Hyperbilirubinemia Benign: ukuxilongwa kanye nokwelashwa

Yellowing of nolwelwesi lwamafinyila, iso sclera nesikhumba kumele waziswe noma yimuphi umuntu. Wonke umuntu uyazi ukuthi lezi zimpawu ukhombe semfene ezithile emsebenzini komgwamanda ezibalulekile ezifana isibindi. Lezi zifo kufanele ngaso sonke isikhathi kumelwe zilawulwe udokotela. Wayezokwenza wafaka le uphethwe ilungile futhi linqume ukwelashwa kudingekile. Emazingeni aphezulu ka bilirubin ngokuvamile kubonakala yellowness. hyperbilirubinemia Benign Ubuye nezimpawu ezifanayo. Isihloko sihlaziye kabanzi mayelana nokuthi yini lesi sifo, yiziphi izimbangela yayo kanye nokwelashwa.

Definition of hyperbilirubinemia ingozi

Empeleni bilirubin - sombala Bile, it has a isici umbala obomvu-ephuzi. Ikhishwa yalolu ketshezi e-hemoglobin kusukela erythrocyte okuyinto iba yingxenye yomhlabathi ngenxa izinguquko involutive lamaseli isibindi, ubende, izicubu zezinzwa futhi umnkantsha.

hyperbilirubinemia Benign isifo ezimele kuhlanganisa sombala steatosis, Holem umndeni elula, jaundice engamahlongandlebe ngakanye, jaundice omkhaya angebona hemolytic, ngokomthethosisekelo isibindi ukungasebenzi, ukusebenza distention jaundice kanye hyperbilirubinemia. Lesi sifo kubonakala promezhayuscheysya noma ezingamahlalakhona jaundice obstructive, baveze isibindi womuntu futhi oyiyo ngaphandle thuthuva abalulekile. Ayikho futhi ngesikhathi esifanayo izimpawu ezisobala cholestasis futhi hemolysis anda.

Benign hyperbilirubinemia (ikhodi ye-ICD-10: E 80 - iziyaluyalu Jikelele kanye porphine bilirubin umzimba) Ubuye amakhodi alandelayo E 80,4 E 80,5 E 80,6 E 80. Ngakho okubhalwe: Gilbert syndrome, Crigler syndromes, nezinye izifo - Dubin-Johnson syndrome Rotor kanye syndromes, ukwephulwa engalungisiwe ka bilirubin umzimba.

izimbangela

hyperbilirubinemia Benign e abadala ezimweni eziningi - is sifo nge uhlamvu umndeni, basuke wadluliselwa uhlobo evelele. Lokhu kuqinisekiswa zokwelapha.

Kube postgepatitnaya hyperbilirubinemia - kuphakama ngenxa yegciwane i-hepatitis oyingozi. Libuye imbangela isifo ongakhonza mononucleosis ezithathelwanayo njengenkinga esidlule, ngemva yokutakula iziguli ingase ibe nezimpawu ezithile ka hyperbilirubinemia.

Imbangela lesi sifo singumbulali ukwehluleka in exchange of bilirubin. I serum ukwanda izidakamizwa, kukhona ukuphazamiseka noma bamba noma uyidlulisele ku kwesibindi plasma amaseli.

isimo esinjalo kungenzeka kulezo zimo lapho kukhona kuliphazamise yokubopha bilirubin futhi glyukuropovoy acid, kuyaqondakala ukushoda unomphela noma okwesikhashana i-enzyme ezifana glyukuroniltrapsferaza.
Lezi izindlela zinezikhathi hyperbilirubinemia Gilbert syndrome, Crigler-Najjar futhi postgepatituyu hyperbilirubinemia. Rotor syndromes futhi Dubin-Johnson serum bilirubin inyuka sombala ekskrektsii ku canaliculi Bile ngokusebenzisa ulwelwesi hepatocyte ngenxa yokuphazamiseka.

izici ngodlame

hyperbilirubinemia kungavamile ukuba ingozi, uphethwe kuqinisekiswa yokuthi ngokuvamile itholwa entsheni, engaveza izimpawu yabo iminyaka eminingi futhi ngisho nokuphila konke. In amadoda, lesi sifo itholwa okuningi njalo kunabantu besifazane.

Ukubonakaliswa zakudala zesifo - yellowing we sclera, umbala icteric kwesikhumba kungenzeka ezimweni ngazinye, ngaso sonke isikhathi. Ukubonakaliswa hyperbilirubinemia kuthiwa ngakanye ngokuvamile, ezimweni ezingajwayelekile, njalo, ngicela ungadluli.

Ukuqinisa jaundice angase avuse lezi zinto ezilandelayo:

• liphinyiselwe ukukhathala ngokomzimba noma wethuke;
• exacerbation ukutheleleka, biliary ipheshana lesion;
• izidakamizwa amasosha omzimba;
• imikhuhlane;
• izinqubo ezihlukahlukene kuhlinzwa;
• nophuzo oludakayo.

izimpawu

Ngaphandle ukuthi we sclera futhi isikhumba ababethi umbala ophuzi, iziguli zizizwe ukusinda ilungelo quadrant engenhla. Kunezikhathi lapho enyakaziswa dyspepsia - a isicanucanu, ukuhlanza, sezinyawo kahle, ukungabi esesuthi, anda ukubuyisa emathunjini.

Izenzo hyperbilirubinemia kungaholela ukubukeka astenovegetativnogo iziyaluyalu okuyinto sibonakalise njengoba ukudana, ukukhathala, ukukhathala ngokushesha.
On udokotela kwasekuqaleni uyazinaka lo yellowing we sclera futhi tinge matte-ephuzi ukuze isikhumba nesineke. Kwezinye izimo, isikhumba akusho phuzi. Isibindi palpated emaphethelweni lwaleli costal, futhi ayikwazi wazizwa. Kwenzeka ukukhuphuka kancane ngosayizi umzimba, isibindi iba ezithambile futhi babe nesineke ngezingane palpation kubuhlungu. Ubende kayikhuli ngosayizi. Okuhlukile lapho hyperbilirubinemia ingozi kwenzeka ngenxa izifo kwesibindi. Postgepatitnaya hyperbilirubinemia Ungase futhi zenzeka ngemuva izifo ezithathelwanayo - mononucleosis.

Syndrome hyperbilirubinemia ingozi

Ukuze hyperbilirubinemia ingozi zihlanganisa syndromes azalwa naso eziyisikhombisa kwezokwelapha:

• Crigler-Najjar syndromes, uhlobo 1 no-2;
• Dubin-Johnson syndromes;
• Gilbert syndrome;
• Rotor syndromes;
• isifo Baylera (ezingavamile);
• Lucy-Driscoll syndrome (ezingavamile);
• yobudala Umkhaya cholestasis ingozi - a hyperbilirubinemia ingozi (ezingavamile).

Konke lokhu syndromes zenzeka ngenxa umzimba bilirubin, uma ezingeni bilirubin unconjugated egazini luyanda, okuyinto aqoqana emisipheni. Conjugation of bilirubin linendima ebalulekile emzimbeni, a bilirubin enobuthi kakhulu iguqulwe ibe ephansi anobuthi e diglyukoronid - kwakwakhe encibilikayo (bilirubin conjugated). Mahhala ifomu bilirubin lingena kalula indwangu nokunwebeka egcinwa nolwelwesi lwamafinyila, kwesikhumba Imithambo yegazi, ngaleyo ndlela ebangela i-jaundice.

Crigler-Najjar syndrome

US nodokotela bezingane Nayyar V futhi J .. Kriegler 1952 ukhombe syndrome entsha futhi wayichaza. Wabizwa Crigler-Najjar syndrome uhlobo 1. Lesi sifo azalwa naso ine ifa autosomal retsensivny. Ukuthuthukiswa lesi sifo kwenzeka ngokushesha emahoreni zokuqala ngemva kokuzalwa. Kukhona nezimpawu ezifanayo ngokuvamile ngokulinganayo kokubili amantombazane nabafana.

I pathogenesis we sifo sibangelwa ukungabakhathaleli nhlobo i-enzyme ezifana UDFGT (enzyme urndin-5-difosfatglyukuroniltransferazy). Lapho yilesi sifo nohlobo 1 UDFGT lingekho ngokuphelele, khulula bilirubin inyuka ngokucijile, izinkomba ukufinyelela 200 Mol / L, ngisho nangokwengeziwe. Emva kokuzalwa ngosuku lokuqala high permeability p. Igazi ebuchosheni. Ebuchosheni (grey ndaba), kukhona olwenzeka okusheshayo sombala ophuzi ekuthuthukiseni kernicterus. ukuhlolwa Kordiamin nge sitholakele hyperbilirubinemia kungavamile ukuba ingozi, phenobarbital - ezingezinhle.

Bilirubin encephalopathy kuholela ukuthuthukiswa nystagmus, imisipha hypertonia, athetosis, opisthotonos, clonic kanye nokugoqana tonic. Onalesi sifo ayiyinhle kakhulu. In the ukungabikho ukwelashwa olunzulu ngosuku lokuqala kungenzeka ebulalayo. Isibindi engashintshwa at kwesidumbu.

Dubin-Johnson syndrome

Syndrome hyperbilirubinemia ingozi Dubin-Johnson waba owokuqala owachaza lesi ngo-1954. Isikhathi esiningi sifo kuvamile phakathi izakhamuzi eMpumalanga Ephakathi. Abesilisa ngaphansi kweminyaka eyi-25 itholakala 0.2-1% of amacala. Ifa yilesi sifo autosomal ngendlela evelele. Lesi sifo has pathogenesis zazo lihlotshaniswa yokuphazamiseka imisebenzi ukuthuthwa hepatocyte bilirubin futhi kuwo ngenxa ukuguquguquka ezokuthutha uhlelo ulwelwesi cell ATP nasekuthengeni. Ngenxa yalokho, sinqamukile ukulethwa bilirubin e Bile, kukhona Reflux ka bilirubin e igazi hepatocyte. Lokhu kuqinisekiswa esiqongweni emakamu udayi egazini amahora amabili ngesikhathi kuqulwa icala lakhe usebenzisa bromsulfaleina.

Morphological isici sici - kwesibindi ushokoledi-ezinemibala, lapho ukuzuza eliphezulu indawo luyimbudumbudu sombala. Izenzo sifo: njalo yellowness noma ukulunywa ngakanye, ubuhlungu ohlangothini lwesokudla e waya engxenyeni engenhla, izimpawu asthenic, dyspepsia, kweNdikimba ubende kanye esibindini. Ukuqala Kwenkathi sifo kungaba ngasiphi yobudala. Kunengozi ngemva kwesikhathi eside ejenti hormone umuthi, kanye ngesikhathi sokukhulelwa.

Ukuhlonza isifo based bromsulfaleinovoy isampula at holetsistografii ngesikhathi excretion kancane ku Bile Ngokuphambene ejenti ngokungabi bikho staining ku gallbladder. Kordiamin ekuhlonzweni hyperbilirubinemia ingozi kulokhu alisetshenziswa.

Ubude bilirubin Inkomba ayidluli kwengu-100 Mol / l, khulula futhi conjugated bilirubin lingemaphesenti 50/50.

Ukwelashwa yalesi sifo engakaze athuthukile. Ubude bempilo syndrome asithinteki, kodwa izinga khona kule zokugula siba sibi.

hyperbilirubinemia Benign - Gilbert Syndrome

Lokhu esiwufuzo yisona esikhulunywa abantu abaningi, ngako kabanzi. Izifo ngobulili kusukela abazali nezingane lihlotshaniswa isici ka isakhi sofuzo, okuyinto ehilelekile umzimba bilirubin. hyperbilirubinemia Benign (ICD - 10 - E80.4) - into njengoba Gilbert Syndrome.

Bilirubin - omunye wemizila ebaluleke kakhulu Bile zombala ukuwohloka Lesisemkhatsini-hemoglobin okuyinto ehilelekile zokuthutha oxygen.

amazinga bilirubin Ukwandiswa (80-100 micromol / L), a preponderance abalulekile amaprotheni-non-igazi (ngokungaqondile) bilirubin kuholela gqwa ukubonakaliswa jaundice (nolwelwesi lwamafinyila, sclera, isikhumba). Kulokhu, umsebenzi ukuhlolwa isibindi, nezinye izinkomba ijwayelekile. In amadoda, Gilbert sika syndrome izikhathi 2-3 zanda kuka kwabesifazane. Ngokokuqala ngqá ingavela abaneminyaka ephakathi kweminyaka emithathu kuya nantathu. Ngokuvamile lesi sifo kuhambisana bonke abantu abaphilayo.
Ngu Gilbert syndrome fermentopaticheskie hyperbilirubinemia ingozi (sombala gepatozy). Kujwayele ukuthi kube ngenxa engaqondile (unconjugated) ingxenyenamba Bile zombala. Lokhu kungenxa ukukhubazeka kwezakhi zofuzo kwesibindi. Okuvela kuyo ingozi - izinguquko ezimbi kakhulu isibindi, uzwakalise hemolysis kwenzeka.

Izimpawu ze-Gilbert Syndrome

Gilbert Syndrome akanayo izimpawu sho kwenzeka nge izimpawu omncane. Abanye odokotela ungacabangi syndrome yisifo, futhi ubheke ku-izici bokuphila umzimba.

Ukubonakaliswa kuphela ezimweni eziningi anganawo jaundice staining ukusebenza nolwelwesi lwamafinyila, isikhumba, iso sclera. Ezinye izimpawu noma omnene, noma kungenzeki nhlobo.
Ubuncane kungenzeka izimpawu zokungasebenzi kahle kwemizwa:

• ngenxa yobuthakathaka;
• isiyezi;
• ukukhathala;
• iziyaluyalu ubuthongo;
• ukuqwasha.

Ngisho izimpawu ngaphezulu ezingavamile Gilbert Syndrome - a dyspepsia (ukuqunjelwa)

• ukungabi noma ukunciphisa ukudla;
• nokubhamuka ebabayo ngemva kokudla;
• isilungulela;
• aftertaste okumunyu emlonyeni; akuvamile ukuhlanza, isicanucanu;
• umuzwa wobukhulu, ukugcwala esiswini;
• sezinyawo kwezinso (uhudo noma ukuqunjelwa);
• ubuhlungu ngakwesokudla quadrant engenhla zibuthuntu uhlamvu ngiyaqaqanjelwa. Kungenzeka uma amaphutha Ukudla, ukuhlukunyezwa ukudla oyingozi futhi okunamafutha;
• kungase kube nokwenyuka ezibalweni ezigcwele isibindi.

hyperbilirubinemia Benign: ukwelashwa

Uma kungekho izifo ezihambisana yokusetshenziswa pheshana emathunjini e udokotela ukuthethelelwa uqoka Ukudla №15. Esigabeni acute, uma kukhona concomitant gallbladder isifo, Ukudla №5 esimisiwe. nokuphathwa ngendlela ehlukile kwabantu iziguli isibindi ayidingekile.

Ngilusizo Kulezimo Vitamin yokwelapha, ukusetshenziswa kwezimali choleretic. Futhi angadingi iziguli ukubamba ukwelashwa okukhethekile spa.
ukwelashwa kagesi noma ezishisayo esibindini, abenzi lokho kuphela akukho okuhle, kodwa futhi nemiphumela elimazayo. Onalesi sifo kuyinto ngempela evumayo. Iziguli ziyakwazi ukuqhubeka isebenze, kodwa kubalulekile ukuba wehlise emsipheni, kanye nemsebenti lotsintsa umtimba.

hyperbilirubinemia Benign futhi Gilbert akudingi ukuphathwa ngendlela ekhethekile. Iziguli kufanele ulandele abanye izincomo ukuze abangele ukubonakaliswa okukhulu lesi sifo.

Ithebula №5.

• Ivunyelwe ukusebenzisa: itiye ababuthakathaka, ujusi, ephansi fat cottage shizi, kakolweni nesinkwa isobho nge yemifino umhluzi, inyama yenkomo, ephansi fat, okusanhlamvu omnandi, inkukhu, izithelo omuncu.
• Akuvunyelwe ukudla: ubhekeni, ehla fresh, isipinashi, Sorrel, inyama amanoni, lwesinaphi, okunamafutha inhlanzi, ice cream, pepper, utshwala, black coffee.
• Ukubambelela - kuyaqedwa ngokuphelele umsebenzi esindayo. Ukusetshenziswa imithi kadokotela: anticonvulsants, imithi elwa namagciwane, ama steroid, uma kunesidingo - analogue hormone ocansini, ezisetshenziswa ekwelapheni ukuphazamiseka kwama-hormone, kanye abasubathi - ukuthuthukisa ukusebenza wezemidlalo.
• Ngokuphelele ukuyeka ukubhema, ukuphuza utshwala.

Uma uhlangabezana izimpawu ka-jaundice, lo Udokotela angase anikeze isiguli eziningi imithi.

• barbiturates Group - izidakamizwa antiepileptic ziyasebenza ekunciphiseni ezingeni bilirubin.
• Bile kusho.
• Kusho, ezithonya umsebenzi we gallbladder futhi Amapayipi yayo. Vimbela ukuthuthukiswa cholelithiasis, cholecystitis.
• Hepatoprotectors (ukuvikela indlela ukuvikela amaseli hepatic ekulimaleni).
• Chelators. Esebenzisa ngcono excretion of bilirubin kusukela esiswini.
• ama-enzyme aqokwa iziyaluyalu dyspeptic (isicanucanu, ukuhlanza, ukubuyisa) - zisiza ekugayeke-.
• Phototherapy - ukuchayeka izibani ekukhanyeni blue kuholela ekubhujisweni kwezicubu bilirubin isilungisiwe. Ukuvimbela iso ukusha kudingeka uvikelwe.

Ukuqaliswa kwalawa izincomo, iziguli amazinga evamile bilirubin, izimpawu anizikhokheli njalo.