Patients with multiple myeloma (MM) often develop renal manifestations. The majority of cases present as cast nephropathy, amyloid light-chain (AL) amyloidosis, and monoclonal immunoglobulin deposition disease. AL amyloidosis usually involves the glomeruli, blood vessels, and interstitium. It is extremely uncommon to find isolated intratubular deposition of AL amyloid. Our patient presented with rapid worsening of renal function due to isolated intratubular deposition of AL amyloid, where the biopsy revealed amyloid proximal tubulopathy and amyloid cast nephropathy. Our case provides new insights into the complicated pathophysiology of the abnormal light chains in MM. This case is, to our knowledge, the second case of amyloid proximal tubulopathy reported in literature.
Collapsing glomerulopathy (CG) usually presents with reduced glomerular filtration rate, heavy proteinuria and has unfavorable prognosis. Numerous associations with CG are found. We encountered a case of CG associated with pulmonary tuberculosis presenting with proteinuria and dialysis-requiring severe renal failure. Our patient made partial recovery of his renal function and became dialysis-independent after antituberculous therapy and oral steroids. Long-term follow-up is needed to assess the progression of the disease.
Immunoglobulin A (IgA) dominant postinfectious glomerulonephritis (IgA PIGN) is a distinct clinical entity increasingly recognized in adult. It usually presents with reduced glomerular filtration rate, heavy proteinuria, and has unfavorable prognosis. Immunofluorescence study of renal biopsy specimens have IgA as dominant or codominant antibody. We encountered two cases of IgA dominant PIGN recently presenting as rapidly progr essive glomerulonephritis and managed conservatively. Both the patients are on follow-up and do not have complete recovery of renal function till date. Long-term follow-up is needed to assess the progression of the disease in these patients.
Dry weight assessment in dialysis patients remains a challenging endeavor owing to the limitations of the available methods for volume assessment. Lung ultrasound is emerging as an invaluable tool to assist in the appropriate assessment and assignment of dry weight. The objectives of this study are (1) to determine the reliability of clinical signs and symptoms for volume assessment, (2) to compare lung ultrasound with High Resolution Computed Tomography (HRCT) chest-A noninvasive gold standard tool for detecting pulmonary congestion and with inferior vena cava diameter (IVCD) – another time-tested volume assessment method, and (3) to analyze if lung ultrasound could detect dialysis induced fluid status variations. The cross-sectional study involves 50 patients on maintenance hemodialysis. Lung ultrasound for B line estimation and ultrasonographic measurement of IVCD performed before and after hemodialysis by a nephrologist trained in ultrasonography. Limited HRCT was obtained just before hemodialysis. Edema, crackles, and dyspnea had a poor sensitivity of 37.9%, 11.5%, and 52.6%, respectively, to detect clinically significant pulmonary congestion by lung ultrasound. A highly significant correlation was obtained between B-line score and HRCT signs of pulmonary congestion (
P
< 0.001) before dialysis. B lines showed statistically significant reduction with dialysis. The absolute reduction of B lines showed significant correlation with ultrafiltration volume and weight loss. Bedside lung ultrasound appears a sensitive tool for evaluating real-time changes in extravascular lung water and would serve to optimize volume status in dialysis patients.
Introduction:
Collapsing glomerulopathy (CG) is a distinct morphologic pattern of proliferative renal parenchymal injury. It differ from focal segmental glomerulosclerosis (FSGS) by clinicopathologic pattern and its adverse outcome. The clinical significance of CG in renal allograft biopsies is not yet clear due to scant data and less occurrence of CG in renal transplant recipients. We conducted this single-center retrospective study to evaluate the prevalence, clinicopathological features, and outcome of post renal transplant CG.
Subjects and Methods:
We studied 127 renal allograft biopsies performed over a period of 45 months (Jan 2015–Oct 2018). A diagnosis of CG was made if at least one glomerulus demonstrated global or segmental collapse of the glomerular capillary walls, associated marked hyperplasia, and hypertrophy of the overlying visceral epithelial cells. We analyzed clinical, biochemical, and pathological characteristics and its impact on renal allograft outcome. Statistical analysis was performed and continuous variables were expressed as means ± standard deviation (SD) or medians (interquartile range and noncontinuous data were expressed in percentage and numerical values.
Results:
The prevalence of CG was 5.3% (7/127) of allograft biopsies. Out of the seven patients, six patients had undergone live donor transplant and one patient had undergone deceased donor renal transplant. The native kidney disease was unknown in these patients except one (IgA nephropathy). The median duration of diagnosis for CG was 17 months after transplantation (range 5–132months). Presenting symptoms were pedal edema and hypertension in 71.4% (5) patients each. All patients had proteinuria of more than 1 gm and renal allograft dysfunction and median serum creatinine of 3.05 mg/dl (1.5–4.8 mg/dl). All patients received standard triple immunosuppression. Over a period of 2–20 months, 57.14% (4) patients developed a graft failure and 43% (3) of the other patients had functioning grafts with serum creatinine of 1.5–4.2 mg/dl.
Conclusions:
CG presents with moderate to severe proteinuria and may lead to rapid graft dysfunction and subsequent graft failure in most of the patients.
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