Mothball Ingestion in the Setting of G6PD Deficiency Causing Severe Hemolytic Anemia, Methemoglobinemia, and Multiple Organ Failure in a Toddler.

Mothballs containing naphthalene or paradichlorobenzene are known to cause hemolysis and methemoglobinemia. They can also affect the other organs, including the kidneys, liver, lungs, and skeletal muscles. The involvement of 1 or 2 organs at a time has been commonly reported.

Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes-a Pediatric Nephrology Research Consortium study.

Background: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab.

Incidence and Risk Factors of Early Onset Neonatal AKI.

Background And Objectives: Neonatal AKI is associated with poor short- and long-term outcomes. The objective of this study was to describe the risk factors and outcomes of neonatal AKI in the first postnatal week.

Design, Setting, Participants, & Measurements: The international retrospective observational cohort study, Assessment of Worldwide AKI Epidemiology in Neonates (AWAKEN), included neonates admitted to a neonatal intensive care unit who received at least 48 hours of intravenous fluids.

Late onset neonatal acute kidney injury: results from the AWAKEN Study.

Background: Most studies of neonatal acute kidney injury (AKI) have focused on the first week following birth. Here, we determined the outcomes and risk factors for late AKI (>7d).

Methods: The international AWAKEN study examined AKI in neonates admitted to an intensive care unit.

Insulin and the phosphatidylinositol 3-kinase signaling pathway regulate Ribonuclease 7 expression in the human urinary tract.

Diabetes mellitus is a systemic disease associated with a deficiency of insulin production or action. Diabetic patients have an increased susceptibility to infection with the urinary tract being the most common site. Recent studies suggest that Ribonuclease 7 (RNase 7) is a potent antimicrobial peptide that plays an important role in protecting the urinary tract from bacterial insult.

Renal epithelial miR-205 expression correlates with disease severity in a mouse model of congenital obstructive nephropathy.

Background: Congenital obstructive nephropathy (CON) is a leading cause of pediatric chronic kidney disease (CKD). Despite optimal surgical and medical care, there is a high rate of CKD progression. Better understanding of molecular and cellular changes is needed to facilitate development of improved biomarkers and novel therapeutic approaches in CON.

Urine Stasis Predisposes to Urinary Tract Infection by an Opportunistic Uropathogen in the Megabladder (Mgb) Mouse.

Purpose: Urinary stasis is a risk factor for recurrent urinary tract infection (UTI). Homozygous mutant Megabladder (Mgb-/-) mice exhibit incomplete bladder emptying as a consequence of congenital detrusor aplasia. We hypothesize that this predisposes Mgb-/- mice to spontaneous and experimental UTI.

Renin Angiotensin System Blocker Fetopathy: A Midwest Pediatric Nephrology Consortium Report.

Objectives: Fetuses continue to be exposed to renin angiotensin system (RAS) blockers despite their known teratogenicity and a black box warning. We hypothesized that fetopathy from in utero exposure to RAS blockers has a broader spectrum of clinical manifestations than described previously and that there are a variety of clinical scenarios leading to such exposures.

Study Design: This was a retrospective study performed through the Midwest Pediatric Nephrology Consortium.

Beneficial effects of intravenous pamidronate treatment in children with osteogenesis imperfecta under 24 months of age.

Osteogenesis imperfecta (OI) is an inherited disorder characterized by bone fragility and low bone mass. Low bone density and fracture is a cause of morbidity. Limited data exists on bisphosphonate treatment in patients under 24 months of age.

Molecular basis of renal adaptation in a murine model of congenital obstructive nephropathy.

Congenital obstructive nephropathy is a common cause of chronic kidney disease and a leading indication for renal transplant in children. The cellular and molecular responses of the kidney to congenital obstruction are incompletely characterized. In this study, we evaluated global transcription in kidneys with graded hydronephrosis in the megabladder (mgb (-/-)) mouse to better understand the pathophysiology of congenital obstructive nephropathy.

Struvite urolithiasis and chronic urinary tract infection in a murine model of urinary diversion.

Objective: To characterize the clinical course after cutaneous vesicostomy (CV) in megabladder (mgb(-/-)) mice with functional urinary bladder obstruction.

Materials And Methods: A total of 45 mgb(-/-) male mice underwent CV at a median age of 25 days. The 34 mice that survived >3 days after CV were evaluated by serial observation and renal ultrasonography.

Ultrasound imaging of the murine kidney.

Ultrasound (US) is the most common and least invasive modality for clinical imaging of the kidney. One important application of US in nephrology is the detection and monitoring of structural changes in the kidney. Recent advances in US technology have facilitated the application of similar techniques to animal models of human disease.

Current perspectives on congenital obstructive nephropathy.

Congenital obstructive nephropathy is the leading cause of chronic renal disease in children. As a result, it represents a tremendous societal burden in terms of morbidity and mortality, as well as in health care expenses of caring for children with chronic kidney disease and end-stage renal disease. The various diagnostic, prognostic, and therapeutic challenges associated with congenital obstructive nephropathy highlight the importance of developing effective experimental models for studying this disease process.

Pathogenesis of renal injury in the megabladder mouse: a genetic model of congenital obstructive nephropathy.

Congenital obstructive nephropathy (CON) is the most common cause of chronic renal failure in children often leading to end-stage renal disease. The megabladder (mgb) mouse exhibits signs of urinary tract obstruction in utero resulting in the development of hydroureteronephrosis and progressive renal failure after birth. This study examined the development of progressive renal injury in homozygous mgb mice (mgb-/-).

Identification of distinct myocardin splice variants in the bladder.

Purpose: Although the importance of myocardin in smooth muscle development is well established, many tissue specific intricacies of smooth muscle differentiation remain to be determined. We characterized myocardin expression in the developing and adult bladder to identify potential tissue specific differences that may have a role in detrusor smooth muscle development.

Materials And Methods: Reverse transcriptase and quantitative polymerase chain reaction were done to determine myocardin expression in the mouse and human bladder vs various other tissues.

Management and etiology of the unilateral multicystic dysplastic kidney: a review.

In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction.

Identification and characterization of novel human transcripts embedded within HMGA2 in t(12;14)(q15;q24.1) uterine leiomyoma.

The high mobility group A2 protein (HMGA2) has been implicated in the pathogenesis of mesenchymal tumors such as leiomyoma, lipoma and hamartoma. HMGA2 was pinpointed by mapping the breakpoints in the chromosomal translocations in 12q15, especially the t(12;14) that is commonly seen in uterine leiomyoma. It is generally assumed that altered expression of HMGA2 is an early event in the pathway to tumor formation.

The incidence of potential interactions between dietary supplements and prescription medications in cancer patients at a Veterans Administration Hospital.

Objective: The concurrent use of dietary supplements and prescription medications is common among patients with cancer. This study examines potential interactions between dietary supplements and prescription medications in a Veteran Hospital cancer population.

Methods: Eligible patients seen at the Hematology/Oncology clinic at the Veterans Administration Medical Center in Cincinnati, OH, were administered a survey to determine their use of dietary supplements.