I-Fanconi syndrome

I-Fanconi syndrome ukuphazamiseka kwamashubhu ezinso lapho izinto ezithile ezijwayele ukungena egazini ngezinso zikhishelwa emchameni esikhundleni.

I-Fanconi syndrome ingabangelwa izakhi zofuzo ezinamaphutha, noma ingadala kamuva empilweni ngenxa yokulimala kwezinso. Kwesinye isikhathi imbangela yeFanconi syndrome ayaziwa.

Izimbangela ezivamile ze-Fanconi syndrome ezinganeni ukukhubazeka kwezakhi zofuzo okuthinta amandla omzimba wokudiliza izinhlanganisela ezithile ezifana nokuthi:

• I-cystine (i-cystinosis)
• I-Fructose (ukungabekezelelani kwe-fructose)
• IGalactose (galactosemia)
• I-Glycogen (isifo sokugcina i-glycogen)

ICystinosis iyimbangela ejwayelekile yeFanconi syndrome ezinganeni.

Ezinye izimbangela ezinganeni zifaka:

• Ukuvezwa kuzinsimbi ezisindayo njenge-lead, mercury, noma i-cadmium
• I-Lowe syndrome, ukuphazamiseka kofuzo okungavamile kwamehlo, kobuchopho, nezinso
• Isifo sikaWilson
• Isifo samazinyo, isifo esingajwayelekile sezakhi zofuzo

Kubantu abadala, i-Fanconi syndrome ingabangelwa izinto ezahlukahlukene ezilimaza izinso, kufaka phakathi:

• Imithi ethile, kufaka phakathi i-azathioprine, i-cidofovir, i-gentamicin, ne-tetracycline
• Ukufakelwa kwezinso
• Isifo sokubekwa kwamaketanga okukhanya
• I-myeloma eminingi
• I-amyloidosis eyinhloko

Izimpawu zifaka:

• Ukudlulisa inani elikhulu lomchamo, okungaholela ekuphelelweni amanzi emzimbeni
• Ukoma ngokweqile
• Ubuhlungu obukhulu bethambo
• Ukuqhekeka ngenxa yobuthakathaka bamathambo
• Ubuthakathaka bemisipha

Ukuhlolwa kwelabhoratri kungakhombisa ukuthi izinto eziningi kakhulu ezilandelayo zingalahleka emchameni:

• Ama-amino acid
• IBicarbonate
• Ishukela
• Magnesium
• I-Phosphate
• I-potassium
• Sodium
• Uric acid

Ukulahleka kwalezi zinto kungaholela ezinkingeni ezahlukahlukene. Ukuhlolwa okwengeziwe nokuhlolwa komzimba kungakhombisa izimpawu ze:

• Ukwehla kwamanzi emzimbeni ngenxa yokuchama ngokweqile
• Ukwehluleka kokukhula
• I-Osteomalacia
• Ama-Rickets
• Thayipha i-acidosis tubular 2 yezinso

Izifo eziningi ezahlukahlukene zingadala iFanconi syndrome. Imbangela eyisisekelo nezimpawu zayo kufanele ziphathwe njengokufanele.

Isibikezelo sincike kwisifo esiyisisekelo.

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma une-dehydration noma ubuthakathaka bemisipha.

Isifo sikaDe Toni-Fanconi-Debré

• Ukwakheka kwezinso

UBonnardeaux A, uBichet DG. Ukuphazamiseka okuzuzwe njenge-tubula yezinso. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ama-eds. IBrenner neRector's The Kidney. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 44.

Induna uJW. I-Fanconi syndrome nokunye ukuphazamiseka kwesifo se-tubule. Ku: Feehally J, Floege J, Tonelli M, Johnson RJ, abahleli. I-Nephrology Yomtholampilo Ephelele. Umhlaka 6. IPhiladelphia, PA: Elsevier; 2019: isahluko 48.