Koj puas muaj ib yam kab mob txawv txawv uas koj tsis nkag siab? Tej zaum nws yog Acute Hepatic Porphyria!

Koj puas xav tias koj mob plab heev, qee zaum nrog lub plawv hnyav, thiab koj txhais ceg tsis muaj zog? Tej zaum txawm tias cov kws kho mob kuj tsis paub tias nws yog dab tsi. Ib qho ntawm cov tsos mob txawv txawv, zoo li tsis muaj feem cuam tshuam uas peb yuav tham txog hnub no yog Acute Hepatic Porphyria (AHP). Tsis txhob txhawj, qhov no nyuaj me ntsis, tab sis cia peb nkag siab yooj yim.

Mob porphyria hepatic (AHP) yog dab tsi tiag?

Hais yooj yim xwb, AHP yog ib yam kab mob caj ces uas tsis tshua muaj. Nws pib hauv koj lub siab . Tab sis nws kuj cuam tshuam rau koj lub paj hlwb , ua rau muaj cov tsos mob thoob plaws koj lub cev. Xav txog nws, muaj ib yam dab tsi hu ua heme, uas yog ib feem ntawm hemoglobin hauv peb cov ntshav. Peb lub cev xav tau ntau yam enzymes los ua cov heme no. Ib tug neeg uas muaj AHP muaj qhov tsis txaus, lossis tsis txaus, ntawm qee cov enzymes uas xav tau los ua cov heme no.

Thaum qhov no tshwm sim, yuav ua li cas? Qee cov tshuaj uas yuav tsum tau siv los ua heme tseem tshuav. Peb hu cov khoom seem no ua cov tshuaj porphyrin precursors . Thaum cov no sib sau ua ke, lawv lom rau lub cev. Hauv AHP, cov tshuaj lom no ua ntej sib sau ua ke hauv lub siab. Tom qab ntawd, thaum lawv nkag mus rau hauv cov ntshav thiab cuam tshuam nrog cov hlab ntsha, cov tsos mob pib tshwm sim. Koj puas nkag siab?

Puas muaj ntau hom AHP sib txawv?

Yog, muaj plaub hom AHP tseem ceeb. Plaub hom no muab faib ua cov hom enzymes uas kuv tau hais ua ntej, nyob ntawm seb hom enzyme twg tsis txaus. Txhua hom yog muab lub npe tom qab qhov hu ua "hepatic" vim nws pib hauv lub siab, thiab qhov hu ua "acute" vim tias cov tsos mob tshwm sim sai sai. Raws li kev txiav txim los ntawm hom feem ntau mus rau hom tsawg tshaj plaws, ntawm no yog cov hom:

1. Kab mob Porphyria uas mob ib ntus xwb (AIP): Qhov no yog qhov feem ntau. Kev hloov pauv hauv HMBS gene ua rau tsis muaj enzyme hydroxymethylbilane synthase (HMBS) (tseem hu ua porphobilinogen deaminase (PBGD)). Qhov no tuaj yeem yog kev hloov pauv tshiab ntawm cov noob caj noob ces, lossis nws tuaj yeem tau txais los ntawm kev yug los ua tus cwj pwm autosomal dominant (piv txwv li, tus kab mob tuaj yeem tshwm sim txawm tias tsuas yog ib tug niam txiv tau txais cov noob caj noob ces).

2. Variegate Porphyria (VP): Kev hloov pauv hauv PPOX gene ua rau muaj qhov tsis txaus ntawm cov enzyme protoporphyrinogen oxidase (PPO lossis PPOX). Qhov no kuj tuaj yeem tau txais los ntawm noob caj noob ces raws li autosomal dominant lossis autosomal recessive.

3. Kab mob Coproporphyria uas los ntawm noob caj noob ces (HCP): Kev hloov pauv hauv cov noob caj noob ces 'CPOX' ua rau tsis muaj cov enzyme 'coproporphyrinogen oxidase (CPOX) txaus.' Qhov no kuj tuaj yeem yog los ntawm noob caj noob ces ua autosomal dominant lossis autosomal recessive.

4. ALAD-Deficiency Porphyria (ALAD-Deficiency Porphyria - ADP):Kev hloov pauv hauv cov noob caj noob ces 'ALAD' ua rau muaj qhov tsis txaus ntawm cov enzyme 'delta-aminolevulinic acid dehydratase (ALAD). Nws yog los ntawm kev yug los ntawm 'autosomal recessive' (piv txwv li, tus kab mob tsuas yog tshwm sim tau yog tias ob leeg niam txiv tau txais cov noob caj noob ces).

AHP cuam tshuam li cas rau kuv?

Qhov no yog qhov xav tsis thoob, vim AHP tsis cuam tshuam rau txhua tus tib yam. Ib txhia neeg muaj kev hloov pauv ntawm cov noob caj noob ces rau nws tab sis yeej tsis muaj tsos mob dab tsi . Lwm tus yuav muaj "kev tawm tsam" ntawm cov tsos mob uas tsuas yog tshwm sim ib lossis ob zaug hauv lawv lub neej. Tab sis ib txhia neeg muaj cov kev tawm tsam no ntau zaus, thiab lwm tus muaj lawv ntev , txhais tau tias lawv tseem muaj cov tsos mob uas cuam tshuam rau lawv lub neej txhua hnub. Qee zaum kev tawm tsam tuaj yeem hnyav heev uas koj yuav tsum tau maj nrawm mus rau chav kho mob ceev hauv tsev kho mob. Koj yuav muaj mob hlab ntsha hnyav, hloov pauv ntawm daim tawv nqaij, thiab cov tsos mob ntsig txog lub paj hlwb.

Leej twg yuav muaj feem tau qhov no tshaj?

AHP tuaj yeem cuam tshuam rau cov neeg ntawm txhua haiv neeg lossis xim. Nws tuaj yeem tau txais los ntawm ib tug lossis ob leeg niam txiv uas muaj kev hloov pauv gene. Qhov xav tsis thoob, ntau tus neeg tsis muaj tsos mob . Yuav kom cov tsos mob tshwm sim, kev hloov pauv gene ib leeg tsis txaus. Yuav tsum muaj ntau lwm yam 'ua rau' .

Cov yam ua rau muaj teeb meem no yog dab tsi?

• Kev hloov pauv hormonal (tshwj xeeb tshaj yog thaum lub sijhawm puberty, cev xeeb tub, thiab lub voj voog ntawm lub cev)
• Qee cov tshuaj
• Kev siv cawv
• Haus luam yeeb
• Kev ntxhov siab hnyav
• Kev tswj hwm kev noj zaub mov kom nruj (tshwj xeeb tshaj yog kev txwv calorie)

Nws tseem ceeb heev tias 80% ntawm cov neeg uas muaj cov tsos mob yog cov poj niam uas muaj hnub nyoog yug tau menyuam .

AHP feem ntau npaum li cas?

Nyuaj hais kom meej, vim tias feem ntau tus kab mob no tsis tau kuaj pom zoo. Thoob ntiaj teb, kwv yees li ntawm 5 ntawm 100,000 tus neeg yuav muaj nws. Ntawm cov hom uas tau hais ua ntej, 'Acute Intermittent Porphyria (AIP)' yog hom uas pom tsuas yog 80% ntawm cov neeg mob uas tau tshaj tawm. 'ALAD-Deficiency Porphyria (ADP)' yog hom tsawg tshaj plaws, tsuas yog 9 tus neeg mob tau tshaj tawm thoob ntiaj teb. Xav txog, tsuas yog ib tus ntawm txhua 10 tus neeg uas muaj kev hloov pauv gene cuam tshuam nrog AHP yuav muaj cov tsos mob.

Cov tsos mob ntawm AHP yog dab tsi?

Cov tsos mob ntawm AHP txawv heev. Lawv tuaj yeem nyuaj rau cov neeg mob thiab cov kws kho mob nkag siab, vim tias cov tsos mob tuaj yeem tshwm sim sai sai, hnyav heev, thiab zoo li tsis muaj feem cuam tshuam.

Nov yog qee yam ntawm cov yam ntxwv no:

Mob hlab ntsha

Qhov mob no tuaj yeem tshwm sim rau ntau qhov chaw hauv lub cev:

• Mob plab hnyav heev - Qhov no yog thawj cov tsos mob uas tshwm sim rau feem ntau ntawm cov neeg.
• Mob hauv siab
• Mob nraub qaum
• Mob tes thiab taw

Teeb meem ntawm lub plab zom mov

Vim tias cov hlab ntsha raug cuam tshuam, teeb meem nrog lub cev zom zaub mov kuj tuaj yeem tshwm sim:

• Xeev siab thiab ntuav
• Kev zom zaub mov tsis zoo
• Kab mob cem quav
• Mob raws plab

Cov tsos mob ntawm lub paj hlwb hauv nruab nrab

Cov no muaj feem cuam tshuam ncaj qha rau lub hlwb thiab lub siab:

• Kev ntxhov siab
• Tsis tsaug zog
• Kev Nyuaj Siab
• kev chim siab
• Kev pom tsis tseeb - pom lossis hnov ​​​​​​tej yam uas tsis muaj tiag
• Mob qaug dab peg/qaug dab peg ('Qaug dab peg')

Cov tsos mob ntawm lub paj hlwb peripheral

Cov no txuas nrog cov hlab ntsha hauv lwm qhov ntawm lub cev:

• Cov leeg tsis muaj zog
• Kev tsis muaj zog thiab tingling hauv cov ceg
• Kev qaug zog
• Kev poob ntawm kev hnov ​​​​​​lus
• Cov leeg nqaij tuag tes tuag taw
• Kev ua pa tsis taus - Qhov no txaus ntshai heev thiab ua rau ua pa nyuaj.

Cov tsos mob ntawm lub paj hlwb autonomic

Cov no yog tej yam uas tshwm sim yam tsis tau npaj tseg, tej yam uas peb tswj tsis tau:

• Lub plawv dhia ceev
• Ntshav siab

Cov tsos mob ntawm daim tawv nqaij (tshwj xeeb tshaj yog nyob rau hauv 'Variegate Porphyria' thiab 'Hereditary Coproporphyria')

Cov neeg uas muaj ob hom no yuav muaj teeb meem ntawm daim tawv nqaij thaum raug tshav ntuj :

• Kev rhiab heev rau lub hnub ci
• Mob pob khaus
• Kev hloov xim ntawm daim tawv nqaij (Pigmentation)
• Kev ua kom mob

Qhov tseem ceeb tshaj plaws yog thaum koj muaj mob porphyria, koj cov zis yuav hloov xim . Thaum cov tshuaj porphyrin uas kuv hais txog sib sau ua ke hauv koj lub cev thiab raug tso tawm hauv koj cov zis, koj cov zis tuaj yeem hloov xim liab-ntshav . Lo lus "porphyria" los ntawm lo lus Greek "porphura," uas txhais tau tias "ntshav." Cov porphyrins hauv peb cov qe ntshav liab yog cov uas ua rau cov ntshav liab.

Kev tawm tsam AHP yog dab tsi?

Rau ntau tus neeg uas muaj cov tsos mob AHP, cov no tuaj ua ib qho "kev tawm tsam" qee zaum. Ib txhia neeg ntsib lawv ntau zaus dua, ib txhia tsis tshua muaj. Ib txhia neeg ntsib lawv hnyav dua, thaum lwm tus tsis ntsib. Feem ntau, cov kev tawm tsam no kav ntev li ob peb hnub, thiab tom qab ntawd maj mam nce thiab txo qis. Yog tias koj muaj cov tsos mob hnyav, txaus ntshai, koj yuav tsum mus rau tsev kho mob. Zoo li kuv tau hais, tej yam xws li kev ua pa tsis taus, uas ua rau nws nyuaj rau ua pa, yog cov mob uas xav tau kev kho mob thaum muaj xwm txheej ceev.

Cov tsos mob feem ntau ntawm kev tawm tsam AHP yog mob plab hnyav heev uas tsis paub meej.. Ntau tshaj 90% ntawm cov neeg uas muaj AHP mus ntsib kws kho mob vim yog qhov mob plab no. Txawm li cas los xij, vim muaj ntau yam ua rau mob plab, thiab vim tias tsis yog X-ray lossis CT scan tuaj yeem nrhiav tau qhov ua rau mob no (vim nws yog mob hlab ntsha), nws nyuaj heev rau kev kuaj mob. Vim tias qhov mob plab no nrog rau tej yam xws li xeev siab, ntuav, thiab cem quav, cov kws kho mob yuav xav tias nws yog kab mob zom zaub mov. Yog tias koj kuj muaj teeb meem kev puas siab puas ntsws thiab kev xav, lawv kuj yuav xav tias nws yog kab mob hlab ntsha. Thaum cov tsos mob zoo li tsis muaj feem cuam tshuam no los ua ke, nws yog qhov tsim nyog mus ntsib kws kho mob uas paub txog nws kom paub tias nws yuav yog porphyria.

AHP ua rau muaj cov tsos mob ntev li cas?

Cov neeg uas muaj kab mob tsis hnyav heev (tsis muaj enzyme txaus) tsis tshua muaj cov tsos mob ntev. Lawv tsuas muaj ib lossis ob zaug xwb hauv lawv lub neej. Yog tias lawv pom cov yam ua rau lawv mob, lawv tuaj yeem zam tau. Txawm li cas los xij, qee tus neeg muaj mob ntau zaus, thiab qee cov tsos mob tshwm sim ntau zaus uas lawv suav tias yog mob ntev. Cov tsos mob no suav nrog:

• Mob
• Kev nkees nkees
• Xeev siab
• Neuropathy ( mob, loog, lossis tsis muaj zog hauv ob txhais ceg)

Cov teeb meem ntawm AHP yog dab tsi?

Cov tsos mob ntsig txog lub paj hlwb hauv nruab nrab (xws li kev ntxhov siab, kev pom tsis meej, thiab qaug dab peg) feem ntau tsuas yog tshwm sim thaum muaj kev tawm tsam xwb. Txawm li cas los xij, cov tsos mob ntsig txog lub paj hlwb sab nraud (xws li cov leeg tsis muaj zog thiab tuag tes tuag taw) tuaj yeem tshwm sim ntau zaus, thiab kev puas tsuaj mus tas li tuaj yeem tshwm sim tom qab muaj kev tawm tsam hnyav. Kev sib sau ua ke ntawm cov porphyrin precursors no kuj tseem tuaj yeem ua rau muaj kev puas tsuaj mus sij hawm ntev rau qee lub cev. Cov no yuav suav nrog:

• Mob ntshav siab ntev
• Kab mob siab ntev
• Kab mob raum ntev
• Mob qog nqaij hlav thawj zaug
• Kev nyuaj siab thiab kev ntxhov siab

Dab tsi ua rau AHP?

Qhov ua rau AHP yog kev hloov pauv caj ces . Tab sis zoo li kuv tau hais ua ntej, cov tsos mob tsis tshwm sim tam sim ntawd tom qab muaj kev hloov pauv caj ces. Feem ntau, cov tsos mob thawj zaug tshwm sim thaum lub sijhawm laus, nrog rau kev hloov pauv hormonal. Tsis tas li ntawd, qee cov tshuaj, tshuaj yeeb, haus dej cawv ntau dhau, kev txwv calorie hnyav, thiab cov kab mob uas ua rau lub cev ntxhov siab ntau kuj tuaj yeem ua rau nws.

Qhov uas ua rau cov tshuaj no zoo sib xws yog tias lawv ua rau lub siab ua haujlwm tsim cov heme. Txawm li cas los xij, hauv lub siab ntawm ib tus neeg uas muaj AHP, cov khoom seem ntawm kev tsim cov heme (xws li cov porphyrin precursors) tsis tau metabolized kom zoo, uas yog, siv tag. Yog li ntawd, cov porphyrin precursors seem no yuav sib sau ua ke hauv lub siab. Tsuas yog thaum cov no sib sau ua ke mus rau qee qib uas lawv pib cuam tshuam rau lub paj hlwb thiab ua rau muaj cov tsos mob.

Yuav kuaj mob AHP li cas? (Kev kuaj mob)

Rau cov neeg uas muaj AHPKev kuaj mob kom raug yuav nyuaj heev, vim tias cov tsos mob tsis yog ib txwm muaj rau tus mob no thiab tej zaum yuav zoo li tsis muaj feem cuam tshuam. Txawm li cas los xij, tus kws kho mob uas paub txog AHP yuav xav tias muaj AHP yog tias koj mob plab nrog rau cov tsos mob ntawm lub paj hlwb hauv nruab nrab thiab sab nraud. Kev sib xyaw ua ke ntawm peb cov tsos mob no suav tias yog "classic triad" ntawm AHP.

Yog tias tus kws kho mob xav tias AHP, lawv yuav ua ntau qhov kev kuaj kom paub meej.

• Kev kuaj zis: Qhov no ua tau sai thaum muaj mob hnyav. Cov zis yuav zoo li qub thaum koj tsis muaj mob hnyav, tab sis thaum muaj mob hnyav, cov zis yuav qhia txog cov tshuaj porphyrin precursors (PBG thiab ALA) ntau ntxiv. Qhov no tsis yog kev kuaj uas ua rau AHP xwb, tab sis nws yog kev kuaj yooj yim uas tuaj yeem pab cov kws kho mob taw qhia koj mus rau txoj kev raug.
• Kev kuaj noob caj noob ces: Qhov no yog txoj hauv kev zoo tshaj plaws thiab meej tshaj plaws los kuaj mob AHP (tus qauv kub) . Txawm koj muaj tsos mob lossis tsis muaj, nws tuaj yeem lees paub seb koj puas muaj kev hloov pauv noob caj noob ces uas cuam tshuam nrog AHP. Nws kuj tseem tuaj yeem qhia koj tias koj muaj hom AHP twg thiab qhov tsis txaus enzyme hnyav npaum li cas. Qhov no suav nrog kev coj cov ntshav lossis cov qaub ncaug. Koj lub tsev kho mob hauv zos kuj tseem yuav tsum xa cov qauv mus rau chav kuaj mob tshwj xeeb.

Yuav kho AHP li cas?

Kev kho mob rau AHP tsom mus rau kev tiv thaiv thiab tswj kev tawm tsam . Tej zaum koj yuav tsum tau mus pw hauv tsev kho mob thaum muaj kev tawm tsam. Tej zaum koj yuav xav tau ntau hom tshuaj los txo koj cov tsos mob. Thaum lub sijhawm uas koj tsis muaj kev tawm tsam, koj yuav xav tau lwm yam tshuaj los tswj cov yam ua rau koj cov tsos mob hnyav dua. Yog tias koj thiab koj tus kws kho mob ua haujlwm ua ke los txheeb xyuas cov yam ua rau koj mob tshaj plaws, koj txoj kev kho mob yuav zoo dua.

Kev kho mob rau qhov mob hnyav heev:

• Kev txhaj tshuaj Hemin: Yog tias muaj mob hnyav, tus kws kho mob yuav muab tshuaj hemin rau koj txhaj rau hauv cov leeg ntshav. Qhov no pab txo cov porphyrin hauv koj cov ntshav. Hemin yog ib yam khoom uas tau los ntawm cov qe ntshav liab thiab txo cov porphyrin hauv koj lub cev.
• Kev txo qhov mob: Thaum muaj mob hnyav, tej zaum yuav xav tau cov tshuaj tua mob muaj zog xws li opioids.
• Phenothiazines: Cov no yog siv los tswj kev xeev siab thiab ntuav hnyav.
• Cov kua dej IV thiab kev noj haus: Cov tsos mob xws li mob plab, xeev siab, ntuav, raws plab, thiab cem quav tuaj yeem ua rau lub cev poob calories thiab dej thaum muaj kev tawm tsam. AHP kuj tseem tuaj yeem ua rau poob cov khoom xws li sodium thiab magnesium. Yog li ntawd, cov kua dej uas muaj carbohydrates thiab electrolytes tuaj yeem muab rau hauv cov hlab ntsha.
• Cov tshuaj kho mob qaug dab peg:Kwv yees li ntawm 20% ntawm cov neeg mob yuav xav tau kev kho mob rau tus kab mob qaug dab peg thaum lub sijhawm tawm tsam.

Cov kev xaiv kho mob mus sij hawm ntev:

• Tshuaj hemin tiv thaiv: Kev muab tshuaj hemin tsawg tsawg ib zaug ib lub lim tiam rau cov neeg uas muaj mob ntau zaus tuaj yeem tiv thaiv kev sib sau ua ke ntawm porphyrins.
• Givosiran (GIVLAARI®): Qhov no yog ib hom kev kho mob gene . Nws txo cov porphyrin precursors. Tam sim no nws tau pom zoo ua ib qho kev txhaj tshuaj txhua hli los tiv thaiv cov tsos mob AHP rau cov neeg uas muaj mob hnyav heev.
• Kev Kho Mob Hormone: Yog tias koj qhov kev tawm tsam AHP yog los ntawm koj lub voj voog kev coj khaub ncaws, koj tus kws kho mob yuav sau ntawv rau cov tshuaj xws li GnRH (Gonadotropin-releasing hormone) analogs. Cov no txo ​​​​​​cov tshuaj hormones estrogen thiab progesterone hauv lub cev.
• Tshuaj kho mob ntshav siab: Yog tias muaj ntshav siab ntev, yuav tsum tau tswj nws nrog cov tshuaj tshwj xeeb.
• Kev Hloov Siab: Cov neeg uas muaj mob hnyav heev thiab tsis tau teb rau lwm yam kev kho mob yuav xav txog kev hloov siab. Qhov no tuaj yeem kho tau tus kab mob tag nrho.

Puas tiv thaiv tau AHP?

Nws tsis yog ib qho ua tau los tiv thaiv kom tus kab mob tsis txhob tshwm sim. Txawm li cas los xij, koj tuaj yeem pab tiv thaiv cov tsos mob los ntawm kev zam cov yam ua rau mob . Yog tias koj yeej tsis tau muaj mob, nws yog qhov zoo tshaj plaws kom zam txhua yam uas ua rau mob. Yog tias koj tau muaj mob thiab tau txheeb xyuas cov yam ua rau mob, nws yuav txaus kom zam cov yam ua rau mob ntawd xwb.

Nov yog qee qhov feem ntau pom cov teeb meem ua rau muaj teeb meem no:

Cov hom tshuaj:

• Cov tshuaj Antihistamines (qee cov tshuaj rau mob khaub thuas thiab ua xua)
• Cov tshuaj loog
• Cov tshuaj tiv thaiv kev xeeb tub hauv qhov ncauj
• Kev kho mob hloov tshuaj hormones

Kev siv cov khoom siv:

• Luam yeeb ('Tobacco')
• Tshuaj yeeb
• Cawv
• Cov tshuaj lom zem

Kev Nyuaj Siab:

• Kev kis kab mob
• Kev phais mob
• Kev noj zaub mov tsawg calorie (tshwj xeeb tshaj yog kev noj zaub mov tsawg carbohydrate) ('Calorie deprivation')
• Kev ntxhov siab ntawm lub hlwb

Yav tom ntej rau cov neeg uas muaj AHP yog dab tsi? (Kev kwv yees)

Qhov no txawv ntawm ib tus neeg mus rau ib tus neeg. Muaj coob tus neeg yeej tsis muaj tsos mob . Ntawm cov neeg uas muaj, feem ntau cov neeg tsuas muaj ib zaug lossis ob peb zaug xwb hauv lawv lub neej. Txawm hais tias kev tawm tsam tuaj yeem ua rau tuag taus, feem ntau cov neeg rov zoo tag nrho nrog kev kho mob sai . Kwv yees li 5% ntawm cov neeg uas muaj AHP muaj cov tsos mob ntau zaus lossis ntev. Rau cov neeg no, cov tshuaj tiv thaiv feem ntau pab tau, thiab kev hloov siab yuav raug suav hais tias yog qhov kawg.

Kuv yuav saib xyuas kuv tus kheej li cas thaum kuv tseem muaj AHP?

• Tsis txhob noj tej yam uas ua rau koj mob. Cawv, haus luam yeeb, thiab siv yeeb tshuaj yuav tsum yog tej yam uas koj yuav tsum tsis txhob noj. Tej zaum koj yuav xav nrog koj tus kws kho mob tham txog lwm yam tshuaj uas koj noj.
• Noj zaub mov kom zoo thiab sib npaug. Tsis txhob noj zaub mov ntau dhau thiab noj zaub mov uas tsis muaj carbohydrate ntau. Yog tias koj yoo mov ua ntej mus kuaj mob lossis phais mob, nrog koj tus kws kho mob tham txog qhov no.
• Mus kuaj mob tsis tu ncua. Txawm tias koj tsis muaj tsos mob los xij, koj tus kws kho mob yuav kuaj koj lub siab, lub raum, thiab ntshav siab tsis tu ncua.

Kab mob siab porphyria yog ib yam kab mob tsis tshua muaj, yog li ntawd, kev paub txog nws ntawm cov pej xeem feem ntau tsis tshua muaj. Thaum muaj kev tawm tsam sai sai nrog cov tsos mob hnyav, nws ua rau tsis meej pem thiab ntshai heev. Ib txhia neeg siv sijhawm ntau xyoo los sim xav seb muaj dab tsi tsis zoo rau lawv thiab lawv tuaj yeem ua dab tsi. Yog tias koj xav tias koj muaj AHP, kev kuaj noob caj noob ces tuaj yeem lees paub nws. Thaum koj paub, nws yuav yog ib qho kev pab zoo rau koj los tiv thaiv kev tawm tsam thiab tswj lawv.

Cov ntsiab lus tseem ceeb tshaj plaws uas koj yuav tsum paub (Cov Lus Qhia Coj Mus Tsev)

Zoo, yog li cia kuv sau qee yam uas koj yuav tsum nco ntsoov los ntawm qhov peb tau tham txog:

• AHP yog ib yam kab mob caj ces tsis tshua muaj uas pib hauv lub siab thiab cuam tshuam rau lub paj hlwb .
• Qhov no yog vim muaj qhov tsis txaus ntawm cov enzymes uas xav tau hauv cov txheej txheem ntawm kev ua heme , uas ua rau cov tshuaj lom hu ua porphyrin precursors kom sib sau ua ke hauv lub cev.
• Cov tsos mob yuav txawv, xws li mob plab tsis sib xws, mob hlab ntsha, teeb meem kev puas siab puas ntsws, teeb meem ntawm daim tawv nqaij, thiab txawm tias hloov xim ntawm koj cov zis.
• Txawm tias muaj kev hloov pauv caj ces los xij, cov tsos mob tsis tshua tshwm sim yam tsis muaj cov tshuaj ua rau ( xws li cov tshuaj hormones, qee yam tshuaj, cawv, thiab kev ntxhov siab).
• Kev kuaj zis thiab kev kuaj noob caj noob ces yog qhov tseem ceeb rau kev kuaj mob.
• Qhov tseem ceeb tshaj plaws yog kev tswj thiab tiv thaiv kev tawm tsam los ntawm kev kho mob. Siv tshuaj tiv thaiv kab mob 'Hemin', tshuaj tua kab mob, thiab qee cov tshuaj tshwj xeeb. Hauv cov xwm txheej hnyav tshaj plaws, kev hloov siab kuj yog ib qho kev daws teeb meem.
• Koj tuaj yeem ua neej zoo nrog tus kab mob los ntawm kev zam cov teeb meem uas ua rau koj mob, ua neej noj qab nyob zoo, thiab mus kuaj mob tsis tu ncua .

Vam tias cov ntaub ntawv no yuav pab tau koj. Nco ntsoov, yog tias koj muaj ib qho ntawm cov tsos mob no, tsis txhob ntshai mus ntsib kws kho mob thiab tham txog nws.

Kab mob siab porphyria mob hnyav, AHP, daim siab, lub paj hlwb, cov kab mob caj ces, porphyrins, cov tsos mob, kev kho mob