CDC has received reports from 13 pediatric centers in New York, Massachusetts, Virginia, and the District of Columbia of 32 cases of nephrologist-diagnosed hemolytic-uremic syndrome (HUS), with onsets between June 22, and October 31, 1983. Onset of illness occurred in June (2 cases), July (6), August (4), September (13), and October (7).

The 32 children ranged in age from 11 months to 12 years (mean 3.3 years); all were white, and 56% were female. Twenty-two (69%) required dialysis. Two patients currently are on chronic dialysis and have significant neurologic sequelae 2 months after initial hospitalization.

New York: Twenty-one cases have been reported; onsets occurred between June 22 and October 25 throughout upstate and Long Island, New York. Six cases were reported from Long Island. Syracuse and Albany had four cases each, referred in an 8-week period; three of the four had prodromes consisting of bloody diarrhea.

Massachusetts: Five HUS patients were reported, with onsets between September 1 and September 15. All had bloody diarrhea prodromes. Two of the patients lived in Boston.

Virginia: Four cases have been reported; onsets occurred between September 14 and October 31, with prodromes consisting of nonbloody diarrhea in three and bloody diarrhea in one.

District of Columbia: Two cases occurred, one each on September 18 and September 27; both patients had vomiting and bloody diarrhea prodromes. Reported by R Spitzer, MD, Upstate Medical Center, Syracuse, MR Kaplan, MD, New York Hospital, Cornell Medical Center, New York City, F Kaskel, MD, Stoneybrook, Long Island, B Gauthier, MD, Long Island Jewish Hospital, New Hyde Park, L Feld, MD, Children's Hospital of Buffalo, J Largent, MD, Albany Medical Center, H Cohen, MD, Strong Memorial Hospital, Rochester, R Rothenberg, MD, State Epidemiologist, New York State Dept of Health; W Harris, MD, Boston Children's Hospital, B Strechenberg, MD, Bay State Medical Center, Springfield, NJ Fiumara, MD, State Epidemiologist, Massachusetts State Dept of Health; RL Chevalier, MD, University of Virginia, Charlottesville, RS Buddington, MD, Johnston Memorial Hospital, Abingdon, GB Miller, MD, State Epidemiologist, Virginia State Dept of Health; GH Bock, MD, CC Porter, MD, EJ Ruley, MD, Children's Hospital National Medical Center, ME Levy, MD, State Epidemiologist, Dept of Human Svcs, Washington, DC; Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: First described in 1955, HUS is defined by the classic triad of microangiopathic hemolytic anemia, acute nephropathy, and thrombocytopenia (1). HUS is usually preceded by a prodromal gastrointestinal illness, or less commonly, an upper respiratory illness. The gastrointestinal illness consists of vomiting, bloody and/or nonbloody diarrhea, and abdominal cramps. Renal failure is common, often requiring dialysis; death has been reported in approximately 6%-10% of children (2,3).

HUS occurs primarily among white infants and children less than 5 years of age, with equal distribution among males and females. The disease has been reported with greatest frequency from California, Argentina, the Netherlands, and South Africa. Clusters of between nine and 14 cases have been reported from Sacramento, California (4), Canada (5,6), Wales (7), Bangladesh, and Central America (8). In one outbreak in Toronto, Canada, 13 children developed HUS following ingestion of fresh apple juice at a local fair. No common organism or toxin was isolated from children or juice (5).

Although the cause of HUS is unknown, both viral and bacterial pathogens have been associated with the illness. Enteroviruses, including Coxsackie A and B and echoviruses, have been reported, and several investigators have noted a summer-fall seasonality (7,9,10). Recently, Vero-toxin producing Escherichia coli were associated with 11 of 15 children with sporadic cases of HUS (11). E. coli 0157: H7, a rare serotype associated with hemorrhagic colitis (12), was isolated in two of these 11 cases, as well as in three others from the United Kingdom (13). Other bacterial pathogens isolated from patients with HUS include Shigella (8), Campylobacter (14), and Yersinia (15).

When investigating cases of HUS, stool specimens for viral and bacterial culture should be obtained as early as possible--preferably within 7 days of onset of the diarrheal illness. Specimens that will not be processed immediately should be stored at -70 C (-94 F).

The Division of Viral Diseases, Center for Infectious Diseases, CDC, is interested in obtaining reports of new and recent cases of HUS in children.

References

1. Kaplan BS, Thomson PD, de Chadarevian JP. The hemolytic uremic syndrome. Pediatr Clin of North Am. 1976;23:761-77.

2. Dolislager D, Tune B. The hemolytic-uremic syndrome: spectrum of severity and significance of prodrome. Am J Dis Child 1978;132:55-8.

3. Sorrenti LY, Lewy PR. The hemolytic-uremic syndrome: experience at a center in the Midwest. Am J Dis Child 1978;132:59-62.

4. CDC. Unpublished data.

5. Steele BT, Murphy N, Arbus GS, Rance CP. An outbreak of hemolytic uremic syndrome associated with ingestion of fresh apple juice. J Pediatr 1982;101:963-5.

6. O'Regan S, Robitaille P, Mongeau JG, McLaughlin B. The hemolytic-uremic syndrome associated with ECHO 22 infection. Clin Pediatr 1980;19:125-7.

7. McLean MM, Jones CH, Sutherland DA. Haemolytic-uraemic syndrome: a report of an outbreak. Arch Dis Child 1966;41:76-81.

8. Koster F, Levin J, Walker L, et al. Hemolytic-uremic syndrome after Shigellosis: relation to endotoxemia and circulating immune complexes. N Engl J Med 1978;298:927-33.

9. Ray CG, Tucker VL, Harris DJ, Cuppage FE, Chin DY. Enteroviruses associated with the hemolytic-uremic syndrome. Pediatrics 1970;46:378-88.

10. Austin TW, Ray CG. Coxsackie virus group B infections and the hemolytic-uremic syndrome. J Infect Dis 1973;127:698-701.

11. Karmali MA, Petric M, Steele BT, Lim C. Sporadic cases of haemolytic-uraemic syndrome associated with faecal cytotoxin and cytotoxin-producing Escherichia coli in stools. Lancet 1983;1:619-20.

12. Riley LW, Remis RS, Helgerson SD, et al. Hemorrhagic colitis associated with a rare Escherichia coli serotype. N Eng J Med 1983;308:681-5.

13. Hemolytic-uremic syndrome. Communicable disease report, weekly edition, CDR 83/36, Sept 9, 1983.

14. Chamovitz BN, Hartstein AI, Alexander SR, Terry AB, Short P, Katon R. Campylobacter jejuni-associated hemolytic-uremic syndrome in a mother and daughter. Pediatr 1983;71:253-6.

15. Prober CG, Tune B, Hoder L. Yersinia pseudotuberculosis septicemia. Am J Dis Child 1979;133:623-4.

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