Incidence and index of severity of hemolytic uremic syndrome in a 26 year period in Fukushima Prefecture, Japan.

BACKGROUND: There have been a number of reports on large outbreaks of hemolytic uremic syndrome (HUS), but there have been no long-term studies of sporadic HUS in Japan. This study therefore investigated the epidemiology and prognosis of HUS in Fukushima Prefecture over a 26 year period. METHODS: The medical records of 26 patients with HUS between 1987 and 2012 were collected. These children were divided into two groups: those with HUS following an episode of gastroenteritis, often with bloody diarrhea (D + HUS; n = 24) and those with HUS not associated with prodromal diarrhea (D-HUS; n = 2). The D + HUS group was further subdivided into group A (11 patients requiring dialysis) and group B (13 patients not requiring dialysis). The epidemiological and clinical data, as well as prognosis, were retrospectively investigated for each group. RESULTS: Approximately 90% of HUS patients belonged to the D + HUS group. In this group, the mean number of patients per year from 1987 to 1999, and from 2000 to 2012 was 0.92 ± 0.95, and 1.08 ± 0.86, respectively. On admission, lactate dehydrogenase (LDH), alanine aminotransferase (ALT), blood urea nitrogen (BUN), serum creatinine and serum fibrinogen degradation product (FDP) levels in group A were all higher than in group B. Serum albumin level and estimated glomerular filtration rate (eGFR) in group A were lower than in group B. At 6 months after the onset of HUS in the D + HUS group, renal function was normal. CONCLUSIONS: The frequency of HUS was constant from 1987 to 2012 in Fukushima. and serum LDH, ALT, BUN, creatinine, and FDP levels as well as eGFR might be risk factors for dialysis in D + HUS children.

The incidence and severity of Henoch-Schönlein purpura nephritis over a 22-year period in Fukushima Prefecture, Japan.

BACKGROUND: Recently, there have been a few reports on the decrease in the incidence of severe Henoch-Schönlein purpura nephritis (HSPN) in Japan. To evaluate the incidence and the severity of HSPN, we examined the epidemiology and clinical manifestations of HSPN in Fukushima Prefecture over a 22-year period. METHODS: We enrolled 61 patients (Group 1) diagnosed with HSPN between 1987 and 1997 and a further 59 patients (Group 2) diagnosed with HSPN between 1998 and 2008. Epidemiology, clinical features, laboratory data, pathological findings, treatment and outcome were retrospectively compared between the two groups. RESULTS: The mean number of patients per 100,000 children per year was 3.5 ± 1.2 in Group 1 and 3.6 ± 0.8 in Group 2. The clinical manifestations and laboratory findings at onset were comparable between Groups 1 and 2. Pathological findings in Group 1 and Group 2 were classified as ISKDC grade IIIa in 16 (26%) and 14 cases (24%), grade IIIb in 26 (43%) and 27 cases (46%), grade IV in 5 (8%) and 6 cases (10%)), grade V in 1 (2%) and 5 cases (8%), and grade VI in 2 (3%) and 2 cases (3%), respectively. There were no inter-group differences in ISKDC classification or rate of crescentic formation. CONCLUSIONS: Our findings suggest that the incidence of HSPN and the severity of HSPN in patients between 1987 and 1997 were similar to those in patients between 1998 and 2008 and that the number of patients with severe HSPN has not decreased.