Safety,tolerability, and effectiveness of oral zonisamide therapy in comparison with intramuscular adrenocorticotropic hormone therapy in infants with West syndrome

West syndrome is a distinct, infantile onset, epileptic encephalopathy, associated with poor neurodevelopmental outcome. The present study was designed as a randomized, open-label, pilot study to evaluate the safety, feasibility, and effectiveness of oral zonisamide therapy in comparison with adrenocorticotropic hormone therapy in infants with West syndrome. Thirty infants with West syndrome were randomized to receive treatment with either synthetic, intramuscular adrenocorticotropic hormone (30–60 IU) or oral zonisamide (4–25 mg/kg/day). The study participants had a long treatment lag and preponderance of male sex (90%). The primary effectiveness outcome measure was the cessation of epileptic spasms at 2 weeks of initiation of therapy and persistent till 6 weeks as per West Delphi consensus statement recommendations. Comparison of efficacies of zonisamide versus adrenocorticotropic hormone was as following: the cessation of epileptic spasms (27% vs. 40%, p = 0.70), resolution of hypsarrhythmia at 14 days (20% vs. 33%, p = 0.68) and resolution of hypsarrhythmia at 6 weeks (36% vs. 71%, p = 0.14). Overall, the study observed a poor efficacy of both adrenocorticotropic hormone and zonisamide therapy, which is probably due to long treatment lag and a high proportion of structural aetiology. However, oral zonisamide appeared to be safe and tolerable in the study.     

Alterations in the concentration of an apolipoprotein E-containing subfraction of plasma high density lipoprotein in coronary heart disease

The concentrations of high density lipoprotein (HDL) subfractions in 100 healthy male subjects were compared with 100 newly presenting patients with myocardial infarction (MI) within 12 h of the onset of chest pain. A subfraction of HDL enriched in apolipoprotein E (apo E), separated by heparin-Sepharose affinity chromotography, was present in lower concentrations (P < 0.001) in the plasma of the coronary patients than in the control subjects. This finding was confirmed by a lower content (P < 0.02) of apo E, measured by ELISA, in the total HDL fraction isolated from the coronary patients. Gradient gel electrophoresis of the total HDL demonstrated that the coronary patients had a significantly decreased concentration of the large HDL particles, HDL_2b, of mean diameter 10.57 nm and a higher concentration of the smaller-sized HDL₃, especially HDL_3c, of mean diameter 7.62 nm. The coronary patients had a lower concentration of HDL cholesterol than the control subjects, attributable to the HDL₂ fraction, with no difference in HDL_2a between the two groups. There was no difference in the concentration of plasma cholesterol or triglyceride. The distribution of apo E phenotypes was similar in the two groups. HDL_2b produced the highest discriminant power between the two groups, followed by apo E-rich HDL, HDL₂ and HDL_3c Plasma cholesterol correlated strongly with apo E-rich HDL for control subjects but not for MI survivors. This study demonstrates that the inverse relationship between HDL cholesterol and coronary risk shown in epidemiological studies is attributable to the large, apo E-containing HDL subspecies which under some circumstances are implicated in cholesterol removal by reverse cholesterol transport. This study also suggests that the concentration of the large, ago E-containing HDL may provide a sensitive predictor for subjects at risk of developing coronary heart disease.     

Bilateral spontaneous hyphema in juvenile xanthogranuloma

This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management.     

Association of raised titres of antibodies to Chlamydia pneumoniae with a history of pre-eclampsia

OBJECTIVE: To establish the prevalence of Chlamydia pneumoniae (C. pneumoniae) infection in a pregnant UK population and to investigate whether C. pneumoniae infection is more common in women with a previous history of pre-eclampsia. DESIGN: Prospective study. SETTING: Academic Hospital. POPULATION: Ninety-one pregnant women (54 parous and 37 nulliparous) at 16-22 weeks of gestation were studied. Of the parous women, 32 had a previous history of pre-eclampsia. METHODS: Peripheral blood was drawn for C. pneumoniae antibodies between 16-22 and 28-40 weeks of gestation. C. pneumoniae antibodies were measured using a solid-phase enzyme immunoassay. According to pregnancy outcome, women were categorised into normal, gestational hypertension and pre-eclampsia groups. MAIN OUTCOME MEASURES: Serum levels of IgG, IgA and IgM C. pneumoniae antibodies. RESULTS: Prevalence of seropositivity to C. pneumoniae was 77%. Parous women had significantly higher levels of IgA and IgM C. pneumoniae antibodies than nulliparous women (P < 0.04). Parous women with previous pre-eclampsia were found to have higher levels of antibodies than parous women with a normal obstetric history (P< or = 0.003). There was no difference in the antibody levels in women with different pregnancy outcomes. CONCLUSIONS: The longitudinal data do not indicate an association between C. pneumoniae infection and pre-eclampsia. However, the subgroup analysis of parous women demonstrated raised C. pneumoniae antibodies in the women with previous pre-eclampsia, and therefore suggests that there may be an association between C. pneumoniae and the disease in this group.