Chosaopsa hyperbilirubinemia: matenda ndi mankhwala

Chikasu wa mucous zimagwira ntchito zosiyanasiyana, sclera maso ndi khungu ayenera kukadziwitsa wa munthu aliyense. Aliyense akudziwa kuti zizindikiro zonsezi zikuloza kwa monyanyira zina mwa ntchito zimene munthu thupi zofunika monga chiwindi. matenda awa ayenera nthawi zonse ziyenera kulamulidwa ndi dokotala. Anali kuika matenda lolondola ndi mankhwala mankhwala kutero. Misinkhu apamwamba bilirubin zambiri amapezeka yellowness. Chosaopsa hyperbilirubinemia komanso ali ndi zizindikiro zofanana. Nkhaniyi yosanthula mwatsatanetsatane zimene matenda izi, kodi zimayambitsa matendawo ndi mankhwala.

Tanthauzo la chosaopsa hyperbilirubinemia

Kwenikweni bilirubin - ndi pigment ya ndulu, ali ndi khalidwe pabuka-chikasu. Yopangidwa ndi mankhwala mu hemoglobin ku erythrocytes amene apasuke chifukwa ndi kusintha involutive maselo chiwindi, ndulu, zimakhala connective, ndi mafupa.

Chosaopsa hyperbilirubinemia ndi matenda palokha zikuphatikizapo pigment steatosis, yosavuta Holem banja, zaumbanda likukulira matenda a chikasu ndiyo, si hemolytic matenda a chikasu ndiyo banja, oyendetsera dziko chiwindi kukanika zinchito distention matenda a chikasu ndiyo ndipo hyperbilirubinemia. matenda akuwonetseredwa promezhayuscheysya kapena aakulu obstructive matenda a chikasu ndiyo, kusonyeza chiwindi anthu ndi matabwa ake popanda chisokonezo kwambiri. No ndipo nthawi yomweyo waledzera wa cholestasis ndi kuchuluka hemolysis.

Chosaopsa hyperbilirubinemia (malamulo ICD 10: E 80 - matenda General ndi porphine bilirubin kagayidwe) ali ndi zizindikiro zotsatirazi E 80,4 E 80,5 E 80,6 E 80. Choncho ndiyotengera: Gilbert syndrome, Crigler syndromes, matenda ena - Dubin-Johnson syndrome ozungulira ndi syndromes, unadjusted kuphwanya kagayidwe bilirubin.

zimayambitsa

Chosaopsa hyperbilirubinemia akuluakulu nthawi zambiri - Kodi matenda ndi khalidwe banja, iwo kudzera mwa mtundu wamphamvu. Izi zikutsimikiziridwa ndi yochizira matenda.

Pali postgepatitnaya hyperbilirubinemia - ukapezeka monga chotsatira cha tizilombo pachimake chiwindi. Ndi chifukwa cha matenda angathe kutumikira monga kale opatsirana mononucleosis, pambuyo ayambe odwala kuona zizindikiro za hyperbilirubinemia.

Chifukwa cha matenda ndi kulephera nzeru bilirubin. The seramu wa ukuwonjezeka thunthu, pali chisokonezo kapena adani kapena kusamusa mu chiwindi ku maselo plasma.

okhumudwa ndi zotheka milandu anthu pamene pali chisokonezo mu ankameteramo ubweya wa bilirubin acid glyukuropovoy, n'zomveka chokhalitsa kapena zosakhalitsa kuchepa kwa enzyme ndi monga glyukuroniltrapsferaza.
njira izi yodziwika ndi hyperbilirubinemia Gilbert syndrome, Crigler-Najjar ndi postgepatituyu hyperbilirubinemia. Ozungulira syndromes ndi Dubin-Johnson seramu bilirubin ukuwonjezeka chifukwa chisokonezo ekskrektsii pigment mu ya ndulu canaliculi mwa hepatocyte Kakhungu.

zinthu kukulitsa

Chosaopsa hyperbilirubinemia, matenda zikutsimikiziridwa ndi chakuti nthawi zambiri wapezeka mu unyamata, angasonyeze zizindikiro zawo kwa zaka zambiri ndipo ngakhale moyo. Anthu, matenda wapezeka kwambiri kawirikawiri kuposa akazi.

Mawonetseredwe tingachipeze powerenga za matenda - chikasu sclera ndi, icteric mtundu wa khungu kumachitika milandu, osati nthawi zonse. Chiwonetsero cha hyperbilirubinemia ali likukulira kawirikawiri, nthawi zina, ndi okhazikika, Sizilowana.

Kulimbikitsa matenda a chikasu ndiyo imfayo zinthu zotsatirazi:

• kutchulidwa thupi kapena mantha kutopa;
• exacerbation la matenda, biliary thirakiti chotupa;
• Chitetezo chokwanira mankhwala;
• chimfine;
• Various opaleshoni njira;
• mowa wambiri.

zizindikiro

Kupatula wa sclera ndi khungu utoto mtundu wachikasu, odwala kumva chisoni mu lamanja nusu chapamwamba. Pali nthawi zina pamene kusokonezedwa dyspepsia - ndi nseru, kusanza, mkhutu chopondapo, kusafuna kudya, kuchuluka flatulence mu intestine lapansi.

Mawonetseredwe hyperbilirubinemia kungachititse kuti maonekedwe astenovegetativnogo matenda amene kumawonetsera ngati maganizo, kutopa, kufooka mwauzimu.
Kuzionetsetsa, dokotala mu malo oyamba amasamalirira ku chikasu sclera ndi matte-chikasu kum'chitira kwa khungu la mtima. Nthawi zina, khungu sabwerera chikasu. Chiwindi palpated pa nsonga ya Chipilala costal, ndipo sangathe n'komwe. Zimachitika kuwonjezeka pang'ono kukula thupi, chiwindi zofewa ndi odekha ndi palpation ndi chopweteka. Ndulu si ikukula. Kuchotserapo ndi pamene chosaopsa hyperbilirubinemia kumachitika monga chotsatira cha matenda a chiwindi. Postgepatitnaya hyperbilirubinemia akhoza zimachitika pambuyo matenda opatsirana - mononucleosis.

Syndrome chosaopsa hyperbilirubinemia

Pakuti chosaopsa hyperbilirubinemia monga asanu syndromes kobadwa nako mu yochizira matenda:

• Crigler-Najjar syndromes, mtundu 1 ndi 2;
• Dubin-Johnson syndromes;
• Gilbert syndrome;
• Ozungulira syndromes;
• matenda Baylera (osowa);
• Lucy-Driscoll syndrome (osowa);
• zaka banja chosaopsa cholestasis - mtima wabwino hyperbilirubinemia (osowa).

Onse syndromes izi zimachitika chifukwa kagayidwe wa bilirubin, ngati mlingo wa unconjugated bilirubin mu magazi kwambiri chifukwa, amene amasonkhana zimakhala ndi. Conjugation wa bilirubin kungathandize kwambiri m'thupi, ndi bilirubin kwambiri poizoni n'kukhala otsika-poizoni diglyukoronid - sungunuka pawiri (conjugated bilirubin). Free mawonekedwe bilirubin mosavuta likulowerera nsalu zotanuka ndi anapitiriza mu nembanemba wa mucous, khungu pa Mitsempha, motero chimayambitsa matenda a chikasu ndiyo.

syndrome Crigler-Najjar

madokotala US Nayyar V. ndi J .. Kriegler mu 1952 anatchula syndrome latsopano ndipo analongosola. Iye ankatchedwa Crigler-Najjar syndrome mtundu 1. Matenda kobadwa nako ali autosomal retsensivny cholowa. Kukula kwa matendawa amapezeka nthawi yomweyo maola woyamba atabadwa. Pali zizindikiro zofanana mofanana zambiri anyamata ndi atsikana.

The pathogenesis matendawa amayamba ndi kusowa wathunthu wa enzyme ndi monga UDFGT (enzyme urndin-5-difosfatglyukuroniltransferazy). Pamene syndrome mtundu 1 UDFGT kwina kwathunthu, free bilirubin ukuwonjezeka zikuchepa, zizindikiro kufika 200 mol / L, ndipo ngakhale zambiri. Atabadwa tsiku loyamba mkulu permeability p. Magazi ubongo chotchinga. Mu ubongo (imvi kanthu), pali kudzikundikira mofulumira pigment chikasu osauka kernicterus. Kordiamin mayeso ndi matenda a mtima wabwino hyperbilirubinemia, phenobarbital - negative.

Bilirubin encephalopathy kumabweretsa chitukuko cha nystagmus, minofu hypertonia, athetosis, opisthotonos, clonic ndi khunyu zimandilimbikitsa. Kuti madokotala ananena zawo ndi osauka kwambiri. Popeza mankhwala tima tsiku loyamba mwina amapha. Chiwindi ndi sanasinthe pa autopsy.

syndrome Dubin-Johnson

Syndrome chosaopsa hyperbilirubinemia Dubin-Johnson anafotokozedwa koyamba mu 1954. Makamaka matenda ndi ofala pakati pa anthu a Middle East. Amuna pansi zaka 25 amapezeka mu 0.2-1% ya milandu. Madalitso amapezeka mu autosomal unsembe lalikulu. syndrome uyu ali pathogenesis amene amagwirizana ndi chisokonezo ntchito zoyendera za hepatocyte bilirubin ndipo Kuchokera m'menemo chifukwa kusatsatira zoyendera ATP amadalira dongosolo khungu nembanemba. Motero, yobereka bilirubin mu ya ndulu wasweka, pali Reflux wa bilirubin mu mwazi wa hepatocyte lapansi. Izi zikutsimikiziridwa ndi pachimake cha utoto ndende mu magazi maola awiri pa mlandu ntchito bromsulfaleina.

Morphological khalidwe Mbali - ndi chokoleti-akuda chiwindi, kumene mkulu kudzikundikira coarse granular pigment. Kuwonekera kwa matendawa: yellowness zonse kapena likukulira kuyabwa, kupweteka kumanja mu nusu chapamwamba, zizindikiro asthenic, dyspepsia, kukulitsa a ndulu ndi chiwindi. Isanayambike matenda kungakhale pa msinkhu uliwonse. Pali chiopsezo pambuyo ntchito yaitali wothandizila m'thupi kulera zosiyanasiyanazi, komanso pa mimba.

Kupeza Vuto matenda zochokera bromsulfaleinovoy chitsanzo pa holetsistografii pa excretion pang'onopang'ono mu ya ndulu Mosiyana wothandizila, pakalibe kudetsa mu ndulu ya. Kordiamin mu Kupeza Vuto chosaopsa hyperbilirubinemia mu nkhani iyi si ntchito.

Total bilirubin index si upambana 100 mol / l, free ndi conjugated bilirubin chiŵerengero ndi 50/50.

Chithandizo cha matenda alibe yapangidwa. Moyo amayembekezeka syndrome si bwanji, koma khalidwe la nzeru kudwala izi worsens.

Chosaopsa hyperbilirubinemia - syndrome Gilbert a

matenda otengera ndi ambiri, kumuuza za izo mwatsatanetsatane. Matenda opatsirana kwa makolo ana amagwirizana ndi chilema cha mbewu, amene kumatanthauza kagayidwe wa bilirubin. Chosaopsa hyperbilirubinemia (ICD - 10 - E80.4) - kanthu matenda Gilbert a.

Bilirubin - imodzi yofunika kwambiri ya ndulu inki wapakatikati kuwonongedwa hemoglobin umene nawo mayendedwe a oxygen.

kuchepetsa bilirubin (80-100 micromol / L), ndi preponderance kwambiri omwe sanali magazi mapuloteni (mozungulira) bilirubin kumabweretsa nthawi mawonetseredwe matenda a chikasu ndiyo (nembanemba mucous, sclera, khungu). Pankhaniyi, chiwindi mayesero ntchito, zizindikiro zina zachilendo. Anthu, matenda Gilbert ndi 2-3 wamba kuposa akazi. Kwa nthawi yoyamba akhoza kuoneka zaka zapakati pa zaka zitatu ndi atatu. Kawirikawiri matenda limodzi ndi moyo wa anthu onse.
Ndi matenda Gilbert ndi fermentopaticheskie chosaopsa hyperbilirubinemia (pigment gepatozy). Pali zambiri chifukwa mosadziwika (unconjugated) nusu ya ndulu inki. Ichi ndi chifukwa nthenda ya kumtundu wa chiwindi. Nthawi ya chosaopsa - kusintha aakulu chiwindi, anasonyeza hemolysis amapezeka.

Zizindikiro za matenda Gilbert a

Gilbert ndi matenda alibe zizindikiro chitaperekedwa amapezeka ndi zizindikiro kochepa. Madokotala ena osaganizira za matenda monga matenda, ndipo Chibwezeni kwa makhalidwe thupi la chamoyo.

Mawonetseredwe okha nthawi zambiri wofatsa matenda a chikasu ndiyo kudetsa ntchitoyo nembanemba wa mucous, khungu, diso sclera. Zizindikiro zina kapena wofatsa, kapena sadzafika konse.
Osachepera zotheka zizindikiro ubongo:

• kufooka;
• chizungulire;
• kutopa;
• matenda tulo;
• kusowa tulo.

Ngakhale zizindikiro yosowa kwambiri za matenda Gilbert - pamenepa dyspepsia (kudzimbidwa)

• pakalibe kapena kuchepetsa kudya;
• owawa belching pambuyo kudya;
• kutentha;
• owawa aftertaste pakamwa; kawirikawiri kusanza;
• Ndikumva chisoni, chidzalo pamimba;
• kuvutika chopondapo (m'mimba kapena kudzimbidwa);
• ululu ufulu chapamwamba nusu kukometsa khalidwe kupweteka. Angayambe pamene zolakwa zakudya nkhanza zakudya pachimake ndi mafuta;
• pangakhale kuwonjezeka mu chiwindi.

Chosaopsa hyperbilirubinemia: chithandizo

Ngati mulibe matenda ena okhudzidwa wa kapepalako m'mimba mwa chikhululukiro dokotala chimaika zakudya №15. Mu gawo pachimake, ngati pali concomitant ndulu matenda, zakudya №5 yoikidwiratu. chithandizo chapadera odwala chiwindi si chofunika.

Zothandiza mu milandu, vitamini mankhwala ntchito ndalama choleretic. Ndipo musati amafuna odwala kugwira wapadera spa chithandizo.
Magetsi kapena matenthedwe mankhwala mu chiwindi, osati kuchita zabwino, komanso ndi mavuto. Kuti madokotala ananena zawo ndi zabwino ndithu. Odwala amatha ntchito, koma m'pofunika kuchepa mitsempha, komanso zolimbitsa thupi.

Chosaopsa hyperbilirubinemia ndi Gilbert sikutanthauza chithandizo chapadera. Odwala ayenera kutsatira ena ayamikira, kuti chifukwa mawonetseredwe pachimake cha matenda.

Table №5.

• Anandilola ntchito: tiyi ofooka, madzi, otsika-mafuta kanyumba tchizi, tirigu mkate msuzi ndi msuzi masamba, ng'ombe, otsika-mafuta, khirisipi monga chimanga, nkhuku, wowawasa zipatso.
• Ndi zoletsedwa kudya: nyama yankhumba, mitanda atsopano, sipinachi, sorelo, nyama mafuta, mpiru, mafuta nsomba, ayisikilimu, tsabola, mowa, wakuda khofi.
• Kupembedza - ali kwathunthu inathetsedwa zolimbitsa thupi katundu. Kugwiritsa ntchito mankhwala mankhwala: anticonvulsants, mankhwala anabolic mankhwala ngati n'koyenera - analogues mahomoni kugonana, amene ntchito zochizira kusokonezeka m'thupi, komanso othamanga wa - kulimbikitsa ntchito othamanga.
• Kwathunthu kusiya kusuta, kumwa mowa.

Ngati mwakumana ndi zizindikiro za matenda a chikasu ndiyo, adokotala akhoza mankhwala angapo mankhwala.

• barbiturates Gulu - mankhwala antiepileptic zimathandiza kuchepetsa mlingo wa bilirubin.
• Ya ndulu zikutanthauza.
• Njira, amene kusonkhezera pa ntchito ya ndulu ndi ducts ake. Kupewa chitukuko cha cholelithiasis, cholecystitis.
• Hepatoprotectors (kuteteza njira kuteteza maselo kwa chiwindi motsutsana kuwonongeka).
• Chelators. Njira utithandize excretion wa bilirubin kwa m'matumbo.
• michere m'mimba amaikidwa ndi matenda dyspeptic (nseru, kusanza, flatulence) - chithandizo chimbudzi.
• Phototherapy - kukhudzana ndi nyali kuwala buluu kumabweretsa kuwonongedwa kwa minofu atathana bilirubin. Pofuna kupewa diso amayaka afunika kutetezedwa.

Kukhazikitsa malangizo awa, odwala ndi milingo wabwinobwino wa bilirubin, zizindikiro zikayamba kwambiri kamodzikamodzi.