Hemolytic uremic syndrome (HUS) is a multisystem thrombotic microangiopathic disease characterized by the triad of renal failure, hemolytic anemia and thrombocytopenia. It is the most common cause of renal failure in infancy and childhood requiring dialysis.
There are two forms of this syndrome:
- typical or D+ HUS: corresponds to >90% of all HUS, occurs in childhood and is caused by Shiga toxin-producing Escherichia coli
- atypical or D- HUS: can occur after infections, use of certain drugs, following other pathologies (e.g. malignancy), or, rarely, due to defective cobalamin metabolism 3
This article is based on the features related to the typical HUS.
HUS affects ~ 2:100,000 people worldwide 3.
It is most commonly seen in young children.
Usually, there is an interval of 2-12 days between the ingestion of contaminated food and the first day of diarrhea and abdominal pain. After 1-3 days the diarrhea becomes bloody (~90%). Fever is usually absent.
HUS usually occurs (>90%) following a gastrointestinal infection with Shiga toxin-producing E. coli (STEC). Some cases are associated with medical conditions such as collagen vascular conditions like SLE, underlying malignancy, or medications such as cyclosporine, OCP, or 5 FU.
Injury to the endothelium of the capillaries results in mechanical destruction of the RBCs. Kidneys can be the only organ involved; however involvement of the other organ like liver, pancreas, heart, intestine, muscles also possible.
History and etymology
The term was coined by the Swiss doctor Conrad von Gasser in 1955. The relation of E. coli as an aetiological factor was proposed by Mohamed A. Karmali in 1985 2,4.
- 1. Pollard B. Handbook of Clinical Anaesthesia 3E. CRC Press. ISBN:144410862X. Read it at Google Books - Find it at Amazon
- 2. Textbook of Clinical Pediatrics. Springer. ISBN:3642022022. Read it at Google Books - Find it at Amazon
- 3. Gulati S. Childhood Renal Disorders - ECAB. Elsevier Health Sciences. (2014) ISBN:8131239365. Read it at Google Books - Find it at Amazon
- 4. Karmali MA, Petric M, Lim C et-al. The association between idiopathic hemolytic uremic syndrome and infection by verotoxin-producing Escherichia coli. J. Infect. Dis. 1985;151 (5): 775-82. Pubmed citation