Matenda a Beckwith-Wiedemann

Matenda a Beckwith-Wiedemann ndimatenda okula omwe amachititsa kukula kwa thupi, ziwalo zazikulu, ndi zizindikilo zina. Ndi chikhalidwe chobadwa nacho, chomwe chimatanthauza kuti chimakhalapo pakubadwa. Zizindikiro za matendawa zimasiyanasiyana mwana ndi mwana.

Khanda limakhala nthawi yovuta mwa makanda omwe ali ndi vutoli chifukwa chotheka:

• Shuga wamagazi ochepa
• Mtundu wa hernia wotchedwa omphalocele (akakhalapo)
• Lilime lokulitsidwa (macroglossia)
• Kuchuluka kwa kukula kwa chotupa. Zotupa za Wilms ndi hepatoblastomas ndizotupa zofala kwambiri kwa ana omwe ali ndi vutoli.

Matenda a Beckwith-Wiedemann amayamba chifukwa cha vuto lomwe limayambitsa ma chromosome 11. Pafupifupi 10% yamilandu imatha kuperekedwa kudzera m'mabanja.

Zizindikiro za matenda a Beckwith-Wiedemann ndi awa:

• Kukula kwakukulu kwa wakhanda
• Chizindikiro chofiira chakumaso pamphumi kapena zikope (nevus flammeus)
• Amapanga m'makutu akumakutu
• Lilime lalikulu (macroglossia)
• Shuga wamagazi ochepa
• Mimba yam'mimba yolakwika (umbilical hernia kapena omphalocele)
• Kukulitsa kwa ziwalo zina
• Kukula kwa mbali imodzi ya thupi (hemihyperplasia / hemihypertrophy)
• Kukula kwa chotupa, monga zotupa za Wilms ndi hepatoblastomas

Wothandizira zaumoyo adzayesa thupi kuti aone ngati ali ndi matenda a Beckwith-Wiedemann. Nthawi zambiri izi ndizokwanira kuti mupeze matenda.

Mayeso a vutoli ndi awa:

• Kuyezetsa magazi kwa shuga wotsika magazi
• Kafukufuku wa Chromosomal wazovuta mu chromosome 11
• Ultrasound pamimba

Makanda omwe ali ndi shuga wotsika kwambiri amathandizidwa ndimadzimadzi omwe amaperekedwa kudzera mumitsempha (intravenous, IV). Ana ena angafunike mankhwala kapena chithandizo china ngati shuga wotsika magazi akupitilira.

Zofooka m'makoma am'mimba zimafunika kukonzedwa. Ngati lilime lokulitsa likuvutitsa kupuma kapena kudya, opaleshoni ingafunike. Ana omwe akuchulukirachulukira mbali imodzi ya thupi ayenera kuyang'aniridwa ndi msana wopindika (scoliosis). Mwanayo ayeneranso kuyang'aniridwa bwino ndikukula kwa zotupa. Kuwunika kwa chotupa kumaphatikizapo kuyesa magazi ndi m'mimba ma ultrasound.

Ana omwe ali ndi matenda a Beckwith-Wiedemann amakhala moyo wabwinobwino. Kupitiliza maphunziro kumafunika kuti pakhale chidziwitso chotsatira chotsatira.

Zovuta izi zitha kuchitika:

• Kukula kwa zotupa
• Kudyetsa mavuto chifukwa cha kukulitsa lilime
• Mavuto opumira chifukwa chokulitsa lilime
• Scoliosis chifukwa hemihypertrophy

Ngati muli ndi mwana yemwe ali ndi matenda a Beckwith-Wiedemann ndipo zizindikiro zowopsa zimayamba, pitani kuchipatala nthawi yomweyo.

Palibe choletsa kudziwika cha matenda a Beckwith-Wiedemann. Upangiri wa chibadwa ungakhale wofunikira kwa mabanja omwe angafune kukhala ndi ana ambiri.

• Matenda a Beckwith-Wiedemann

Devaskar SU, Garg M. Kusokonezeka kwa kagayidwe kabakiteriya m'thupi mwa mwana wakhanda. Mu: Martin RJ, Fanaroff AA, Walsh MC, olemba., Eds. Fanaroff ndi Martin's Neonatal-Perinatal Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015: chap 95.

Madan-Khetarpal S, Arnold G. Matenda amtundu komanso zovuta za dysmorphic. Mu: Zitelli, BJ, McIntire SC, Nowalk AJ, eds. Zitelli ndi Davis ’Atlas of Pediatric Physical Diagnosis. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 1.

Kutulutsa MA. Matenda osokoneza bongo. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 111.