Acute porphyria uveza: iimpawu, isifo, unyango

Izifo zofuzo i entsonkothileyo kakhulu kwaye kunzima, kuba ukufumana ngokupheleleyo, lahlani kubo akunakwenzeka. Esinye isifo okunjalo porphyria yondele ngakanye. Oku kubangelwa sisifo ilifa apho inani porphyrins landa egazini. Olu hlobo leengxaki igqalwa omnye ethandwa kakhulu phakathi kwazo zonke iintlobo porphyria yemfuza.

Ngexesha sifo kukho zemali izinto eziyityhefu kwizigqeba yabantu. Kakhulu sivame ukuxilongwa ngezifo besini bubuhle. Kwaye nga kuxhokonxwa ekhulelwe naxa ezala.

symptomatology kwesifo

Ukuba isiguli porphyria etsolo ngakanye, iimpawu ziquka:

• Ezinye ndindisholo kwesikhumba.
• Polyneuritis (ukonakala luvo ezininzi).
• Angawokubhuda.
• Ububomvu kunye ekweneni ulusu.
• Nombala kunye namadyungudyungu kwi kwimimandla yesikhumba zivuleleke ngokuqhelekileyo.
• Microcysts (umthamo isinyi yancitshiswa, apho kuqatshelwe ukuphula ezingisileyo imisebenzi yayo).
• Psychosis.
• utshintsho yezifo Organic esibindini ezichaphazela ukusebenza kwayo.
• Anemia.
• Isiqaqa.
• Ubuhlungu esiswini, kwaye sinamandla kakhulu. kokuba zihamba kunye isicaphucaphu vetshe kunye nokugabha.
• Ukutshatyalaliswa isikhonkwane nembasa.
• Umfutho.
• echama disorder.
• Ukudideka, ixhala, nemibono.
• Ukukhubazeka izihlunu zemiphunga.

Kufuneka kuqatshelwe ukuba iimpawu zokuqala waphawula kwizigulane abaneminyaka eyi-20 ukuya kuma-40 ngohlobo uhlaselo. Ngaphezu koko, ukuba ingaphinda-phindwa amaxesha amaninzi okanye kwenzeka kanye kuphela ebomini. Ukuba isifo ukuhlaselwa iphuhlisa, ukuze kubonakale ukugxojwa phezu inxalenye brainstem, luvo cranial (oculomotor kunye nobuso), NS yawo.

Iimpawu porphyria sibonakalalisiwe hayi bonke abathwali. Umzekelo, ama-80% kubo ndingaze ndibe akayiboni le ngxaki. Uphawu zawahlasela sifo kukuba kuzo zonke iintlobo yayo nomchamo yomguli itshintsha umbala kwaye lube pink, omdaka okanye obomvu.

iintlobo Acute of porphyria zinqabile. Symptomatology kwesifo kubonakaliswa ngokwahlukileyo.

uxilongo zokugula

Kwimeko izifo ezifana porphyria etsolo ngakanye, uphethwe kufuneka silumke. Adla ngokusetyenziswa laboratory umchamo negazi, nekufuneka ukutyhila isixa porphobilinogen, porphyrin amanqanaba plasma. Ukuba ubuncinane enye isampula ezingaqhelekanga, isigulane kufuneka yabelwe uviwo olongezelelweyo.

Umzekelo, kubalulekile ukuba wenze ray le kwisisu, nto leyo eza kunceda ukuchonga ileus. Ukongeza, kukho ezinye iindlela yoxilongo gciwane ngokuchanekileyo kangangoko kunokwenzeka:

1. Ebudeni kuhlaselwa: i umchamo kakhulu idubule ngokukhawuleza PBG kunye ALA.
2. Xa ukuhlolwa ngenxa iqondo kokunciphisa deaminase ingenziwa ngexesha kuxolelwe.

Kuzo zombini ezi meko, uxilongo umahluko kufuneka ibe unyango lwezifo sasebenza ngokwenene. Ukuba yoviwo lwenziwe ngokufanelekileyo, kwaye unyango ninganqeni, isigulane alindele ukuba ukufa (60% amatyala).

Kwakhona kubalulekile ukuba iimvavanyo zigqithile onke amalungu entsapho yesigulane. Oku kuya kunceda kwixesha elizayo ukuthintela uphuhliso porphyria kwi inzala.

Njani na esi sifo?

Indlela yophuhliso leengxaki ilula. Heme - non-protein inxenye mpilo - phantsi kwempembelelo yeemeko ezithile esikwaziyo ukuguqula into inetyhefu kakhulu. Ngoko, ke, iparafini izicwili phantsi kwesikhumba.

Ngenxa yoko, i-inshorensi liqala ukuba mdaka, olindelwe. Ekuhambeni kwexesha, isikhumba isigulane wambu amanxeba kunye nezilonda, ingakumbi xa zidibene nelanga. Inyaniso yokuba ukukhanya yelanga ebangela umonakalo kunye amalaphu ubuncwane kangaka.

Ukuphuhlisa, inkqubo sifo sihlasela kuphela sangaphandle. Kwakhona ezichaphazelekayo nezihlunu cartilaginous empumlo, iindlebe. Ngokuqhelekileyo, oko deforms kubo. Loo nto porphyria yondele ngakanye izisa nje ukubandezeleka kuphela emzimbeni kodwa ngengqondo umntu akakwazi ukujonga ubuso bakhe babonakala esipilini.

oonobangela

Acute porphyria uveza - esi sifo entsonkothileyo nzima. Izizathu uphuhliso zayo zimi ngolu hlobo lulandelayo:

1. yofuzo: ngezifo into njengelifa.
2. Isantya enkulu kakhulu yokuba lipid peroxidation.
3. isifo sesibindi. Oku kuyinyani ngakumbi kwesibindi.
4. chemical Ityhefu Strong okanye iityuwa ze enzima.
5. iiseli Siderosis Kupffer.
6. A lot of yentsimbi egazini.
7. ukusetyenziswa elide amayeza enzima: barbiturates, iihomoni.
8. utshwala Chronic.

Ziziphi izinto kwandisa ubungozi sifo?

Porphyria (yintoni na - sele ukwazi) unako kuxhokonxwa:

• Ukudla engqongqo. Yena, kakhulu, kuba umthwalo elibi kwesibindi, kunyanzelekile ukuba asebenze ngaphezulu kunesiqhelo.
• Uxinezeleko.
• Ukufumana inani elikhulu iziyobisi.
• contact rhoqo kunye iikhemikhali eziyingozi.
• utshintsho hormone ngenxa ukukhulelwa okanye sexesheni kwabasetyhini.
• ezimbi ezasulelayo Complex ezifana kwesibindi C.
• kukutya kakhulu utywala.

Ukuze ngezifo akukho ukhathazekile malunga ngokuphindaphindiweyo, kufuneka nje ukuphelisa zonke izinto ezidweliswe apha ngentla, kwakunye yokuphila esempilweni.

Outlook

Ukuba isigulane banesifo porphyria etsolo ngakanye, i-ALS ixhomekeke iqondo iziphelo-luvo. Ngexesha unyango liqale ishenxisa iimpawu kwiintsuku 2-4.

Ukuba isigulane kuphuhliswa kakhulu motor neuropathy, ngoko inokuba akukho mpawu ukuba ndinyamalale iinyanga kunye neminyaka. I omdala umntu uba, ukuba novakalelo kancinci ukuya izizathu ezibangela izifo. Yayinciphisa nezihlandlo uhlaselo.

Iimpawu zonyango

Ukuba isigulane banesifo porphyria zosulele ngakanye, unyango kufuneka aveze segazi kunye endocrinologist. Kakade ke, unyango kufuneka kuhlanganiswe. Oku kuquka:

• Plasmapheresis (igazi ukuhlanjululwa ityhefu encediswa nezixhobo ezizodwa).
• Ukukhuselwa elangeni ngqo.
• Splenectomy (ukususwa udakada).
• Sebenzisa imithi iintlungu.
• Ukusetyenziswa kweziyobisi ukuthoba uxinzelelo lwegazi.
• Sebenzisa le isisombululo iswekile for ukumnika.
• и "Рибоксин". Ukusetyenziswa kweziyobisi ezifana kwakunye denozinmonofosfat yaye "Riboksin".
• Ukusebenzisa izixhobo negalelo kususwe isikhumba porphyrins kwi yokuchama.

Porphyria (yintoni na le - ezichazwe apha ngentla) kufuna ukuba kwenziwe ngendlela emanyeneyo unyango. Est therapy scheme kungaba:

1. Okokuqala, isigulane kufuneka ungeniswe esibhedlele. Apha kuyimfuneko ukuba esweni rhoqo izinga kwentliziyo, uxinzelelo lwegazi kunye neminye imiqondiso ebalulekileyo.
2. Kubalulekile ecaleni ngokupheleleyo zonke izinto engabangela zisiba mbi yesi sifo.
3. Ukuba ityala ngqo imeko zophendlo lwezifo okwangoku uzimisele, isigulana unako ukwenza nokubandakanywa glucose. Noko ke, le unyango kunceda kuphela xa porphyria ayilo ukuhlaselwa.
4. Kubalulekile kwakhona ukuba ukuqala ngokukhawuleza unyango nomonde usebenzisa hemin. Ukuphucula kulo mzekelo, zize emva zonyango 2-4.
5. Ukuphelisa iimpawu kwakhona yinxalenye ebalulekileyo ye nkqubo yonyango. Kuyimfuneko ukuba ulandele yokusela kwirejimeni ukuba isigulana kungenzeki emzimbeni. Kuba ubuhlungu kufuneka paracetamol kunye analgesics ezidakumbisayo (iingcebiso ugqirha). Kuba unyango ukuphazamiseka ngentliziyo, kwaye iingxaki kunye noxinzelelo lwegazi zingasetyenziswa beta-blocker. Nosulelo kufuneka kujongwane nayo penicillin okanye cephalosporins.

yokwahlukanisa jikelele

porphyria Acute ngakanye na iintlobo ezifana:

1. Pathology okubangwa dehydratase intswelo.
2. coproporphyria zamafa.
3. porphyria zinaso.

Ziziphi amayeza ukuba batye?

porphyria Acute ngakanye esinokubakho ngenxa yeziphumo iziyobisi ezithile. Ngoko kuyimfuneko ukwazi nguwuphi na kubo akumele ithathwe ukuze bandiqumbise uhlaselo.

Ngoko ke, ukuba umntu iye porphyria etsolo ngakanye, uluhlu kweziyobisi ezithintelweyo kuyimfuneko ukwazi ngentloko:

• Ezi iziyobisi eziyingozi kakhulu, "dapsone", "Danazol," "Difenilgidanton" "Diclofenac", "carbamazepine", "meprobamate," "Novobiocin" "Chloroquine."
• Ezinokuba yingozi "clonazepam" "Ketamine", "Clonidine", "Nortriptyline", "rifampicin" "Theophylline" "Erythromycin", "Spironolactone".

zokuthintela izifo

Ngelishwa, oogqirha ngeke ngoku zinika iinkonzo amanyathelo othintelo ukuba akwazi ukuthintela isifo. Noko ke, ezinye iingcebiso inokwenziwa ukuze baxhase ngokubanzi umzimba:

1. Kungcono ukuyeka ukutshaya nokusela.
2. Kubalulekile ukuba badle kutya, njengenxalenye eziquka imali ebonakalayo weevithamini, ingakumbi iqela B
3. Khusela isikhumba sakho elangeni ngqo: iiglasi, iimpahla itshixiwe, iminqwazi okanye iminqwazi xa ungena esitratweni efunekayo!
4. Kubhetele ukuba ukuphepha zonke ezo zinto engabangela umsi.
5. Kubalulekile ukuba adibane isazi, ngoko wanceda ukuba ukukhetha ukutya kakhulu.

Kukho ezinye iingcebiso eziluncedo oogqirha ukuba isigulane kufuneka athobele. Umzekelo, ukuba kufuneka kusoloko nesacholo, oluya kubhalwa, loo ndoda isifo.

Nantso zonke iimpawu ebonelelwe ngezifo. Hlala uphilile!