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IgA nephropathy: clinical features and natural history--a pediatric perspective
1Department of Pediatrics, Baylor University Medical Center, Dallas, TX 75246.
Insights
IgA nephropathy (IgAN) in children often presents with hematuria after infections. Increased awareness is needed among healthcare providers to ensure timely diagnosis and appropriate management of this kidney disease.
Area of Science:
- Pediatric Nephrology
- Immunology
- Glomerular Diseases
Background:
- IgA nephropathy (IgAN) is a kidney disease with varied global presentations in children.
- Typical symptoms include hematuria post-respiratory infection, but awareness among clinicians is often low.
- Screening programs reveal IgAN through microscopic hematuria or proteinuria, especially in Japan.
Purpose of the Study:
- To review the clinical spectrum of IgA nephropathy in pediatric patients worldwide.
- To highlight diagnostic challenges and the need for increased physician awareness.
- To discuss disease progression and potential therapeutic strategies.
Main Methods:
- Review of clinical features and presentations of IgA nephropathy in pediatric populations.
- Analysis of diagnostic pathways and awareness levels among healthcare professionals.
- Examination of disease progression, histological findings, and associated clinical outcomes.
Main Results:
- Macroscopic hematuria post-infection is a common presentation, often leading to unnecessary urologic evaluations.
- Microscopic hematuria/proteinuria detected via screening is frequent in some regions, but underdiagnosis may occur.
- A subset of patients (10-30%) shows disease progression, with hypertension and proteinuria indicating chronic kidney disease.
Conclusions:
- Improved awareness of IgA nephropathy is crucial for pediatricians, family practitioners, and urologists.
- Early diagnosis through urinalysis and appropriate biopsy practices are essential.
- Multicenter studies are needed to evaluate therapies for progressive IgAN in children with severe histological findings.
Abstract:
This review describes the spectrum of clinical features observed in pediatric patients with IgA nephropathy (IgAN) in different parts of the world. The typical clinical presentation consists of an episode of macroscopic hematuria within 24 to 48 hours of an upper respiratory infection. However, many children who present with macroscopic hematuria are subjected to a battery of urologic studies before the appropriate procedure is performed. This sequence highlights the lack of awareness of IgAN among pediatricians, family practitioners, and urologists. The finding of microscopic hematuria or, less commonly, proteinuria, in a urinalysis carried out as part of a school screening program is the most frequent "presentation" of IgAN in Japanese children. However, it is possible that many children with IgAN expressed as microscopic hematuria and/or mild proteinuria remain undiagnosed in this country because routine urinalysis is not done and many pediatric nephrologists are reluctant to perform renal biopsies when such children are identified. It is now recognized that some patients with IgAN and nephrotic range proteinuria exhibit a state of steroid responsiveness. The renal biopsy in such patients often reveals "minimal change." Several recent studies have shown progressive deterioration in approximately 10% of all pediatric patients found to have IgAN and in 15% to 30% of the subset of patients with more severe histologic findings. Hypertension and proteinuria are observed frequently in patients who progress to chronic renal failure. It is proposed that multicenter collaborative studies be designed to evaluate proposed therapies for children with IgAN associated with proliferative glomerular lesions, particularly those in whom hypertension, proteinuria, and depressed glomerular filtration rate are found.