Successful treatment of non-familial haemophagocytic lymphohistiocytosis with interferon and gammaglobulin.

Two cases of non-familial haemophagocytic lymphohistiocytosis (HLH) are presented in which treatment with interferon alfa and gammaglobulin was associated with complete clinical remission. In one case, serological evidence of recent Epstein-Barr virus infection was found. Natural killer cell activity was within normal limits in both children, compatible with a secondary form of HLH. The combination of interferon alfa and intravenous gammaglobulin requires further evaluation in the treatment of non-familial HLH.

Children with seizures presenting to accident and emergency.

OBJECTIVE: To analyse the diagnoses made in children presenting to an accident and emergency (A&E) department with seizures. METHODS: All children who presented to a district general hospital A&E department with seizures over a 1 year period were identified. A retrospective review of A&E and hospital records was performed one year after they were first seen. The types of seizure, investigations performed, treatment given, and the range of associated diagnoses were ascertained. RESULTS: 199 children (of 21,795 attending A&E in the year) had 254 visits with seizures, which represented 1.2% of the child attendances at A&E. Self referral occurred in 87%, and 52 patients had established epilepsy. Febrile seizures were far the commonest type of first seizure (n = 75), but there were also 13 cases of symptomatic seizures resulting from various metabolic and neurological causes. CONCLUSIONS: The children studied had a very different spectrum of problems from adults. A&E staff should be aware of the range of problems in children who present with seizures. Experienced paediatric staff should be available to assist all A&E departments where children are seen.

Copper associated childhood cirrhosis.

Several papers have reported severe liver disease in association with massive hepatic copper accumulation, which do not seem to be either of the recognised copper associated liver diseases, namely Wilson's disease and Indian childhood cirrhosis. A further case is reported in which novel copper kinetic studies were carried out using the stable isotope 65Cu, showing that this patient did not suffer from Wilson's disease. It is suggested that these cases can be divided into two groups on the basis of age, clinical course, and history of excessive copper ingestion. The benefits of using 65Cu for in vivo studies of copper metabolism is discussed.

Neonatal death at the University Hospital of the West Indies.

PIP: Neonatal deaths were investigated at the University Hospital of the West Indies (UHWI) over a 4-year period (1974-77). Principal pathological diagnosis at autopsy was compared with clinical diagnosis according to the following criteria: intrapartum hypoxia, hypoxia, respiratory distress syndrome (RDS), infection and congenital malformation. Babies were classified according to the place of birth: "inborn" (born in UHWI) and "outborn" (born elsewhere). Neonatal mortality, mortality by weight, proportion of babies born at low birth weight, clinico-pathological agreement in principal cause of neonatal deaths, and comparison of first week neonatal deaths between 3 countries (Canada, United Kingdom and UHWI) were detailed in Tables 1-5. 49.6% of neonatal deaths occurred on the first day and 83.5% at the end of the first week; this confirmed the study of Lowry, Hall and Sparke (1976) which showed that 58% of neonatal deaths happen on the 1st extra-uterine day, and 89% within the 1st week. Neonatal mortality in the UHWI was attributed in part to the deficient neonatal care (limited nursing staff, equipment maintenance and supplies). For the period '74-'77, the neonatal mortality rate was 16.87/1000 live births; low-birthweight babies significantly accounted for this mortality. Common causes of death were congenital malformation, infection, RDS, intrapartum hypoxia and hypoxia in very immature babies. In Table 5, UHWI had the highest rate of infection which contributed to neonatal deaths. Prevention of neonatal infection, along with a general improvement in neonatal care would reduce neonatal mortality in UHWI.^ieng