Cilladda Fanconi

Cilladda Fanconi waa cilad ku timaadda tuubooyinka kelyaha taas oo walxaha qaarkood sida caadiga ah u nuugaan dhiigga ee kelyaha lagu sii daayo kaadida halkii.

Cilladda Fanconi waxaa sababi kara hiddo-wadaha cilladaysan, ama waxay sababi kartaa goor dambe oo nolosha ka mid ah dhaawac kelyaha ah. Mararka qaar lama oga sababta keenta cudurka Fanconi syndrome.

Sababaha guud ee keena cilladda Fanconi syndrome ee carruurta waa cilladaha hidda-socodka ee saameeya awoodda jirka ee ah inuu burburiyo xeryahooda qaarkood sida:

• Cystine (cystinosis)
• Fructose (dulqaad la'aan fructose)
• Galactose (galactosemia)
• Glycogen (cudurka kaydinta glycogen)

Cystinosis waa sababta ugu badan ee keenta cudurka Fanconi syndrome ee carruurta.

Sababaha kale ee carruurta waxaa ka mid ah:

• Soo-gaadhista biraha culus sida leedhka, meerkuriga, ama cadmium
• Cudurka 'Lowe syndrome', oo ah cillad hidde ah oo dhif ah oo indhaha, maskaxda, iyo kelyaha ah
• Cudurka Wilson
• Cudurka ilkaha, cillad hidde ah oo kelyaha ah

Dadka qaangaarka ah, Fanconi syndrome waxaa sababi kara waxyaabo kala duwan oo waxyeeleeya kilyaha, oo ay ka mid yihiin:

• Daawooyinka qaarkood, oo ay ku jiraan azathioprine, cidofovir, gentamicin, iyo tetracycline
• Qalitaanka kilyaha
• Cudurka dabinka silsiladda fudud
• Myeloma badan
• Amyloidosis aasaasi ah

Calaamadaha waxaa ka mid ah:

• Kaadida oo aad u tiro badan, taasoo u horseedi karta fuuqbax
• Haraad xad dhaaf ah
• Lafo xanuun daran
• Jabka jabka daciifnimada lafaha
• Murqaha oo daciifa

Tijaabooyinka shaybaarka ayaa laga yaabaa inay muujiyaan in inbadan oo ka mid ah walxaha soo socda ay ku lumi karaan kaadida:

• Acids amino
• Bicarbonate
• Gulukooska
• Magnesium
• Fosfateerka
• Botaasiyam
• Sodium
• Uric acid

Lumidda walxahaas waxay kuu horseedi kartaa dhibaatooyin kala duwan. Tijaabooyin dheeri ah iyo baaritaan jireed ayaa laga yaabaa inay muujiyaan calaamadaha:

• Fuuqbax kaadi saa’id ah awgeed
• Kobaca koritaanka
• Osteomalacia
• Rickets
• Nooca 2-aad ee acidosis renal tubular

Cuduro badan oo kala duwan ayaa sababi kara cudurka Fanconi syndrome. Sababta asalka ah iyo astaamaheeda waa in loola dhaqmaa sida ugu haboon.

Saadaashu waxay kuxirantahay cudurka salka ku haya.

Wac daryeel caafimaad bixiyahaaga haddii aad leedahay fuuqbax ama daciifnimo muruq.

De Toni-Fanconi-Debré syndrome

• Anatomy kilyaha

Bonnardeaux A, Bichet DG. Ciladaha laga dhaxlo tuubada kelyaha. Gudaha: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner iyo Raktarka kilyaha. 11aad. Philadelphia, PA: Elsevier; 2020: cutubka 44.

Horjooge JW. Cilladda Fanconi iyo dhibaatooyinka kale ee tuubada kudhowaad. Gudaha: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Neefroloji Caafimaad oo dhameystiran. 6aad ed. Philadelphia, PA: Elsevier; 2019: cutubka 48.