Atypical Hemolytic Uremic Syndrome in Children
S Chelghoum1*, F Haddoum1, MT Hamlaoui2 and K Djenouhat3
1Nephrology Department, Mustapha University Hospital Center, Algiers, Algeria
2Pediatric Nephrology Department, Nafissa Hammoud University Hospital Center, Algiera
3Biochemistry and Immunology Department, Rouiba Public Hospital, Algiers, Algeria
*Corresponding Author: S Chelghoum, Nephrology Department, Mustapha University Hospital Center, Algiers, Algeria.
December 31, 2021; Published: May 27, 2022
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy, mostly is a reflection of serious pathological state that can be life-threatening. HUS has renal tropism. 90% of childhood HUS caused by an E.coli infection producing shigatoxin (STEC). Atypical HUS (aHUS) is a genetic disease caused by deregulation of the alternate complement pathway, the mortality rate is around 10% with more than 50% progression to ESRD. Renal sequelae (proteinuria, hypertension, CKD) may be considered. Several prognostic factors have been identified, the length of anuria making up the main one. Evaluating these severity factors for long-term kidney damage is an important issue. In our cohort of 21 children had aHUS, the rate of renal function recovery and death was 43% and 52%, respectively. Overall survival was fatal for the first 3 months, neurological involvement was significantly associated, at the age of <2 years old, and time limit to dialysis> 48 hours. In multivariate analysis, delayed dialysis multiplies the risk of death by 15. At 4 years of development, 80% of the children had renal sequelae. Event-free survival (proteinuria, hypertension and/or CKD) is significantly related to neurological damage and delayed dialysis. In multivariate analysis, delayed dialysis increases the risk of renal sequelae by a factor of 21. Early diagnosis and treatment are important prognostic factors in aHUS.
Keywords: Atypical Hemolytic Uremic Syndrome; Risk Factors; Renal Sequelae; Long-term Prognosis
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