Ambulatory blood pressure monitoring in children during the COVID-19 pandemic.

The pandemic caused by severe acute respiratory syndrome corona virus-2 (SARS-CoV-2) had profound effects on healthcare delivery in the USA and abroad. Although ambulatory blood pressure monitoring (ABPM) is the recommended method for confirming hypertension (HTN) diagnosis and management, it is unclear how the pandemic affected ABPM utilization. We surveyed 81 pediatric nephrologists from 54 pediatric nephrology centers regarding their ABPM practices during the pandemic; 56.

Diabetes Insipidus Complicating Management in a Child with COVID-19 and Multiorgan System Failure: A Novel Use for Furosemide.

Judicious balance of fluids is needed for optimal management of acute respiratory distress syndrome (ARDS). Achieving optimal fluid balance is difficult in patients with disorders of fluid homeostasis such as diabetes insipidus (DI). There is little data on the use of Furosemide to aid in balancing fluid and electrolytes in patients with DI.

Cumulative Application of Creatinine and Urine Output Staging Optimizes the Kidney Disease: Improving Global Outcomes Definition and Identifies Increased Mortality Risk in Hospitalized Patients With Acute Kidney Injury.

Objectives: Acute kidney injury is diagnosed according to creatinine and urine output criteria. Traditionally, both are applied, and a severity stage (1-3) is conferred based upon the more severe of the two; information from the other criteria is discarded. Physiologically, however, rising creatinine and oliguria represent two distinct types of renal dysfunction.

Pediatric Immunization Practices in Nephrotic Syndrome: An Assessment of Provider and Parental Knowledge.

Children with nephrotic syndrome (NS) are at high risk for vaccine-preventable infections due to the immunological effects from the disease and concurrent treatment with immunosuppressive medications. Immunizations in these patients may be deferred due to their immunosuppressive treatment which may increase the risk for vaccine-preventable infections. Immunization practices in children with NS continue to vary among pediatric nephrologists.

Hypertension in Childhood Nephrotic Syndrome.

Arterial hypertension (HTN) is commonly encountered by clinicians treating children with steroid sensitive (SSNS) and steroid resistant nephrotic syndrome (SRNS). Although the prevalence of HTN in SSNS is less documented than in SRNS, recent studies reported high prevalence in both. Studies have estimated the prevalence of HTN in different patient populations with NS to range from 8 to 59.

Left Ventricular Hypertrophy in Pediatric Hypertension: A Mini Review.

Adults with arterial hypertension (HTN) have stroke, myocardial infarction, end-stage renal disease (ESRD), or die at higher rates than those without. In children, HTN leads to target organ damage, which includes kidney, brain, eye, blood vessels, and heart, which precedes "hard outcomes" observed in adults. Left ventricular hypertrophy (LVH) or an anatomic and pathologic increase in left ventricular mass (LVM) in response to the HTN is a pediatric surrogate marker for HTN-induced morbidity and mortality in adults.

Renal and Cardiovascular Morbidities Associated with Status among African-American and Non-African-American Children with Focal Segmental Glomerulosclerosis.

Background And Objectives: African-American (AA) children with focal segmental glomerulosclerosis (FSGS) have later onset disease that progresses more rapidly than in non-AA children. It is unclear how genotypes contribute to kidney disease risk, progression, and cardiovascular morbidity in children.

Design Setting Participants And Measurements: We examined the prevalence of genotypes and associated cardiovascular phenotypes among children with FSGS in the Chronic Kidney Disease in Children (CKiD) study; an ongoing multicenter prospective cohort study of children aged 1-16 years with mild to moderate kidney disease.

Krüppel-Like Factor 15 Mediates Glucocorticoid-Induced Restoration of Podocyte Differentiation Markers.

Podocyte injury is the inciting event in primary glomerulopathies, such as minimal change disease and primary FSGS, and glucocorticoids remain the initial and often, the primary treatment of choice for these glomerulopathies. Because inflammation is not readily apparent in these diseases, understanding the direct effects of glucocorticoids on the podocyte, independent of the immunomodulatory effects, may lead to the identification of targets downstream of glucocorticoids that minimize toxicity without compromising efficacy. Several studies showed that treatment with glucocorticoids restores podocyte differentiation markers and normal ultrastructure and improves cell survival in murine podocytes.

APOL1-associated glomerular disease among African-American children: a collaboration of the Chronic Kidney Disease in Children (CKiD) and Nephrotic Syndrome Study Network (NEPTUNE) cohorts.

Background: Individuals of African ancestry harboring two variant alleles within apolipoprotein L1 ( APOL1 ) are classified with a high-risk (HR) genotype. Adults with an HR genotype have increased risk of focal segmental glomerulosclerosis and chronic kidney disease compared with those with a low-risk (LR) genotype (0 or 1 variants). The role of APOL1 risk genotypes in children with glomerular disease is less well known.

Assessment of Left Ventricular Mass and Hypertrophy by Cardiovascular Magnetic Resonance Imaging in Pediatric Hypertension.

Cardiovascular magnetic resonance (CMR) imaging in adults is considered the gold standard for assessment of left ventricular mass (LVM) and left ventricular hypertrophy (LVH). The authors aimed to evaluate agreement of LVM measurements and LVH determination between echocardiography (ECHO) and CMR imaging in children with hypertension (HTN) confirmed by 24-hour ambulatory blood pressure monitoring (ABPM). The children (n=22) underwent contemporaneous ECHO, CMR imaging, and ABPM.

Rituximab in post-transplant pediatric recurrent focal segmental glomerulosclerosis.

Background: Focal segmental glomerulosclerosis (FSGS) recurs in 20-40 % of allografts. Plasmapheresis (TPE) has been one of the mainstays of treatment with variable results. Rituximab (RTX), a monoclonal antibody to the protein CD20, is being used for treatment of recurrent FSGS (recFSGS) but pediatric experience is limited.

Recurrent focal segmental glomerulosclerosis in renal allograft recipients: role of human leukocyte antigen mismatching and other clinical variables.

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation impacts long-term graft survival and limits access to transplantation. We hypothesized that HLA donor/recipient matching could be used as a surrogate marker of recurrence. In a retrospective study of 42 pediatric and 77 adult subjects with primary FSGS, transplanted from 1990 to 2007 at a single center, we analyzed the degree of donor/recipient HLA compatibility and other clinical variables associated with FSGS recurrence.

Hypertension, chronic kidney disease, and renal pathology in a child with hermansky-pudlak syndrome.

We report a child with Hermansky-Pudlak Syndrome (HPS) and chronic kidney disease (stage II) with histological diagnosis of focal segmental glomerulosclerosis (FSGS). A 15-year-old male of Puerto Rico ancestry with history of HPS, hypertension (HTN), asthma, obesity, and chronic kidney disease (CKD) stage II presented with new-onset proteinuria without edema. His blood pressure had been controlled, serum creatinine had been 0.

Progression of glomerular and tubular disease in pediatrics.

Chronic kidney disease may be stimulated by many different etiologies, but its progression involves a common, yet complex, series of events that lead to the replacement of normal tissue with scar. These events include altered physiology within the kidney leading to abnormal hemodynamics, chronic hypoxia, inflammation, cellular dysfunction, and activation of fibrogenic biochemical pathways. The end result is the replacement of normal structures with extracellular matrix.

Laser capture microdissection of kidney tissue.

Kidney tissue laser capture microdissection (LCM) is of great clinical relevance since genome wide studies on total kidney messenger RNA (mRNA) potentially miss important factors involved in the pathogenesis of the disease in glomeruli and tubules. This technique is readily applicable to study mRNA from isolated glomeruli and tubules of human kidney biopsy material. In this chapter we present a "cook-book" practical approach of utilizing LCM in combination with RNA isolation technique in downstream applications in nephrology.

Increased prevalence of renal and urinary tract anomalies in children with congenital hypothyroidism.

Objective: We investigated the prevalence of congenital renal and urologic anomalies in children with congenital hypothyroidism to determine whether further renal and urologic investigations would be of benefit.

Study Design: Prevalence of congenital hypothyroidism was obtained from the New York State Congenital Malformation Registry. The occurrence of urinary tract anomalies were calculated for children with congenital hypothyroidism and compared to children without congenital hypothyroidism.

Outcome of dialysis in children with human immunodeficiency virus infection.

Human immunodeficiency virus (HIV) infection accounts for an unknown percentage of children with end-stage kidney disease (ESKD). Our objective was to compare the outcome of renal replacement therapy (RRT) in subjects with ESKD due to HIV and other diagnoses and to examine the prevalence of ESKD due to HIV. We analyzed Kt/V, morbidity, mortality, echocardiography, nutritional, and transplant status in 12 dialysis patients with HIV and 32 without HIV followed at our center between February 2002 and February 2007.

The role of osteopontin in the development of albuminuria.

Several gene array studies have suggested that osteopontin (Opn) expression strongly correlates with albuminuria and glomerular disease. Urinary Opn concentration and kidney Opn immunoreactivity were found to be increased in patients with steroid-sensitive nephrotic syndrome. In addition, renal Opn mRNA was increased in the Ins2(Akita) mouse model of type 1 diabetic nephropathy, in the LPS-induced albuminuria model, and in glomeruli of puromycin aminonucleotide-induced nephrotic rats.

Urinary cytokines and steroid responsiveness in idiopathic nephrotic syndrome of childhood.

Background/aim: Steroid-resistant nephrotic syndrome (SRNS) has been associated with activation of TGF-beta(1) and progression to chronic kidney disease. Steroid-sensitive nephrotic syndrome (SSNS) has been associated with activation of T-cells and favorable outcome. Our objective was to distinguish SRNS from SSNS and focal segmental glomerulosclerosis (FSGS) from minimal change disease (MCD) on the basis of urinary cytokine profile.

Genetics of focal segmental glomerulosclerosis.

The recent advances in understanding the pathophysiology of focal segmental glomerulosclerosis (FSGS) and molecular function of glomerular filtration barrier come directly from genetic linkage and positional cloning studies. The exact role and function of the newly discovered genes and proteins are being investigated by in vitro and in vivo mechanistic studies. Those genes and proteins interactions seem to change susceptibility to kidney disease progression.

Association of steroid and cyclosporin resistance in focal segmental glomerulosclerosis.

Given the variable response of steroid-resistant nephrotic syndrome (SRNS) to treatment with cyclosporin (CsA), it may be inappropriate to expose all SR patients to additional immunosuppression. How to determine from which patients to withhold CsA is unclear. We tested the hypothesis that in patients with primary focal segmental glomerulosclerosis (FSGS), steroid resistance predicts CsA resistance.

Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhood.

The response to steroid therapy is used to characterize the idiopathic nephrotic syndrome (INS) of childhood as either steroid-sensitive (SSNS) or steroid-resistant (SRNS), a classification with a better prognostic capability than renal biopsy. The majority (approximately 80%) of INS is due to minimal change disease but the percentage of focal and segmental glomerulosclerosis is increasing. We applied a new technological platform to examine the urine proteome to determine if different urinary protein excretion profiles could differentiate patients with SSNS from those with SRNS.

Glomerular expression of transforming growth factor-beta (TGF-beta) isoforms in mice lacking CD2-associated protein.

Mice lacking CD2-associated protein (CD2AP-/-) develop glomerular lesions resembling human focal segmental glomerulosclerosis (FSGS) between 3-4 weeks of age and die approximately 2 weeks later from massive proteinuria and renal failure. The mechanisms involved in the glomerular injury in this model are unclear. In this study, we used laser capture microdissection (LCM) and real-time PCR, and examined expression of TGF-ss isoforms in CD2AP-/- mice at the level of isolated glomeruli.