Hemolytic-uremic syndrome risk factors
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2], Anila Hussain, MD [3] Parth Vikram Singh, MBBS[4]
Overview
The most potent risk factor in the development of hemolytic uremic syndrome (HUS) in childhood is infection with verocytotoxin (shiga-like toxin)-producing bacteria, usually enterohemorrhagic Escherichia coli (VTEC/STEC),and in some tropical regions Shigella dysenteriae type I . Other risk factors include genetic mutations in complement factors, pnemumococcal infections, autoimmune diseases like SLE and antiphospholipid syndrome, pregnancy, antineoplastic and immunosupressive drugs, HIV infection and organ transplantation.
Risk Factors
The most potent risk factor in the development of HUS is etiology advanced and clinical associations.
etiology advance
- Shiga and verocytotoxin (shiga-like toxin)-producing bacteria; enterohemorrhagic Escherichia coli, Shigella dysenteriaen type 1, Citrobacter
- Streptococcus pneumoniae, neuraminidase, and T-antigen exposure
- Disorders of complement regulation[3][4]
- Complement factor H (CFH) mutation/ factor H defeciency (autosomal dominant)
- Complement factor I(CFI) defeciency (acquired antibody mediated)
- Membrane co-factor protein defeciency (MCP; CD46)
- Factor B overactivity (Complement factor B mutation)
- Diacylglycerol kinase epsilon gene mutations
- von Willebrand proteinase, ADAMTS13 deficiency
- Genetic disorders of ADAMTS13[5]
- Acquired von Willebrand proteinase deficiency; autoimmune, drug induced
- Defective cobalamine metabolism[6].
Clinical associations with following diseases:
- Malignancy, cancer chemotherapy and ionizing radiation
- Calcineurin inhibitors and transplantation
- Pregnancy, HELLP syndrome and oral contraceptive pill
- Systemic lupus erythematosis and antiphospholipid antibody syndrome
- Glomerulopathy
- Familial, not included in part 1
- Unclassified
Risk factors for progression from high-risk STEC infection to HUS include the following unmodifiable factors:[7]
- younger age below 5 years, older age above 75 years,
- female sex,
- bloody diarrhea,
- vomiting,
- absence of Shiga toxin 1 when Shiga toxin 2 is present,
- white-cell count of at least 13,000/mm3,
- and an initial platelet count below 250,000/mm3.
Potentially modifiable or possibly modifiable risk factors include:
- antibiotic exposure,
- narcotic or antidiarrheal medication use,
- dehydration,
- elative hemoconcentration, and
- hyponatremia.
Risk factors for progression to severe HUS include:
- shorter diarrheal prodrome,
- hyponatremia,
- lack of parenteral volume expansion,
- delayed pathogen identification,
- antibiotic administration, and,
- at the time of HUS diagnosis, hypocalcemia, central nervous system involvement, increased neutrophil count, relative hemoconcentration, hyponatremia, hypoalbuminemia, and antecedent respiratory infection.
References
- ↑ Majowicz SE, Scallan E, Jones-Bitton A, Sargeant JM, Stapleton J, Angulo FJ; et al. (2014). "Global incidence of human Shiga toxin-producing Escherichia coli infections and deaths: a systematic review and knowledge synthesis". Foodborne Pathog Dis. 11 (6): 447–55. doi:10.1089/fpd.2013.1704. PMC 4607253. PMID 24750096.
- ↑ Mark Taylor C (2008). "Enterohaemorrhagic Escherichia coli and Shigella dysenteriae type 1-induced haemolytic uraemic syndrome". Pediatr Nephrol. 23 (9): 1425–31. doi:10.1007/s00467-008-0820-3. PMC 2459235. PMID 18493800.
- ↑ Frémeaux-Bacchi V (2013). "[Pathophysiology of atypical hemolytic uremic syndrome. Ten years of progress, from laboratory to patient]". Biol Aujourdhui. 207 (4): 231–40. doi:10.1051/jbio/2013027. PMID 24594571.
- ↑ Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher MA, Niaudet P, Guest G; et al. (2007). "Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome". J Am Soc Nephrol. 18 (8): 2392–400. doi:10.1681/ASN.2006080811. PMID 17599974.
- ↑ Feng S, Eyler SJ, Zhang Y, Maga T, Nester CM, Kroll MH; et al. (2013). "Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome". Blood. 122 (8): 1487–93. doi:10.1182/blood-2013-03-492421. PMC 3750341. PMID 23847193.
- ↑ Adrovic A, Canpolat N, Caliskan S, Sever L, Kıykım E, Agbas A; et al. (2016). "Cobalamin C defect-hemolytic uremic syndrome caused by new mutation in MMACHC". Pediatr Int. 58 (8): 763–5. doi:10.1111/ped.12953. PMID 27324188.
- ↑ Freedman SB, van de Kar NC, Tarr PI (October 2023). "Shiga Toxin-Producing Escherichia coli and the Hemolytic-Uremic Syndrome". N Engl J Med. 389 (15): 1402–1414. doi:10.1056/NEJMra2108739. PMID 37819955 Check
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