De novo thrombotic microangiopathy induced by cytomegalovirus infection leading to renal allograft loss

After kidney transplantation, thrombotic microangiopathy (TMA) can occur de novo or as recurrent disease. Risk factors for de novo posttransplant TMA include ischemia-reperfusion injury, immunosuppressive drugs, viral infections, acute humoral rejection, and complement gene abnormalities. Cytomegalovirus infection as a trigger for posttransplant TMA in kidney transplant recipients has only been reported in 7 cases, all of them between 4 weeks and 8 years after transplantation. We describe a new case of de novo TMA in association with cytomegalovirus infection 25 years after kidney transplantation.     

Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft

Amyloidosis results from the deposition of amyloid proteins in organ and tissues. It can be systemic or localized. Localized primary amyloidosis in renal allograft is an extremely rare clinical condition. We describe here a 46-year-old man who developed proteinuria and re-renal failure 5 years after receiving deceased renal allograft and proved to be a case of isolated primary AL amyloidosis in renal allograft confirmed by clinical investigations and renal biopsy.     

De novo renal carcinoma arising in non-functional kidney graft: a national retrospective study

International Urology and Nephrology - Characteristics of renal carcinoma arising in non-functional graft in renal transplant recipients (RTR) are unknown. We studied a large national retrospective...     

Schistosomal specific nephropathy leading to end-stage renal failure

In this study 17 patients, 11 with end-stage renal failure and six with nephrotic syndrome were selected. The selection criteria were presence of active intestinal schistosomiasis and absence of any surgical or other medical disease which could explain the renal disease. When examined by light microscopy, kidney biopsies showed membranoproliferative glomerulonephritis in nine, membranous in four, focal segmental glomerulosclerosis in two, sclerosing glomerulonephritis in one case, and no changes in another case. Direct immunofluorescence showed IgG deposits in 13 cases, IgM in 10 and different complement components (C3, C1q) in eight cases. Eluates from the kidney biopsies of the 17 schistosomal as well as six control cases were examined by ELISA against schistosoma mansoni adult worm antigen (AWA). This test showed the presence of antibodies against the AWA in 12 out of 17 of the schistosomal cases, and zero out of six of the controls. When examined by direct IFA using sheep anti-circulating anodic antigen/FITC and by indirect IFA using monoclonal antischistosomal CAA IgG3, kidney biopsies of the ELISA positive cases showed granular deposits of circulating anodic antigen (CAA). We conclude that schistosomal specific nephropathy does exist in the clinical settings and can lead to end-stage renal disease, with CAA probably being a major responsible antigen.     

Familial hypomagnesaemia — hypercalciuria leading to end-stage renal failure

Several disorders of hypomagnesaemia of hetary renal origin are now recognised. The cases of two sisters from a consanguineous marriage with the syndrome of renal magnesium wasting, hypercalciuria and nephrocalcinosis are presented. Pathological examination of the heterozygous parental kidneys revealed mild focal interstitial fibrosis. This condition is a previously unreported cause of end-stage renal failure in chilhhood, and this report suggests that transplantation from heterozygous parental donors can be successfully undertaken without recurrence currence of the syndrome.     

Ask-Upmark kidney and tubulointerstitial nephritis in a woman with severe renal failure

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered.     

A case of acute renal failure after exercise with renal hypouricemia demonstrated compound heterozygous mutations of uric acid transporter 1

Familial renal hypouricemia is a hereditary disease characterized by extraordinary high renal uric acid (UA) clearance and is associated with acute renal failure (ARF). A 17-year-old Japanese male developed ARF after anerobic exercise. Renal function improved completely after approximately 2 weeks of hydration treatment. After remission, hypouricemia became evident (1.0 mg/dL) from the initial level of UA (4.8 mg/dL) and fractional excretion of uric acid (FEUA) was >50%. His parents showed normal levels of UA and FEUA. Polymerase chain reaction of a urate anion exchanger known to regulate UA level [SLC22A12 gene: UA transporter 1 (URAT1)] demonstrated compound heterozygous mutations (Q297X and R90H). Thus, we describe a Japanese male with hypouricemia complicated by anerobic exercise-induced ARF, with definite demonstration of a genetic abnormality in the responsible gene, URAT1.     

Lymphoma-associated hemophagocytic syndrome: a rare cause of severe prerenal acute renal failure

We report on a 79-year-old woman having hemophagocytosis and capillary hyperpermeability syndrome who presented with anuric prerenal acute renal failure. The patient eventually died of a hypovolemic shock. Post-mortem biopsies evidenced a highly aggressive B cell intravascular lymphoma without amyloidosis. Physicians should be aware of the risk of anuric prerenal acute renal failure in the course of lymphoma-associated hemophagocytic syndrome.     

Maternal ingestion of diclofenac leading to renal failure in newborns

Background  

The use of non-steroidal anti-inflammatory drugs, such as indomethacin, ibuprofen, and nimesulide, during pregnancy has been reported to cause nephrotoxicity in the fetus. However, neonatal renal failure following antenatal exposure to diclofenac has not been reported in the literature. We report three cases of neonatal renal failure, including a pair of twins, following ingestion of diclofenac by the mother during pregnancy.     

Acute renal failure due to severe Landry-Guillain-Barre syndrome

Background: Blood urea nitrogen (BUN) >60 mg/dl has been reported to occur commonly in patient's with severe Landry-Guillain-Barré syndrome. Aims: To find out the cause for this high BUN we compared the renal function tests of 30 consecutive cases with severe Landry-Guillain-Barré syndrome to those of 30 controls. Results: Acute renal failure occurred in seven patients with Landry-Guillain-Barré syndrome and one of the control group. Acute renal failure was found more in cases with Landry-Guillain-Barré syndrome compared to controls (P=0.0049). Six out of seven cases with Landry-Guillain-Barré syndrome and acute renal failure had dysautonomia and became oliguric while being in a hypotensive state. Of 30 patients with Landry-Guillain-Barré syndrome seven cases died. From eight patients with dysautonomia six cases who had acute renal failure died. The mortality rate was higher in cases with dysautonomia and acute renal failure (P=0.0001 and 0.00001, respectively). Interestingly no glomerular disease was found. Conclusion: In conclusion acute renal failure can occur commonly in cases with severe Landry-Guillain-Barré syndrome particularly in those with dysautonomia, causing high mortality.     

Mild renal ischemia activates primed neutrophils to cause acute renal failure.

The role of neutrophils (PMN) in acute renal failure (ARF) is controversial. Although the development of acute renal failure (ARF) frequently occurs in situations where there is partial activation of PMN (primed PMN) and mild renal ischemia, the interaction between primed PMN and ischemic organs has not been studied in any biological system. To define the interaction between primed PMN and mild renal ischemia, kidneys were made ischemic for 10 minutes in situ and reperfused by the isolated kidney technique with untreated PMN or PMN primed with low concentrations of lipopolysaccharide (LPS) or phorbol myristate acetate (PMA). We found that primed PMN had no effect on control (non-ischemic) kidneys and that untreated PMN did not cause injury to kidneys previously subjected to mild ischemia. However, addition of primed PMN to mildly ischemic kidneys caused severe injury. To determine the nature of renal injury, ischemic kidneys were reperfused with primed PMN and catalase (CAT) or the elastase inhibitor, Eglin C. In ischemic kidneys reperfused with LPS-primed PMN, Eglin C (but not CAT) was partially protective while in ischemic kidneys reperfused with PMA-primed PMN, CAT (but not Eglin C) was partially protective. Reperfusion with both CAT and Eglin C completely prevented the damaging effects of either LPS- or PMA-primed PMN. In conclusion, addition of primed but not untreated PMN causes ARF in mildly ischemic kidneys by PMN oxidant- and/or protease-mediated mechanisms. This synergism could account for the high frequency of ARF in conditions associated with prerenal azotemia and primed PMN.     

Angiodysplasia as a cause of severe hematochezia in a child with end-stage renal failure

Angiodysplasia is a frequent cause of gastrointestinal bleeding in adults with chronic renal failure (CRF); however, there is no data about this association in children. The mechanism of this association is not known. We report a 4.5-year-old boy with CRF presenting with hematochezia due to colonic angiodysplasia. He was on hemodialysis for the previous 9 months. Treatment with argon plasma coagulation (APC) was commenced following a short course of octreotide therapy. During the 3 years of follow-up, no occult or gross bleeding occurred. This case illustrates that octreotide and APC therapy seems to be useful for arresting bleeding from angiodysplasia and prevention of recurrent bleeding in children with CRF.     

Hepatorenal syndrome: a severe, but treatable, cause of kidney failure in cirrhosis

Hepatorenal syndrome (HRS) is a unique type of kidney failure that occurs in advanced cirrhosis. It is characterized by functional impairment of the kidneys due to vasoconstriction of the renal arteries in the setting of preserved tubular function and absence of significant histologic abnormalities. Renal vasoconstriction in HRS is due to severe vasodilation of the splanchnic arteries associated with portal hypertension, leading to a decrease in effective arterial blood volume and arterial pressure. HRS commonly develops after a trigger, usually a bacterial infection, that disrupts the arterial circulation, but it also may occur spontaneously. There are 2 forms of HRS: type 1 is characterized by an acute progressive decrease in kidney function and very short survival without treatment, whereas type 2 features stable less severe kidney failure and longer survival compared with type 1. A liver transplant is the preferred treatment for HRS. Pharmacologic treatment with vasoconstrictors to reverse splanchnic vasodilation, together with albumin, is effective in 40%-50% of patients with type 1 HRS and improves survival. The drug of choice is the vasopressin analogue terlipressin. Renal replacement therapy should not be used as first-line therapy.     

The long-term prognosis of acute kidney injury: acute renal failure as a cause of chronic kidney disease

There is a widespread opinion that acute kidney injury (AKI) is a rather harmless complication and that survival is determined not by renal dysfunction per se, but by the severity of the underlying disease. This opinion is in sharp contrast to evidence from several recent experimental and clinical investigations indicating that AKI is a condition which exerts a fundamental impact on the course of the disease, the evolution of associated complications and on prognosis, independently from the type and severity of the underlying condition. In conclusion, severe AKI in the critically ill patient is associated with high rates of morbidity, mortality and consumption of health care resources.     

Retroperitoneal hematoma compressing a single functioning kidney: an unusual cause of obstructive renal failure

We report a case of a retroperitoneal hematoma occurring in a patient under anticoagulation therapy for deep-venous thrombosis and presenting as an anuric acute renal failure. A coexisting polycythemia vera led to misdiagnosis that could have been life-threatening. A woman, known for polycythemia vera and a single functioning right kidney, was admitted with mild abdominal pain in a context of recent deep venous thrombosis under low-molecular weight heparin. Clinical examination revealed hepatomegaly associated with polycythemia vera. Biochemical evaluation disclosed an acute renal failure, and renal ultrasonography showed no dilation of the renal pelvis. Retroperitoneal hematoma resulted in shock, progressive anemia and obstructive renal failure, related to renal pelvic compression. A right renal indwelling catheter was introduced to restore urine flow after one hemodialysis session, and an inferior vena cava filter was placed because of anti-coagulation contra-indication. However, pulmonary embolism occurred, so that oral anticoagulants were introduced. The hematoma resorbed spontaneously, and a year after this episode, the patient is still alive and well. Retroperitoneal hematoma is a rare cause of obstructive acute renal failure and a life-threatening complication of anti-coagulation therapy.