Ekisinga obukulu ekibaawo mu mibiri gyaffe kwe kuba nti emmere gye tulya etuwa amaanyi. Kiba ng’okuteeka ggaasi mu mmotoka. Wano, omubiri gwaffe gufuna amaanyi okuva mu sukaali (glucose). Enkola eno yonna efugibwa obusimu obuyitibwa
insulin . Insulini eno y’egamba ssukaali ali mu musaayi okusindikibwa mu butoffaali obwetaaga amaanyi. Kiba ng’okuggulawo enzigi z’obutoffaali n’oleka ssukaali okuyingira Kyokka mu mubiri gw’omuntu alina obulwadde bwa Rabson-Mendenhall Syndrome, enkola eno tegenda bulungi. Kwe kugamba, omubiri gwabwe tegusobola kukozesa bulungi insulini. Eno mbeera ya bbula nnyo nnyo.
Kale ddala obulwadde buno kye ki?
Insulin bw’eba tesobola kukola bulungi mulimu gwayo, ekosa butereevu enkula y’omwana. Teebereza, ebiva mu kino bitandika ne bwe kiba nti omwana tannazaalibwa, kwe kugamba, ng’akyali mu lubuto lwa maama. Abalongo abalina embeera eno batera okuba abatono mu sayizi. Ne bwe baba bamaze okuzaalibwa,
okugejja kwabwe n’okukula kw’omubiri bibaawo mpola nnyo. Mu by’obusawo, obulwadde bwa Rabson-Mendenhall bulwadde obubeera mu kibinja ekinene ekiyitibwa severe insulin resistance syndromes. Obulwadde bwa Donohue syndrome ne type A insulin resistance syndrome bwe ndwadde endala bbiri ezibeera mu kibinja kino.
Kiki ekivuddeko embeera eno?
Mu ngeri ennyangu, eno mbeera ya buzaale. Kwe kugamba,
kisikira okuva ku bazadde okutuuka ku mwana . Kino kiva ku glitch mu gene eyitibwa `INSR` mu mubiri gwaffe. Kati ka tulabe engeri kino gye kibaawo. Mu buli katoffaali k’omubiri gwaffe, buli buzaale bulimu kkopi bbiri. Omu okuva ku maama, n’omulala okuva ku taata. Omwana okusobola okufuna obulwadde bwa Rabson-Mendenhall syndrome, kkopi zombi ez’obuzaale bwa `INSR` omwana z’afuna zirina okuba n’ekikyamu ekyogerwako. Singa kkopi emu yokka eba n’ekikyamu, obulwadde tebujja kukula. Mu ngeri endala, omwana okusobola okufuna obulwadde buno, maama ne taata bombi balina okuba ne kkopi emu ey’obuzaale buno obulema ne kkopi emu ey’obuzaale obulamu. Olwo, omwana alina okufuna kkopi zombi ezitali nnungi okuva ku bombi mu butanwa. Eno y’ensonga lwaki tekitera kubaawo kubanga ebiseera ebisinga tekibaawo emirundi esatu ku mirundi ena.
Bubonero ki obulaga embeera eno?
Obubonero butera okutandika okulabika
mu mwaka ogusooka ogw’obulamu bw’omwana. Ate era, obuzibu bw’obubonero buno buyinza okwawukana okusinziira ku muntu. Ka tulabe obubonero obukulu obuyinza okulabibwa.
| Ekitundu/enkola y’omubiri | Ebintu ebirabika |
|---|
| Omutwe ne ffeesi | Olususu olukaluba, ekituli ekigazi wakati w’amaaso, ebituli ebiwanvu ku lulimi, ebinene okusinga amatu aga bulijjo, emimwa n’akawanga. |
| Emisumaali | Ekinene okusinga ekya bulijjo. |
| Omubiri | Olususu olukalu. Okuddugala n’okugonza ebitundu ebimu eby’olususu (naddala mu bifo ebikutte ng’enkwawa n’ensingo). Kino mu by'obujjanjabi kimanyiddwa nga acanthosis nigricans . Ebitundu bino biyinza okuwulira nga bya velvet ng’obikwatako. |
| Amanyo | Okubeera omunene okusinga ku bulijjo, okujjula wamu oba okuvaamu amannyo nga tegannaba kutuuka. |
| Ebitundu by’omubiri eby’omunda | Ensigo, omutima n’ebitundu by’ekyama (obusajja mu balenzi, obukyala mu bawala) biba binene okusinga ebya bulijjo. |
| Ebintu ebirala ebitera okubeerawo | Enviiri z’omubiri ezisukkiridde okukula, okukula mpola nga tonnazaala n’oluvannyuma lw’okuzaalibwa, okuzimba olubuto, amasavu matono nnyo wansi w’olususu, n’okunafuwa kw’ebinywa. |
Endwadde endala eziyinza okubaawo olw’ensonga eno
Ng’oggyeeko obubonero buno obukulu, obulwadde buno era busobola okuleeta embeera endala ez’amaanyi.
Okuzuula obulwadde buno kukolebwa kutya?
Ekimu ku bisomooza mu kuzuula embeera eno kwe kugyawula ku mbeera endala ezifaanagana (nga Donahue syndrome).
Omusawo w’omwana wo ajja kukukebera omubiri mu bujjuvu, akubuuze ku bubonero bw’omwana wo n’ebyafaayo by’obulamu bw’amaka, era kirabika ajja kulagira okukeberebwa omusaayi emirundi egiwerako okukebera
ssukaali n’obungi bwa insulini mu musaayi gw’omwana wo . Eno y’engeri yokka ey’okuzuula obulwadde mu ngeri entuufu.
Kijjanjabwa kitya?
Okujjanjaba obulwadde bwa Rabson-Mendenhall syndrome ebiseera ebisinga kyetaagisa okuyambibwako ttiimu ennene, omuli abakugu ab’enjawulo, abalongoosa, n’abasawo b’amannyo. Okubuulirira nakyo kiyinza okuba ekikulu ennyo eri amaka okuddukanya situleesi n‟enneewulira ebijja n‟okuzaala omwana alina embeera eno etali ya bulijjo.
Mu kiseera kino tewali ddagala lya nkalakkalira eriwonya embeera eno. Obujjanjabi bugendereddwamu okufuga n’okuddukanya obubonero.
Obujjanjabi butera okuba obw’enjawulo ku buli kabonero. Okugeza, okulongoosa okuggyawo ebizimba by’enkwaso, okujjanjaba amannyo ku bizibu by’amannyo n’ebirala Mu mbeera ezimu, abasawo bawandiika ddoozi nnyingi eza insulini oba eddagala eddala erisitula okukozesa insulini, naye bino bitera obutakola bulungi mu bbanga eggwanvu.
Obujjanjabi obupya bunoonyezebwa
Abakugu bakyagenda mu maaso n’okunoonyereza ku bujjanjabi obulungi obw’okujjanjaba ssukaali omungi (hyperglycemia) obukwatagana n’okuziyiza insulini okw’amaanyi. Wadde ng’obujjanjabi buno bulaze ebimu ebisuubiza, kyetaagisa okwongera okunoonyereza.
- Biguanides: Zino kika kya ddagala erikendeeza ku ssukaali mu mubiri n’okwongera okukozesa insulini .
- Leptin: Puloteeni eno ezuuliddwa nti eyamba okufuga ssukaali mu musaayi ne insulini.
- rhIGF-I (Recombinant insulin-like growth factor I): Puloteeni eno ekozesebwa okujjanjaba obulwadde bwa ketoacidosis, embeera eva ku buziyiza bwa insulini obw’amaanyi .
Obubaka Obutwala Awaka
- Obulwadde bwa Rabson-Mendenhall Syndrome mbeera ya buzaale etali ya bulijjo nnyo ng’omubiri tegusobola kukozesa bulungi insulini .
- Kino kiva ku buzaale obulema (obuzaale bwa `INSR`) omwana bw’asikira okuva mu bazadde bombi.
- Obubonero butandikira mu buto era bukosa enkula y’omubiri , endabika ya ffeesi , olususu, amannyo, n’ebitundu by’omunda.
- Kino tewali ddagala lya lubeerera era obujjanjabi bugendereddwamu okuddukanya obubonero. Kino kyetaagisa obuwagizi bwa ttiimu y’abasawo abakugu.
- Omwana wo bw’aba alaga obubonero buno bwonna, kikulu nnyo okulaba omusawo (omusawo) alina ebisaanyizo mu bwangu okufuna amagezi.
Rabson-Mendenhall Syndrome, okuziyiza insulini, endwadde z’obuzaale, endwadde z’abaana, acanthosis nigricans, ketoacidosis, sukaali
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