Acute intermittent porphyria: zviratidzo, kuongororwa, kurapwa

Genetic pathologies ndiyo yakaoma uye yakaoma, sezvo zvisingakwanisi kubvisa zvachose. Chimwe chezvirwere izvi inonyanya kubuda pakvirariya. Ichi chirwere chekuzvarwa, umo muwandu we porphyrines inowedzera muropa. Urwu hutano hwehutano hunofananidzwa seimwe yeiyo inowanzoonekwa pakati pemarudzi ese eine genetic porphyria.

Panguva yechirwere ichi, kune kuwedzera kwezvinhu zvinotyisa muzvikamu zvevanhu. Kazhinji kacho, hutachiona hunowanikwa mune vakadzi. Uye zvinokuvadza zvinogona kuva nepamuviri uye kubereka.

Symptomatology yechirwere

Kana murwere achikura zvakanyanya mukati memhepo, zviratidzo zvinotevera:

• Unhu hweimwe nzvimbo dzeganda.
• Polyneuritis (kukuvadza kwakasiyana-siyana kwemagariro ekuguma).
• Rash.
• Tsvuku uye kuzvimba kweganda.
• Pigmentation uye blister pane dzimwe nzvimbo dzeganda dzinowanzozarurwa.
• Microcyst (kuderera mumubhadharo wechipfuva, umo mune kupesana kusingaperi kwemabasa ayo).
• Psychosis.
• Organic pathological inoshandiswa mukati mechiropa inobata basa rayo.
• Anemia.
• Coma.
• Pain mumimba, uye yakasimba kwazvo. Izvo zvinowirirana neyeusa yakawedzerwa uye kurutsira.
• Kuparadzwa kweail plates.
• Kuwedzera mukumanikidza.
• Kupopotedzana kwekuzora.
• Kuvhiringidzika, kutya, kukanganisa.
• Kuparara kwemapfupa ekufema.

Zvinofanira kuonekwa kuti zviratidzo zvekutanga zvinowanikwa mumurwere ane makore makumi maviri nemakumi mana nemafungiro ekudzivirira. Uye zvinogona kudzokororwa kazhinji kana kuitika kamwe chete munguva yehupenyu. Kana kukanganiswa kwechirwere ichi kuchikura, ipapo pangave nokuputsa kubva muhutano hwehuropi, mitsipa yedehenya (oculomotor uye chiso), iyo yakazvimirira NA.

Zviratidzo zve porphyria hazvisi pachena kune vose vanotakura. Somuenzaniso, 80% yevamwe vangave vasingazivi nezvechinetso. Ihwo hunoshamisa hwekurwiswa kwechirwere ndechokuti, mumhando dzose dzayo, murwere wemurwere unochinja ruvara uye unova wepenki, webhuru kana kunyange wakatsvuka.

Acute porphyria mafomu haawanzoitiki. Chiratidzo chechirwere chinoratidzwa nenzira dzakasiyana.

Kuongororwa kwehutachiona

Muchiitiko chechirwere chakadai sechirwere chekati chinonzi porphyria, chirwere chinofanira kuve chakakwana. Chaizvoizvo, laboratory test of urine neropa zvinoshandiswa, izvo zvinofanira kuzarura chiyero che porphobilinogens, iyo ye porphyrin mu plasma. Kana imwe samuenzaniso inorwara, murwere anogona kugoverwa zvakare kuongorora.

Somuenzaniso, zvakakosha kuita radiograph yedumbu remuviri, iyo inobatsira kuziva kubatwa kwepamuviri. Mukuwedzera, pane zvimwe zviratidzo zvinoshandiswa zvinobatsira kuita kuti kuongororwa kwakarurama sezvinobvira:

1. Panguva yekubatwa: PBG neAA zvakakurumidza kubudiswa mumugini.
2. Panguva yekuregererwa, kuongororwa kwehuwandu hwekuderera kwebasa rinogona kuitwa.

Chero zvazvingava, chirwere chacho chinofanira kunge chakasiyana, kuitira kuti chirwere chechirwere chinoshanda chaizvo. Kana kuongororwa kwaitwa zvisina kururama, uye kurapwa kwakanga kusina basa, ipapo murwere anotarisirwa kufa (mumakumi matanhatu ematambudziko).

Zvakakoshawo kuti nhengo dzemhuri yese murwere dzinopa kuedza. Izvi zvichabatsira mune ramangwana kuchengetedza kukurudzirwa kwe porphyria muvana.

Chirwere chacho chinokura sei?

Iyo nzira yekukura kwehutano yakanyanya nyore. Hem - isiri-proteine chikamu che hemoglobin - pasi pesimba rezvimwe zviitiko iyo inokwanisa kuchinja kuva chinhu chakanyanya chepfu. Izvo, inotorawo, inoputira mazinga ari pasi peganda.

Somugumisiro, chivharo chinotanga kuchinja, kuonda. Nokufamba kwenguva, ganda romurwere rinofukidzwa nemaronda uye maronda, kunyanya kana yakaiswa chiedza chezuva. Ichokwadi ndechokuti mihizha ye ultraviolet inokonzera kukanganisa kwemaviri matete.

Kukura, hutachiona hwehutachiona hunosimbisa kwete epidermis chete. Uyewo unofananidzwa ndeyezviputi zvinotapira zvemhino nenzeve. Zvinonzwisisika, izvi zvinovhiringidza ivo. Izvozvo zvinoreva kuti, kuwedzera kwepachifiriya inonyanyisa kunounza kwete muviri chete, asiwo kutambura kwemitemo, sezvo munhu asingakwanisi kutarisa kuratidzwa kwake kusina kujeka mugirazi.

Zvinokonzera kukura

Acute intermittent porphyria chirwere chakaoma uye chakaoma. Zvikonzero zvekufambisa kwayo ndezvizvi:

1. Genetic predisposition: chirwere chinotora nhaka.
2. Kunyanya kukurumidza kwepidyididation ye lipid.
3. Zvirwere zvechiropa. Kunyanya, izvi zvinoshanda kune hepatitis.
4. Yakanyanya kupisa chepfu nemakemikari kana heavy metal salts.
5. Siderosis of Kupffer masero.
6. Iko yakawanda yedare muropa.
7. Kushandiswa kwenguva refu kwemishonga inorema: barbiturates, mahomoni.
8. Kunyanya kunwa doro.

Ndezvipi zvinhu zvinogona kuwedzera njodzi yekurapa?

Porphyria (chii icho - iwe unotoziva kare) chinogona kutsamwiswa:

• Zvokudya zvakaoma. Iyo, zvakare, inetso yakakomba pachiropa, iyo inofanira kushanda kupfuura kupfuura nguva dzose.
• Kunetseka mupfungwa.
• Kubvuma nhamba yakawanda yemishonga.
• Kuonana kakawanda nemakemikari ane ngozi.
• Kuchinja muhutori hunobva pamusana pekubata pamuviri kana kumwedzi kune vakadzi.
• Zvirwere zvinotapukira zvinokonzerwa nechipatara C.
• Kushandisa zvakanyanya doro.

Mukudaro kuti hutachiona hahuvhiringidzi nekudzoka, iwe unongoda kuisa kunze kwezvinhu zvose pamusoro apa, uyewo kutungamira mararamiro akanaka.

Forecast

Kana murwere wacho akaonekwa kuti ane chirwere chakakomba chinonzi porphyria, kufungidzira kwakanyanya kunobva pachiyero chemagetsi. Nokufamba kwenguva, kurapwa kwakatanga kunogona kubvisa zviratidzo mukati mezuva 2-4.

Kana murwere akadzidza zvakanyanya motokari yeuropathy, zvino zviratidzo zvingasapera kwemwedzi nemakore. Munhu akakura munhu anova, zvisinganyanyi kunzwisisika ndezvikonzero zvinokonzera chirwere chacho. Kurwisana kwake kunowedzera.

Zvikamu zvekurapa

Kana murwere wacho akaonekwa kuti ane chirwere chakakomba chinonzi porphyria, kurapwa kunofanira kuitwa nehematologist uye endocrinologist. Zvinonzwisisika, kurapa kunofanira kunge kwakakwana. Inosanganisira:

• Plasmapheresis (kucheneswa kweropa kubva kune chepfu nekubatsira kwemidziyo chaiyo).
• Zvidzivirire kubva kune zuva rakananga.
• Splenectomy (kubviswa kwepleen).
• Kushandiswa kwemishonga inorwadza.
• Kushandisa zvinodhaka kuderedza ropa.
• Kushandisa glucose solution yekukanganiswa.
• и "Рибоксин". Kushandiswa kwemishonga yakadaro sey denosin monophosphate uye Riboxin.
• Kushandisa zvinodhaka zvinokurudzira kubviswa kwe porphyrins yeganda pamwe nemuyo.

Porphyria (chii ichocho - chinotsanangurwa pamusoro) chinoda nzira yakabatana yekurapa. Chirongwa chechirongwa chekurapa chinogona kuva chakadai:

1. Pamusoro pokutanga murwere anofanira kupinda muchipatara muchipatara. Iko unofanirwa kugara uchicherechedza kutengesa kwemwoyo, kushanda kweropa, nezvimwe zviratidzo zvinokosha.
2. Zvakakosha kusabvisa zvachose zvikonzero zvinokonzera kuwedzera kwechirwere ichi.
3. Kana chikonzero chaicho chemamiriro ekurwara chisati chagadziriswa, murwere anokwanisa kupiwa mukana weglucose. Zvisinei, iyi nzira yekurapa inobatsira chete kana kurwisa kwe porphyria kusina simba.
4. Zvakakoshawo kuti utange kutanga kurapa nehana. Kuvandudza munyaya iyi kunogona kuitika mushure mekuita 2-4 maitiro.
5. Kuparara kwezviratidzo zvakare chikamu chinokosha chekurapa. Zvakakosha kuongorora hurumende yekunwa kuitira kuti murwere arege kushaya mvura. Kuti ubvise marwadzo, iwe unoda paracetamol, pamwe neancotic analgesics (yakapiwa nemishonga). Beta-blockers inogona kushandiswa kurapa mwoyo arrhythmias uye zvinetso zvezvinetso. Utachiona hunofanira kurapwa ne penicillin kana cephalosporins.

General classification

Acute intermittent porphyria inogona kuva yemhando inotevera:

1. Pathology inokonzerwa nekushaikwa kwe dehydratase.
2. Hereditary coproporphyria.
3. Variegate porphyria.

Ndeipi mishonga isingagoni kudyiwa?

Acute intermittent porphyria inogona kuitika nekuda kwekuiswa kune dzimwe mishonga. Nokudaro zvakakosha kuziva kuti ndeupi wavo asingagoni kutorwa kuti arege kukuvadza kurwisa.

Saka, kana munhu ane chirwere chakakomba chinonzi porphyria, urongwa hwemishonga inorambidzwa unofanirwa kuyeukwa:

• Mishonga inotyisa zvikuru: Dapsone, Danazol, Diphenylhydantone, Diclofenac, Carbamazepine, Meprobamate, Novobiocin, uye Chlorokhin.
• Inogona kutyisa: Clonazepam, Ketamine, Clonidine, Nortriptyline, Rifampicin, Theophylline, Erythromycin, uye Spironolactone.

Kudzivirira zvirwere

Zvinosuruvarisa, nhasi vanachiremba havagoni kupa chero chinetso chekudzivirira chinogona kudzivirira chirwere. Zvisinei, dzimwe mazano anogona kuitwa kuitira rubatsiro rukuru rwemuviri:

1. Zvakanakisisa kurega kusvuta uye kunwa.
2. Zvakakosha kudya izvo zvikafu zvinenge zvakawanda zvevhithamini, kunyanya boka re B.
3. Inofanira kuchengetedza ganda kubva kune zvakananga kune chiedza chezuva: magirazi, maguzu akavharwa, hove kana hofu kana uchienda kumigwagwa inodiwa!
4. Zviri nani kudzivisa zvinhu zvose izvo zvinogona kukonzera kuwedzera.
5. Zvakakosha kutsvaga mazano evanhu vane chikafu, kuitira kuti iye anobatsira kusarudza kudya kwakakwana.

Kune mamwe mazano anonyanya kukosha echiremba uyo murwere anofanira kugara achicherechedza. Semuenzaniso, pamwe naye pane nguva dzose inofanira kuva nehomberi iyo ichanyorwa kuti munhu ane chirwere ichi.

Ndizvo zvose zvinoratidzwa nehutano hwehutano. Iva noutano!