Mob sib quas ntus porphyria: cov tsos mob, mob, kev kho mob

Caj kab mob yog cov feem ntau nyuaj thiab tsis yooj, vim hais tias mus kiag li tau tshem ntawm lawv yog tsis yooj yim sua. Ib tug xws li tus kab mob yog mob sib quas ntus porphyria. Qhov no yog tshwm sim los ntawm roj ntsha kab mob nyob rau hauv uas lub xov tooj ntawm porphyrins nyob rau hauv cov ntshav qab. Qhov no hom ntawm pathology yog suav tias yog ib qho ntawm feem nrov ntawm tag nrho cov hom ntawm kev tshuaj ntsuam genetic porphyria.

Thaum lub sij hawm tus kab mob no muaj ib tug cia hnab khib tej yam tshuaj lom nyob rau hauv tib neeg lub nruab nrog. Feem ntau cov feem ntau thiaj paub hais tias pathology ntawm lub fairer nrog txiv neej pw. Thiab nws yuav tau triggered thaum uas cev xeeb tub thiab yug menyuam.

symptomatology ntawm tus kab mob

Yog hais tias ib tug neeg mob muaj mob sib quas ntus porphyria, cov tsos mob muaj xws li:

• Ib txhia xws li loog loog ntawm daim tawv nqaij.
• Polyneuritis (ntau hlab hlwb puas tsuaj).
• Pob.
• Lub qhov koob liab thiab o ntawm daim tawv nqaij.
• Pigmentation thiab hlwv ntawm daim tawv nqaij qhov chaw uas yog feem ntau qhib.
• Microcysts (txo zais zis muaj peev xwm, nyob qhov twg sau tseg pheej cuam tshuam ntawm nws functions).
• Psychosis.
• Organic pathological kev hloov nyob rau hauv lub siab hais tias muaj kev cuam tshuam nws functionality.
• Anemia.
• Coma.
• Mob nyob rau hauv lub plab mog, thiab nws yog ib tug heev muaj zog. lawv nrog ntxiv xeev siab thiab ntuav.
• Cov kev puas tsuaj ntawm tus ntsia thawv phaj.
• Ua kom siab.
• tso zis teeb meem.
• Feeb tsis meej, ntxhov siab vim, hallucinations.
• Tuag tes tuag taw ntawm ua pa nqaij.

Nws yuav tsum tau muab sau tseg tias thawj cov tsos mob cai nyob rau hauv cov neeg mob muaj hnub nyoog 20 mus rau 40 xyoos nyob rau hauv daim ntawv ntawm cov tawm tsam. Ntxiv mus, lawv yuav tau rov qab feem ntau los yog tshwm sim ib zaug xwb nyob rau hauv ib tug lub neej. Yog hais tias tus nres kab mob kev, tej zaum nws yuav tshwm sim ib tug ua txhaum nyob rau ib feem ntawm lub brainstem, cranial paj (oculomotor thiab lub ntsej muag), autonomic NS.

Cov tsos mob ntawm porphyria yog manifested tsis yog txhua txhua nqa. Piv txwv li, 80% ntawm lawv yuav puas yog unaware ntawm qhov teeb meem. Ib tug feature ntawm tus kab mob tawm tsam yog hais tias nyob rau hauv tag nrho nws cov ntaub ntawv nyob rau hauv tus neeg mob cov zis hloov xim thiab yuav liab, xim av los yog xim liab.

Mob ntaub ntawv ntawm porphyria tsis tshua muaj. Symptomatology ntawm tus kab mob yog hais txawv.

pathology diagnostics

Nyob rau hauv cov ntaub ntawv ntawm cov kab mob xws li mob sib quas ntus porphyria, mob yuav tsum tau ceev faj. Feem ntau siv kuaj ntsuam xyuas ntawm cov zis thiab cov ntshav, uas yuav tsum qhia tus nqi ntawm cov porphobilinogen, porphyrin theem nyob rau hauv ntshav. Yog hais tias tsawg kawg yog ib qho hnoos qeev yog txawv, tus neeg mob tej zaum yuav muab ib tug ntxiv kev soj ntsuam.

Piv txwv li, nws yog ib qho tseem ceeb los mus ua kom ib tug radiography ntawm lub plab kab noj hniav, uas yuav pab kom paub tias ileus. Nyob rau hauv tas li ntawd, muaj ib co diagnostic luj rau pab tshawb nrhiav raws li meej li sai tau:

1. Thaum lub sij hawm nres: cov zis yog sai heev tas PBG thiab ALA.
2. Nyob rau hauv kev soj ntsuam kuaj rau cov neeg kawm ntawv uas yuav txo tau deaminase kev ua si yuav tsum ua thaum lub sij hawm txoj kev zam txim.

Nyob rau hauv cov ntaub ntawv yog, lub differential mob yuav tsum tau rau cov kab mob kev kho mob yog tiag tiag zoo. Yog hais tias xeem tau lawm kom zoo, thiab cov kev kho mob yog tsis zoo thiab tus neeg mob tos rau txoj kev tuag (60% ntawm cov neeg).

Nws tseem yog ib qho tseem ceeb uas cov kev ntsuam xyuas tau dhau tag nrho cov mej zeej ntawm tus neeg mob lub tsev neeg. Qhov no yuav pab tau nyob rau hauv lub neej yav tom ntej los mus tiv thaiv cov kev loj hlob ntawm porphyria nyob rau hauv lub offspring.

Yuav ua li cas yog muaj tus kab mob?

Lub mechanism ntawm kev loj hlob ntawm pathology yog heev yooj yim. Heme - uas tsis yog-protein feem ntawm hemoglobin - nyob rau hauv tus ntawm tej yam tseem ceeb uas muaj peev xwm ntawm transforming ib tug heev tshuaj lom tshuaj. Nws, nyob rau hauv lem, erodes cov ntaub so ntswg nyob rau hauv daim tawv nqaij.

Li ntawd, cov cover pib ua xim av, thinning. Thaum lub sij hawm, ntawm daim tawv nqaij ntawm tus neeg mob yog them nrog qhov txhab thiab ulcers, tshwj xeeb tshaj yog yog hais tias nws yog raug tshav ntuj. Qhov tseeb hais tias ultraviolet lub teeb ua kev puas tsuaj thiab thiaj li ilv fabrics.

Tsim, tus kab mob no cov txheej txheem muaj feem xyuam rau tsis tsuas cov epidermis. Tsis tas li ntawd cuam tshuam thiab cartilaginous ntaub so ntswg ntawm lub qhov ntswg, pob ntseg. Lawm, nws deforms lawv. Qhov ntawd yog mob sib quas ntus porphyria theem tsis tau tsuas yog lub cev tab sis kuj puas siab puas ntsws txoj kev txom nyem raws li ib tug txiv neej tsis tau saib nws disfigured lub ntsej muag nyob rau hauv daim iav.

ua rau

Mob sib quas ntus porphyria - tus kab mob no yog txoj thiab tsis yooj yim. Yog vim li cas rau nws txoj kev loj hlob yog raws li nram no:

1. Caj predisposition: pathology yog pub.
2. Ntau dhau lawm ceev ntawm lipid peroxidation.
3. mob siab. Qhov no yog tshwj xeeb tshaj yog muaj tseeb ntawm tus kab mob siab.
4. Muaj zog tshuaj lom los yog ntsev ntawm hnyav hlau.
5. Siderosis Kupffer hlwb.
6. Ib tug ntau ntawm cov hlau nyob rau hauv cov ntshav.
7. Ntev siv ntawm hnyav tshuaj: barbiturates, cov tshuaj hormones.
8. Mob alcoholism.

Yuav ua li cas yam yuav ua rau kom txoj kev pheej hmoo ntawm tus kab mob?

Porphyria (nws yog dab tsi - koj twb paub) yuav tau triggered thaum uas:

• Ib tug nruj kev noj haus. Nws, dhau lawm, yog ib tug loj tab kaum rau hauv lub siab, uas yog yuam ua hauj lwm mus ua hauj lwm ntau tshaj li niaj zaus.
• Kev nyuaj siab.
• Tau txais ib tug loj tus naj npawb ntawm cov tshuaj.
• Nquag sib cuag nrog yus cov tshuaj.
• Hormonal hloov vim lub cev xeeb tub los yog ua poj niam nyob rau hauv cov poj niam.
• Complex kab pathologies xws li kab mob siab C.
• Ntau tau ntawm dej cawv.

Yuav kom pathology yog tsis muaj kev txhawj xeeb txog tshaj thiab tshaj dua, koj cia li xav tau los mus tshem tawm tag nrho cov ntawm cov yam tseem ceeb uas muaj npe saum toj no, raws li zoo raws li ib tug noj qab nyob zoo txoj kev ua neej.

outlook

Yog hais tias tus neeg mob yog mob mob sib quas ntus porphyria, lub raug nyob rau hauv lub degree ntawm lub paj txoj. Raws sij hawm pib txoj kev kho eliminates cov tsos mob tsis pub dhau 2-4 hnub.

Yog hais tias tus neeg mob tsim mob loj heev lub cev muaj zog neuropathy, ces tej zaum yuav muaj tsis muaj cov tsos mob kom ploj rau lub hlis thiab xyoo. Cov laus ib tug neeg tiav niam txiv, cov tsawg rhiab rau yam uas ua rau tus kab mob. Nws tau txo lub zaus ntawm tawm tsam.

Nta ntawm kev kho mob

Yog hais tias tus neeg mob yog mob mob sib quas ntus porphyria, cov kev kho mob yuav tsum ua ib tug hematologist thiab ib tug endocrinologist. Ntawm cov hoob kawm, kev kho yuav tsum tau kev. Nws muaj xws li:

• Plasmapheresis (ntshav ntxuav toxins nrog kev pab los ntawm tshwj xeeb cov khoom).
• Kev pab tiv thaiv los ntawm ncaj qha tshav ntuj.
• Splenectomy (tshem tawm ntawm tus po).
• Siv analgesic tshuaj.
• Kev siv cov tshuaj kom txo tau cov ntshav siab.
• Siv ntawm ib tug zib tov rau Txoj kev lis ntshav.
• и "Рибоксин". Cov kev siv ntawm tej cov tshuaj raws li zoo denozinmonofosfat thiab "Riboksin".
• Siv cov cuab yeej uas ua rau kom qhov kev tshem tawm ntawm daim tawv nqaij ntawm porphyrins nyob rau hauv cov zis.

Porphyria (dab tsi yog no - piav saum toj no) yuav tsum tau ib tug kev mus kom ze rau kev kho mob. Yog txoj kev kho tswvyim yuav yog:

1. Yuav pib, tus neeg mob yuav tsum tau mus tsev kho mob. Ntawm no nws yog tsim nyog los lossi saib xyuas lub plawv dhia, cov ntshav siab thiab lwm yam tseem ceeb heev tshwm sim.
2. Nws yog ib qho tseem ceeb mus kaws kiag li tag nrho cov yam tseem ceeb uas ua rau lub ntsuas ntawm tus kab mob.
3. Yog hais tias lub caij nyoog ua kom muaj tus pathological mob twb tsis tau tau txiav txim siab, tus neeg mob yuav ua ib qho kev Txoj kev lis ntshav ntawm qabzib. Txawm li cas los, qhov no kev kho mob pab yog hais tias porphyria tsis yog ib tug muaj zog nres.
4. Nws tseem ceeb heev kom sai pib tus neeg mob kev kho mob siv hemin. Improvement nyob rau hauv cov ntaub ntawv no, tej zaum yuav tuaj tom qab 2-4 kev kho mob.
5. Ntaus cov tsos mob kuj yog ib qho tseem ceeb heev ib feem ntawm txoj kev kho mob. Nws yog tsim nyog los ua raws li cov haus regimen tias tus neeg mob tsis tshwm sim lub cev qhuav dej. Rau qhov mob xav tau kev paracetamol thiab narcotic analgesics (tshuaj ntawm tus kws kho mob). Rau cov kev kho mob ntawm lub plawv mob, thiab cov teeb meem uas muaj ntshav siab kuj yuav siv beta-blockers. Nrog kab mob yuav tsum tau hais txog nrog los ntawm penicillin los yog cephalosporins.

General kev faib

Mob sib quas ntus porphyria yog xws hom:

1. Pathology tshwm sim los ntawm ib tug tsis muaj peev xwm dehydratase.
2. Mob muaj keeb coproporphyria.
3. Variegated porphyria.

Yuav ua li cas cov tshuaj yuav tsis txhob noj?

Mob sib quas ntus porphyria yuav tshwm sim vim cov teebmeem ntawm tej yam tshuaj. Yog li ntawd nws yog tsim nyog los paub uas lawv yuav tsum tsis txhob coj thiaj li tsis mus ntxias ib tug nres.

Yog li ntawd, yog tias ib tug neeg tau mob sib quas ntus porphyria, txwv tsis pub siv yeeb tshuaj daim ntawv teev yog tsim nyog los paub los ntawm lub plawv:

• Cov no yog cov heev txaus ntshai tshuaj, "dapsone", "Danazol," "Difenilgidanton" "Diclofenac", "carbamazepine", "meprobamate," "Novobiocin" "Chloroquine."
• Uas yus "clonazepam" "Ketamine", "Clonidine", "Nortriptyline", "Rifampicin" "Theophylline" "Erythromycin", "Spironolactone".

tiv thaiv kab mob

Tu siab, cov kws kho mob yuav tsis tam sim no muaj ib tug ntau ntawm kev tiv thaiv uas yuav tiv thaiv tau cov kab mob. Txawm li cas los, ib co tswv yim yuav ua tau rau lub zuag qhia tag nrho cov nyiaj them yug ntawm lub cev:

1. Nws yog qhov zoo tshaj plaws muab kev haus luam yeeb thiab haus dej haus cawv.
2. Nws yog ib qho tseem ceeb rau noj cov zaub mov, raws li ib feem ntawm cov uas muaj teeb meem loj nqi ntawm cov vitamins, tshwj xeeb tshaj yog B pab pawg neeg
3. Tiv thaiv koj daim tawv nqaij los ntawm ncaj qha tshav ntuj: tsom-iav, muab xauv khaub ncaws, kaus mom los yog kaus mom thaum nkag mus kawm rau txoj kev yuav tsum tau!
4. Nws yog zoo dua kom tsis txhob tag nrho cov yam tseem ceeb uas muaj peev xwm ua ib tug flare.
5. Nws yog ib qho tseem ceeb mus tham ib tug dietitian, li ntawd, nws tau pab xaiv qhov zoo tshaj plaws cov khoom noj.

Muaj ib co tswv yim pom zoo pab ntawm cov kws kho mob uas tus neeg mob yuav tsum nco ntsoov ua raws li. Piv txwv li, yog hais tias muaj yuav tsum yeej ib txwm yog ib tug saws tes, uas yuav tau sau ntawv, hais tias txiv neej yog tus kab mob.

Hais tias yog tag nrho cov nta uas muab los ntawm lub pathology. Nyob twj ywm noj qab nyob zoo!