• Childhood Kidney Diseases
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• v.1 no.2
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• pp.170-175
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• 1997

Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.149-153
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• 2013

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

• Journal of Microbiology and Biotechnology
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• v.26 no.2
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• pp.432-439
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• 2016

Shiga toxins (Stxs) produced by Shiga toxin-producing Escherichia coli (STEC) strains are major virulence factors that cause fatal systemic complications, such as hemolytic uremic syndrome and disruption of the central nervous system. Although numerous studies report proinflammatory responses to Stx type 1 (Stx1) or Stx type 2 (Stx2) both in vivo and in vitro, none have examined dynamic immune regulation involving cytokines and/or unknown inflammatory mediators during intoxication. Here, we showed that enzymatically active Stxs trigger the dissociation of lysyl-tRNA synthetase (KRS) from the multi-aminoacyl-tRNA synthetase complex in human macrophage-like differentiated THP-1 cells and its subsequent secretion. The secreted KRS acted to increase the production of proinflammatory cytokines and chemokines. Thus, KRS may be one of the key factors that mediate transduction of inflammatory signals in the STEC-infected host.

• Journal of Preventive Medicine and Public Health
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• v.36 no.1
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• pp.77-84
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• 2003

Objectives : Two related cases of Hemolytic-Uremic Syndrome (HUS) were reported to the Korea National Institute of Health in May, 2001. Shiga toxin 2 genes were detected in both stool samples. We suspected an enterohemorrhagic Escherichia coli (EHEC) infection as the cause of the HUS, and conducted an investigation to find the source of the infection and its route of transmission. Methods : We peformed case investigations on these two related HUS cases, and obtained interviews and rectal swabs form the family members and other close contacts. Additionally, we peformed rectal swabs on the cattle raised by the household of the index patient. Results : We found a 20 month old index patient and a 6 year-old cousin had developed HUS, where there had been a 2 day history of contact with the index, and bacteriological examinations for these two patients revealed, indistinguishably, the same E. coli O171. The grandmother of the index patient was found to be asymptomatic, but E. coli O26 was isolated. We also found a probable case in the mother of the cousin. She reported a history of contact with the index, and developed bloody diarrhea of 3 days duration. The test results for the cattle revealed E. coli O26 in one cow, and E. coli O26 and O55 in another. E. coli O26, which was isolated in both cows and the grandmother of the index, were indistinguishably the same. Conclusions : We found that the E. coli O26 in the grandmother had originated from the cows, and that the E. coli O171 found in the index patient had been transmitted to the cousin through person-to-person contact.

• Journal of the Korean Society of Food Science and Nutrition
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• v.33 no.3
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• pp.571-575
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• 2004

Escherichia coli O157:H7 cause hemorrhagic colitis and the extraintestinal complication of hemolytic-uremic syndrome, with their higher incidence occurring in children. Lipopolysaccharide (LPS) of E. coli O157:H7 is very important to make IgG anti-LPS with bactericidal activity. To identify the characteristic of E. coli OI57:H7, we isolated 60 MDa plasmid and amplified stx genes of shiga-like toxin (Stx) 1, 2 of E. coli O157:H7 by polymerase chain reaction (PCR) method. Using the simple purification method which contained phenol extract, ethanol precipitation and gel filtration steps, the LPS of E. coli O157:H7 was isolated and purified. Finally, we confirmed the purity of LPS through SDS-PAGE and silver nitrate staining.

• Korean Journal of Food Science and Technology
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• v.50 no.4
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• pp.414-419
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• 2018

Escherichia coli O157:H7 is one of the most common foodborne pathogens responsible for outbreaks of hemorrhagic colitis, which can lead to the life-threatening hemolytic-uremic syndrome. In this study, we identified phytochemicals that specifically inhibit the expression of LEE operon in E. coli O157:H7. Among phytochemicals, diosmin decreased the adherence of E. coli O157:H7 towards Caco-2 cells in vitro (p<0.01) and its biofilm formation activity (p<0.05). Quantitative RT-PCR analysis revealed that the transcripts of Ler-regulated genes and genes related to curli production were significantly reduced in the presence of diosmin. However, diosmin does not affect bacterial viability, indicating that the resistance rate to diosmin was remarkably low. Overall, these results provide significant insights into the development of a novel anti-infective agent that is different from conventional antibiotics.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.1-5
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• 2013

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.

• Journal of Dairy Science and Biotechnology
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• v.33 no.1
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• pp.59-67
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• 2015

Enterohemorrhagic Escherichia coli (EHEC) O157:H7 is well-characterized as an important food-borne pathogen worldwide and causes human diseases such as diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS) by producing shiga-like toxin (Stx). It has been reported that a number of dairy foods, including cheese, can act as the source of EHEC O157:H7 infections. In addition to the toxicity of Stx, recently it has been indicated that EHEC O157:H7 possesses virulence factors related to attachment, quorum sensing, and biofilms. Moreover, these novel virulence factors might become critical points to be considered in the future production of food derived from animals. Here, we review the evidences that support these insights on new virulence factors and discuss the potential mechanisms mediating the pathogenesis of EHEC O157:H7 in the dairy food industry.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Korean Journal of Veterinary Service
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• v.39 no.2
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• pp.87-92
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• 2016

Porcine edema disease (ED) is a communicable disease of pigs caused by infection with Shiga toxin (Stx)-producing Escherichia coli (STEC) which expresses F18 fimbriae and/or Stx type 2e (Stx2e). While STEC causes a severe illness including hemorrhagic colitis and hemolytic-uremic syndrome in humans, it induces damage to the vascular endothelium, which results in edema, hemorrhage, and microthrombosis, leading in high mortality in pigs. In the present study, we cultured Stx2e-producing E. coli field isolates from conventional pig farms that experienced sudden deaths previously with symptoms similar to porcine edema disease, which were further investigated with Shiga toxin profiles. A total of 43 strains were identified from the collected samples by F18 or Stx2e specific PCR. Based on the PCR, 42 isolates out of 43 isolates were proved to carry one of F18 or Stx2e genes and 14 isolates to carry both F18 and Stx2e genes. All of the 30 isolates that harbored Stx2e gene induced the cytopathic effect (CPE) in vero cells and especially, the isolate 150229 produced the highest level of Shiga toxin. Therefore, we identified the virulence genes (F18 and Stx2e) and demonstrated Shiga toxin-producing abilities from porcine edema disease causing E. coli filed isolates. These results suggested that one of the isolates could be a vaccine antigen candidate against STEC through further investigating to elicit an immune response.