Benign hyperbilirubinemia: kuongorora chirwere uye kurapwa

Yellowing of mucous membranes, ziso sclera uye ganda inofanira yazivisa kuti munhu. Munhu wose anoziva kuti zviratidzo izvi zvinoratidza mamwe hadzina kufambiswa zvakanaka mubasa muviri chinokosha sezvo chiropa. zvirwere izvi vanofanira kudzorwa nachiremba. Aiisa yakarurama zvokuongorora uye varayire anodiwa kurapwa. At dzakakwirira kwevari bilirubin kazhinji anooneka yellowness. Benign hyperbilirubinemia anewo zviratidzo zvakafanana. Nyaya ongorora muudzame chii chirwere ichi, chii ayo chinokonzera uye kurapwa.

Definition of benign hyperbilirubinemia

Chaizvoizvo bilirubin - munhu bile ruvara, ine maitiro yakatsvukuruka-yellow ruvara. Yakabudiswa neZvapupu fuma iyi Chizenga kubva erythrocytes izvo dziparare nokuda ane involutive rokuchinja chiropa masero, chirwatata, connective munyama, uye mwongo.

Benign hyperbilirubinemia iri rakazvimirira chirwere zvinosanganisira ruvara steatosis, nyore mhuri Holem, hwanana inoratidza jaundice, vasiri hemolytic jaundice mhuri, yepamutemo chiropa dysfunction, chadzo distention jaundice uye hyperbilirubinemia. The chirwere kunoratidzwa promezhayuscheysya kana chirwere obstructive jaundice, kuratidza chiropa dzevanhu uye ayo chivako pasina zvinokosha vachivavhiringidza. No uye panguva imwecheteyo zviratidzo pachena cholestasis ikawanda hemolysis.

Benign hyperbilirubinemia (bumbiro ICD 10: E 80 - General zvirwere uye porphine bilirubin muviri) anewo zvinotevera Codes E 80,4 E 80.5 E 80,6 E 80. Naizvozvo encoded: Gilbert urwere, Crigler syndromes, zvimwe zvirwere - Dubin-Johnson urwere rotor uye syndromes, unadjusted kutyora bilirubin muviri.

zvinokonzera

Benign hyperbilirubinemia nedzevakuru kakawanda - iri hosha nemhuri hunhu, ivo pachishandiswa kureva mhando. Izvi kunosimbiswa okurapa.

Pane postgepatitnaya hyperbilirubinemia - chinovapo Somugumisiro nemavhairasi rikwanise chiropa. Ndirwowo mhaka chirwere rinogona kare kutapuriranwa mononucleosis, pashure kuzoita varwere tinganetseka zviratidzo hyperbilirubinemia.

Chikonzero hosha iri kukundikana iri kupanana bilirubin. The serum of fuma inowedzera, pane nyonganyonga kana kubatwa kana uchatamisa akaukandira chiropa kubva yeplasma masero.

Ezvinhu akadaro zvinogoneka vaya zviitiko apo pane nyonganyonga iri nokusungwa bilirubin uye glyukuropovoy acid, zvinonzwisisika zvachose kana kwenguva kuperevedza imwe enzayemu akadai glyukuroniltrapsferaza.
zvigadziriswe izvi vanoonekwa ne hyperbilirubinemia Gilbert urwere, Crigler-Najjar uye postgepatituyu hyperbilirubinemia. Rotor syndromes uye Dubin-Johnson serum bilirubin inowedzera nokuda vachivavhiringidza ekskrektsii ruvara ari bile canaliculi kubudikidza hepatocyte membrane.

sembura zvinhu

Benign hyperbilirubinemia, chirwere kunosimbiswa chokuti kazhinji zviri dzakaonekwa mu kuyaruka, anogona kuratidza zviratidzo zvavo kwemakore akawanda uye kunyange kwoupenyu hwose. Varume, chirwere ichi dzakaonekwa zvikuru kakawanda kupfuura vakadzi.

The klassiske nekuratidza chirwere - yellowing of sclera, icteric Ruvara ganda kunogona kuitika munguva nomumwe nguva kwete nguva dzose. Kuratidzwa hyperbilirubinemia vari inoratidza kazhinji, zvisingawanzoitiki nguva ndekwechigarire, musapfuura.

Kusimbisa jaundice anogona kumutsa zvinhu zvinotevera:

• akazivisa chaiko kana huta kuneta;
• exacerbation chirwere, biliary turakiti lesion;
• Kutapata zvinodhaka;
• chikosoro;
• Various maoparesheni;
• doro yaunodya.

zviratidzo

Asi zviri sclera uye ganda iri nechepadivi mu tsvuku ruvara, varwere vanonzwa kufundumwara zvakarurama repamusoro quadrant. Pane nguva apo anetseka dyspepsia - a kuda kurutsa, kurutsa, havachanyatsogoni chetsoka, kushaya kudya, yakawedzera flatulence ari ura.

Kuratidza hyperbilirubinemia kungaita kuti chitarisiko astenovegetativnogo zvinetso izvo kuratidza sezvo mwoyo, kurukutika, nokukurumidza kuneta.
On kuongororwa, chiremba pakutanga anoteerera kusvikira yellowing pamusoro sclera uye matte-yellow tinge kuti ganda murwere. Mune zvimwe zviitiko, ganda rikasauya tsvuku. Chiropa palpated panguva kumucheto costal chematenga, uye haagoni akanzwa. Zvinoitika zvishoma kuwedzera kukura mumuviri, chiropa inova nyoro uye kushivirira palpation kunorwadza. The chirwatata haisi kukura ukuru. Anosiyiwa vari apo benign hyperbilirubinemia kunoitika somunhu mugumisiro zvirwere chiropa. Postgepatitnaya hyperbilirubinemia zvinogonawo kuitika mushure zvirwere - mononucleosis.

Syndrome benign hyperbilirubinemia

For benign hyperbilirubinemia zvinosanganisira syndromes vachiberekwa manomwe okurapa:

• Crigler-Najjar syndromes, mhando 1 uye 2;
• Dubin-Johnson syndromes;
• Gilbert urwere;
• Rotor syndromes;
• Baylera chirwere (kushoma);
• Lucy-Driscoll syndrome (kushoma);
• Family zera benign cholestasis - a benign hyperbilirubinemia (isingawanzowanikwi).

Vose syndromes izvi zvichiitika nokuda muviri ose bilirubin, kana pachiyero unconjugated bilirubin muropa rwuchiwanzwa, iyo rinoungana munyama. Conjugation kuti bilirubin inoita basa rinokosha mumuviri, zvikuru hunokachidza bilirubin iri dzikwanise yakaderera-chepfu ari diglyukoronid - soluble remubatanidzwa (conjugated bilirubin). Free chimiro bilirubin nyore chinopinda chinokosha kwakavharwa nenyama iri rakaramba mune mucous membranes, ganda pamusoro pamasvingo midziyo ropa, saka anoita jaundice.

Crigler-Najjar urwere

US pediatricians Nayyar V. uye J .. Kriegler muna 1952 akataura chirwere chitsva vakarondedzera. Akanzi Crigler-Najjar chirwere mhando 1. Izvi vachiberekwa rebipolar ane autosomal retsensivny nhaka. Kukura chirwere kunoitika pakarepo pakutanga maawa pashure pokuberekwa. Pane kufanana zviratidzo zvakafanana kazhinji nevasikana nevakomana.

The pathogenesis chirwere kunokonzerwa nokushayiwa ane enzayemu zvizere akadai UDFGT (enzayemu urndin-5-difosfatglyukuroniltransferazy). Kana ichi chirwere mhando 1 UDFGT ndisipo zvachose, vakasununguka bilirubin inowedzera hasha, zviratidzo vanosvika 200 mol / L, uye kunyange zvakawanda. Aberekwa rokutanga refu permeability p. Blood-pfungwa nokuidzivirira. In uropi (pfumbu nyaya), pane nokukurumidza kuunganidza ruvara yeyero dzichiri kernicterus. Kordiamin bvunzo pamwe nechirwere pamusoro benign hyperbilirubinemia, phenobarbital - kunaka.

Bilirubin encephalopathy inotungamirira kutanga nystagmus, tsandanyama hypertonia, athetosis, opisthotonos, clonic uye tonic nokugwinha. The fungidziro murombo chaizvo. Pasina zvakasimba kurapwa rokutanga zuva zvichida zvinouraya. Chiropa haina chakashanduka kuvhuya chitunha.

Dubin-Johnson urwere

Syndrome benign hyperbilirubinemia Dubin-Johnson itange kurondedzerwa muna 1954. Kunyanya chirwere chinoremedza vagari kuMiddle East. Men pasi makore 25 unowanikwa 0.2-1% nyaya. Nhaka kunoitika munzvimbo autosomal kureva nenzira. chirwere ichi ane pathogenesis uko chokuita kuvhiringidzika chokufambisa mashandiro anoita hepatocyte bilirubin uye pauri nokuda kusawirirana chokufambisa ATP-zvaanoda Kamudzira sero hurongwa. Somugumisiro, chokuendesa bilirubin mu bile yaputswa, pane reflux pakati bilirubin iri muropa hepatocyte. Izvi kunosimbiswa yepamusoro dhayi wevasungwa muropa maawa maviri mudare uchishandisa bromsulfaleina.

Morphological maitiro chinhu - chiropa chokoreti-mavara, apo mukuru kuunganidza zvinonyadzisa granular ruvara. Zvinoratidza chirwere: yellowness rusingaperi kana inoratidza kuvaviwa, marwadzo kurudyi chokumusoro quadrant, asthenic zviratidzo, dyspepsia, akawedzera chirwatata uye chiropa. Kutanga chirwere kunogona chero zera. Pane mukana mushure kushandisa hormone pamuviri yomuchipatara kwenguva refu, uyewo panguva yepamuviri.

Diagnosing chirwere yakavakirwa bromsulfaleinovoy yokuenzanisira panguva holetsistografii panguva kunonoka excretion kupinda bile musiyano mumiriri pasina staining ari gallbladder. Kordiamin mu diagnosing benign hyperbilirubinemia mune iyi haina kushandiswa.

Total bilirubin indekisi hakuiti pfuura 100 mol / L, vakasununguka uye conjugated bilirubin reshiyo ndiyo 50/50.

Pakurapa chirwere haina dzakabudirira. Life anotarisirwa chirwere haina kukanganiswa, asi kunaka aripo zvokurwara ichi Zvinoipisisa.

Benign hyperbilirubinemia - Gilbert kuti urwere

chirwere ichi nhaka ndicho, nezvazvo zvizere. Chirwere chinotapuriranwa kubva vabereki kuti vana kusonganirana kuremara majini, iyo rinobatanidzwa muviri kuti bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) - hapana sezvo Gilbert kuti syndrome.

Bilirubin - chimwe chezvinhu zvinokosha bile pigments zvenguva nokuputsika Chizenga iyo rinobatanidzwa kutakurwa okisijeni.

Inosimudzira bilirubin yomuropa (80-100 micromol / L), chinokosha preponderance vasiri ropa mapuroteni (zvisina kunanga) bilirubin kunotungamirira nenguva dzirikure kuratidzwa jaundice (mucous membranes, sclera, ganda). Muchiitiko chino, chiropa basa nemiedzo, zvimwe zviratidzo zviri muupenyu. Varume, Gilbert kuti chirwere ndiyo 2-3 nguva anonyanya kupfuura vakadzi. Kokutanga anogona kuonekwa pakati pemakore matatu namatatu makore. Kazhinji maperembudzi pamwe upenyu hwose hwomunhu.
By Gilbert chirwere vari fermentopaticheskie benign hyperbilirubinemia (ruvara gepatozy). Pane kazhinji nokuda zvisina kunanga (unconjugated) hacho bile pigments. Izvi imhaka vaberekwe vakaremara chiropa. The panowanikwa benign - kwakakomba zvachinja chiropa, akaratidza hemolysis kunoitika.

Zviratidzo Gilbert kuti urwere

Gilbert kuti chirwere asina kutaurwa zviratidzo, kunoitika shoma zviratidzo. Vamwe vanachiremba musafunga chirwere sezvo chirwere, uye kuMutongi vasiri zvechadenga muviri.

The chete kuratidzwa kakawanda vari munyoro jaundice staining chokupika ari mucous membranes, ganda, ziso sclera. Zvimwe zviratidzo kana munyoro, kana kwete kuitika zvachose.
Minimum zvinogoneka chetsinga zviratidzo:

• simba;
• dzungu;
• nekuneta;
• hope zvirwere;
• kushaya hope.

Kunyange zvakawanda zvisingawanzoitiki zviratidzo Gilbert kuti chirwere - a dyspepsia (fufune)

• Kusavapo kana zvinoderedza kuti havi;
• shungu belching pashure kudya;
• chirungurira;
• shungu aftertaste mumuromo; kashoma kurutsa, kuda kurutsa,
• kunzwa kuchema, kuzara mudumbu;
• chetsoka abnormalities (manyoka kana kupatira);
• marwadzo zvakarurama repamusoro quadrant mikukutu achirwadza hunhu. Anogona kuitika kana zvikanganiso zviri zvokudya, kushungurudzwa chakasimba uye mafuta zvokudya;
• pangava nokuwedzera chiropa.

Benign hyperbilirubinemia: kurapwa

Kana pasina kuyeuka zvirwere nemudumbu mu rekukangamwirwa chiremba unogadza zvokudya №15. Munguva chenjera chokutambudzika, kana pane concomitant gallbladder chirwere, kudya №5 akagadzwa. Special kurapwa nokuti chiropa varwere haina kuzvireva.

Anobatsira Muzviitiko izvi, vhitamini kurapa, kushandisa choleretic mari. Uye regai zvinoda varwere kubata Spa kurapwa chairo.
Electrical kana chafariz kurapwa vari chiropa, kwete chete kuita zvakanaka, asi vanewo zvinokonzerwa. The fungidziro iwayo zvakanaka. Varwere vanokwanisa ishande, asi zvinokosha kutapudzwa etsinga, uyewo kurovedza muviri.

Benign hyperbilirubinemia uye Gilbert haatarisiri vavashandire. Varwere vanofanira kutevera vamwe kunenge kuti varege kukonzera chakasimba zvinoratidza chirwere.

Table №5.

• Kubvumirwa kushandisa: simba tii, muto, yakaderera-mafuta koteji chizi, gorosi chingwa muto pamwe miriwo muto, nyama, yakaderera-mafuta, Crisp bota, huku, yakashapa zvibereko.
• Zvinonzi rambidzwa kudya: bhekoni, matsva pastries, sipinachi, zvakarungwa, nyama mafuta, netsanga, mafuta hove, aizikirimu, mhiripiri, doro, dema kofi.
• Nemazvo - vari zvachose zvachose rinorema muviri. Kushandiswa inonyorwa nachiremba: anticonvulsants, nemishonga, anabolic steroids, kana zvakakodzera - analogues dzepabonde mahormone, izvo inoshandiswa pakurapa hormone Kuvhiringidza, uyewo vatambi - kunatsiridza mitambo unongotaura.
• Chose kurega kusvuta, kunwa doro.

Kana mukawana Zviratidzo jaundice, chiremba anogona mishonga zvinoverengeka mishonga.

• barbiturates Group - mishonga antiepileptic dzinobudirira mukuderedza padanho bilirubin.
• Bile zvinoreva.
• Means, izvo kufurira pamusoro kushanda gallbladder yayo ducts. Kudzivirira kukura cholelithiasis, cholecystitis.
• Hepatoprotectors (yaidzivirira zvinoreva kuchengetedza hepatic masero pamusoro kukuvara).
• Chelators. Nzira kuwedzera excretion ose bilirubin kubva dumbu.
• Zvomudumbu makemikari vanogadzwa nomudzimu dyspeptic zvinetso (kuda kurutsa, kurutsa, flatulence) - rubatsiro kugaya.
• Phototherapy - kugarotamba chiedza yebhuruu mwenje unotungamirira kuparadzwa zvinyama yakatarwa bilirubin. Kuti adzivise ziso kutsva vanoda kudzivirirwa.

The Implementation pamusoro vanorumbidza izvi, varwere vane zvinoitika kwevari bilirubin, zviratidzo kuitika ndoda kakawanda.