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