Indwara ya Rabson-Mendenhall ni iki? Reka tuvuge kuri iyi ndwara idasanzwe.

Ikintu cy'ibanze kibaho mu mibiri yacu ni uko ibiryo turya biduha imbaraga. Ni nko gushyira umwuka mu modoka. Aha, umubiri wacu ubona ingufu zikomoka ku isukari (glucose). Iyi gahunda yose igenzurwa na hormone yitwa insuline . Iyi insuline ni yo ibwira isukari iri mu maraso koherezwa mu turemangingo dukeneye ingufu. Ni nko gufungura inzugi z'uturemangingo no kureka isukari ikagera. Ariko, mu mubiri w'umuntu urwaye indwara ya Rabson-Mendenhall, iyi gahunda ntibaho neza. Ni ukuvuga ko umubiri we udashobora gukoresha insuline neza. Iyi ni indwara idasanzwe cyane.

None se iyi syndrome ni iki mu by'ukuri?

Iyo insuline idashoboye gukora akazi kayo neza, igira ingaruka ku mikurire y'umwana. Tekereza, ingaruka z'ibi zitangira na mbere y'uko umwana avuka, ni ukuvuga akiri mu nda ya nyina. Abana bafite iyi ndwara akenshi baba ari bato. Ndetse na nyuma yo kuvuka, kwiyongera ibiro byabo no gukura kw'umubiri wabo bigenda buhoro cyane. Mu buvuzi, indwara ya Rabson-Mendenhall ni indwara iri mu itsinda rinini ryitwa severe insuline resistance syndrome. Donohue syndrome na type A insuline resistance syndrome ni izindi ndwara ebyiri ziri muri iri tsinda.

Impamvu y'iki kibazo ni iyihe?

Mu magambo make, iyi ni indwara iterwa n’uturemangingo. Ni ukuvuga ko ikomoka ku babyeyi ku mwana . Ibi biterwa n’ikibazo kiri muri gene yitwa `INSR` mu mubiri wacu. Noneho reka turebe uko ibi bibaho. Muri buri turemangingo tw’umubiri wacu, hari kopi ebyiri za buri gene. Imwe ikomoka kuri nyina, n’indi ikomoka kuri se. Kugira ngo umwana agire indwara ya Rabson-Mendenhall, kopi zombi za gene ya `INSR` umwana ahabwa zigomba kuba zifite iyo nenge. Niba kopi imwe gusa ifite iyo nenge, indwara ntizakura. Mu yandi magambo, kugira ngo umwana agire iyi ndwara, nyina na se bagomba kuba bafite kopi imwe y’iyi gene ifite inenge n’ikopi imwe y’iyi gene nzima. Hanyuma, umwana agomba kubona kopi zombi zifite inenge ziturutse kuri bombi ku bw’amahirwe. Niyo mpamvu bibaho gake cyane kuko akenshi bitabaho inshuro eshatu kuri enye.

Ni ibihe bimenyetso by'iyi ndwara?

Ibimenyetso bikunze kugaragara mu mwaka wa mbere w'ubuzima bw'umwana. Nanone, ubukana bw'ibi bimenyetso bushobora gutandukana bitewe n'umuntu ku wundi. Reka turebe ibimenyetso by'ingenzi bishobora kugaragara.

Izindi ndwara zishobora guterwa n'ibi

Uretse ibi bimenyetso by'ibanze, iyi ndwara ishobora no gutera izindi ndwara zikomeye.

• Ibisebe mu ntanga z'abakobwa.
• Diyabete . Ibi rimwe na rimwe bishobora gutera indwara ishobora gushyira ubuzima mu kaga yitwa ketoacidosis .
• Ibibazo by'impyiko .

Isuzuma rikorwa rite?

Imwe mu mbogamizi mu gusuzuma iyi ndwara ni ukuyitandukanya n'izindi ndwara zisa (nk'indwara ya Donahue). Muganga w'umwana wawe azasuzuma umubiri we neza, abaza ibimenyetso by'umwana wawe n'amateka y'ubuzima bwe mu muryango, kandi ashobora gutegeka ko apimwa amaraso menshi kugira ngo arebe urugero rw'isukari mu maraso y'umwana wawe n'ingano ya insuline . Ubu ni bwo buryo bwonyine bwo gusuzuma neza.

Ivurwa ite?

Ubusanzwe kuvura indwara ya Rabson-Mendenhall bisaba ubufasha bw'ikipe nini, harimo inzobere zitandukanye, abaganga b'inzobere mu kubaga, n'abaganga b'amenyo. Ubujyanama bushobora kandi kuba ingenzi cyane ku miryango kugira ngo icunge imihangayiko n'amarangamutima bizanwa no kubyara umwana ufite iyi ndwara idasanzwe. Ubuvuzi akenshi bushingira kuri buri kimenyetso. Urugero, kubagwa kugira ngo hakurweho udukoko two mu miyoboro y'intanga, kuvura amenyo ku bibazo by'amenyo, nibindi. Hari ubwo abaganga batanga imiti myinshi ya insuline cyangwa indi miti itera imbaraga ikoreshwa rya insuline, ariko akenshi ntabwo igira ingaruka nziza mu gihe kirekire.

Uburyo bushya bwo kuvura burimo gukorwaho iperereza

Impuguke zikomeje gukora ubushakashatsi ku buryo bwiza bwo kuvura isukari nyinshi mu maraso (hyperglycemia) ifitanye isano no kudakora insuline cyane. Nubwo ubu buryo bwo kuvura bwagaragaje ibisubizo byiza, hakenewe ubundi bushakashatsi.

• Biguanides: Iyi ni ubwoko bw'imiti igabanya umusaruro w'isukari mu mubiri kandi ikongera ikoreshwa rya insuline .
• Leptin: Iyi poroteyine yagaragaye ko ifasha mu kugenzura isukari mu maraso n'urugero rwa insuline.
• rhIGF-I (Recombinant insulin-like growth factor I): Iyi poroteyine ikoreshwa mu kuvura ketoacidosis, indwara iterwa no kudakomera cyane kwa insuline .

Indwara ya Rabson-Mendenhall, ubudahangarwa bwa insuline, indwara zikomoka ku ngirabuzimafatizo, indwara z'abana, acanthosis nigricans, ketoacidosis, diyabete