Cor pulmonale and acute liver necrosis, due to upper airway obstruction as part of pycnodysostosis.

A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma.

Sexuality as a treatment issue with a special population of adolescents.

The handicapped adolescent population heretofore has not been properly identified or serviced. These adolescents have to deal with body image and sexual identity problems. Working with the adolescent individually, in groups, and in a therapeutic community setting, we have developed methods of dealing with the issue of sexuality.

Human placental 5'-nucleotidase: purification and properties.

Subcellular fractionation of human term placenta showed that the highest relative specific activity of 5'-nucleotidase and alkaline phosphatase resided in the microsomal fraction; of the total 5'-nucleotidase activity present, 7 per cent was in the cytosol. 5'-Nucleotidase was reproducibly purified over 500-fold in 17 per cent yield from the insoluble component of homogenates of term placenta to give a single major glycoprotein with two minor inactive protein contaminants. Purified placental 5'-nucleotidase was free from non-specific or alkaline phosphatase, hydrolysed 12 to 22 mumol AMP/min/mg of protein at 30 degrees C, and was activated up to fivefold by Triton X-100.

Susceptible Escherichia coli cells can actively excrete tetracyclines.

Escherichia coli shows severalfold less susceptibility to tetracyclines when grown in enriched medium than in minimal medium. Transport studies with cells harvested from these media showed different handling of the drugs. Whereas an energy-dependent uptake of tetracycline and minocycline was observed in susceptible K-12 and wild-type E.

Assay of AHF concentrates and standards: failure to eliminate variability with a monographed assay.

Previous calibration studies have shown a high interlaboratory variability in the potency of the proposed Office of Biologics (National Center for Drugs and Biologics, US-FDA) AHF standard relative to the 2nd International Standard for Factor VIII (Factor VIII:C) (WHO 73/552). This led to the formation of an Industry Collaborative Study group whose objective was to reduce the assay variability. The group, in collaboration with the Office of Biologics and the National Institute for Biological Standards and Control (UK), designed a study based on a monographed one-stage assay protocol, which specified all materials, assay methods, equipment, dilution technique, reagents, assay order, and calculation methodology.

Plasma derivatives and viral hepatitis.

Plasma derivatives can be separated into those with either a low or a high risk of transmitting viral hepatitis. Low-risk products, with few exceptions, will remain low-risk irrespective of the plasma from which they are manufactured because they are heated at 60 degrees C for 10 hours (Albumin, Plasma Protein Fraction) or because they contain protective antibodies (Immune Globulin). This would appear to be the case not only for hepatitis B but also for non-A, non-B hepatitis.

The diagnosis of meniscus pathology.

A study of 281 patients evaluated for a meniscus pathologic condition was performed to assess the accuracy of the clinical diagnosis and arthrogram and delineate the role of arthroscopy. Following the clinical examination, the surgeon stated his level of confidence in his diagnosis, as did the radiologist in his interpretation of the arthrogram. In 134 cases inspected by arthroscopy or arthrotomy, the clinician proved to be correct 74% of the time, and the arthrogram accuracy in evaluating the medial meniscus was 89%; the majority of the errors were false positive diagnoses.

Uptake of ornithine by rat liver mitochondria.

Uptake of [14C]-L-ornithine by rat liver mitochondria has been measured by using the silicone sampling technique. The uptake of ornithine measured after 20-45 s of incubation exhibits stereospecificity, pH dependence, and a lack of dependence on respiratory energy. A slower subsequent increase in [14C]-L-ornithine counts associated with the mitochondria, which is blocked by the transaminase inhibitor aminooxyacetate, is attributed to metabolism of the labeled ornithine.

International collaborative study on the assay of commerically available high and low molecular weight urokinases.

Urokinases of different molecular weights are now commerically available. An international collaborative study (eight laboratories) has been conducted to investigate the effect of type and concentration of plasminogen on the assay of two different urokinase preparations against the International Reference Preparation (IRP). Considerable inter-laboratory variation in relative potency estimation was found, and a small effect of plasminogen concentration, independent of type, was apparent.

Carpal-tunnel syndrome. Results of a prospective trial of steroid injection and splinting.

In order to define the role of steroid injection and splinting as a method of treatment of carpal-tunnel syndrome, a prospective study was performed on fifty hands in forty-one consecutive patients. All hands were treated with a single injection and three weeks of splinting. Follow-up ranged from a minimum of six months to a maximum of twenty-six months, with a mean of eighteen months.

Effect of mersalyl on mitochondrial Mg++ flux.

The mercurial mersalyl has little effect either on rapid Mg++ binding by isolated rat liver mitochondria or on the total Mg++ content of these organelles measured after 0.75 min of incubation at 20 degrees C. The data do not support the previous suggestion that the increased permeability to K+ of mitochondria treated with mersalyl results from release of endogenous Mg++.

Contact-activated factors: contaminants of immunoglobulins preparations with coagulant and vasoactive properties.

The intramuscular or intravenous administration of ISG prepared from human plasma by ethanol fractionation can elicit such reactions as pain at the injection site, flushing, and even hypotension. Similar adverse reactions to plasma protein fraction, a volume expander also made by ethanol fractionation, have been associated with PKA (Hageman factor fragments) in the product. Twenty-five lots of commercial ISG were therefore analyzed for PKA and kallikrein, components of the contact activation system which could mediate such reactions through the generation of kinins in recipients.

Removal of hepatitis-B-virus infectivity from factor-IX complex by hepatitis-B immune-globulin. Experiments in chimpanzees.

Hepatitis-B-virus infectivity can be removed from a heat-labile clotting factor concentrate by the addition of hepatitis-B immune-globulin. Three chimpanzees were each inoculated with samples of factor-IX complex (factor IX) which had been deliberately contaminated with 10(3.5) chimpanzee infectious doses of hepatitis-B virus from a known infectious inoculum.

The inhibition of high and low molecular weight urokinase in plasma.

The rates of inhibition of high molecular weight (HMW) and low molecular weight (LMW) urokinase (UK) incubated in plasma or with purified antithrombin III (AT-III) were compared. Using a fibrinolytic assay system to determine residual biologic activity, polyacrylamide gel electrophoresis to demonstrate the formation of complexes, and selective immunoprecipitation techniques to identify the plasma inhibitors participating in the neutralization process, it was established that: (A) HMW-UK is inhibited more rapidly than LMW-UK, both in plasma and with purified AT-III; (B) heparin (3--10 U/ml accelerates the neutralization process in both systems, but only slightly; and (C) in plasma, several inhibitors, alpha 2-macroglobulin, alpha 1-antitrypsin, and antithrombin III, neutralize the activity of HMW-UK and LMW-UK.