Molecular studies of haemophilia B in Sweden

SummaryA mixture of adsorbed normal human plasma and chicken plasma was prepared as reagent for factor IX measurement using a one-stage method. The substrate was found to be specific for factor IX. Its performances tested on samples displaying factor IX activity ranging from <l%–2,500% compared favorably with those obtained when using the plasma of severe haemophilia B patients as substrate.

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Management of Haemophilia in Sweden

Thrombosis and Haemostasis ◽

10.1055/s-0038-1647991 ◽

1976 ◽

Vol 35 (03) ◽

pp. 510-521 ◽

Cited By ~ 13

Author(s):

Inga Marie Nilsson

Keyword(s):

Factor Viii ◽

Total Population ◽

Age Groups ◽

Factor Ix ◽

Haemophilia A ◽

Severe Haemophilia ◽

Haemophilia B ◽

Human Fraction ◽

Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

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The Formation of Intermediate Product I in a Purified System The Role of Factor IX or of its Precursor and of a Hageman Factor-PTA Fraction

Thrombosis and Haemostasis ◽

10.1055/s-0038-1654555 ◽

1961 ◽

Vol 6 (02) ◽

pp. 224-234 ◽

Cited By ~ 4

Author(s):

E. T Yin ◽

F Duckert

Keyword(s):

Intermediate Product ◽

Factor Ix ◽

Assay Method ◽

Lag Phase ◽

Factor X ◽

Haemophilia B ◽

Hageman Factor ◽

One Stage ◽

The One

Summary1. The role of two clot promoting fractions isolated from either plasma or serum is studied in a purified system for the generation of intermediate product I in which the serum is replaced by factor X and the investigated fractions.2. Optimal generation of intermediate product I is possible in the purified system utilizing fractions devoid of factor IX one-stage activity. Prothrombin and thrombin are not necessary in this system.3. The fraction containing factor IX or its precursor, no measurable activity by the one-stage assay method, controls the yield of intermediate product I. No similar fraction can be isolated from haemophilia B plasma or serum.4. The Hageman factor — PTA fraction shortens the lag phase of intermediate product I formation and has no influence on the yield. This fraction can also be prepared from haemophilia B plasma or serum.

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Darwin's two competing phylogenetic trees: marsupials as ancestors or sister taxa?

Archives of Natural History ◽

10.3366/anh.2012.0091 ◽

2012 ◽

Vol 39 (2) ◽

pp. 217-233 ◽

Cited By ~ 4

Author(s):

J. David Archibald

Keyword(s):

Fossil Record ◽

Evolutionary Biology ◽

Phylogenetic Trees ◽

Charles Darwin ◽

The Other ◽

Charles Lyell ◽

Single Origin ◽

Molecular Studies ◽

Richard Owen ◽

First Time

Studies of the origin and diversification of major groups of plants and animals are contentious topics in current evolutionary biology. This includes the study of the timing and relationships of the two major clades of extant mammals – marsupials and placentals. Molecular studies concerned with marsupial and placental origin and diversification can be at odds with the fossil record. Such studies are, however, not a recent phenomenon. Over 150 years ago Charles Darwin weighed two alternative views on the origin of marsupials and placentals. Less than a year after the publication of On the origin of species, Darwin outlined these in a letter to Charles Lyell dated 23 September 1860. The letter concluded with two competing phylogenetic diagrams. One showed marsupials as ancestral to both living marsupials and placentals, whereas the other showed a non-marsupial, non-placental as being ancestral to both living marsupials and placentals. These two diagrams are published here for the first time. These are the only such competing phylogenetic diagrams that Darwin is known to have produced. In addition to examining the question of mammalian origins in this letter and in other manuscript notes discussed here, Darwin confronted the broader issue as to whether major groups of animals had a single origin (monophyly) or were the result of “continuous creation” as advocated for some groups by Richard Owen. Charles Lyell had held similar views to those of Owen, but it is clear from correspondence with Darwin that he was beginning to accept the idea of monophyly of major groups.

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Molecular studies in gorgonian alloimmunity : search for gene homologs of the immunoglobulin gene superfamily in Swiftia exserta

10.25148/etd.fi14051878 ◽

1991 ◽

Author(s):

Julia Bundschuh

Keyword(s):

Immunoglobulin Gene ◽

Molecular Studies ◽

Immunoglobulin Gene Superfamily

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On Riccia marginata and related species (Ricciaceae, Marchantiophyta)

Novosti sistematiki nizshikh rastenii ◽

10.31111/nsnr/2012.46.298 ◽

2012 ◽

Vol 46 ◽

pp. 298-305 ◽

Cited By ~ 1

Author(s):

A. D. Potemkin ◽

T. Ahti

Keyword(s):

Related Species ◽

Lake Ladoga ◽

Leningrad Region ◽

Sem Images ◽

North Shore ◽

The North ◽

Molecular Studies ◽

The Future ◽

The Town

Riccia marginata Lindb. was described by S. O. Lindberg (1877) from the outskirts of the town of Sortavala near the north shore of Lake Ladoga, Republic of Karelia, Russia. The species has been forgotten in most recent liverwort accounts of Europe, including Russia. Lectotypification of R. marginata is provided. R. marginata shares most characters with R. beyrichiana Hampe ex Lehm. It differs from “typical” plants of R. beyrichiana in having smaller spores, with ± distinctly finely areolate to roughly papillose proximal surfaces and a narrower and shorter thallus, as well as in scarcity or absence of marginal hairs. It may represent continental populations of the suboceanic-submediterranean R. beyrichiana, known in Russia from the Leningrad Region and Karelia only. The variability of spore surfaces in R. beyrichiana is discussed and illustrated by SEM images. A comparison with the spores of R. bifurca Hoffm. is provided. The question how distinct R. marginata is from R. beyrichiana needs to be clarified by molecular studies in the future, when adequate material is available. R. marginata is for the time being, provisionally, included in R. beyrichiana.

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