Hemochromatosis (cirrhosis pigmenti o ka pāpaʻi, keleawe keleawe)

- he maʻi hoʻoilina i hōʻike ʻia e ka nui o ka komo ʻana o ka hao ma loko o ka ʻōpū a me ka hoʻihoʻi ʻana i nā pōpoki hao i loko o nā ʻōpū a me nā ʻili (ʻoi aku ka ʻano o ka hemosiderin) me ka hoʻomohala ʻana o ka fibrosis.

(Yu.N. Tokarev, D.A. Settarova, 1988, me nā hoʻohui).

1. Hereditary (idiopathic, kumu) hemochromatosis.

2. Hemochromatosis lua, hoʻopiha:

2,1. ʻO ka hoʻokuʻu-maʻi (ma ke ʻano o ka anemia maʻi, i ka mālama ʻia ʻana o ke kahe koko e hoʻohana ʻia no ka manawa lōʻihi).

2.2. Alimentary (hemochromatosis o ka ʻohana Bantu ʻAmelika ma muli o kaʻai nui o ka hao me ka meaʻai a me ka wai, ka waiʻī o ka ʻakaʻū o ka ate, ʻo ia paha, kahi maʻi Kashin-Beck, etc.).

2.3. ʻO ka metabola (pākuhi hao hao ʻoi aku ka waena o ka B-thalassemia, i nā mea maʻi me ka cirrhosis o ka ate me ka hoʻomohala a i ʻole noi ana i kahi anastomosis portocaval, me ka pani o ka auhau pancreatic, cutaneous porphyria, etc.).

2.4. No ka huki i hui ʻia (nui thalassemia, kekahi mau ʻano o ka anemia dyserythropoietic - ka hao ka hao, siderohrestical, sideroblastic).

I kēia manawa, ua hōʻoia ʻia ka hana o nā mea genetic i ka hoʻomohala ʻana o ka hemochromatosis idiopathic. ʻO ka prevalence o ka huahelu hemochromatosis hoʻoilina (e kālele ʻia ana ma ka lima pōkole o ka chromosome VI a pili kokoke loa i ka ʻāina o nā antigens o ka pūnaehana HLA histocompatibility) ʻo 0,03-0.07% me ka heterozygosity frequency ma ka lehulehu o ʻEulopa ma kahi o 10%. Hoʻomohala ka maʻi ma nā hihia he 3-5 i kēlā me kēia 1000 mau mea lawe o ka nui o ka hemochromatosis hoʻohele a hoʻouna ʻia e kahi ʻano rectos autosomal. Ua hoʻokūpaʻa ʻia kahi pilina ma waena o ka hemochromatosis hoʻoilina - he mau kaohi o ka huina a me ke ʻano he nui i ka hōʻuluʻulu o ka hao ma loko o nā ʻōpū o loko, a me nā antigens histocompatibility o ka pūnaehana H1A - AZ, B7, B14, Ac

I ka hemochromatosis idiopathic, ka nui o ka hewa o ka hana hewa ʻana o ka hōʻiliʻili o ka hao e ka huina a nā huina o ka mucosa tract gastrointestinal, e alakaʻi ana i ka palena ʻole o ka hao, e ukali ʻia e ka nui o ka hao me ka pigos hemosiderin i loko o ke ʻōpala, pancreas, naʻau, hōʻike, a me nā kino ʻē aʻe (i nele i ka "palena ʻole o ka hao"). Ke alakaʻi nei kēia i kahi make o nā hana hana a me ka hoʻomohala ʻana i ke kaʻina hana sclerotic. Nā hōʻailona maʻi o ka cirrhosis o ka pepi, ka maʻi diabetes mellitus, hele mai nā metabolic cardiomyopathy.

ʻO ke kino o ka mea olakino e loaʻa ana he 3-4 g o ka hao, me ka hemochromatosis - 20-60 g. ʻO kēia ke kumu no ka pilina o 10 mg o ka hao i kēlā me kēia lā i ka hemochromatosis, aʻo kahi pākeke olakino pili ana i ka 1,5 mg (maximum 2 mg. ) No laila, i kahi makahiki he 3 g o ka nui o ka hao i hōʻiliʻili ʻia i loko o ke kino o ka mea maʻi me ka hemochromatosis. ʻO ia ke kumu i hōʻike ʻia ai nā hōʻailona koʻikoʻi o ka hemochromatosis ma kahi o 7-10 mau makahiki ma hope o ka hoʻomaka ʻana o ka maʻi (L. N. Valenkevich, 1986).

Hoʻoulu pinepine ka hemochromatosis me ka cirrhosis o ke akeʻa, ka hōʻemi kino ʻana, ka hoʻonaʻa ʻana i ka protein sakano.

Me ka cirrhosis ate, ka hoʻohaʻahaʻa ʻana o ka transferrin, kahi i hoʻopaʻa ʻia ka hao i loko o ke koko a hāʻawi aku ia i ka ʻōpū o ka iwi (no ka erythropoiesis), i ka kiko (no ka hana o nā enzymes respiratory), a i ka depot hao. Me ka nele o ka transferrin, loaʻa ka hōʻiliʻili o ka hao i hoʻohana ʻole ʻia no ka hana metabolism. Eia kekahi, me ka cirrhosis o ka ate, ka wā o ka ferritin, kahi ʻano kīpana hao.

Ma muli o ka inuʻana i ka waiʻona ka nui o ka hoʻokomo ʻana o ka hao i loko o ka ʻōpū, kahi i hāʻawi ʻia i ka wikiwiki o ka hoʻomaka ʻana o nā hōʻailona o ka hemochromatosis hoʻohele a hōʻeha i ka naʻau a me ka hoʻomohala ʻana o kahi ʻano lua o ka maʻi.

ʻO ka heleʻana o nā anastomoses ma ka waihona pūnaewele e hoʻonui ai i ka waiho ʻana o ka hao i loko o ke akeʻa.

Me ka hao refractory (siderohrestical) anemia a me ka thalassemia nui, ʻaʻole i hoʻohana ʻia ka hao i hoʻopili ʻia, ua kumulāʻu ia a kau ʻia i loko o ka ate, myocardium a me nā mea kino a me nā ʻala.

ʻO ka hapanui ke nānā aku i nā kāne (ratio o nā kāne a me nā wahine 20: 1), kahi ʻano hoʻomohala o kahi maʻi e hōʻike nei iā ia iho i ka makahiki 40-60 mau makahiki. ʻO ka haʻahaʻa haʻahaʻa o ka maʻi i ka wahine ma muli o ka nalowale i ka wahine me ka hao me ke koko menstrual i loko o 25-35 mau makahiki (ʻAi.

ʻO nā hōʻailona koʻikoʻi lapaʻau:

1. ʻO ka pigmentation kiri (melasma) e nānā ʻia ana ma 52-94% o nā mea maʻi (S. D. Podymova, 1984). Hoʻokumu ʻia e ka haʻalele ʻana i ka epidermis o nā pig-free-hao (melanin, lipofuscin) a me hemosiderin. Ma muli o ka paʻakikī o ka pigmentation e pili ana i ka lōʻihi o ka maʻi. He ʻula ʻula, keleawe, hina ka pulu, ʻike ʻia i nā wahi ākea o ke kino (alo, lima), ma nā wahi ʻalā mua, i loko o nā pulima, ma nā wahi kāne.

2. Hoʻomaopopo ka hoʻonui ʻana i ka ate i ka 97% o nā mea maʻi i ka hiki ke hoʻomohala o ka maʻi, maʻi ka palepa, ʻeha pinepine. I ka wā e hiki mai ana, ke kiʻi kino o ka cirrhosis ate me ascites, ulu hypertension, splenomegaly e ulu.

3. ʻO ka maʻi diabetes mellitus e nānā ana i ka 80% o nā mea maʻi, nā maʻi no ka lawe ʻana o ka hao i nā mokupuni o Langerhans, i hōʻike ʻia e ka makewai, polyuria, hyperglycemia, glycosuria. ʻAʻole maʻamau ka paʻakikī o ka acidosis a me ka coma.

4. I kekahi mau pilikia o ka endocrine - hypogenitalism (hoʻohaʻahaʻa i ka potency, ka hoʻoliʻi testicular, ka nalowale ʻana o nā hiʻohiʻona lua, nā wahine, i ka wahine - amenorrhea, ka maʻi), hypocorticism (nāwaliwali koʻikoʻi, hōʻemi i ke koko, hōʻemi ka nui o ka kaumaha).

5. ʻO ka Cardiomyopathy me ka piʻi ʻana o ka puʻuwai, nā hana hoʻonā, ka ulu ʻana i ka ulu ʻana o ka hōʻeha ʻana o ka naʻau, kūpaʻa i ka mālama ʻana me nā glycosides cardiac. 35% o nā mea maʻi me ka hemochromatosis make mai congestive heart failed.

6. Hoʻokomo ʻia ka maʻi metabolic malabsorption e ka disfunction o ka pūpū liʻiliʻi a me ka pancreas ma muli o ka lawe ʻana o ka pigment-hao i loko o kēia mau mea.

He lōʻihi ka papa o ka hemochromatosis maʻamau (15 mau makahiki a ʻoi aku paha), me ka hoʻomohala ʻana o ka cirrhosis ate, ʻaʻole ola ka ola.

keu aku he 10 mau makahiki. Me ka hemochromatosis kekua, ua pōkole ke ola.

1. UAC: nā hōʻailona o ka anemia (ʻaʻole i nā maʻi āpau), hoʻonui ESR.

2. OAM: hiki i ka proteinuria haʻahaʻa, urobilinuria, glucosuria; hiki ke hoʻoulu ʻia ka hao a me ka urine a hiki i ka 10-20 mg i kēlā me kēia lā (maʻamau - a hiki i 2 mg / lā).

3. LHC: ʻoi aku ka nui o ka serum hao ma mua o 37 μmol / L, serum ferritin ma mua o 200 μmol / L, ka nui o ka waihona o ka selaation transferrin me ka hao ʻoi aku ma mua o 50%, hoʻonui i ka ALAT, tg-globulins, hoʻoliʻi thymol, ʻokoʻa hoʻoliʻelima glucose i ʻole pālahalaha maʻi.

4. Hōʻemi ka nui o ke koko o ka 11-ACS, 17-ACS, sodium, chloride, hydrocortisone, kahi hōʻemi i nā lā urine i kēlā me kēia lā o 17-ACS, 17-KS, kahi hōʻemi i ka pae o ka sex sex i ke koko a me ka urine.

5. ʻO kahi puncture mau loa: dictate he kiʻekiʻe ka manaʻo hao.

6. I nā ʻōmaʻomaʻo biopsy o ka ʻili - ka hoʻoneʻe loa o ka melanin, i loko o nā koina biopsy o ke akeʻa - ka haʻalele ʻana o ka hemosiderin, lipofuscin, kiʻi kiʻi o ka cirrhosis micronodular. Wahi a Sartap (1982), ʻo ka nui o ka hao i loko o ke akea i ka wā hemochromatosis primer e hoʻonui ʻia ana he 40 mau manawa hoʻohālikelike ʻia me ke ʻano maʻamau, a ma o 3-5 mau manawa i ka hemochromatosis lua.

7. Hoʻololi i ka hopena - pili i ka hiki o Desferal e hoʻopaʻa i ka ferritin a me ka hao hemosiderin a hoʻoneʻe i ke kino. Hōʻike maikaʻi ʻia kahi hōʻike inā inā ma hope o ke kaha o ka intramuscular 0.5-1 g o ka desferal i kēlā me kēia lā, ʻoi aku ma mua o 2 mg o ka hao ka mea i hoʻopiʻi ʻia i ka urine.

1. ʻO ka ultrasound a me nā radioisotope scanning: hoʻonui i ka nui o ka puhiluhi, pancreas, nā loli i loko o lākou, splenomegaly.

2. FEGDS: me ka hoʻomohala ʻana o ka cirrhosis o ka ate, ua ʻike ʻia nā maʻi varicose o ka esophagus a me ka ʻōpū.

3. Echocardiography: kahi mahuahua o ka nui o ka puʻuwai, ka hōʻemi ʻana i ka hana myocardial contractile.

4. ECG: e hoʻohuli i nā loli i ka myocardium (hoʻemi i ka neʻe o ka T, ka 8-T ka manawa), ka lōʻihi o ke kikowaena (^ -T, nā mea hemahema o ka cardiac.

1. ʻOA o ke koko, kaomi, kaomi ʻana o ka wai no ka glucose, urobilin, bilirubin.

2. LHC: bilirubin, transaminases, ka nui o ka protein a me nā ʻōnaehana o ka protein, glucose, potasiuma, sosa, chlorides, serum hao, serum ferritin, pākēneka transferrin saturation me ka hao. Me ka glycemia maʻamau, kahi hōʻike hoʻoliʻi glucose.

3. Hoʻopili i ka pēpē o ka ate, spleen, pancreas, nā keiki.

6. Ka manaʻo o ka ʻike ʻana o nā hāmeʻa wahine i ke koko, hydrocortisone.

7. Ka hoʻoholo ʻana i ka maʻi maʻamau i kēlā me kēia lā i ka 17-OKS, 17-KS.

ʻO ka cirrhosis pigmentary, aka hemochromatosis: nā hōʻailona a me nā lula o ka mālamaʻana i nā pathology

ʻO Hemochromatosis mua i wehewehe ʻia he maʻi kaawale i ka makahiki 1889. Eia nō naʻe, hiki i ka hana kūpono ke hoʻonohonoho pono i nā kumu o nā maʻi me ka hoʻomohala ʻana i nā ʻano lapaʻau lāʻau.

Kahi ʻia ka ʻano o ka maʻi a me kona neʻina liʻiliʻi.

No laila, e like me ka ʻike o kēia mau lā, ʻo 0.33% o ka poʻe noho o ka honua i mua ka pilikia o ka ulu ʻana o ka hemochromatosis. He aha ke kumu o ka maʻi a he aha nā hōʻailona?

Hemochromatosis - he aha ia?

He hoʻoilina kēia maʻi a hōʻailona ʻia me ka nui o nā hōʻailona a me kahi kiʻekiʻe o nā hōʻeha koʻikoʻi a me nā ala e pili ana.

Ua hōʻike ʻia nā haʻawina ʻo ia ka hemochromatosis i hoʻohana pinepine ʻia e kahi mutation ma ka HFE gene.

I ke hopena o ka hopena o ka gen, ua hoʻohālikelike ka hana o ka hao o ka hao i ka duodenum.. ^ E Ha yM. Ke alakaʻi nei kēia i ka mea i loaʻa i ke kino kahi ʻōlelo wahaheʻe e pili ana i ka nele o ka hao i loko o ke kino a hoʻomaka i ka hoʻoikaika a me nā nui he nui i synthesize i ka protein kūikawā e hoʻopaʻa ana i ka hao.

Ke alakaʻi nei kēia i ka haʻalele nui ʻana o ka hemosiderin (ka puaʻa glandular) i loko o nā kino o loko. Me ka hoʻonui ʻana o ka synthesis protein, hana ʻia ka gastrointestinal tract, e alakaʻi ana i ka hoʻopiʻi nui ʻana o ka hao mai ka meaʻai ma loko o ka ʻawa.

No laila me kaʻai maʻamau, ʻo ka nui o ka hao i loko o ke kino he nui ka manawa ma mua o ka mea maʻamau. Ke alakaʻi kēia i ka luku ʻana i nā kihi o nā kino o loko, nā pilikia me ka ʻōnaehana endocrine, a me ka pale ʻole.

ʻO ke ʻano ma nā ʻano, nā ʻano a me nā pae

Ma ke ʻano lapaʻau, hoʻokaʻawale ʻia nā ʻano maʻi mua a me ke ʻano kiʻekiʻe. I kēia hihia, ka mea nui, i kapa ʻia hoʻi he hoʻoilina, ʻo ia ka hopena o kahi genis predisposition. ʻO ka hemochromatosis lua ka hopena o ka hoʻomohala ʻana i nā ʻoihana i ka hana o nā ʻōnaehana o ka enzyme i pili i ka hana a ka molome.

ʻEhā ʻano ʻano hoʻoilina (genetic) o nā maʻi mai ka maʻi:

• ʻōpio
• hoʻōla
• Kaumaka HFE-unassociated species,
• ua luna autosomal.

Hoʻohui ka ʻaoʻao mua me ke ʻano o ka hana o ka papa chromosome aono. Hōʻike ʻia kēia ʻano ma ka hapa nui o nā hihia - ʻoi aku ma mua o 95 pakeneka o nā mea maʻi e loaʻa mai i ka hemochromatosis papa.

ʻO ke ʻano ala o ka maʻi ʻalani e like me ka hopena o ka mutation i loko o kekahi ʻano ʻē aʻe, ʻo HAMP. Ma lalo o ka hopena o kēia hoʻololi, ke ʻano o ka synteph o hepcidin, ka hoʻāoli a me ke kuleana no ka lawe ʻana i ka hao i loko o nā kino, e hoʻonui nui ʻia. ʻO ka maʻamau, hōʻike ka maʻi i waena o nā makahiki he ʻakū a he kanakolu.

Hoʻohui ka ʻano HFE-unassociated i ka wā i pau ʻole ka hua H HE. Loaʻa kēia ʻano i ka hana o ka hyperactivation o nā mea hoʻokele transferrin-2. A ke hopena, hoʻonui ʻia ka hana hepcidin. ʻO ka ʻokoʻa me ka ʻano ala o ka maʻi ʻulaʻī, aia i ka hihia mua, he male kekahi, ka mea pili pono i ka hana ʻana o ka hoʻōna o ka hao.

ʻO kahi aʻe, hiki i ke kino ke ʻano o kahi nui o ka hao i ka meaʻai, ke alakaʻi nei i ka pae ʻana o ka ʻaina.

ʻO ka hā ʻehā o ka hemochromatosis hoʻoilina i pili pū me kahi hana hewa o ka gen SLC40A1.

Hōʻike ka maʻi iā ia iho i ka wā ʻelemakule a pili pū me ke kāpili pololei ʻana o ka protein ferroportin, nona ka halihali i ka lawe ʻana i nā mea hao i loko o nā wahi.

Hoʻopilikia ka hewa a me ke kumu pōpilikia

ʻO kahi ʻano genetic i loko o kahi ʻano hoʻoilina o ke ʻano he hopena no ka predisposition o kekahi kanaka.

Hōʻike nā haʻawina ʻo ka hapa nui o nā mea maʻi e noho keʻokeʻo o ʻAmelika ʻAmelika a me ʻEulopa, me ka nui o nā mea maʻi me ka hemochromatosis i ʻike ʻia i waena o nā malihini no Ireland.

ʻO ke ʻano o nā ʻōiwi like ʻole o nā ʻano like ʻole o ka honua. Hiki i nā kāne ke maʻalahi i ka maʻi i nā manawa pinepine i ka manawa o nā wahine. I ka hopena hope, ulu pinepine nā hōʻailona ma hope o nā loli o ka hormonal i ke kino e hopena ana i ka menopause.

Ma waena o nā mea maʻi i hoʻopaʻa inoa ʻia, ʻo nā wāhine he 7-10 mau manawa ma mua o nā kāne. ʻAʻole maopopo paha nā kumu no ka hoʻololi. ʻO ke ʻano hoʻoilina wale nō ka maʻi i hōʻoia ʻia i hōʻike ʻia, a ua ʻike ʻia ka pilina ma waena o ka hemo o ka hemochromatosis a me ka fibrosis ngo.

ʻOiai e hiki ʻole ke wehewehe pololei i ka ulu ʻana o ka hao ma o ka hōʻuluʻulu ʻana o ka hao ma ke kino, a hiki i ka 70% o nā maʻi me ka hemochromatosis ua loaʻa i ka fibrosis ngo.

Eia kekahi, ʻaʻole pono kahi predisposition genetic e hoʻokau i ka hoʻomohala ʻana o ka maʻi.

Eia kekahi, aia kahi kekona o ka hemochromatosis, i nānā ʻia i nā poʻe me nā ʻano genetics maʻamau. Loaʻa kekahi mau wahi ʻē aʻe i kekahi mau kalakal. No laila, ua hoʻololi i ka steatohepatitis (ka wai ʻole o ka waiʻona o ka kiko adipose), ka hoʻomohala ʻana o ka hepatitis kūlohelohe o nā ʻano etiology, a me ka pale ʻana o ka pancreas e kōkua i ka hōʻike o ka maʻi.

Hiki i kekahi neoplasms malignant ke lilo i mākeke no ka hoʻomohala ʻana i ka hemochromatosis.

Nā hōʻailona o ka hemochromatosis i nā wahine a me nā kāne

Hoʻowahāwahā ka maʻi maʻi i kēia lāʻau e like me ke ahi!

Pono ʻoe e koi ...

I ka wā ma mua, ʻo ka hoʻolālā ʻana o ka nui o nā hōʻike hōʻailona koʻikoʻi i hiki i ka maʻi ke nānā i kēia maʻi.

ʻO ka mea maʻi me ka hōʻemi nui o ka hao e manaʻo i ka hoʻoluhi palupalu, nāwaliwali.

ʻO kēia hōʻailona ka hiʻohiʻona o ka 75% o nā mea maʻi me ka hematochromatosis. Hoʻonui ʻia ka pigmentation kino, ʻaʻole i pili kēia kaʻina o ka melanin. E lilo nā maka ma muli o ka hōʻiliʻili ʻana o nā mea hao ma laila. Hoʻomaopopo ka pouli i nā mea he 70% o nā maʻi.

ʻO ka hopena maikaʻi loa o ka hao i hōʻiliʻili ʻia i nā pōpoki paleʻai i alakaʻi i ka nāwaliwali ʻana o ka pale ʻana. No laila, me ka papa o ka maʻi, ka hiki i ka hoʻokaʻawale ʻana o ka mea maʻi i ka maʻi - mai ke koʻikoʻi a hiki ʻole i ka pāpā a hōʻino ʻole i nā kūlana maʻamau.

Ma kahi o ka hapalua o nā mea maʻi e hōʻeha ʻia i nā ala pūlama e hōʻike ʻia ana ma ka hopena o ka ʻeha.

Aia kekahi ʻōpala hoʻi i kā lākou hana. Hoʻomaʻamaʻa kēia hōʻailona no ka mea ʻo ka nui o nā mea hao hao e hōʻiliʻili ai i nā waiho ʻana o nā calcium ma nā ʻāpana.

ʻO Arrhythmias a me ke kūkulu ʻana o ka hōʻeha o ka naʻau, hiki pū kekahi. ʻO ka hopena maikaʻiʻole i ka pancreas e alakaʻi pinepine i ka maʻi diabetes. ʻO ka hao hao ke kumu i ka hana ʻana i ka lāpū palu. I nā pilikia ākea, ʻike ʻia ka ʻeha.

ʻO ka hoʻomohala ʻana o ka maʻi mai ka hala ʻana i nā kāne. ʻO ka emi ʻana o ka hana hana i hōʻike ʻia i nā hōʻailona o ka hoʻopau ʻana i ke kino me nā huahana pūlima hao. I nā wahine, hiki ke hoʻemi ʻia ka pohō nui i ka wā o ka hoʻoponopono.

ʻO kahi hōʻailona koʻikoʻi ka hoʻonui ʻia o ka puʻuwai, a me ka ʻeha hoʻi o ka ʻōpū, i ke ʻano o ka hiki ʻole ke hoʻomaopopo i ka systemic..

ʻO ka loaʻa ʻana o kekahi mau hōʻailona hōʻike i ka pono no ke ake ʻana o ka lāʻau makana ʻo ka maʻi.

ʻO kahi hōʻailona o ka maʻi he maʻi hemoglobin kiʻekiʻe i ke koko, me ka ʻike haʻahaʻa o ia mea i loko o nā ʻāpana koko ʻula. ʻO nā palena transferrin i lalo o 50% e manaʻo ʻia he hōʻailona o ke kikowaena o ka hemochromatosis.

ʻO ka heleʻana o nā heterozygotes paʻakikī a i ka mutations homozygous o kekahi ʻano i ka ʻano o ka HFE me nā hōʻike ʻoi ʻana o ka hōʻiliʻili nui o ka hao e hōʻike i ka hoʻomohala ʻana o ka hemochromatosis.

ʻO ke hoʻonui nui loa i ke ate me ke koikoi kiʻekiʻe o kāna mau ʻiʻo kahi hōʻailona o ka maʻi. Eia kekahi, me ka hemochromatosis, kahi discoloration o ka nui o ka peʻa e nānā ʻia.

Pehea e hōʻike ai i kahi keiki?

ʻO ka hemochromatosis ka mua he nui o nā hiʻohiʻona - mai nā mutations i kumu i kēlā me nā wahi chromosome kūpono i nā kiʻi kauka a me nā hōʻike.

ʻO ka mea mua, ʻo nā hōʻailona o ka maʻi i ka wā ʻōpiopio he polymorphic.

Hōʻike ʻia nā keiki e ka hoʻokumu ʻana o nā hōʻailona e hōʻike ana i ka hypertension portal. ^ E Ha yM. Hoʻoulu i ka hoʻonāukiuki ʻana i ka ʻai ʻana o ka meaʻai, kahi hoʻoulu hou i ka pōpoki a me nā ate.

Me ka hoʻomohala ʻana o nā pathology, kaumaha a kū ʻole i nā hopena curative ascites hoʻomaka - dropsy i hoʻokumu i ka ʻōpū o ka ʻōpū. ʻO ka hoʻomohalaʻana o nā veins varicose o ka esophagus he mea maʻamau.

Maʻa ke ʻano o ka maʻi, a me ka ʻike pono ʻana o ka hoʻomaʻamaʻa ʻana i nā wā a pau ʻole. I ka aneane āpau, hoʻopilikia ka maʻi i kahi ʻehaʻeha o ka palaualelo o ka ʻaihue.

He aha nā hōʻike a me nā hana hoʻokolohua kōkua e maopopo ai i ka pathology?

No ka hoʻomaopopo ʻana i ka maʻi, ua hoʻohana ʻia nā ʻano diagnostic like ʻole.

I ka mua, ua hana ʻia ka ʻolile koko e aʻo i ke kiʻekiʻe o ka hemoglobin i loko o nā kōnela koko a me ka plasma.

Hoʻomaka ʻia kahi loiloi o ka hao ʻo ka hao.

Kōkua ka desferal hōʻoia e hōʻoia i ka maʻi maʻi. No ka hana ʻana i kēia, ua mālama ʻia kahi maʻi o ka hopena glandular, a ma hope o ʻelima mau hola e lawe ʻia kahi tauira urine. Eia kekahi, hoʻokō ʻia ka CT a me ka MRI o nā kino i loko e hoʻoholo i ko lākou mau hoʻololi pathological - he hoʻonui o ka nui, ka pigmentation, a me ka hoʻololi ʻana i ke ʻano o nā kiko.

ʻO ka nānā ʻana i ka genetic scanning e hiki ai iā ʻoe ke hoʻoholo i ka hele ʻana o kahi hapa i hoʻopau ʻia o ka chromosome. ʻO kēia noiʻi, i hana ʻia i waena o nā ʻohana ʻohana o ka mea maʻi, hiki iā mākou ke loiloi i ka hopena o ka hiki ʻana o ka maʻi mai mua aʻe o ka hoʻomaka ʻana o kāna mau lapaʻau e hōʻole ʻia ai ka mea maʻi.

Nā kuʻina mālama

ʻO nā hana nui o ka mālama ʻana ka mea maʻamau o ka heluhelu ʻana o ka ʻike hao ma loko o ke kino a me ka pale ʻana i nā pōʻino i nā kino o loko. Eia naʻe, ʻaʻole maopopo ka lāʻau lapaʻau hou i ke ʻano o ka normalization o nā apana gen.

ʻO kahi ala maʻamau e mālama ai ke koko. Me ka hana hoʻomaka, 500 mg o ke koko e hoʻopau i ka hebedoma. Ma hope o ka hoʻoponopono ʻana i ka hōʻailona o ka hao, ua huli lākou i ka mālama mālama ʻia, ke kūlohelohe ʻia ka pili koko i kēlā me kēia ʻekolu mahina.

Hoʻohana ʻia ka ʻohi ʻana i nā lāʻau hao hao. No laila, ka poʻe chelators e ʻae iā ʻoe e wehe i nā mea momona loa me ka urine a i ʻole nā ​​mea hao. Akā naʻe, ʻo kahi manawa pōkole o ka hana e hana i ka sākena subutaneus maʻamau me ke kōkua ʻana o nā pulu pono e pono ai.

Hoʻopili ʻia ka mālama ʻana ʻo Laboratory i kēlā me kēia mahina ʻekolu mahina. Pēlā e helu ai i nā ʻike hao, me ka hoʻokaʻawale ʻana i nā hōʻailona o ka anemia a me nā hopena ʻē aʻe o ka maʻi.

Hiki i nā hoʻopiʻi a me ka prognosis

Me ka maʻi maʻamau, hiki ke hoʻokele maikaʻi ʻia ka maʻi.

ʻO ka lōʻihi a me ka maikaʻi o ke ola o nā maʻi e loaʻa ma ka mālama maʻamau e kūleʻa ʻole mai nā mea ola olakino wale nō.

Eia kekahi, alakaʻi ka luhi kūpono ʻole i nā pilikia hōʻeha. Hoʻopili kēia i ka hoʻomohala ʻana o ka cirrhosis a me ka hāʻule ʻole o ka ate, ka maʻi maʻi, ka hoʻopōʻino i nā veins a hiki i ke kahe koko.

Aia ka pilikia nui o ka hoʻomohala ʻana i ka cardiomyopathy a me ka maʻi kanesa, ʻike ʻia hoʻi nā maʻi intercurrent.

Hemochromatosis

Wehewehe He maʻi ʻo Hemochromatosis me ka hōʻiliʻili nui ʻana o ka hao i loko o nā papa o nā meaʻokoʻa like me ka pōʻino a me ka hana ʻole o kēia mau mea.

ICD10: E83.1 - Hoʻopilikia iā metabolism.

ʻOiʻala. Aia ka hemochromatosis idiopathic mua a loaʻa i ka lua hemochromatosis hōʻailona.

ʻO ka hemochromatosis mua he mea maʻi maʻi neʻeomomal a me nā maʻi i loaʻa i kahi hema i loko o ka hopena e kau ana ma ka lima pōkole o ka chromosome VI.

Hiki i ka hemochromatosis lua ke loaʻa i ka wā e hoʻoluhi nui ʻia ka hao i loko o ke kino, ke kaohi ʻana i nā iwi i ka hāhiʻi pinepine ʻana o ke koko koko. Hōʻike pinepine ʻia i ka hoʻoili ʻana o ka hao e ka system hematopoietic i nā mea maʻi me ka anemia siderochrestic, thalassemia. Hiki ke kumu paha i ka hōʻino ʻana i ka lāʻau hao mea hoʻopili, vitamona C. Hoʻonui ka mea inu wai i ka hōʻiliʻili o ka hao ma ke kino. ʻO ka hapalua o nā mea maʻi me ka hemochromatosis hōʻailona.

Hōʻailona. I nā poʻe olakino, hoʻopili ʻia ka nui o ka hao i ke koko i kahi ʻano insoluble i loko o ke ʻano o ka hemosiderin. I loko o ka iwi nā hua ʻōloi nā makcrophage i loaʻa nā granule hemosiderin (sideroblasts). Me ka nele o ka hoʻopili ʻana a i ʻole ka waiwai nui o ka hao i loko o ke kino, ʻoi ka nui o nā sideroblasts i ka ʻōpū o ka iwi. Ua kapa ʻia kēia kūlana ʻo ka hemosiderosis. Hoʻihoʻi ʻia ia, ʻaʻole lauʻa ʻia e nā pōʻino o nā kino o loko.

Me ka nui o ka hoʻokomo i loko o ke kino, hoʻomaka ka hao e waiho i nā wahi maʻamau - i ka ate, ka puʻuwai, ka pancreas, ka pā ʻana o ka ʻōpū, a me nā mea i kapa ʻia kēia hemochromatosis. Ua ukali ʻia me nā hana hewa koʻikoʻi o ka hoʻonohonoho a me nā hana o nā ʻāpana ke waiho i ka hao. ʻO ka hōʻiliʻili pathological o ka hao ke kumu etiological o nā maʻi koʻikoʻi e like me ka cirrhosis, ka maʻi diabetes mellitus, ka cardiomyopathy palena.

ʻO ka pilina nui i ka pathogenesis o ka hemochromatosis congenital idiopathic he kuleana kūlohelohe i nā ʻōnaehana o ka enzyme e hoʻoponopono i ka hoʻopio ʻana o ka hao ma loko o ka ʻōpū. I kēlā mau mea maʻi, me ka hana maʻamau o ka hao me ka meaʻai, e hoʻonui ʻia ana kāna kīpū mai ka ʻōpū mai - a hiki i 10 mg i kēlā me kēia lā ma kahi o 1,5 mg maʻamau. ʻAʻole hoʻololi ʻia ka hoʻohana ʻana a me ka wehe ʻana i ka hao mai ke kino. ʻO ka huina o ka piha hao ma ke kino o nā mea maʻi me ka IG e loaʻa ai i ka 20-60 g, aʻo nā poʻe olakino ʻaʻole eʻoi aku i ka 3-4 g.

ʻO ke kiʻi haukapila. E maʻi pinepine ʻia nā kāne. ʻO ka hoʻomaka ʻana o ka maʻi ka wī. Hōʻike nā hōʻailona hiʻohiʻona 1-3 mau makahiki ma hope o ke ʻano o nā loli morphological i loko o nā kino o loko. I ka hoʻomaka mua o ka maʻi, ʻike ʻia ka nāwaliwali nui, nā luhi, ke kaumaha nui, ke maloʻo a me ka hoʻololi ʻana o ka atrophic i ke kino, ka lauoho o ka lauoho, a me ka hoʻonaninani ʻana i nā kāne a me nā wahine.

ʻO ka hōʻike kinoʻana o ka maʻi i ka hapa nui i loaʻa i nā makahiki he 40-60 mau makahiki. Ke hōʻike ʻia nei e ka triad papa:

ʻO ka pigmentation o ka ʻili a me nā membrane membous.

Loaʻa ka Pigmentation i ka hapanui o nā maʻi. Ma muli o kāna hōʻeha i ka lōʻihi o ka maʻi. He paona keleawe ka ʻili. Ua ʻōlelo hou ʻia ka Pigmentation i nā ʻāpana ākea o ke kino, i nā pale lima, ma ka māhele kāne.

Hoʻomaopopo ka hoʻonuiʻana i ka ate i ka aneane o nā mea maʻi me ka hemochromatosis. Puhi ka ate, pīhoihoi, me kahi ʻaoʻao i kuhikuhi mua i kekahi manawa, ʻeha ka hōʻeha ma ka palpation. Loaʻa pinepine ʻia nā Splenomegaly a me "nā hōʻailona kahikaha". I ka hana o ke kikowaena, e ʻike i nā hōʻailona o ka cirrhosis decompensated o ka nui - ka hepatic cell failed, portal hypertension, ascites, hypoproteinemic edema.

ʻO ka lawe ʻana i ka hao i ka pancreas i loko o nā kōpana o nā mokupuni o Langerhans e alakaʻi i ka mellitus e pili ana i ka insulin, i hōʻike ʻia e polyuria, makewai. ʻO ka hoʻopiʻi o ka maʻi diabetes ma ke ʻano o ka acidosis, coma nā mea ʻolua.

ʻO ka hapa nui o nā poʻe maʻi me ka hōʻehaʻeha o ka naʻau - ka palena hoʻopili āpau o cardiomyopathy ʻO ka mea kuhi, ua hōʻike ʻia e ka cardiomegaly, kahi ʻano meʻe a me ka haunaele e hoʻomau ʻia, a me ka piʻi pinepine ʻana i ka puʻuwai naʻau. ʻO ke kumu o ka make o kēlā me kēia puʻukū me ka hemochromatosis me ka hoʻokaʻawale o ka puʻuwai ʻole.

Hiki i ka maʻi Malabsorption ke ulu - ulu ka maʻi me ka hoʻoliʻi ʻole o nā protein, nā kahe, ka momona i loko o ka ʻawaʻawa. Aia kēia i pili i ka hana pohihihi o ka paia o ka ʻili a me ka pancreas e pili ana i ka lawe ʻana o ka hemosiderin i loko o nā puna o kēia mau kino.

Hoʻopau pinepine ʻo arthropathy me ka nalowale o nā pūlima liʻiliʻi o nā lima, nā hips, nā kuli. I ka hapa hapa o kēia mau hihia, he pseudogout kēia - pale i ka membov synovial o ka calcium pyrophosphate.

ʻO 1/3 o nā mea maʻi he hōʻailona i ka hemahema o ka endocrine polyglandular: pituitary, pituitary, adrenal, thyroid gland, testicular atrophy i nā kāne, amenorrhea i nā wahine.

Naʻi hoʻolika Ka helu koko piha: hiki paha. Ma nā hihia kaʻawale, ʻike ʻia nā hōʻailona o ka anemia, hoʻonui ʻia ka nui o ka ESR.

ʻO ka nānāʻana biochemical i ke koko: ka nui o ka ʻike o ka hao serum, ferritin, saturation transferrin me ka hao, hyperglycemia, he hoʻonui i ka hana o ALT, AST. Hiki i ke hypoproteinemia (me ka decompensation o cirrhosis).

Urinalysis: glucosuria, proteinuria hōʻona, hoʻonui ʻia ka hao excretion.

ʻO ka hoʻāʻo ʻana ma ka hopena: ma hope o ka hoʻokele intramuscular o 0.5-1 g o ka desferal me ka urine, ʻoi aku ka nui o 2 mg o ka hao i hoʻokuʻuia i kēlā me kēia lā.

ʻO kahi mālama mau iho: i ka iwi ʻōiwi ka nui o nā sideroblasts - macrophage i loaʻa nā kuhi hemosiderin.

Hoʻopili nā biopsy: ʻili ʻia ka epidermis, i loko o ka epithelium ka nui o ka melatonin pigment, hemosiderin.

Biopsy nono: hōʻailona hōʻailona morphological o ka hepatitis, cirrhosis micronodular. I ka wā mua, me ka hoʻohana ʻana i ka hopena Perls, ʻike ʻia nā huna hao i ke ʻano o ka ferritin a me ka hemosiderin i nā ʻāpana periportal, i nā pū Kupffer, i nā hanana hope loa - i nā cell epithelial o nā dile bile, i nā septa fibrous.

ECG: neʻe ʻana i nā loli i ka myocardium he hapa o ka ventricle hema, a ʻano ʻoe i nā ʻano hana hoʻokūkū a me ka haunaele.

Echocardiography: dilatation of cavities of all room of the heart, paglabag of diastolic function (restrictive cardiomyopathy), hōʻemi o ka ejection fraction, stroke a me ka hopena o ka cardiac.

ʻO ka nānāʻana i ka ultrasound: nā hōʻailona o ka cirrhosis, portal hypertension, ke hoʻololi nei i nā loli i ke ʻano o ka pancreas, nā ʻōpū.

ʻOkoʻa ka maʻi ʻokoʻa. ʻO ka mea mua, pono ia e hoʻokaʻawale i ka hemochromatosis mai hemosiderosis. Ma ka hoʻomaʻamaʻa hīnikalana o nā punctates hepatic, kahi ʻano ʻokoʻa ma waena o ka hemosiderosis kūleʻa a me ka hemochromatosis ʻo ia ka mālama o ka hana lobular maʻamau o ka parenchyma. Me ka hemochromatosis, kahi kiʻi histological o ka maʻi hepatitis a me / a cirrhosis e kū nei.

ʻO ka cirrhosis alikaʻi o ke akea hiki ke loaʻa nā hōʻailona e like me ka idmoathic hemochromatosis: ka hemahema, ka hoʻomaʻemaʻe o ka ʻili, ka lilo o ka lauoho, ka maʻi diabetes, cardiopathy. ʻO ka hapa nui o ka poʻe me ka waiʻona ka mea hao e hōʻike nei i ka hao o ka hao i loko o ke akea (hemosiderosis kekona). Eia nō naʻe, ua ʻike ʻia nā kino ʻo Mallory, kahi reticulum endoplasmic maʻa ka palē, i ʻike ʻia i loko o nā pakanā biopsy ate me ka cirrhosis alkohol, i kū ʻole ʻia me ka hemochromatosis. Eia nō naʻe, i ka nui o nā mea inu wai, poino ka maʻi ma muli o ka hoʻokumu ʻana o ka hemochromatosis kūmole.

Kūʻai koko nui.

ʻO ka nānāʻana biochemical i ke koko: hao, ferritin, hao saturation o transferrin, gula, bilirubin, huina piha protein, AST, ALT.

Urinalysis me ka manaʻo o ka hao hao excretion.

Hoʻololi i ka hoʻāʻo ʻana no ka hoʻonui ʻia ʻana o ka hao i ka urine.