Matenda a von Gierke

Matenda a Von Gierke ndi omwe thupi silitha kuwononga glycogen. Glycogen ndi mtundu wa shuga (shuga) womwe umasungidwa m'chiwindi ndi minofu. Nthawi zambiri imasweka kukhala glucose kuti ikupatseni mphamvu zambiri mukafuna.

Matenda a Von Gierke amatchedwanso Type I glycogen matenda osungira (GSD I).

Matenda a Von Gierke amapezeka thupi likasowa puloteni (enzyme) yomwe imatulutsa shuga kuchokera ku glycogen. Izi zimayambitsa kuchuluka kwa glycogen m'matenda ena. Glycogen ikasweka bwino, imayambitsa shuga wotsika magazi.

Matenda a Von Gierke amatengera cholowa, zomwe zikutanthauza kuti amapatsira kudzera m'mabanja. Ngati makolo onse atenga mtundu wosagwira ntchito wokhudzana ndi vutoli, mwana wawo aliyense ali ndi mwayi 25% (1 mwa 4) wokhala ndi matendawa.

Izi ndi zizindikiro za matenda a von Gierke:

• Nthawi zonse njala ndikusowa kudya pafupipafupi
• Kuvulaza kosavuta ndi kutulutsa magazi m'mphuno
• Kutopa
• Kukwiya
• Masaya otukumula, chifuwa chowonda ndi miyendo, ndi mimba yotupa

Wothandizira zaumoyo wanu adzakuyesani.

Mayesowa atha kuwonetsa zizindikiro za:

• Kuchedwa kutha msinkhu
• Kukulitsa chiwindi
• Gout
• Matenda otupa
• Zotupa za chiwindi
• Shuga wambiri wamagazi
• Kukula pang'onopang'ono kapena kulephera kukula

Ana omwe ali ndi vutoli nthawi zambiri amapezeka asanakwanitse zaka 1.

Mayeso omwe angachitike ndi awa:

• Chiwindi cha chiwindi kapena impso
• Mayeso a shuga wamagazi
• Kuyesedwa kwachibadwa
• Lactic acid kuyesa magazi
• Mulingo wa Triglyceride
• Uric acid kuyesa magazi

Ngati munthu ali ndi matendawa, zotsatira zake ziziwonetsa shuga wotsika magazi komanso kuchuluka kwa lactate (yopangidwa kuchokera ku lactic acid), mafuta am'magazi (lipids), ndi uric acid.

Cholinga cha chithandizo ndikupewa shuga wotsika magazi. Idyani pafupipafupi masana, makamaka zakudya zomwe zimakhala ndi chakudya. Ana okalamba ndi akulu amatha kumwa chimanga pakamwa kuti awonjezere zakumwa zamahydrate.

Kwa ana ena, chubu chodyetsera chimayikidwa m'mphuno m'mimba usiku wonse kuti apereke shuga kapena chimanga chosaphika. Chitoliro chimatha kutulutsidwa m'mawa uliwonse. Kapenanso, chubu cha gastrostomy (G-chubu) chitha kuyikidwa kuti chibweretse chakudya kumimba usiku wonse.

Mankhwala oti muchepetse uric acid m'magazi ndikuchepetsa chiopsezo cha gout atha kulembedwa. Wothandizira anu amathanso kukupatsirani mankhwala ochizira matenda a impso, lipids, komanso kuwonjezera ma cell omwe amalimbana ndi matenda.

Anthu omwe ali ndi matenda a von Gierke sangathe kuwononga shuga kapena zipatso mkaka. Ndikofunika kupewa izi.

Mgwirizano wa Matenda Osungira a Glycogen - www.agsdus.org

Ndi chithandizo, kukula, kutha msinkhu, ndi moyo wabwino zasintha kwa anthu omwe ali ndi matenda a von Gierke. Omwe amadziwika ndi kusamalidwa mosamala ali aang'ono amatha kukhala achikulire.

Chithandizo choyambirira chimachepetsanso mavuto azovuta monga:

• Gout
• Impso kulephera
• Shuga wotsika magazi wowopsa
• Zotupa za chiwindi

Zovuta izi zitha kuchitika:

• Matenda pafupipafupi
• Gout
• Impso kulephera
• Zotupa za chiwindi
• Osteoporosis (kupatulira mafupa)
• Khunyu, ulesi, kusokonezeka chifukwa cha magazi otsika shuga
• Kutalika kwakanthawi
• Khalidwe lachiwerewere lomwe silikukula (mawere, ubweya)
• Zilonda mkamwa kapena matumbo

Itanani omwe akukuthandizani ngati muli ndi mbiri yoti banja lanu lili ndi matenda osungira glycogen kapena kufa kwa ana ali wakhanda chifukwa chotsika shuga.

Palibe njira yosavuta yopewera matenda osungira glycogen.

Mabanja omwe akufuna kukhala ndi mwana atha kufunsa upangiri wa majini ndi kukayezetsa kuti adziwe ngati ali pachiwopsezo chotenga matenda a von Gierke.

Lembani matenda osungira glycogen

Bonnardeaux A, Bichet DG. Matenda obadwa nawo a aimpso tubule. Mu: Skorecki K, Chertow GM, Marsden PA, Taal MW, Yu ASL, olemba. Brenner ndi Rector a Impso. 10th ed. Philadelphia, PA: Elsevier; 2016: chap 45.

Kishnani PS, Chen YT. Zofooka mu kagayidwe chakudya. 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 105.

Santos BL, Souza CF, Schuler-Faccini L, ndi al. Matenda osungira a Glycogen mtundu 1: mbiri yazachipatala komanso labotale. J Pediatra (Rio J). 2014; 90 (6): 572-579. PMID: 25019649 www.ncbi.nlm.nih.gov/pubmed/25019649.