Hemolytic Non-uremic Syndrome
Chen, K. S., Neuneright, C. E., Crary, S. E., & Buchanan, G. R. (2012). Hemolytic non-uremic syndrome. Pediatric blood & cancer, 59(1), 167–169. https://doi.org/10.1002/pbc.23264
View Original Source →Abstract
More than 50% of patients with non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) progress to ESRD. Kidney transplant failure for disease recurrence is common; hence, whether renal transplantation is appropriate in this clinical setting remains a debated issue. The aim of this study was to identify possible prognostic factors for renal transplant outcome by focusing on specific genetic abnormalities associated with the disease. All articles in literature that describe renal transplant outcome in patients with ESRD secondary to non-Stx-HUS, genotyped for CFH, MCP, and IF mutations, were reviewed, and data of patients who were referred to the International Registry of Recurrent and Familial HUS/TTP and data from the Newcastle cohort were examined. This study confirmed that the overall outcome of kidney transplantation in patients with non-Stx-HUS is poor, with disease recurring in 60% of patients, 91.6% of whom developed graft failure. No clinical prognostic factor that could identify patients who were at high risk for graft failure was found. The presence of a factor H (CFH) mutation was associated with a high incidence of graft failure (77.8 versus 54.9% in patients without CFH mutation). Similar results were seen in patients with a factor I (IF) mutation. In contrast, graft outcome was favorable in all patients who carried a membrane co-factor protein (MCP) mutation. Patients with non-Stx-HUS should undergo genotyping before renal transplantation to help predict the risk for graft failure. It is debatable whether a kidney transplant should be recommended for patients with CFH or IF mutation. Reasonably, patients with an MCP mutation can undergo a kidney transplant without risk for recurrence.
Case Details
Disease Location
Systemic
Personal Characteristics
3 -year-old male
Clinical Characteristics
Briefly admitted for bloody diarrhea with negative stool cultures and a hemoglobin concentration of 12.4 g/dl ten days after discharge he returned with pallor and edema. His hemoglobin was 3.7 g/dl with an elevated reticulocyte count and schistocytes on peripheral smear. Platelet count was 135,000/mm3 hemolytic uremic syndrome (hus) was suspected, and edema was presumed to be from renal failure or hypoalbuminemia
Remission Characteristics
Following transfusion, his hemoglobin concentration stabilized and then increased after hydration, serum creatinine was 0.5 mg/dl. Following transfusion, his hemoglobin concentration stabilized and then increased two weeks later his hemoglobin concentration was 11.5 g/dl without evidence of hemolysis.
Treatment & Mechanisms
Proposed Remission Mechanisms
The children reported here perhaps therefore represent a variant of hus which we have termed ‘‘hemolytic non-uremic syndrome’’. Its transient and limited nature implies that it, too, is toxin-mediated, perhaps by a novel mechanism not heretofore identified. Alternatively, host factors may possibly protect these children from developing overnight renal failure through down-regulation of renal gb3 receptors or a polymorphism that confers resistance to shiga toxin..
Clinical Treatment
Transfusion with red packed cells. Hydration
Non-Clinical Treatment
The cases described above do not fit into these well-defined entities