Blockade of the Terminal Complement Cascade Using Ravulizumab in a Pediatric Patient With Anti-complement Factor H Autoantibody-Associated aHUS: A Case Report and Literature Review.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease in pediatrics with 6-10% of cases associated with complement factor H autoantibodies Ravulizumab is a new treatment option available for long-term management through blockage of the terminal complement cascade. We report a case of a previously healthy eight-year-old female who presented with hemolytic anemia, thrombocytopenia, and acute kidney injury. Low complement C3, normal ADAMTS13, and negative rheumatology and infectious disease panels suggested aHUS.

Renal Involvement and Its Treatment in Pediatric Patients With Proteinase-3 Anti-Neutrophil Cytoplasmic Antibody Positive Granulomatosis With Polyangiitis: A Case Series.

We describe three pediatric patients between the ages of 10 and 14 years old who were diagnosed with granulomatosis with polyangiitis (GPA) between 2014 and 2019. Each case involves variations in presentation, symptomatology, diagnostics, and induction and maintenance therapy regimens. Patient 1 presented with significant renal involvement, hypertensive emergency, and focal alveolar hemorrhage, a rare presentation of GPA that causes up to 60% mortality.

Complete Renal Recovery in Pediatric Patient with C3 Glomerulonephritis: A Case Report.

C3 glomerulonephritis (C3GN) is a rare kidney disease resulting from dysregulation of the alternative complement cascade. Without treatment, approximately 70% of affected children and 30-50% of affected adults will develop worsening of proteinuria and progress to end-stage renal disease within 10 years of diagnosis. Here, we describe a 9-year-old Sudanese girl with no significant past medical history who presented to the Emergency Department with a 2-month history of fatigue, poor oral intake, and worsening facial and lower extremity edema, and subsequently found to have anemia, hypoalbuminemia, microscopic hematuria, and proteinuria.

Urinary sodium to creatinine ratio in healthy infants.

Rationale Sodium is essential for adequate growth; the daily requirement in infants is 2 to 3 mmol/kg/d. Objectives The study aimed to establish a reference range for the ratio of urinary sodium (UNa) to creatinine (UCr) in healthy infants in a suburban setting in the United States. Methods UNa and UCr were measured in healthy infants and UNa:UCr was calculated.

Protein S deficiency manifesting as spontaneous aortic thrombosis in a neonate.

Purpose: This article describes a neonate with protein S deficiency who presented with life-threatening thrombosis of the aorta. This is the first case report of a neonate with protein S deficiency presenting with aortic thrombosis.

Patients And Methods: A 9-day-old boy presented with a history of dark colored urine, vomiting, and apnea.