Common presentation of micro hematuria and proteinuria in an uncommon disease: Answers
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CLINICAL QUIZ
Common presentation of micro hematuria and proteinuria in an uncommon disease: Answers Marwa Mansour 1 & Danielle Young 1 & Mohammad Ilyas 1 & Asad Tolaymat 1 Received: 22 March 2020 / Revised: 27 March 2020 / Accepted: 31 March 2020 # IPNA 2020
Keywords Child . Monoclonal gammopathy of renal significance . Proliferative glomerulonephritis with monoclonal immunoglobulin deposits . PGNMID . Enalapril . Prednisolone
Answers 1. What is your differential diagnosis? Hematuria and proteinuria is the common presentation of glomerulonephritis. To confirm the underlying pathology, the patient had a kidney biopsy that revealed membranoproliferative glomerulonephritis (MPGN) with monoclonal IgG lambda deposits. 2. What further studies would you recommend? With histology revealing monoclonal IgG lambda deposits, serum and urine protein electrophoresis was performed that did not show detectable monoclonal immunoglobulin. Her blood flow cytometry was negative. 3. What are the causes and pathophysiology for the above diagnosis? Monoclonal gammopathy of renal significance (MGRS) is a rare cause of her acute glomerulonephritis. 4. What is the initial treatment for acute glomerulonephritis in this patient?
This refers to the article that can be found at https://doi.org/10.1007/ s00467-020-04565-9. * Marwa Mansour [email protected] 1
University of Florida College of Medicine, Jacksonville, FL, USA
She was started on enalapril 10 mg daily, solumedrol 500mg IV daily for 3 days, and followed by prednisolone 60 mg daily. She was given calcium and vitamin D supplements. 5. What are the long-term treatment options to prevent progression of disease? One month later, her gross hematuria subsided. Two months later, her protein-to-creatinine ratio improved from 1897 to 871-mg/g Cr. Prednisolone was gradually tapered off over 6 months. The cushingoid features improved after prednisolone was stopped. Her blood pressure and serum sugar continued to be normal. One year after initial presentation, she had no hematuria and minimal proteinuria (urine proteinto-creatinine ratio 372-mg/g Cr). Her serum BUN and creatinine continued to be normal. She was continued on ACEI. 6. What is the prognosis? She has been monitored for the last 2 years with normal kidney function but persistent minimal proteinuria. Her blood pressure remains normal.
Discussion MGRS represents a group of disorders in which a monoclonal immunoglobulin secreted by a nonmalignant or premalignant B cell or plasma cell clone causes kidney damage [1]. MGRS includes immunoglobulin-associated kidney disorders such as amyloidosis, the monoclonal immunoglobulin deposition diseases (MIDDs) (light-chain deposition disease, heavy-chain deposition disease, and light and heavy–chain deposition disease), C3 glomerulopathy with monoclonal gammopathy, light-chain proximal tubulopathy (Fanconi’s
Pediatr Nephrol
syndrome), and proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). PGNMID is a disease entity characterized by a pattern of prolif
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