Ese uterwa ikinya n'imiti igabanya ububabare? Reka twige ku bijyanye n'(Pseudocholinesterase Deficiency)?

Ese wigeze wumva ufite ubwoba gato ku bijyanye n'ikinya uhabwa mu gihe cyo kubagwa? Ni ibisanzwe ko hari abantu bumva bameze batyo. Ariko tekereza uko byagenda iyo imwe mu miti igabanya ikinya uhabwa itari ikwiriye umubiri wawe. Uyu munsi tugiye kuvuga ku kibazo kimwe kidasanzwe, ariko cy'ingenzi cyane ugomba kumenya. Iyo ni indwara yitwa `(Pseudocholinesterase Deficiency)`.

`(Kubura Pseudocholinesterase)` ni iki? Reka tubisobanukirwe mu buryo bworoshye, si byo se?

Yego, iri zina rirumvikana nk'irirerure gato, si byo se? Reka tubikomeze byoroshye. `(Pseudocholinesterase)` ni enzyme yihariye `(Enzyme)` mu mubiri wacu. Imwe mu nshingano z'ingenzi z'iyi `(Enzyme)` ni ugusenya (ni ukuvuga `(metabolize)`) mu mubiri imwe mu miti itangwa kugira ngo iruhure imitsi , cyane cyane iyo duhawe ikinya (tuyita `(Choline esters)`). Tekereza, mu gihe cyo kubagwa, imitsi yawe igomba guhagarara igihe runaka. Iyi ni yo miti itangwa kuri ibyo.

Noneho, mu gihe cy’indwara yitwa `(Pseudocholinesterase deficiency)`, ikibaho ni uko iyi `(Enzyme)` yitwa `(Pseudocholinesterase)` mu mubiri wawe iba idakozwe bihagije , cyangwa idakora neza . None se bigenda bite? Imiti itangwa kugira ngo iruhure imitsi (`(Succinylcholine)` na `(Mivacurium)` ni ingero ebyiri z’ibi) ntikurwa mu mubiri vuba. Iguma mu mubiri igihe kirekire.

Kubera iyo mpamvu, imitsi yawe ishobora kugumana ibinya igihe kirekire nyuma yo gusinzira, ndetse ushobora no kugira ikibazo cyo guhumeka. Ibi bivuze ko ushobora kudashobora kwimuka cyangwa guhumeka wenyine kugeza igihe ingaruka z'imiti zirangiye.

Iyi ndwara ikunze kugaragara gute?

Mu by’ukuri, iyi ndwara yitwa `(Pseudocholinesterase Deficiency)` ntabwo ikunze kugaragara cyane . Ugereranyije, umuntu umwe gusa kuri 3.200 cyangwa 5.000 mu baturage ni we uhura n’iyi ndwara. Ni ukuvuga umubare muto cyane.

Ibimenyetso ni ibihe? Ubimenya ute?

Abantu benshi bafite iyi ndwara ntibabimenya kugeza igihe bakiriye ubwoko bw'umuti utera ikinya navuze mbere, witwa ``Choline esters.'' Ni bwo ibimenyetso by'ingenzi bigaragara:

• Imitsi iruhutse cyane (ni ukuvuga idafite ubuzima) igihe kirekire kurusha uko byari bisanzwe byitezwe.
• Ndetse n'imitsi igufasha guhumeka iba idakora by'agateganyo .

Bishobora gufata amasaha make kugira ngo ibi bimenyetso bishire. Iki gihe gitandukanye bitewe n'umuntu ku giti cye. Muri iki gihe, itsinda ry'abaganga rizagukomeza munsi y'ikinya kandi rigufashe guhumeka binyuze muri "ventilator" y'ubukorano, kugeza igihe uzabasha guhumeka wenyine.

Impamvu ni izihe? Ibi bibaho bite?

Iyi ndwara (Pseudocholinesterase Deficiency) ishobora kuragwa (ni ukuvuga kuragwa), cyangwa ishobora no kugaragara nyuma y'ubuzima..

Impamvu zarazwe

Abantu bavukanye iyi ndwara bagira impinduka, cyangwa impinduka, muri gene yitwa `(BCHE)`. Iyi gene `(BCHE)` niyo ibwira umubiri wacu gukora enzyme `(Pseudocholinesterase)`. Rero, iyo habayeho impinduka muri iyi gene, umubiri wawe ukora bike cyane muri iyi enzyme, cyangwa ushobora kutayikora na gato.

Impamvu zibaho mu buzima (ziboneka)

Hari igihe iyi ndwara (Pseudocholinesterase Deficiency) ishobora kubaho mu buzima bwose. Ishobora guterwa n'indwara zitandukanye n'imiti imwe n'imwe.

Zimwe mu ndwara zishobora gutera iyi ndwara:

• Imirire mibi
• Ibikomere bikomeye byo gushya
• Indwara y'impyiko
• Indwara y'umwijima
• Hypothyroidism (tiyode idakora neza)
• Kanseri
• Gutwita

Imiti imwe ishobora kongera ibyago byo kurwara iyi ndwara irimo:

• MAO inhibitor (urugero: Phenelzine (Nardil®))
• Ibinini byo kuboneza urubyaro (imiti yo kuboneza urubyaro inyobwa)
• Ibitonyanga by'amaso bya Echothiophate
• Metoclopramide
• Cyclofosfamide
• Piridostigmine

Abaganga bapima iki kibazo gute?

Niba ufite ikibazo cyo kugenzura imitsi yawe cyangwa guhumeka nyuma yo gusinziriza indwara rusange, muganga wawe ashobora gukeka ko hari ikibazo cyo kubura pseudocholinesterase. Kugira ngo byemezwe, bashobora kugupima amaraso kugira ngo barebe urugero rwa pseudocholinesterase muri enzyme yawe.

Niba hari umuntu wo mu muryango wawe ufite iyi ndwara, muganga ashobora gukora isuzuma ry’uturemangingo kugira ngo amenye niba nawe uyifite. Ibi bisaba gufata ikizamini cy’amaraso no gusuzuma niba ufite ihinduka ry’uturemangingo (BCHE).

Niba umuganga wawe akeka ko ufite iyi ndwara, azakoresha ubundi bwoko bw'imitsi iruhura imitsi idashingiye kuri enzyme ``Pseudocholinesterase''.

Uburyo bwo kuvura ni ubuhe?

Niba ugaragaje ibimenyetso bya `(Pseudocholinesterase Deficiency)` nyuma yo guhabwa ikinya rusange, ushobora gukenera ubuvuzi bwihuse. Itsinda ry’abaganga rizagukurikiranira hafi kugeza ukize. Nibiba ngombwa, bazagukomeza munsi y’ikinya kandi bakoreshe imashini ya ``mechanical ventilation`` kugira ngo igufashe guhumeka kugeza ushoboye guhumeka wenyine.

Ni iki wakwitega niba ufite iyi ndwara?

Niba urwaye indwara ya `(Pseudocholinesterase deficiency)`, ikintu cyiza wakora ni ukumenyesha abaganga bawe ibyayo – cyane cyane abaganga bazagusura kugira ngo ubagwe .

Nanone, ugomba kubwira umuryango wawe ibi., hanyuma bashobora no gupimwa kugira ngo barebe niba bafite `(BCHE)` mutation. Niba ari uko bimeze, bagomba no kubimenyesha abaganga babo mbere yo kubagwa.

Ni byiza kandi kwambara umukandara w'indangamuntu yo kwa muganga kugira ngo abaganga bamenye niba ukeneye kubagwa byihutirwa.

Ese ibi bishobora kwirindwa?

Kubura Pseudocholinesterase ni indwara umuntu avukana idashobora kwirindwa. Ariko, niba hari umuntu wo mu muryango wawe uyifite, ushobora gupimwa BCHE gene mutation mbere yo gukorerwa anesthesia rusange. Ibi bishobora gufasha gukumira ingorane mu gihe cyo kubagwa.

Niba ufite imiterere y’uturemangingo (genetic mutation), muganga wawe ashobora kwirengagiza imiti ya Choline esters agahitamo indi miti igabanya ububabare. Rocuronium ni yo miti igabanya ububabare ikoreshwa cyane ku bantu bafite ikibazo cyo kubura Pseudocholinesterase.

Ushobora kugabanya ibyago byo kurwara indwara yo kubura Pseudocholinesterase nyuma y'ubuzima bwawe uvura indwara zibi, nko kurya nabi cyangwa indwara zidakira z'impyiko (CKD). Nanone, niba ufata imiti iyo ari yo yose igabanya ikorwa rya enzyme Pseudocholinesterase, ushobora kuvugana na muganga wawe ku bijyanye no guhindura imiti yawe.

Ni izihe mbogamizi zitesha agaciro, ni ukuvuga imiti idakwiye gukoreshwa ku bantu bafite iyi ndwara?

Niba waranduye `(Pseudocholinesterase Deficiency)`, ugomba kwirinda ubwoko bukurikira bw'imiti igabanya ububabare :

• Succinylcholine `(Succinylcholine)`
• Mivacurium
• Procaine
• Chloroprocaine
• Benzocaine
• Tetracaine

Ibibazo byo kubaza muganga wawe

Niba ufite indwara yitwa "Pseudocholinesterase Deficiency", waba warayivukanye cyangwa wayirwaye nyuma, ushobora kubaza muganga wawe ibibazo bimwe na bimwe nka:

• Ni izihe mpamvu nyazo zituma ntafata imiti, ni ukuvuga imiti nkwiye kwirinda?
• Ese iyi ndwara ni ikintu navukanye cyangwa ni indwara yatewe n'indi ndwara?
• Ese ni byiza ko umuryango wanjye upimwa imiterere y'uturemangingo?

Kujya kubagwa bishobora kugutera imihangayiko. Benshi muri twe tuba twibanda cyane ku kubagwa ku buryo tutanatekereza ku kuba hari ingaruka mbi zishobora guterwa no gusinziriza. Nta muti uhari wo kubura Pseudocholinesterase. Ariko, kumenya niba ufite iyo ndwara bishobora kugufasha kwirinda intege nke z'igihe gito z'imitsi n'ibibazo byo guhumeka bishobora kubaho nyuma yo kubagwa. Niba ufite Pseudocholinesterase Deficiency, bwira abagize umuryango wawe ibyayo. Bashobora kandi gupimwa gene no kwirinda ingorane zitari ngombwa zishobora kuvuka mu rugendo rwabo rw'ubuzima.

Amaherezo, ibintu byo kwibuka

Rero, `(Pseudocholinesterase Deficiency)` ni izina riteye ubwoba, ariko ni ingenzi cyane kurimenya. Cyane cyane niba ugiye kubagwa, cyangwa niba hari umuntu wo mu muryango wawe wahuye n'ikintu nk'iki , ni byiza kuganiriza abaganga bawe kuri ibi. Wibuke ko kumenya akenshi ari bwo buryo bwiza bwo kudufasha kwirinda ibibazo bitari ngombwa!

Anesthesia , pseudocholinesterase, kubura enzymes, ingorane zo kubaga, imiterere y'uturemangingo, imikorere mibi y'imitsi, imiti igabanya ububabare