Lefu la Fanconi

Fanconi syndrome ke bokuli ba methapo ea liphio moo lintho tse ling tse atisang ho kenella maling ke liphio li lokolloang ka morong ho fapana.

Fanconi syndrome e ka bakoa ke liphatsa tsa lefutso tse fosahetseng, kapa e ka baka hamorao bophelong ka lebaka la tšenyo ea liphio. Ka linako tse ling sesosa sa lefu la Fanconi ha se tsejoe.

Lisosa tse tloaelehileng tsa Fanconi syndrome ho bana ke liphoso tsa lefutso tse amang bokhoni ba 'mele ba ho senya metsoako e meng e kang:

• Cystine (cystinosis)
• Fructose (ho hloka mamello ha fructose)
• Galactose (galactosemia)
• Glycogen (lefu la polokelo ea glycogen)

Cystinosis ke sesosa se tloaelehileng haholo sa lefu la Fanconi baneng.

Lisosa tse ling ho bana li kenyelletsa:

• Ho hlahisa lihlahisoa tse boima tse kang lead, mercury kapa cadmium
• Lowe syndrome, lefu le sa tloaelehang la liphatsa tsa lefutso la mahlo, boko le liphio
• Lefu la Wilson
• Lefu la meno, lefu le sa tloaelehang la liphatsa tsa lefutso la liphio

Ho batho ba baholo, Fanconi syndrome e ka bakoa ke lintho tse fapaneng tse senyang liphio, ho kenyelletsa:

• Meriana e meng, ho kenyeletsoa azathioprine, cidofovir, gentamicin, le tetracycline
• Ho kenyelletsa liphio
• Lefu la ho beha liketane tse bobebe
• Myeloma e mengata
• Amyloidosis ea mantlha

Matšoao a kenyelletsa:

• Ho fetisa moroto o mongata, o ka lebisang ho felloa ke metsi 'meleng
• Lenyora le feteletseng
• Bohloko bo boholo ba masapo
• Ho robeha hoa mmele ka baka la bofokodi ba masapo
• Bofokoli ba mesifa

Liteko tsa laboratori li ka bonts'a hore lintho tse ngata tse latelang li ka lahleha morong:

• Li-amino acid
• Bicarbonate
• Tsoekere
• Magnesiamo
• Phosphate
• Potasiamo
• Sodium
• Asiti ea Uric

Ho lahleheloa ke lintho tsena ho ka baka mathata a fapaneng. Liteko tse ling le tlhahlobo ea 'mele li ka bontša matšoao a:

• Ho felloa ke metsi ka lebaka la ho ntša metsi haholo
• Ho hloleha ho hola
• Osteomalacia
• Li-rickets
• Mofuta oa 2 renal tubular acidosis

Maloetse a mangata a fapaneng a ka baka lefu la Fanconi. Sesosa le matšoao a sona a lokela ho tšoaroa ka tsela e nepahetseng.

Phatlalatso e ipapisitse le lefu le ka sehloohong.

Bitsa mofani oa tlhokomelo ea bophelo haeba u na le ho felloa ke metsi 'meleng kapa bofokoli ba mesifa.

Lefu la De Toni-Fanconi-Debré

• Anatomy ea liphio

Bonnardeaux A, Bichet DG. Mathata a futsitsoeng a renal tubule. Ka: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, li-eds. Brenner le Rector ea Liphio. 11th ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 44.

Molaoli JW. Fanconi syndrome le mathata a mang a haufi le lefuba. Ho: Feehally J, Floege J, Tonelli M, Johnson RJ, bangoli. Kakaretso ea Nephrology ea Kliniki. La 6th. Philadelphia, PA: Elsevier; 2019: khaolo ea 48.