Porphyria

Porphyrias rukuni ne na cututtukan gado da ba a san su ba. Wani muhimmin bangare na haemoglobin, wanda ake kira heme, ba a yin shi da kyau. Hemoglobin wani furotin ne a cikin jajayen ƙwayoyin jini wanda ke ɗauke da iskar oxygen. Hakanan ana samun Heme a cikin myoglobin, sunadarin da ke cikin wasu tsokoki.

A yadda aka saba, jiki yana sanya shi a cikin tsari mai matakai da yawa. Ana yin Porphyrins yayin matakai da yawa na wannan aikin. Mutanen da ke fama da cutar porphyria ba su da wasu ƙwayoyin enzymes da ake buƙata don wannan aikin. Wannan yana haifar da yawan sinadarin jujjuyawar jiki ko kuma wasu sinadarai masu dangantaka cikin jiki.

Akwai siffofin daban-daban na porphyria. Mafi yawan nau'ikan nau'ikan shine porphyria cutanea tarda (PCT).

Magunguna, kamuwa da cuta, giya, da kuma sinadarai irin su estrogen na iya haifar da hare-hare na wasu nau'ikan cutar sankara.

An gaji Porphyria. Wannan yana nufin cewa cutar ta shiga cikin dangi.

Porphyria tana haifar da manyan alamu guda uku:

• Ciwon ciki ko ƙyama (kawai a wasu nau'ikan cutar)
• Hankali ga haske wanda zai iya haifar da rashes, blistering, da tabo na fata (photodermatitis)
• Matsaloli tare da tsarin jijiyoyi da tsokoki (kamuwa, rikicewar hankali, lalacewar jijiya)

Hare-hare na iya faruwa ba zato ba tsammani. Sau da yawa sukan fara ne da tsananin ciwon ciki mai biyo baya da amai da maƙarƙashiya. Kasancewa a cikin rana na iya haifar da ciwo, jin zafi, blister, da jan fata da kumburi. Fusho suna warkarwa a hankali, galibi tare da tabo ko canjin launin fata. Scaryallen tabo na iya zama wata illa. Fitsari na iya zama ja ko ruwan kasa bayan hari.

Sauran cututtukan sun hada da:

• Ciwon tsoka
• Raunin jijiyoyi ko nakasa jiki
• Nutsawa ko kunci
• Jin zafi a hannaye ko ƙafa
• Jin zafi a baya
• Yanayin mutum yana canzawa

Hare-hare a wasu lokuta na iya zama barazanar rai, suna haifar da:

• Pressureananan hawan jini
• Matsanancin rashin daidaiton lantarki
• Shock

Mai ba da lafiyar ku zai yi gwajin jiki, wanda ya haɗa da sauraron zuciyar ku. Kuna iya samun bugun zuciya mai sauri (tachycardia). Mai ba da sabis ɗin na iya gano cewa ƙwanƙwasawar jijiyar ka (gwiwa ko wasu) ba sa aiki da kyau.

Gwajin jini da fitsari na iya bayyana matsalolin koda ko wasu matsaloli. Wasu daga cikin sauran gwaje-gwajen da za'a iya yi sun haɗa da:

• Iskar gas
• M rayuwa panel
• Matakan Porphyrin da matakan wasu sunadarai masu alaƙa da wannan yanayin (ana duba shi cikin jini ko fitsari)
• Duban dan tayi
• Fitsari

Wasu daga cikin magungunan da ake amfani dasu don magance saurin kamuwa da cutar porphyria na iya haɗawa da:

• Hematin da aka bayar ta jijiya (intravenously)
• Maganin ciwo
• Propranolol don sarrafa bugun zuciya
• Magungunan kwantar da hankali don taimaka maka samun nutsuwa da rashin damuwa

Sauran jiyya na iya haɗawa da:

• Abubuwan Beta-carotene don rage tasirin hoto
• Chloroquine a cikin ƙananan allurai don rage matakan porphyrins
• Ruwan ruwa da glucose don haɓaka matakan carbohydrate, wanda ke taimakawa iyakance samar da sinadarin porphyrins
• Cire jini (phlebotomy) don rage matakan porphyrins

Ya danganta da nau'in porphyria da kake da shi, mai ba ka sabis na iya gaya maka:

• Guji duk giya
• Guji magunguna waɗanda zasu iya haifar da hari
• Guji cutar da fata
• Guji hasken rana gwargwadon iko kuma a yi amfani da fuska a yayin waje
• Ku ci abinci mai-carbohydrate

Wadannan albarkatu na iya ba da ƙarin bayani game da porphyria:

• Gidauniyar Amurka Porphyria - www.porphyriafoundation.org/for-patients/patient-portal
• Cibiyar Nazarin Ciwon Suga ta Duniya da Cututtukan narkewar abinci da Koda - www.niddk.nih.gov/health-information/liver-disease/porphyria
• Nationalungiyar forasa don Rare Rashin Lafiya - rarediseases.org/rare-diseases/porphyria

Porphyria cuta ce mai tsawon rai tare da alamun bayyanar cututtuka waɗanda ke zuwa da tafiya. Wasu nau'ikan cutar suna haifar da alamun bayyanar fiye da wasu. Samun magani mai kyau da nisantar abubuwan da ke haifar da hakan na iya taimakawa tsawan lokacin tsakanin hare-hare.

Matsaloli na iya haɗawa da:

• Coma
• Duwatsu masu tsakuwa
• Shan inna
• Rashin numfashi (saboda rauni na tsokoki na kirji)
• Satar fata

Nemi taimakon likita da zaran ka sami alamun mummunan hari. Yi magana da mai ba ka sabis game da haɗarinka ga wannan yanayin idan kana da dogon tarihin rashin ciwon ciki da ba a gano shi ba, matsalolin tsoka da jijiyoyi, da ƙwarewar hasken rana.

Bayar da shawara game da kwayar halitta na iya amfanar da mutane da ke son haihuwar yara kuma waɗanda ke da tarihin iyali na kowane irin cutar sankara.

Porphyria cutanea tarda; Interananan porphyria; Kwayar cutar gado; Hanyar erythropoietic porphyria; Ciwon kwayar cutar Erythropoietic

• Porphyria cutanea tarda akan hannayen

Bissell DM, Anderson KE, Bonkovsky HL. Porphyria. N Engl J Med. 2017; 377 (9): 862-872. PMID: 28854095 www.ncbi.nlm.nih.gov/pubmed/28854095.

Ful SJ, Wiley JS. Heme biosynthesis da cuta: porphyrias da sideroblastic anemias. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 38.

Habif TP. Cututtuka masu nasaba da haske da rikicewar launi. A cikin: Habif TP, ed. Clinical Dermatology: Jagoran Launi don Bincikowa da Far. Na 6 ed. Philadelphia, PA: Elsevier; 2016: babi na 19.

Hift RJ. A porphyrias. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: babi na 210.