Fanconi Syndrome (Syndrome, Fanconi)

A hereditary or acquired form of generalized dysfunction of the PROXIMAL KIDNEY TUBULE without primary involvement of the KIDNEY GLOMERULUS. It is usually characterized by the tubular wasting of nutrients and salts (GLUCOSE; AMINO ACIDS; PHOSPHATES; and BICARBONATES) resulting in HYPOKALEMIA; ACIDOSIS; HYPERCALCIURIA; and PROTEINURIA.
Also Known As:
Syndrome, Fanconi; Fanconi Renotubular Syndrome; Idiopathic De Toni-Debre-Fanconi Syndrome; Neonatal De Toni-Debre-Fanconi Syndrome; Primary Toni-Debre-Fanconi Syndrome; Toni-Debre-Fanconi Syndrome; De Toni Debre Fanconi Syndrome; Idiopathic De Toni Debre Fanconi Syndrome; Lignac Fanconi Syndrome; Neonatal De Toni Debre Fanconi Syndrome; Primary Toni Debre Fanconi Syndrome; Syndrome, De Toni-Debre-Fanconi; Syndrome, Fanconi Renotubular; Syndrome, Lignac-Fanconi; Toni Debre Fanconi Syndrome; Toni-Debre-Fanconi Syndrome, Primary; De Toni-Debre-Fanconi Syndrome; Lignac-Fanconi Syndrome; Proximal Renal Tubular Dysfunction; Renal Fanconi Syndrome
Networked: 527 relevant articles (12 outcomes, 36 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cystinosis
2. Renal Tubular Acidosis (Distal Renal Tubular Acidosis)
3. Disease Progression
4. Osteomalacia
5. Glycosuria


1. Gahl, William A: 7 articles (06/2015 - 12/2003)
2. Levtchenko, Elena N: 6 articles (01/2015 - 11/2005)
3. Norden, Anthony G W: 6 articles (02/2012 - 01/2002)
4. Kleta, Robert: 6 articles (02/2011 - 08/2002)
5. Unwin, Robert J: 6 articles (08/2007 - 01/2002)
6. van den Heuvel, Lambertus P: 5 articles (09/2013 - 11/2005)
7. Cutillas, Pedro R: 5 articles (02/2012 - 05/2003)
8. Watanabe, Toru: 5 articles (06/2005 - 01/2002)
9. Sarwal, Minnie M: 4 articles (03/2015 - 01/2010)
10. Sansanwal, Poonam: 4 articles (03/2015 - 01/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fanconi Syndrome:
1. maleic acid (maleate)IBA
2. Cystine (L-Cystine)IBA
3. Cysteamine (Mercaptamine)FDA Link
4. Uric Acid (Urate)IBA
5. CalciumIBA
6. Glucose (Dextrose)FDA LinkGeneric
7. Carnitine (L-Carnitine)FDA LinkGeneric
8. PotassiumIBA
08/01/2010 - "Urine potassium levels were elevated, and the transtubular potassium gradient (TTKG) was 6.6, consistent with hypokalemic RTA with associated Fanconi syndrome, which presented as hyperphosphaturia, uricaciduria, and loss of protein and sugar in the urine. "
01/01/1971 - "The mechanism of renal potassium wasting in renal tubular acidosis associated with the Fanconi syndrome (type 2 RTA) was investigated in 10 patients, each of whom had impaired proximal renal tubular reabsorption of bicarbonate as judged from a greater than 15-20% reduction of renal tubular bicarbonate reabsorption (THCO(3) (-)) at normal plasma bicarbonate concentrations. "
01/01/1971 - "On the mechanism of renal potassium wasting in renal tubular acidosis associated with the Fanconi syndrome (type 2 RTA)."
03/01/1971 - "IN TWO PATIENTS WITH CLASSIC RENAL TUBULAR ACIDOSIS (RTA) AND IN TWO PATIENTS WITH RTA ASSOCIATED WITH THE FANCONI SYNDROME, RENAL POTASSIUM WASTING PERSISTED DESPITE SUSTAINED CORRECTION OF ACIDOSIS: (a) during moderate degrees of hypokalemia, daily urinary excretion of potassium exceeded 80 mEq in each patient; (b) during more severe degrees of hypokalemia, daily urinary excretion of potassium exceeded 40 mEq in two patients and 100 mEq in another. "
09/01/2005 - "Laboratory studies showed profound hypokalemia with renal potassium (K) wasting, hyperchloremic metabolic acidosis, hypophosphatemia with hyperphosphaturia, hypouricemia with hyperuricosuria, and glycosuria, consistent with Fanconi syndrome. "
9. IfosfamideFDA LinkGeneric
10. Amino AcidsFDA Link

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Kidney Transplantation
3. Transplants (Transplant)
4. Aftercare (After-Treatment)
5. Transplantation (Transplant Recipients)