Matenda a Fanconi

Matenda a Fanconi ndi matenda amachubu ya impso momwe zinthu zina zomwe zimalowa m'magazi ndi impso zimatulutsidwa mumkodzo m'malo mwake.

Matenda a Fanconi amatha kuyambitsidwa ndi majini olakwika, kapena atha kubwera pambuyo pake chifukwa cha kuwonongeka kwa impso. Nthawi zina chifukwa cha matenda a Fanconi sichidziwika.

Zomwe zimayambitsa matenda a Fanconi mwa ana ndi zofooka zamtundu zomwe zimakhudza kuthekera kwa thupi kuwononga zinthu zina monga:

• Cystine (cystinosis)
• Fructose (kusagwirizana kwa fructose)
• Galactose (galactosemia)
• Glycogen (matenda osungira glycogen)

Cystinosis ndiye chifukwa chofala kwambiri cha matenda a Fanconi mwa ana.

Zina zomwe zimayambitsa ana ndi izi:

• Kuwonetsedwa pazitsulo zolemera monga lead, mercury, kapena cadmium
• Matenda a Lowe, matenda osowa amtundu wamaso, ubongo, ndi impso
• Matenda a Wilson
• Matenda a mano, matenda osowa a impso

Kwa akuluakulu, matenda a Fanconi amatha kuyambitsidwa ndi zinthu zosiyanasiyana zomwe zimawononga impso, kuphatikizapo:

• Mankhwala ena, kuphatikizapo azathioprine, cidofovir, gentamicin, ndi tetracycline
• Kuika impso
• Matenda oyatsira magetsi
• Myeloma yambiri
• Pulayimale amyloidosis

Zizindikiro zake ndi izi:

• Kupititsa mkodzo wambiri, zomwe zingayambitse kuchepa kwa madzi
• Ludzu lokwanira
• Kupweteka kwambiri kwa mafupa
• Kutyoka chifukwa cha kufooka kwa mafupa
• Minofu kufooka

Mayeso a labotale atha kuwonetsa kuti zinthu zotsatirazi zitha kutayika mu mkodzo:

• Amino zidulo
• Bicarbonate
• Shuga
• Mankhwala enaake a
• Mankwala
• Potaziyamu
• Sodium
• Uric asidi

Kutaya zinthu izi kumatha kubweretsa mavuto osiyanasiyana. Mayeso ena ndi kuyezetsa thupi kumatha kuwonetsa:

• Kutaya madzi m'thupi chifukwa chokodza kwambiri
• Kukula kulephera
• Osteomalacia
• Zolemba
• Lembani 2 renal tubular acidosis

Matenda osiyanasiyana amatha kuyambitsa matenda a Fanconi. Choyambitsa komanso zizindikiro zake ziyenera kuchitidwa moyenera.

Kulosera kumatengera matenda omwe amabwera.

Itanani omwe akukuthandizani ngati muli ndi vuto lakutaya madzi m'thupi kapena kufooka kwa minofu.

Matenda a De Toni-Fanconi-Debré

• Matenda a impso

Bonnardeaux A, Bichet DG. Matenda obadwa nawo a aimpso tubule. Mu: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, olemba. Brenner ndi Rector a Impso. 11th ed. Philadelphia, PA: Elsevier; 2020: chap 44.

Woyang'anira JW. Matenda a Fanconi ndi matenda ena ofala a tubule. Mu: Feehally J, Floege J, Tonelli M, Johnson RJ, olemba. Chachikulu Chachipatala Nephrology. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2019: chap 48.