Paraquat-induced Fanconi syndrome

The ingestion of paraquat, a non-selective herbicide, can be fatal in humans. Paraquat is toxic to multiple organs, including the kidney, heart, gastrointestinal tract and central nervous system. Although paraquat has been established as one cause of acute tubular necrosis, Fanconi syndrome presenting as severe hypophosphataemia after paraquat intoxication has not been reported. Here, we report the case of a 44-year-old Korean woman who presented with generalized proximal tubular dysfunction including aminoaciduria, phosphaturia and glycosuria after paraquat intoxication. We found that severe hypophosphataemia induces deep drowsiness. Renal biopsy findings indicated the presence of acute tubular necrosis that may be reversible.     

Acute renal failure following multiple wasp stings

We report the cases of two patients who developed acute renal failure following multiple wasp stings. Both patients required dialysis and recovered within 4 weeks. The kidney biopsy from one patient showed acute tubular necrosis with interstitial nephritis. One patient had complete recovery of renal function on follow-up, while the other was lost to follow-up.     

Acute kidney injury following multiple wasp stings

Background

Common manifestations of hypersensitivity reactions to toxins of stinging insects range from local swelling to angioedema and anaphylaxis. Sometimes it may result in unusual manifestations like intravascular hemolysis, disseminated intravascular coagulation, rhabdomyolysis, etc. Acute kidney injury (AKI) due to immune-mediated acute interstitial nephritis is an extremely uncommon manifestation of insect stings.

Case-Diagnosis/Treatment

A 9-year-old boy who developed renal failure from acute interstitial nephritis 7?days after getting stung by a swarm of wasps at multiple sites is described. He regained normal renal function after eight sessions of hemodialysis.

Conclusions

Acute interstitial nephritis resulting in AKI may be either due to immune-mediated tubulointerstitial injury or acute cellular injury caused by obstruction by pigments like hemoglobin and myoglobin. Timely and appropriate supportive management usually cures the patient without any residual damage. The objective of reporting this case is to draw the attention of fellow clinicians towards the possibility of this unusual but life-threatening delayed complication in multiple wasp stings, even if there are no significant immediate reactions.     

Acute renal failure following multiple hornet stings

Five patients who developed acute renal failure due to acute tubular necrosis following multiple hornet (Vespa orientalis) stings are described. All of them had intravascular hemolysis. Evidence for rhabdomyolysis was present in 2 patients. Two patients had elevated transaminase and alkaline phosphatase levels and in 1 of these, liver biopsy showed centrilobular necrosis. Two patients had thrombocytopenia in the absence of disseminated intravascular coagulation. Two patients died of infections while the remaining 3 recovered completely. Acute renal failure following multiple hornet stings appears to result mainly from intravascular hemolysis or rhabdomyolysis although a direct nephrotoxic effect of venom cannot be excluded.     

Saethre-Chotzen syndrome presenting with incomplete renal Fanconi syndrome

Here we report on a patient with findings of acrocephaly, craniosynostosis, low frontal hairline, ptosis of eyelids, deviated nasal septum, broad great toes, moderate hallux valgus, bilateral symmetrical complete soft tissue syndactyly of toes 2 and 3, and partial soft tissue syndactyly of toes 4 and 5 consistent with the diagnosis of Saethre-Chotzen syndrome. Additionally, the patient had some unusual findings as part of generalized dysfunction of the renal tubules including hypophosphatemia with renal phosphate wasting, normocalcemic hypercalciuria, hypomagnesemia with renal magnesium wasting, low-molecular-weight proteinuria, decreased serum PTH levels, osteopenia, and nephrolithiasis. In the light of these findings, the diagnosis of incomplete renal Fanconi syndrome was made. In conclusion, on the basis of the present findings, it is difficult to say whether renal tubular dysfunction are somehow connected to the Saethre-Chotzen syndrome or not. Therefore, we consider that this is probably just a coincident. However, further studies may show the connection between renal tubular dysfunction and Saethre-Chotzen syndrome.     

An Acadian variant of Fanconi syndrome

The Acadians were French settlers to Nova Scotia in the seventeenth century. In 1755, they were expelled by the British to various sites in the Americas, including Louisiana, where they are referred to as Cajuns. Many later migrated back to the Maritime Provinces of Canada. The objective of this study was to describe a series of pediatric patients representing an Acadian variant of Fanconi syndrome (AVFS). Nineteen children were diagnosed with AVFS between 1971 and 2006 and followed regularly. Data concerning demographics, growth, bone disease, and renal function at presentation and last observation were collected. The commonest reason for referral was assessment of genu valgum at 8.5 ± 4.2 years (mean ± SD) with hypophosphatemic rickets confirmed in all patients. Small-body habitus and short stature were confirmed in all patients. Therapy consisting of alkali replacement and phosphate and vitamin D supplements resulted in improvement of rickets and leg alignment but not stature (median height Z-score at presentation −2.05, range −3.6 to 0.21, vs. −2.05 at last observation, range −3.36 to 0.47). Creatinine clearance decreased (65.4 ± 24.6 vs. 48.0 ± 36.0 ml/min per 1.73 m², P < 0.05) and proteinuria increased (0.38 ± 0.25 vs. 1.46 ± 1.52 g/d, P < 0.05) during follow up of 8.4 ± 6.1 years. Chronic kidney disease developed in 50% by age 13 years. No extrarenal manifestations were identified, although two patients developed lethal pulmonary fibrosis postrenal transplantation. AVFS is characterized by rickets responsive to solute therapy, short stature, and loss of renal function, with progressive proteinuria with age.     

Angioimmunoblastic lymphadenopathy and transient Fanconi syndrome

A case of angioimmunoblastic lymphadenopathy (AIL) is presented in which the transient presence of a Fanconi syndrome was detected. Both the AIL and the Fanconi syndrome were ameliorated after steroid therapy.     

儿童范可尼综合征19例临床分析

范可尼综合征(FS)主要由于近端肾小管重吸收障碍而引起葡萄糖尿、全氨基酸尿、磷酸盐尿、碳酸氢盐尿等,亦可同时累及远端肾小管,伴发远端肾小管酸中毒.现对我院收治的19例儿童FS进行回顾性分析.     

Acute renal failure following massive attack by Africanized bee stings

Bee venom is a complex substance, which acts in several tissues. Although severe allergic reactions have occurred after one or more stings, several deaths have been reported without allergic manifestations, emphasizing the toxic effects of massive poisoning. A number of about 500 stings have been considered necessary to cause death by direct toxicity, but as few as 30-50 stings have proved fatal in children. Among the major toxic effects are hemolytic anemia, acute renal failure (ARF), and shock. ARF may be due to a common toxic-ischemic mechanism with hypovolemic or anaphylactic shock, pigment tubulopathy (myoglobinuria and hemoglobinuria), or acute tubular necrosis (ATN) from a direct kidney toxicity of the venom. We present a case of rhabdomyolysis and hemolysis with consequent ARF which developed after about 800 bee stings. The patient recovered completely after peritoneal dialysis.     

Light chain tubulopathy without Fanconi syndrome.

A 59-year-old white male was found to have mild proteinuriaand renal insufficiency during a life insurance test in 2003.His serum creatinine has been stable at 1.6 mg/dl since then.His 24 h urine protein was 720 mg in 2003 and 861     

Combined Alport syndrome,Klinefelter syndrome and Fanconi syndrome in a Chinese boy

Alport syndrome (AS) is a progressive renal disease characterized by hematuria and progressive renal failure. X-linked dominant (XLAS) is the major inheritance form, accounting for almost 80% of the cases, caused by mutations in COL4A5 genes. Klinefelter syndrome (KS) is the most common genetic cause of human male gonadal dysgenesis. AS and KS are both rare disease, there are only three cases of combined AS and KS in the literatures. Fanconi syndrome (FS) caused by AS is also very rare. We report here the first case combined AS, KS and FS in a Chinese boy. We suggest that the severe renal phenotype and FS might be due to the two homozygous COL4A5 variants in our boy, and cases of AS combined KS will be good research objects for X chromosome inactivation.     

Secondary renal Fanconi syndrome caused by valproate therapy

Although renal Fanconi syndrome resulting from valproate (VPA) has occasionally been reported, the detailed clinical characteristics of this disease remain unclear. To clarify the clinical features of patients with VPA-induced Fanconi syndrome, we analyzed the clinical and laboratory data of seven affected patients. All patients were children, were severely disabled and required tube feeding. Five patients required treatment with multiple anticonvulsant agents. Hypophosphatemia and hypouricemia were found in all patients. Mild proteinuria, increased excretion of urinary beta2-microglobulin (beta2MG) and generalized hyperaminoaciduria were present in all patients. The renal biopsy of one patient exhibited tubulointerstitial nephritis without any structural abnormalities of the mitochondria in proximal renal tubular cells. All patients recovered from the Fanconi syndrome after the cessation of VPA therapy without any long-term renal sequellae. These results indicate that young age and being severely disabled with tube feeding and anticonvulsant polytherapy are contributory factors to the development of VPA-induced Fanconi syndrome. Serum phosphate and uric acid concentrations and urinary beta2MG levels in addition to serum electrolytes and urinalysis should be examined regularly in patients receiving VPA therapy, especially in those with the contributory factors outlined above. Patients with Fanconi syndrome caused by VPA have a favorable renal outcome.     

Lowe's syndrome with Fanconi syndrome for ocular surgery: perioperative anesthetic considerations

Lowe's syndrome is a rare inherited metabolic disorder characterized by mental retardation, kidney malfunction, and abnormalities of the eyes and bones. A 4 month-old child with Lowe's and Fanconi's syndrome, undergoing bilateral congenital cataract surgery, is presented. Preoperative electrolyte imbalance was corrected by potassium, calcium, magnesium, phosphate, and bicarbonate supplementation. Anesthesia was administered uneventfully using appropriate anesthetic agents and monitoring. Adequate preoperative evaluation and optimization, along with selection of anesthetic agents and fluid and electrolyte management with appropriate perioperative monitoring, is key to a successful outcome.     

Nephrotic syndrome with mesangial proliferative glomerulonephritis induced by multiple wasp stings

We report the case of a young male who developed severe nephrotic syndrome within 2-3 weeks after being stung by 3 wasps. A percutaneous kidney biopsy specimen revealed mesangioproliferative glomerulonephritis with occasional subepithelial deposits suggestive of early membranous nephropathy. The patient was treated with oral prednisone 60 mg/day with no significant clinical response after 4 weeks, at which point he was started on oral cyclophosphamide, 100 mg/day, while the prednisone dose was tapered to 20 mg every other day over a 2-week period. Six months after initiation of cyclophosphamide, he still has severe nephrotic syndrome. We also briefly review the literature on Hymenoptera sting associated nephrotic syndrome.     

Late-onset downregulation of NaPi-2 in experimental Fanconi syndrome

The pathogenesis of renal phosphate (Pi) leak in Fanconi syndrome is unknown. Disorders of apical membrane transporters, leaky apical membrane, depleted cellular Pi and ATP, and impaired sodium (Na) pumps have been proposed as underlying defects. The present study examined the role of type II Na-Pi cotransport system (NaPi-2) in experimental Fanconi syndrome in rats. Following a single injection of maleic acid (MA), 75 mg/kg body weight IP, rats were sacrificed after 90 min, 4 h, and 24 h. Renal cortical expression of NaPi-2 mRNA was determined by Northern blotting, and brush border membrane (BBM) NaPi-2 protein by Western blotting. Increased urinary excretion of phosphate was demonstrated as soon as 90 min after MA injection, and was sustained at 4 and 24 h. NaPi-2 mRNA expression and NaPi-2 protein were not decreased after 90 min. NaPi-2 mRNA decreased after 4 h, while NaPi-2 protein decreased only at 24 h. Hence, the immediate phosphaturia in experimental Fanconi syndrome may be independent of NaPi-2 downregulation, possibly resulting from energy depletion or membrane dysfunction. The decrease in NaPi-2 mRNA expression and the subsequent NaPi-2 protein decrease may account for the second-phase phosphaturia. Received: 21 August 2000 / Revised: 18 December 2000 / Accepted: 22 December 2000