Recurrent, Atypical Anti-Glomerular Basement Membrane Disease.

Anti-glomerular basement membrane disease (GBM) (Goodpasture's disease) typically presents with acute manifestations of rapidly progressive glomerulonephritis often accompanied by lung haemorrhage. Anti-GBM disease is usually monophasic. However, atypical presentations with indolent renal involvement are being increasingly recognized. Herein we report a patient who presented with lung haemorrhage, minimal renal involvement, and negative result for serum anti-GBM antibody, while immunofluorescence examination of the renal biopsy provided the diagnosis leading to the institution right treatment with excellent response. Interestingly, he had presented 10 years earlier with lung hemorrhage, more significant renal involvement clinically and histologically, with positive serum anti-GBM antibody. The present case is intended to increase our awareness regarding the variable presentations of anti-GBM disease, such as with negative serology and recurrence of anti-GBM disease. The presentation of anti-GBM nephritis with non-proliferative, non-crescentic glomerulonephritis is also highlighted. The possible explanations for negative serum anti-GBM antibody are explored with a brief review of literature.

Open partial nephrectomy for a collision renal cell carcinoma in a transplant kidney: A case report.

A collision tumour of the kidney is a very rare condition defined by two immediately adjacent but histologically distinct neoplasms that coexist within one organ without histological admixture. We present a collision tumour in a transplant kidney treated with open partial nephrectomy. This case also highlights key surgical principles the enhanced risk of oncogenesis in transplant recipients, and some key principles for surgical resection of a tumour in a transplant kidney.

Optimal use of plasma and urine BK viral loads for screening and predicting BK nephropathy.

BACKGROUND: BK virus is a polyoma virus causing renal allograft nephropathy. Reduction of immunosuppression with the early recognition of significant BK viral loads in urine and plasma can effectively prevent BKV associated nephropathy (BKVN), however the optimal compartment and frequency of BK viral load measurement post renal transplantation are undetermined. Our purpose was to examine time to detection and viral loads in urine compared to plasma, and establish viral load cut-offs associated with histological BKVN. METHODS: We performed a retrospective analysis of the BKV screening frequency and compartment(s) of 277 adult renal transplant recipients (RTR). RESULTS: BKVN was histologically diagnosed in 17 (6.1 %) RTR. In cases where both urine and plasma were tested fortnightly for 6 months (n = 53), BKV was detected in the urine 29 days earlier than plasma. Fortnightly (n = 72) versus 3-monthly (n = 78) testing demonstrated that BKV was detected in the urine significantly earlier (median 63 versus 97 days, p = 0.001) and at a lower level (median 3.27 versus 6.71 log10 c/mL, p < 0.001) with more frequent testing, but this difference was not evident in plasma first detection (80 versus 95 days, p = 0.536) or first positive viral load (3.18 versus 3.30 log10 c/mL, p = 0.603). The optimum cut-off BK viral load for histological diagnosis of BKVN was 4.10 log10 c/mL for the first positive urine, 3.79 log10 c/mL for the first positive plasma, 9.24 log10 c/mL for the peak urine, and 4.53 log10 c/mL for the peak plasma. CONCLUSIONS: Frequent urinary BK viral load screening for the prevention of BKVN is suggested due to its high sensitivity and earlier detection.

Renal biopsy findings among Indigenous Australians: a nationwide review.

Australia's Indigenous people have high rates of chronic kidney disease and kidney failure. To define renal disease among these people, we reviewed 643 renal biopsies on Indigenous people across Australia, and compared them with 249 biopsies of non-Indigenous patients. The intent was to reach a consensus on pathological findings and terminology, quantify glomerular size, and establish and compare regional biopsy profiles. The relative population-adjusted biopsy frequencies were 16.9, 6.6, and 1, respectively, for Aboriginal people living remotely/very remotely, for Torres Strait Islander people, and for non-remote-living Aboriginal people. Indigenous people more often had heavy proteinuria and renal failure at biopsy. No single condition defined the Indigenous biopsies and, where biopsy rates were high, all common conditions were in absolute excess. Indigenous people were more often diabetic than non-Indigenous people, but diabetic changes were still present in fewer than half their biopsies. Their biopsies also had higher rates of segmental sclerosis, post-infectious glomerulonephritis, and mixed morphologies. Among the great excess of biopsies in remote/very remote Aborigines, females predominated, with younger age at biopsy and larger mean glomerular volumes. Glomerulomegaly characterized biopsies with mesangiopathic changes only, with IgA deposition, or with diabetic change, and with focal segmental glomerulosclerosis (FSGS). This review reveals great variations in biopsy rates and findings among Indigenous Australians, and findings refute the prevailing dogma that most indigenous renal disease is due to diabetes. Glomerulomegaly in remote/very remote Aboriginal people is probably due to nephron deficiency, in part related to low birth weight, and probably contributes to the increased susceptibility to kidney disease and the predisposition to FSGS.

Acute irreversible oxalate nephropathy in a lung transplant recipient treated successfully with a renal transplant.

We report a 29 year old male cystic fibrosis patient with end stage lung disease and normal renal function who underwent a sequential double lung transplant. Medical history included: an ileal resection and pancreatic exocrine dysfunction. The postoperative period was complicated with haemorrhage and repeat surgery, requiring multiple blood transfusions and extensive antibiotic cover. Pancreatic supplements were interrupted. Acute renal failure attributed to haemodynamically-mediated acute tubular necrosis was managed expectantly. He remained dialysis dependent 8 weeks post surgery and was maintained on triple immunosuppression with tacrolimus, mycophenolate and prednisolone. A DTPA study was consistent with ATN. Renal biopsy revealed features consistent with tubular injury due to acute oxalate nephropathy (AON). Further biochemical characterization excluded primary hyperoxaluria but confirmed increased 24 hour urinary oxalate. He was maintained on enhanced frequency HDF and subsequently received an uncomplicated live related renal transplant 10 months post lung transplant with only additional basiliximab. Calcium carbonate was continued to manage post transplant hyperoxaluria and an early renal biopsy excluded recurrent oxalate injury. Enteric hyperoxaluria due to malabsorption in patients with CF especially with ileal resection, in addition to loss of gut Oxalobacter formigenes due to prolonged antimicrobials, increases the risk of AON. Increased awareness of this condition and screening prior to lung transplant is recommended.

Sclerosing lipogranuloma of the penis.

We present a case of sclerosing lipogranuloma of the penis in a 25-year-old man of Burmese origin complicating injection of an unknown non-biodegradable oily foreign material into his external genitalia. Despite frequent complications, penile augmentation with exogenous paraffin material is still practised in some parts of the world. Sclerosing lipogranuloma is a rare condition in Australia that dermatologists need to consider in the differential of a genital ulcer or indurated penile mass, particularly in young men from South-East Asia. A causal relationship between the procedure and adverse events may not be made because complications are frequently delayed for many years. A high degree of clinical suspicion and a skin biopsy is essential, as a history of injection may not be disclosed.

CKD in Aboriginal Australians.

Chronic kidney disease (CKD) is one component of a spectrum of chronic disease in Aboriginal Australians. CKD is marked by albuminuria, which predicts renal failure and nonrenal natural death. Rates vary greatly by community and region and are much higher in remote areas. This reflects the heterogeneous characteristics and circumstances of Aboriginal people. CKD is multideterminant, and early-life influences (notably low birth weight), infections (including poststreptococcal glomerulonephritis), metabolic/hemodynamic parameters, and epigenetic/genetic factors probably contribute. CKD is associated intimately with cardiovascular risk. Albuminuria progresses over time, with a high incidence of new onset of pathologic levels of albuminuria in all age groups. All the usual morphologic findings are found in renal biopsy specimens. However, glomerular enlargement is notable in individuals from remote regions, but not those living closer to population centers. Glomerulomegaly probably represents compensatory hypertrophy caused by low nephron number, which probably underlies the accentuated susceptibility to renal disease. In the last decade, health care services have been transformed to accommodate systematic chronic disease surveillance and management. After a relentless increase for 3 decades, rates of Aboriginal people starting renal replacement therapy, as well as chronic disease deaths, appear to be stabilizing in some regions. Official endorsement of these system changes, plus ongoing reductions in the incidence of low birth weight and infections, hold promise for continued better outcomes.

Complete resolution of recurrent calciphylaxis with long-term intravenous sodium thiosulfate.

A 35-year-old morbidly obese woman on home haemodialysis presented with painful indurated subcutaneous nodules histologically characteristic of calciphylaxis. After failing to respond to conventional treatment, she was commenced on an intravenous infusion of 25 g of sodium thiosulfate three times per week. Two weeks after commencing sodium thiosulfate, the pain resolved completely. By 12 weeks, the lesions had healed and the infusions were ceased. Two months later, skin lesions recurred, but resolved again within 3 months of recommencement of sodium thiosulfate treatment, which was continued for 8 months. The treatment was well tolerated. There has been no recurrence of lesions in the 18 months since the cessation of sodium thiosulfate. Clinical trials to determine the optimum dose and duration of therapy for sodium thiosulfate treatment of calciphylaxis should be given priority because of its high rate of success in treating what is otherwise a severe and mostly lethal condition.