POSSIBLE ETIOLOGY OF HEMORRHAGIC-SHOCK AND ENCEPHALOPATHY SYNDROME INTHE NEGEV AREA OF ISRAEL

A retrospective study was performed for all patients diagnosed with ha emorrhagic shock and encephalopathy syndrome (HSES) over an 11 year pe riod (1984-94). Soroka University Medical Centre is the only medical f acility in the southern Negev region of Israel serving a population of about 400 000 residents, consisting primarily of two ethnic populatio ns, Jews and Bedouins. Twenty patients, 17 Bedouin and three Jews, wer e diagnosed with HSES. The annual incidence of HSES for infants under the age of 1 year was 5:10 000 for Bedouins and 0.6:10 000 for Jews. P atients ranged in age from 6 to 32 weeks and arrived at the hospital. late at night or early morning (2.00 am to 11.00 am), during the winte r or early spring (November to April). All were healthy before admissi on, with short prodromal symptoms of upper respiratory tract or gastro intestinal infection noted in 10 cases. Most infants had markedly high body temperature on arrival. A history of overwrapping and/or excessi ve heating was obtained in four of 20 infants. Bacteriological and vir ological cultures were negative in all infants. One infant died and ne urological sequelae were observed in all survivors. The high prevalenc e of hyperpyrexia during sleep in the presence of negative microbiolog ical results with no evidence of excessive heating, and the high incid ence of HSES among a dosed and culturally isolated society known to ha ve a high incidence of congenital malformations, may support previous assumptions that HSES results from hyperpyrexia, originating in most c ases from a 'physiological' heat induced trigger, which starts and pea ks during the night in previously healthy infants who are genetically susceptible.