Treatment-resistant PLA2R-negative membranous nephropathy responsive to low-density lipoprotein apheresis.

Idiopathic membranous nephropathy is the most common cause of nephrotic syndrome in nondiabetic adults. The antibody most often implicated is the M-type phospholipase A2 receptor (PLA2R) antibody, found in >70% of primary membranous nephropathy cases. First-line therapy is immunosuppressive in nature, but for patients who are treatment-resistant there is a significant risk of end-stage renal disease and mortality. Hypercholesterolemia is not only a side effect of nephrotic syndrome, but also its presence may worsen renal function. A recent single-arm observational study in Japan found that low-density lipoprotein apheresis (LDL-A) was able to ameliorate nephrotic syndrome in half of patients who were resistant to medication. We present a case of treatment resistant PLA2R negative membranous nephropathy who had significant improvement following two courses of LDL-A. To our knowledge, this is the first such reported case in the United States.

Primary epithelial tumours of the appendix in a black population: a review of cases.

AIM: To determine the prevalence, histologic types and clinical features of primary epithelial tumours of the vermiform appendix in a predominantly black population. METHODS: All cases of primary tumours of the appendix identified by review of the histopathology records at the University of the West Indies between January 1987 and June 2007 were selected. Relevant pathologic and clinical data were extracted with supplementation from patient charts where available. Non-epithelial tumours were excluded. The total number of appendectomy specimens over the period was also ascertained. RESULTS: Forty-two primary epithelial tumours were identified out of 6,824 appendectomies yielding a prevalence rate of approximately 0.62%. Well-differentiated neuroendocrine cell tumours (carcinoids, 47.6%) and benign non-endocrine cell tumours (adenomas, 45.2%) were most common with nearly equal frequency. The median age was 43 years, with no sex predilection. Carcinoid tumours occurred in younger patients (mean age 32 years), with a male-to-female ratio of 1.2:1. A clinical diagnosis of acute appendicitis was the most common reason for appendectomy (57.1%) and was histologically confirmed in 75% (18 of 24) of cases. In total, 16.7% of cases were diagnosed after incidental appendectomy. CONCLUSION: Appendiceal epithelial tumours are rare in our experience, and are represented principally by carcinoid tumours and adenomas. Carcinoid tumours occurred in younger patients but were slightly more common in men than women. Tumours were not suspected clinically and were diagnosed incidentally in specimens submitted for acute appendicitis supporting the need for histological evaluation in all resection specimens.