Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes in the kidney are limited.

Methods: To characterize kidney pathology in HLH, a retrospective review of 30 patients' clinical and laboratory data, and kidney tissue was performed (18 from autopsy, and 12 biopsied patients).

The Fatty Kidney and Beyond: A Silent Epidemic.

As the prevalence of obesity rises in the United States, so does the incidence of obesity-related kidney disease. Obesity itself is an independent risk factor for chronic kidney disease where the pathophysiology is complex, involving altered hemodynamics, renin-angiotensin-aldosterone system overactivation, and adipokines leading to inflammation and fibrosis. Obesity-related kidney disease comprises both obesity-related glomerulopathy and fatty kidney disease.

Finerenone: A Novel Third-Generation Mineralocorticoid Receptor Antagonist.

Finerenone is a novel third-generation, selective nonsteroidal mineralocorticoid receptor antagonist (MRA) that was approved by the Food and Drug Administration in July of 2021 for its use in adults with chronic kidney disease and Type II diabetes mellitus. Randomized controlled trials The Finerenone in Reducing Kidney Failure and Disease Progression in Diabetic Kidney Disease and Finerenone in Reducing Cardiovascular Mortality and Morbidity in Diabetic Kidney Disease showed decreased adverse kidney and cardiovascular outcomes, respectively, in this population. The incidence of hyperkalemia, while higher in the study versus placebo group, was lower than older generations of MRAs (spironolactone and eplerenone) and proved to be an infrequent cause for drug discontinuation.

Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment.

Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection.

Hypertension in Pregnancy: What We Now Know.

Purpose Of Review: Hypertensive disorders of pregnancy remain a highly morbid condition that affects both the mother and fetus, complicate approximately 10% of pregnancies worldwide, and contribute to immediate and long-term cardiovascular outcomes. There is still much to learn regarding pathogenesis and treatment goals.

Recent Findings: There is updated information on the pathogenesis of preeclampsia and treatment thresholds for HTN in pregnancy.

Obesity-related glomerulopathy in the presence of APOL1 risk alleles.

Nephropathic apolipoprotein L1 (APOL1) risk alleles (G1/G2) have been associated with focal segmental glomerulosclerosis, HIV-associated nephropathy, Systemic lupus erythematosus (SLE)-associated collapsing glomerulopathy and other glomerulonephritides. These alleles confer protection from infections which are enriched in sub-Saharan African populations. We present a young woman with obesity, hypertension, subnephrotic range proteinuria who was found to have obesity-related glomerulopathy on kidney biopsy while harbouring two high-risk APOL1 alleles (G1/G2).

Impaired renal reserve contributes to preeclampsia via the kynurenine and soluble fms-like tyrosine kinase 1 pathway.

To understand how kidney donation leads to an increased risk of preeclampsia, we studied pregnant outbred mice with prior uninephrectomy and compared them with sham-operated littermates carrying both kidneys. During pregnancy, uninephrectomized (UNx) mice failed to achieve a physiological increase in the glomerular filtration rate and during late gestation developed hypertension, albuminuria, glomerular endothelial damage, and excess placental production of soluble fms-like tyrosine kinase 1 (sFLT1), an antiangiogenic protein implicated in the pathogenesis of preeclampsia. Maternal hypertension in UNx mice was associated with low plasma volumes, an increased rate of fetal resorption, impaired spiral artery remodeling, and placental ischemia.

Utility of Cardiac Troponins in Patients With Chronic Kidney Disease.

Cardiovascular disease is a major cause of death worldwide especially in patients with chronic kidney disease (CKD). Troponin T and troponin I are cardiac biomarkers used not only to diagnose acute myocardial infarction (AMI) but also to prognosticate cardiovascular and all-cause mortality. The diagnosis of AMI in the CKD population is challenging because of their elevated troponins at baseline.

Acute Kidney Injury in Obstetric Patients.

Obstetric-related acute kidney injury (obstetric AKI) is an important and complex public health problem; its early recognition and proper treatment are key in preventing maternal and fetal adverse outcomes. While the incidence of obstetric AKI has drastically declined in some developing countries due to reduction of sepsis-related causes, the opposite has been observed in other developed nations in the last decade due to advanced maternal age and the presence of comorbidities. The diagnosis of obstetric AKI has been made difficult by the physiologic decrease in serum creatinine of pregnancy as well as the absence of a uniform definition for AKI in this population.

Atypical presentation of allergic interstitial nephritis likely induced by ciprofloxacin.

Though the prevalence of drug induced allergic interstitial nephritis (AIN) appears to be increasing, the diagnostic and treatment strategies still remain vague. We present a 56-year-old man with a history of hypertension, chronic kidney disease stage IIIa, recent exposure to ciprofloxacin who presented with acute kidney injury. Though the suspicion of AIN was high, his urinary sediment was bland, that is, no leucocytes or leucocyte casts.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits associated with parvovirus B19.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is included in the group of dysproteinaemias causing renal disease. Only a minority of cases are associated with a haematological malignancy. Two cases have been linked to acute parvovirus B19 infections.

Light chain proximal tubulopathy with cast nephropathy in monoclonal gammopathy of renal significance.

Kidney tubular disorders due to monoclonal immunoglobulin light chains are common manifestations of B-cell neoplasm. Cast nephropathy (CN) is the most frequent type of these disorders and may present with acute kidney injury (AKI) due to the presence of excess light chains in the distal tubules. Light chain proximal tubulopathy (LCPT) is an uncommon form of renal disease and may present as Fanconi syndrome due to proximal tubular cell damage by intracellular deposition of light chains.

Complement-Mediated Disorders in Pregnancy.

Complement-mediated disorders in pregnancy span a large spectrum and have been implicated in all three complement pathways: classical, lectin, and alternative. Our understanding of these disorders in recent years has advanced due to a better understanding of complement regulatory proteins, such as complement factor H, complement factor I, membrane cofactor protein, and thrombomodulin that particularly affect the alternative complement pathway. Enthusiasm in genotyping for mutations that encode these proteins has allowed us to study the presence of genetic variants which may predispose women to develop conditions such as pregnancy-associated hemolytic uremic syndrome (P-aHUS), thrombotic thrombocytopenic purpura, preeclampsia/hemolysis, elevated liver enzymes, low platelets (HELLP), systemic lupus erythematosus/antiphospholipid syndrome, and peripartum cardiomyopathy.

Acute kidney injury in pregnancy.

Purpose Of Review: Pregnancy-related acute kidney injury (Pr-AKI) is associated with increased maternal and fetal morbidity and mortality and remains a large public health problem.

Recent Findings: Pr-AKI incidence has globally decreased over time for the most part. However, the cause presents a disparity between developing and developed countries, reflecting differences in socioeconomic factors and healthcare infrastructure - with the noteworthy outlier of increased incidence in the United States and Canada.

Hypertension and Pregnancy: Management and Future Risks.

Pregnancy-induced hypertension is a major cause of maternal and fetal morbidity and mortality. The overall strategies of defining and managing these conditions are aimed at preventing cardiovascular and cerebrovascular complications in the mother without jeopardizing fetal well-being. Our understanding of the origin of these disorders is evolving.

Recurrent case of pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS).

Pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS) is a rare condition characterised by microangiopathic haemolytic anaemia, thrombocytopenia and renal failure. It accounts for approximately 7% of total HUS cases. Here, we present a case of recurrent P-aHUS in a 25-year-old Hispanic woman.

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles.

Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II.

Mentorship in Medicine and Nephrology: More Important Than Ever.

Mentorship has always been important in medicine. In fact, one can argue that that is how doctors are trained. Moreover, mentorship has been proven to preserve and elevate those who wish to pursue academic medicine.

Proteinuric Kidney Diseases: A Podocyte's Slit Diaphragm and Cytoskeleton Approach.

Proteinuric kidney diseases are a group of disorders with diverse pathological mechanisms associated with significant losses of protein in the urine. The glomerular filtration barrier (GFB), comprised of the three important layers, the fenestrated glomerular endothelium, the glomerular basement membrane (GBM), and the podocyte, dictates that disruption of any one of these structures should lead to proteinuric disease. Podocytes, in particular, have long been considered as the final gatekeeper of the GFB.

Unusual case of levamisole-induced dual-positive ANCA vasculitis and crescentic glomerulonephritis.

Cocaine adulterated levamisole is an increasingly reported cause of skin necrosis, arthralgia and systemic vasculitis, but renal involvement is uncommon. We present a case of a 40-year-old Hispanic man with a history of cocaine abuse who presented with acute kidney injury to the rheumatology clinic where he was being treated for chronic inflammatory arthritis. He was found to have a serum creatinine of 2.

Acute Kidney Injury in Pregnancy: The Changing Landscape for the 21st Century.

Pregnancy-related acute kidney injury (Pr-AKI) remains a large public health problem, with decreasing incidences in developing countries but seemingly increasing incidences in the United States and Canada. These epidemiologic changes are reflective of the advances in medical and obstetric care, as well as changes in underlying maternal risk factors. The risk factors associated with advanced maternal age, such as hypertension, diabetes, chronic kidney disease, and those associated with reproductive technologies such as multiple gestations, are increasing.

Autosomal Dominant Tubulointerstitial Kidney Disease Due to MUC1 Mutation.

Mucin 1 kidney disease, previously referred to as medullary cystic kidney disease type 1, is a rare hereditary kidney disease. It is one of several diseases now termed autosomal dominant tubulointerstitial kidney disease, as proposed by a KDIGO (Kidney Disease: Improving Global Outcomes) consensus report in 2014. Autosomal dominant tubulointerstitial kidney diseases share common clinical findings, such as autosomal dominant inheritance, bland urinary sediment, absent to mild proteinuria, and progressive loss of kidney function.

ANCA and IgA glomerulonephritis all in one: prognosis and complications.

We present the case of a 75-year-old Hispanic woman with known stage 3 chronic kidney disease, long-standing hypertension and type 2 diabetes mellitus who presented with right-sided abdominal pain and acute kidney injury, nephrotic range proteinuria with positive antimyeloperoxidase antibody. A renal biopsy revealed IgA nephropathy with superimposed pauci-immune antineutrophilic cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis. The patient was treated with pulse intravenous methylprednisolone, cyclophosphamide and plasmapheresis.