scholarly journals Idiopathic Adult Onset Fanconi’s Syndrome in a Patient Treated with Oxaliplatin

2021 ◽  
Vol 8 (4) ◽  
Author(s):  
Nguyen A ◽  
◽  
Benz S ◽  
Keyword(s):  
Urine Analysis ◽  
Signs And Symptoms ◽  
Proximal Convoluted Tubule ◽  
Adult Onset ◽  
Tubular Injury ◽  
Fanconi’S Syndrome ◽  
Proximal Tubular ◽  
Fanconi's Syndrome ◽  
Electrolyte Replacement

Proximal tubular injury is known complication of chemotherapy such as carboplatin. However, there are far more other causes of injury and in many situations, the etiology is difficult to elucidate. Here we describe a case of a patient who presented with a urine analysis consistent of Fanconi’s syndrome with signs and symptoms of the disorder prior to chemotherapy but requiring admission for aggressive electrolyte replacement soon after the chemotherapy was completed. We further discuss causes of Fanconi’s syndrome and the importance of evaluation of the proximal convoluted tubule prior to administration of chemotherapy.

Disorders of tubular electrolyte handling

2020 ◽  
pp. 5112-5123
Author(s):  
Nine V.A.M. Knoers ◽  
Elena N. Levtchenko
Keyword(s):  
Serum Magnesium ◽  
Serum Phosphate ◽  
Magnesium Concentration ◽  
Main Site ◽  
Fanconi’S Syndrome ◽  
Tubular Transport ◽  
Bone Changes ◽  
Proximal Tubular ◽  
Magnesium Excretion ◽  
Fanconi's Syndrome

Glycosuria—glucose reabsorption in the proximal tubule is carried out by two different pairs of apical Na+-dependent (SGLT1 and -2) and basolateral Na+-independent (GLUT1 and -2) glucose transporters. Abnormalities in renal glucose transport can be seen in association with other defects of proximal tubular transport. Familial renal glycosuria is a rare autosomal recessive condition caused by mutations in the SGLT2-encoding gene, SLC5A2. Phosphate-handling disorders—the plasma concentration of inorganic phosphate depends on the balance between intestinal absorption, renal excretion, and the internal contribution from bone. Changes of serum phosphate levels can be caused by numerous inherited and acquired conditions. Disorders associated with increased urinary phosphate excretion and low serum phosphate levels produce symptoms that mainly affect the bones: rickets in children and osteomalacia in adults. Magnesium-handling disorders—normal plasma magnesium concentration is achieved by variation of urinary magnesium excretion in response to altered uptake by the intestine. The main site of magnesium absorption is the small bowel, via paracellular simple diffusion at high intraluminal concentrations, and via active transcellular uptake through the magnesium channel TRPM6 at low concentrations. Regulation and fine-tuning of serum magnesium concentration occurs primarily in the kidney. Genetic disorders of magnesium handling include Gitelman’s syndrome. Aminoaciduria and renal Fanconi’s syndrome—most amino acids (except for tryptophan, which is protein bound) are freely filtered by the glomerulus, after which 95 to 99.9% are reabsorbed in the proximal tubules by apical Na+-dependent cotransporters and Na+-independent cotransporters. Aminoaciduria is defined as urinary excretion of more than 5% of the filtered load of an amino acid. Renal Fanconi’s syndrome is characterized by a generalized defect of both Na+-coupled and receptor-mediated proximal tubular transport.

Unilateral nephrectomy and cisplatin as risk factors of ifosfamide-induced nephrotoxicity: analysis of 120 patients.

1994 ◽  
Vol 12 (1) ◽  
pp. 159-165 ◽  
Author(s):  
R Rossi ◽  
A Gödde ◽  
A Kleinebrand ◽  
M Riepenhausen ◽  
J Boos ◽  
...  
Keyword(s):  
Risk Factors ◽  
Renal Function ◽  
Unilateral Nephrectomy ◽  
Tubular Dysfunction ◽  
Schwartz Formula ◽  
Cytostatic Treatment ◽  
Fanconi’S Syndrome ◽  
Fractional Excretion Of Sodium ◽  
Proximal Tubular ◽  
Fanconi's Syndrome

PURPOSE This study was performed to identify risk factors of ifosfamide-induced renal damage. PATIENTS AND METHODS Renal function was assessed in 120 patients at a minimum of 3 months after completion of chemotherapy including ifosfamide. The cumulative ifosfamide dose ranged from 2 to 95 g/m2 (median, 30 g/m2). Ten patients had undergone unilateral nephrectomy; combination cytostatic treatment included cisplatin in 51 and methotrexate in 57. Sixty-eight patients had received gentamicin treatment. The glomerular filtration rate was estimated using the Schwartz formula. Proximal tubular function was assessed by the percent reabsorptions of glucose and 16 amino acids, the fractional excretion of sodium, and the fractional reabsorption of phosphate. In addition, the serum bicarbonate level was measured. RESULTS Proximal tubular dysfunction--with a predominance of renal amino acid (66.3%) and phosphate loss (38.3%)--was much more frequent than both glomerular impairment and acidosis. Seven patients were identified as having renal Fanconi's syndrome, and generalized tubulopathy was noted in another 15 patients. Ifosfamide-induced nephrotoxicity was dose-dependent, with a weak linear inverse correlation between cumulative ifosfamide dose and fractional phosphate reabsorption. Unilateral nephrectomy proved to be the single most important risk factor (odds ratio for the development of renal Fanconi's syndrome, 11.4), but cisplatin also significantly enhanced ifosfamide-mediated nephrotoxicity. Methotrexate, gentamicin, and patient age at primary diagnosis had no influence on renal function. CONCLUSION Ifosfamide chemotherapy should probably be restricted in patients after unilateral nephrectomy.

scholarly journals Intracellular prostaglandin E2 contributes to hypoxia-induced proximal tubular cell death

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Coral García-Pastor ◽  
Selma Benito-Martínez ◽  
Ricardo J. Bosch ◽  
Ana B. Fernández-Martínez ◽  
Francisco J. Lucio-Cazaña
Keyword(s):  
Hypoxia Inducible Factor ◽  
Renal Diseases ◽  
Prostaglandin E ◽  
Relevant Factor ◽  
Tubular Injury ◽  
Proximal Tubular Cells ◽  
Tubular Cells ◽  
Proximal Tubular ◽  
Cox 2 ◽  
Induced Apoptosis

AbstractProximal tubular cells (PTC) are particularly vulnerable to hypoxia-induced apoptosis, a relevant factor for kidney disease. We hypothesized here that PTC death under hypoxia is mediated by cyclo-oxygenase (COX-2)-dependent production of prostaglandin E2 (PGE2), which was confirmed in human proximal tubular HK-2 cells because hypoxia (1% O2)-induced apoptosis (i) was prevented by a COX-2 inhibitor and by antagonists of prostaglandin (EP) receptors and (ii) was associated to an increase in intracellular PGE2 (iPGE2) due to hypoxia-inducible factor-1α-dependent transcriptional up-regulation of COX-2. Apoptosis was also prevented by inhibitors of the prostaglandin uptake transporter PGT, which indicated that iPGE2 contributes to hypoxia-induced apoptosis (on the contrary, hypoxia/reoxygenation-induced PTC death was exclusively due to extracellular PGE2). Thus, iPGE2 is a new actor in the pathogenesis of hypoxia-induced tubular injury and PGT might be a new therapeutic target for the prevention of hypoxia-dependent lesions in renal diseases.

scholarly journals A Case of Fanconi's Syndrome due to Chinese Herb Nephropathy

2007 ◽  
Vol 30 (2) ◽  
pp. 101 ◽  
Author(s):  
Hye Sung Won ◽  
In Jeong Cho ◽  
Seung Hyun Yoo ◽  
Mi Na Yu ◽  
Dong Ryeol Ryu ◽  
...  
Keyword(s):  
Chinese Herb ◽  
Fanconi’S Syndrome ◽  
Fanconi's Syndrome

Evidence for electroneutral sodium chloride transport in rat proximal convoluted tubule

1986 ◽  
Vol 250 (4) ◽  
pp. F644-F648
Author(s):  
K. J. Howlin ◽  
R. J. Alpern ◽  
C. A. Berry ◽  
F. C. Rector
Keyword(s):  
Sodium Chloride ◽  
Active Transport ◽  
Sodium Transport ◽  
Chloride Transport ◽  
Proximal Convoluted Tubule ◽  
Organic Solutes ◽  
Nacl Transport ◽  
Proximal Tubular ◽  
High Chloride

One- to two-thirds of NaCl absorption in the late proximal convoluted tubule (no luminal organic solutes present) is inhibited by cyanide and thus is dependent on active transport. To examine whether this active transport-dependent NaCl transport is electrogenic or electroneutral, the effect of cyanide on transepithelial potential difference (PD) was measured in the rat proximal convoluted tubule microperfused in vivo. In the presence of an ultrafiltrate-like luminal perfusate containing glucose and alanine, cyanide addition caused the transepithelial PD to change from -0.44 +/- 0.04 to -0.05 +/- 0.03 mV (P less than 0.001). In the presence of a late proximal tubular fluid (high chloride, low bicarbonate, no organics), the transepithelial PD was 1.23 +/- 0.06 mV and was unchanged at 1.19 +/- 0.05 mV after cyanide addition (NS). To eliminate the possibility that an effect of cyanide on a putative acidification-dependent lumen-positive PD was concealing an effect on an electrogenic sodium transport-dependent lumen-negative PD, the above studies were repeated in the presence of acetazolamide. Cyanide did not affect the transepithelial PD (1.17 +/- 0.05 vs. 1.07 +/- 0.06 mV, NS). We conclude that, although cyanide-inhibitable NaCl transport is electrogenic in the presence of luminal organic solutes, it does not generate a transepithelial PD in their absence and therefore is electroneutral.

Ifosfamide, Fanconi's syndrome, and rickets.

1991 ◽  
Vol 9 (8) ◽  
pp. 1495-1499 ◽  
Author(s):  
C B Pratt ◽  
W H Meyer ◽  
J J Jenkins ◽  
L Avery ◽  
C P McKay ◽  
...  
Keyword(s):  
Total Dose ◽  
Side Effect ◽  
Fanconi’S Syndrome ◽  
Fanconi's Syndrome

Three of 218 children treated with ifosfamide plus the uroprotectant mesna, in single- or combination-agent protocols, have developed Fanconi's renal syndrome, all of whom were in a subgroup of 86 children who had also received cisplatin or carboplatin therapy. Patients receiving ifosfamide who have received prior cisplatin (or carboplatin) are at significantly higher risk of developing Fanconi's syndrome than are those who have received no prior nephrotoxic therapy (P = .04). The role of prior nephrotoxic therapy, including cisplatin and its derivatives, and the total dose of ifosfamide should be considered in the assessment of this rare but serious and apparently irreversible side effect.

scholarly journals The Incidence of Leukemia in Families of Patients with Hypoplasia of the Marrow

Blood ◽  
1959 ◽  
Vol 14 (9) ◽  
pp. 1008-1014 ◽  
Author(s):  
SERGIO GARRIGA ◽  
WILLIAM H. CROSBY
Keyword(s):  
Bone Marrow ◽  
Special Interest ◽  
Mesenchymal Origin ◽  
Fanconi’S Syndrome ◽  
High Incidence ◽  
Fanconi's Syndrome

Abstract The authors have reviewed the published cases of Fanconi’s syndrome of hypoplasia of the bone marrow. They have tabulated the features of the syndrome, pointing out that atrophy of the spleen is a common fault and may reflect a generalized dystrophy of the tissues of mesenchymal origin. Of special interest is the demonstration of a high incidence of leukemia in the families of patients with hereditary hypoplasia of the bone marrow.

scholarly journals Constitutional Anemia (Fanconi’s Syndrome) and Leukemia in Two Brothers

Blood ◽  
1955 ◽  
Vol 10 (8) ◽  
pp. 788-801 ◽  
Author(s):  
R. H. COWDELL ◽  
P. J. R. PHIZACKERLEY ◽  
D. A. PYKE
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
Short Stature ◽  
Left Kidney ◽  
The Other ◽  
Congenital Defects ◽  
Fanconi’S Syndrome ◽  
Bone Marrow Hypoplasia ◽  
Fanconi's Syndrome ◽  
Hypoplastic Anemia

Abstract Two brothers physically very alike became ill in their third decade, one with severe bone marrow hypoplasia and the other with acute leukemia. Both died and the autopsy findings are described. The two shared certain congenital defects, notably short stature with small heads, cutaneous pigmentation, pituitary and genital hypoplasia, and deformity of the thumbs. The brother with hypoplastic anemia also had aortic hypoplasia and his left kidney was absent. No previous report has been discovered of the association of Fanconi’s syndrome (of bone marrow hypoplasia with congenital defects) and leukemia with similar congenital defects in brothers. In two reports of leukemia affecting cousins of patients with Fanconi's syndrome, the leukemic cousins were without congenital defects.

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