End-stage and chronic kidney disease in classic bladder exstrophy: A retrospective muti-institutional cohort study.

Introduction: While most children with classic bladder exstrophy (CBE) are born with normal kidneys, some experience renal deterioration in adulthood. Little is known about the incidence of end-stage and chronic kidney disease (ESKD and CKD, respectively) in this population. Our group has recently published on surgical outcomes in a multi-institutional cohort of 216 people with CBE.

Responding to the workforce crisis: consensus recommendations from the Second Workforce Summit of the American Society of Pediatric Nephrology.

Importance: Pediatric patients with complex medical problems benefit from pediatric sub-specialty care; however, a significant proportion of children live greater than 80 mi. away from pediatric sub-specialty care.

Objective: To identify current knowledge gaps and outline concrete next steps to make progress on issues that have persistently challenged the pediatric nephrology workforce.

DNA gene-dosage regulates the kidney innate immune response during upper urinary tract infection.

Antimicrobial peptides (AMPs) are host defense effectors with potent neutralizing and immunomodulatory functions against invasive pathogens. The AMPs α-Defensin 1-3/ participate in innate immune responses and influence patient outcomes in various diseases. DNA copy-number variations in have been associated with severity and outcomes in infectious diseases including urinary tract infections (UTIs).

Intercalated cell function, kidney innate immunity, and urinary tract infections.

Intercalated cells (ICs) in the kidney collecting duct have a versatile role in acid-base and electrolyte regulation along with the host immune defense. Located in the terminal kidney tubule segment, ICs are among the first kidney cells to encounter bacteria when bacteria ascend from the bladder into the kidney. ICs have developed several mechanisms to combat bacterial infections of the kidneys.

Generation of -Cre mice for investigation of intercalated cells and the collecting duct.

Kidney intercalated cells (ICs) maintain acid-base homeostasis and recent studies have demonstrated that they function in the kidney's innate defense. To study kidney innate immune function, ICs have been enriched using vacuolar ATPase (V-ATPase) B1 subunit ()-Cre (B1-Cre) mice. Although is considered kidney specific, it is expressed in multiple organ systems, both in mice and humans, raising the possibility of off-target effects when using the Cre-lox system.

Establishment of Baseline Urinary Antimicrobial Peptide Levels by Age: A Prospective Observational Study.

Background: Antimicrobial peptides (AMPs) are key effectors of urinary tract innate immunity. Identifying differences in urinary AMP levels between younger and older adults is important in understanding older adults' susceptibility and response to urinary tract infections (UTI) and AMP use as diagnostic biomarkers. We hypothesized that uninfected older adults have higher urinary human neutrophil peptides 1-3 (HNP 1-3), human alpha-defensin-5 (HD-5), and human beta-defensin-2 (hBD-2), but lower urinary cathelicidin (LL-37) than younger adults.

Using aquapheresis with continuous hematocrit monitoring to guide ultrafiltration.

Background: Management of edema and volume overload in patients with hypoalbuminemia, either due to nephrotic syndrome or other disease processes, can be extremely challenging.

Methods: We describe the management of five patients with hypoalbuminemia and severe fluid overload using the Aquadex FlexFlow device with continuous hematocrit monitoring to guide ultrafiltration.

Results: We report five pediatric patients ranging in age from 7 days to 11 years and in size from 2.

Safety and timeliness of telemedicine initiation of continuous kidney replacement therapy.

Background: During the COVID-19 pandemic, some continuous kidney replacement therapy (CKRT) initiations were transitioned to telemedicine to improve the timeliness of initiation, and minimize COVID-19 transmission. While telemedicine would appear acceptable for many clinical settings, safety and timeliness of telemedicine CKRT initiation is undescribed.

Methods: We conducted a single-center retrospective cohort study of pediatric patients on CKRT from January 2021-September 2022.

FOXD1 is required for 3D patterning of the kidney interstitial matrix.

Background: The interstitial extracellular matrix (ECM) is comprised of proteins and glycosaminoglycans and provides structural and biochemical information during development. Our previous work revealed the presence of transient ECM-based structures in the interstitial matrix of developing kidneys. Stromal cells are the main contributors to interstitial ECM synthesis, and the transcription factor Forkhead Box D1 (Foxd1) is critical for stromal cell function.

Human neutrophil peptides 1-3 protect the murine urinary tract from uropathogenic challenge.

Antimicrobial peptides (AMPs) are critical to the protection of the urinary tract of humans and other animals from pathogenic microbial invasion. AMPs rapidly destroy pathogens by disrupting microbial membranes and/or augmenting or inhibiting the host immune system through a variety of signaling pathways. We have previously demonstrated that alpha-defensins 1-3 () are AMPs expressed in the epithelial cells of the human kidney collecting duct in response to uropathogens.

MAP3K7 is an innate immune regulatory gene with increased expression in human and murine kidney intercalated cells following uropathogenic Escherichia coli exposure.

Understanding the mechanisms responsible for the kidney's defense against ascending uropathogen is critical to devise novel treatment strategies against increasingly antibiotic resistant uropathogen. Growing body of evidence indicate Intercalated cells of the kidney as the key innate immune epithelial cells against uropathogen. The aim of this study was to find orthologous and differentially expressed bacterial defense genes in human versus murine intercalated cells.

Suspect screening of exogenous compounds using multiple reaction screening (MRM) profiling in human urine samples.

Thousands of chemical compounds produced by industry are dispersed in the human environment widely enough to reach the world population, and the introduction of new chemicals constantly occurs. As new synthetic molecules emerge, rapid analytical workflows for screening possible presence of exogenous compounds in biofluids can be useful as a first pass analysis to detect chemical exposure and guide the development and application of more elaborate LC-MS/MS methods for quantification. In this study, a suspect screening workflow using the multiple reaction monitoring (MRM) profiling method is proposed as a first pass exploratory technique to survey selected exogenous molecules in human urine samples.

Association Between Continuous Kidney Replacement Therapy Clearance and Outcome in Pediatric Patients With Hyperammonemia Not Due to Inborn Error of Metabolism.

Objectives: To describe a single-center experience of pediatric patients with hyperammonemia not due to inborn errors of metabolism and determine the association between use of continuous kidney replacement therapy (CKRT) treatment and outcomes.

Design: Retrospective cohort study.

Setting: Tertiary-care children's hospital.

A Pilot Single Cell Analysis of the Zebrafish Embryo Cellular Responses to Uropathogenic Infection.

Background: Uropathogenic (UPEC) infections are common and when they disseminate can be of high morbidity.

Methods: We studied the effects of UPEC infection using single cell RNA sequencing (scRNAseq) in zebrafish. Bulk RNA sequencing has historically been used to evaluate gene expression patterns, but scRNAseq allows gene expression to be evaluated at the single cell level and is optimal for evaluating heterogeneity within cell types and rare cell types.

Ribonuclease 7 polymorphism rs1263872 reduces antimicrobial activity and associates with pediatric urinary tract infections.

Ribonuclease 7 (RNase 7) is an antimicrobial peptide that prevents urinary tract infections (UTI); however, it is yet unknown how RNASE7 genetic variations affect its antimicrobial activity and its mitigation of UTI risk. This study determined whether the RNASE7 SNP rs1263872 is more prevalent in children with UTI and defined how rs1263872 affects RNase 7's antimicrobial activity against uropathogenic E. coli (UPEC).

Placement on COVID-19 Units Does Not Increase Seroconversion Rate of Pediatric Graduate Medical Residents.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its associated disease COVID-19 (coronavirus disease 2019) has presented graduate medical education (GME) training programs with a unique set of challenges. One of the most pressing is how should hospital systems that rely on graduate medical residents provide appropriate care for patients while protecting trainees. This question is of particular concern as healthcare workers are at high risk of SARS-CoV-2 exposure.

Kidney intercalated cells are phagocytic and acidify internalized uropathogenic Escherichia coli.

Kidney intercalated cells are involved in acid-base homeostasis via vacuolar ATPase expression. Here we report six human intercalated cell subtypes, including hybrid principal-intercalated cells identified from single cell transcriptomics. Phagosome maturation is a biological process that increases in biological pathway analysis rank following exposure to uropathogenic Escherichia coli in two of the intercalated cell subtypes.

3D Mapping Reveals a Complex and Transient Interstitial Matrix During Murine Kidney Development.

Background: The extracellular matrix (ECM) is a network of proteins and glycosaminoglycans that provides structural and biochemical cues to cells. In the kidney, the ECM is critical for nephrogenesis; however, the dynamics of ECM composition and how it relates to 3D structure during development is unknown.

Methods: Using embryonic day 14.

Variation in COVID-19 Diagnosis by Zip Code and Race and Ethnicity in Indiana.

To describe variations in coronavirus disease 2019 (COVID-19) diagnosis by zip code race and ethnicity in Indiana. Cross-sectional evaluation of subjects with SARS-CoV-2 at Indiana University Health. We performed two separate analyses, first evaluating likelihood of COVID-19 diagnosis by race (Caucasian, African American, Asian, or other) and ethnicity (Hispanic vs.

Assessment of Seroconversion to SARS-CoV-2 in a Cohort of Pediatric Kidney Transplant Recipients.

The occurrence of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and the associated coronavirus disease 2019 (COVID-19) have profoundly affected adult kidney disease patients. In contrast, pediatric solid organ transplant recipients, including pediatric kidney transplant (KT) recipients, do not seem to be at particularly higher risk for SARS-CoV-2 infection or for severe COVID-19 disease. This patient population might be protected by certain mechanisms, such as the immunosuppressive medications with their anti-inflammatory properties or simply being well-versed in self-protection techniques.

Coronavirus disease 2019 (COVID-19) in two pediatric patients with kidney disease on chronic immunosuppression: A case series.

Coronavirus disease 2019 (COVID-19) is a highly infectious disease caused by the severe acute respiratory syndrome coronavirus 2 virus (SARS-CoV-2). While children appear to experience less severe disease than adults, those with underlying conditions such as kidney disease may be more susceptible to infection. Limited data are present for children with kidney disease, and there are limited prior reports of pediatric hemodialysis patients with COVID-19.