I-Porphyria (iVampire Syndrome): oonobangela, iimpawu, isifo, isifo, izinto ezinobungozi kunye nonyango

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Isifo sevampire sisifo esinqabileyo semfuza segazi esihlala sichaphazela ulusu kunye nenkqubo yovalo. Ngokwezonyango, yaziwa ngokuba yi-porphyria ^[1] . Le meko ibizwa ngokuba yi-'vampire 'ngenxa yeempawu zayo ezifanayo nevampire yasentsomini yenkulungwane ye-18.

I-Porphyria yafunyanwa kwakudala, ngaphambi kokuba kwenziwe i-antibiotics, ucoceko kunye nefriji. Ngezo ntsuku, abantu abanale meko babethathwa njenge-'vampire 'ngenxa yeempawu zabo ezinje nge-vampire ezibandakanya amazinyo, imijikelezo emnyama ejikeleze amehlo, umchamo obomvu kunye nokungakhathaleli ukukhanya kwelanga. Nangona kunjalo, le meko yafundwa kamva ziingcali zonyango kwaye unyango lwayo lwayilwa ^[Mbini] .

Iithiyori zesayensi emva kwePorphyria

Ngokuka-Desiree Lyon Howe, umseki weAmerican Porphyria Foundation kunye nogulayo we-porphyria ebukhali, esi sifo sinqabile sasixhaphakile phakathi kwabantu base-Yurophu ngexesha eliphakathi xa abantu babehlala kude nonxibelelwano lwala maxesha. umhlaba ^[25] .

URoger Luckhurst, unjingalwazi kwiNcwadi yaMhlanje neyangoku (eLondon) kunye nomhleli wencwadi 'iDracula' ebhalwe nguBram Stoker wakhankanya izinto ezininzi kunye neziganeko ezinoxanduva lwe-porphyria kwii-1730s. Uye wakhankanya ukuba ngexesha lamaxesha aphakathi, intlekele yabetha kwiindawo ezikude zaseYurophu yabangela indlala, isibetho kunye nezifo ezininzi ezinje ngecatalepsy (ukuqina komzimba kunye nokulahleka kwemvakalelo) ^[26] .

Ngenxa yokungabikho konxibelelwano nehlabathi langaphandle kunye nokungonelanga kwamayeza, abantu abane-porphyria bakhubazeka ngokwengqondo ngenxa yoloyiko, uxinzelelo kunye nezinye izinto kwaye baqala ukuzitya ngokwabo ngenxa yendlala enkulu. Kananjalo, ngenxa yokungaziwa kogonyo lwale mihla kunye neziyobisi, izifo ezibangelwe kukulunywa zizilwanyana njenge-rabies zisasazeke ngesixa esikhulu ngexesha ebangele ukuba baphike amanzi kunye nokukhanya, ukubona izinto ezingekhoyo kunye nobundlongondlongo.

Esinye isizathu, njengoko kuchaziwe nguNjingalwazi Roger Luckhurst, sibonisa ukuba njengoko abahlali baseYurophu bahlala bodwa ixesha elide, kwabangela ukungondleki ngenxa yokutya okungalunganga kunye nokuchaphazeleka kwizifo ezininzi ngenxa yokuba ii-genes zabo bezinokuthi ziguqukele ekwenzeni i-vampire- njengeempawu.

Njengokuba ixesha lihamba kunye nemitshato, ukungaqheleki kofuzo kudlula kubazali kuye ebantwaneni kwaye kwabangela ukusasazeka kwale meko.

Unobangela wePorphyria

Emntwini, ioksijini evela emiphungeni idluliselwa kwamanye amalungu omzimba ngeprotein ekhethekileyo kwiiseli ezibomvu zegazi ezibizwa ngokuba yihemoglobin ekwanoxanduva lombala obomvu wegazi. I-Hemoglobin iqulethe iqela lokufakelwa elibizwa ngokuba yi-heme eliquka i-porphyrin kunye ne-iron-ion kwiziko. Ihlala yenziwe kwiiseli ezibomvu zegazi, umongo wethambo kunye nesibindi.

I-Heme yenziwa ngamanqanaba asibhozo ngokulandelelana nganye yi-enzyme eyahlukileyo eveliswa yi-porphyrin. Ukuba kukho naliphi na kula manyathelo asibhozo asilelayo ngexesha lokwakha i-heme ngenxa yokutshintsha kwemfuza okanye ityhefu yokusingqongileyo, ukudityaniswa kwee-enzymes kuyaphazamiseka kubangele ukusilela kwayo kwaye kukhokelele kwi-porphyria. Kukho iintlobo ezahlukeneyo ze porphyria kwaye imeko inxulunyaniswa nohlobo lwe-enzyme engekhoyo ^[3] .

Iindidi zePorphyria

Kukho iindidi ezi-4 ze-porphyria apho ezimbini zibonakaliswa ziimpawu zayo kwaye ezi zimbini zokugqibela zahlulwe yipathophysiology.

1. I-porphyria esekwe kwimpawu

• I-porphyria efanelekileyo (AP): Le meko isongela ubomi ibonakala ngokukhawuleza kwaye ichaphazela inkqubo yeemvakalelo. Iimpawu ze-AP zihlala iveki enye okanye ezimbini kwaye emva kokubonakala kwazo, iimpawu ziqala ukuphucula. I-AP ayifane yenzeke ngaphambi kokufikisa nasemva kokuyeka ukuya exesheni ^[4] .
• I-porphyria esisikiweyo (CP): Zahlulahlulwe kakhulu zaziindidi ezi-6 kwaye uhlobo ngalunye lubonisa iimeko ezinxulumene nezifo eziqatha zolusu njengokuqaqamba kwelanga, iblisters, i-edema, ubomvu, amanxeba kunye nokwenza mnyama kolusu. Iimpawu ze-CP ziqala ngexesha lobuntwana ^[5] .

2. IPathophysiology esekwe kwi-porphyria

• I-Erythropoietic porphyria: Ubonakaliswe ngokuveliswa kakhulu kweporfyrin, ngakumbi kumongo wethambo ^[6] .
• I-hepatic porphyria Iphawuleka ngokuveliswa kakhulu kweeporphyrins esibindini ^[7] .

Iimpawu zePorphyria

Iimpawu ze-porphyria ngokweentlobo zazo zezi zilandelayo.

I-porphyria efanelekileyo

• Ukudumba kunye neentlungu ezibuhlungu esiswini
• Ukuqunjelwa, ukugabha okanye urhudo
• Ukubetha kwentliziyo
• Iimeko zengqondo ezinje ngoxinzelelo, ukubona izinto ezingekhoyo, okanye iparanoia ^[8]
• Ukuphuthelwa
• Ukuxhuzula ^[8]
• Umchamo obomvu okanye omdaka ^[9]
• Ubuhlungu bemisipha, ubuthathaka, ukuba ndindisholo okanye ukukhubazeka
• Uxinzelelo lwegazi

I-porphyria yesikhumba

• Ukujonga ngaphezulu kwelanga ^[10]
• Intlungu evuthayo ekukhanyeni kwelanga okanye ukukhanya okungekuko
• Ukudumba okubuhlungu kolusu
• Ubomvu besikhumba
• Amabala kunye nolusu ^[10]
• Ukwanda kokukhula kweenwele
• Amadyungudyungu avela kwizikrelemnqa ezincinci
• Umchamo o-Blue-tinged
• Ukukhula okungaqhelekanga ebusweni ^{[Shumi elinanye]}
• Ubumnyama besikhumba esivelileyo
• Ukuqaqamba kwesikhumba okukhokelela ekuveleni kwamazinyo afana namazinyo kunye nemilebe ebomvu.

Imiba yomngcipheko wePorphyria

Xa i-porphyria ifunyenwe, ikakhulu ngenxa yeetyhefu zokusingqongileyo ezibangela iimpawu ze-vampirism. Zezi zilandelayo:

• Ukukhanya kwelanga ^[1]
• Ukutya igalikhi okanye ukutya okusekwe kwigalikhi ^[12]
• Amayeza eHormonal anje ngehomoni yokuya exesheni
• Ukutshaya ^[13]
• Uxinzelelo lomzimba okanye lweemvakalelo ^[14]
• Usulelo
• Ukuhlukunyezwa kwesiyobisi
• Ukutya okanye ukuzila ukutya
• Iziyobisi ezinje ngeepilisi zokulawula ukuzalwa okanye iziyobisi ezisebenza ngengqondo
• Ukuqokelelwa kakhulu kwentsimbi emzimbeni ^{[Shumi elinantlanu]}
• Isifo sesibindi

Iingxaki zePorphyria

Iingxaki ze-porphyria zezi zilandelayo:

• Ukusilela kwezintso ^[16]
• Umonakalo osisigxina wolusu ^[5]
• Umonakalo wesibindi
• Ukuphelelwa ngamandla emzimbeni ^[4]
• Hyponatremia, isodiyam ephantsi emzimbeni
• Iingxaki zokuphefumla ezinzima ^[4]

Ukuchongwa kwePorphyria

I-Porphyria ngamanye amaxesha kunzima ukuyifumana njengoko iimpawu zayo zifana nesifo sikaGuillain-Barre. Nangona kunjalo, ukuxilongwa kwenziwa ngolu vavanyo lulandelayo:

• Uvavanyo lwegazi, umchamo kunye nokugcina isitulo: Ukufumana iingxaki zezintso kunye nesibindi kunye nohlobo kunye nenqanaba le-porphyrins emzimbeni ^[17] .
• Uvavanyo lwe-DNA: Ukuqonda unobangela wokutshintsha kwemfuza ^[18] .

Unyango lwePorphyria

Unyango lwe-porphyria lusekwe kwiindidi zalo. Zezi zilandelayo:

• Amayeza afakwa ngaphakathi: IHematin, iswekile kunye namanye amayeza alulwelo anikwa ngaphakathi ukuze kugcinwe amanqanaba ehemme, iswekile kunye nolwelo emzimbeni. Unyango lwenziwa ikakhulu kwi-porphyria AP ebukhali ^[4] .
• Iphlebotomy: Kwi-CP, inani elithile legazi liyarhoxiswa kwimithambo yomntu ukunciphisa inqanaba lesinyithi emzimbeni ^[19] .
• Beta iziyobisi zeCarotene: Ukuphucula ukunyamezelana kolusu kwilanga ^{[Amashumi amabini]} .
• Amayeza okugoma: Iziyobisi ezinjengehydroxychloroquine kunye ne-chloroquine, ezisetyenziselwa ukunyanga iimpawu ze-malaria zisetyenziselwa ukufunxa inani eligqithisileyo le-porphyrins emzimbeni ^{[amashumi amabini ananye]} .
• Vitamin D ezongezelelweyo: Ukuphucula iimeko ezibangelwa kukunqongophala kukavithamini D ^[22] .
• Ukutshintshwa komongo wethambo: Ukuveliswa kweeseli zegazi ezintsha nezisempilweni emzimbeni ^{[2. 3]} .
• Ukufakelwa kweseli: Oku kwenziwa ngokusebenzisa intambo yegazi engumthombo otyebileyo weeseli zeziqu kunomongo wethambo ^[24] .

Iingcebiso zokujongana nePorphyria

• Nxiba izixhobo zokukhusela xa ungaphandle elangeni.
• Gwema iziyobisi okanye utywala ukuba unayo i-porphyria.
• Ungayityi igalikhi kuba inokubangela iimpawu zale meko ^[12] .
• Yeka ukutshaya ^[13]
• Musa ukuzila ukutya ixesha elide njengoko kunokukhokelela kunqongophalo lwezakhamzimba ezithile emzimbeni.
• Yenza ukucamngca okanye i-yoga ukunciphisa uxinzelelo.

Ukuba ufumana usulelo, lunyange ngokukhawuleza.

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