Use and outcomes of kidneys from donors with renal angiomyolipoma: A systematic review.

BACKGROUND: Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney. Although there is a rare possibility of malignant transformation of AML, this risk has not been studied in immunosuppressed patients. The safety of donors with AML and their kidney transplant recipients has not been well established. METHODS: A literature search was conducted utilizing MEDLINE, EMBASE, and Cochrane databases from inception through May 15, 2018 (updated on October 2019). We included studies that reported the outcomes of kidney donors with AML or recipients of donor with AML. The protocol for this meta-analysis is registered with PROSPERO (International Prospective Register of Systematic Reviews; no. CRD42018095157). RESULTS: Fourteen studies with a total of 16 donors with AML were identified. None of the donors had a diagnosis of tuberous sclerosis complex (TSC), pulmonary lymphangioleiomyomatosis (LAM), or epithelioid variant of AML. Donor age ranged from 35 to 77 years, and recipient age ranged from 27 to 62 years. Ninety-two percent of the donors were female. Only 8% were deceased donor renal transplant. The majority underwent ex vivo resection (65%) before transplantation, followed by no resection (18%), and the remaining had in vivo resection. Tumor size varied from 0.4 cm to 7 cm, and the majority (87%) were localized in the right kidney. Follow-up time ranged from 1 to 107 months. Donor creatinine prenephrectomy ranged 0.89-1.1 mg/dL and postnephrectomy creatinine 1.0-1.17 mg/dL. In those who did not have resection of the AML, tumor size remained stable. None of the donors with AML had end-stage renal disease or died at last follow-up. None of the recipients had malignant transformation of AML. CONCLUSION: These findings are reassuring for the safety of donors with AML (without TSC or LAM) as well as their recipients without evidence of malignant transformation of AML. As such, this can also positively impact the donor pool by increasing the number of available kidneys.

Epidemiology of cannabis use and associated outcomes among kidney transplant recipients: A meta-analysis.

OBJECTIVE: Cannabis is the most commonly used recreational drug in the United States, and transplant acceptability for cannabis using candidates varies among transplant centers. However, the prevalence and impact of cannabis use on outcomes of kidney transplant recipients remain unclear. This study aimed to summarize the prevalence and impact of cannabis use on outcomes after kidney transplantation. METHODS: A literature search was performed using Ovid MEDLINE, EMBASE, and The Cochrane Library Databases from inception until September 2019 to identify studies assessing the prevalence of cannabis use among kidney transplant recipients, and reported adverse outcomes after kidney transplantation. Effect estimates from the individual studies were obtained and combined utilizing random-effects, generic inverse variance method of DerSimonian-Laird. RESULTS: A total of four cohort studies with a total of 55 897 kidney transplant recipients were enrolled. Overall, the pooled estimated prevalence of cannabis use was 3.2% (95% CI 0.4%-20.5%). While the use of cannabis was not significantly associated with all-cause allograft failure (OR = 1.31, 95% CI 0.70-2.46) or mortality (OR = 1.52, 95% CI 0.59-3.92), the use of cannabis among kidney transplant recipients was significantly associated with increased death-censored graft failure with pooled OR of 1.72 (95% CI 1.13-2.60). CONCLUSIONS: The overall estimated prevalence of cannabis use among kidney transplant recipients is 3.2%. The use of cannabis is associated with increased death-censored graft failure, but not mortality after kidney transplantation.

Hepatitis A hospitalizations among kidney transplant recipients in the United States: nationwide inpatient sample 2005-2014.

BACKGROUND: This study aimed to evaluate the hospitalization rate for Hepatitis A virus (HAV) among kidney transplant (KTx) recipients and its outcomes as well as resource utilization. METHODS: The 2005-2014 National Inpatient Sample database was used to identify all hospitalized KTx recipients with an associated diagnosis of HAV. The hospital mortality, resource utilization, and associated liver conditions were compared between patients with and without HAV, adjusting for potential confounders. RESULTS: Of 871 024 KTx recipients identified, 204 had HAV. The overall inpatient prevalence of HAV in KTx recipients over 10 years in the United States was 23.42 cases per 100 000 admissions. There were no statistically significant changes in the inpatient prevalence of HAV in KTx recipients during the study period (P = 0.77), ranging from 9.2 to 34.3 per 100 000 admissions. Among hospitalized KTx recipients with HAV, 27.9% were from Northeast, 29.2% were from Midwest, 23.8% were from South, and 19.1% were from West. HAV was not significantly associated with increased hospital mortality, multiorgan failure, need for abdominal ultrasound, hospital length of stay, and total hospitalization costs and charges when compared with those without HAV. However, it is significantly associated with increased ICU stay, coexisting hepatitis B and C infection, and liver failure. CONCLUSION: Overall, inpatient prevalence of HAV in KTx recipients in the United States (years 2005-2014) was 23.42 cases per 100 000 admissions. Hospitalization for HAV after KTx is associated with increased ICU stay, coexisting hepatitis B and C infection, and liver failure.

Epidemiology of parvovirus B19 and anemia among kidney transplant recipients: A meta-analysis.

BACKGROUND: Persistent anemia has been described in kidney transplant (KTx) recipients with parvovirus B19 virus infection. However, the epidemiology of parvovirus B19 and parvovirus B19-related anemia after KTx remains unclear. We conducted this systematic review (1) to investigate the incidence of parvovirus B19 infection after KTx and (2) to assess the incidence of parvovirus B19 among KTx patients with anemia. MATERIALS AND METHODS: A systematic review was conducted in EMBASE, MEDLINE, and Cochrane databases from inception to March 2019 to identify studies that reported the incidence rate of parvovirus B19 infection and/or seroprevalence of parvovirus B19 in KTx recipients. Effect estimates from the individual studies were extracted and combined using random-effects, generic inverse variance method of DerSimonian and Laird. The protocol for this systematic review is registered with PROSPERO (no. CRD42019125716). RESULTS: Nineteen observational studies with a total of 2108 KTx patients were enrolled. Overall, the pooled estimated seroprevalence of parvovirus B19 immunoglobulin G was 62.2% (95% confidence interval [CI]: 45.8%-76.1%). The pooled estimated incidence rate of positive parvovirus B19 DNA in the 1st year after KTx was 10.3% (95% CI: 5.5%-18.4%). After sensitivity analysis excluded a study that solely included KTx patients with anemia, the pooled estimated incidence rate of positive parvovirus B19 DNA after KTx was 7.6% (95% CI: 3.7%-15.0%). Among KTx with anemia, the pooled estimated incidence rate of positive parvovirus B19 DNA was 27.4% (95% CI: 16.6%-41.7%). Meta-regression analysis demonstrated no significant correlations between the year of study and the incidence rate of positive parvovirus B19 DNA (P = 0.33). Egger's regression asymmetry test was performed and demonstrated no publication bias in all analyses. CONCLUSION: The overall estimated incidence of positive parvovirus B19 DNA after KTX is 10.3%. Among KTx with anemia, the incidence rate of positive parvovirus B19 DNA is 27.4%. The incidence of positive parvovirus B19 DNA does not seem to decrease overtime.

COVID-19 and Renal Failure: Challenges in the Delivery of Renal Replacement Therapy.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), first officially reported in December 2019 in Wuhan City, Hubei province, China, and has since lead to a pandemic. Most cases result in minor symptoms such as cough, fever, sore throat, myalgia, fatigue, nausea, diarrhea, loss of smell, and abdominal pain. As of April 8, 2020, more than 1,485,000 cases of COVID-19 have been reported in more than 200 countries and territories, resulting in over 90,000 deaths. Outcomes are worse in elderly patients, particularly males, and those with comorbidities, but can affect any age group. The incidence of acute kidney injury in patients with COVID-19 infection is about 3-15%; and in patients with severe infection requiring care in the intensive care unit, the rates of acute kidney injury increased significantly from 15% to 50%. Acute kidney injury is an independent risk factor for mortality in COVID-19 patients. The nephrologists, as well as intensivists, are facing immense daily challenges while providing care for these patients in the inpatient setting as well as end-stage renal disease patients on chronic dialysis in both inpatient and outpatient settings. In the current review article, we discussed the epidemiology and etiology of acute kidney injury, management of acute kidney injury including renal replacement therapy options (both hemodialysis and peritoneal dialysis) for inpatient floor, as well as intensive care unit settings. We also discussed the challenges faced by the outpatient dialysis units with COVID-19 infection. We discussed measures required to limit the spread of infection, as well as summarized the guidance as per the Centers for Disease Control and Prevention (CDC), American Society of Nephrology (ASN), American Society of Diagnostic and Interventional Nephrology (ASDIN) and the Vascular Access Society of the Americas (VASA).

Atypical Presentation of Novel Coronavirus Disease 2019 in a Peritoneal Dialysis Patient.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is a rapidly spreading disease causing increased morbidity and mortality across the globe. There is limited available knowledge regarding the natural history of the SARS-CoV-2 infection. Other factors that are also making this infection spread like a pandemic include global travelers, lack of proven treatment, asymptomatic carriers, potential reinfection, underprepared global health care systems, and lack of public awareness and efforts to prevent further spread. It is understood that certain preexisting medical conditions increase the risk of mortality with COVID-19; however, the outcome of this disease in traditionally vulnerable chronic illnesses such as end-stage renal disease is not well documented. We present a case of a 56-year-old African American lady with end-stage renal disease on the peritoneal dialysis who presented predominantly with nausea, vomiting, and subsequently found to have COVID-19. We use this case to illustrate an atypical presentation of the COVID-19 in a vulnerable patient and discuss the literature.

Medical Management of COVID-19: Evidence and Experience.

Coronavirus disease 2019 is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), and this infectious disease is termed COVID-19 in short. On a global scale, as of June 1, 2020, the World Health Organization (WHO) published statistics of 6,057,853 infected patients and 371,166 deaths worldwide. Despite reported observational data about the experimental use of certain drugs, there is no conclusively proven curative therapy for COVID-19 as of now; however, remdesivir received emergency use authorization (EUA) by the Food and Drug Administration (FDA) recently for use in patients hospitalized with COVID-19. There are several ongoing clinical trials related to the pharmacological choices of therapy for COVID-19 patients; however, drug trials related to observational studies so far have yielded mixed results and therefore have created a sense of confusion among healthcare professionals (HCPs). In this review article, we seek to collate and provide a summary of treatment strategies for COVID-19 patients with a variable degree of illness and discuss pharmacologic and other therapies intended to be used either as experimental medicine/therapy or as part of supportive care in complicated cases of COVID-19.

Serum Klotho in Living Kidney Donors and Kidney Transplant Recipients: A Meta-Analysis.

α-Klotho is a known anti-aging protein that exerts diverse physiological effects, including phosphate homeostasis. Klotho expression occurs predominantly in the kidney and is significantly decreased in patients with chronic kidney disease. However, changes in serum klotho levels and impacts of klotho on outcomes among kidney transplant (KTx) recipients and kidney donors remain unclear. A literature search was conducted using MEDLINE, EMBASE, and Cochrane Database from inception through October 2019 to identify studies evaluating serum klotho levels and impacts of klotho on outcomes among KTx recipients and kidney donors. Study results were pooled and analyzed utilizing a random-effects model. Ten cohort studies with a total of 431 KTx recipients and 5 cohort studies with a total of 108 living kidney donors and were identified. After KTx, recipients had a significant increase in serum klotho levels (at 4 to 13 months post-KTx) with a mean difference (MD) of 243.11 pg/mL (three studies; 95% CI 67.41 to 418.81 pg/mL). Although KTx recipients had a lower serum klotho level with a MD of = -234.50 pg/mL (five studies; 95% CI -444.84 to -24.16 pg/mL) compared to healthy unmatched volunteers, one study demonstrated comparable klotho levels between KTx recipients and eGFR-matched controls. Among kidney donors, there was a significant decrease in serum klotho levels post-nephrectomy (day 3 to day 5) with a mean difference (MD) of -232.24 pg/mL (three studies; 95% CI -299.41 to -165.07 pg/mL). At one year following kidney donation, serum klotho levels remained lower than baseline before nephrectomy with a MD of = -110.80 pg/mL (two studies; 95% CI 166.35 to 55.24 pg/mL). Compared to healthy volunteers, living kidney donors had lower serum klotho levels with a MD of = -92.41 pg/mL (two studies; 95% CI -180.53 to -4.29 pg/mL). There is a significant reduction in serum klotho levels after living kidney donation and an increase in serum klotho levels after KTx. Future prospective studies are needed to assess the impact of changes in klotho on clinical outcomes in KTx recipients and living kidney donors.

Rare but not beyond care: a young female with altered mental status and seizures.

A 40-year-old Caucasian lady with focal crescentic glomerulonephritis (p-ANCA) demonstrated by kidney biopsy, was treated with intravenous pulse steroids followed by weekly outpatient rituximab infusions (375 mg/m2). Five days after the fourth and final rituximab infusion, she developed headaches, altered mental status and seizures. Upon transfer to our facility, magnetic resonance imaging of the brain revealed cortical white matter changes suggestive of possible progressive multifocal leukoencephalopathy (PML) or posterior reversible encephalopathy syndrome (PRES). She was aggressively treated with antihypertensives, anti-seizure medications, intravenous steroids, plasmapheresis and ventilatory support while awaiting cerebrospinal fluid analysis and polymerase chain reaction on John Cunningham virus DNA. She had a complete recovery and, at 1 year follow up, was found to be doing well. Awareness of potential complications of rituximab therapy, such as PRES or PML is critical in providing appropriate treatment.

Epicardial Adipose Tissue and Renal Disease.

Epicardial adipose tissue (EAT) is derived from splanchnic mesoderm, localized anatomically between the myocardium and pericardial visceral layer, and surrounds the coronary arteries. Being a metabolically active organ, EAT secretes numerous cytokines, which moderate cardiovascular morphology and function. Through its paracrine and vasocrine secretions, EAT may play a prominent role in modulating cardiac function. EAT protects the heart in normal physiological conditions by secreting a variety of adipokines with anti-atherosclerotic properties, and in contrast, secretes inflammatory molecules in pathologic conditions that may play a dynamic role in the pathogenesis of cardiovascular diseases by promoting atherosclerosis. Considerable research has been focused on comparing the anatomical and biochemical features of EAT in healthy people, and a variety of disease conditions such as cardiovascular diseases and renal diseases. The global cardiovascular morbidity and mortality in renal disease are high, and there is a paucity of concrete evidence and societal guidelines to detect early cardiovascular disease (CVD) in this group of patients. Here we performed a clinical review on the existing evidence and knowledge on EAT in patients with renal disease, to evaluate its application as a reliable, early, noninvasive biomarker and indicator for CVD, and to assess its significance in cardiovascular risk stratification.

Alport's syndrome and intracranial aneurysm: mere coincidence or undiscovered causal relationship.

A 44-year-old Caucasian man with a history of deceased donor renal transplant for end-stage renal disease from Alport's syndrome (AS), presented with a spontaneous subarachnoid haemorrhage and hydrocephalus. Following an external ventricular drain for the hydrocephalus, a CT angiography revealed a dissection of the left vertebral artery extending into vertebro-basilar junction necessitating a bypass between left occipital artery to left posterior inferior cerebellar artery. He had a posterior fossa Craniectomy, C1 laminectomy and coiling off, of the left vertebral artery. Postprocedure course was prolonged but uneventful with complete recovery and normal renal function 18 months postpresentation. AS, a disease caused by abnormalities in the synthesis of type IV collagen, can cause aneurysms with severe and permanent neurological sequalae. We present a case of AS with intracranial arterial dissection with potential life-threatening consequences and discuss the genetic and molecular basis of AS along with review of the relevant literature.

Seizures, renal failure and acute respiratory failure: not your typical case of Henoch-Schonlein purpura.

A 30-year-old Caucasian woman with no prior medical history presented with pedal oedema, arthralgias and abdominal pain with diarrhoea, following a respiratory infection. She had mild abdominal tenderness along with a purpuric rash on the extremities and was anaemic. Following initial workup for anaemia and rash, her condition deteriorated with renal impairment, respiratory failure and seizures necessitating ventilatory support, dialysis and steroids. Serologies were negative, and skin biopsy showed leucocytoclastic vasculitis without vascular IgA deposition, and renal biopsy showed subendothelial, mesangial deposits of IgA with C3 indicative of Henoch-Schonlein purpura (HSP). She was treated with steroids, haemodialysis and on 6-month follow-up recovered renal function. We present the case to illustrate that HSP, though rare in adults, can present with multiorgan failure, with renal, pulmonary and central nervous system involvement, and the need for early diagnosis and prompt treatment for rapid clinical recovery.

Guillain-Barre syndrome with concurrent posterior reversible encephalopathy syndrome and hyponatraemia: mere coincidence or rare coexistence.

A 61-year-old Caucasian woman with a history of hypertension presented with a week's history of confusion falls and back pain was found to have hyponatraemia from secretion of antidiuretic hormone and treated appropriately. Given her persistent symptoms, despite a normal CT head on presentation, an MRI head was obtained, showing vasogenic oedema in line with posterior reversible encephalopathy syndrome (PRES). Despite aggressive antihypertensives and supportive measures, unfortunately, her condition deteriorated, with increased confusion, new left-sided flaccid paresis, paraesthesias and worsening of the back pain. Following further testing including a cerebrospinal fluid analysis, finally diagnosed with an atypical presentation of Guillain-Barre syndrome (GBS), and prompt management with intravenous immunoglobulins was initiated. She recovered clinically and returned to near-normal function on follow-up. We use this case to suggest the importance of dysautonomia in GBS and various clinical manifestations it can present with, including PRES and hyponatraemia.

Methamphetamine (N-methylamphetamine)-induced renal disease: underevaluated cause of end-stage renal disease (ESRD).

A 26-year-old Caucasian man with no medical history, except years of oral and intravenous drug abuse, presented with fatigue, shortness of breath, epistaxis and uncontrolled hypertension. He was pale with skin ecchymosis over his thighs and was anaemic, with severe renal failure and metabolic acidosis. Following initial clinical stabilisation of the patient, a renal biopsy was obtained, which showed vascular and glomerular changes consistent with thrombotic microangiopathic injury and advanced glomerulosclerosis. He was treated with antihypertensives and required haemodialysis. He admitted using 'crystal meth' regularly for many years, which is likely responsible for his renal failure. We present the case to illustrate methamphetamine-induced renal disease leading to end-stage renal disease and to bring awareness among practising clinicians, ancillary healthcare workers and public health professionals of this often undervalued cause of renal failure, which can be prevented.

Dialysis Disequilibrium Syndrome: Rare Serious Complication of Hemodialysis and Effective Management.

Dialysis disequilibrium syndrome (DDS) is an extremely rare central nervous system complication that occurs in patients receiving hemodialysis and can be potentially fatal. The prognosis is poor when this manifests with serious neurological manifestations like seizures and obtundation. Evidence in effective management is sparse. Herein, we present the case of a patient who developed seizures and altered mental status during hemodialysis. The patient successfully recovered with the administration of mannitol and 3% hypertonic saline without any long-term neurologic sequelae.

An Elderly Man with Syncope, Hypoxia, and Confusion: A Case Report and Review of Literature.

Controversies exist regarding the treatment of acute massive pulmonary embolism (PE) with anticoagulation alone or with thrombolytic therapy. Paradoxical embolism in the presence of a patent foramen ovale (PFO) is a rare but well-known entity and should always be looked for in case of a PE associated with systemic thromboembolism. We report a case of acute sub-massive PE treated with thrombolytic therapy in an elderly gentleman who had a paradoxical embolism and ischemic stroke as a result of a clot traversing through a PFO. We discussed diagnostic modalities, treatment of choice, and associated controversies in management.

A Rare Manifestation of a Rare Disease: Mantle Cell Lymphoma Presenting With Aseptic Meningitis.

Mantle cell lymphoma (MCL) is a rare form of non-Hodgkin lymphoma characterized by clonal proliferation of follicular mantle zone B lymphocytes. It is caused by abnormal chromosomal translocation t(11;14) resulting in aberrant expression of cyclin D1. This leads to activation of anti-apoptotic pathways and abnormal proliferation of MCL cells. Patients can present with an indolent course or a fulminant disease with short overall survival. The disease frequently involves extranodal organs, but rarely manifests with neurological symptoms. We report a rare case of aberrant CD5-negative MCL presenting with aseptic meningitis.

Adrenal Incidentaloma: Challenges in Diagnosing Adrenal Myelolipoma.

Adrenal myelolipomas (AMLs) are rare benign adrenal tumors, containing adipose and hematopoietic tissue, a result of reticuloendothelial cell metaplasia. Incidence on autopsy has been reported from 0.08% to 0.4%. AMLs are generally considered nonsecretory. The functional aspect of adrenal incidentaloma should be evaluated. In this article, we report a case of a 40-year-old male, who presented with uncontrolled hypertension and renal failure, with imaging revealing an adrenal incidentaloma. He was started on dialysis for acute fluid overload, and workup for pheochromocytoma revealed an elevated serum norepinephrine level of 1181 pg/mL. Free metanephrine and normetanephrine levels were low when checked pre- and post-dialysis. Complete resection of the encapsulated right adrenal mass was performed. Pathology of the adrenal tumor demonstrates an 11.5 × 9.5 × 7.5 cm well-circumscribed, partially encapsulated proliferation of mature adipose tissue with admixed hemopoietic elements consistent with myelolipoma weighing 29.3 g. This case highlights the inclusion of a full metabolic workup for all adrenal incidentalomas, including AML.

Acetazolamide Therapy in Patients with Heart Failure: A Meta-Analysis.

BACKGROUND AND OBJECTIVES: Fluid overload and central sleep apnea are highly prevalent in patients with heart failure (HF). We performed this meta-analysis to assess the effects of acetazolamide therapy on acid/base balance and apnea indexes. METHODS: A literature search was conducted using EMBASE, MEDLINE, and Cochrane Database from inception through 18 November 2017 to identify studies evaluating the use of acetazolamide in HF. Study results were analyzed using a random effects model. The protocol for this systematic review is registered with PROSPERO (International Prospective Register of Systematic Reviews; no. CRD42017065401). RESULTS: Nine studies (three randomized controlled trials and six cohort studies) with a total of 229 HF patients were enrolled. After acetazolamide treatment, there were significant decreases in serum pH (mean difference (MD) of -0.04 (95% CI, -0.06 to -0.02)), pCO2 (MD of -2.06 mmHg (95% CI, -3.60 to -0.53 mmHg)), and serum bicarbonate levels (MD of -6.42 mmol/L (95% CI, -10.05 to -2.79 mmol/L)). When compared to a placebo, acetazolamide significantly increased natriuresis (standardized mean difference (SMD) of 0.67 (95% CI, 0.08 to 1.27)), and decreased the apnea-hypopnea index (AHI) (SMD of -1.06 (95% CI, -1.75 to -0.36)) and central apnea index (CAI) (SMD of -1.10 (95% CI, -1.80 to -0.40)). Egger's regression asymmetry tests revealed no publication bias with p = 0.20, 0.75 and 0.59 for analysis of the changes in pH, pCO2, and serum bicarbonate levels with use of acetazolamide in HF patients. CONCLUSION: Our study demonstrates significant reduction in serum pH, increase in natriuresis, and improvements in apnea indexes with use of acetazolamide among HF patients.