Pachimake likukulira porphyria: zizindikiro, matenda, mankhwala

matenda majini ndi zovuta kwambiri ndi zovuta, chifukwa kwathunthu tichotse iwo n'zosatheka. matenda china pachimake likukulira porphyria. Izi zimachitika chifukwa cha matenda chawo chimene chiwerengero cha porphyrins mu ukuwonjezeka magazi. Mtundu wa kudwala ndi chimodzi cha wotchuka kwambiri pakati pa mitundu yonse ya porphyria chibadwa.

Pa matenda pali kudzikundikira poizoni ziwalo. Ambiri zambiri matenda kudwala akazi fairer. Ndipo akhoza zidzayambike ndi pakati komanso pobereka.

symptomatology matendawa

Ngati wodwala akufotokozera pachimake likukulira porphyria, zizindikiro ndi monga:

• Ena dzanzi wa khungu.
• Polyneuritis (angapo mitsempha kuwonongeka).
• Totupa.
• Redness ndi kutupa kwa khungu.
• Mtundu ndi matuza pa malo khungu zimene ndi lotseguka.
• Microcysts (yafupika chikhodzodzo mphamvu, komwe taonera kulimbikira siudzalandilidwa ngathi lamulo ntchito zake).
• Maganizo.
• Organic kusintha pathological mu chiwindi zimene zimakhudza magwiridwe ake.
• Magazi m'thupi.
• Chikomokere.
• Kupweteka m'mimba ndipo ndi amphamvu kwambiri. iwo limodzi ndi nseru zina ndi kusanza.
• Kuwonongedwa kwa mbale misomali.
• Kuonjezera kuthamanga.
• pokodza matenda.
• Chisokonezo, nkhawa, kuyerekezera zinthu m'maganizo.
• Ziwalo minofu kupuma.

Tikumbukenso kuti zizindikiro woyamba anati odwala zaka 20 mpaka 40 mwa mawonekedwe kuukira. Komanso, iwo akhoza kawirikawiri kapena kuchitika kamodzi kokha moyo. Ngati matenda kuukira akufotokozera, zingaoneke kuphwanya pa mbali ya brainstem, mitsempha cranial (oculomotor ndi nkhope), autonomic NS.

Zizindikiro za porphyria ali kuwonetseredwa osati onyamula onse. Mwachitsanzo, 80% ya iwo akanakhoza konse sangadziwe vuto. Mbali A kuukira matenda n'chakuti mtundu uliwonse mu mkodzo mtima kusintha mtundu ndipo pinki brown kapena wofiira.

mitundu wokonda porphyria n'zochepa. Symptomatology matendawa chimaoneka mosiyana.

kudwala diagnostics

Pankhani ya matenda monga pachimake likukulira porphyria, matenda ayenera kusamala. Amagwiritsidwa ntchito mayesero zasayansi ya mkodzo ndi mwazi limene uwulule kuchuluka kwa porphobilinogen, porphyrin misinkhu mu madzi. Ngati chitsanzo chimodzi wosasangalala, wodwalayo ingaperekedwe kuunikidwa zina.

Mwachitsanzo, ndikofunika kupanga radiography wa zonse m'mimba, zomwe zimathandizanso kudziwa ileus. Komanso, pali mfundo zina ziti matenda kuthandiza oterewa molondola:

1. Pa nthawi ya: ndi mkodzo ndi kwambiri mofulumira excreted PBG ndi Ala.
2. Mu mosamala kwa mlingo wa ntchito kuchepetsa deaminase zikhoza kupangidwa panthawi chikhululukiro.

Mulimonsemo, matenda masiyanidwe ayenera matenda mankhwala anali kwenikweni ogwira. Ngati Kupenda wapanga bwino, ndi mankhwala anali osamveka ndi mtima amadikirira imfa (60% milandu).

N'kofunikanso kuti mayesero zapita mamembala onse a m'banja la wodwalayo. Izi zidzathandiza m'tsogolo kupewa chitukuko cha porphyria mu mbewu.

Kodi matenda?

Limagwirira chitukuko cha kudwala ndi wosavuta. Heme - sanali mapuloteni gawo la hemoglobin - mchikakamizo cha zinthu zina amatha yozindikira chinthu kwambiri poizoni. Izo, nawonso, imawononga minofu pansi pa khungu.

Choncho chivundikiro wayamba kukhala bulauni, kupatulira. M'kupita kwa nthawi, khungu la mtima ali ndi mabala ndi zilonda, makamaka ngati dzuŵa. chakuti kuwala ultraviolet umawononga ndi nsalu kotero wosakhwima.

Osauka, ndondomeko matenda amakhudza osati khungu. Anakhudzanso ndipo zimakhala cartilaginous mphuno, makutu. Mwachibadwa, deforms iwo. Kuti ndi pachimake likukulira porphyria umabweretsa osati mavuto komanso maganizo ngati munthu sungakhoze kuyang'ana pa nkhope yake wosawoneka pa galasi.

zimayambitsa

Pachimake likukulira porphyria - matenda ndi zovuta ndi zovuta. Zifukwa chitukuko ali motere:

1. Majini chingawapangitse kukhala zidakwa: kudwala ndi akatengela apabanja.
2. liwiro kwambiri wa zamadzimadzi peroxidation.
3. chiwindi matenda. Izi ndi zoona makamaka chiwindi.
4. Wamphamvu mankhwala poyizoni kapena mchere wa zitsulo.
5. Siderosis Kupffer maselo.
6. A zambiri chitsulo magazi.
7. Yaitali ntchito mankhwala lolemera: barbiturates, mahomoni.
8. Matenda uchidakwa.

N'chiyani chingachititse azitaya matenda?

Porphyria (icho chiri - mukudziwa kale) Kodi zidzayambike ndi:

• A zakudya kwambiri. Iyenso, ndi katundu lalikulu chiwindi, amene anakakamizika ntchito kwambiri kuposa masiku.
• Kupanikizika.
• Kulandira ambiri mankhwala.
• Pafupipafupi kukhudzana ndi mankhwala oopsa.
• kusintha m'thupi chifukwa cha mimba kapena kusamba mu akazi.
• Zovuta pathologies matenda monga a chiwindi C.
• Kugwiritsa kumwa mowa.

Kuti kudwala si nkhawa mobwereza bwereza kachiwiri, inu muyenera kuthetsa onse a zinthu tatchulazi, komanso moyo wathanzi.

kaonedwe

Ngati wodwalayo ndi akapezeka ndi pachimake likukulira porphyria, kuti madokotala ananena zawo zimatengera pa mlingo wa molekezera mitsempha. Panthawi yake anayambitsa mankhwala kumatha zizindikiro pasanathe masiku 2-4.

Ngati wodwala anayamba aakulu galimoto neuropathy, ndiye pasakhale zizindikiro kutha kwa miyezi ndi zaka. The munthu wamkulu amakhala, chindichepere tcheru zinthu zimene zimayambitsa matenda. Iye kuchepetsa pafupipafupi kuukira.

Features mankhwala

Ngati wodwalayo ndi akapezeka ndi porphyria pachimake likukulira, mankhwala ayenera kutulutsa panthawiyi, dokotala ndi m'thupi yemwenso. Kumene, mankhwala ayenera Integrated. Pamafunikanso:

• Plasmapheresis (magazi kuyeretsa poizoni ndi thandizo la zipangizo zina zapadera).
• Chitetezo dzuwa.
• Splenectomy (kuchotsa ndulu ya).
• Gwiritsani ntchito mankhwala analgesic.
• Kugwiritsa ntchito mankhwala magazi kuchepetsa.
• Ntchito ya shuga njira yothetsera kulowetsedwa.
• и "Рибоксин". Kugwiritsa ntchito mankhwala monga bwino denozinmonofosfat ndi "Riboksin".
• Kugwiritsa ntchito zipangizo zimene zimachititsa kuchotsa khungu la porphyrins mu mkodzo.

Porphyria (ichi - tafotokozazi) amafuna munthu njira Integrated mankhwala. Est mankhwala chiwembu zikhoza kukhala:

1. Kuyamba, wodwala ayenera kuvomereza kuchipatala. Apa m'pofunika zonse kuyan'ana kugunda kwa mtima, kuthamanga kwa magazi ndipo zizindikiro zina zofunika.
2. Nkofunika kuti achotse mwamtheradi zinthu zonse hule akuchuluka matendawa.
3. Ngati chifukwa chenicheni cha chikhalidwe pathological silinakwaniritsidwe mtima, wodwala apange kulowetsedwa wa mphamvu. Komabe, mankhwala amathandiza pokhapokha ngati porphyria si vuto amphamvu.
4. M'pofunikanso mwamsanga kuyamba mankhwala wodwala ntchito hemin. Kupititsa patsogolo maphunziro Pankhaniyi, akhoza kudza pambuyo mankhwala 2-4.
5. Kucotsa zizindikiro ndi mbali yofunika ya ndondomeko mankhwala. M'pofunika kutsatira kumwa kamwedwe kuti wodwalayo sizichitika madzi m'thupi. Kupweteka muyenera paracetamol ndi analgesics chomwa mankhwalawa (mankhwala a dotolo). Zochizira matenda a mtima, ndi mavuto ndi magazi angagwiritsidwe ntchito beta-blockers. Ndi matenda ayenera anachitira ndi mwa penicillin kapena cephalosporins.

General gulu

Pachimake likukulira porphyria ndi mitundu ngati:

1. Kudwala chifukwa cha kuperewera dehydratase.
2. Cholowa coproporphyria.
3. Variegated porphyria.

Kodi mankhwala sangathe kudya?

Pachimake likukulira porphyria kumachitika chifukwa zotsatira za mankhwala ena. Choncho m'pofunika kudziwa ndani wa iwo sayenera kumwedwa kuti tifulumizane nkhondo.

Choncho, ngati munthu wakhala pachimake likukulira porphyria, analetsa mndandanda mankhwala m'pofunika kudziŵa ndi mtima:

• Awa ndi mankhwala owopsa kwambiri, "dapsone", "Danazol," "Difenilgidanton" "Diclofenac", "carbamazepine", "meprobamate," "Novobiocin" "Chloroquine".
• Zikanavulaza "clonazepam" "Ketamine", "Clonidine", "Nortriptyline", "Rifampicin" "theophylline" "mankhwalawa", "Spironolactone".

kupewa matenda

Mwatsoka, madokotala sindingakhoze tsopano kupereka osiyanasiyana zodzitetezera kuti chingalepheretse matenda. Koma ena nsonga angathe kuchitidwa thandizo chonse cha thupi:

1. Ndi bwino kusiya fodya ndi kumwa mowa.
2. Nkofunika kudya zakudya zimenezi, monga mbali ya amene ali ndalama zofunika kwambiri za mavitamini, makamaka B gulu
3. Kuteteza khungu lanu ku dzuwa: magalasi, zovala chokhoma, zipewa kapena zisoti pamene kulowa msewu chofunika!
4. Ndi bwino kupewa zinthu amene lingachititse ndi zinayambira.
5. Nkofunika kukaonana ndi dietitian, kotero anathandiza kusankha zakudya zabwino.

Pali ena ayamikira zothandiza madokotala kuti wodwalayo ayenera kutsatira. Mwachitsanzo, ngati inayenera kukhala chibangili, zomwe zinalembedwa, munthu ali ndi matendawa.

Ndi mbali zonse zidaperekedwa ndi kudwala kwa. Khalani wathanzi!