Studies of the pathophysiology of acquired von Willebrand's disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies.

In seven patients with acquired von Willebrand's disease (AvWD) associated with lymphoproliferative disorders or benign monoclonal gammopathies, the platelet contents of von Willebrand factor antigen and ristocetin cofactor (vWF:Ag and vWF:RiCof, respectively) were normal. All the multimers of vWF:Ag could be seen in the 1.6% SDS-agarose gel electrophoresis patterns of plasma and platelet lysates.

A variant of von Willebrand's disease characterized by recessive inheritance and missing triplet structure of von Willebrand factor multimers.

A 10-yr-old girl had bleeding symptoms of moderate severity; her mother and maternal aunt had milder bleeding symptoms, and other members of the kindred were asymptomatic. In the child, factor VIII coagulant activity (VIII:C) and von Willebrand factor antigen (vWF:Ag) were normal, ristocetin cofactor very low, and the bleeding time (BT) markedly prolonged. These values were normal in the rest of the kindred, but the mother and maternal aunt had prolonged BT and a high VIII:C/vWF:Ag ratio.

Deamino-8-D-arginine vasopressin shortens the bleeding time in uremia.

In a randomized double-blind cross-over trial we gave either 1-deamino-8-D-arginine vasopressin or placebo to 12 patients with uremia, hemorrhagic tendencies, and prolonged bleeding times. After vasopressin infusion, all patients had shortened bleeding times, with the effect lasting for at least four hours in most cases. Platelet count, platelet cyclic AMP levels, platelet retention on glass beads, plasma fibronectin, serum thromboxane B2 and residual prothrombin, hematocrit, and plasma osmolarity were unchanged after vasopressin.

Type IIB von Willebrand's disease: differential clearance of endogenous versus transfused large multimer von willebrand factor.

The abnormal multimeric composition of plasma von Willebrand factor in type IIB von Willebrand's disease is transiently corrected after infusion of 1-deamino-[8-D-arginine]-vasopressin. However, the larger multimers released into the circulation disappear more rapidly in these patients than in type I von Willebrand's disease or normals. We demonstrate that the larger multimers of normal von Willebrand factor transfused into a type IIB patient are cleared from the circulation more slowly than multimers of similar size endogenously released from tissue stores.

Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC).

A variant of von Willebrand's disease has been identified in which sodium dodecyl sulfate agarose electrophoresis provides evidence that the von Willebrand factor present is structurally abnormal. Rather than the repeating triplet seen in normal subjects and in patients with the IIA and IIB variants, a repeating doublet was present in the propositus. None of the bands had the same mobility as bands in normal subjects or previously described von Willebrand's disease patients.

Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes.

We have studied the modifications in the multimeric composition of plasma factor VIII/von Willebrand factor and the bleeding time response following administration of 1-Deamino-[8-D-arginine]-Vasopressin (DDAVP) to patients with different subtypes of von Willebrand's disease. In type I, all multimers were present in plasma in the resting state, though they were decreased in concentration. Administration of DDAVP resulted in an increased concentration of these forms as well as the appearance of larger forms than were previously present.

Alterations of factor VIII von Willebrand factor in clinical conditions associated with an increase in its plasma concentration.

Factor VIII-related antigen (VIIIR:Ag) was consistently higher than factor-VIII procoagulant activity (VIII:C) in 57 patients with clinical conditions characterized by acute-phase reactions. Two different methods for measuring VIII:C (one- and two-stage assays) and VIIIR:Ag (electroimmunodiffusion and immunoradiometric assay) gave concordant results in the majority of cases. In 43% of plasma samples, crossed immunoelectrophoresis in agarose gel was characterized by the appearance of an additional, fast-moving precipitin peak which was immunologically identical with the major, slower-moving VIIIR:Ag peak.

Quantification of vestibular compensation in unilateral Meniere's disease.

The main parameters of the vestibulo-ocular reflex (i.e. the gain or reflectivity, the long-time constant of the semicircular canals, the time constant of the mechano-neural transduction, and that of the central leaky integrator) were computed from the post-rotational nystagmic responses of unilateral Meniere's patients.

[A bladder calculus of unusual dimensions].

A case of giant vesical calculosis is presented on account of its statistical and radiographical unusualness. Its aetiopathogenesis is briefly discussed.

Factors mediating against excellence in dental esthetics.

Factors mediating against excellence in dental esthetics have been classified and enumerated in this article. A formula for producing unesthetic prostheses can be hypothesized as follows: Educational de-emphasis + Lack of research + Technical orientation + Technical tradition + Delegation (abdication) + Poor economics + Fatique + Poor office design + Convention + Conditioning + Schemata leads to POOR DENTAL ESTHETICS. It is postulated that a formula for excellent dental esthetics can be produced by reversing these factors: Altered schemata + Deconditioning + Altered convention + Adequate office design + Elimination of fatique + Favorable economics + Personal participation + Research + Educational emphasis leads to ESTHETIC EXCELLENCE.

Treatment of neoplasia of the bladder with topical application of a synthetic peptide complex preparation: preliminary results.

Preliminary results are reported on the endo-vesical use of a complex of synthetic peptides ('Peptichemio') in 15 patients suffering from bladder neoplasia who were judged to be beyond surgery or radiotherapy. The agent was introduced into bladder in 20 mg doses diluted in 50 ml of 5% glucose and retained for approximately 30 minutes. Treatment was repeated every 5 to 7 days.