The Resolution of a Cerebrovascular Accident With Phlebotomy in a Hemodialysis Patient With Sickle Cell Disease.

Anemia is commonly observed in patients with end-stage renal disease (ESRD) on maintenance hemodialysis (HD) and can be quite severe, particularly when there is an additional comorbidity. With the use of erythropoietin-stimulating agents (ESAs), anemia is effectively treated, but the complete normalization of hemoglobin is not recommended since these agents increase the risk of thrombosis. With improvements in the therapy of sickle cell disease (SCD), patients now survive longer and may more frequently reach end-stage renal disease and require renal replacement therapy. Their anemia can be severe but does respond to ESAs. The goal hemoglobin in these patients is not established and likely should be lower than others on maintenance hemodialysis (HD) since SCD patients already have an increased risk of thrombosis, and the use of ESAs may exacerbate this risk. We present a 57-year-old African-American female with SCD on maintenance HD admitted with an acute cerebrovascular accident (CVA) that occurred in spite of the fact that her hemoglobin was within the accepted range for the general population on maintenance HD. Her neurologic status did not improve with blood pressure control and exchange transfusions, the suggested initial therapy for an acute CVA in a patient with sickle cell disease (SCD). However, with phlebotomy, the patient's symptoms rapidly improved when her hemoglobin was lowered and subsequently maintained with a lower dose of ESAs. Our experience suggests that the hemoglobin goal in SCD patients on maintenance HD should be lower than in other HD patients. The role of phlebotomy during an acute thrombotic event needs to be explored further.

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.

High Incidence of Thrombotic Thrombocytopenic Purpura Exacerbation Rate Among Patients With Morbid Obesity and Drug Abuse.

This study aims to identify the baseline patient characteristics, clinical presentation, and response to treatment of 11 patients who were diagnosed with thrombotic thrombocytopenic purpura (TTP) between 2014 and 2020 at Brookdale University Hospital Medical Center, Brooklyn, NY. Laboratory and clinical parameters were recorded for 29 patients who received plasmapheresis in this time period. Of 29 patients, 11 had confirmed TTP and one was diagnosed with hereditary TTP. Young, black, and female patients made up the majority of our patient population. A high prevalence of obesity and drug abuse were seen among our patients. Five out of 11 were obese and four of them were morbidly obese; six out of 11 patients were positive for the drug screen including cannabinoids (3), opiates (2), benzodiazepines (1), PCP (1), and methadone (1). Four patients with a positive drug screen had acute kidney injury (AKI), and plasmapheresis helped them enhance their kidney function. We observed a high incidence of AKI and high TTP exacerbation rates in patients who were drug abusers and those who were morbidly obese. There is a paucity of data on the relationship of TTP with obesityor drug abuse and this needs further study.

Hyponatremia and Encephalopathy in a 55-Year-old Woman with Syndrome of Inappropriate Antidiuretic Hormone Secretion as an Isolated Presentation of SARS-CoV-2 Infection.

BACKGROUND During the coronavirus disease 2019 (COVID-19) pandemic of 2020, varied presentations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been reported. The present report is of a case of hyponatremia and encephalopathy due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) as the main presentation of SARS-CoV-2 infection in a 55-year-old woman. CASE REPORT A 55-year-old woman with type II diabetes mellitus presented with confusion and slurring of speech, with a temperature of 38.5°C, heart rate of 120 bpm, blood pressure of 159/81 mmHg, and oxygen saturation of 98% on room air. She did not have edema on examination. Laboratory testing showed a low sodium level of 116 mEq/L (reference range, 135-145 mEq/L) with urine osmolarity of 364 mOsm/kg, urinary sodium of 69 mEq/L, urinary potassium of 15.6 mEq/L, and serum osmolarity of 251 mOsm/kg. The patient had normal serum thyroid-stimulating hormone and cortisol levels. A chest X-ray should no pulmonary infiltrates nor did a lumbar puncture reveal signs of infection. A real-time SARS-CoV-2 polymerase chain reaction assay was positive for COVID-19. Brain imaging with computed tomography was negative for acute infarct, intracranial hemorrhage, and mass effect. Based on findings from laboratory testing and physical examination, a diagnosis of SIADH was made. The patient was treated with 3% hypertonic saline, followed by salt tablets and fluid restriction, with improvement in her clinical symptoms and serum sodium level. CONCLUSIONS The present report is of a rare but previously reported association with SARS-CoV-2 infection. Encephalopathy and hyponatremia associated with SIADH without pneumonia or other symptoms of infection should be an indication for testing for SARS-CoV-2 infection.

Acute Kidney Injury in COVID-19 Patients: An Inner City Hospital Experience and Policy Implications.

BACKGROUND: Although diffuse alveolar damage and respiratory failure are the key features of coronavirus disease 2019 (COVID-19), the involvement of other organs such as the kidney has also been reported. The reports of the incidence of acute kidney injury (AKI) in COVID-19 patients vary widely. In this study, we report our unique experience with AKI in COVID-19 patients in a low socioeconomic and predominantly ethnic minority group and provide its incidence, risk factors, and prognosis to expand the current understanding of this complication. METHODS: In this single-center, retrospective cohort study, we analyzed the data of 469 COVID-19 patients admitted to the Brookdale University Hospital in Brooklyn, NY, from March 18 through April 23, 2020. Information regarding demographics, comorbidities, medications, clinical and laboratory data, and outcomes was collected from the electronic medical records. Both univariate and multivariate analyses were performed to determine the association of AKI with in-hospital mortality. RESULTS: The median age was 66 years (interquartile range [IQR] 25-75; range 19-101 years), and 268 (57.14%) patients were male. Estimated glomerular filtration rate (eGFR) as determined by the Modification of Diet in Renal Disease Study Equation was low (<60 mL/min/1.73 m2) in 207 (44.1%) patients. During hospitalization, 128 (27.3%) patients developed AKI, and the incidence was significantly higher in those patients presenting with a low eGFR (N = 81, 39.1%; p < 0.001). Male sex, hypertension, the use of angiotensin-converting enzyme inhibitors and non-steroidal anti-inflammatories, hemodynamic instability, mechanical ventilation, acute respiratory distress syndrome, and admission elevated ferritin, creatinine kinase, brain natriuretic peptide, and troponin 1 were identified as the risk factors for in-hospital AKI. Ninety-seven (28.45%) patients died in the non-AKI group versus 91 (71.1%) in the AKI group (p < 0.001). The Cox proportional hazard model after adjusting for age, gender, comorbidities, hemodynamic status, and PF ratio (arterial oxygen partial pressure [PaO2]/fractional inspired oxygen [FiO2]) determined that on admission, an elevated blood urea nitrogen (hazard ratio [HR]: 1.75; 95% confidence interval [CI] 1.23-2.48), a low eGFR (HR 1.43; CI 1.1-2.03), AKI stage 1 (HR 1.14; CI 0.64-2.03), AKI stage 2 (HR 1.86; CI 1.03-3.56), and AKI stage 3 (HR 2.1; CI 1.3-2.81) were independent risk factors for in-hospital mortality. Renal replacement therapy (RRT) did not improve survival in stage III AKI. CONCLUSION: AKI in our hospitalized COVID-19 patients was common and carried a high mortality, especially in patients with AKI stage 3. RRT did not improve survival. Policy changes and planning for this high incidence of AKI in COVID-19 patients and its associated high mortality are necessary at the local and national levels.

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.

Severe Symptomatic Hypermagnesemia Associated with Over-the-Counter Laxatives in a Patient with Renal Failure and Sigmoid Volvulus.

Hypermagnesemia is an uncommon but a potentially serious clinical condition. Over-the-counter magnesium containing products are widely used as antacids or laxatives. Although generally well tolerated in patients with normal renal function, their unsupervised use in the elderly can result in severe symptomatic hypermagnesemia, especially in those patients with concomitant renal failure and bowel disorders. We report a case of severe symptomatic hypermagnesemia associated with over-the-counter laxatives in a 70-year-old male patient with renal failure and sigmoid volvulus, who was successfully treated with hemodialysis.

Left ventricular geometry and renal function in hypertensive patients with diastolic heart failure.

BACKGROUND: The objective is to define the relationship between cardiac geometry and renal function in hypertensive subjects with and without diastolic heart failure (DHF). METHODS: This is a prospective observational study in a tertiary-care teaching institute in a 15-month period of consecutive hospitalized hypertensive patients. Patients on dialysis therapy or with atrial fibrillation, systolic heart failure, gross proteinuria, and glomerular diseases were excluded. Two-dimensional echocardiography was performed and stable glomerular filtration rate (GFR) was calculated by using the Modification of Diet in Renal Disease formula. Patients were classified into stage 1 to 5 chronic kidney disease (CKD). RESULTS: Five hundred forty hypertensive patients were separated into 2 groups: 286 patients with DHF and 254 patients without DHF. Mean age was 69.1 +/- 13.7 (SD) years in general. In patients with DHF, from stages 1 to 5 CKD, there was a significant graded increase in left ventricular mass index (from 117.3 to 162.4 g/m(2)) and relative wall thickness (from 0.42 to 0.52) and a significant graded decrease in aortic cusp separation (from 1.85 to 1.55 cm). Among echocardiographic variables, left ventricular mass index and relative wall thickness were associated inversely and aortic cusp separation was associated directly with GFR. In the absence of DHF, only left ventricular mass index was associated inversely with GFR, suggesting a prominent role of aortic cusp separation and relative wall thickness in the variability in GFR in patients with DHF through a hemodynamic disturbance. CONCLUSION: Hemodynamic alterations have a prominent role in the variability of GFR in patients with CKD with DHF. Adverse cardiac geometry is linked to the severity of CKD in hypertensive patients, raising the possibility of preserving both cardiac and renal function by means of hypertension control.

Independent and additive impact of blood pressure control and angiotensin II receptor blockade on renal outcomes in the irbesartan diabetic nephropathy trial: clinical implications and limitations.

Elevated arterial pressure is a major risk factor for progression to ESRD in diabetic nephropathy. However, the component of arterial pressure and level of BP control for optimal renal outcomes are disputed. Data from 1590 hypertensive patients with type 2 diabetes in the Irbesartan Diabetic Nephropathy Trial (IDNT), a randomized, double-blind, placebo-controlled trial performed in 209 clinics worldwide, were examined, and the effects of baseline and mean follow-up systolic BP (SBP) and diastolic BP and the interaction of assigned study medications (irbesartan, amlodipine, and placebo) on progressive renal failure and all-cause mortality were assessed. Other antihypertensive agents were added to achieve predetermined BP goals. Entry criteria included elevated baseline serum creatinine concentration up to 266 micromol/L (3.0 mg/dl) and urine protein excretion >900 mg/d. Baseline BP averaged 159/87 +/- 20/11 mmHg. Median patient follow-up was 2.6 yr. Follow-up achieved SBP most strongly predicted renal outcomes. SBP >149 mmHg was associated with a 2.2-fold increase in the risk for doubling serum creatinine or ESRD compared with SBP <134 mmHg. Progressive lowering of SBP to 120 mmHg was associated with improved renal and patient survival, an effect independent of baseline renal function. Below this threshold, all-cause mortality increased. An additional renoprotective effect of irbesartan, independent of achieved SBP, was observed down to 120 mmHg. There was no correlation between diastolic BP and renal outcomes. We recommend a SBP target between 120 and 130 mmHg, in conjunction with blockade of the renin-angiotensin system, in patients with type 2 diabetic nephropathy.

Atherosclerotic Renovascular Disease.

The patient, a 78-year-old Asian male, was brought to the hospital because of acute shortness of breath that had progressively worsened over the course of the day. He complained of a nonproductive cough and claudication after walking 1 block. His past medical history was significant for mild renal insufficiency (serum creatinine 1.5--2.0 mg/dl), the etiology of which was never explored. Although there was a recent history of mild to moderate hypertension, at presentation his blood pressure was noted to be 240/118 mm Hg in both arms. His physical exam at the time of admission was remarkable for grade II hypertensive retinopathy, an S4 gallop, periumbilical systolic bruits, audible femoral arterial bruits and absent distal lower extremity pulses. Initial complete blood count, serum electrolytes and cardiac enzymes (including lactate dehydrogenase) were normal. His blood urea nitrogen and serum creatinine concentrations were 51 and 3.6 mg/dl, respectively, and his urinalysis showed 1+ protein (both by dipstick and sulfasalicylic acid) with a "benign" sediment (0--1 WBC/HPE, 1--2 RBCs/HPF) with occasional granular casts. His electrocardiogram, apart from demonstrating left ventricular hypertrophy with secondary ST-T wave abnormalities, showed no acute changes; his chest X-ray demonstrated cardiomegaly and pulmonary vascular congestion. He was intubated and subsequently treated with increasing parenteral doses of furosemide (40--240 mg) and a nitroglycerine drip (up to 15 mcg/min). Over the course of the first 48 h, his blood pressure was gradually lowered to 170/100 mm Hg. His urine output increased from 20 ml/h to 125/ml/h, and his respiratory status improved, allowing him to be extubated. In spite of adequate control of his blood pressure in the ensuing days (150--170/80--90 mm Hg), his renal function continued to deteriorate. Renal sonography (without Doppler) demonstrated a right kidney of 9.6 cm and a left kidney of 9.3 cm in length without evidence of hydronephrosis. Both kidneys were noted to be echogenic. Assays for antinuclear antibodies and antineutrophilic cytoplasmic antibodies were negative, and the patient's serum complement levels were normal. For several days after his admission, his serum creatinine gradually rose to 10.7 mg/dl, and hemodialysis was initiated for uremic encephalopathy. Because of the high index of suspicion for renal artery stenosis as the case of both his hypertension and renal failure, a renal angiogram was performed. It revealed a 90% occlusion of the right renal artery with ostial involvement and a 70% occlusion of the left renal artery; both kidneys had poor distal renal vasculature and there was marked atherosclerotic disease of the aorta. After being hemodialyzed for 3 treatments, his renal function began to improve spontaneously. His serum creatinine returned to 3.4 mg/dl, and a subsequent 24-hour urine demonstrated a creatinine clearance of 20 ml/min and an excretion of 1.2 g of protein. Following his discharge from the hospital, his renal function remained unchanged for 3 years, and his blood pressure was easily controlled on monotherapy with a long-acting calcium channel blocker. He recently died from pneumonia.