Renal Fanconi syndrome

Cutillas, P.R., Norden, A.G., Cramer, R., Burlingame, A.L., Unwin, RJ. (2003). Detection and analysis of urinary peptides by on-line liquid chromatography and mass spectrometry application to patients with renal Fanconi syndrome. Clin. Sci. (Lond.) 104, 483 490. 

Negro A, Regolisti G, Perazzoli F, et al Ifosfamide-induced renal Fanconi syndrome with associated... 

Galactose in blood and urine renal Fanconi syndrome... 

Generalized renal tubulopathy (renal Fanconi syndrome) + + + +... 

Wu C, Zhang H, Qian Y, Wang L, Gu X, Dai Z. Hypophosphatemic osteomalacia and renal Fanconi syndrome induced by low-dose adefovir dipivoxil a case report and literature review suggesting ethnic predisposition. J Clin Pharm Ther 2013 38(4) 321-6. 

This complex contains 11 polypeptide subunits of which only one is encoded by mtDNA. Defects of complex III are relatively uncommon and clinical presentations vary. Fatal infantile encephalomyopathies have been described in which severe neonatal lactic acidosis and hypotonia are present along with generalized amino aciduria, a Fanconi syndrome of renal insufficiency and eventual coma and death. Muscle biopsy findings may be uninformative since abnormal mitochondrial distribution is not seen, i.e., there are no ragged-red fibers. Other patients present with pure myopathy in later life and the existence of tissue-specific subunits in complex III has been suggested since one of these patients was shown to have normal complex 111 activity in lymphocytes and fibroblasts. 

Nephrotoxicity IDV potentially TDF Onset IDV—months after therapy TDF—weeks to months after therapy Symptoms IDV—asymptomatic rarely develop end-stage renal disease TDF—asymptomatic to symptoms of nephrogenic diabetes insipidus, Fanconi syndrome 1. History of renal disease 2. Concomitant use of nephrotoxic drugs Avoid use of other nephrotoxic drugs adequate hydration if on IDV monitor creatinine, urinalysis, serum potassium and phosphorus in patients at risk D/C offending agent, generally reversible supportive care electrolyte replacement as indicated... 

NS (acute) (general population) Renal Aminoaciduria Fanconi syndrome >80 (children) Chisolm 1962 Pueschel et al. 1972... 

Bl. Baber, M. D., A case of congenital cirrhosis of the liver with renal tubular defects akin to those in the Fanconi syndrome. Arch. Disease Childhood 31, 335-339 (1956). 

Benign Not evident while sole nutrition is breast milk Severe hypoglycemia and lactic acidosis after fructose ingestion Vomiting, apathy, diarrhea Liver damage and jaundice Proximal renal tubule disorder resembling Fanconi syndrome Treatment eliminate sources of fructose from diet... 

Renal function impairment Renal impairment, including cases of acute renal failure and Fanconi syndrome, has been reported in association with the use of tenofovir. Avoid tenofovir with concurrent or recent use of a nephrotoxic agent. Carefully monitor patients at risk for, or with a history of, renal dysfunction and patients... 

Adverse effects are not a major concern with the use of tenofovir. The occurrence of acute renal failure and Fanconi syndrome is rare. Lactic acidosis and severe hepatomegaly with steatosis, including fatal cases, have also been observed. 

Fanconi syndrome (metabolic acidosis secondary to malfunction of proximal renal tubules, resulting in urinary excretion of amino acids, glucose, phosphate, bicarbonate, uric acid, and other substances) secondary to longterm valproic acid has been described in an 8-year-old boy with severe developmental disability (1170). In a review of 10 previous reports of Fanconi syndrome secondary to long-term valproic acid therapy the authors found that all occurred at 4-14 years, all had taken valproic acid for 10 months to 10 years, and symptoms were fully reversible within 2-14 months after withdrawal of valproic acid. Most of the patients (9 of 11) were severely disabled, bedridden, or wheelchair-bound. 

Mori Y, lesato K, Ueda S, et al. 1984. Renal tubular disturbances induced by tributyl-tin oxide in guinea pigs A secondary Fanconi syndrome. Clin Nephrol 21 118-128. 

D22. de Toni, G., Renal rickets with phospho-glucoamino renal diabetes (de Toni-Debre-Fanconi Syndrome). Ann. Paediat. 187, 42-80 (1956). 

M3. McCune, D. J., Mason, H. H., and Clarke, H. T., Intractable hypophospha-temic rickets with renal glycosuria and acidosis (the Fanconi syndrome). Report of a case in which increased urinary organic acids were detected and identified, with a review of the literature. Am. J. Diseases Children 66, 81-146 (1943). 

R3. Robson, E. B., and Rose, G. A., The effect of intravenous lysine on the renal clearances of cystine, arginine and ornithine in normal subjects, in patients with cystinuria and Fanconi syndrome and in their relatives. Clin. Sci. 16, 75-93 (1957). 

Children and adolescents given cumulative doses of 32-112 g/m had only transient disturbances in renal function (9). In five children with renal tubular Fanconi syndrome caused by ifosfamide, all went on to develop rickets in the face of declining renal function. None had had pre-existing tubular damage and the syndrome developed at cumulative doses of ifosfamide of 39-99 g/m. There were low serum bicarbonate and phosphate concentrations, and supplementation of these resulted in bone healing but not renal recovery (10). 

Acquired Lignac-De Toni-Fanconi syndrome, with polyuria, polydipsia, glycosuria, aminoaciduria, hyper-phosphaturia, and hypercalciuria, was described in a number of patients treated with outdated tetracycline formulations. The degeneration products responsible for the toxic action are probably epitetracycline, anhydro-4-epitetracycline, and anhydrotetracycline (117), as similar renal damage was produced in rats with anhydro-4-epite-tracycline (118). 

Enuresis in children can occasionally be a problem (88). There have been single cases of Fanconi syndrome (SED-13, 151) (89), and hematuria with acute renal insufficiency (SEDA-20, 69). 

Verhelst D, Monge M, Meynard JL, Fouqueray B, Mougenot B, Girard PM, Ronco P, and Rossert J. 2002. Fanconi syndrome and renal failure induced by tenofovir a first case report. AmT/CdneyD/s 40 1331-1333. 

Malik, A, Abraham P, Malik N. Acute renal failure and Fanconi syndrome In an AIDS patient on tenofovir treatment - case report and review of the literature. J Infection 2005 51 e61-65. 

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