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1.  Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment 
Emerging Infectious Diseases  2012;18(6):901-907.
The book on iatrogenic Creutzfeldt-Jakob disease (CJD) in humans is almost closed. This form of CJD transmission via medical misadventures was first detected in 1974. Today, only occasional CJD cases with exceptionally long incubation periods still appear. The main sources of the largest outbreaks were tissues from human cadavers with unsuspected CJD that were used for dura mater grafts and growth hormone extracts. A few additional cases resulted from neurosurgical instrument contamination, corneal grafts, gonadotrophic hormone, and secondary infections from blood transfusions. Although the final solution to the problem of iatrogenic CJD is still not available (a laboratory test to identify potential donors who harbor the infectious agent), certain other measures have worked well: applying special sterilization of penetrating surgical instruments, reducing the infectious potential of donor blood and tissue, and excluding donors known to have higher than normal risk for CJD.
PMCID: PMC3358170  PMID: 22607808
Creutzfeldt-Jakob disease; dura mater; human growth hormone; iatrogenic disease; PRNP codon 129; variant Creutzfeldt-Jakob disease; prions and related diseases
2.  Etymologia: Prion 
Emerging Infectious Diseases  2012;18(6):157-1031.
PMCID: PMC3358176  PMID: 22607731
prion; Etymologia; transmissible spongiform encephalopathies; viruses
3.  Survival of blood transfusion recipients identified by a look-back investigation 
Blood Transfusion  2014;12(1):67-72.
Survival of blood transfusion recipients is a critical consideration in assessing the outcomes of transfusion. Data from the USA on the short- and long-term survival of recipients are limited.
Materials and methods
Blood product recipients were identified through a look-back study of Creutzfeldt-Jakob disease. Survival data were obtained from searches of the National Death Index or the Social Security Death Master File. Short- and long-term survival of recipients was analysed through descriptive statistics, Kaplan-Meier survival analysis, and stratified Cox proportional hazard modelling.
This study includes data from 575 blood product recipients. One half of the recipients died within the first year of transfusion and the median time to death was 1.1 years. Survival rates at 5, 10, 15, 20, and 25 years after transfusion were 32%, 22%, 15%, 12%, and 9%, respectively. Survival rates varied with age at transfusion and type of component received, but not by gender. Survival after transfusion varied by year of transfusion, with recipients transfused in 1980–1989 having longer post-transfusion survival than those transfused in 2000–2010 (p=0.049). In multivariate models, the type of component transfused, but not the year of transfusion, was a significant predictor of survival among recipients; this effect varied by age.
We provide an estimate of survival time from a geographically diverse sample of blood product recipients in the USA. Predictors of post-transfusion survival are numerous and complex, and may include year of transfusion and type of component transfused.
PMCID: PMC3926731  PMID: 24333056
blood transfusion; post-transfusion survival; recipient survival; transfusion recipients; year of transfusion
4.  Evaluation of Potential Infectivity of Alzheimer and Parkinson Disease Proteins in Recipients of Cadaver-Derived Human Growth Hormone 
JAMA neurology  2013;70(4):462-468.
Growing evidence of cell-to-cell transmission of neurodegenerative disease (ND)–associated proteins (NDAPs) (ie, tau, Aβ, and α-synuclein) suggests possible similarities in the infectious prion protein (PrPsc) in spongiform encephalopathies. There are limited data on the potential human-to-human transmission of NDAPs associated with Alzheimer disease (AD) and other non-PrPsc ND.
To examine evidence for human-to-human transmission of AD, Parkinson disease (PD), and related NDAPs in cadaveric human growth hormone (c-hGH) recipients.
We conducted a detailed immunohistochemical analysis of pathological NDAPs other than PrPsc in human pituitary glands. We also searched for ND in recipients of pituitary-derived c-hGH by reviewing the National Hormone and Pituitary Program (NHPP) cohort database and medical literature.
University-based academic center and agencies of the US Department of Health and Human Services.
Thirty-four routine autopsy subjects (10 non-ND controls and 24 patients with ND) and a US cohort of c-hGH recipients in the NHPP.
Main Outcome Measures
Detectable NDAPs in human pituitary sections and death certificate reports of non-PrPsc ND in the NHPP database.
We found mild amounts of pathological tau, Aβ, and α-synuclein deposits in the adeno/neurohypophysis of patients with ND and control patients. No cases of AD or PD were identified, and 3 deaths attributed to amyotrophic lateral sclerosis (ALS) were found among US NHPP c-hGH recipients, including 2 of the 796 decedents in the originally confirmed NHPP c-hGH cohort database.
Conclusions and Relevance
Despite the likely frequent exposure of c-hGH recipients to NDAPs, and their markedly elevated risk of PrPsc-related disease, this population of NHPP c-hGH recipients does not appear to be at increased risk of AD or PD. We discovered 3 ALS cases of unclear significance among US c-hGH recipients despite the absence of pathological deposits of ALS-associated proteins (TDP-43, FUS, and ubiquilin) in human pituitary glands. In this unique in vivo model of human-to-human transmission, we found no evidence to support concerns that NDAPs underlying AD and PD transmit disease in humans despite evidence of their cell-to-cell transmission in model systems of these disorders. Further monitoring is required to confirm these conclusions.
PMCID: PMC3678373  PMID: 23380910
5.  Iatrogenic Creutzfeldt-Jakob Disease from Commercial Cadaveric Human Growth Hormone 
Emerging Infectious Diseases  2013;19(4):682-684.
PMCID: PMC3647424  PMID: 23750511
Creutzfeldt-Jakob disease; iatrogenic Creutzfeldt-Jakob disease; human growth hormone; case report; commercial; cadaveric; prions and related diseases
6.  Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries 
Emerging Infectious Diseases  2012;18(10):1574-1579.
Current diagnostic criteria should be sufficient to detect new cases of vCJD.
Variant Creutzfeldt-Jakob disease (vCJD) has been reported in 12 countries. We hypothesized that a common strain of agent is responsible for all vCJD cases, regardless of geographic origin. To test this hypothesis, we inoculated strain-typing panels of wild-type mice with brain material from human vCJD case-patients from France, the Netherlands, Italy, and the United States. Mice were assessed for clinical disease, neuropathologic changes, and glycoform profile; results were compared with those for 2 reference vCJD cases from the United Kingdom. Transmission to mice occurred from each sample tested, and data were similar between non-UK and UK cases, with the exception of the ranking of mean clinical incubation times of mouse lines. These findings support the hypothesis that a single strain of infectious agent is responsible for all vCJD infections. However, differences in incubation times require further subpassage in mice to establish any true differences in strain properties between cases.
PMCID: PMC3471642  PMID: 23017202
Prion; strain; variant Creutzfeldt-Jakob disease; transmissible spongiform encephalopathy; Creutzfeldt-Jakob disease; transmission properties; CJD; vCJD; TSE; BSE; prions; United States; the Netherlands; France; Italy; United Kingdom; USA; UK
7.  Disparities in Infectious Disease Hospitalizations for American Indian/Alaska Native People 
Public Health Reports  2011;126(4):508-521.
We described disparities in infectious disease (ID) hospitalizations for American Indian/Alaska Native (AI/AN) people.
We analyzed hospitalizations with an ID listed as the first discharge diagnosis in 1998–2006 for AI/AN people from the Indian Health Service National Patient Information Reporting System and compared them with records for the general U.S. population from the Nationwide Inpatient Survey.
The ID hospitalization rate for AI/AN people declined during the study period. The 2004–2006 mean annual age-adjusted ID hospitalization rate for AI/AN people (1,708 per 100,000 populiation) was slightly higher than that for the U.S. population (1,610 per 100,000 population). The rate for AI/AN people was highest in the Southwest (2,314 per 100,000 population), Alaska (2,063 per 100,000 population), and Northern Plains West (1,957 per 100,000 population) regions, and among infants (9,315 per 100,000 population). ID hospitalizations accounted for approximately 22% of all AI/AN hospitalizations. Lower-respiratory--tract infections accounted for the largest proportion of ID hospitalizations among AI/AN people (35%) followed by skin and soft tissue infections (19%), and infections of the kidney, urinary tract, and bladder (11%).
Although the ID hospitalization rate for AI/AN people has declined, it remains higher than that for the U.S. general population, and is highest in the Southwest, Northern Plains West, and Alaska regions. Lower-respiratory-tract infections; skin and soft tissue infections; and kidney, urinary tract, and bladder infections contributed most to these health disparities. Future prevention strategies should focus on high-risk regions and age groups, along with illnesses contributing to health disparities.
PMCID: PMC3115210  PMID: 21800745
8.  Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report 
BMC Neurology  2011;11:136.
Sporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare human prion diseases.
Case Presentation
We report a case of a 33-year-old female who died of a prion disease for whom the diagnosis of sFI or FFI was not considered clinically. Following death of this patient, an interview with a close family member indicated the patient's illness included a major change in her sleep pattern, corroborating the reported autopsy diagnosis of sFI. Genetic tests identified no prion protein (PrP) gene mutation, but neuropathological examination and molecular study showed protease-resistant PrP (PrPres) in several brain regions and severe atrophy of the anterior-ventral and medial-dorsal thalamic nuclei similar to that described in FFI.
In patients with suspected prion disease, a characteristic change in sleep pattern can be an important clinical clue for identifying sFI or FFI; polysomnography (PSG), genetic analysis, and nuclear imaging may aid in diagnosis.
PMCID: PMC3214133  PMID: 22040318
9.  Clinical Assessment of Self-Reported Acute Flaccid Paralysis in a Population-Based Setting in Guatemala 
Historically, poliovirus infection has been an important cause of acute flaccid paralysis (AFP) worldwide; however, successful elimination of wild-type poliovirus in much of the world has highlighted the importance of other causes of AFP. Despite the evolving etiology, AFP surveillance in most developing countries still focuses on poliovirus detection and fails to detect many AFP cases, particularly among adults. We assessed 41 subjects self-reporting symptoms suggestive of AFP during a population-based health survey in the Department of Santa Rosa, Guatemala. Thirty-five (85%) of the suspected cases were not hospitalized. Most subjects (37) did not have features consistent with AFP or had other diagnoses explaining weakness. We identified two adults who had not received medical attention for a clinical illness consistent with Guillain-Barré syndrome, the most important cause of non-poliovirus AFP. Usual surveillance methods for AFP, particularly in developing countries, may underestimate the true burden of non-poliovirus AFP.
PMCID: PMC2844551  PMID: 20348524
10.  Variably Protease-Sensitive Prionopathy: A New Sporadic Disease of the Prion Protein 
Annals of neurology  2010;68(2):162-172.
The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV).
Fifteen affected subjects with 129MM, 129MV, and 129VV underwent comparative evaluation at the National Prion Disease Pathology Surveillance Center for clinical, histopathologic, immunohistochemical, genotypical, and PrP characteristics.
Disease duration (between 22 and 45 months) was significantly different in the 129VV and 129MV subjects. Most other phenotypic features along with the PrP electrophoretic profile were similar but distinguishable in the 3 129 genotypes. A major difference laid in the sensitivity to protease digestion of the disease-associated PrP, which was high in 129VV but much lower, or altogether lacking, in 129MV and 129MM. This difference prompted the substitution of the original designation with “variably protease-sensitive prionopathy” (VPSPr). None of the subjects had mutations in the PrP gene coding region.
Because all 3 129 genotypes are involved, and are associated with distinguishable phenotypes, VPSPr becomes the second sporadic prion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920. However, the characteristics of the abnormal prion protein suggest that VPSPr is different from typical prion diseases, and perhaps more akin to subtypes of Gerstmann-Sträussler-Scheinker disease.
PMCID: PMC3032610  PMID: 20695009
11.  Racial/Ethnic Differences in the Incidence of Kawasaki Syndrome among Children in Hawai‘i 
Hawaii Medical Journal  2010;69(8):194-197.
To describe the occurrence of Kawasaki syndrome (KS) among different racial/ethnic groups in Hawai‘i.
Retrospective analysis of children <18 years of age, with a focus on children <5 years of age, living in Hawai‘i who were hospitalized with KS using the 1996–2006 Hawai‘i State Inpatient Data.
Children <5 years of age accounted for 84% of the 528 patients <18 years of age with KS. The average annual incidence among this age group was 50.4 per 100,000 children <5 years of age, ranging from 45.5 to 56.5. Asian and Pacific Islander children accounted for 92% of the children <5 years of age with KS during the study period; the average annual incidence was 62.9 per 100,000. Within this group, Japanese children had the highest incidence (210.5), followed by Native Hawaiian children (86.9), other Asian children (84.9), and Chinese children (83.2). The incidence for white children (13.7) was lower than for these racial/ethnic groups. The median age of KS admission for children <5 years of age was 21 months overall, 24 months for Japanese children, 14.5 months for Native Hawaiian children and 26.5 months for white children.
The high average annual KS incidence for children <5 years of age in Hawai‘i compared to the rest of the United States population reflects an increased KS incidence among Asian and Pacific Islander children, especially Japanese children. The incidence for white children was slightly higher than or similar to that generally reported nationwide.
PMCID: PMC3118023  PMID: 20845285
12.  Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States 
PLoS ONE  2010;5(1):e8765.
Variant Creutzfeldt–Jakob disease (vCJD) is a prion disease thought to be acquired by the consumption of prion-contaminated beef products. To date, over 200 cases have been identified around the world, but mainly in the United Kingdom. Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects.
Methodology/Principal Findings
Neuropathological and genetic examinations were carried out using standard procedures. We assessed the presence and characteristics of protease-resistant prion protein (PrPres) in brain and 23 other organs and tissues using immunoblots performed directly on total homogenate or following sodium phosphotungstate precipitation to increase PrPres detectability. The brain showed a lack of typical spongiform degeneration and had large plaques, likely stemming from the extensive neuronal loss caused by the long duration (32 months) of the disease. The PrPres found in the brain had the typical characteristics of the PrPres present in vCJD. In addition to the brain and other organs known to be prion positive in vCJD, such as the lymphoreticular system, pituitary and adrenal glands, and gastrointestinal tract, PrPres was also detected for the first time in the dura mater, liver, pancreas, kidney, ovary, uterus, and skin.
Our results indicate that the number of organs affected in vCJD is greater than previously realized and further underscore the risk of iatrogenic transmission in vCJD.
PMCID: PMC2808239  PMID: 20098730
13.  Human Prion Diseases in the United States 
PLoS ONE  2010;5(1):e8521.
Prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form of human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encephalopathy.
This study describes the occurrence and epidemiology of CJD and vCJD in the United States.
Methodology/Principal Findings
Analysis of CJD and vCJD deaths using death certificates of US residents for 1979–2006, and those identified through other surveillance mechanisms during 1996–2008. Since CJD is invariably fatal and illness duration is usually less than one year, the CJD incidence is estimated as the death rate. During 1979 through 2006, an estimated 6,917 deaths with CJD as a cause of death were reported in the United States, an annual average of approximately 247 deaths (range 172–304 deaths). The average annual age-adjusted incidence for CJD was 0.97 per 1,000,000 persons. Most (61.8%) of the CJD deaths occurred among persons ≥65 years of age for an average annual incidence of 4.8 per 1,000,000 persons in this population. Most deaths were among whites (94.6%); the age-adjusted incidence for whites was 2.7 times higher than that for blacks (1.04 and 0.40, respectively). Three patients who died since 2004 were reported with vCJD; epidemiologic evidence indicated that their infection was acquired outside of the United States.
Surveillance continues to show an annual CJD incidence rate of about 1 case per 1,000,000 persons and marked differences in CJD rates by age and race in the United States. Ongoing surveillance remains important for monitoring the stability of the CJD incidence rates, and detecting occurrences of vCJD and possibly other novel prion diseases in the United States.
PMCID: PMC2797136  PMID: 20049325
14.  A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease 
Annals of neurology  2008;63(6):697-708.
To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) that, contrary to the common prion diseases, is predominantly sensitive to protease digestion.
Eleven subjects were investigated at the National Prion Disease Pathology Surveillance Center (NPDPSC) for clinical, histopathological, immunohistochemical, genotypical and PrP characteristics.
Patients presented with behavioral and psychiatric manifestations on average at 62 years while mean disease duration was 20 months. The type of spongiform degeneration, the PrP immunostaining pattern and the presence of micro-plaques distinguished these cases from those with known prion diseases. Typical protease-resistant PrP was undetectable in the cerebral neocortex with standard diagnostic procedures. Following enrichment, abnormal PrP was detected at concentrations 16 times lower than common prion diseases; it included nearly four times less protease-resistant PrP, which formed a distinct electrophoretic profile. The subjects examined comprised about 3% of sporadic cases evaluated by the NPDPSC. Although several subjects had family history of dementia, no mutations were found in the PrP gene open reading frame (ORF).
The distinct histopathological, PrP immunohistochemical and physical-chemical features along with the homogeneous genotype indicate that this is a previously unidentified type of disease involving the prion protein, which we designated “protease-sensitive prionopathy or PSPr”. PSPr is not very rare among prion diseases, and might be even more prevalent than our data indicate since PSPr cases are likely to be also classified within the group of non-Alzheimer dementias.
PMCID: PMC2767200  PMID: 18571782
15.  Infectious Disease Hospitalizations Among Older American Indian and Alaska Native Adults 
Public Health Reports  2006;121(6):674-683.
American Indians and Alaska Natives (AI/AN) adults ≥65 years of age (older adults) have the second highest age group-specific infectious disease (ID) hospitalization rate. To assess morbidity and disparities of IDs for older AI/AN adults, this study examined the epidemiology of overall and specific infectious disease hospitalizations among older AI/AN adults.
ID hospitalization data for older AI/AN adults were analyzed by using Indian Health Service hospital discharge data for 1990 through 2002 and comparing it with published findings for the general U.S. population of older adults.
ID hospitalizations accounted for 23% of all hospitalizations among older AI/AN adults. The average annual ID hospitalization rate increased 5% for 1990–1992 to 2000–2002; however, the rate increased more than 20% in the Alaska and the Southwest regions. The rate for older AI/AN adults living in the Southwest region was greater than that for the older U.S. adult population. For 2000–2002, lower respiratory tract infections accounted for almost half of all ID hospitalizations followed by kidney, urinary tract, and bladder infections, and cellulitis.
The ID hospitalization rate increased among older AI/AN adults living in the Southwest and Alaska regions, and the rate for the older AI/AN adults living in the Southwest region was higher than that for the U.S. general population. Prevention measures should focus on ways to reduce ID hospitalizations among older AI/AN adults, particularly those living in the Southwest and Alaska regions.
PMCID: PMC1781909  PMID: 17278402
16.  Variant Creutzfeldt-Jakob Disease Death, United States 
Emerging Infectious Diseases  2005;11(9):1351-1354.
Reports of secondary bloodborne transmission of vCJD add to the uncertainty about the future of the vCJD outbreak.
The only variant Creutzfeldt-Jakob disease (vCJD) patient identified in the United States died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. The patient likely acquired the disease while growing up in Great Britain before immigrating to the United States in 1992. Additional vCJD patients continue to be identified outside the United Kingdom, including 2 more patients in Ireland, and 1 patient each in Japan, Portugal, Saudi Arabia, Spain, and the Netherlands. The reports of bloodborne transmission of vCJD in 2 patients, 1 of whom was heterozygous for methionine and valine at polymorphic codon 129, add to the uncertainty about the future of the vCJD outbreak.
PMCID: PMC3310634  PMID: 16229761
Creutzfeldt-Jakob disease; variant Creutzfeldt-Jakob disease; prion disease; transmissible spongiform encephalopathy; epidemiology; surveillance; public health; research
17.  Barriers to Creutzfeldt-Jakob Disease Autopsies, California 
Emerging Infectious Diseases  2004;10(9):1677-1680.
Creutzfeldt-Jakob disease (CJD) surveillance relies on autopsy and neuropathologic evaluation. The 1990–2000 CJD autopsy rate in California was 21%. Most neurologists were comfortable diagnosing CJD (83%), but few pathologists felt comfortable diagnosing CJD (35%) or performing autopsy (29%). Addressing obstacles to autopsy is necessary to improve CJD surveillance.
PMCID: PMC3320312  PMID: 15498177
Creutzfeldt-Jakob Disease; CJD; variant; transmissible spongiform encephalopathy; TSE; prion; surveillance; autopsy; dispatch
18.  Chronic Wasting Disease and Potential Transmission to Humans 
Emerging Infectious Diseases  2004;10(6):977-984.
Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.
PMCID: PMC3323184  PMID: 15207045
Chronic wasting disease; prion diseases; transmissible spongiform encephalopathy; interspecies transmission; prions
19.  Kawasaki syndrome hospitalizations and associated costs in the United States. 
Public Health Reports  2003;118(5):464-469.
OBJECTIVES: To describe the epidemiologic characteristics of patients hospitalized with Kawasaki syndrome (KS) and estimate associated costs in the United States, using a large national hospital discharge dataset. METHODS: Hospitalization discharge records with KS for 1997 through 1999 for U.S. residents <18 years of age were selected from Solucient's hospital discharge records. These records are collected from most of the self-governing children's hospitals and approximately one-third of short-term, non-federal general hospitals in the United States. RESULTS: A total of 7,431 hospital discharges with a KS diagnosis were identified; 2,270 of the discharges were in 1997, 2,700 in 1998, and 2,461 in 1999. Boys comprised 60.0% of the discharges, and 76.4% of discharges were among children ages <5 years. For the 44 states and the District of Columbia with at least one hospital reporting KS, the average annual KS hospitalization rate was 10.2 per 100,000 children ages <5 years. The KS hospitalization rate for boys (12.0 per 100,000) was higher than that for girls (8.3 per 100,000) (risk ratio 1.45; 95% confidence interval 1.37, 1.52). Extrapolation to the U.S. population showed an estimated average annual KS hospitalization rate of 21.6. The median KS hospitalization cost for children <5 years of age during the study period was $6,169 US dollars. CONCLUSIONS: The KS hospitalization rate was consistent with that of previous U.S. studies, although the extrapolated rate may be an overestimation. The median hospitalization cost for KS was higher than that for respiratory syncytial virus-associated bronchiolitis and diarrheal diseases. Large hospitalization datasets can be used to monitor the occurrence of KS in the United States.
PMCID: PMC1497579  PMID: 12941859
20.  Hemolytic-Uremic Syndrome—An Outbreak in Sacramento, California 
Western Journal of Medicine  1986;144(2):169-173.
Between July and November 1982, 14 cases of the hemolytic-uremic syndrome occurred in the Sacramento, California, metropolitan area; 9 of the 14 patients lived within a 7.5-mile radius in northeast Sacramento, 10 were female, 12 were white non-Hispanic and 13 were children with a mean age of 3.6 years. Of the 14 patients, 13 were admitted to hospital; 7 required peritoneal dialysis. The 14th child, a 3-month-old white female infant, was found dead in her crib and had renal histopathologic findings consistent with the hemolytic-uremic syndrome. Of the 13 nonfatal cases, 12 patients had diarrhea before being admitted to hospital. A case-control study involving 11 cases and 22 controls did not show any significant differences in exposure to a variety of possible risk factors including restaurants, specific foods and water supply. Stool specimens were negative for enteric bacterial pathogens by culture and for viruses by tissue culture assay, suckling mouse inoculation and immune electron microscopy; no serologic evidence was found for infection due to enteroviruses, respiratory viruses or arenaviruses. Two of four children tested, however, showed serologic evidence of infection by Vero-cytotoxin-producing Escherichia coli. These 14 cases represent one of the largest reported outbreaks of the hemolytic-uremic syndrome in the United States.
PMCID: PMC1306553  PMID: 3953085

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