Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999-2008.
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.



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.



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 %).



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.
Department of Infectious Disease Epidemiology, Norwegian Institute of Public Health (Nasjonalt Folkehelseinstitutt), Postboks 4404, Nydalen, NO 0403, Oslo, Norway. gautereier@gmail.com.|Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway. gautereier@gmail.com.
Gaute Reier
GR
Jenssen
Department of Infectious Disease Epidemiology, Norwegian Institute of Public Health (Nasjonalt Folkehelseinstitutt), Postboks 4404, Nydalen, NO 0403, Oslo, Norway.
Line
L
Vold
Department of Infectious Disease Epidemiology, Norwegian Institute of Public Health (Nasjonalt Folkehelseinstitutt), Postboks 4404, Nydalen, NO 0403, Oslo, Norway.|Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
Eirik
E
Hovland
Department of Pediatrics, Oslo University Hospital, Oslo, Norway.
Hans-Jacob
HJ
Bangstad
Department of Infectious Disease Epidemiology, Norwegian Institute of Public Health (Nasjonalt Folkehelseinstitutt), Postboks 4404, Nydalen, NO 0403, Oslo, Norway.
Karin
K
Nygård
Department of Pediatrics, Oslo University Hospital, Oslo, Norway.
Anna
A
Bjerre
D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D003967:Diarrhea / Q000209:etiology; D004927:Escherichia coli Infections / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006331:Heart Diseases / Q000209:etiology; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007223:Infant; D007668:Kidney; D007676:Kidney Failure, Chronic / Q000209:etiology; D016030:Kidney Transplantation; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D009664:Norway; D010956:Plasmapheresis; D011507:Proteinuria / Q000209:etiology; D006435:Renal Dialysis; D051436:Renal Insufficiency, Chronic / Q000209:etiology; D017582:Renal Replacement Therapy; D012121:Respiration, Artificial; D012140:Respiratory Tract Diseases / Q000209:etiology; D012189:Retrospective Studies; D018805:Sepsis / Q000209:etiology; D054323:Shiga-Toxigenic Escherichia coli
D000900:Anti-Bacterial Agents
Enterohaemorrhagic E. coli - EHEC; Epidemiology; Haemolytic uraemic syndrome; SP-HUS; Shiga toxin producing E. coli – STEC; aHUS; clinical outcome