Johan L. Willemse

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Two siblings with cerebral calcifications are described, clinically characterized by the early onset of general regression and epileptic seizures, followed by cerebral blindness and spastic tetraplegia. No remarkable biochemical abnormalities were found. Death ensued before the age of seven years. Radiology of the skull in the second case showed widespread(More)
The authors describe a case of predominantly right-sided arthrogryposis in an infant with contralateral congenital cerebral hypoplasia and associated unilateral hydrocephalus. Diagnosis was made by prenatal ultrasonography and confirmed by postnatal cerebral CT-scanning. In addition to a variety of congenital neuromuscular disorders, arthrogryposis may be(More)
BACKGROUND & AIM The ability of Staphylococcus aureus to successfully colonize (a)biotic surfaces may be explained by biofilm formation and the actions of virulence factors. The aim of the present study was to establish the presence of 52 proteins, including virulence factors such as alpha-toxin, during biofilm formation of five different (methicillin(More)
Procarboxypeptidase U [proCPU, thrombin-activatable fibrinolysis inhibitor (TAFI), EC 3.4.17.20] belongs to the metallocarboxypeptidase family and is a zymogen found in human plasma. ProCPU has been proposed to be a molecular link between coagulation and fibrinolysis. Upon activation of proCPU, the active enzyme (CPU) rapidly becomes inactive due to its(More)
A group of 43 patients suffering from cerebral palsy and cerebral visual impairment was compared with a group of 24 cerebral palsy patients with normal visual acuity, with regard to a functional level in daily life. Four categories were considered: communication, emotional contact, self-care and intelligence. Cerebral visual impairment-cerebral palsy(More)
The influence of the P1 amino acid on the substrate selectivity, the catalytic parameters K(m) and k(cat), of carboxypeptidase M (CPM) (E.C. 3.4.17.12) was systematically studied using a series of benzoyl-Xaa-Arg substrates. CPM had the highest catalytic efficiency (k(cat)/K(m)) for substrates with Met, Ala and aromatic amino acids in the penultimate(More)
A prospective clinical study is presented of 75 patients with multiple congenital contractures. With the data from medical history, child neurologic examinations, laboratory tests including chromosome and dermatoglyphic analysis, and neuropathology in 23 cases with perinatal death, a nosological or syndromal diagnosis was made in 61 cases. These cases were(More)
BACKGROUND Measurement of procarboxypeptidase U (TAFI) in plasma by activity-based assays is complicated by the presence of plasma carboxypeptidase N (CPN). Accurate blank measurements, correcting for this interfering CPN activity, should therefore be performed. A selective CPU substrate will make proCPU determination much less time-consuming. METHODS We(More)
CT scanning was done to calculate the volume of intracranial spaces in children with the purpose of developing a reliable method of differentiating the various causes of macrocephaly. The technique has been applied to the CT scans of 60 apparently normal children, and the resulting graphs show the normal relationship between intracranial volumes and age(More)