Acute kidney injury following treatment with CD19-specific CAR T-cell therapy in children, adolescent, and young adult patients with B-cell acute lymphoblastic leukemia.

Background: CD19-specific chimeric antigen receptor (CAR) T-cell therapy has shown promising disease responses in patients with high-risk B-cell malignancies. However, its use may be related to complications such as immune-mediated complications, infections, and end-organ dysfunction. The incidence of post-CAR T-cell therapy acute kidney injury (AKI) in the children, adolescent, and young adult (CAYA) patient population is largely unreported.

Acute kidney injury following treatment with CD19-specific CAR T-cell therapy in children, adolescent and young adult patients with B-cell acute lymphoblastic leukemia.

CD19-specific chimeric antigen receptor (CAR) T-cell therapy has shown promising disease responses in patients with high-risk B-cell malignancies. Treatment with CD19-CAR T-cell therapy is also associated with the risk of morbidity and mortality, primarily related to immune-mediated complications (cytokine release syndrome [CRS] and neurotoxicity [NTX]), infections, and end-organ dysfunction. Despite these well-described systemic toxicities, the incidence of post-CAR T-cell therapy acute kidney injury (AKI) in the children, adolescent and young adult (CAYA) patient population is largely unreported.

Elucidation of Renal Scars in Children With Vesicoureteral Reflux Using Contrast-Enhanced Ultrasound: A Pilot Study.

Introduction: Vesicoureteral reflux is a common disorder in children but can result in kidney scarring following acute pyelonephritis. The gold standard diagnostic to detect renal scars in children is Tc-dimercaptosuccinic acid (DMSA) scintigraphy. DMSA has a number of limitations including radiation exposure, need for sedation, and radiotracer supply shortages.

Comprehensive renal function evaluation in patients treated for synchronous bilateral Wilms tumor.

Objectives: The purpose of this study was to perform a comprehensive assessment of long-term renal function in patients treated at our institution for synchronous bilateral Wilms tumor (BWT) and to determine the optimal method for estimating glomerular filtration rate (eGFR).

Methods: Surgical approach, adjuvant therapy, and pathology reports were reviewed for patients with at least six months follow-up from definitive surgery. eGFRs, as assessed by the Schwartz and Chronic Kidney Disease in Children (CKiD) formulas, were compared to measured GFR (mGFR) determined by Tc-DTPA scanning.

Overall Survival and Renal Function of Patients With Synchronous Bilateral Wilms Tumor Undergoing Surgery at a Single Institution.

Objectives: Wilms tumor is the most common renal cancer in children. Approximately 5% of children with Wilms tumor present with disease in both kidneys. The treatment challenge is to achieve a high cure rate while maintaining long-term renal function.

Renal function in survivors of nonsyndromic Wilms tumor treated with unilateral radical nephrectomy.

Background: Partial nephrectomy is considered by some for children with unilateral Wilms tumor (UWT) to avoid the theoretical complication of renal insufficiency. In the current study, the authors evaluated the prevalence of hypertension and impaired renal function in long-term survivors of nonsyndromic UWT who were treated without nephrotoxic chemotherapy or ionizing radiation.

Methods: Eligibility included age ≤15 years at the time of diagnosis of nonsyndromic UWT, treatment receipt before 2002, and maintenance of disease remission after unilateral nephrectomy without receipt of abdominal irradiation or nephrotoxic chemotherapy.

Central neurogenic hyperventilation and renal tubular acidosis in children with pontine gliomas.

Central neurogenic hyperventilation (CNH) is a rare neurologic condition that develops secondary to brainstem involvement. Approximately 35 noncomatose patients with CNH have been reported, including 7 with either a diffuse (patients 2–10 in the table) or focal (patient 1 in the table) brainstem glioma. Although diffuse intrinsic pontine glioma (DIPG) is the most common pediatric brainstem cancer, only 1 case report described a child with DIPG who experienced CNH.

Characterization of the residues in helix 8 of the human beta1-adrenergic receptor that are involved in coupling the receptor to G proteins.

Several key amino acids within amphipathic helix 8 of the human beta1-adrenergic receptor (beta1-AR) were mutagenized to characterize their role in signaling by G protein-coupled receptors. Mutagenesis of phenylalanine at position 383 in the hydrophobic interface to histidine (F383H) prevented the biosynthesis of the receptor, indicating that the orientation of helix 8 is important for receptor biosynthesis. Mutagenesis of aspartic acid at position 382 in the hydrophilic interface to leucine (D382L) reduced the binding and uncoupled the receptor from G protein activation.

C1q nephropathy: features at presentation and outcome.

The study population comprised all 20 patients followed since 1990 through December 2004 at the Le Bonheur Children's Medical Center with diagnosis of C1q nephropathy (55% boys; 60% African Americans). All were aged under 18 years at biopsy (mean 11.2 years, 65% aged 11 or over); the youngest presented at age 10 months and progressed to end-stage renal disease at 14 months.

Pediatric IgA nephropathies: clinical aspects and therapeutic approaches.

The pediatric IgA nephropathies are IgA nephrothapy (Berger's Disease) and Henoch-Schönlein purpura nephritis. Both conditions are reviewed in detail with respect to epidemiology, clinical features, outcome, prognostic markers, and therapeutic approaches. For both conditions variable disease severity and outcome along with the lack of conclusive evidence for efficacy of treatment based on randomized clinical trials makes it difficult to make strong recommendations regarding therapy.

Role of the cyclic AMP-dependent protein kinase in homologous resensitization of the beta1-adrenergic receptor.

A fundamental question in biology is how the various motifs in G protein-coupled receptors participate in the divergent functions orchestrated by these molecules. Here we describe a fundamental role for a serine residue at position 312 in the third intracellular loop of the human beta(1)-adrenergic receptor (beta(1)-AR) in endocytic recycling of the agonist-internalized receptor. In receptor recycling experiments that were monitored by confocal microscopy, the agonist-internalized wild-type (WT) beta(1)-AR recycled with a t(0.