A child with homozygous protein C deficiency was treated at age 20 months by orthotopic hepatic transplantation. Postoperatively there was complete reconstitution of protein C activity and resolution of the thrombotic condition.

In a peri-urban shanty town in Lima, Peru, that was hyperendemic for Giardia lamblia, 44 children aged between 0.9 months and 10 years were effectively treated for Giardia lamblia with tinidazole. Stools were examined weekly in the 6 months after treatment to determine the rate of reinfection, and after reinfection stools continued to be examined. 98% of the children became reinfected with Giardia lamblia within 6 months, and after reinfection stool excretion of the parasite lasted a mean (SD) of 3.2 (3.3) months. The children's mean stool pH and their mean stool fat index was unaffected by Giardia lamblia reinfection. Treatment of all symptomless Giardia lamblia infections in a developing country hyperendemic for the disease is of questionable value because of rapid reinfection.

A randomised trial of 367 patients with acute myocardial infarction was performed to determine whether an invasive strategy combining thrombolysis with recombinant tissue-type plasminogen activator (rTPA), heparin, and acetylsalicylic acid, and immediate percutaneous transluminal coronary angioplasty (PTCA) would be superior to a noninvasive strategy with the same medical treatment but without immediate angiography and PTCA. Intravenous infusion of 100 mg rTPA was started within 5 h after onset of symptoms (median 156 min). Angiography was performed 6-165 min later in 180 out of 183 patients allocated to the invasive strategy; 184 patients were allocated to the non-invasive strategy. Immediate PTCA reduced the percentage stenosis of the infarct-related segment, but this was offset by a high rate of transient (16%) and sustained (7%) reocclusion during the procedure and recurrent ischaemia during the first 24 h (17%). The clinical course was more favourable after non-invasive therapy, with a lower incidence of recurrent ischaemia within 24 h (3%), bleeding complications, hypotension, and ventricular fibrillation. Mortality at 14 days was lower in patients allocated to non-invasive treatment (3%) than in the group allocated to invasive treatment (7%). No difference between the treatment groups was observed in infarct size estimated from myocardial release of alpha-hydroxybutyrate dehydrogenase or in left ventricular ejection fraction after 10-22 days. Since immediate PTCA does not provide additional benefit there seems to be no need for immediate angiography and PTCA in patients with acute myocardial infarction treated with rTPA.

The best management of fetal hydronephrosis is controversial. Despite the lack of experimental evidence that prenatal drainage of the obstructed urinary tract substantially improves ultimate renal function, various forms of percutaneous intervention on the fetal bladder and kidney have been used. To evaluate the efficacy of intervention for suspected fetal obstructive uropathy, all published reports of drainage of the fetal urinary tract up to December, 1985, were reviewed. In the 57 reported cases, the most common type of intervention was placement of a vesicoamniotic shunt (37%). Complications occurred in 25 cases (44%), including inadequate shunt drainage or migration (19%), onset of premature labour within 48 h (12%), urinary ascites (7%), and chorioamnionitis (5%). Of 28 fetuses with associated oligohydramnios, only 6 (21%) survived. 2 of these survivors had vesicoamniotic shunts, 2 single or multiple bladder aspirations, 1 an external renal drainage catheter, and 1 in-utero vesicostomy. Because of the high complication rate and lack of evidence of improved survival from in-utero drainage procedures, a prospective, randomised trial is needed to compare survival with and without vesicoamniotic shunt placement.

In 17 healthy young men who had a parent with documented early coronary disease, ratings of type A behaviour correlated with upregulated lymphocyte beta 2 receptor density and inversely with the ratio of platelet alpha 2 to lymphocyte beta 2 receptor density ratio. This indicates a correlation of type A behaviour with receptor-based determinations of increased peripheral alpha-adrenergic balance, consistent with increased coronary arterial vasoconstriction, perhaps leading to coronary artery disease.

Unusual cutaneous vascular neoplasms distinct from Kaposi's sarcoma were observed in five patients with the acquired immunodeficiency syndrome (AIDS) or human immunodeficiency virus (HIV)-1 infection. The cutaneous lesions were solitary or multiple papules and nodules. In some patients the lesions also affected internal organs. Histologically the neoplasms were composed of proliferating blood vessels and cells with epithelioid features. Immunoperoxidase studies of one lesion showed that the cells expressed both factor VIII antigen, a maker for endothelial cells, and alpha 1-anti-chymotrypsin, a marker for histiocytes. In some patients the lesions gradually disappeared but in two they were the cause of death, in one case from disseminated intravascular coagulation and in the other from laryngeal obstruction by the tumour.

During studies of T-cell recognition of autologous tumour cells, a number of tumour cell lines derived from patients with lymphoma proved to be poor stimulators of both autologous and allogeneic T-cell responses. Analysis of the tumour cell surface molecules indicated that expression of the lymphocyte-function-associated antigen, LFA-1, was lacking, whereas normal leucocytes from these patients expressed normal levels of LFA-1. Examination of other lymphoid tumours revealed that most high grade lymphomas, but not most low or intermediate grade lymphomas, do not express the LFA-1 molecule. Furthermore, in an initial survey, the tumours from 5 of 7 patients with non-relapsing large cell lymphomas expressed LFA-1 whereas only 3 of 18 patients with relapsing lymphomas had tumours that did so. These findings suggest that tumour cells lacking surface LFA-1 cannot initiate an effective immune response in vivo. This lack of immunogenicity might contribute to escape from immunosurveillance.

Seven patients with hepatitis delta virus (HDV) cirrhosis underwent liver transplantation. In every case the HDV infection was florid but accompanied by an inactive hepatitis B virus (HBV) infection. The patients were given anti-HB surface antigen (HBsAg) serum globulins and HBV vaccine. Two patients cleared the HBsAg and the HDV, and are alive and well 14 and 15 months, respectively, after transplantation. HDV infection recurred in the other five patients: hepatitis developed in three, another died, and the fifth was re-transplanted for causes unrelated to viral hepatitis (reinfection was shown by the presence of HD antigen in the graft). Liver transplantation is feasible in patients with HDV disease but involves a high risk of HDV reinfection that cannot be predicted by the virological pattern of the native HBV infection or prevented by conventional HBV prophylaxis.