Siegler RL, Pavia AT, Sherbotle JR, Cook JB, Wallace S
Familial hemolytic uremic syndrome (HUS).
ASN 30th Annual Meeting, San Antonio
J Am Soc Nephrol (Sep) 8:130A 1997

Non-concurrent familial HUS is usually considered to have a different etiology and prognosis from epidemic or diarrheal HUS which affects multiple family members. However, authors from a large center with wide experience treating diarrheal HUS draw attention to 3 families in which siblings or cousins developed diarrheal HUS separated by > 2 years. Their course and outcome suggest an etiology similar to other cases of diarrheal HUS. In the experience of this center, non-concurrent diarrheal HUS is more common than familial HUS.

Comment: This report makes several interesting observations. First, as the incidence of diarrheal HUS increases (perhaps as a result of a greater prevalence of E. Coli O157:H7), non-concurrent cases in a family will be less likely to represent an hereditary, relapsing, non-diarrheal form of HUS. Such an observation counters previously held notions about distinguishing between these two distinct forms of HUS. Second, there is likely a genetic predisposition which explains why only a small minority of patients with enteric infection with verotoxin-producing organisms develop HUS. These families provide a window into that predisposition. (Susan Mendley, M.D., Northwestern University, Chicago, IL)

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ASN 30th Annual Meeting, San Antonio
ARF etiology : Hemolytic/uremic syndrome and TTP
Proteinuria/Hematuria : Hereditary hematuria/proteinuria