Hyperbilirubinemia caanood: ogaanshaha iyo daaweynta

Hurdi xub-axaleedka, sclera indhaha iyo maqaarka waa in la keentay qof. Qof walba waa ogyahay in calaamadahan farta khaladaad qaar ka shaqaynayeen sida jidhka muhiim ah sida beerka. nno waa in had iyo jeer la gacanta ku dhakhtar. Waxa uu lahaa cudurka saxda ah iyo qori daaweynta lagama maarmaanka ah. At heer sare ah bilirubin badanaa u muuqata jaalle. hyperbilirubinemia caanood ayaa sidoo kale waxa uu leeyahay astaamo la mid ah. article The falanqeeyaan faahfaahsan waxa cudurkan waa, waa maxay sababaha iyo daaweynta ay.

Qeexidda hyperbilirubinemia benign

In bilirubin nuxurka - Midabka bile ah, waxa uu leeyahay midab guduudan oo huruud ah dabeecad u. Soo saaray walax this ee unugga dhiigga ka erythrocytes oo muuq beelayo ay sabab u tahay isbedel ah involutive unugyada beerka, beeryarada, unugyada connective, iyo dhuuxa lafaha.

hyperbilirubinemia caanood waa cudur ka mid ah madax-bannaan tobnad Midabka, qoyska fudud Holem, joonis dhallinta go'aysa, qoyska joonis non-hemolytic, cillad beerka dastuurka, joonis distention functional iyo hyperbilirubinemia. Cudurku wuxuu la muujiyey promezhayuscheysya ama cagaarshow obstructive chronic, muujiyaan beerka aadanaha iyo qaab-dhismeedka aan rabshooyin weyn. No iyo waqti isku mid ah calaamadaha muuqda ee cholestasis iyo hemolysis kordhay.

Caanood (ICD code 10: E 80 - xanuunada guud iyo Dheef bilirubin porphine) hyperbilirubinemia sidoo kale leeyahay codes soo socda E 80.4 E 80,5 E 80,6 E 80. Iyadoo la raacayo encoded: Gilbert syndrome, syndromes Crigler, cudura kale - Dubin-Johnson syndrome rotor iyo syndromes, xadgudub unadjusted shiid bilirubin.

sababaha

hyperbilirubinemia caanood ee dadka waaweyn kiisaska intooda badan - waa cudur la dabeecadda qoyska, waxay kala qaado by nooca xukuma. Tani waxaa xaqiijiyay by dhaqanka caafimaad.

Waxaa postgepatitnaya hyperbilirubinemia - baxdo sidii natiijo ah oo hepatitis ba'an viral. Waxa kale oo sabab u ah cudurka faafa shaqayn karo mononucleosis sida ee la soo dhaafay, ka dib markii dib u soo kabashada dadka bukaanka ah la kulmi karaan astaamaha hyperbilirubinemia.

Sababta cudurka waa failure ah ee is-dhaafsiga ee bilirubin. serum walax uu kordho, waxaa jira khalkhal ama qabashada ama u wareejiyo galay beerka ka unugyada plasma.

gobolka noocan oo kale ah waxaa suurto gal ah in xaaladaha kuwa marka ay jirto rabshad ee ku qabanaya of bilirubin iyo glyukuropovoy acid, waa yaraanta joogto ah ama ku meel gaar ah enzyme la fahmi karo sida glyukuroniltrapsferaza.
hababka Kuwan waxaa lagu gartaa hyperbilirubinemia Gilbert syndrome, Crigler-Najjar oo hyperbilirubinemia postgepatituyu. syndromes Rotor iyo Dubin-Johnson bilirubin serum kordhay sababo la rabshooyin Midabka ekskrektsii in canaliculi bile ah iyada oo loo marayo xuub hepatocyte ah.

Waxyaabaha uga sii dara

hyperbilirubinemia caanood, cudurka waxaa la xaqiijiyay by xaqiiqada ah in inta badan waxaa loo ogaado in ay qaan, muujin karaa calaamadaha ay sanado badan oo xitaa uu nool yahay. In ragga, cudurkan la ogaado wax badan oo dhowr jeer oo ka haweenka.

muujinta The classic cudurka - hurdi sclera ah, midabka icteric maqaarka dhici kara in kiisas gaar ah, had iyo jeer ma. Astaanta of hyperbilirubinemia waxaa inta badan go'aysa, marmar dhif ah, waa mid joogto ah, aan ku aflaxin.

Xoojinta joonis laga yaabaa cadhaysiiyaan arrimaha soo socda:

• dhawaaqaa daal jireed ama neerfayaasha,
• sii daridda cudurka, dhaawaca biliary mareenka,
• daroogada Xasaanadda;
• hargab,
• qalliin kala duwan;
• qaadashada khamriga.

calaamadaha

Marka laga reebo in ka mid ah sclera iyo maqaarka la rinjiyeeyay midab huruud ah, bukaan dareemaan tiiraanyooday in Rubuca midig sare. Waxaa jira jeer marka dhibtay dyspepsia - a lallabo, matag, saxarada oo yaraada, rabitaan la'aan cunto, kor u yaaqyaaqsi xiidmaha.

Eebbe hyperbilirubinemia waxay keeni kartaa in muuqaalka astenovegetativnogo xanuunada taas oo muujin sida depression, daal, daal degdeg ah.
On baaritaan, dhakhtarka in meesha ugu horeysa bixisaa in ay hurdi ah oo ka mid ah sclera iyo galyadeena matte-jaalle ah in ay maqaarka ka mid ah bukaanka ah dareenka. Xaaladaha qaarkood, maqaarka ma doorsoon. Beerka taataabasho ee cidhifka xafiiltamaan ee Xeebaha, oo aan gacanta lagu dareemi karo. Waxaa dhacaya koror yar ee jirka, beerka jilcin iyo bukaanka la taataabasho xanuun. beeryarada The aan la sii kordhaya ee size. Ka reeban marka hyperbilirubinemia benign dhacdaa sidii natiijo ah oo cudurrada hepatitis. Postgepatitnaya hyperbilirubinemia sidoo kale dhici karaa ka dib markii cudurada faafa - mononucleosis.

Syndrome hyperbilirubinemia benign

Waayo, hyperbilirubinemia benign ka mid ah toddobadii syndromes lagu dhasho ee dhaqanka caafimaad:

• syndromes Crigler-Najjar, type 1 iyo 2,
• syndromes Dubin-Johnson,
• Gilbert syndrome,
• syndromes Rotor;
• cudurka Baylera (dhif);
• Lucy-Driscoll syndrome (dhif);
• da'da Qoyska cholestasis benign - hyperbilirubinemia-caanood (naadir).

All of syndromes dhacaan sabab u shiid of bilirubin, haddii heerka bilirubin unconjugated in dhiiggiisa la soo kordhay, kaas oo ururayaa in unugyo ah. Conjugation of bilirubin door muhiim ah ee jirka ku jira, bilirubin aad u sun ah waxaa beddelay low-sunta ee diglyukoronid - xarunta L (bilirubin conjugated). foomka Free bilirubin si fudud u guskiisa dhar ah laastikada waxaa la hadhay ee xub-axaleedka, maqaarka on derbiyada marinnada dhiigga, sidaas sababa cagaarshow.

Crigler-Najjar syndrome

takhaatiirta US Nayyar V. iyo J .. Kriegler 1952 aqoonsaday a syndrome cusub oo u ku tilmaamay. Wuxuu la odhan jiray Crigler-Najjar syndrome type 1. disorder Tani lagu dhasho ayaa dhaxal retsensivny autosomal ah. Horumarinta ee syndrome ku dhacdaa si degdeg ah in saacadaha ugu horreeya dhalashada ka dib. Waxaa jira calaamado la mid ah si isku mid ah inta badan gabdhaha iyo wiilasha labadaba.

pathogenesis cudurka waxaa keena la'aan dhamaystiran oo Insaymkaas ah sida UDFGT (enzyme urndin-5-difosfatglyukuroniltransferazy). Marka ay tani syndrome type 1 UDFGT gebi ahaanba maqan, bilirubin korodhka free soo maratay, tilmaamayaasha gaaraan 200 mol / L, iyo xataa more. Ka dib dhalashada maalinta ugu horeysa kala ooday Dhiig-maskaxda permeability p sare.. In maskaxda (sida cawl), waxaa jira raasamaal si degdeg ah u koraya Midabka jaalaha ah kernicterus. imtixaanka Kordiamin la ogaado hyperbilirubinemia benign, phenobarbital - xun.

encephalopathy Bilirubin keenaysaa in horumarka nystagmus, hypertonia muruqa, athetosis, opisthotonos, clonic iyo garaac tonic. saadaal waa mid aad u liita. Maqnaanshaha daaweynta degdegga ah maalinteedii kowaad ayaa suurto gal dilaa. beerka ma beddelmin at qaliin.

Dubin-Johnson syndrome

Syndrome hyperbilirubinemia benign Dubin-Johnson markii ugu horaysay la tilmaamay in 1954. Badanaa cudurka ka mid ah dadka deegaanka Bariga Dhexe caadi ah. Ragga da'doodu ka yar tahay 25 sano waxaa laga helaa 0.2-1% ee kiisaska. Dhaxalka dhacdaa autosomal hab adag. syndrome waxa uu leeyahay pathogenesis ah oo la xidhiidha rabshooyin in hawlaha gaadiidka ee bilirubin hepatocyte iyo ka ay sabab u tahay gaadiidka gudeed nidaamka cell xuub ATP-tiirsane ah. Taa awgeed, gaarsiinta bilirubin in bile waa la dumiyey, waxaa jira reflux ah bilirubin dhiigga hepatocyte ah. Arrintan waxa xaqiijiyay sarreysey fiirsashada dheeh in dhiigga laba saacadood inta lagu jiro maxkamad la isticmaalayo bromsulfaleina.

feature sifo Morphological - beerka shukulaatada-midab, halkaas oo raasamaal sare ee Midabka granular aan dhab ahayn. Eebbe syndrome, oo joogto ah jaalle ama cuncun go'aysa, xanuun dhanka midig ee Rubuca sare, calaamadaha asthenic, dyspepsia, wayneeyo oo ka mid ah beeryarada iyo beerka. Soo ridashada cudurka noqon karaa da 'kasta. Waxaa jira halis ka dib isticmaalka muddada-dheer ee wakiilada ka hortag oo hormoon, iyo sidoo kale inta aad uurka leedahay.

Soo ogaanshaha saamiga bromsulfaleinovoy cudur ku salaysan ee holetsistografii inta lagu guda jiro Digo gaabis galay wakiilka duwan bile ay ka maqan yihiin xayd in kaadi ah. Kordiamin soo ogaanshaha hyperbilirubinemia benign kiiskan looma isticmaalo.

Total index bilirubin kama badna 100 mol / l, ratio bilirubin xor ah oo conjugated waa 50/50.

Daaweynta syndrome ayaa la soo saaray. Cimriga syndrome kama beddelayso, laakiin tayada jiritaanka in cudurada this ka sii daro.

hyperbilirubinemia caanood - Gilbert ayaa syndrome

Cudurka Tani waxay la kala dhaxlo waa kuwa ugu badan, sheeg waxa ku saabsan in faahfaahin dheeraad ah. Cudurada lagu kala qaado waalidka carruurtiisa waxa uu la xidhiidhaa iin ah oo hiddo, kaas oo ku lug-shiid ee bilirubin. hyperbilirubinemia caanood (ICD - 10 - E80.4) - waa wax sida Gilbert ayaa syndrome.

Bilirubin - mid ka mid ah pigments bile ugu muhiimsan ee burburka dhexe ee unugga dhiigga cas oo ku lug leh gaadiidka ee ogsijiin.

heerarka bilirubin kordhay (80-100 micromol / L), qaalib weyn oo protein non-dhiig (aan toos ahayn) bilirubin keenaysaa in daliilka wareegsan cagaarshow (xub-axaleedka, sclera, maqaarka). Xaaladdan oo kale, baaritaano beerka, tilmaamayaasha kale waa caadi. In ragga, Gilbert ayaa syndrome waa 2-3 jeer ka badan yihiin haweenka. Waayo, markii ugu horeysay ka soo muuqan kara ay da'doodu tahay saddex ilaa saddex iyo toban sannadood ka dhexeeya. Inta badan cudurka waxaa la socda nolosha aadanaha oo dhan.
By Gilbert syndrome waa hyperbilirubinemia benign fermentopaticheskie (gepatozy Midabka). sida caadiga ah waxaa jira sabab u dadban pigments bile (unconjugated) jajab. Tan waxa u sabab cilladaha hidaha ee beerka. dhacdo ee benign - isbedel guud ee beerka, ayaa muujiyay hemolysis dhacdaa.

Calaamadaha Gilbert ayaa syndrome

Gilbert ayaa syndrome ma laha calaamado dhawaaqaa, waxay dhacdaa oo leh calaamado yar. dhakhaatiirta qaarkood ma fiirsan syndrome sida cudur, iyo ku Dooddaan Arrin u sifooyinka nafsaaniga ah ee u shaqeeyo.

muujinta kaliya ee kiisaska intooda badan waa qaab fudud xayd joonis ee xuubka, maqaarka, indhaha sclera. Calaamadaha kale ama khafiif ah, ama ma dhacaan oo dhan.
calaamado ay suuragal tahay dareemayaasha Ugu Yar:

• itaaldarro,
• wareer;
• daal,
• hurdo xumo,
• hurdo la'aan.

Xitaa calaamado dheeraad ah oo dhif ah Gilbert ayaa syndrome - a dyspepsia (saxarada)

• Maqnaanshaha ama hoos u luma;
• belching qadhaadh ka dib markii wax cunaya,
• laab;
• aftertaste qadhaadh oo afka, dhif ah matag, yalaalugo,
• dareen ah u tiiraanyooday, buuxnaanta caloosha;
• aan caadi ahayn saxarada (shuban ama calool);
• xanuun Rubuca sare dabeecadda xaq caajis xanuun. waxay dhici karaan marka khaladaadka ku jira cuntada, xadgudub ka mid ah cuntooyinka ba'an iyo dufan;
• waxaa laga yaabaa in la kordhiyo beerka.

hyperbilirubinemia caanood: daaweynta

Haddii aanay jirin cudurada la xiriira of mareenka caloosha iyo mindhicirka ee dhakhtarka dembidhaafka magacaabay №15 cunto. Wajiga ba'an, haddii ay jiraan waa cudur kaadi haysta saameen, cunto №5 magacaabay. daaweynta gaarka ah ee bukaan beerka looma baahna.

Waxtar leh in kiisaskan, vitamin therapy, isticmaalka lacagaha choleretic. Oo aan u baahnayn bukaanka si uu u qabto daaweynta HBS gaar ah.
daaweynta korontada ama kaamerada beerka, uusan innaba wanaag ku samayn waxyaalahaas oo keliya, laakiin sidoo kale waxay leeyihiin waxyeellada daran. saadaal waa arrin wanaagsan. Bukaan-socodka waa ay awoodaan in ay shaqeeyaan, laakiin waxaa lagama maarmaan ah in ay hoos u dareemaha, iyo sidoo kale dhaqdhaqaaqa jirka.

hyperbilirubinemia caanood iyo Gilbert uma baahna daaweyn gaar ah. Bukaan-socodka waa in ay raacaan qaar ka mid ah talooyin si aan loo keeni daliilka ba'an cudurka.

Shaxda №5.

• Ogol yahay in la isticmaalo: shaaha daciif ah, juice, jiis subagga ku yar, oo sarreen ah maraq kibis kula maraq khudradda, hilibka lo'da, subagga ku yar, badarka tegayo, digaag, miraha dhanaan.
• Waa mamnuuc in la cuno, hilib doofaar, la dubo cusub, isbinaajka, sorrel, baruurta hilibka, iniin khardal le'eg, kalluunka dufanka leh, jalaatada, basbaas, khamriga, kafeega madow.
• U hogaansanaanta - waxaa gebi ahaanba ka reebtay jimicsiga culus. Isticmaalka dawada la qoray: anticonvulsants, antibiotics, steroids dhiska, haddii loo baahdo - analogues hormoonnada jinsiga, kuwaas oo loo isticmaalo daweynta ee curyaaminta hormoon, iyo sidoo kale ciyaartoyda ka - si kor loogu qaado waxqabadka ciyaaraha fudud.
• Gebi ahaanba la joojiyo sigaar cabidda, khamriga la cabbo.

Haddii aad isku aragto calaamadaha cagaarshow, dhakhtarku kuu qori karaa tiro ka mid ah daawooyinka.

• barbiturates Group - daroogada antiepileptic waa wax ku ool yaraynta heerka bilirubin.
• Bile ka dhigan tahay.
• Means, taas oo saamayn ku function ka mid ah kaadi haysta iyo marinnada ay. Looga hortago horumarinta cholelithiasis, cholecystitis.
• Hepatoprotectors (ilaalinta hab ilaaliyaan unugyada cagaarshowga khasaarada).
• Chelators. Means of qaadidda Digo ee bilirubin ka mindhicirka.
• ensaymes dheefshiidka waxaa la xanuunada dyspeptic (yalaalugo, matag, yaaqyaaqsi) - dheefshiidka gargaarka.
• Daawaynta - gaadhista laambadihii buluug iftiin keenaysaa in burburinta ah unugyada bilirubin go'an. Si looga hortago gubashada indhaha u baahan in la ilaaliyo.

Hirgelinta talooyinka, bukaanka waxay leeyihiin heer caadi ah bilirubin, calaamadaha dhacaan wax badan ka yar si joogto ah.