Into eyisisekelo eyenzeka emizimbeni yethu ukuthi ukudla esikudlayo kusinika amandla. Kufana nokufaka igesi emotweni. Lapha, imizimba yethu ithola amandla kushukela (i-glucose). Lonke lolu hlelo lulawulwa yi-hormone ebizwa ngokuthi
i-insulin . Yile insulin etshela ushukela egazini ukuthi uthunyelwe kumaseli adinga amandla. Kufana nokuvula iminyango yamaseli nokuvumela ushukela ungene. Kodwa-ke, emzimbeni womuntu one-Rabson-Mendenhall Syndrome, lolu hlelo alwenzeki kahle. Okusho ukuthi, imizimba yabo ayikwazi ukusebenzisa i-insulin kahle. Lesi yisimo esingavamile kakhulu.
Ngakho-ke iyini ngempela le syndrome?
Uma i-insulin ingakwazi ukwenza umsebenzi wayo kahle, ithinta ngqo ukukhula kwengane. Cabanga nje, umphumela walokhu uqala ngisho nangaphambi kokuba ingane izalwe, okungukuthi, isesesibelethweni sikanina. Izingane ezinalesi simo zivame ukuba zincane ngobukhulu. Ngisho nangemva kokuzalwa,
ukukhuluphala kwazo nokukhula komzimba kwenzeka kancane kakhulu. Ngokwezokwelapha, i-Rabson-Mendenhall syndrome yisifo esiseqenjini elikhulu elibizwa ngokuthi ama-insulin resistance syndromes anzima. I-Donohue syndrome kanye ne-type A insulin resistance syndrome yizinye izifo ezimbili ezikuleli qembu.
Iyini imbangela yalesi simo?
Kalula nje, lesi yisimo sofuzo. Okusho ukuthi,
sizuzwe njengefa kubazali kuya enganeni . Lokhu kubangelwa iphutha ku-gene ebizwa ngokuthi i-'INSR' emzimbeni wethu. Manje ake sibone ukuthi lokhu kwenzeka kanjani. Kuwo wonke amaseli omzimba wethu, kunezikhophi ezimbili ze-gene ngayinye. Elilodwa elivela kumama, nelilodwa elivela kubaba. Ukuze ingane ibe ne-Rabson-Mendenhall syndrome, womabili amakhophi e-gene ye-'INSR' ingane eyitholayo kumele abe nesici esishiwoyo. Uma ikhophi eyodwa kuphela inesici, lesi sifo ngeke sikhule. Ngamanye amazwi, ukuze ingane ibe nalesi sifo, bobabili umama nobaba kumele babe nekhophi eyodwa yalesi gene esinesici kanye nekhophi eyodwa ye-gene enempilo. Ngemuva kwalokho, ingane kumele ithole womabili amakhophi anesici kubo bobabili ngengozi. Yingakho kungavamile kangaka ngoba ngokuvamile akwenzeki kathathu noma kane.
Ziyini izimpawu zalesi simo?
Izimpawu zivame ukuqala ukuvela
phakathi nonyaka wokuqala wokuphila kwengane. Futhi, ubukhali balezi zimpawu bungahluka kuye ngomuntu nomuntu. Ake sibheke izimpawu eziyinhloko ezingabonakala.
| Ingxenye/uhlelo lomzimba | Izici ezibonakalayo |
|---|
| Ikhanda nobuso | Isikhumba esiqinile, isikhala esikhulu phakathi kwamehlo, imisele ejulile olimini, emikhulu kunezindlebe, izindebe, kanye nomhlathi ojwayelekile. |
| Izinzipho | Kukhulu kunokujwayelekile. |
| Isikhumba | Isikhumba esomile. Ukufiphala nokujiya kwezindawo ezithile zesikhumba (ikakhulukazi emagobolondweni afana namakhwapha nentamo). Lokhu kwaziwa ngokwezokwelapha ngokuthi i-acanthosis nigricans . Lezi zindawo zingase zizizwe zithambile uma uzithinta. |
| Amazinyo | Ukuba mkhulu kunokujwayelekile, ukuminyana ndawonye, noma ukuqhakaza kwamazinyo ngaphambi kwesikhathi. |
| Izitho zangaphakathi | Izinso, inhliziyo, kanye nezitho zobulili (ipenisi kubafana, isitho sangasese ... |
| Ezinye izici ezivamile | Ukukhula kwezinwele emzimbeni ngokweqile, ukukhula kancane ngaphambi nangemva kokubeletha, ukuvuvukala kwesisu, amafutha amancane kakhulu ngaphansi kwesikhumba, kanye nobuthakathaka bemisipha. |
Ezinye izifo ezingase zivele ngenxa yalokhu
Ngaphezu kwalezi zimpawu eziyisisekelo, lesi sifo singabangela nezinye izimo ezimbi kakhulu.
- Ama-cysts kuma-ovari amantombazane.
- Isifo sikashukela . Lokhu ngezinye izikhathi kungaholela esimweni esisongela ukuphila esibizwa ngokuthi i-ketoacidosis .
- Izinkinga zezinso .
Kwenziwa kanjani ukuxilongwa?
Enye yezinselele ekuxilongeni lesi simo ukusehlukanisa nezinye izimo ezifana naso (njenge-Donahue syndrome).
Udokotela wengane yakho uzokwenza ukuhlolwa okujulile ngokomzimba, abuze ngezimpawu zengane yakho kanye nomlando wempilo yomndeni, futhi cishe uzoyala ukuhlolwa kwegazi okuningana ukuze ahlole
amazinga kashukela egazini lengane yakho kanye ne-insulin . Lena ukuphela kwendlela yokwenza ukuxilongwa okunembile.
Iphathwa kanjani?
Ukwelashwa kwe-Rabson-Mendenhall syndrome kuvame ukudinga usizo lweqembu elikhulu, okuhlanganisa ochwepheshe abahlukahlukene, odokotela abahlinzayo, nodokotela bamazinyo. Ukwelulekwa kungaba yinto ebaluleke kakhulu emindenini ukuze kulawulwe ukucindezeleka nemizwa ehambisana nokuba nomntwana onalesi simo esingavamile.
Okwamanje akukho ukwelashwa okuhlala njalo kwalesi simo. Ukwelashwa kuhloselwe ukulawula nokuphatha izimpawu.
Ukwelashwa kuvame ukuqondene ngqo nophawu ngalunye. Isibonelo, ukuhlinzwa kokususa ama-cyst esibelethweni, ukwelashwa kwamazinyo ngezinkinga zamazinyo, njll. Kwezinye izimo, odokotela banikeza imithamo ephezulu ye-insulin noma eminye imithi ekhuthaza ukusetshenziswa kwe-insulin, kodwa lokhu akuvamile ukusebenza isikhathi eside.
Ukwelashwa okusha kuyaphenywa
Ochwepheshe bayaqhubeka nokucwaninga ngezinketho zokwelapha ezingcono zamazinga aphezulu kashukela egazini (i-hyperglycemia) ehlotshaniswa nokumelana okukhulu kwe-insulin. Nakuba lokhu kwelashwa kubonise imiphumela ethembisayo, kudingeka ucwaningo olwengeziwe.
- Ama-Biguanides: Lawa uhlobo lomuthi onciphisa ukukhiqizwa kukashukela emzimbeni futhi wandise ukusetshenziswa kwe-insulin .
- I-Leptin: Le phrotheni itholakale isiza ukulawula ushukela egazini kanye namazinga e-insulin.
- i-rhIGF-I (Isici sokukhula esifana ne-insulin I): Le phrotheni isetshenziselwa ukwelapha i-ketoacidosis, isimo esibangelwa ukumelana okukhulu kwe-insulin .
Umlayezo Wokuya Nawe Ekhaya
- I-Rabson-Mendenhall Syndrome iyisimo esingavamile kakhulu sofuzo lapho umzimba ungakwazi ukusebenzisa i-insulin ngendlela efanele.
- Lokhu kubangelwa yi-gene enephutha (i-gene ethi `INSR`) ezuzwa yingane kubazali bobabili.
- Izimpawu ziqala ebuntwaneni futhi zithinta ukukhula komzimba , ukubukeka kobuso , isikhumba, amazinyo, kanye nezitho zangaphakathi.
- Akukho ukwelashwa okuhlala njalo kwalokhu, futhi ukwelashwa kuhloselwe ukulawula izimpawu. Lokhu kudinga ukwesekwa yithimba lodokotela abangochwepheshe.
- Uma ingane yakho ikhombisa noma yiziphi zalezi zimpawu, kubaluleke kakhulu ukubona udokotela ofanelekayo (udokotela) ngokushesha ukuze uthole iseluleko.
I-Rabson-Mendenhall Syndrome, ukumelana ne-insulin, izifo zofuzo, izifo zezingane, i-acanthosis nigricans, i-ketoacidosis, isifo sikashukela
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