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Published on: November 1, 2018
Crescentic glomerulonephritis in children
Ulrike Mayer1, Jessica Schmitz2, Jan Hinrich Bräsen2
1Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany.
Insights
Early diagnosis and aggressive treatment are crucial for managing pediatric crescentic glomerulonephritis (cGN). Cyclosporine A and mycophenolate mofetil show promise as effective alternatives for treating this kidney disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Crescentic glomerulonephritis (cGN) is a significant cause of acute kidney injury in children, yet knowledge regarding its nuances remains limited.
- Understanding the diverse etiologies and clinical trajectories of pediatric cGN is essential for improving patient outcomes.
Purpose of the Study:
- To investigate the underlying causes, clinical presentations, and treatment outcomes of pediatric crescentic glomerulonephritis.
- To identify factors influencing treatment success and explore alternative therapeutic strategies.
Main Methods:
- Retrospective analysis of kidney biopsy results, clinical data, and laboratory findings in 60 pediatric patients diagnosed with cGN over 16 years.
- Evaluation of various treatment regimens, including corticosteroids, immunosuppressants (mycophenolate mofetil, cyclosporine A, cyclophosphamide, rituximab, azathioprine, tacrolimus), and plasmapheresis.
Main Results:
- Immune complex glomerulonephritis (ICGN) was the most common underlying cause (75%), with IgA nephropathy being the most frequent subtype.
- Patient outcomes varied based on the class of cGN, diagnostic delay, biopsy findings (crescent percentage, tubular atrophy, interstitial fibrosis, necrosis), and clinical factors like nephrotic syndrome and hypertension.
- A significant proportion of patients required intensive immunosuppressive therapy, including multiple corticosteroid pulses and combination therapies.
Conclusions:
- Successful treatment of pediatric cGN hinges on early diagnosis, prompt and aggressive therapeutic interventions, and consideration of the specific underlying pathology.
- Cyclosporine A (CsA) and mycophenolate mofetil (MMF) demonstrate potential as effective therapeutic alternatives to cyclophosphamide in managing pediatric cGN.
Background:
To date, there is insufficient knowledge about crescentic glomerulonephritis (cGN), the most frequent immunologic cause of acute kidney injury in children.
Methods:
Over a period of 16 years, we retrospectively analyzed kidney biopsy results, the clinical course, and laboratory data in 60 pediatric patients diagnosed with cGN.
Results:
The underlying diseases were immune complex GN (n = 45/60, 75%), including IgA nephropathy (n = 19/45, 42%), lupus nephritis (n = 10/45, 22%), Henoch-Schoenlein purpura nephritis (n = 7/45, 16%) and post-infectious GN (n = 7/45, 16%), ANCA-associated pauci-immune GN (n = 10/60, 17%), and anti-glomerular basement-membrane GN (n = 1/60, 2%). Patient CKD stages at time of diagnosis and at a median of 362 days (range 237-425) were CKD I: n = 13/n = 29, CKD II: n = 15/n = 9, CKD III: n = 16/n = 7, CKD IV: n = 3/n = 3, CKD V: n = 13/n = 5. Course of cGN was different according to class of cGN, duration of disease from first clinical signs to diagnosis of cGN by biopsy, percentage of crescentic glomeruli, amount of tubular atrophy/interstitial fibrosis and necrosis on renal biopsy, gender, age, nephrotic syndrome, arterial hypertension, dialysis at presentation, and relapse. Forty-eight/60 children were treated with ≥ 5 (methyl-) prednisolone pulses and 53 patients received oral prednis(ol)one in combination with mycophenolate mofetil (n = 20), cyclosporine A (n = 20), and/or cyclophosphamide (n = 6), rituximab (n = 5), azathioprine (n = 2), tacrolimus (n = 1), and plasmapheresis/immunoadsorption (n = 5).
Conclusions:
The treatment success of cGN is dependent on early diagnosis and aggressive therapy, as well as on the percentage of crescentic glomeruli on renal biopsy and on the underlying type of cGN. CsA and MMF seem to be effective alternatives to cyclophosphamide.
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