Cutar jini wacce ke sanadin tabin hankali

 

Porphyria
Left figure is urine on the first day while the right figure is urine after three days of sun exposures showing the classic change in color to purple.
Specialty	Endocrinology
Symptoms	Depending on subtype—abdominal pain, chest pain, vomiting, confusion, constipation, fever, seizures, blisters with sunlight
Usual onset	Recurrent attacks that last days to weeks
Causes	Usually genetic
Diagnostic method	Blood, urine, and stool tests, genetic testing
Differential diagnosis	Lead poisoning, alcoholic liver disease
Treatment	Depends on type and symptoms
Frequency	1 to 100 in 50,000 people

Porphyria rukuni ne na cututtuka wanda abubuwan da ake kira porphyrins ke haɓakawa, suna cutar da fata ko tsarin juyayi . Nau'in da ke shafar tsarin jijiyoyi kuma ana san su da m porphyria, kamar yadda alamun suna da sauri a farkon farawa da gajeren lokaci. Alamomin harin sun hada da ciwon ciki, ciwon kirji, amai, rudewa, maƙarƙashiya, zazzabi, hawan jini, da hawan jini . Hare-haren kan wuce zuwa makonni. Matsalolin na iya haɗawa da inna, ƙananan matakan sodium na jini, da kamewa . Ana iya haifar da hare-hare ta barasa, shan taba, canjin hormonal, azumi, damuwa, ko wasu magunguna. Idan fata ta shafi, blisters ko ƙaiƙayi na iya faruwa tare da hasken rana.

Mafi yawan nau'in porphyria ana gadonsu daga daya ko duka biyu na iyayen mutum kuma suna faruwa ne sakamakon maye gurbin daya daga cikin kwayoyin halittar da ke yin heme . Ana iya gadar su a cikin mafi rinjaye na autosomal, recessive autosomal, ko kuma mai alaƙa da X. Nau'i ɗaya, porphyria cutanea tarda, na iya zama saboda ƙarar ƙarfe a cikin hanta, hepatitis C, barasa, ko HIV / AIDs . Tsarin da ke ciki yana haifar da raguwa a cikin adadin heme da aka samar da kuma haɓaka abubuwan da ke tattare da yin heme. Hakanan ana iya rarraba Porphyrias ta hanyar hanta ko marrow na kashi . Ana yin ganewar asali ta hanyar gwajin jini, fitsari, da stool. Ana iya yin gwajin kwayoyin halitta don tantance takamaiman maye gurbi.

Jiyya ya dogara da nau'in porphyria da alamun mutum. Maganin ciwon kai na fata gabaɗaya ya haɗa da nisantar hasken rana, yayin da jiyya ga rashin ƙarfi na porphyria na iya haɗawa da ba da heme na jini ko maganin glucose . Da wuya, ana iya yin dashen hanta .

Madaidaicin mitar porphyria ba a sani ba, amma an kiyasta zai shafi tsakanin 1 zuwa 100 a cikin mutane 50,000. Farashin ya bambanta a duniya. An yi imanin Cutar jini wacce ke sanadin tabin hankali cutanea tarda shine nau'in da ya fi kowa. An kwatanta cutar a farkon 370 BC ta Hippocrates . Masanin kimiyyar ilmin lissafi na Jamus Felix Hoppe-Seyler ya fara bayanin tsarin da ke ƙarƙashinsa a cikin 1871. Sunan gwagwalad porphyria ya fito ne daga Girkanci πορφύρα, porphyra, ma'ana " purple ", nuni ga launi na fitsari wanda zai iya kasancewa a lokacin. hari.