Hyperbilirubinemia Koló: tātaritanga me te maimoatanga

kia kōwhaitanga o te kiriuhi mucous, mā karu kanohi me te kiri kia mataara o tetahi tangata. Te tangata matau e tohu enei tohu ki etahi kōtītiti i te mahi o te taua tinana nui rite te ate. kia tonu ēnei ma'i e whakahaeretia e te tākuta. e hoatu e ia te tātaritanga tika, me te whakahau i te maimoatanga tika. I ngā taumata teitei o te bilirubin te tikanga puta yellowness. hyperbilirubinemia Koló hoki he rite tohumate. Ko te tuhinga tātari i roto i te taipitopito atu he aha tenei mate ko, he aha e ona take me te maimoatanga.

Whakamāramatanga o te hyperbilirubinemia Koló

I roto i te ngako bilirubin - he ngarahu ate, te mea he tae whero-kōwhai āhuatanga. I whakaputahia e tenei rawa i roto i te pūtoto i erythrocytes e fatifati i tika ki te huringa involutive o ngā pūtau ate, kōateate, kopa tūhonohono, me te wheua wheua.

hyperbilirubinemia Koló he mate motuhake ngā ngarahu steatosis, whānau Holem ohie, jaundice küao taratahi, kore-hemolytic whānau jaundice, pōrearea ate ture, jaundice distention mahi me te hyperbilirubinemia. whakakitea te mate te promezhayuscheysya ma'i jaundice taunutia ranei, faaite ate tangata, me tona hanganga, kahore raruraru nui. No ka i te wa ano tohu kitea o te cholestasis me nui haere hemolysis.

Koló hyperbilirubinemia (waehere ICD 10: E 80 - disorders General me te pākia bilirubin porphine) hoki kua te waehere e whai ake nei E 80,4 E 80,5 E 80.6 E 80. Fakatatau ki whakawaeheretia: Gilbert syndrome, Crigler syndromes, atu disorders - Dubin-Johnson syndrome rotor me syndromes, ofatiraa unadjusted o bilirubin pākia.

take o

hyperbilirubinemia Koló i roto i ngā pakeke i roto i te nuinga o te wā - ko te mate ki te pūāhua whānau, kua tuku ratou i te momo tino. whakapumautia tenei e mahi hauora.

He postgepatitnaya hyperbilirubinemia - whakatika rite te putanga o te kakā whakapeka viral. Ko reira ano hoki te take o te mate e taea te mahi mononucleosis hopuhopu rite mua, i muri kia ite i whakaora o te tūroro tohu o hyperbilirubinemia.

Meinga o te mate ko te kore i roto i te utu o te bilirubin. Ko te serum o nga piki taonga, i reira te mea he ngangau hopu ranei whakawhiti reira ki te ate i ngā pūtau wē ranei.

Ko taea i roto i te hunga wā i te āhua taua ka reira te mea he ngangau i roto i te here o te bilirubin me te waikawa glyukuropovoy, he reira kaimahi pūmau rangitahi ranei mārama o te paru pērā i glyukuroniltrapsferaza.
Kei te āhuatanga e hyperbilirubinemia Gilbert syndrome, Crigler-Najjar me postgepatituyu hyperbilirubinemia enei tikanga. syndromes rotor me piki serum bilirubin Dubin-Johnson tika ki raruraru ngarahu ekskrektsii i roto i te canaliculi ate i roto i te membrane hepatocyte.

āhuatanga whakahōhā

hyperbilirubinemia Koló, whakapumautia tātaritanga e te meka e maha te kitea te reira i roto i te taiohitanga, taea faaite mai ratou tohu mō ngā tau maha, me te ara te ora. I roto i te tangata, kitea tenei mate te nui atu pinepine atu i te wahine.

Ko te faaiteraa matarohia o te mate - kōwhaitanga o te teatea, tae icteric o te kiri kia puta i roto i ngā take takitahi, e kore tonu. E taratahi faaiteraa o hyperbilirubinemia maha, i roto i wā onge, he pūmau, e kore e haere.

Te whakapakari i jaundice kia whakapataritari i te āhuatanga e whai ake nei:

• korerotia te rohirohi tinana ranei io;
• exacerbation o mate, biliary lesion tuleki;
• raau taero papare;
• maremare;
• ngā tukanga hāparapara Various;
• horomanga waipiro.

tohu

Te kore e o te teatea me te kiri pania te i roto i te tae kōwhai, tūroro ite pouri i roto i te hautahi o runga tika. He wā ka ohorere dyspepsia - he whakapairuaki, ruaki, kumete waimaero, kore o te hiahia, nui haere flatulence i te whēkau.

Ka taea e Faaiteraa hyperbilirubinemia arahi ki te ahua astenovegetativnogo disorders i whakaatu rite te korekore, rohirohi, rohirohi tere.
I te whakamātautau, te tākuta i te wahi tuatahi utu whakarongo ki te kōwhaitanga o te teatea me te urukehu Hāmā-kōwhai ki te kiri o te manawanui. I roto i ētahi wā, e kore e te kiri tahuri kōwhai. palpated ate i te mata o te kikorangi costal, a kore e taea te hakiri. tupu te reira i te hua paku i roto i te rahi te tinana, riro ngohengohe te ate, me te manawanui ki te palpation he mamae. E kore e tipu haere te kōateate i roto i te rahi. He okotahi ina puta hyperbilirubinemia Koló rite te putanga o te kakā mate. Ka taea hoki e Postgepatitnaya hyperbilirubinemia puta i muri i mate hopuhopu - mononucleosis.

Syndrome hyperbilirubinemia Koló

Hoki hyperbilirubinemia Koló ngā syndromes congenital e whitu i roto i te mahi hauora:

• syndromes Crigler-Najjar, momo 1 me 2;
• syndromes Dubin-Johnson;
• Gilbert syndrome;
• syndromes rotor;
• mate Baylera (onge);
• syndrome Lucy-Driscoll (onge);
• Family tau cholestasis Koló - he hyperbilirubinemia Koló (onge).

puta katoa o enei syndromes tika ki te pākia o bilirubin, ki te nui haere te taumata o te bilirubin unconjugated i roto i te toto e, e piki i te kopa. Conjugation o bilirubin e whai wāhi nui i roto i te tinana, kei te tahuri te bilirubin tino paitini ki iti-paitini i roto i te diglyukoronid - pūhui wairewa (bilirubin conjugated). puka bilirubin Free ngāwari te ô i puritia te papanga tāwariwari te i roto i te kiriuhi mucous, te kiri i runga i nga taiepa o oko toto, ko te kupu ai jaundice.

Crigler-Najjar syndrome

tāutuhia US pediatricians Nayyar V. ko J .. Kriegler i roto i te 1952 i te syndrome hou, a whakaahuatia ana e ia. I huaina e ia Crigler-Najjar momo syndrome 1. Tenei mate congenital he kainga autosomal retsensivny. Ko te whanaketanga o te syndrome puta tonu i roto i te haora tuatahi i muri i whanau. He tohu rite ōrite maha e rua ngā kōtiro me mau tamaroa.

meinga te pathogenesis o te mate e oti kore o te paru pērā i UDFGT (whākōkī urndin-5-difosfatglyukuroniltransferazy). Ia matou e ngaro tenei syndrome momo 1 UDFGT rawa, piki bilirubin free koi, tae tohu 200 mol / L, a tae noa atu. I muri i te whanautanga o te ra tuatahi he uruhanga nui p. Toto-roro arai. I roto i te roro (mea hina), i reira te mea he whakaeminga tere o te ngarahu whakawhanake kōwhai kernicterus. whakamātautau Kordiamin ki te tātaritanga o hyperbilirubinemia Koló, phenobarbital - tōraro.

arata'i Bilirubin encephalopathy ki te whanaketanga o te nystagmus, uaua hypertonia, athetosis, opisthotonos, clonic me haehae toniki. Ko tino rawakore te waitohunga. I roto i te ngaro o te maimoatanga kaha i roto i te ra tuatahi pea mate. E kore te te ate ke i tūpāpaku.

Dubin-Johnson syndrome

i whakaahuatia tuatahi syndrome hyperbilirubinemia Koló Dubin-Johnson i roto i te 1954. Te nuinga he noa i roto i noho o te Middle Te Tai Rāwhiti i te mate. Tangata i raro i te tau o te 25 tau kitea i roto i 0.2-1% o te wā. puta tupu i roto i te tikanga tino autosomal. Tenei syndrome he pathogenesis o nei e pā ana te ki raruraru i roto i nga mahi waka o hepatocyte bilirubin me i reira e tika ana ki te pūnaha membrane pūtau taupatupatu waka ATP-ti'aturi. Ka rite ki te putanga, pakaru tuku o bilirubin i ate te, i reira te mea he Reflux o bilirubin i te toto o te hepatocyte. whakapumautia tenei e te tihi o te kukū tae i roto i te toto e rua haora i roto i te tamataraa te whakamahi i bromsulfaleina.

Morphological āhuatanga āhuatanga - te ate tiakarete-tae, te wahi whakaeminga tiketike o te ngarahu potakataka makaka. Faaiteraa o te syndrome: tamau yellowness ranei minamina taratahi, te mamae i runga i te taha ki matau i roto i te hautahi o runga, tohu asthenic, dyspepsia, whakarahi o te kōateate me ate. Tīmatanga o te mate e taea kia i tetahi tau. He he he tūpono i muri i te whakamahi wā roa-o āpiha ārai hormonal, me te i roto i te hapūtanga.

Tātarihia te te mate e hāngai ana tauira bromsulfaleinovoy i holetsistografii i roto i excretion puhoi ki ate kaihoko rerekē i roto i te ngaro o te poke i roto i te gallbladder. Kordiamin i roto i Tātaritia hyperbilirubinemia Koló i roto i tenei take e kore e whakamahia.

e kore e taupū bilirubin Tapeke nui 100 mol / l, free, me te conjugated ōwehenga bilirubin ko 50/50.

Kua kore i whakawhanakehia maimoatanga o te syndrome. e kore te ora tümanako syndrome pānga, engari worsens te kounga o te oraraa i roto i tenei māuiuitanga.

hyperbilirubinemia Koló - syndrome o Gilbert

Ko te noa tino tenei mate tuku, korero e pā ana ki reira i roto i te kōrero atu. Mate tuku i ngā mātua ki ngā tamariki e pā ana te ki te koha o te ira, whai wāhi nei i roto i te pākia o bilirubin. hyperbilirubinemia Koló (ICD - 10 - E80.4) - he mea rite syndrome o Gilbert.

Bilirubin - tetahi o nga uhi tino nui ate wehenga takawaenga o te pūtoto whai wāhi nei i roto i te transportation o te hāora.

taumata bilirubin nui haere (80-100 micromol / L), he Tuhinga nui o toto-kore pūmua (autaki) bilirubin arata'i ki faaiteraa wā jaundice (kiriuhi mucous, mā karu, kiri). I roto i tenei take, whakamātautau mahi ate, he noa atu tohu. I roto i te tangata, ko te 2-3 wā atu noa atu i roto i nga wahine syndrome o Gilbert. Hoki te wā tuatahi ka taea e puta i waenganui i nga tau o e toru ki te kotahi tekau ma toru nga tau. E mea pinepine tahi te mate e te ora katoa tangata.
E Gilbert syndrome he hyperbilirubinemia Koló fermentopaticheskie (ngarahu gepatozy). He nuinga o tika ana ki autaki pigments ate (unconjugated) hautanga. he tika ki te hē iranga o te ate tenei. Ko te takanga o mārire - huringa matotoru i roto i te ate, faaite puta hemolysis.

Tohu o te syndrome o Gilbert

syndrome o Gilbert e kore e whai tohumate korerotia, puta ki te tohu iti. e kore e ētahi kaiwhakaako whakaaro te syndrome rite te mate, a ka kōrero ai ki te āhuatanga whaiaroaro o te rauropi.

Te faaiteraa anake i roto i te nuinga o te wā e ngawari mahi jaundice poke o nga kiriuhi mucous, kiri, mā karu kanohi. Ētahi atu tohumate ranei ngawari, e kore ranei tupu i te katoa.
Mōkito tohu neurological taea:

• ngoikore;
• whanoke;
• rohirohi;
• disorders moe;
• ohoroa.

Ahakoa tohu nui atu te utu o te syndrome o Gilbert - te dyspepsia (tokopā)

• ngaro whakaiti o te hiahia ranei;
• te paowa kawa i muri kai;
• tokopā;
• aftertaste kawa i roto i te mangai; uaua ruaki, whakapairuaki;
• he mana'o o pouri, e hua ana i roto i te puku;
• abnormalities kumete (korere kōroke ranei);
• mamae i roto i te tika hautahi o runga pūāhua ate puhoi. Ka taea e puta, no te hapa i roto i te kai, te tūkino o ngā kai whakapeka, me te fatty;
• reira kia kia he hua i roto i te ate.

hyperbilirubinemia Koló: maimoatanga

Ki te mea i reira e kore mate pānga o te wahi gastrointestinal i roto i te murunga tākuta e whakaturia ana kai №15. I roto i te wā whakapeka, ki te mea i reira te mea he mate gallbladder te rere, kai №5 whakaritea. e kore te maimoatanga Special mō tūroro ate hiahiatia.

Whai hua i roto i enei take, huaora therapy, te whakamahi o moni choleretic. A e kore e rapua e tūroro ki te pupuri maimoatanga kaukau motuhake.
maimoatanga Electrical waiariki ranei i te ate, mahi kore anake kahore pai, engari ano hoki i ngā pānga kino. Ko te tino pai te waitohunga. E taea e ki te mahi tūroro, engari he mea e tika ana ki te whakaiti i te karere, me te mahi tinana.

e kore e hyperbilirubinemia Koló me Gilbert rapu maimoatanga motuhake. kia whai tūroro etahi tūtohutanga i roto i te tikanga e kore e ki te meinga faaiteraa whakapeka o te mate.

Ripanga №5.

• Whakaaetia ki te whakamahi i: tea ngoikore, wai, ngako-iti tīhi, witi taro hupa ki hupa huawhenua, mīti, ngako-iti, totokore e hōu, heihei, hua kawa.
• Kei te riria te reira ki te kai: pēkana, pōhā hou, kōkihi, he kopurepure, ngako kai, pua nani, fatty ika, ice cream, pepa, waipiro, kawhe pango.
• Pipiki - Kei te tino whakakorea mahi taimaha. Ko te whakamahi o rongoā whakahaunga: anticonvulsants, paturopi, steroids anabolic, ki te tika - analogues o hormones sex, e kua whakamahia mo te maimoatanga o te tauwhatinga hormonal, me te te kaitäkaro - ki te whakarei ake i mahi sipoti.
• Mutu hoatu ake paowa, inu waipiro.

Ki te wheako koe tohu o jaundice, kia whakahau i te tākuta he maha o rongoā.

• barbiturates Group - he whai hua i roto i te whakaiti i te taumata o te bilirubin taero antiepileptic.
• tikanga ate.
• Founga, e whakaawe i te mahi o te gallbladder me ona aerodynamics. Ārai i te whanaketanga o te cholelithiasis, cholecystitis.
• Hepatoprotectors (tiaki tikanga tiaki i ngā pūtau ate ki kino).
• Chelators. Founga o te whakarei ake i te excretion o bilirubin i te punuatuna.
• E whakaritea whākōkī kūnatu ki disorders dyspeptic (whakapairuaki, ruaki, flatulence) - āwhina nakunaku.
• Kūmanu - te rongo ki rama puru marama arata'i ki te whakangaromanga o te kiko bilirubin whakaritea. Me ki kia tiakina ki te ārai i te kanohi weranga.

Ko te whakatinanatanga o enei tūtohutanga, tūroro whai taumata noa o bilirubin, nui iti pinepine tohu puta.