Journal of Clinical & Experimental Nephrology

Description

Kidney amyloidosis typically presents with nephrotic-range proteinuria. Rare cases of crescentic glomerulonephritis have been reported in patients with kidney amyloidosis but most cases were in the setting of patients with AA amyloidosis from long-standing inflammation and malignancy. We present a case of a previously healthy man in his 70s who was admitted with severe acute kidney injury, nephrotic-range proteinuria, and nephritic urinary sediment. Initial serologic testing for causes of rapidly progressive glomerulonephritis was negative. Kidney biopsy demonstrated the presence of active cellular and fibro cellular crescents with Congo red–positive staining in glomeruli and microvasculature on light microscopy and amyloid fibrils in glomerular basement membrane on electron microscopy. Urinary protein electrophoresis revealed monoclonal λ light chains, leading to a diagnosis of kidney AL amyloidosis, which was confirmed with bone marrow biopsy.

Combined Cryoglobulinemia Cause an Immune Complex-Mediated Glomerulonephritis

Our case illustrates that AL amyloidosis can present with findings suspicious for rapidly progressive glomerulonephritis and crescent formation on kidney biopsy specimens. Kidney injury related to infections is one of the important preventable causes of morbidity and mortality, especially in tropical regions. Kidney involvement in scrub typhus infection ranges from bland urinary sediments to the requirement of Renal Replacement Therapy (RRT). Earlier studies have shown renal histopathological findings consistent with acute tubular necrosis, interstitial nephritis, and mild meningeal glomerulonephritis in patients with scrub typhus and renal injury. Trichosporon colonizes the skin, vagina, gastrointestinal and respiratory tract of humans. Superficial infections are common, while disseminated trichosporonosis is rare, specifically seen among immunocompromised patients and often associated with high mortality. Molecular identification of the isolate was confirmed by sequencing IGS1 region of rDNA. Our study adds to a rather limited literature on renal complications of Trichosporonosis. Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease and usually involves the skin, musculoskeletal system, and kidneys. More than 30 genes have been to monogenic lupus, so far. Monogenic lupus is often characterized by an early-onset, similar family history, and syndromic appearance. Kidney amyloidosis commonly affords with nephrotic-variety proteinuria. Rare instances of crescentic glomerulonephritis have been pronounced in sufferers with kidney amyloidosis however maximum instances had been withinside the putting of sufferers with AA amyloidosis from long-status infection and malignancy. Our case illustrates that AL amyloidosis can gift with findings suspicious for hastily modern glomerulonephritis and crescent formation on kidney biopsy specimens. Kidney harm associated with infections is one in every of the essential preventable reasons of morbidity and mortality, especially in tropical regions. Kidney involvement in scrub typhus contamination tiers from bland urinary sediments to the requirement of renal substitute therapy (RRT). Earlier studies have proven renal histopathological findings regular with acute tubular necrosis, interstitial nephritis, and slight mesangial glomerulonephritis in sufferers with scrub typhus and renal harm. Trichosporon colonizes the pores and skin, vagina, gastrointestinal and breathing tract of humans. Superficial infections are common, even as disseminated trichosporonosis is uncommon, especially visible amongst immunocompromised sufferers and regularly related to excessive mortality. Our have a look at provides to a as an alternative constrained literature on renal complications of Trichosporonosis. Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune ailment and generally includes the pores and skin, musculoskeletal system, and kidneys. More than 30 genes were to monogenic lupus, so far. Monogenic lupus is regularly characterised with the aid of using an early-onset, comparable family history, and syndromic appearance.

Living Associated Transplantation Gives Doubtlessly Higher HLA Matching

Herein we gift a pediatric affected person with DNASE1L3 deficiency, tormented by each urticarial pores and skin lesions, recurrent hemoptysis, and renal involvement, in the end identified as this uncommon monogenic lupus. In anti-glomerular basement membrane glomerulonephritis antibodies and T cells directed in opposition to the Goodpasture antigen, the non-collagenous area of the α3-chain of kind IV collagen initiate renal infection ensuing in hastily progressing crescentic GN. Antibody remedy became additionally useless in a healing putting with pre-present autoantibodies and T cells. In conclusion, our outcomes indicate that even though the blockade of IL-6 impairs the improvement of autoimmunity in opposition to α3 (VI) NC1, this remedy does now no longer ameliorate crescentic GN each in a preemptive and a healing approach. Hepatitic C virus-related cryoglobulinemia and combined cryoglobulinemia may also cause an immune complex-mediated glomerulonepritis. Its remedy consists of RAAS (Renin Angiotensin Aldosterone System) inhibitors, steroids, mycofenolate mofetil, cyclophosphamide, or rituximab. Primary glomerulonephritis can recur after kidney transplantation and might jeopardize the survival of the renal allograft. The dangers of residing-associated kidney transplantation stay debatable on this institution of sufferers. Living associated transplantation gives doubtlessly higher HLA matching; consequently enhance the long-time period graft survival. However, the priority for elevated charges of recurrence of the number one glomerulonephritis withinside the transplanted kidney from residing associated donors complicates the choice of donors. This increases the query of whether or not sufferers with number one glomerulonephritis ought to acquire residing donor kidneys thru paired kidney trade packages to obtain the advantages of a residing donor kidney transplant even as additionally lowering the chance of recurrent glomerulonephritis. Our evaluation of the literature shows that even though the recurrence of number one glomerulonephritis happens greater regularly when donation happens from a residing associated donor in comparison to an unrelated donor, the graft survival benefit of residing associated donation is commonly maintained no matter the recurrence. We advocate that no matter the elevated chance of recurrence, residing associated donation ought to now no longer be averted in sufferers with number one glomerulonephritis because the motive in their end-level renal ailment.