What colour are the alligators? A critical look at methods used to assess communication skills in adults with learning difficulties.

This paper describes the assessment of communication skills in 120 adults with severe to moderate learning difficulties. Each subject was assessed on the Communication Assessment Profile (CASP), a new communication assessment procedure for use with this client group. Qualitative and quantitative comparisons were made between three different methods of assessment used in CASP. These included observational assessment, elicited conversation and a structured language task. The relative usefulness of each method was evaluated, and the implications for communication assessment outlined.

Platelet aggregation studies during transient hypoglycaemia: a potential method for evaluating platelet function.

The effect of acute hypoglycaemia on platelet function was examined in patients undergoing an insulin stress test. Enhanced platelet aggregation was observed in all cases but platelet count, platelet adenine nucleotides, and the plasma level of von Willebrand factor were unchanged overall. The onset of the hypoglycaemia-induced increase in platelet aggregation coincided with the lowest blood glucose levels recorded and with the clinical signs of adrenaline release. Increased platelet aggregation was maintained thereafter for the two-hour test period. There was no apparent correlation with changes in cortisol, growth hormone, and prolactin. No change in platelet function was observed after the administration of L-dopa. We suggest that the measurement of platelet aggregation during a standard insulin stress test may provide a means of evaluating platelet function in vivo and the influence of drugs thereon.

Immune complexes and abnormal liver function in haemophilia.

Abnormal 125I-Clq-binding activity was found in the sera of 94% of 55 haemophiliacs. Sera from 66% of these patients inhibited macrophage uptake of labelled aggregated human IgG in a competitive radiobioassay. These results suggest that large molecular weight immune complexes are present in these sera. Analysis of the precipitates obtained directly from the sera by addition of 4% polyethylene glycol showed either a mixture of IgG and IgM or IgM alone. There was poor correlation between the radiobioassay results and the Clq-binding activities in the whole group of 55 patients, suggesting a heterogeneous population of complexes. Nevertheless, a significant correlation was found between Clq-binding activities and the radiobioassay results in seven patients with antibodies to factor VIII, suggesting a homogeneous population of complexes. There was poor correlation between the level of immune complexes and the amount of replacement therapy the patient had received in the previous six months. Abnormal liver function tests were found in 55% of the patients studied but there was poor correlation between these abnormal levels and the Clq binding activities and radiobioassay results. Only two patients had clinical evidence of liver disease.

Regional co-ordinator for haemophilia in domiciliary practice.

The North-east Thames Region has appointed a nursing sister to co-ordinate the organisation of care for haemophiliacs in the region. As a result of the appointment, facilities for home treatment have expanded rapidly. Several associate centres providing care to haemophiliacs have been set up around the region in addition to the four main haemophilia centres, which are all in the south-west corner of the region. As well as providing support and supervision of patients on home treatment, the co-ordinator helps to place haemophiliac children in suitable schools, maintains the regional register of haemophiliacs, and has a more general role in ensuring that services are available where they are needed throughout the region.

Serial plasmapheresis in a haemophiliac with antibodies to FVIII.

Serial plasmaphereses were performed on a 23-year-old haemophiliac, with antibodies to factor VIII (FVIII), in order to reduce the antibody level before multiple dental extractions. Eight phereses were carried out in which approximately 1.5 litres plasma was exchanged with isotonic saline. By the ninth exchange, which was carried out immediately before the extraction, the antibody level had fallen from 4 u/ml to 0.8 u/ml. On this occasion fresh frozen plasma and FVIII concentrate were infused immediately after the pheresis. A normal post-infusion level of FVIII was achieved with a normal half disappearance time of the infused FVIII.

Health of the intensively treated hemophiliac, with special reference to abnormal liver chemistries and splenomegaly.

Liver function abnormalities have been noted in intensively treated hemophiliacs, and have led to less aggressive application of pooled plasma products by some physicians. In a prospective study, liver function was abnormal in 68 of 98 hemophiliacs. The abnormalities of hepatic function tended to persist over a 1-yr study period. There was no correlation between these abnormalities and the age of the patient, the presence of hepatitis-associated antigen or antibody, the presence or absence of splenomegaly (which was found in 26 of 98 patients), the number of infusions of plasma products, the type of hemophilia, or the type of product infused. Titers of antibodies to cytomegalovirus were generally higher in hemophilic patients than in a control group of healthy volunteers. These abnormalities did not suggest that a less aggressive infusion regimen was indicated for the hemophiliac, but did suggest the need for careful long-term observation of such patients.

Ultrasonography in the management of haemophilia.

Ultrasonography was used to demonstrate retroperitioneal haemorrhages and intramuscular haematoma in three patients with antibodies to factor VIII and one with von Willebrand's disease in whom major bleeding episodes occurred. Scans were useful in demonstrating the presence, location, size and regression of haemorrhages.

Acquired von Willebrand syndrome with inhibitors both to factor VIII clotting activity and ristocetin-induced platelet aggregation.

A case of acquired von Willebrand's syndrome (vWs) is described which appeared to be due to antibodies directed against factor VIII clotting activity (FVIIIC), factor VIII-related antigen (FVIIIRAg) and von Willebrand factor. The antibodies directed against FVIIIRAg was demonstrated by the inhibitory effect of a platelet eluate on Ristocetin-induced aggregation of normal platelets. This effect was not shown by the patient's platelet-poor plasma alone, nor could it be demonstrated in platelet eluates from 13 other patients who had antibodies to FVIIIC but in whom there was no evidence of an acquired vWs.

Factor-VIII-related antigen: measurement by enzyme immunoassay.

Factor-VIII-related antigen was measured, both by an enzyme immunoassay using a microplate method and by the Laurell technique, in normal people, patients with von Willebrand's disease, haemophiliacs, and obligatory haemophilia carriers. The enzyme immunoassay was simpler to perform and gave equally reliable and reproducible results. Many more assays could be carried out at any one time.

The suppression of factor VIII antibodies in haemophilia.

Three severely affected haemophiliacs who had developed antibodies to factor VIII are described in whom the administration of cyclophosphamide was thought to have prevented reappearance of the antibody after factor VIII infusion. Some of the possible causes which might have led to the favourable outcome in these and three other, previously reported, patients in whom the antibody, which was raised at the time of IS therapy, dropped to zero and failed to reappear, are discussed. It is suggested that immunosuppressive therapy was effective in these patients as it was given during or soon after the primary immune response. As the antibody response becomes increasingly well established, so the chances of successful immunosuppressive therapy recede.

Effect of oral contraceptives on factor VIII clotting activity and factor VIII related antigen in normal women.

The factor VIII clotting activity (VIIIc1 and factor VIII related antigen (VIIIRAg) were determined repeatedly in 24 pairs of age-matched normal women, one of each pair being on oral contraceptives. No significant differences in either parameter or in the VIIIc/VIIIRAg ration were found between the two groups ,although the mean factor VIII clotting activity and VIIIc/VIIIRAg ratios for women on oral contraceptives were very slightly higher than for those not on oral contraceptives.

The von Willebrand syndrome.

Five patients with an original diagnosis of von Willebrand's disease are described because of their levels of factor VIII related protein, Ristocetin-induced platelet aggregation and/or family studies differed from the main group of patients with classical von Willebrand's disease. Two had normal levels of factor VIII related protein with reduced Ristocetin aggregation when this was tested in platelet rich plasma. In one, however, this was due to a plasma defect and in the other to a platelet abnormality. After cryoprecipitate infusion all abnormal tests were corrected in both these patients. The first patient, however, failed to show a secondary rise of factor VIII whereas the second showed a secondary rise of both factor VIII and of factor VIII related protein. The other three cases, who were all very severely affected, have been separated from the main group as none of their families was segregating for classical von Willebrand's disease. It is suggested that the term von Willebrand's disease should be confined to those patients who have reduced factor VIII related protein and Ristocetin aggregation, and that von Willebrand's syndrome should be used for the various sub-groups that are emerging.