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Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not?
Serra Sürmeli-Döven1, Ali Delibaş1, İclal Gürses2
1Departments of Pediatric Nephrology, Mersin University Faculty of Medicine, Mersin, Turkey.
Insights
This case study explores a rare co-occurrence of hemolytic uremic syndrome (HUS) and IgA nephropathy (IgAN) in a child. The findings suggest a potential, though not fully understood, connection between these two distinct conditions.
Area of Science:
- Pediatrics
- Nephrology
- Hematology
Background:
- Hemolytic uremic syndrome (HUS) is a serious condition characterized by anemia, thrombocytopenia, and acute kidney injury.
- Immunoglobulin A nephropathy (IgAN) is a primary glomerulonephritis often presenting with hematuria.
Observation:
- An 18-month-old boy presented with symptoms of HUS, including diarrhea, anemia, thrombocytopenia, and acute renal failure.
- Despite negative Shiga toxin-producing E. coli (STEC) and atypical HUS (aHUS) genetic testing, the child developed respiratory distress, hypertrophic cardiomyopathy, and seizures.
- Clinical and histological findings confirmed both HUS and IgAN.
Findings:
- The patient demonstrated a positive response to steroid treatment and plasma exchange therapy combined with peritoneal dialysis.
- The simultaneous diagnosis of HUS and IgAN in this pediatric case is unusual and warrants further investigation.
- Genetic testing for aHUS was negative, ruling out common genetic predispositions for atypical HUS.
Implications:
- This case highlights the importance of considering co-existing renal and hematological conditions in pediatric patients with complex presentations.
- The study prompts further research into potential shared pathophysiological mechanisms or triggers linking HUS and IgAN.
- Understanding such rare associations can improve diagnostic approaches and therapeutic strategies for children with severe kidney and blood disorders.
Abstract:
Sürmeli-Döven S, Delibaş A, Gürses İ, Kayacan UR, Coşkun-Yılmaz B, Esen K, Korkmaz E, Özaltın F. Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not? Turk J Pediatr 2018; 60: 81-85. A previously healthy 18-month old boy, presenting with diarrhea, anemia, thrombocytopenia and acute renal failure was admitted to our hospital. Hemolytic uremic syndrome (HUS) was diagnosed with his clinical and laboratory findings. His stool was negative for Shiga toxin producing E. coli (STEC). During follow-up he developed respiratory distress, hypertrophic cardiomyopathy and seizure. His genetic tests for atypical HUS (aHUS) were negative. His clinical and histological findings indicated hemolytic uremic syndrome and immunglobulin A nephropathy (IgAN). The patient responded to steroid treatment and plasma exchange therapy with peritoneal dialysis. We discuss the probable connection between HUS and IgAN.
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