MMIH ​​okanye iBerdon Syndrome

Ukufezeka Kokuzalwa Namaqhinga Alukhulu

I-Megacystis microcolon intestinal hypoperistalsis syndrome (MMIH), eyaziwa nangokuthi yi-Berdon syndrome, ibangelwa yintlupheko yesisipha esisigxina kwisisu nesisu sokutya. Ingxaki ikhusela isisu, amathumbu, izintso, kunye nesibindi ekusebenzeni ngokuchanekileyo.

Ayaziwa kakuhle ukuba kungani okanye i-MMIH syndrome eyenzekayo. Abanye abaphandi bakholelwa ukuba lifa ngenxa yokuba kukho iimeko zabantakwabo abachaphazelekayo.

Phantse onke amacala aziwayo kumantombazana. Esinye sezizathu kuba oku kwenzeka ukuba i-MMIH inokuthi ingabonakali kakuhle njenge- prune belly syndrome , ingakumbi kubafana.

Iimpawu

Iimpawu ze-MMIH zivela kwi-disorder ye-muscle disorder. Iimfumba zamalungu angaphakathi zilahla kwaye azihlazi kakuhle kwi-utero. Iimpawu zeMMIH zi:

Iisisu zomzimba zincinci, okwenza isisu somntwana sibonakale sinyakanyiswa, njenge-prune. I-hypoperistalsis ikhokelela ekumanyeni nasekukhuselweni kwamathumbu.

Ukuxilongwa

I-MMIH kunzima ukuxilonga ngaphambi kokuba umntwana azalwe. Izitho zangaphakathi ezingavumelekanga ezifana nesibhanxa esandisiweyo kunye esingavinjelwanga singabonwa kwi-ultrasound fetal, kodwa kungenakucaca ukuba umntwana unemMMIH, ngaphandle kokuba umntwana ebengumntakwabo onesifo.

Emva kokuba umntwana ezalwe, ukuhlolwa ngokomzimba kunye ne-ultrasound kunokukwazi ukuba i-MMIH ikhona.

Ekuhloliseni ngokomzimba, oogqirha baya kukhangela le ntetho elandelayo yeempawu iimpawu zokuqala:

Unyango

Ngelishwa, akukho nonyango ye-MMIH (i-Berdon Syndrome), kwaye imbono ihlwempu kwiintsana ezalwa nayo; abaninzi bafa ngonyaka wokuqala wokuphila. Iintsana zinokudliwa ngokuzenzekelayo ngxube olukhethekileyo, kodwa olu hlobo lonyango lubangelwa ukungafezeki kwesibindi.

Oogqirha baye bazama ukutshintshela zonke izitho zangaphakathi eziphosakeleyo kwiintsana ezine-MMIH ngokutshintshwa. Inqaku kwiphepha leTransplantation ngo-1999 labika ngamantombazana amathathu kunye noM MMIH owamkele ukufakelwa kwamalungu amaninzi. Ukuguquka kwahluleka komntwana omnye, owafa; Umntwana omnye wasweleka kwiinyanga ezili-17 emva kwempumoniya , kodwa umntwana wesithathu wayephila kwaye ngexesha elifanelekileyo kwinqaku.

Enye inzame kwi-multi-organ transplantation ye-MMIH yenzeke ngoJanuwari 31, 2004. U-Alessia Di Matteo, waseGenoa, e-Italy, wathola izitho ezisibhozo ezisuka kwelinye usana: isibindi, isisu, i-pancreas, intlungu encinci, intumbu enkulu, i-spleen, kunye namibini izintso. Oogqirha kwiYunivesithi yaseMiami / iSibhedlele saseJackson Memorial, apho utyando lwenziwa khona, lwabikwa iiveki ezi-7 kamva ukuba uAlessia wenza kakuhle.

Uya kufuneka ukuba aqhubeke nokuthatha izidakamizwa zokuchaswa ubomi bakhe bonke, kodwa abazali bakhe bacinga ukuba intengo encinci yokuhlawula isipho sobomi esinikwe yona.

Imithombo:

U-Brecher, u-EJ Baby ufumana amalungu amatsha amathathu e-Miami. I-Miami Herald, uLwesihlanu, ngo-Matshi 19, 2004.

InfoBiogen. I-Megacystis-microcolon-intestinal-hypoperistalsis syndrome.

Masetti, M., Rodrigues, MM, Thompson, JF, Pinna, AD, Kato, T., Romaguera, R. l., Nery, JR, De Faria W., Khan, MF, Verzaro, R., Ruiz, P ., ITikis, AG (1999). Ukufakelwa kwe-multivisceral for megacystis microcolon intestinal hypoperistalsis syndrome. Ukutshintshwa , 68 (2), iphe. 228-232.