Benign hyperbilirubinemia:ʻike, a me ka lapaʻau

Yellowing o ka mucous membranes, maka sclera a me ka ili e e Tilly i o kekahi kanaka. Ua noa i ike ia mau symptoms kuhikuhi i kekahi mau paewaewa ma ka hana o ia ka nui kino me ke akepaʻa. Mau maʻi puʻuwai E mau e noho mana ana ma kekahi kauka. He makemake i ka mea pololeiʻike, a me ka hoakaka i ka pono lapaau. Ma ka 'ilikai ai kiekie o ka bilirubin ana ikeia yellowness. Benign hyperbilirubinemia kekahi, ua like symptoms. I ka 'atikala kālailai i hou au mamuli mea keia maʻi ka, mea i kona mea, lalau mai a me ka lapaau.

Definition o benign hyperbilirubinemia

Ma o Mānoa, bilirubin - he bile pigment, ka mea, i ke ano ulaula-yellow waihoʻoluʻu. Hua mai ma kēia waiwai i loko o hemoglobin mai erythrocytes i disintegrate no i ka involutive loli o ke akepaa keena, spleen, connective nā'aʻaʻa, a me ka iwi ka lolo.

Benign hyperbilirubinemia mea kuokoa maʻi kikowaena pigment steatosis, hemahema hoi Holem 'ohana, nā' ōpio intermittent jaundice, ole-hemolytic jaundice ohana, Kumukanawai ake dysfunction, ooieoeiiaeuiiai distention jaundice a me ka hyperbilirubinemia. I ka maʻi ua hoikeia promezhayuscheysya a maʻi obstructive jaundice, ka hoike kanaka ake a me kona 'ole me ka nui ka haunaele. ʻAʻole a ma ka ia manawa maopopo symptoms o cholestasis, a mahuahua hemolysis.

Benign hyperbilirubinemia (kuhi ICD 10: E 80 - General kāna hana, a porphine bilirubin pūnao) no hoi i na kēia nā loina E 80,4 E 80,5 E 80,6 E 80. ¶ encoded: Gilbert maʻi pale, Crigler syndromes,'ē aʻe kāna hana - Dubin-Johnson maʻi pale epaepa huila a me syndromes, unadjusted kue o ka bilirubin pūnao.

kumu o ka

Benign hyperbilirubinemia i loko o nā mākua i loko o ka hapanui o na hihia - ua ka maʻi me ka ohana ano, ka mea i hiki e Hoʻohaku type. Keia ua hookupaa iho la ia ma ka 'inikua hana.

Aia postgepatitnaya hyperbilirubinemia - aicieeaao like me kaʻilihune o ka mea hoʻomaʻi huʻi hepatitis. Ua mea no hoi ke kumu o ka maʻi hiki ke malama i hala infectious mononucleosis, ma hope o ke ola ana o nā mea maʻi i ka mahina symptoms o hyperbilirubinemia.

Kumu o ka maʻi o ka hoʻokō 'ole' ana ma o ka wehe aʻe o ka bilirubin. Ka lāʻau lapaʻau ana o ka waiwai, piʻi pū, aia no he haunaele a me ka hopu a hoolilo ia i loko o ke akepaʻa, mai ka wai koko keena.

Oia ka moku'āina mea hiki i loko o ia mau hihia wā malaila mea he haunaele ma ka paa ana o bilirubin a me ka glyukuropovoyʻakika, ia mea maopopo paʻa paha? Aiaiiuie shortage o ka'ūhū e like me glyukuroniltrapsferaza.
Mau maomeka i wehewehe aku ma ka hyperbilirubinemia Gilbert maʻi pale, Crigler-Najjar a me postgepatituyu hyperbilirubinemia. Epaepa huila syndromes a me Dubin-Johnson lāʻau lapaʻau bilirubin, piʻi pū aie i ka haunaele ekskrektsii pigment i loko o ka bile canaliculi ma ka hepatocyteʻili lahilahi.

ano hoomainoino aʻe

Benign hyperbilirubinemia,ʻike, ua hookupaa iho la ia ma ka mea i pinepine ia ua 'akomi i ka adolescence, ke hoike i ko lakou symptoms no na makahiki, a hiki i ka wā e ola ana. I na kanaka, i kēia maʻi ua 'akomi i ka nui loa aku ma mua o nā wāhine pinepine ia.

Ke Classic hoike ana o ka maʻi - yellowing o ka sclera, icteric kala like 'ole o ka ili kānāwai i loko o kēia hihia,ʻaʻole e mau ana. Hōʻike o ke kīnā o ke hyperbilirubinemia i intermittent pinepine, i pohihihi keia hoopii, ua paʻa, mai kekahi.

E hooikaika ana jaundice e hoʻonāukiuki i nā kēia kumumea:

• haʻi kino ai 'ole ko mākou hopohopo exhaustion;
• exacerbation o ka maʻiʻaʻai, biliary'āpana lesion;
• E hoʻonele ai ';
• ke anu;
• Ke Kula Kaiapuni 'ʻoki lāʻau lapaʻau kaʻina hana;
• waiʻonaʻai ana.

symptoms

Ina aole ia o ka sclera, a me ka ili ua pena i loko o melemele waihoʻoluʻu a pau, e hoomanawanui i manao ehaeha ma ka akau maluna quadrant. Aia nā manawa a ka wā pilikia dyspepsia - he nausea, ka luaʻi, hoʻopōʻino noho, nele o ka ikaika loa ka makemake, mahuahua flatulence i loko o ka naʻau liʻiliʻi.

Hoike ana hyperbilirubinemia ke alakai i ka nānā aku astenovegetativnogo kāna hana i hoike mai ai ia e like me ka kaumaha, ka luhi, kokololio luhi.
Ma hookolokolo, i ke kauka i loko o ka wahi mua uku noonoo ana i ka yellowing o ka sclera a me ka matte-melemele tinge i ka ili o ka hoʻomanawanui. Ma kekahi mau hihia, ma ka ili, aole ia i huli yellow. Ake palpated ma ke kihi o ka costal e pio mai, aʻaʻole hiki ke ikeia. Ua hana kekahi hōʻemi iki mahuahua ma ke kino nui, ke ake i pahee a me ka hoomanawanui me ka palpation mea ola. Ke spleen ua ole e ulu ana i loko o ka nui. 'Ia i ka wā benign hyperbilirubinemia ia lŘlŘ like me kaʻilihune o ka hepatitis maʻi. Postgepatitnaya hyperbilirubinemia hiki no hoi i hoʻomaka ai ma hope o infectious maʻi - mononucleosis.

Maʻi pale benign hyperbilirubinemia

No ka benign hyperbilirubinemia nā ehiku congenital syndromes i loko o ka 'inikua hana:

• Crigler-Najjar syndromes, 'ano 1 a me 2;
• Dubin-Johnson syndromes;
• Gilbert maʻi pale;
• Epaepa huila syndromes;
• Baylera maʻi (pohihihi);
• Lucy-Driscoll maʻi pale (pohihihi);
• Family makahiki benign cholestasis - he benign hyperbilirubinemia (pohihihi).

A pau o kēia mau syndromes ana aie i ka pūnao o bilirubin, ina ua mahuahua ka nui o unconjugated bilirubin i loko o ke koko, i accumulates ma na nā'aʻaʻa. Radio Conjugation o bilirubin kūlana nui i loko o ke kino, he hoomaikaiʻawahia bilirubin ua hoohuliia i loko o haʻahaʻa-ʻawahia i loko o diglyukoronid - wailana hui (conjugated bilirubin). Free palapala bilirubin hoopuni komo iloko o ka laholio lole ua paa ia ma ka mucous membranes, ka ili ma na paia o na moku koko, penei ke kumu jaundice.

Crigler-Najjar maʻi pale

US pediatricians Nayyar V., a J .. Kriegler i ka makahiki 1952 hōʻike 'i ka hou maʻi pale, a kakau lakou ia mea. He Ua kapa Crigler-Najjar maʻi pale type 1. Kēia congenital kāna hana mai ka autosomal retsensivny hooilina. Ka hooulu ana o ka maʻi pale ia lŘlŘ koke i loko o ka hora mua ma hope o ka hānau. Aia i like symptoms like pinepine nā kaikamahine a me nā keiki.

Ke pathogenesis o ka maʻi ua kena ae la e piha ka nele o ka'ūhū e like me UDFGT ('ūhū urndin-5-difosfatglyukuroniltransferazy). A pau keia maʻi paleʻano 1 UDFGT kahi e loa, noa bilirubin, piʻi pū ikaika, nā hoailona hiki 200 Mol / L, a hiki hou aku. Ma hope iho o ka hanau ana o ka mea mua lā he kiʻekiʻe permeability p. Ke koko-lolo ke'ākeʻakeʻa mai. I ka lolo (oho mea), aia no he kokololio hoʻomāhuahua o pigment yellow ulu kernicterus. Kordiamin oʻu ho'āʻo a me kaʻike, a benign hyperbilirubinemia, phenobarbital - io.

Bilirubin encephalopathy hiki aku ai i ke kūpono 'ana o ke nystagmus, Muscle hypertonia, athetosis, opisthotonos, clonic a me ka tonic convulsions. Ke prognosis He'āina ilihune. I ka wa e kaawale aku o ka mālama nui lapaau i ka mua lā hiki ona. Ke ake ia e hoʻololi i autopsy.

Dubin-Johnson maʻi pale

Maʻi pale benign hyperbilirubinemia Dubin-Johnson i ho'ākāka 'ia ma mua i loko o 1954. Ka hapanui o ka maʻi mea a pau mawaena o nā kupa o ka Middle East. Men ma lalo o ka makahiki o nā makahiki 25 Ua loaʻa i loko o 0.2-1% o ka hihia. Hooili ia lŘlŘ i loko o ka autosomal Hoʻohaku ano. Kēia maʻi pale i pathogenesis o ka i pili i ka haunaele ma ka amo mana o ka hepatocyte bilirubin, a mai ia ma muli o ka inconsistency amo ATP-e kaukaʻiʻili lahilahi aeea nenoaiu. E like me ke no, haawi o bilirubin ma ka bile ua wāwahiʻia, aia no he reflux o bilirubin i loko o ke koko o ka hepatocyte. Keia ua hookupaa iho la ia ma ka piko o ka kalameaʻai lehulehu i loko o ke koko, elua no hora i ka hookolokolo ana me ka hoʻohana 'bromsulfaleina.

Morphological ano hiʻona - ke kokoleka-nā pōpō akepaa, kahi kiʻekiʻe hoʻomāhuahua o ka lohi loa granular pigment. Hoike ana mai o ka maʻi pale: yellowness a eia paha intermittent ka ohane, eha ma kaʻaoʻao'ākau i loko o ka luna quadrant, asthenic symptoms, dyspepsia, hoʻonui o ka spleen a me ka akepaʻa. Hookahi o ka maʻi hiki ia ma kekahi mau makahiki. Aia ka he pilikia ma hope loa-makahiki hoʻohana o hormonal contraceptive konohiki, e like me i hāpai keiki.

Diagnosing i ka maʻi ma muli bromsulfaleinovoy hāpana ma holetsistografii iloko o na mea excretion i loko o bile Akä naÿe hope paha i loko o ka wa e kaawale aku o ka i hookohu aku i loko o ka gallbladder. Kordiamin i diagnosing benign hyperbilirubinemia ma keia hihia ua ole i hoʻohana.

Huina bilirubin inideka, aole ia i oi aku mamua o 100 Mol / l, noa a conjugated bilirubin hiʻohiʻona o 50/50.

Lapaau ana i ka maʻi pale i ole, ua ulu. Life expectancy maʻi pale ua ole iʻa, akā, i ka pono o ke ola i loko o kēia pathology worsens.

Benign hyperbilirubinemia - Gilbert ka maʻi pale

Kēia hereditary mai o ka loa, he pono ole, e hai aku ana ia i loko o oi au mamuli. Maʻi pū i hiki mai na makua i na keiki ua pili i ka hewa o ka'ōewe, i komo i loko o ka pūnao o bilirubin. Benign hyperbilirubinemia (ICD - 10 - E80.4) - mea, aohe mea like Gilbert ka maʻi pale.

Bilirubin - kekahi o ka mea nui wale bile pigments waena hālāwai hope o ka hemoglobin i komo i loko o ka halihali o ka oxygen.

Mahuahua bilirubin pae (80-100 micromol / L), he nui piha kūpono o ka ole-koko kumuʻiʻo (ʻauhau'ēʻaʻe) bilirubin hiki aku ai i ka papa'āweʻaweʻa kemika hoike ana jaundice (mucous membranes, sclera, ili). Ma keia hihia, ake hana ho'āʻo ma, 'ē aʻe nā hoailona e maʻamau. I na kanaka, Gilbert ka maʻi pale mea 2-3 manawa hou, he pono ole ma mua i loko o nā wāhine. No ka mea manawa mua keʻikeʻia ma waena o nā makahiki o ka ekolu i ka umikumamakolu makahiki. Pinepine i ka maʻi ua pu ma kanaka a pau ke ola.
By Gilbert maʻi pale i fermentopaticheskie benign hyperbilirubinemia (pigment gepatozy). Aia i IeAUPIIe, IAa IO ma muli o keʻauhau'ēʻaʻe (unconjugated) mahele bile pigments. Kēia mea ma muli o ke aaiaoe mā o ke akepaʻa. Ka hanana o benign - loli loa i loko o ke akepaa, kaheaia hemolysis ia lŘlŘ.

Symptoms o Gilbert ka maʻi pale

Gilbert ka maʻi pale aʻole i haʻi symptoms, ia lŘlŘ me ka palena haʻahaʻa symptoms. Kekahi kauka mai i noonoo i ka maʻi pale i ka maʻi, a i kapa ia i ka physiological ano o ka meaola.

Ke wale hoike ana i loko o ka hapanui o na hihia i mehana jaundice i hookohu aku i hana o ka mucous membranes,ʻili, maka sclera. Other symptoms a mehana, aʻaʻole i hoʻomaka ai i nā mea a pau.
Palena iki hiki neurological symptoms:

• nawaliwali;
• pōniuniu;
• luhi;
• hiamoe kāna hana;
• Kanikani'āʻula.

E like hou pohihihi keia symptoms o Gilbert ka maʻi pale - he dyspepsia (indigestion)

• wa e kaawale aku ai ka ho'ēmiʻana o ka ikaika loa ka makemake;
• awaawa belching ma hope o kā lākouʻahaʻaina;
• heartburn;
• awaawa aftertaste i loko o ka waha; kākaʻikahi ka luaʻi, nausea;
• i ka manao o ka naau, nui i loko o ka abdomen;
• noho abnormalities (diarrhea ole constipation);
• eha ma ka akau maluna quadrant hoopaa aching külana. Hiki ke hana ia ka wahaheʻe i loko o kaʻai, i ka hainā a huʻi a me ka momomaʻai;
• aole paha e ka hua i loko o ke akepaʻa.

Benign hyperbilirubinemia: lapaʻau

Inā he mea i hui pu maʻi o ka gastrointestinal'āpana i loko o ke kala kauka e koho iʻai №15. Ma ka huʻi pae, ina he mea kekahi concomitant gallbladder maʻi,ʻai №5 hoonohoia. Special lapaʻau no ka ake nā mea maʻi ua i koi 'ia.

Pono keia mau mea i loko o kēia mau hihia, wikamina Inc, ka hoʻohana 'ana o choleretic kālā. A mai koi nā mea maʻi e paa kuikawa Spa lapaau.
Uila a thermal kūlana kanaka i loko o ke akepaa,ʻaʻole wale nō ke hana i ka maikaʻi, akā, no hoi i nā palapala 'ole. Ke prognosis mea he maikaʻi. e hiki ke hana nā mea maʻi, akā, he mea pono e emi ke aʻalolo, like hoʻi me physical haʻawina.

Benign hyperbilirubinemia a Gilbert 'aʻole i koi kuikawa lapaau. Nā mea maʻi e hahai i kekahi koi ma ka mea,ʻaʻole i ka i huʻi hoike ana mai o ka maʻi.

Oaaeeoea №5.

• 'Ae' ia ka hoʻohana: nawaliwali kī, wai, haʻahaʻa-momona e eiooaa? Ka waiū, palaoa berena ai me nō kai, pipi, haʻahaʻa-momona, crisp cereal, moa,ʻawaʻawa hua.
• Ua Ua papaia e ai ai: puaʻa, hou pastries, spinach, kikokiko, ai momona, mākeke, momoma iʻa, hau kalima, kekahi pahūpahū, ka waiʻona, eleele ke kope.
• Adherence - e loa eliminated kaumaha hoounauna. Ka hoʻohana a kuhikuhi lapaʻauʻia: anticonvulsants, antibiotics, anabolic steroid, ina pono - analogues o ke keka hormones, a i hoʻohana ai no ka iapaau ana o hormonal hoʻohuikau, e like me ka 'âlapa - e hōʻoi' kekahi kime pāʻani hana.
• Loa e haawi mai i ka uiki e, inu waiʻona.

Inā e 'ike symptoms o jaundice, ke kauka i hoakaka ia i ka nui o ka lapaʻauʻia.

• barbiturates Group - antiepileptic ai 'i mana ma ka hoemi i ka nui o ka bilirubin.
• Bile 'o ia hoʻi.
• Mea, a ho ololi i ma ka hana o ka gallbladder a me kona ducts. Pale aku i ka hooulu ana o ka cholelithiasis, cholecystitis.
• Hepatoprotectors (mālama hoʻi hepatic keena e ku e poho hoʻopale).
• Chelators. Kumu o ka 'ō ka excretion o bilirubin mai ka gut.
• pau i waeia Digestive nā'ūhū me dyspeptic kāna hana (nausea, ka luaʻi, flatulence) - kōkua digestion.
• Phototherapy - waiū i ka malamalama uliuli kukui hele aku ai a hiki i ka make ana o ka'aʻaʻa paa bilirubin. E pale maka'ā Pono e e hoomaluia.

I ka manaʻo o kēia mau koi, nā mea maʻi i mahana maʻamau o ka bilirubin, symptoms i hoʻomaka ai ka emi pinepine ia.