A Contrast Frugal Approach to Transcatheter Aortic Valve Replacement in Chronic Kidney Disease: A Pilot Study.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is now regarded as a viable treatment option for all cases of severe aortic stenosis (AS). Acute kidney injury (AKI) is common and lowers the survival of patients after TAVR and iodine-based contrast-induced nephropathy (CIN) plays a significant adverse role in AKI. Therefore, in chronic kidney disease (CKD) patients requiring pre-operative evaluation for TAVR, the risk of CIN is of particular concern. METHODS: It was a single-center study including eight CKD patients who underwent pre-operative evaluation for TAVR with minimized contrast exposure by means of pre-operative contrast-sparing evaluation and intra-operative contrast minimization. All patients had glomerular filtration rate (eGFR) calculated before TAVR and on a follow-up about one month and one year post-operatively to document the impact of this TAVR protocol on prognosis of kidney function in patients with advanced CKD. RESULTS: New York Heart Association (NYHA) functional classification demonstrated significant improvement of symptomatology (p = 0.0001) by one-year post-TAVR. Patients' mean AS gradient was significantly improved (p = 0.00004) after the TAVR procedure. No significant post-operative paravalvular aortic regurgitation was noted on follow up echocardiogram. eGFR data showed mean eGFR for the group was slightly better (27.38 ml/min/per 1.73 m2 BSA vs. 30.38 ml/min/per 1.73 m2 BSA) after TAVR. CONCLUSIONS: "Contrast frugal" approach is feasible and safe for pre-TAVR evaluation and the procedure itself. Our pilot study showed no significant paravalvular leak of the prosthetic valve following this proposed protocol. No statistically significant decrease in eGFR was noted on a one-year follow-up.

Transition of Renal Patients Using AlloSure Into Community Kidney Care (TRACK): Protocol for Long-Term Allograft Surveillance in Renal Transplant Recipients.

BACKGROUND: Patients with end-stage kidney disease require complex and expensive medical management. Kidney transplantation remains the treatment of choice for end-stage kidney disease and is considered superior to all other modalities of renal replacement therapy or dialysis. However, access to kidney transplant is limited by critical supply and demand, making it extremely important to ensure longevity of transplanted kidneys. This is prevented through lifelong immunosuppression, with caution not to overly suppress the immune system, resulting in toxicity and harm. Transition of care to community nephrologists after initial kidney transplantation and monitoring at a transplant center is an important process to ensure delivery of effective and patient-centric care closer to home. Once transplanted, laborious surveillance of the immune system and monitoring for potential rejection and injury are undertaken through an armamentarium of screening modalities. Posttransplant surveillance for kidney function and injury remains key to follow-up care. While kidney function, quantified by estimated glomerular filtration rate and serum creatinine, and kidney injury, measured by proteinuria and hematuria, are standard biomarkers used to monitor injury and rejection posttransplant, they have recently been demonstrated to be inferior in performance to that of AlloSure (CareDx Inc, Brisbane, CA) circulating donor-derived, cell-free DNA (dd-cfDNA). OBJECTIVE: The outcomes and methods of monitoring renal transplant recipients posttransplant have remained stagnant over the past 15 years. The aim of this study is to consider intensive surveillance using AlloSure dd-cfDNA in an actively managed protocol, assessing whether it increases long-term allograft survival in kidney transplant recipients compared with current standard clinical care in community nephrology. METHODS: The study protocol will acquire data from a phase IV observational trial to assess a cohort of renal transplant patients managed using AlloSure dd-cfDNA and patient care managers versus 1000 propensity-matched historic controls using United Network for Organ Sharing U.S. Scientific Registry of Transplant Recipients data. Data will be managed in a centralized electronic data server. The primary outcome will be superior allograft survival, as a composite of return to dialysis, retransplant, death due to allograft failure, and death with a functional graft (infection, malignancy, and cardiovascular death). The secondary endpoints will assess improved kidney function through decline in estimated glomerular filtration rate and immune activity through development of donor-specific antibodies. RESULTS: The total sample is anticipated to be 3500 (2500 patients managed with AlloSure dd-cfDNA and 1000 propensity-matched controls). Active enrollment began in November 2020. CONCLUSIONS: Based on a significant literature base, we believe implementing the surveillance of dd-cfDNA in the kidney transplant population will have a positive impact on graft survival. Through early identification of rejection and facilitating timely intervention, prolongation of allograft survival versus those not managed by dd-cfDNA surveillance protocol should be superior. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/25941.

Thrombocytopenia: A Diagnostic Dilemma and Incidental Detection of Systemic Lupus Erythematosus in a Middle-Aged Asian Male.

Systemic lupus erythematosus is a multisystem disorder much more common in females than males due to the effect of the hormone estrogen. There are also specific differences in clinical presentation in men and women. We present a unique case of a 54-year-old middle-aged Asian male presenting with only generalized weakness without other systemic features and with only incidental finding of thrombocytopenia. Notable laboratory values were positive for antinuclear antibody (ANA) and anti-double-stranded DNA (dsDNA), low complement 3 level with normal complement 4 levels, along with severe thrombocytopenia and mild anemia. The patient was eventually diagnosed with systemic lupus erythematosus based on these parameters. Bone marrow biopsy revealed an increased number of megakaryocytes without hypocellular or hypercellular marrow and no dysplasia of cell lines. He was initiated on oral prednisone, and his symptoms recovered remarkably with normalization of lab values upon discharge. The case's importance lies in the fact that the diagnosis of lupus can be missed in male patients with nonspecific clinical features due to certain differences in presentation from females. This diagnosis should be included in the workup of any thrombocytopenia.

Acute Kidney Injury in a Case Series of Patients with Confirmed COVID-19 (Coronavirus Disease 2019): Role of Angiotensin-Converting Enzyme 2 and Renin-Angiotensin System Blockade.

The renin-angiotensin system plays a very critical role in hypertension, diabetes, and kidney and heart diseases. The blockade of the renin-angiotensin system results in the prevention of progression of renal and cardiac damage. There have been controversial hypotheses raised regarding the safety of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers in COVID-19 (coronavirus disease 2019). We present the case series of four patients (2 men and 2 women; 1 Caucasian and 3 African Americans; two survived and two died) with confirmed COVID-19, presenting with respiratory symptoms and acute kidney injury, who have been on angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. Membrane-bound angiotensin-converting enzyme 2 (ACE2) has been implicated as the gateway for viral entry into the human cell in causing the infection. The factors contributing to acute kidney injury are diuretics, iodinated contrast administration, hemodynamic instability apart from ACE inhibitors, and angiotensin receptor blockers. The ACE inhibitors and ARBs were stopped in these patients due to acute kidney injury. We also discussed the role of ACE2 and the renin-angiotensin system (RAS) blockade in patients with COVID-19 infection along with pathogenesis.

Cocaine and Alcohol Co-Ingestion-Induced Severe Rhabdomyolysis With Acute Kidney Injury Culminating in Hemodialysis-Dependent End-Stage Renal Disease: A Case Report and Literature Review.

Cocaine toxicity is associated with several organ dysfunctions, including acute kidney injury (AKI). Rhabdomyolysis is the most likely mechanism that mediates AKI, and associated alcohol co-ingestion can amplify the situation. AKI, if severe, can result in end-stage renal disease (ESRD) requiring renal replacement therapy (RRT). All patients with cocaine intoxication should be screened for rhabdomyolysis and AKI along with testing for other drug toxicity, including alcohol. Aggressive measures should be taken to treat the underlying cause that contributes to AKI, and the patients need to be educated about this severe condition. Our patient is a unique case where cocaine and alcohol co-ingestion led to severe rhabdomyolysis, AKI, and subsequently developed ESRD requiring ongoing hemodialysis (HD). He was on daily cocaine and alcohol co-ingestion for seven days and subsequently developed AKI with oliguria from rhabdomyolysis. His creatine kinase (CK) was significantly elevated to 141974 IU/L, and his serum creatinine was 11 mg/dl. Despite aggressive intravenous hydration, his kidney function did not improve, and he ended up needing HD for more than one year despite abstaining from cocaine.

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.

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).

Reply to Comment on "An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female".

We sincerely thank Dr Andrew Whyte, who keenly reviewed our case report and came up with critical reasoning to justify his thoughts and critique with regard to our published article, "An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female." We agree with the author that hypocomplementemic urticarial vasculitis can be a reasonable contender as a diagnosis in this case. There are indeed some features in this case that do not entirely fit either classic presentation of acquired angioedema or hypocomplementemic urticarial vasculitis. Both diseases being equally rare, we tried to focus on the association of proliferative glomerulonephritis with angioedema-like features in this patient and considered acquired angioedema as the unifying diagnosis.

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.

Intensive Home Hemodialysis Survival Comparable to Deceased Donor Kidney Transplantation.

INTRODUCTION: Kidney transplantation (KT) remains the treatment of choice for end-stage kidney disease (ESKD), but access to transplantation is limited by a disparity between supply and demand for suitable organs. This organ shortfall has resulted in the use of a wider range of donor kidneys and, in parallel, a reexamination of potential alternative renal replacement therapies. Previous studies comparing Canadian intensive home hemodialysis (IHHD) with deceased donor (DD) KT in the United States reported similar survival, suggesting IHHD might be a plausible alternative. METHODS: Using data from the Scientific Registry of Transplant Recipients and an experienced US-based IHHD program in Lynchburg, VA, we retrospectively compared mortality outcomes of a cohort of IHHD patients with transplant recipients within the same geographic region between October 1997 and June 2014. RESULTS: We identified 3073 transplant recipients and 116 IHHD patients. Living donor KT (n = 1212) had the highest survival and 47% reduction in risk of death compared with IHHD (hazard ratio [HR]: 0.53; 95% confidence interval [CI]: 0.34-0.83). Survival of IHHD patients did not statistically differ from that of DD transplant recipients (n = 1834) in adjusted analyses (HR: 0.96; 95% CI: 0.62-1.48) or when exclusively compared with marginal (Kidney Donor Profile Index >85%) transplant recipients (HR: 1.35; 95% CI: 0.84-2.16). CONCLUSION: Our study showed comparable overall survival between IHHD and DD KT. For appropriate patients, IHHD could serve as bridging therapy to transplant and a tenable long-term renal replacement therapy.

Acute Pancreatitis Due to Disseminated Varicella Zoster Infection in an Individual with Newly Diagnosed Human Immunodeficiency Virus.

Varicella-zoster virus (VZV) infection is generally considered as a benign and self-limiting disease. However, individuals with VZV infection can have disseminated to various organs leading to serious complications, particularly in adults. This pattern is more prevalent in immunosuppressed individuals. Disseminated varicella is historically known to involve the central nervous system (CNS), liver, and lungs. However, dissemination of varicella to the pancreas and subsequently causing acute pancreatitis has been rarely reported. We present a case of disseminated varicella infection in a newly diagnosed human immunodeficiency virus (HIV) patient causing acute pancreatitis at initial disease presentation and subsequently leading to multi organ dysfunction. A 42-year-old African American female who was initially being treated for Pneumocystis carinii pneumonia (PCP) at an inner-city hospital developed severe epigastric pain radiating to back along with nausea on day 2 of admission. Physical findings revealed tachycardia, epigastric tenderness and newly formed vesicular rash involving the neck and face classical of varicella infection. Skin biopsy and serum sample confirmed varicella infection by VZV polymerase chain reaction (PCR) test. Labs revealed elevated lipase, amylase at a level diagnostic of acute pancreatitis. The patient had no other risk factors for pancreatitis. She was started on intravenous Acyclovir and intravenous hydration with isotonic normal saline. She was managed conservatively for other systemic complications. Pancreatitis resolved after five days of clinical presentation. She completed two weeks of Acyclovir, her condition steadily improved and she was successfully discharged home with no further recurrence. Acute pancreatitis is a rare infectious association of disseminated varicella infection. Clinicians should always be mindful of this infectious etiology as one of the rare differentials for acute pancreatitis as this is a treatable cause and could prevent morbidity, mortality associated with this condition if treated timely.

An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.

Acquired angioedema due to deficiency of C1 esterase inhibitor is also called acquired angioedema and is abbreviated as C1INH-AAE. It is a rare syndrome of recurrent episodes of angioedema, without urticaria, and in some patients, it is associated with B-cell lymphoproliferative disorders. Kidney involvement is rare in this condition. The monoclonal immunoglobulin secreted by a nonmalignant or premalignant B-cell or plasma cell clone, causing renal damage that represents a group of disorders which are termed as monoclonal gammopathy of renal significance (MGRS). In this article, we report a rare case of acquired C1 esterase deficiency angioedema and acute kidney injury with renal biopsy-proven MGRS. We present a 64-year-old Caucasian woman who presented with 2 weeks of recurring urticaria and new onset of acute kidney injury. She was diagnosed with monoclonal gammopathy-associated proliferative glomerulopathy through kidney biopsy, and serological workup came back positive for C1 esterase deficiency, implying acquired angioedema. Acquired angioedema is a rare disease with systemic involvement. Recurrent allergic manifestations and acute kidney injury should prompt MGRS as a differential.

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.

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.

An Overlapping Case of IgG4-Related Disease and Systemic Lupus Erythematosus.

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti-double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.

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.

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.

Case Report of Lethal Toxin Lurking in an Edible Plant.

Cyanide is notoriously known to the public for more than a century now as a weapon of mass destruction (Zyklon B gas - hydrogen cyanide used by Nazis), an agent for chemical warfare during World War I (hydrogen cyanide) and very infamous "Suicide Pill" used in the past by military and espionage organizations during World War II (potassium cyanide). During the modern industrial era, cyanide poisoning is commonly associated with the industrial exposure and domestic fires. But there is little awareness about potentially fatal consequences of cyanide poisoning from common food sources. Here, we present the case report of a 79-year-old female with acute cyanide poisoning from improperly prepared cassava leaves. Symptoms from ingested toxin may start a few hours after exposure, which include headache, confusion, ataxia, seizures, palpitations, nausea, vomiting, abdominal pain, flushing, and itching of the skin. Patients may develop hypotension, cardiac arrhythmias, renal failure, hepatic necrosis, rhabdomyolysis, and metabolic acidosis; a multisystem manifestation of hypoxia at the cellular level. Multiple treatment strategies are available to treat cyanide poisoning, including sodium nitrite, sodium thiosulfate, and hydroxycobalamine. This is one of the scenarios where a thorough history, awareness of agents causing cyanide toxicity and knowledge of clinical manifestations can help avoid delays in prompt decision-making for appropriate treatment, thus reducing morbidity, mortality, and prolonged hospital course.