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2016 ; 16
(ä): 285
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Clinical features, therapeutic interventions and long-term aspects of
hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study
from 1999-2008
#MMPMID27297224
Jenssen GR
; Vold L
; Hovland E
; Bangstad HJ
; Nygård K
; Bjerre A
BMC Infect Dis
2016[Jun]; 16
(ä): 285
PMID27297224
show ga
BACKGROUND: Hemolytic-uremic syndrome (HUS) is a clinical triad of
microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia,
primarily affecting pre-school-aged children. HUS can be classified into
diarrhea-associated HUS (D(+)HUS), usually caused by Shiga toxin-producing
Escherichia coli (STEC), and non-diarrhea-associated HUS (D(-)HUS), both with
potentially serious acute and long-term complications. Few data exists on the
clinical features and long-term outcome of HUS in Norway. The aim of this paper
was to describe these aspects of HUS in children over a 10-year period. METHODS:
We retrospectively collected data on clinical features, therapeutic interventions
and long-term aspects directly from medical records of all identified HUS cases
<16 years of age admitted to Norwegian pediatric departments from 1999 to 2008.
Cases of D(+)HUS and D(-)HUS are described separately, but no comparative
analyses were possible due to small numbers. Descriptive statistics are presented
in proportions and median values with ranges, and/or summarized in text. RESULTS:
Forty seven HUS cases were identified; 38 D(+)HUS and nine D(-)HUS. Renal
complications were common; in the D(+)HUS and D(-)HUS group, 29/38 and 5/9
developed oligoanuria, 22/38 and 3/9 needed dialysis, with hemodialysis used most
often in both groups, and plasma infusion(s) were utilized in 6/38 and 4/9
patients, respectively. Of extra-renal complications, neurological complications
occurred in 9/38 and 2/9, serious gastrointestinal complications in 6/38 and 1/9,
respiratory complications in 10/38 and 2/9, and sepsis in 11/38 and 3/9 cases,
respectively. Cardiac complications were seen in two D(+)HUS cases. In patients
where data on follow up ?1 year after admittance were available, 8/21 and 4/7 had
persistent proteinuria and 5/19 and 4/5 had persistent hypertension in the
D(+)HUS and D(-)HUS group, respectively. Two D(+)HUS and one D(-)HUS patient were
diagnosed with chronic kidney disease and one D(+)HUS patient required a renal
transplantation. Two D(+)HUS patients died in the acute phase (death rate; 5 %).
CONCLUSIONS: The HUS cases had a high rate of complications and sequelae,
including renal, CNS-related, cardiac, respiratory, serious gastrointestinal
complications and sepsis, consistent with other studies. This underlines the
importance of attention to extra-renal manifestations in the acute phase and in
renal long-term follow-up of HUS patients.