Haemodilution induces a hypercoagulable state

It has been suggested that haemodilution with saline may increase whole blood coagulation. This study was conducted in two parts. First, we investigated the effect of in vitro dilution of blood with saline on whole blood coagulation as measured by the thrombelastogram (TEG). Blood (4 ml) was diluted with 0.9% saline 1 ml and coagulation compared with that of an undiluted control specimen obtained concurrently from the same subject. In the second part, the study was repeated using a modified gelatin colloidal solution (Haemaccel) as the diluent. The r time, k time and r + k time were decreased relative to control in both diluent groups. The alpha angles were increased compared with control in both groups while maximum amplitude was unchanged in the Haemaccel diluted group. We conclude that haemodilution per se increases the coagulability of whole blood in vitro, but that saline haemodilution has a more marked effect on final clot strength.      

Subacute and chronic extradural haematomas

Over an 8-year period (1982-1989) 43 patients with subacute and chronic extradural haematomas were treated. The clinical picture of the patients, computed tomography (CT) as well as operative findings and prognosis were evaluated. The radiological picture as seen on CT was correlated with the time interval elapsing between initial trauma and surgery. Operative findings are discussed and the terms 'subacute' and 'chronic' are defined.     

Epiphyseal stippling in acrodysostosis

Widespread epiphyseal stippling was demonstrated radiologically during the early months of life in 7 children with acrodysostosis. This finding, which does not seem to have been previously documented in this disorder, is important in the differential diagnosis.     

Prenatal diagnosis in autosomal dominant Beckwith-Wiedemann syndrome

A 20-year-old woman with Beckwith-Wiedemann syndrome (BWS) was ultrasonographically appraised at intervals during her pregnancy. Unequivocal evidence for a diagnosis of BWS was obtained in the fetus and this was confirmed postnatally. Early ultrasound diagnosis enabled appropriate genetic counselling to be given; neonatal complications, such as hypoglycaemic episodes, were prevented.     

Alpha-glucosidase activity and sperm motility

We correlated the activity of alpha-glucosidase in seminal plasma with the motility and differential motility of sperm. Significant positive correlations were found between the alpha-glucosidase activity and both motility and the percentage sperm with good forward progression. This supports the use of alpha-glucosidase in semen as a marker of epididymal function and specifically of the development of motility.     

Costello syndrome with growth hormone deficiency and hypoglycemia: a new report and review of the endocrine associations

We describe an 18-month-old boy with Costello syndrome (CS) with proven partial growth hormone (GH) deficiency and hypoglycemic episodes. The hypoglycemia is deemed to be due to cortisol deficiency. This report represents the second published case of cortisol deficiency in the CS. A brief review of the endocrine disturbances in the syndrome is provided. We highlight the need for careful endocrinological evaluation of individuals with CS.     

Heterogeneity of pseudoxanthoma elasticum: delineation of a new form?

Sixty-four patients with pseudoxanthoma elasticum (PXE) were investigated in a nationwide study within South Africa and Zimbabwe. Thirty-nine individuals formed a distinct clinical subgroup. These persons were found exclusively among people of Afrikaner descent, whose origins are mainly derived from Dutch and French-Huguenot stock. This disorder was inherited as an autosomal recessive trait and presented mild to moderate cutaneous and cardiovascular manifestations. However, after the third decade of life severe visual impairment developed and culminated in blindness in 8 people by the age of 50. The cause of the visual defect was progressive extension of angioid streaks into the macula with neovascularization and haemorrhage. Laser therapy may have prevented further bleeding in 4 instances. The severity of ocular involvement contrasted with the mildness of the skin changes, and in this respect the condition seems to differ from previously delineated autosomal recessive forms of PXE.