Chifukwa Chavuto Iri Lagawanika mu Syndromes Awiri Osiyana
Zaka zapitazi, matenda a Laurence-Moon-Bardet-Biedl (LMBBS) ndi mawu omwe amagwiritsidwa ntchito pofotokoza za chibadwa cha mabadwa omwe anabadwa pafupifupi 1 mwa ana 100,000 obadwa. Anatchulidwa madokotala anayi amene poyamba anafotokoza zizindikiro za matenda.
Kuchokera apo, LMBBS yadziwika kuti si yolondola. M'malo mwake, anapeza kuti LMBBS inali ndi mavuto awiri osiyana: Matenda a Biedl-Bardet (BBS) ndi matenda a Laurence-Moon (LMS).
Kusiyanitsa pakati pa Biedl-Bardet Syndrome ndi Laurence-Moon Syndrome
BBS ndi matenda ovuta kwambiri omwe amachititsa kuti masomphenya awonongeke, zala zala kapena zala, kunenepa m'mimba ndi m'mimba, matenda a impso , ndi mavuto ophunzirira. Masomphenya akuwongolera mofulumira; anthu ambiri adzakhala akhungu kwathunthu. Mavuto ochokera ku zizindikiro zina, monga mavuto a impso, akhoza kuika moyo pachiswe.
Monga BBS, LMS ndi matenda obadwa nawo. Zimakhudzana ndi zovuta, kuphunzira, kuchepetsa kugonana kwa mahomoni, ndi kuuma kwa minofu ndi ziwalo. BBS ndi LMS ndi ofanana kwambiri koma amaonedwa ngati osiyana chifukwa odwala a LMS samasonyeza zizindikiro zochuluka kapena kunenepa kwambiri m'mimba.
Zifukwa za BBS ndi LMS
Nthawi zambiri mabungwe a BBS amachotsedwa. Zimakhudza amuna ndi akazi mofanana koma si zachilendo. BBS imakhudza 1 pa 100,000 ku North America ndi Europe. Ndizofala kwambiri m'mayiko a Kuwait ndi Newfoundland, koma asayansi sadziwa chifukwa chake.
LMS ndi nthenda yobadwa nayo. LMS ndi yodabwitsa kwambiri, kutanthauza kuti imangowoneka ngati makolo onse ali ndi majeremusi a LMS. Kawirikawiri, makolo okhawo alibe LMS koma amanyamula jini kuchokera kwa makolo awo omwe.
Momwe Akudziwira
BBS nthawi zambiri imapezeka kuti ali mwana. Kuyezetsa maonekedwe ndi kuyerekezera zamagetsi kudzayang'ana zosiyana ndi zochedwa.
Nthaŵi zina, kuyezetsa mafupa kungathe kuzindikira kuti pali matendawa asanakhalepo.
LMS kawirikawiri imapezeka poyesedwa kuti ikulepheretsedwe, monga kuwonetseratu zolepheretsa kulankhula, kulephera kuphunzira, komanso mavuto ovomerezeka .
Kuchiza kwa Zinthu ziwiri
Kuchiza kwa BBS kumaphatikizapo kuchiza zizindikiro za matendawa, monga kukonza masomphenya kapena kusintha kwa impso. Kuwongolera mofulumira kungathandize ana kuti azikhala ndi moyo wathanzi komanso kuthetsa zizindikiro, koma palibe chithandizo cha matendawa panthawiyi.
Kwa LMS, palibe mankhwala omwe amavomerezedwa kuti athetse vutoli. Monga BBS, chithandizo chimayang'ana kuthetsa zizindikiro. Thandizo la ophthalmic, monga magalasi kapena zinthu zina zothandizira, zingathandize kuwopsya masomphenya. Pofuna kuthandizira kuchepetsa msinkhu komanso kukula kwa kuchepa, mankhwala opangira mahomoni angakhale othandizidwa. Kulankhula ndi chithandizo chamaganizo kungathandize kuti anthu azikhala osagwirizana komanso akhale ndi luso labwino. Mankhwala othandizira odwala matendawa ndi ofunika.
Matenda a Biedl-Bartet Syndrome kapena Laurence-Moon Syndrome
Kwa omwe ali ndi matenda a Laurence-Moon, nthawi ya moyo nthawi yayitali ndi yofupika kuposa anthu ena. Chifukwa chofala kwambiri cha imfa chimagwirizanitsidwa ndi vuto lachinyengo kapena impso.
Kwa matenda a Biedel-Bartet, kuchepa kwa nthendayi kumakhala kofala kwambiri ndipo ndi chifukwa chachikulu chomwe chimayambitsa imfa. Kusamalira zokhudzana ndi nsomba kungapangitse chiyembekezo cha moyo ndi khalidwe la moyo.
Zotsatira:
"Biedl-Bartet Syndrome". National Organization for Rare Disorders, 2015.
"Laurence-Moon Syndrome". Patient.Info, 2015.