zog hemolysis.

Benign hyperbilirubinemia (code ICD 10: E 80 - General mob thiab porphine bilirubin metabolism) kuj muaj cov nram qab codes E 80,4 E 80,5 E 80,6 E 80. Raws li encoded: Gilbert syndrome, Crigler syndromes, lwm yam mob - Dubin-Johnson syndrome rotor thiab syndromes, unadjusted ua txhaum ntawm bilirubin metabolism.

ua rau

Benign hyperbilirubinemia rau cov neeg laus nyob rau hauv feem ntau - yog tus kab mob nrog tsev neeg tus cwj pwm, lawv yog kis los ntawm hom hom. Qhov no yog paub tseeb hais tias los ntawm kev kho mob xyaum.

Muaj postgepatitnaya hyperbilirubinemia - tshwm sim raws li ib tug rau txim ntawm tus kab mob mob kab mob siab. Nws tseem yog qhov ua rau ntawm tus kab mob no yuav pab tau raws li yav tag los kis mononucleosis, tom qab rov qab los ntawm cov neeg mob tej zaum yuav muaj cov tsos mob ntawm hyperbilirubinemia.

Ua ntawm tus kab mob yog ib tug tsis ua hauj lwm nyob rau hauv cov kev pauv ntawm bilirubin. Cov ntshav dej ntawm lub substance nce, muaj yog ib tug ntxaug los yog capture los yog hloov nws mus rau hauv lub siab ntawm ntshav hlwb.

Xws li ib tug lub xeev yog tau nyob rau hauv cov neeg mob thaum muaj ib tug ntxaug nyob rau hauv lub thwj toob ntawm bilirubin thiab glyukuropovoy acid, nws yog to taub tas mus li los yog ib ntus shortage ntawm ib qho enzyme xws li glyukuroniltrapsferaza.
Cov mechanisms yog yus muaj los ntawm hyperbilirubinemia Gilbert syndrome, Crigler-Najjar thiab postgepatituyu hyperbilirubinemia. Rotor syndromes thiab Dubin-Johnson ntshiab bilirubin qab vim teeb ekskrektsii xim nyob rau hauv cov kua tsib canaliculi los ntawm cov hepatocyte membrane.

thab plaub yam

Benign hyperbilirubinemia, mob yog paub tseeb hais tias los ntawm qhov tseeb hais tias feem ntau nws yog kuaj nyob rau hauv thaum tiav hluas, yuav manifest lawv cov tsos mob ntau lub xyoo thiab txawm ib tug lub neej. Nyob rau hauv cov txiv neej, tus kab mob no yog kuaj npaum li cas tshaj li cov poj niam.

Lub classic manifestation ntawm tus kab mob - daj sclera, icteric xim ntawm daim tawv nqaij yuav tshwm sim nyob rau hauv tus neeg mob, tsis yeej ib txwm. Manifestation ntawm hyperbilirubinemia yog sib quas ntus feem ntau, nyob rau hauv uas tsis tshua muaj mob, yog tas mus li, tsis txhob kis tau.

Ntxiv dag zog rau daj ntseg yuav ntxias yam nram qab no:

• cov lus hais rau lub cev los yog tshee qaug;
• exacerbation ntawm tus kab mob, biliary ib ntsuj av tau lesion;
• Tiv thaiv cov tshuaj;
• mob khaub thuas;
• Ntau yam kev phais cov txheej txheem;
• cawv kom tsawg.

cov tsos mob

Tshwj hais tias ntawm lub sclera thiab daim tawv nqaij yog xim nyob rau hauv daj xim, cov neeg mob xav heaviness nyob rau hauv txoj cai qaum quadrant. Muaj tej lub sij hawm thaum ntxhov dyspepsia - ib tug xeev siab, ntuav, impaired quav, tsis muaj qab los noj mov, nce flatulence nyob rau hauv cov hnyuv.

Txoj kev ua hyperbilirubinemia yuav ua mus rau lub tsos astenovegetativnogo mob uas tshwm sim raws li kev nyuaj siab, qaug zog, ceev ceev qaug zog.
Nyob rau kev xeem, tus kws kho mob nyob rau hauv thawj qhov chaw them xim rau cov daj sclera thiab ib tug matte-daj tinge rau ntawm daim tawv nqaij ntawm tus neeg mob. Nyob rau hauv tej rooj plaub, ntawm daim tawv nqaij tsis tig daj. Siab palpated rau ntawm ntug ntawm lub costal koov, thiab tsis tau muaj. Nws tshwm sim ib me ntsis nce nyob rau hauv lub cev loj, lub siab yuav mos thiab siab ntev rau palpation yog mob heev. Tus po yog tsis loj hlob nyob rau hauv loj. Tsuav txhob yog cov thaum benign hyperbilirubinemia tshwm sim raws li ib tug rau txim ntawm tus kab mob siab cov kab mob. Postgepatitnaya hyperbilirubinemia kuj tshwm sim tom qab kis kab mob - mononucleosis.

Syndrome benign hyperbilirubinemia

Rau benign hyperbilirubinemia muaj xws li xya congenital syndromes nyob rau hauv cov kev kho mob xyaum:

• Crigler-Najjar syndromes, hom 1 thiab 2;
• Dubin-Johnson syndromes;
• Gilbert syndrome;
• Rotor syndromes;
• Baylera kab mob (tsawg heev li);
• Lucy-Driscoll syndrome (muaj tsawg heev);
• Tsev Neeg uas muaj hnub nyoog benign cholestasis - ib tug benign hyperbilirubinemia (muaj tsawg heev).

Tag nrho cov syndromes tshwm sim vim cov metabolism hauv bilirubin, yog hais tias tus theem ntawm unconjugated bilirubin nyob rau hauv cov ntshav yog nce, uas accumulates nyob rau hauv cov ntaub so ntswg. Conjugation ntawm bilirubin plays ib qho tseem ceeb luag hauj lwm nyob rau hauv lub cev, ib tug heev tshuaj lom bilirubin yog hloov dua siab tshiab rau hauv uas tsis muaj tshuaj lom chemical nyob rau hauv diglyukoronid - soluble compound (conjugated bilirubin). Dawb daim ntawv bilirubin yooj yim penetrates rau hauv lub elastic ntaub yog muab khaws cia tseg nyob rau hauv lub qog ua kua week, ntawm daim tawv nqaij ntawm cov phab ntsa hauv cov hlab ntsha, yog li ua rau daj ntseg.

Crigler-Najjar syndrome

US pediatricians Nayyar V. thiab J .. Kriegler nyob rau hauv 1952 qhia tau ib tug tshiab syndrome thiab piav nws. Nws tau raug hu Crigler-Najjar syndrome hom 1. Qhov no congenital teeb meem muaj ib tug autosomal retsensivny qub txeeg qub teg. Cov kev loj hlob ntawm lub syndrome tshwm sim tam sim ntawd nyob rau hauv thawj lub sij hawm tom qab yug me nyuam. Muaj zoo xws li cov tsos mob sib npaug feem ntau ob ntxhais thiab cov tub hluas.

Lub pathogenesis ntawm tus kab mob yog tshwm sim los ntawm ua kom tiav tsis muaj ib qho enzyme xws li UDFGT (enzyme urndin-5-difosfatglyukuroniltransferazy). Thaum zoo li no syndrome hom 1 UDFGT uas tsis mus kawm kiag li, free bilirubin nce sharply, ntsuas txog 200 mol / L, thiab txawm ntau. Tom qab tus me nyuam yug ntawm thawj hnub ib siab permeability p. Ntshav-hlwb barrier. Nyob rau hauv lub paj hlwb (grey teeb meem), muaj ib tug sai txuam nrog ntawm xim daj tsim kernicterus. Kordiamin xeem nrog rau cov mob ntawm cov benign hyperbilirubinemia, phenobarbital - tsis zoo.

Bilirubin encephalopathy ua rau txoj kev loj hlob ntawm nystagmus, nqaij hypertonia, athetosis, opisthotonos, clonic thiab tonic convulsions. Cov raug yog tsis tshua muaj neeg pluag. Nyob rau hauv lub qhaj ntawv ntawm intensive kev kho mob nyob rau hauv thawj hnub tejzaum tuag taus. Lub siab yeej tsis hloov li ntawm kuaj lub cev uas tau tuag.

Dubin-Johnson syndrome

Syndrome benign hyperbilirubinemia Dubin-Johnson twb xub piav nyob rau hauv 1954. Feem ntau tus kab mob no yog ib qho ntawm cov pej xeem hauv lub Middle East. Txiv neej nyob rau hauv lub hnub nyoog ntawm 25 xyoo yog pom nyob rau hauv 0.2-1% ntawm cov neeg. Qub txeeg qub teg no tshwm sim nyob rau hauv ib tug autosomal hom yam. Qhov no syndrome muaj pathogenesis uas yog txuam nrog cuam tshuam nyob rau hauv lub thauj zog ntawm hepatocyte bilirubin thiab los ntawm nws vim inconsistency thauj ATP-dependent membrane cell system. Raws li ib tug tsim nyog tau, tus me nyuam ntawm bilirubin nyob rau hauv cov kua tsib lawm, muaj ib tug reflux ntawm bilirubin nyob rau hauv cov ntshav ntawm hepatocyte. Qhov no yog paub tseeb hais tias los ntawm lub ncov ntawm cov xim concentration hauv cov ntshav ob teev thaum lub sij hawm mus sib hais siv bromsulfaleina.

Morphological yam ntxwv feature - tus chocolate-dawb lias siab, nyob qhov twg siab txuam nrog ntawm ntxhib granular xim. Txoj kev ua ntawm lub syndrome: yellowness qhov los yog sib quas ntus khaus, mob nyob rau sab xis rau hauv lub Upper quadrant, asthenic cov tsos mob, dyspepsia, o ntawm tus po thiab lub siab. Pib ntawm tus kab mob muaj peev xwm yuav nyob rau hauv tej uas muaj hnub nyoog. Muaj yog ib tug uas yuav tom qab ntev-lub sij hawm siv hormonal contraceptive cov neeg ua hauj, raws li zoo raws li thaum lub sij hawm cev xeeb tub.

Diagnosing tus kab mob raws li bromsulfaleinovoy qauv ntawm holetsistografii thaum lub sij hawm qeeb feem rau hauv cov kua tsib zoo neeg sawv cev nyob rau hauv lub qhaj ntawv ntawm uas tseg tau nyob rau hauv lub gallbladder. Kordiamin nyob rau hauv diagnosing benign hyperbilirubinemia nyob rau hauv cov ntaub ntawv no yog tsis siv.

Tag nrho cov bilirubin index tsis pub tshaj 100 mol / l, dawb thiab conjugated bilirubin piv 50/50.

Kev kho mob ntawm lub syndrome tsis tau tsim. Lub neej expectancy syndrome yog tsis cuam tshuam, tab sis qhov zoo tshaj ntawm cov hav zoov nyob rau hauv no pathology worsens.

Benign hyperbilirubinemia - Gilbert lub syndrome

Qhov no raws roj ntsha kab mob no yog cov feem ntau ntau, qhia txog nws nyob rau hauv ntau yam. Kab Mob kis los ntawm cov niam txiv rau cov me nyuam yog txuam nrog ib tug kho raws ntawm cov noob, uas yog muab kev koom tes nyob rau hauv lub metabolism ntawm bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) - yog tsis muaj dab tsi li Gilbert lub syndrome.

Bilirubin - ib qho ntawm feem tseem ceeb cov kua tsib pigments intermediate neej puas hemoglobin uas yog muab kev koom tes nyob rau hauv lub tsheb thauj mus los ntawm cov pa.

Nce bilirubin ntau ntau (80-100 micromol / L), ib tug tseem ceeb preponderance uas tsis yog-ntshav protein (indirect) bilirubin ua rau periodic ces daj ntseg (qog ua kua week, sclera, daim tawv nqaij). Nyob rau hauv cov ntaub ntawv no, daim siab muaj nuj nqi kev ntsuam xyuas, lwm yam kev ntsuas yog ib txwm. Nyob rau hauv cov txiv neej, Gilbert lub syndrome yog 2-3 lub sij hawm ntau dua nyob rau hauv cov poj niam. Rau cov thawj lub sij hawm yuav tshwm sim nruab nrab ntawm cov muaj hnub nyoog ntawm peb mus rau kaum peb xyoos. Feem ntau tus kab mob yog nrog los ntawm tag nrho cov tib neeg lub neej.
Los ntawm Gilbert syndrome yog fermentopaticheskie benign hyperbilirubinemia (xim gepatozy). Muaj feem ntau yog vim indirect (unconjugated) feem kua tsib pigments. Qhov no yog vim kev tshuaj ntsuam genetic tsis xws luag ntawm lub siab. Cov tshwm sim ntawm benign - tag nrho cov kev hloov nyob rau hauv lub siab, qhia hemolysis tshwm sim.

Cov tsos mob ntawm Gilbert lub syndrome

Gilbert lub syndrome tsis muaj cov lus hais cov tsos mob, muaj tshwm sim nrog tsawg heev cov tsos mob. Ib txhia kws kho mob tsis xav txog qhov syndrome raws li ib tug kab mob, thiab xa nws mus rau lub physiological yam ntxwv ntawm tus kab mob.

Lub tsuas manifestation nyob rau hauv feem ntau yog me me daj ntseg uas tseg tau kev kawm ntawm cov qog ua kua week, daim tawv nqaij, qhov muag sclera. Lwm yam tsos mob los yog me me, los yog tsis tshwm sim ntawm tag nrho cov.
Yam tsawg kawg nkaus tau neurological cov tsos mob:

• tsis muaj zog;
• kiv taub hau;
• qaug zog;
• pw tsaug zog mob;
• insomnia.

Txawm ntau tsawg tsos mob ntawm Gilbert lub syndrome - ib tug dyspepsia (kem plab)

• tsis tuaj kawm ntawv los yog yuav txo tau ntawm qab los noj mov;
• iab belching tom qab noj mov;
• kub siab;
• iab aftertaste nyob rau hauv lub qhov ncauj; tsis tshua muaj ntuav, xeev siab;
• ib tug kev xav ntawm heaviness, fullness nyob rau hauv lub plab mog;
• quav txawv txav (raws plab los yog cem quav);
• mob nyob rau hauv txoj cai qaum quadrant npub mob tej cim. Yuav tshwm sim thaum uas tsis nyob rau hauv kev noj haus, kev tsim txom ntawm mob thiab zaub mov;
• tej zaum yuav muaj ib qho kev nce nyob rau hauv lub siab.

Benign hyperbilirubinemia: kev kho mob

Yog hais tias muaj yog tsis muaj kab mob ntawm tus mob huam nyob rau hauv txoj kev zam txim tus kws kho mob appoints noj cov zaub mov №15. Nyob rau hauv lub mob theem, yog hais tias muaj yog ib tug concomitant gallbladder tus kab mob, noj cov zaub mov №5 taw. Tshwj xeeb kev kho mob rau daim siab cov neeg mob yog tsis yuav tsum tau.

Pab nyob rau hauv cov neeg mob, vitamin txoj kev kho, kev siv ntawm choleretic nyiaj. Thiab tsis txhob yuav tsum tau tus neeg mob los tuav tshwj xeeb spa kev kho mob.
Hluav taws xob cov los yog thermal kho mob nyob rau hauv lub siab, tsis tsuas yog ua tsis zoo, tab sis kuj muaj teeb meem los. Cov raug yog heev paaj. Cov neeg mob yuav tau khiav lag luam, tab sis nws yog tsim nyog kom txo tau cov paj, raws li zoo raws li lub cev ua si.

Benign hyperbilirubinemia thiab Gilbert tsis yuav tsum tau tshwj xeeb kev kho mob. Cov neeg mob yuav tsum tau ua raws li tej tswv yim pom zoo nyob rau hauv thiaj li tsis mus ua mob ces ntawm tus kab mob.

Rooj №5.

• Pub siv: tsis muaj zog tshuaj yej, kua txiv, uas tsis muaj roj tsev cheese, nplej kua zaub nrog zaub broth, nqaij nyuj, tsawg-rog, crisp cereal, nqaij qaib, qaub txiv hmab txiv ntoo.
• Nws yog txwv tsis pub rau noj: nqaij npuas kib, tshiab pastries, spinach, sorrel, nqaij rog, mustard, fatty ntses, mis nyuj khov, kua txob, haus dej cawv, kas fes dub.
• Mas - yog kiag li tshem tawm heavy ce. Kev siv cov tshuaj: anticonvulsants, tshuaj tua kab mob, anabolic steroids, Yog hais tias tsim nyog - analogues pw ua niam txiv cov tshuaj hormones, uas yog siv rau cov kev kho mob ntawm hormonal cuam tshuam, raws li zoo raws li lub ncaws pob - lug txhim khu kev ntaus kis las ua tau zoo.
• Kiag li muab up kev haus luam yeeb, haus dej cawv.

Yog hais tias koj muaj kev cov tsos mob ntawm daj ntseg, tus kws kho mob yuav muab tshuaj rau ib tug xov tooj ntawm cov tshuaj.

• barbiturates Group - antiepileptic tshuaj yog zoo nyob rau hauv txo cov theem ntawm bilirubin.
• Kua tsib txhais tau tias.
• Txhais tau tias, uas feem xyuam rau txoj nyob hauv cov nuj nqi ntawm lub gallbladder thiab nws ducts. Tiv thaiv kom txhob loj hlob ntawm cholelithiasis, cholecystitis.
• Hepatoprotectors (tiv thaiv txhais tau tias kev tiv thaiv hepatic hlwb thaiv kev puas tsuaj).
• Chelators. Txhais tau tias kev mus pab tau txoj lub feem ntawm bilirubin los ntawm lub plab.
• Digestive enzymes tsa nrog dyspeptic mob (xeev siab, ntuav, flatulence) - pab digestion.
• Phototherapy - raug mus rau lub teeb xiav teeb ua rau kev puas tsuaj ntawm cov ntaub so ntswg tas bilirubin. Yuav kom tiv thaiv lub qhov muag kub hnyiab yuav tsum tau muaj kev tiv thaiv.

Cov kev siv ntawm cov tswv yim pom zoo, cov neeg mob muaj ib txwm theem ntawm bilirubin, cov tsos mob tshwm sim ntau npaum li cas tsawg zaus.