Fanconi chirwere

Fanconi syndrome kusagadzikana kweiyo itsvo machubhu umo zvimwe zvinhu zvinowanzo nyudzwa muropa neitsvo zvinoburitswa mu weti panzvimbo.

Fanconi syndrome inogona kukonzerwa nemajini asina kunaka, kana inogona kukonzeresa mushure mehupenyu nekuda kwekukuvara kweitsvo. Dzimwe nguva chikonzero cheFanconi syndrome hachizivikanwe.

Zvakajairika zvikonzero zveFanconi syndrome muvana hurema hunokanganisa kugona kwemuviri kuputsa mamwe macomputer akadai se:

• Cystine (cystinosis)
• Fructose (fructose kusashivirira)
• Galactose (galactosemia)
• Glycogen (chirwere chekuchengetedza glycogen)

Cystinosis ndiyo inonyanya kukonzera Fanconi syndrome muvana.

Zvimwe zvikonzero muvana zvinosanganisira:

• Kuratidzirwa kune simbi dzinorema senge lead, mercury, kana cadmium
• Lowe syndrome, isingawanzoitika geni kusagadzikana kwemaziso, huropi, neitsvo
• Chirwere cheWilson
• Chirwere chemazino, chisingawanzoitika geni kusagadzikana kweitsvo

Muvanhu vakuru, Fanconi syndrome inogona kukonzerwa nezvinhu zvakasiyana siyana zvinokuvadza itsvo, kusanganisira:

• Mimwe mishonga, kusanganisira azathioprine, cidofovir, gentamicin, uye tetracycline
• Itsvo kuisazve
• Chiedza cheni deposition chirwere
• Akawanda myeloma
• Primary amyloidosis

Zviratidzo zvinosanganisira:

• Kupfuura yakawanda weti, izvo zvinogona kukonzeresa kupera mvura
• Nyota yakanyanya
• Kutambudzika kwakanyanya kwepfupa
• Kutsemuka nekuda kwekushaya simba kwepfupa
• Tsvina kushaya simba

Rabhoritari bvunzo dzinogona kuratidza kuti zvakawandisa zvezvinhu zvinotevera zvinogona kurasikirwa mumuti.

• Amino acids
• Bicarbonate
• Glucose
• Magnesium
• Phosphate
• Potassium
• Sodium
• Uric asidhi

Kurasikirwa kwezvinhu izvi kunogona kutungamira kune akasiyana matambudziko. Kuwedzera bvunzo uye bvunzo dzepanyama dzinogona kuratidza zviratidzo zve:

• Kupererwa nemvura mumuviri nekuda kwekunyanyisa kuita weti
• Kukura kutadza
• Osteomalacia
• Rickets
• Nyora 2 renal tubular acidosis

Zvirwere zvakawanda zvakasiyana zvinogona kukonzera Fanconi syndrome. Chikonzero chiri kukonzera uye zviratidzo zvacho zvinofanirwa kurapwa sezvakakodzera.

Kufungidzira kunoenderana nechirwere chiri pasi.

Fonera wako mupi wehutano kana iwe uine kupera mvura kana kushaya simba kwemhasuru.

De Toni-Fanconi-Debré chirwere

• Itsvo anatomi

Bonnardeaux A, Bichet DG. Nhaka kusagadzikana kweiyo renal tubule. Mune: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner uye Rector Iyo Itsvo. 11th ed. Philadelphia, PA: Elsevier; 2020: chitsama 44.

Foromani JW. Fanconi syndrome uye zvimwe zvinokanganisa tubule kusagadzikana. Muna: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Yakakwana Kliniki Nephrology. 6th ed. Philadelphia, PA: Elsevier; 2019: chitsuwa 48.