Hyperbilirubinemia ebubonisayo: isifo kwaye afumane unyango

Yellowing ye inwebu yangaphakathi, sclera amehlo kunye isikhumba kufuneka yaziswe nawuphi na umntu. Wonke umntu uyazi ukuba ezi mpawu zibonisa nezitenxo ezithile kumsebenzi kwiqumrhu ezibalulekileyo ezifana esibindini. Ezi zifo kufuneka kusoloko ilawulwe ngugqirha. Wabeka uxilongo oluchanekileyo kwaye amisele unyango kuyimfuneko. Kumanqanaba aphezulu yibilirubin kudla kubonakala yellowness. hyperbilirubinemia ingozi kwakhona uneempawu ezifanayo. Eli nqaku uhlaziye iinkcukacha ngakumbi nokuba siyintoni esi sifo, yintoni unobangela wayo kunye nonyango.

Ukuchazwa hyperbilirubinemia ebubonisayo

Enyanisweni yibilirubin - sombala bile, inombala bomvu-yellow isici. Iveliswe yile kweziyobisi ngehemoglobin evela erythrocytes leyo iba ngenxa utshintsho involutive weeseli zesibindi, udakada, tissue nokuqina, kunye umongo wethambo.

hyperbilirubinemia ebubonisayo izifo ozimeleyo kuquka sakhi steatosis, usapho elula Holem, senyongo zoLutsha ngakanye, usapho senyongo non-hemolytic, siseko isibindi kwekhono, esebenza distention senyongo kunye hyperbilirubinemia. Isifo kubonakala promezhayuscheysya okanye senyongo ezondeleyo obstructive, ukuvakalisa isibindi oluntu kunye nesakhiwo sayo ngaphandle ukuphazamiseka ezibalulekileyo. Akukho kwaye ngaxeshanye iimpawu ezicacileyo of cholestasis kunye hemolysis zanda.

Ebubonisayo hyperbilirubinemia (ikhowudi ICD 10: E 80 - Ukuphazamiseka Jikelele kunye porphine yibilirubin metabolism) Kwakhona unalo ezi khowudi zilandelayo E 80.4 E 80.5 E 80.6 E 80. Ngako oko beminwe: Gilbert syndrome, Crigler syndromes, nezinye izifo - yeeFom ze-Johnson syndrome plane kunye syndromes, waphula unadjusted of yibilirubin ukutya.

oonobangela

hyperbilirubinemia ebubonisayo abadala kwiimeko ezininzi - na isifo kunye nomlinganiswa usapho, kodwa ayihambi ngokohlobo lubalaseleyo. Oku kuqinisekiswa yezonyango.

Kukho postgepatitnaya hyperbilirubinemia - kuvela njengesiphumo sohlobo egazini ahlekise. Kwakhona unobangela sifo ukukhonza mononucleosis njengokuba esosulelayo, emva ukufunyanwa izigulane zinokuba iimpawu hyperbilirubinemia.

Unobangela sifo ukwahluleka ngokutshintshiselana yibilirubin. I-serum le ukunyuka iziyobisi, kukho ukuphazamiseka okanye ukubanjwa okanye idluliselwe esibindini kwiiseli plasma.

Loo urhulumente kunokwenzeka kwezo meko xa kukho isiphazamiso nomphetho owujikelezileyo yibilirubin kunye glyukuropovoy acid, kodwa ukunqongophala eqondakalayo isigxina okanye okwethutyana enzyme ezifana glyukuroniltrapsferaza.
Ezi ndlela luphawulwa hyperbilirubinemia Gilbert syndrome, Crigler-Najjar kunye postgepatituyu hyperbilirubinemia. syndromes Rotor kunye yeeFom ze-Johnson serum yibilirubin ukunyuka mbala ekskrektsii kwi canaliculi inyongo ngokusebenzisa inwebu hepatocyte ngenxa uqhushululu.

mandundu

hyperbilirubinemia ingozi, uxilongo ingqinwa kukuba kaninzi oko labonwa elivisayo, ngazo iimpawu zazo iminyaka emininzi nkqu ubomi. Xa abantu, esi sifo eshiyekileyo ifunyanwa kakhulu ngakumbi rhoqo kuneentombi.

Nokubonakaliswa ezaziwa isifo - yellowing le sclera, umbala icteric ulusu zingenzeka kwiimeko ngamnye, akusoloko. Kubonakaliswa hyperbilirubinemia ukuba ngakanye rhoqo, kwiimeko ezinqabileyo, esisigxina, musa ukudlula.

Ukuqinisa senyongo ukuze bawaphikise zinto zilandelayo:

• libizwa ngokomzimba okanye luvo;
• exacerbation losulelo, biliary elithi sisilonda esidumbileyoesingaphakathi;
• iziyobisi okukwazi;
• imikhuhlane;
• iinkqubo zotyando;
• ukusebenzisa utywala.

iimpawu

Ngaphandle kwalowo sclera kunye ulusu ipeyintiweyo ngombala etyheli, izigulane va ubunzima ikota yesangqa ephezulu ngasekunene. Kukho amaxesha xa baphazamiseke ukuqunjelwa - a isicaphucaphu, ukugabha, isitulo kakuhle, ukungabi namdla wokutya, ukwanda flatulence ethunjini.

Obubonakala hyperbilirubinemia kungakhokelela inkangeleko astenovegetativnogo nezifo ibonakale njengoba ukudakumba, ukudinwa, ukudinwa ngokukhawuleza.
Ngosuku loviwo, ugqirha kwindawo yokuqala byi ingqalelo yellowing le sclera kunye matte-mthubi namona ukuya isikhumba isigulane. Kwezinye iimeko, isikhumba angabuyi mthubi. Isibindi iviwe kwi ngohlangothi arch costal, kwaye ukungavakali. Kwenzeka ukunyuka okungephi ubukhulu emzimbeni, isibindi uba ithambile kunye nomonde Ngokucofa lubuhlungu. Le spleen ayikhuli ubukhulu. Ngaphandle xa kusenzeka hyperbilirubinemia elihle ngenxa yezifo kwesibindi. Postgepatitnaya hyperbilirubinemia nako kwenzeka emva kwezifo ezosulelayo - mononucleosis.

Syndrome hyperbilirubinemia ebubonisayo

Kuba hyperbilirubinemia elihle zibandakanya syndromes ezisixhenxe iziphene kulo mkhuba lwezonyango:

• Crigler-Najjar syndromes, uhlobo 1 no-2;
• syndromes yeeFom ze-Johnson;
• syndrome Gilbert;
• syndromes Rotor;
• isifo Baylera (ezinqabileyo);
• Lucy-Driscoll syndrome (ezinqabileyo);
• yobudala Family cholestasis elihle - a hyperbilirubinemia elihle (ezinqabileyo).

Zonke ezi syndromes zenzeka ngenxa nethe of yibilirubin, ukuba inqanaba yibilirubin unconjugated egazini inyuswe, nto leyo ebangelwa emzimbeni. Conjugation of yibilirubin idlala indima ebaluleke kakhulu emzimbeni, a yibilirubin inetyhefu kakhulu iguqulelwa ezisezantsi yityhefu kwi diglyukoronid - ezimbaxa enyibilikayo (yibilirubin conjugated). Free ifomu yibilirubin ngokulula ingena laphu elastic lugciniwe kwi inwebu yangaphakathi, amaxolo iindonga imithambo yegazi, oko kubangela senyongo.

Crigler-Najjar syndrome

US bezifo Nayyar V. kunye J .. Kriegler ngo-1952 iveze syndrome entsha wayichaza. Wabizwa Crigler-Najjar uhlobo syndrome 1. Esi sifo azalwa iye ilifa autosomal retsensivny. Uphuhliso onaso lwenzeka nangoko kwiiyure zokuqala emva kokuzalwa. Kukho iimpawu ezifanayo rhoqo ngokulinganayo ngamantombazana namakhwenkwe.

Le Pathogenesis esi sifo sibangelwa kukunqongophala okupheleleyo enzyme ezifana UDFGT (enzyme urndin--difosfatglyukuroniltransferazy 5). Xa olu syndrome hlobo 1 UDFGT nabangekhoyo ngokupheleleyo, ukunyuka yibilirubin free kabukhali, izikhombisi ukufikelela 200 Mol / L, yaye ngakumbi. Emva kokuzalwa kosuku lokuqala eliphezulu permeability p. Umqobo Blood-ingqondo. Ebuchotsheni (mbandela grey), kukho olwenzeka ngokukhawuleza ebala etyheli asakhasayo kernicterus. test Kordiamin kunye lokufunyaniswa hyperbilirubinemia nabungozi, phenobarbital - negative.

Yibilirubin Encephalopathy kukhokelela kuphuhliso nystagmus, hypertonia kwezihlunu, ATHETOSIS, opisthotonos, clonic kunye yombono tonic. I-ALS ulihlwempu kakhulu. Xa engekho unyango olunzulu ngemini yokuqala mhlawumbi ebulalayo. Isibindi akawatshintshi kwi kwesidumbu.

YeeFom ze-Johnson syndrome

Syndrome hyperbilirubinemia ebubonisayo yeeFom ze-Johnson iye ochazwe kuqala ngo-1954. Ikakhulu isifo kuqhelekile phakathi kwabahlali yakuMbindi Mpuma. Amadoda abangaphantsi kweminyaka engama-25 ifumaneka 0.2-1% yamatyala. Ilifa kwenzeka ngendlela lubalaseleyo autosomal. Esi sifo iye Pathogenesis apho lu dityanisiwe yokuphazamiseka nemisebenzi zezithuthi hepatocyte yibilirubin kwaye kulo ngenxa kwinkqubo cell inwebu ATP-oxhomekeke zothutho ukungangqinelani. Ngenxa yoko, ukuziswa yibilirubin kwi inyongo wonakele, kukho reflux of yibilirubin kwi egazini hepatocyte. Oku kungqinwe kwincopho idayi yoxinaniso igazi iiyure ezimbini ngexesha lokuthethwa kwetyala usebenzisa bromsulfaleina.

phawu Morphological iimpawu - esibindini yetshokolethi-obugolide, apho olwenzeka eliphezulu ontlakantlaka granular ebala. Mbonakaliso syndrome: ukusoloko yellowness okanye ukurhawuzelela ngakanye, iintlungu kwicala lasekunene kwi ikota yesangqa eliphezulu, iimpawu asthenic, ukuqunjelwa, wawandisa udakada wesibindi. Ekuqaleni esi sifo kuba na ubudala. Kukho ingozi emva ukusetyenziswa ixesha elide arhente hormone zokuthintela ukukhulelwa, kwakunye ngexesha lokukhulelwa.

Uxilongo ezisekwe sifo bromsulfaleinovoy wesampula holetsistografii ngexesha zinomgutyulo kade kwi arhente nshita inyongo xa engekho bala kule senyongo. Kordiamin ku ekuxilongweni hyperbilirubinemia elihle kule meko lisusiwe.

Iyonke Isalathisi yibilirubin ayidluli 100 Mol / l, ezamahala kunye conjugated ratio yibilirubin ngu 50/50.

Unyango lwe onaso iye yaphuhliswa. Iminyaka Life syndrome kakubi, kodwa umgangatho ubukho kule ngezifo mandundu.

hyperbilirubinemia ebubonisayo - Gilbert engqondweni

Esi sifo ilifa lo eqhelekileyo kakhulu, xelela ngayo ngokweenkcukacha ngakumbi. Isifo sivela kubazali abantwana inxulunyaniswa onesiphene zofuzo, nto leyo ebandakanyekileyo nethe of yibilirubin. hyperbilirubinemia ebubonisayo (ICD - 10 - E80.4) - nto njengoko Gilbert engqondweni.

Yibilirubin - omnye zombala ezibaluleke kakhulu inyongo Ukucalucalulwa eliphakathi mpilo leyo obandakanyeka ekuthutheni oksijini.

amanqanaba yibilirubin Ukwanda (80-100 micromol / L), kukho preponderance okubalulekileyo protein non-gazi (ngqo) yibilirubin ikhokelela obubonakala ngamaxesha senyongo (inwebu yangaphakathi, sclera, isikhumba). Kulo mzekelo, iimvavanyo umsebenzi isibindi, ezinye izikhombisi kuqhelekile. Ngabantu, Gilbert kaThixo syndrome kukuphinda 2-3 ivame kakhulu kunokuba kwabasetyhini. Kuba okokuqala angavela phakathi kweminyaka kwiminyaka emithathu ukuya nantathu. Ngokufuthi sifo kunye bonke ubomi babantu.
Ngu Gilbert syndrome hyperbilirubinemia fermentopaticheskie ebubonisayo (sombala gepatozy). Kukho ngokuqhelekileyo ngenxa ngqo zombala inyongo (unconjugated) amaqhekeza. Oku kungenxa ziziphene zemvelo kwesibindi. Kukuvela nabungozi - utshintsho ezinzulu esibindini, wavakalisa hemolysis kwenzeka.

Impawu Gilbert engqondweni

syndrome Gilbert akuthethi iimpawu zomlomo wakhe, kwenzeka iimpawu engephi. Abanye oogqirha musa ingqalelo syndrome njenge sifo, kwaye iyithumele kwiimpawu komzimba yento ephilayo.

Kuzibonakalisa kuphela kwiimeko ezininzi abanomoya yokusebenza senyongo amabala inwebu yangaphakathi, ulusu, amehlo sclera. Ezinye iimpawu okanye nobulali, okanye kwenzeke konke konke.
Ubuncinane mpawu kunokwenzeka luvo:

• yobuthathaka;
• nesiyezi;
• nokudinwa;
• zokulala;
• ugwayi.

Nkqu zesifo onge ka Gilbert engqondweni - a ukuqunjelwa (ukungacoleki kokutya)

• ukungabikho okanye ukuncitshiswa emnyama;
• belching ukrakra emva ukudla;
• heartburn;
• kuba krakra emlonyeni; kunqabile uyagabha, isicaphucaphu;
• imvakalelo edandatheka, nenzaliseko esiswini;
• ukungaqheleki isitulo (isisu okanye ukuqhina);
• intlungu ekunene ikota yesangqa ephezulu buthuntu uphawu ndiyaqaqanjelwa. Ngaba kwenzeka xa iimpazamo ekutyeni, kakubi ukutya etsolo uyadinga;
• kubekho ukwanda esibindini.

hyperbilirubinemia ebubonisayo: unyango

Ukuba akukho izifo enxulumene iphecana zesisu xa ugqirha uxolelo lityumbe ukutya №15. Kwisigaba etsolo, ukuba kukho isifo senyongo ezihamba, ukutya №5 amisiweyo. akuyomfuneko unyango olulodwa kwizigulane zesibindi.

Iluncedo kwezi meko, vitamin unyango, ukusetyenziswa kwemali choleretic. Kwaye musa ukuba izigulane ukubamba unyango Spa ekhethekileyo.
zonyango lombane okanye thermal esibindini, hayi kuphela ukwenza okulungileyo, kodwa nemiphumo eyingozi. I-ALS kakhulu intle. Izigulane banako ukusebenza, kodwa kuyimfuneko ukunciphisa luvo, kwakunye eyahlukahlukeneyo.

hyperbilirubinemia ebubonisayo kunye Gilbert ayifuni unyango olulodwa. Izigulane kufuneka balandele ezinye iingcebiso ukuze enze izinto zale sifo.

Uluhlu №5.

• Ukuvunyelwa ukuba usebenzise: iti buthathaka, juice, libya-fat cottage itshizi, ingqolowa isonka isuphu umhluzi yemifuno, inyama yenkomo, ephantsi amanqatha, cereal mnandi, inkuku, iziqhamo ubumuncu.
• Akuvunyelwe ukuba adle: Bacon, ehla fresh, isipinatshi, nangwevu, nenqatha inyama, lwemostade, okunamafutha intlanzi, ice cream, ipepile, utywala, zekofu emnyama.
• Ukubambelela - eceleni ngokupheleleyo umthambo onzima. Ukusetyenziswa imithi: anticonvulsants, imithi, steroid, ukuba kuyimfuneko - analogue zamadlala ngesondo, ezisetyenziswa ukuba unyango ukuphazamiseka kwamadlala, kwakunye iimbaleki - ukukhuthaza ukusebenza lweembaleki.
• Ngokupheleleyo ukuyeka ukutshaya, ukusela utywala.

Ukuba ngaba wehlelwa iimpawu senyongo, ugqirha unakho ukumisela iqela amayeza.

• barbiturates Group - amachiza antiepileptic iyasebenza ekunciphiseni amanqanaba yibilirubin.
• Enyongweni, ithetha.
• Iindlela, apho ifuthe lwemisebenzi eyenziwa senyongo kunye imigudu yayo. Iyaluthintela uphuhliso cholelithiasis, cholecystitis.
• Hepatoprotectors (ukukhusela iindlela ukukhusela iiseli hepatic umonakalo).
• Chelators. Iindlela zokuthuthukisa kukhuthaza yibilirubin ukusuka ethunjini.
• enzyme baqeshwa nokuphazamiseka dyspeptic (isicaphucaphu, ukugabha, flatulence) - zoncedo ekugayeni.
• Phototherapy - Ukwazi izibane blue ukukhanya kukhokelela ekutshatyalalisweni kwezihlunu yibilirubin ezisisigxina. Ukuthintela iliso ukutsha kufuneka bakhuselwe.

Ukuphunyezwa kwezi ziphakamiso, izigulane kufuneka amanqanaba eqhelekileyo yibilirubin, iimpawu kwenzeka alufane.