Hemochromatosis (matenda a chiwindi, matenda amkuwa)

- matenda obadwa nawo omwe amadziwika ndi kuwonjezeka kwachitsulo m'matumbo ndikuyika ma pigment okhala ndi chitsulo mu ziwalo ndi minofu (makamaka mawonekedwe a hemosiderin) ndi kukula kwa fibrosis.

(Yu.N. Tokarev, D.A. Settarova, 1988, ndi zowonjezera).

1. Herederal (idiopathic, pulayimale) hemochromatosis.

2. Second hemochromatosis, mitundu:

2.1. Kuthiridwa magazi pambuyo pake (kuchepa kwa magazi m'thupi, mankhwalawa komwe kuthiridwa magazi kumagwiritsidwa ntchito kwanthawi yayitali).

2.2. Alimentary (hemochromatosis wa fuko la Africa Bantu chifukwa chakumwa kwambiri iron ndi chakudya ndi madzi, kuledzera kwa chiwindi, mwina, matenda a Kashin-Beck, etc.).

2.3. Metabolic (opuwala chitsulo kagayidwe kachakudya B-thalassemia, odwala cirrhosis chiwindi ndi chitukuko kapena kugwiritsa ntchito anastomosis portocaval, kufalikira kwa pancreatic duct, cutaneous porphyria, etc.).

2.4. Mwa magwero osakanikirana (thalassemia yayikulu, mitundu ina ya kukomoka magazi - zotengera zachitsulo, siderohrestical, sideroblastic).

Pakadali pano, mbali ya chibadwa cha zinthu pakukula kwa idiopathic hemochromatosis yatsimikiziridwa. Kukula kwa jini la chibadwa cha hemochromatosis (kumalowetsedwa pakatikati kakang'ono ka chromosome VI ndipo ndikugwirizana kwambiri ndi dera la antigen of the HLA histocompatibility system) ndi 0.03-0.07% ndi pafupipafupi heterozygosity ku Europe pafupifupi 10%. Matendawa amatenga matenda obwera chifukwa cha 3-5 paziwonetsero za 1000 zamtundu wa chibadwa cha hemochromatosis ndipo zimafalikira ndi mtundu wofundira wa patosomal. Ubwenzi unakhazikitsidwa pakati pa cholowa cha hemochromatosis - cholakwika chobadwa nacho chopita kutsogolo kwazitsulo zamkati, komanso ma antigenoculat antigen a H1A dongosolo - AZ, B7, B14, Ac

Mu idiopathic hemochromatosis, chilema chachikulu cha magwiridwe antchito ndicho kukokedwa kwachitsulo ndi maselo am'mimba am'mimba thirakiti, zomwe zimayambitsa kuyamwa kwachitsulo, ndikutsatiridwa kwachulukidwe kokhala ndi pigos hemosiderin m'chiwindi, kapamba, mtima, kuyesa ndi ziwalo zina. Izi zimabweretsa kumwalira kwa zinthu zomwe zimagwira ntchito ndikukula kwa njira ya sclerotic. Zizindikiro zamatenda a cirrhosis a chiwindi, matenda a shuga, matenda a mtima metabolism amachitika.

Thupi la munthu wathanzi limakhala ndi mchere wa 3-4 g, womwe umakhala ndi hemochromatosis - 20-60 g. Izi ndichifukwa choti pafupifupi 10 mg wachitsulo amalowetsedwa tsiku lililonse mu hemochromatosis, pomwe munthu wamkulu amakhala wathanzi pafupifupi 1.5 mg (pazipita 2 mg ) Chifukwa chake, mchaka pafupifupi 3 g zachitsulo zochuluka zimadziunjikira m'thupi la wodwalayo ndi hemochromatosis. Ichi ndichifukwa chake zizindikiro zazikulu zamatenda a hemochromatosis zimawonekera pafupifupi zaka 7-10 pambuyo poti matendawa atayamba (L. N. Valenkevich, 1986).

Sekondale hemochromatosis nthawi zambiri imayamba ndi chiwindi cha chiwindi, mowa, kusowa kwa protein.

Ndi chiwindi cirrhosis, kapangidwe ka transerrin kamachepa, komwe kumangiriza chitsulo m'magazi ndikuupereka kwa mafuta a mafupa (a erythropoiesis), kwa minofu (yogwira minyewa yopumira ya minofu) komanso kwa malo achitsulo. Ndikusowa kwa transerrin, kudzikundikira kwa chitsulo chosagwiritsidwa ntchito kagayidwe kamachitika. Kuphatikiza apo, ndi cirrhosis ya chiwindi, kapangidwe ka ferritin, womwe ndi mawonekedwe amtundu wachitsulo, amasokonekera.

Kuledzeretsa kumabweretsa kuyamwa kwachuma m'matumbo, zomwe zimapangitsa kuti chizindikiritso cha chibadwa cha matenda obadwa nawo chiwindi kapena kuwonongeka kwa chiwindi chikhale ndi mtundu wina wa matendawa.

Kupezeka kwa anastomoses mu portal system kumathandizira kutsika kwachitsulo mu chiwindi.

Ndi anem-Refractory (siderohrestical) anemia ndi thalassemia yayikulu, chitsulo cholowetsedwa sichimagwiritsidwa ntchito, chimakhala chosafunikira ndipo chimayikidwa m'chiwindi, myocardium ndi ziwalo zina.

Kwambiri amuna amakhudzidwa (chiŵerengero cha amuna ndi akazi 20: 1), mtundu wopangika wa matendawa umadziwoneka wokha wazaka 40-60. Kukula kotsika kwa matendawa mwa azimayi kumachitika chifukwa chakuti azimayi amataya chitsulo ndimagazi a kusamba mkati mwa zaka 25- 35 (O.

Zizindikiro zazikulu zakuchipatala:

1.Khungu la khungu (melasma) limawonedwa mu 52-94% ya odwala (S. D. Podymova, 1984). Zimayambitsidwa ndikuwonekera kwa khungu la khungu lopanda zitsulo (melanin, lipofuscin) ndi hemosiderin. Kukula kwa pigmentation kumatengera kutalika kwa matendawa. Khungu limakhala losuta, la ubweya, laimvi, lomwe limadziwika kwambiri m'malo otseguka a thupi (nkhope, manja), m'malo omwe kale anali ndi nkhungu, kumiyendo, kumaliseche.

2. Kuwonjezeka kwa chiwindi kumawonedwa mu 97% ya odwala omwe ali ndi gawo lalikulu la matendawa, chiwindi chimakhala chambiri, nthawi zambiri chimakhala chopweteka. Mtsogolomo, chithunzi cha matenda a chiwindi cirrhosis ndi ascites, portal matenda oopsa, splenomegaly amakula.

3. Matenda a shuga amawonedwa mu 80% ya odwala, amayamba chifukwa cha kutsitsidwa kwachitsulo m'mabwalo a Langerhans, owonetsedwa ndi ludzu, polyuria, hyperglycemia, glycosuria. Sichovuta kwenikweni ndi acidosis ndi chikomokere.

4. Mavuto ena a endocrine - hypogenitalism (kuchepa kwa potency, testicular atrophy, kutha kwa machitidwe achiwiri achigololo, ukazi, mwa akazi - amenorrhea, kusabereka), hypocorticism (kufooka kwambiri, kuchepa kwa magazi, kuchepa thupi).

5. Cardiomyopathy imayendera limodzi ndi kuwonjezeka kwa mtima, kusokonezeka kwa mitsempha, kukula kwapang'onopang'ono kwa mtima kulephera, kosagwirizana ndi chithandizo cha mtima glycosides. 35% ya odwala omwe ali ndi hemochromatosis amafa chifukwa cha kupunduka mtima.

6. metabolic malabsorption syndrome amayamba chifukwa cha kusakhazikika kwamatumbo am'mimba komanso kapamba chifukwa chazitsulo zamagetsi zomwe zimakhala ndi ziwalozi.

Njira ya hemochromatosis yayitali ndi yayitali (zaka 15 kapena kuposerapo), ndi chitukuko cha matenda amchiwindi, chiyembekezo chamoyo sichikhala

amapitilira zaka 10. Ndi yachiwiri hemochromatosis, chiyembekezo cha moyo ndi chochepa.

1. UAC: Zizindikiro za kuchepa magazi (osati mwa odwala onse), ESR yowonjezera.

2. OAM: proteinuria woyenera, urobilinuria, glucosuria ndizotheka; iron and excretion wa mkodzo umakwera mpaka 10-20 mg patsiku (wabwinobwino - mpaka 2 mg / tsiku).

3. LHC: mulingo wa ma seramu azitsulo woposa 37 μmol / L, serum ferritin ndi woposa 200 μmol / L, kuchuluka kwa kuchuluka kwa transferin ndi chitsulo ndi zoposa 50%, kuchuluka kwa ALAT, tg-globulins, mayeso a thymol, kuvutitsidwa kwa glucose kapena hyperglycemia.

4. Madzi achepetsa kuchuluka kwa magazi a 11-ACS, 17-ACS, sodium, chloride, hydrocortisone, kuchepa kwa zotupa za tsiku ndi tsiku za 17-ACS, 17-KS, kuchepa kwa kuchuluka kwa mahomoni ogonana m'magazi ndi mkodzo.

5. Kuboola kunja: kulamula kumakhala ndi chitsulo chachikulu.

6. Mu biopsy toyesa khungu - kuchuluka kwambiri kwa melanin, mu biopsy toyesa chiwindi - mawonekedwe a hemosiderin, lipofuscin, chithunzi cha micronodular cirrhosis. Malinga ndi Sartap (1982), zitsulo zomwe zimakhala m'chiwindi nthawi yayikulu hemochromatosis imachulukanso pafupifupi nthawi 40 poyerekeza ndi nthawi zonse, komanso katatu munthawi ya sekondaleti.

7. Kuyesa kwa Desferal - kutengera kuthekera kwa Desferal kumangira ferritin ndi hemosiderin iron ndikuchotsa m'thupi. Kuyesedwa kumawoneka ngati kwabwino ngati, pambuyo pakubayidwa mu mnofu wa 0,5-1 g wa desferal patsiku, kuposa 2 mg ya chitsulo amuchotsedwa mkodzo.

1. Kujambula kwa Ultrasound ndi radioisotope: kukulitsa chiwindi, kapamba, kuyambitsa kusintha kwa iwo, splenomegaly.

2. FEGDS: Ndi chitukuko cha matenda a chiwindi, mitsempha ya varicose ya esophagus ndi m'mimba imapezeka.

3. Echocardiography: kuchuluka kukula kwa mtima, kuchepa kwa myocardial contractile function.

4. ECG: sinthani kusintha kwa myocardium (kutsitsa kwa mafunde a T, maulendo a 8-T), kukulitsa nthawi yayitali (^ -T, zovuta zamtima

1. OA ya magazi, mkodzo, kusanthula kwa mkodzo kwa glucose, urobilin, bilirubin.

2. LHC: bilirubin, transaminases, mapuloteni athunthu ndi mapuloteni, glucose, potaziyamu, sodium, chloride, seramu iron, serum ferritin, kuchuluka kwa transfer. Ndi glycemia wabwinoko, kuyesa kwa glucose.

3. Kujambula kwa Ultrasound kwa chiwindi, ndulu, kapamba, impso.

6. Kudziwitsa zomwe zili mu mahomoni ogonana m'magazi, hydrocortisone.

7. Kudziwitsa tsiku ndi tsiku kwamikodzo yokumira 17-OKS, 17-KS.

Pigmentary cirrhosis, aka hemochromatosis: Zizindikiro ndi mfundo za mankhwala a matenda amisempha

Hemochromatosis adayamba kutchulidwa ngati matenda osiyana mu 1889. Komabe, zinali zotheka kukhazikitsa ndendende zomwe zimayambitsa matendawa pokhapokha ndikupanga ma genetics azachipatala.

Kugawana mochedwa koteroko kunalimbikitsa mtundu wa matendawa komanso kufalikira kochepa.

Chifukwa chake, malinga ndi deta yamakono, 0,33% ya anthu padziko lapansi ali pachiwopsezo chotenga hemochromatosis. Chimayambitsa matendawa ndi chiyani ndi zomwe?

Hemochromatosis - ndi chiyani?

Matendawa ndi chobadwa nawo ndipo amadziwika ndi kuchulukitsa kwa Zizindikiro komanso chiwopsezo chachikulu cha zovuta zovuta komanso matendawa.

Kafukufuku wasonyeza kuti hemochromatosis nthawi zambiri imayamba chifukwa cha masinthidwe amtundu wa HFE.

Chifukwa cha kulephera kwa jini, makina azitsulo zomwe zimatulukira mu duodenum amasokonekera. Izi zimadzetsa kuti thupi limalandira uthenga wabodza wokhudza kusowa kwachitsulo mthupi ndipo limayamba kugwira ntchito mwachangu ndipo zochulukirapo zimapanga protein yapadera yomwe imamanga chitsulo.

Izi zimadzetsa kuchuluka kwa hemosiderin (glandular pigment) mkati. Pamodzi ndi kuwonjezeka kwa kaphatikizidwe kamapuloteni, kutseguka kwa m'mimba thirakiti kumachitika, zomwe zimayambitsa kuyamwa kwambiri kwachitsulo ku chakudya m'mimba.

Chifukwa chake ngakhale ndimakudya abwinobwino, kuchuluka kwazitsulo zomwe zimapezeka m'thupi kumakhala kambiri kuposa nthawi yayitali. Izi zimabweretsa kuwonongeka kwa zimakhala zamkati, mavuto ndi endocrine system, komanso chitetezo chokwanira.

Gulu la mitundu ndi mitundu, mitundu ndi magawo

Muzochita zamankhwala, mitundu yayikulu komanso yachiwiri yamatenda imagawidwa. Pankhaniyi, choyambirira, chomwe chimatchedwanso kuti cholowa, ndi chifukwa cha chibadwa chamunthu. Sekondale hemochromatosis ndi chotsatira cha kukula kwa zopatika mu ntchito ya enzyme machitidwe omwe amakhudzidwa ndi glandular metabolism.

Mitundu inayi yamatenda amtundu (genetic) yamatenda amadziwika:

• chapamwamba
• wachinyamata
• mitundu yachilengedwe ya HFE
• Autosomal wamkulu.

Mtundu woyamba umalumikizidwa ndi kusintha kwapadera kwa dera la 6 la chromosome. Mtunduwu umapezeka mu milandu yambiri - oposa 95 peresenti ya odwala omwe ali ndi classical hemochromatosis.

Matendawa amatenga matenda obwera chifukwa cha masinthidwe amtundu wina, HAMP. Mothandizidwa ndi kusintha kumeneku, kapangidwe ka hepcidin, puloteni yomwe imayambitsa kuyika kwazitsulo ziwalo, imakulitsidwa kwambiri. Nthawi zambiri, matendawa amawonekera azaka zapakati pa khumi ndi makumi atatu.

Mtundu wa HFE-osalumikizana umayamba mtundu wa HJV utalephera. Izi matenda akuphatikiza limagwirira hyperactivation wa transerrin-2 zolandilira. Zotsatira zake, kupanga kwa hepcidin kumakulitsidwa. Kusiyana ndi mtundu wa matenda ndikuti poyamba, jini imalephera, yomwe imayambitsa mwachindunji kupanga enzyme yomanga chitsulo.

Pomwe mu nkhani yachiwiri, thupi limapanga mawonekedwe a chitsulo chochuluka mu chakudya, zomwe zimatsogolera pakupanga enzyme.

Mtundu wachinayi wa chibadwa cha hemochromatosis umalumikizidwa ndi vuto la chibadwa cha SLC40A1.

Matendawa amawonekera okalamba ndipo amagwirizanitsidwa ndi kaphatikizidwe kosayenera ka mapuloteni a ferroportin, omwe amachititsa kuti zitsulo zizipanga ma cell.

Zovuta zomwe zimayambitsa masinthidwe oyipa ndi zomwe zimayambitsa ngozi

Kusintha kwa chibadwa cha mtundu wamatenda ndi chotsatira cha mkhalidwe wa munthu.

Kafukufuku akuwonetsa kuti odwala ambiri ndi oyera azungu ku North America ndi Europe, ndi kuchuluka kwakukulu kwa odwala omwe ali ndi hemochromatosis omwe amawonekera pakati osamukira ku Ireland.

Komanso, kufalikira kwa mitundu yosiyanasiyana ya masinthidwe amadziwika m'madera osiyanasiyana padziko lapansi. Amuna amatenga matendawa kangapo konse kuposa azimayi. Mapeto ake, zizindikiro zimayamba pambuyo pa kusintha kwa mahomoni m'thupi chifukwa chosiya kusamba.

Mwa odwala olembetsedwa, azimayi ndi ocheperapo kasanu ndi kawiri kuposa amuna. Zomwe zimasinthira sizikudziwikabe bwinobwino. Ndi chikhalidwe chobadwa nacho cha matendawa chomwe chimatsimikiziridwa mosatsata, komanso kulumikizana kwa kukhalapo kwa hemochromatosis ndi chiwindi fibrosis kumayambikiranso.

Ngakhale kukula kwa minofu yolumikizika sikungafotokozedwe mwachindunji ndi kudziunjikira kwachuma m'thupi, mpaka 70% ya odwala omwe ali ndi hemochromatosis anali ndi chiwindi fibrosis.

Kuphatikiza apo, kubadwa kwamtundu sikungachititse kuti matendawo akule.

Kuphatikiza apo, pali mtundu wachiwiri wa hemochromatosis, womwe umawonedwa mwa anthu omwe ali ndi genetics yoyambirira. Zowopsa zimaphatikizanso ndi ma pathologies ena. Chifukwa chake, kusamutsidwa kwa steatohepatitis (osakhala chidakwa cha kuphatikizika kwa minofu ya adipose), kukula kwa chiwindi cha hepatitis kosiyanasiyana, komanso kufooka kwa kapamba kumathandizira kuwonetsa matendawa.

Ma neoplasms ena oyipa amathanso kukhala othandizira pakukula kwa hemochromatosis.

Zizindikiro za hemochromatosis mwa akazi ndi amuna

Matenda a shuga amawopa mankhwalawa, ngati moto!

Muyenera kungolemba ...

M'mbuyomu, kupezeka kwa zizindikiritso zazikulu zingapo kudapangitsa kuti muzindikire za matendawa.

Wodwala yemwe amakhala ndi chitsulo chochuluka amamva kutopa kwambiri, kufooka.

Chizindikiro ichi chimadziwika ndi 75% ya odwala omwe ali ndi hematochromatosis. Khungu lowongolera khungu limalimbikitsidwa, ndipo njirayi imagwirizanitsidwa ndi kupanga kwa melanin. Khungu limakhala lakuda chifukwa chodzikundikira mankhwala azitsulo pamenepo. Mdima wamdima umawonedwa mwaoposa 70% ya odwala.

Zotsatira zoyipa zomwe zimakhala ndi ma cell a chitetezo chathupi zimabweretsa kufooka kwa chitetezo chathupi. Chifukwa chake, matendawa atadwala, zovuta zomwe wodwalayo amatenga kumatenda zimawonjezeka - kuchokera pazovuta zazikulu mpaka zoletsa komanso zopanda vuto lililonse.

Pafupifupi theka la odwala ali ndi vuto lophatikizika lomwe limanenedwa mwadzidzidzi yopweteka.

Palinso kuwonongeka mu kuyenda kwawo. Chizindikirochi chimachitika chifukwa kuchuluka kwachitsulo kumapangitsa kuti calcium ikhale m'malo.

Arrhythmias ndi kukula kwa kulephera kwa mtima ndizothekanso. Zotsatira zoyipa za kapamba nthawi zambiri zimayambitsa matenda ashuga. Chitsulo chowonjezera chimapangitsa kusokonekera kwa thukuta. Nthawi zina, mutu umawonedwa.

Kukula kwa matendawa kumabweretsa kusabala kwa amuna. Kuchepa kwa zogonana kumawonetsa zizindikiro za poizoni wa thupi ndi zinthu zopangidwa ndi chitsulo. Mwa akazi, kutaya magazi kwambiri panthawi ya malamulo kumatheka.

Chizindikiro chofunikira ndi chiwindi chokulitsidwa, komanso kupweteka kwambiri pamimba, pakuwoneka komwe sikungatheke kuzindikira zadongosolo.

Kukhalapo kwa zizindikiro zingapo kukusonyezera kufunikira kozindikira kwawoko kwa matendawa.

Chizindikiro cha matendawa ndi zinthu zambiri za m'magazi, zomwe zimapezeka munthawi yomweyo m'maselo ofiira a m'magazi. Miyezo yotumizira Iron pansipa 50% imawerengedwa ngati chizindikiro cha labotale ya hemochromatosis.

Kupezeka kwa zovuta za heterozygotes kapena kusintha kwa mtundu wina wa HFE pamtundu wautali wa umboni wa kuchuluka kwazitsulo kumawonetsa kukula kwa hemochromatosis.

Kuwonjezeka kwakukulu kwa chiwindi ndi kuchulukana kwambiri kwa minofu yake ndi chizindikiro cha matendawa. Kuphatikiza apo, ndi hemochromatosis, mawonekedwe a chiwindi amawonedwa.

Kodi zimawonekera bwanji mwa mwana?

Hemochromatosis yoyambirira ili ndi zinthu zingapo - kuchokera ku masinthidwe omwe adapangitsa kuti akhale zigawo zofananira za chromosome kupita ku chithunzi ndi mawonekedwe azachipatala.

Choyamba, Zizindikiro za matendawa ali aang'ono ndi polymorphic.

Ana amadziwika ndi kukula kwa zizindikiro zosonyeza portal matenda oopsa. Amayamba kuphwanya chakudya chimbudzi, kuwonjezeka kofanana ndulu ndi chiwindi.

Ndi chitukuko cha matenda, wolemera komanso kugonjetsedwa ndi curative zotsatira ascites akuyamba - dropsy amene amapezeka pamimba dera. Kukula kwa mitsempha ya varicose ya esophagus ndi khalidwe.

Njira yamatendawa imakhala yolimba, ndipo matendawo amayambitsidwa nthawi zonse osavomerezeka. Pafupifupi nthawi zonse, matendawa amakhumudwitsa mtundu wofooka wa chiwindi.

Ndi mayeso ati ndi njira zodziwira matenda zomwe zimathandiza kuzindikira matenda?

Kuti muzindikire matendawa, njira zingapo zosiyanasiyana zowunika zasayansi zimagwiritsidwa ntchito.

Poyamba, kuyezetsa magazi kumachitika kuti aphunzire kuchuluka kwa hemoglobin m'maselo ofiira am'magazi ndi plasma.

Kuunika kwa kagayidwe kazitsulo kumachitidwanso.

Kuyesedwa kopanda chiyembekezo kumathandizira kutsimikiza. Kuti muchite izi, jakisoni wa mankhwala a glandular amathandizidwa, ndipo atatha maola asanu umodulidwe wa mkodzo umatengedwa. Kuphatikiza apo, CT ndi MRI ya ziwalo zamkati zimapangidwa kuti zitsimikizire kusintha kwazomwe zimachitika - kuchuluka, kukula, kusintha kwa pigment, komanso kusintha kapangidwe kazinthu zina.

Kufufuza zamtundu wa cell kumakupatsani mwayi wodziwa kukhalapo kwa gawo lowonongeka la chromosome. Kufufuza kumeneku, komwe kumachitika pakati pa achibale a wodwalayo, kumatithandizanso kuwunika momwe matendawo angayambire ngakhale isanayambike mawonekedwe ake azaumoyo omwe akusokoneza wodwalayo.

Mfundo zachithandizo

Njira zazikulu zochiritsira ndikusintha kwazitsulo zomwe zimapezeka m'thupi ndi kupewa kuwonongeka kwa ziwalo zamkati ndi machitidwe. Tsoka ilo, mankhwala amakono samadziwa momwe angagwirizanitsire zida za majini.

Njira yodziwika bwino yodziwira magazi ndi kukhetsa magazi. Ndi chithandizo choyambirira, 500 mg yamagazi amachotsedwa sabata iliyonse. Pambuyo pogwirizira zizindikiro zamtundu wachitsulo, amasintha kuti azikonzanso mankhwala, pamene sampu ya magazi imachitika kamodzi miyezi itatu.

Mothandizidwa kulowetsedwa kwa mankhwala omangira chitsulo amachitidwanso. Chifukwa chake, chelators amakulolani kuti muchotse zinthu zochulukirapo ndi mkodzo kapena ndowe. Komabe, kwakanthawi kochepa kumapangitsa jakisoni wokhazikika wa mankhwalawa mothandizidwa ndi mapampu apadera ofunikira.

Kuwunikira labotale kumachitika kamodzi miyezi itatu iliyonse. Zimaphatikizanso kuwerengera zomwe zili zachitsulo, komanso kudziwa zizindikiro za kuchepa kwa magazi ndi zina mwa zotsatirazi za matendawa.

Mavuto omwe angakhalepo komanso chidziwitso cha matendawo

Ndi matenda oyamba, matendawa amatha kuthandizidwa.

Kutalika ndi moyo wa odwala omwe amalandizidwa pafupipafupi samasiyana ndi anthu athanzi.

Komanso, chithandizo chadzidzidzi chimabweretsa zovuta zambiri. Izi zikuphatikizapo kukhazikika kwa matenda a cirrhosis ndi kulephera kwa chiwindi, shuga, kuwonongeka m'mitsempha mpaka kukhetsa magazi.

Pali chiwopsezo chachikulu chotenga mtima ndi khansa ya chiwindi, matenda oyanjana nawo amawonedwanso.

Hemochromatosis

Tanthauzo Hemochromatosis ndi matenda omwe amaphatikizidwa ndi kuchuluka kwa ayironi m'maselo a ziwalo zosiyanasiyana zowonongeka ndi kuwonongeka kwa ziwalozi.

ICD10: E83.1 - Kuphwanya kagayidwe kazitsulo.

Etiology. Pali chachikulu idiopathic hemochromatosis ndi sekondale yopezeka ndi hemochromatosis.

Main hemochromatosis ndi matenda obadwa nawo ozungulira omwe amayamba chifukwa cha chilema cha jini lomwe lili kumanja kwa chromosome VI.

Second hemochromatosis imatha kukhazikika pakakhala chitsulo chochuluka mthupi, kudutsa matumbo ndi ma cell ofiira a magazi. Nthawi zambiri kumachitika ngati chitsulo sichinatengeke ndi hematopoietic dongosolo la odwala a siderochrestic anemia, thalassemia. Zomwe zimayambitsa zimatha kukhala kuzunza kwa mankhwala omwe amakhala ndi chitsulo, vitamini C. Mowa umachulukitsa kuchuluka kwa chitsulo mthupi. Theka la odwala omwe ali ndi vuto la hemochromatosis ndi zidakwa.

Pathogenesis. Mwa anthu athanzi, mchere wambiri m'magazi umayikidwa mu mawonekedwe osakwanira mu hemosiderin. M'mphepete mwa mafupa muli ma macrophages okhala ndi ma grosule a hemosiderin (sideroblasts). Ndi kusamwa kapena kuyamwa kwambiri kwazitsulo mthupi, kuchuluka kwa sideroblasts mumongo wamafupa kumawonjezeka kwambiri. Matendawa amatchedwa hemosiderosis. Zimasinthika, sizotsatira ndi kuwonongeka kwa ziwalo zamkati.

Ndi kudya kwambiri mthupi, chitsulo chimayamba kuyikidwa m'malo osazungulira - m'chiwindi, mtima, kapamba, khoma lamatumbo, ndi zina. Matendawa amatchedwa hemochromatosis. Zimayendera limodzi ndi kuphwanya kwakukulu kwa kapangidwe kake ndi ziwalo zomwe zimayika chitsulo. Kuphatikizika kwazitsulo kumapangitsa kuti munthu azitsata matenda oopsa monga cirrhosis, matenda a shuga, kuletsa mtima.

Cholumikizira chachikulu mu pathogenesis ya kobadwa nako idiopathic hemochromatosis ndi chilema chotsimikizika pamtundu wamagetsi omwe amatsogolera kuyamwa kwachitsulo m'matumbo. Mu odwala oterowo, okhala ndi chitsulo mwachizolowezi ndi chakudya, mayamwidwe ake kuchokera m'matumbo amawonjezereka - mpaka 10 mg tsiku lililonse m'malo 1.5 mg wabwinobwino. Kugwiritsa ntchito komanso kuchotsa chitsulo m'thupi sikusinthidwa. Zazitsulo zathunthu zomwe zimapezeka m'thupi la odwala omwe ali ndi IG zimafika 20-60 g, pomwe mwa anthu athanzi siziposa 3-4 g.

Chithunzi cha chipatala. Amuna amadwala pafupipafupi. Kukhazikika kwa matendawa kumachitika pang'onopang'ono. Zizindikiro zamakhalidwe zimawonekera patapita zaka 1-3 kuchokera ku kuwonekera kwa kusintha kwa morphological mu ziwalo zamkati. Kumayambiriro kwa matendawa, kufooka kwambiri, kutopa, kuchepa thupi, kuwuma komanso kusintha kwa kusintha kwa pakhungu, kuchepa tsitsi, komanso kusowa kwa kugonana kwa amuna ndi akazi kumawonedwa.

Kuwonetsedwa kwamankhwala kwamatenda nthawi zambiri kumachitika pazaka 40-60. Zimawonetsedwa ndi mtundu wapamwamba kwambiri watatu:

Kulimbitsa khungu ndi mucous nembanemba.

Matenda a pigment amawonedwa mwa odwala ambiri. Kuopsa kwake kumatengera kutalika kwa matendawa. Khungu limakhala ndi mafuta amkuwa. Kugonana kumatchulidwa kwambiri padera la thupi, kumapazi, kumaliseche.

Kuwonjezeka kwa chiwindi kumawonedwa mu pafupifupi odwala onse omwe ali ndi hemochromatosis. Chiwindi chimakhala chofewa, chosalala, chokhala ndi m'mphepete kutsogolo, nthawi zina chimapweteka palpation. Splenomegaly ndi "zizindikiro za chiwindi" ndizosowa. Mu siteji yotsika, zizindikiro za kuwonongeka kwa matenda a chiwindi amayamba - chiwindi kulephera, chiwopsezo cha matenda oopsa, ascites, hypoproteinemic edema.

Kukhazikika kwachitsulo mu kapamba mu maselo a zilumba zazing'ono za Langerhans kumapangitsa kuti pakhale shuga wambiri wodwala matenda a shuga, owonetsedwa ndi polyuria, ludzu. Mavuto a shuga a shuga mwa mawonekedwe a acidosis, chikomokere ndizosowa.

Odwala ambiri ali ndi kuwonongeka kwa mtima - yachiwiri yokhala ndi mtima wamtima. Moyenerera, imawonetsedwa ndi cardiomegaly, mawonekedwe osiyanasiyana amiseche ndi kusokonezeka kwapang'onopang'ono, ndikupanga pang'onopang'ono kulephera kwa mtima. Zomwe zimapangitsa kuti odwala atatu aliwonse omwe ali ndi hemochromatosis aperekedwe ka mtima kulephera.

Malabsorption syndrome imatha kukhala - kutsegula m'mimba ndi mapangidwe osokoneza bongo a mapuloteni, chakudya, mafuta m'matumbo. Izi zimakhazikitsidwa chifukwa cha kuwonongeka kwa khoma lamatumbo ndi kapamba komwe kumayenderana ndi kufalikira kwa hemosiderin m'maselo a ziwalozi.

Nthawi zambiri arthropathy imachitika ndikugonjetsedwa kwamanja kwamanja, m'chiuno, mawondo. Mu theka la milandu, iyi ndi pseudogout - mawonekedwe a kuphatikizika kwa calcium pyrophosphate.

1/3 ya odwala ali ndi zizindikiro za kuperewera kwa polyglandular endocrine: pituitary, pituitary, adrenal, chithokomiro, testicular atrophy mwa amuna, amenorrhea mwa akazi.

Zizindikiro Kuwerengera kwamwazi konse: kumatha kukhala kwabwinobwino. Nthawi zina, zizindikiro za kuchepa kwa magazi, ESR yowonjezera imapezeka.

Kusanthula kwa biochemical kwa magazi: kuwonjezeka kwa zomwe zimakhala ndi seramu chitsulo, ferritin, machulukitsidwe amtundu wa iron, hyperglycemia, kuchuluka kwa ntchito ya ALT, AST. Hypoproteinemia ikhoza kuchitika (ndi kuwonongeka kwa cirrhosis).

Urinalysis: glucosuria, proteinuria wolimbitsa, kuchuluka kwa chitsulo.

Mayeso osafunikira: pambuyo poyenda mwamtsempha wa 0,5-1 g wa mkodzo, oposa 2 mg a chitsulo amamasulidwa tsiku lililonse.

Kuboola kwakanthawi: m'mafupa ambiri mwa ma sideroblasts - macrophages okhala ndi hemosiderin inclusions.

Khungu biopsy: khungu limachepera, mu epithelium kuchuluka kwa pigment melatonin, hemosiderin.

Chiwindi biopsy: maumbidwe a hepatitis, micronodular cirrhosis. M'magawo oyambira, pogwiritsa ntchito zomwe a Perls amachita, madipoziti azitsulo amawonekera mu mawonekedwe a ferritin ndi hemosiderin m'malo omwe amatha, mu maselo a Kupffer, m'magawo apambuyo - mu maselo a epithelial a bile ducts, mu fibrous septa.

ECG: imasinthasintha kusintha kwa myocardium makamaka pamphepete yamanzere, mitundu ingapo ndi kusokonezeka kwa conduction.

Echocardiography: kuchepa kwamitsempha yamipanda yonse ya mtima, kuphwanya kwa ntchito ya diastolic (kupumira kwa mtima), kuchepa kwa gawo la episodeion, sitiroko ndi mtima wake.

Kuunika kwa Ultrasound: Zizindikiro za matenda amitsempha yamagazi, matenda oopsa a portal, kupangitsa kusintha kapangidwe ka kapamba, impso.

Kusiyanitsa mitundu. Choyamba, ndikofunikira kusiyanitsa hemochromatosis ku hemosiderosis. Pakufufuza kwadongosolo la hepatic punctates, kusiyana pakati pa sekondemositisosis ndi hemochromatosis ndikusungidwa kwazomwe zimapangidwira mawonekedwe a parenchyma. Ndi hemochromatosis, chithunzi cha mbiri yakale cha hepatitis ndi / kapena cirrhosis chikuchitika.

Mankhwala osokoneza bongo a chiwindi amatha kukhala ndi zizindikiro zofanana ndi idiopathic hemochromatosis: kusabala, khungu pakhungu, kuchepa tsitsi, matenda ashuga, mtima. Anthu ambiri omwe ali ndi zakumwa zoledzeretsa amawonetsa kuwonekera kwa chiwindi (yachiwiri hemosiderosis). Komabe, matupi a Mallory, opindika bwino a endoplasmic reticulum, amapezeka m'chiwonetsero cha chiwindi biopsy ndi chidakwa cha cirrhosis, chomwe sichichitika ndi hemochromatosis. Komabe, mwa zidakwa zambiri, kuwonongeka kwa chiwindi kungayambike chifukwa cha mapangidwe a sekondale hemochromatosis.

Kuyesedwa kwa magazi konse.

Kusanthula kwa biochemical kwa magazi: chitsulo, ferritin, kuchuluka kwa chitsulo chosamutsa, shuga, bilirubin, mapuloteni onse, AST, ALT.

Urinalysis ndi kutsimikiza kwa chitsulo excretion.

Mayeso osowa kwa mkodzo.