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Erschienen in:
27.01.2022 | Clinical Quiz
A boy with purpura and generalized edema: Answers
Erschienen in: Pediatric Nephrology | Ausgabe 6/2022
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1.
What is your diagnosis?
Immunoglobulin A vasculitis (IgAV) with rapidly progressive glomerulonephritis (RPGN) and acute myopericarditis with acute decompensated heart failure.
2.
How can you explain the cause of acute kidney injury?
The causes of acute kidney injury in this patient could be from crescentic glomerulonephritis from IgAV and prerenal azotemia from acute myocarditis.
3.
What should be done next in the management of this patient?
Intensive immunosuppressive therapy with high-dose corticosteroids and intravenous cyclophosphamide is recommended in patients with severe IgAV nephritis, according to the European consensus-based recommendations.
The patient received 30 mg/kg of intravenous methylprednisolone for 5 days, combined with monthly intravenous cyclophosphamide. Then, 60 mg/day of oral prednisolone was continued. He was hospitalized for 23 days. Before discharge, his clinical symptoms were improved, with no dyspnea or edema. His purpura faded. He had urine output approximately 1,300–1,500 mL/day. Serum creatinine was 2.25 mg/dL (eGFR 26 mL/min/1.73 m2). A follow-up echocardiogram showed an improvement of left ventricular ejection fraction (LVEF) at 59% and trivial pericardial effusion. At his follow-up visits, urination decreased to 500 mL/day and serum creatinine gradually increased to 7.18 mg/dL. Despite the intensive immunosuppressive therapy, his kidney function got worse and progressed to kidney failure. Kidney replacement therapy was started in the fourth month after diagnosis.