Maple syrup cutar fitsari (MSUD) wani cututtukan kwayoyin halitta ne da ke haifar da lalacewar kwakwalwa da ci gaba da juyayi tsarin tsarin. Halittar kwayoyin da ke haifar da sakamako na MSUD a cikin lahani a cikin enzyme da ake kira alpha-keto acid dehydrogenase, (BCKD), wanda ya zama dole don raunin amino acid leucine, isoleucine, da valine. Idan ba tare da BCKD enzyme ba, waɗannan amino acid sun bunkasa matakai masu guba a jiki.
MSUD ta samo sunansa daga gaskiyar cewa, a wasu lokuta lokacin da jinin amino acid ya yi tsawo, da fitsari yana ɗauke da ƙanshi mai ban sha'awa.
Maple syrup cutar fitsari yana faruwa a kimanin 1 cikin 180,000 haihuwar haihuwa kuma yana shafar maza da mata. MSUD tana shafar mutane daga dukan kabilanci, amma yawancin ƙwayoyin cuta na faruwa a cikin al'ummomin da akwai matakan auren da yawa, irin su mazaunan Mannonite a Pennsylvania (US).
Cutar cututtuka
Akwai nau'o'in maple syrup mai cutar fitsari. Mafi yawan al'ada (classic) yawanci zai haifar da bayyanar cututtuka a jariri jarirai masu shekaru 4 zuwa 7. Wadannan bayyanar cututtuka sun haɗa da:
- Poor ciyar
- Vomiting
- Matsalar nauyi mara kyau
- Ƙara lethargy (wuya a farka)
- Halin ya ƙone sukari don fitsari
- Canje-canje a cikin sautin tsoka, ƙwayoyin tsoka, da kuma kama
Idan aka bari ba a yi musu ba, waɗannan yara za su mutu tare da farkon watanni na rayuwa.
Mutanen da ke tare da MSUD, na biyu mafi yawan al'amuran irin wannan cuta, sukan ci gaba amma amma idan baza su nuna alamun da aka yi ba.
Matsakaici MSUD wata siffar da ta fi dacewa. Kowane mutum da irin wannan yana da kashi 3-30% na al'ada na al'ada na BCKD, saboda haka alamar cututtuka na iya farawa a kowane zamani.
A cikin sakonni na MSUD, mutane suna nuna wasu ci gaba idan aka ba da matakan thiamine .
Wani mummunan nau'i na rashin lafiya shi ne E3-raunana MSUD, wanda mutane ke da ƙarin rashin ƙarfi a cikin enzymes.
Sanin asali
Idan an yi la'akari da cutar suturar syrup ta hanyar zubar da jini ta jiki, musamman ma alamar wariyar fitsari, za a iya gwada gwajin jini don amino acid. Idan an gano alloisoleucine, an tabbatar da ganewar asali. Ana gudanar da nuni na jariri na MSUD a cikin jihohin da dama a Amurka
Jiyya
Babban maganin maple syrup furotin ƙwayar cuta shine ƙuntatawa da nau'i na abincin abincin na amino acid uku leucine, isoleucine, da valine. Wadannan ƙuntataccen abincin na dole ne ya zama rayuwa. Akwai samfurori da dama da yawa da kuma abinci ga mutane tare da MSUD.
Ɗaya daga cikin damuwa game da maganin MSUD shi ne cewa idan mutum yana da lafiya, ya ji rauni, ko yana da tiyata, cutar ta kara tsanantawa. Yawancin mutane za su buƙaci asibiti a lokacin waɗannan lokuta don kula da lafiyar don hana ƙwayar matsala.
Abin farin ciki, tare da biyan ƙuntataccen abincin da ake amfani da su da kuma likita na yau da kullum da mutanen da ke fama da cutar fitsari na syrup za su iya rayuwa da dogon lokaci.
Sources:
Bodamer, OA (2003). Maple syrup cutar fitsari. eMedicine.
Morton DH, Strauss KA, Robinson DL, et al. Sanin asali da kuma maganin cutar maple syrup: nazari kan marasa lafiya 36. Ilimin yara na yara 2002; 109: 999.