Renal Amyloid-Associated (AA) Amyloidosis in a Sickle Cell Patient: A Case Report and Literature Review.

Renal amyloid-associated (AA) amyloidosis is a rare occurrence in sickle cell disease (SCD). Very little literature is available on renal AA amyloidosis in sickle cell disease. Nephrotic range proteinuria is associated with higher mortality among patients with SCD. We present a case of a young reproductive-age African American woman who presented with massive nephrotic range proteinuria. Other more common causes of AA amyloidosis such as immunologic and infectious etiologies were ruled out by history, physical examination, radiologic investigation, and serology. Renal biopsy showed mesangial expansion with Congo red-positive material. Staining for immunoglobulins was negative. Electron microscopy showed nonbranching fibrils. These findings were consistent with AA amyloidosis. This case report adds to the rare findings of renal AA amyloidosis in sickle cell disease. The patient refused any intervention to decrease her Glomerular Filtration Rate (GFR) in the hopes of potentially reversing the disabling proteinuria. We report sickle cell disease presenting with nephrotic syndrome secondary to AA amyloid.

Complete Resolution of Paraneoplastic Membranous Nephropathy Following Curative Therapy of Triple-Negative Breast Cancer.

A paraneoplastic syndrome, which includes glomerulopathy, is a manifestation of malignancy unexplained by direct tumor burden. Membranous nephropathy (MN) may be associated with malignancies that are primarily solid tumors of the lung, prostate and gastrointestinal tract. It is rarely associated with breast cancer. To our knowledge, we herein report the first case of MN associated with triple-negative carcinoma of the breast. The patient initially presented with MN as a paraneoplastic nephrotic syndrome. Treatment resulting in a complete pathological response of the breast cancer also resolved the MN. Neither has recurred after a 48-month follow-up. The patient exhibited autoantibodies against phospholipase A2 receptor and was also antinuclear antibody (ANA) and anti-Smith (anti-Sm) antibody positive. These results suggest that the neoplasm evoked an autoimmune response, which resolved with treatment. ANA and anti-SM positivity closely correlated with the neoplasm activity supporting this hypothesis.

Collapsing Focal Segmental Glomerulosclerosis with Acute Interstitial Nephritis Associated with Plasmodium Falciparum: A Case Report and Review of the Literature.

BACKGROUND Malaria adversely affects the kidney in a variety of ways. The most common kidney injury is acute tubular necrosis, although various glomerular lesions are also described. Of these, collapsing focal segmental glomerulosclerosis (cFSGS) is the most rarely seen. Thus, the natural history of this lesion and response to treatment are not clear. Herein, we present a case of cFSGS complicated by acute interstitial nephritis caused by Plasmodium falciparum (P. falciparum) unresponsive to prednisone. CASE REPORT A 64-year-old Nigerian man with chronic kidney disease due to hypertensive nephropathy was admitted to the hospital, diagnosed with active P. falciparum malaria infection after returning from Nigeria. He developed acute kidney injury and nephrotic range proteinuria. Renal biopsy showed acute interstitial nephritis and cFSGS. Despite corticosteroid therapy, his kidney function worsened, requiring initiation of renal replacement therapy. This is the fifth case report of cFSGS due to malaria P. falciparum but the first to report the presence of acute interstitial nephritis in association with cFSGS due to malaria. CONCLUSIONS cFSGS is rarely seen as a manifestation of P. falciparum infection. When associated with acute interstitial nephritis, the prognosis seems to be worse. It appears that age and co-morbidities are the risk factors for unresponsiveness to corticosteroids, and treatment of the renal disease should focus on rapidly eradicating the parasitemia and providing supportive care. Our case report is the first to describe a combination of cFSGS and interstitial nephritis caused by P. falciparum unresponsive to corticosteroids.

Erythrocytosis is associated with intradialytic hypotension: a case series.

BACKGROUND: For patients with end stage renal disease undergoing hemodialysis, erythrocytosis occurs rarely. Erythrocytosis increases the risk of thrombosis, which is a common complication in hemodialysis patients. The risk of thrombosis may also be increased by hypotension. The purpose of our report is to examine the relationship between intradialytic hypotension and erythrocytosis. CASE PRESENTATION: We present a series of five patients with end stage renal disease and erythrocytosis (peak hemoglobin range 15.2-18.5 g/dL). All were erythropoiesis-stimulating agent naïve and non-smokers. Prior to developing erythrocytosis, each patient developed recurring episodes of intradialytic hypotension over several months. A statistically significant inverse correlation was observed between nadir intradialytic systolic blood pressure and hemoglobin concentration. In the index case, midodrine treatment resulted in resolution of the hypotension and erythrocytosis. Most of the patients had multiple acquired renal cysts, which is a potential source of erythropoietin. Four of the five cases developed arteriovenous dialysis access or deep venous thrombosis. CONCLUSIONS: An association between intradialytic hypotension and erythrocytosis was observed in five cases. We postulate that chronic intermittent hypotension and renal ischemia may lead to erythropoietin secretion, and this cascade could represent a newly recognized cause of secondary erythrocytosis.

Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report.

BACKGROUND: Non-Shiga toxin-associated hemolytic uremic syndrome is known to be caused by dysregulation of the alternative complement pathway. Infections, drugs, pregnancy, bone marrow transplantation, malignancy, and autoimmune disorders have all been reported to trigger episodes of atypical hemolytic uremic syndrome. To the best of our knowledge, there have been no previous reports of an association between diabetic ketoacidosis and atypical hemolytic uremic syndrome. CASE PRESENTATION: We describe a case of a 26-year-old Spanish man who presented with diabetic ketoacidosis and was found to have the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The patient had a normal ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity level, and his renal biopsy demonstrated predominant changes of diabetic glomerulosclerosis with an area compatible with thrombotic microangiopathy suggestive of superimposed atypical hemolytic uremic syndrome. Complement sequencing subsequently revealed a potential causative mutation in exon 12 of complement factor B with changes of lysine at amino acid position 533 to an arginine (CFB p.K533R). CONCLUSIONS: To the best of our knowledge, this is the first case report of diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult patient.

Hypercalcemia-Induced Hypokalemic Metabolic Alkalosis in a Multiple Myeloma Patient: The Risk of Furosemide Use.

Hypercalcemia is often seen in patients with malignancies, and in the past treatment for this has traditionally included loop diuretics. Clinically, patients with hypercalcemia frequently present with polyuria and volume contraction which may be further exacerbated by diuretic therapy. In the lab, hypercalcemia has been shown to activate the calcium-sensing receptor in the thick ascending limb of Henle and inactivate the 2 chloride sodium potassium co-transporter and induce a hypokalemic metabolic alkalosis, an effect similar to that of the loop diuretic furosemide. We now report what may well be the first clinical correlate of this laboratory finding in a patient who developed a hypokalemic metabolic alkalosis as a consequence of severe hypercalcemia due to multiple myeloma and whose metabolic derangement was corrected without the use of a loop diuretic which may have exacerbated the electrolyte abnormalities.