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Two hereditary syndromes characterized by the association of retinal changes and erythrocytic anomalies have been described: (1) Retinopathy complicating haemoglobin anomalies, thalassaemia (Rudd, Evans, and Peeney, 1953), or sickle-cell anaemia (Lieb, Geeraets, and Guerry, 1959); (2) Atypical retinitis pigmentosa with thorny erythrocytes (acanthocytosis: Bassen and Kornzweig, 1950; Kornzweig a...

We present an updated cumulative size distribution (CSD) for Jupiter Family comet (JFC) nuclei, including a rigourous assessment of the uncertainty on the slope of the CSD. The CSD is expressed as a power law, N(> rN) ∝ r −q N , where rN is the radius of the nuclei and q is the slope. We include a large number of optical observations published by ourselves and others since the comprehensive rev...

Mucopolysaccharidosis type VI, Maroteaux-Lamy syndrome is a lysosomal storage disorder with progressive, multisystem involvement caused by deficiency of the lysosomal enzyme N-acetylgalactosamine-4-sulfatase leading to accumulation of the glycosaminoglycan, keratan sulfate. Enzyme replacement therapy (ERT) has been shown to clinically benefit affected individuals. A combined treatment regime of...

Dr. Lamy that such rates are probably a much better reflection of current risk. These studies do not specify the rates for the subgroup with relevant carotid lesions, however, making the problem of rinding appropriate "historical controls" even more vexing. Fortunately, it appears that definitive resolution will become available in the next few years, when several large European and North Ameri...

A method is presented for the rapid purification of dog thyroid calcyphosine, a protein previously identified as a major substrate for cyclic AMP-dependent protein kinase in dog thyroid slices stimulated by thyrotropin [Lecocq, Lamy and Dumont (1979) Eur. J. Biochem. 102, 147-152]. The protein was previously identified as a spot on two-dimensional gels and is now purified in its native form by ...

MULTIPLE approaches are important when attempting to limit allogeneic blood transfusions. My interest in developing strategies to decrease bleeding and transfusion requirements started in the early 1980s, when I was evaluating perioperative anaphylaxis and noted that transfused blood was an important cause of life-threatening hypersensitivity reactions. There are multiple reasons to reduce tran...