bluuebunny/crossref_metadata_2025_split huggingface dataset:
Chromosome Studies on Bone Marrow from a Male Control Population
Authors: O'Riordan Maureen L. , Berry Elizabeth W. , Tough Ishbel M. 
Published: March 2008

Summary Chromosome analyses of bone marrow cells prepared by the direct method were carried out on 32 male patients, who had no primary haematological disease. One patient was found to have a pericentric inversion of the Y chromosome, which was also present in a skin fibroblast culture and in a lymphocyte culture. Three cases were found to have a high percentage of bone marrow cells with a 45,X chromosome constitution and one case had a 45, X?Y ‐ or G‐ cell line. The high degree of aneuploidy was not found on examination of cells from leucocyte cultures. This observation is discussed in relation with other similar findings in patients with haematological diseases. The remaining 27 patients had a normal male karyo‐type and showed only small deviations from the modal chromosome number.The percentage of bone marrow cells with either chromatid aberrations or stable chromosome abnormalities was not significantly different from previous observations on lymphocyte cultures. However, the proportion of cells with unstable chromosome abnormalities was found to be substantially reduced in comparison with results from cultured lymphocytes examined in their first in vitro division.
Haemolytic Anaemia due to Haemoglobin Torino
Authors: Prato V. , Gallo E. , Ricco G. , Mazza U. , Bianco G. , Lehmann H. 
Published: March 2008

Summary Haematological and clinical features of the haemolytic anaemia due to haemoglobin Torino are reported. The haematological picture is similar to that observed in other haemolytic anaemias due to unstable haemoglobins. The disease, present in three members of the same family, is inherited as a Mendelian co‐dominant, non‐sex‐linked character.The disease is caused by the presence of haemoglobin Torino in erythrocytes and sulphonamide can provoke haemolytic crises. Besides the globin abnormality a decrease in porphobilinogen synthesis was demonstrated. Splenectomy improves the haematological state and clinical course.
A ‘New’ Congenital Haemorrhagic Condition due to the Presence of an Abnormal Factor X (Factor X Friuli): Study of a Large Kindred
Authors: Girolami A. , Molaro G. , Lazzzarin M. , Scarpa R. , Brunetti A. 
Published: March 2008

Summary Three related patients are presented who show a congenital coagulation disorder with laboratory features intermediate between classical factor‐VII and factor‐X deficiencies. A woman and two men had suffered from bleeding since early childhood, with epistaxis, bleeding from the gums, post‐traumatic haemarthroses, bleeding after tooth extractions and other surgical procedures. Investigation demonstrated a prolonged prothrombin time, prolonged partial thromboplastin time, abnormal prothrombin consumption and abnormal thromboplastin generation corrected by normal serum. Platelet and vascular tests were normal and no hyperfibrinolysis was found. Factors I, II, V, VII, IX, XI and XII were within normal limits in all three patients. Mutual correction was demonstrated with a known factor‐VII‐deficient plasma but not with Stuart (X‐deficient) plasma. Factor‐X assay yielded low (4–9%) levels using tissue whole thromboplastin or tissue partial thromboplastin; but the results were normal with a Stypven‐cephalin mixture. In agreement with these results, the Stypven‐cephalin clotting time, the Stypven clotting time and the factor II + factor X level using a Stypven‐cephalin mixture were normal, ‘correction’ being attributable to the Russell's Viper venom. These results were thought to indicate an abnormal factor X rather than a real deficiency.The presence of abnormal factor X was demonstrated by the antibody neutralization technique and by the immunodiffusion studies. The defect, like classical factor X deficiency, is transmitted as an autosomal incompletely recessive trait. The heterozygote population has factor‐X levels varying from 32% to 55% of normal and are usually asymptomatic.The term ‘Factor X Friuli’ is proposed for the abnormality, due to a locally common mutant gene.
Causes of Macroplania of Erythrocytes in Diseases of the Liver and Biliary Tract with Special Reference to Leptocytosis
Authors: Werre J. M. , Helleman P. W. , Verloop M. C. , Gier J. de 
Published: March 2008

Summary In diseases of the liver or biliary tract increased surface area (macroplania) is related either to macrocytosis or to leptocytosis. In a study on 20 patients with macroplania associated with leptocytosis, but without target cells, the following observations were made.

There was a positive correlation between changes in MCD and serum bilirubin.
There was a negative correlation between MCD and osmotic fragility.
There was a positive correlation between MCD and mean amount of cholesterol per erythrocyte and per μm2 of erythrocyte surface area, but there was no correlation between mean amount of cholesterol per erythrocyte and serum concentration of free or total cholesterol.
The osmotic fragility of normal erythrocytes introduced into the circulation of patients with leptocytosis became similar to the (diminished) osmotic fragility of the patients' own erythrocytes.

The order in which the changes occur is probably as follows: changes in the peripheral circulation→increase in the mean amount of cholesterol per erythrocyte and per unit of erythrocyte surface area→increase in mean erythrocyte surface area while the MCV remains normal→increase of MCD and diminution of MCT→diminution of osmotic fragility.
Observations on Folate Metabolism in Rats on a Vitamin B12 ‐Deficient Diet
Authors: Williams D. L. , Spray G. H. 
Published: March 2008

Summary.Unless they were starved, rats fed on a vitamin B12‐deficient diet had significantly higher levels of folate activity for Lactobacillus casei in the plasma than controls receiving the same diet supplemented with cyanocobalamin. The folate activity in the livers of the deficient animals was significantly lower than in the controls, whether the rats were fed or starved. There was a significant linear correlation between the concentrations of folate activity and vitamin B12 activity in liver.No consistent differences were found between the rates of disappearance of intravenously‐injected [5‐14C]methyl tetrahydrofolate in vitamin B12‐deficient and ‐supplemented rats. The latter results do not support the ‘methylfolate block’ hypothesis.
Ferritin Production in the Rat Small Intestine
Authors: Bernier George M. , Schade Stanley G. , Conrad Marcel E. 
Published: March 2008

Summary.Administration of iron to rats increases the amount of [14C]amino acid incorporation into ferritin in the rat intestine. Using a specific anti‐rat ferritin antiserum, several variables affecting de novo synthesis of ferritin in the rat intestine were studied. Synthesis was dose dependent in the range of 1–100 μmoles of oral iron with larger doses producing no greater effect. However, significant ferritin synthesis was evoked by a 150 μmole dose of oral iron in animals pretreated with similar doses of oral iron 3 and 6 hr before the test dose. Cobalt, a cation absorbed by the intestine in a fashion similar to iron, did not stimulate ferritin synthesis. Oral and parenteral iron administration produced different effects on various small intestinal segments. Oral iron induced synthesis chiefly in the proximal small intestine while intravenous iron produced it dominantly in the terminal ileum. These latter findings are consistent with the concept that ferritin functions chiefly as a barrier to excess iron absorption and as an important mechanism for iron excretion.
Milder Variant of Sickle‐Cell Disease in Arabs in Kuwait associated with Unusually High Level of Foetal Haemoglobin
Authors: Ali Syed Amir 
Published: March 2008

Summary Two families with a new form of relatively benign sickle‐cell disease in Arabs are described here. In the first family, born to AS parents, four children, all above the age of 10 years, had mild manifestation of the sickle‐cell disease. The haemoglobin pattern of these four children is that of SF with complete absence of HbA on the Hb electrophoresis. There was no evidence of thalassaemia minor in the parents. In the second family, two brothers with sickle‐cell disease showed on electrophoresis true SF pattern. Both were above the age of 12 and never needed blood transfusion before, except on recent admission of the younger brother to hospital. The elder brother was not anaemic and was free of symptoms though he has homozygous sickle‐cell disease.Haematological details of a few similar individual cases where family studies were not possible are also reported.
Globin Chain Synthesis in Sicilian Thalassaemic Subjects
Authors: Conconi F. , Bargellesi A. , Senno L. Del , Menegatti E. , And S. Pontremoli , Russo G. 
Published: March 2008

Summary. Globin synthesis has been studied in two homozygous β‐thalassaemic subjects, in two homozygous βs patients and in four subjects doubly heterozygous for the two β‐gene abnormalities. All the patients considered were of Sicilian origin. The results obtained can be summarized as follows:

Decreased β‐globin synthesis and excess of α‐globin synthesis was found in all the β‐thalassaemic subjects examined. This finding further suggests the existence of different genetic determinants for the Cooley's disease, directing the synthesis of different amounts of β‐globin in different thalassaemic populations.
Ratio of 3Hα to 3H counterpart globins was higher than 1 in the initial stages of the incubation, both in the total lysates and in purified haemoglobins. These ratios, after 8 hr of incubation, decrease to values lower than unity. These results suggest that the excess of newly synthesized α‐molecules flows initially from the polyribosomes to the haemoglobins, and then more slowly, to a ‘stromal’α‐pool.
Combination of the excess α‐molecules with Hb S in the double hetero‐zygotes considered. This combination indicates that the β‐thalassaemia and the βs genes are present within the same cellular clone.
THE SIGNIFICANCE OF GASTRIC ANTIBODIES
Authors: Fisher June M. , Taylor Keith B. 
Published: March 2008

Chronic inflammatory disease of the acid‐secretory gastric mucosa in man may be associated with the development of circulating antibodies directed against two distinct antigenic components of the gastric parietal cell. The first can be shown by immunofluorescent techniques to be present in the cytoplasm of this cell (Taylor et al, 1962). Immunoabsorption studies suggest that it is present in the microsomal fraction of gastric mucosal extracts and that it may be a lipoprotein (Baur et al, 1965). Immunolocalization using the electron microscope has recently suggested that it may be a component of the micro‐villi of the canalicular system of the parietal cell (Hoedemaeker & Ito, 1970).The other antigen is Castle's intrinsic factor (IF), a protein of mol. wt 55 000, which is produced by the parietal cell in man and some other mammals (Hoedemaeker et al, 1964; Fisher & Taylor, 1969) and which mediates the absorption of vitamin B12 in the distal small intestine.
The Autoxidation of Human Red Cell Lipids Induced by Hydrogen Peroxide
Authors: Stocks J. , Dormandy T. L. 
Published: March 2008

Summary.Malonyldialdehyde (MDA) formation, a measure of polyunsaturated fat autoxidation, was estimated in normal human red cells incubated in vitro. Exposure to oxygen under a variety of conditions did not induce autoxidation. Exposure to hydrogen peroxide was either by the addition of a hydrogen peroxide solution or by incubation in an atmosphere saturated with hydrogen peroxide vapour. A pattern of MDA formation was established with both methods. Lack of reproducibility, a feature of previous methods of measuring the susceptibility of red cells to exogenous peroxide, could be overcome by (i) catalase inhibition, (ii) attention to the non‐linear relation between packed‐cell volume and MDA formation, and (iii) elimination of potentially misleading coloured complexes on spectroscopy. A number of recognized antioxidants inhibited peroxide‐induced MDA formation. Inhibition was proportional to the logarithm of the antioxidant concentration. Under certain conditions pre‐incubation with nucleosides and with lecithin protected the cells against autoxidation on subsequent exposure to peroxide. Peroxide‐induced cell autoxidation was also influenced by plasma, bovine albumin, and ascorbic acid. Haemolysis was an invariable consequence of autoxidation. Similarities and dissimilarities between autoxidation and antioxidation in free lipid emulsions and in organized biological structures were examined and discussed in the light of the experimental findings.
Studies of Splenic Function in the Myeloproliferative Disorders and Generalized Malignant Lymphomas
Authors: Pettit J. E. , Williams E. D. , Glass H. I. , Lewis S. M. , Szur L. , Wicks C. J. 
Published: March 2008

Summary.The relative importance of splenic red‐cell pooling, sequestration and cell destruction in the causation of anaemia has been studied in 29 patients—16 with generalized lymphoproliferative disease, 12 with myeloproliferative disease and one with idiopathic autoimmune haemolytic anaemia.A scanning method with [11C]carbon monoxide was used for direct in vivo measurement of splenic red‐cell volume, and the spleen was delineated by‐a scan after injection of 81Rb‐labelled red cells, damaged with non‐radioactive 1‐mercuri‐2‐hydroxypropane (MHP). The clearance time of the damaged cells from the circulation was used as an index of splenic function. The fraction of red cells in the spleen varied from 2.9% to 32% and the splenic red‐cell volume ranged from 38 to 1000 ml. In patients with lymphoproliferative disorders their spleens contained a smaller proportion of red cells, relative to splenic size, than patients with myeloproliferative disease. Clearance of cells damaged with 1‐mercuri‐2‐hydroxypropane (MHP) was 30–60 min in normal subjects. Slow clearances were found in some patients with lymphosarcoma; fastest clearances occurred in patients with obvious haemolytic anaemia. No clear relationship was noted between the rate of clearance and splenic size or splenic red‐cell volume.
Serum Lysozyme and Vitamin B12 Binding Capacity in Myeloproliferative Disorders
Authors: Catovsky D. , Galton A. G. , Griffin Chantal , Hoffbrand A. V. , Szur L. 
Published: March 2008

Summary Estimations of serum lysozyme (muramidase), total B12 binding capacity (TB12BC) and folate were performed in 122 patients: 69 with myeloproliferative disorders (39, polycythaemia rubra vera (PRV); 10, chronic granulocytic leukaemia, Ph1‐chromosome positive (CGL); 20, myelosclerosis) and 53 with other disease which affect leucocyte production or turnover (17, monocytic leukaemias; 20, megaloblastic anaemia; 15, neutropenia; and a single case of red cell aplasia progressing to a myeloproliferative disorder).The highest serum lysozyme levels were found in monocytic leukaemia. Serum lysozyme concentrations were also elevated in myelosclerosis and to a lesser extent in CGL and in PRV. High levels in PRV occurred in patients developing myelosclerosis or with active disease, resistant to treatment. Significantly higher concentrations of lysozyme were present in patients with myelosclerosis following PRV than in patients with the primary form of the disease. Patients with myeloproliferative diseases and high lysozyme concentrations tended to have low serum folate levels.Slightly raised levels were found in about 50% of patients with megaloblastic anaemia. Moderately high serum lysozyme concentrations also occurred in patients with neutropenia and hypercellular bone marrows but not in those with hypocellular marrows.TB12BC concentrationswere increased in the myeloproliferative disorders, especially in CGL and to a lesser extent in myelosclerosis and least of all in PRV. The results suggest that serum lysozyme reflects granulocyte turnover (except in monocytic leukaemia) while TB12BC (providing liver function is normal) correlates closely with the total blood (and possibly marrow and splenic) granulocyte pool. The ratio between serum lysozyme and TB12BC proved to be a useful index of the balance between granulocyte turnover and total granulocyte pool in individual patients.
The Hereditary Transmission of Congenital ‘True’Hypoprothrombinaemia
Authors: Girolami A. 
Published: March 2008

SUMMARY. The family pedigrees of three patients with congenital ‘true’hypoprothrombinaemia are reported. By means of a specific prothrombin assay three groups of patients could be separated, namely: homozygote, heterozygote and normal.The one‐stage factor II level in the homozygotes varied between 9% and 16%. Similar levels were obtained by means of other prothrombin assays.The prothrombin level in the heterozygotes varied between 43% and 75% (average 58.1·9.7).The prothrombin level in the normal relatives of the homozygote patients varied between 84% and 130% (average 96.7·10.2).The prothrombin level in a group of normal subjects unrelated to the homozygotes varied between 86% and 115% (average 98.0·7.9).These data are compatible with the assumption that the disorder is transmitted as an autosomal incompletely recessive trait. The heterozygotes are usually asymptomatic but may have epistaxis, excessive bleeding after tooth extraction and menorrhagia.
Ultrastructural Localization of Iron in the Human Jejunum in Iron Overload (Bantu Siderosis)
Authors: Theron J. J. , Mekel R. C. P. M. 
Published: March 2008

Summary. Light and electron microscopical studies of intestinal biopsies from 22 patients with advanced Bantu siderosis revealed that ferritin‐iron was deposited in dense membrane‐bound ferritin bodies, in apical vesicles and in Golgi vesicles of the jejunal absorptive cells. Acid phosphatase was demonstrated within the ferritin bodies and it was concluded that these bodies were lysosomes. Ferritin molecules were also observed in the intercellular space between epithelial cells and cells of the lamina propria, and within the lumen of capillaries.Electron‐dense material, presumably ‘haemosiderin’ occurred in the Golgi cisternae of the concave face of the Golgi complex of jejunal epithelial cells. In the lamina propria, iron in the form of ferritin and haemosiderin was observed mainly in macrophages, but ferritin was also deposited within fibroblasts.
Prognosis in Hodgkin's Disease Related to the Lymphocyte Count
Authors: Swan H. T. , Knowelden J. 
Published: March 2008

Summary. Case notes of 328 histologically‐diagnosed patients with Hodgkin's disease have been studied in respect to sex, age of presentation, lymphocyte count in the peripheral blood and survival. Lymphocyte counts were accepted only if they had been made before the first course of radiotherapy or cytotoxic chemotherapy.A clear difference in prognosis has been demonstrated in three lymphocyte groups. The worst prognosis was associated with lymphocyte counts of less than 1000/μl in which group the proportion of patients surviving 5 yr was only 12%. The prognosis was better where the initial counts were between 1000 and 1500/μl, the 5‐yr survival being 26%, and was best where the lymphocyte counts were above 1500/μl, the 5‐yr survival being 35%.
Iron Kinetics in the Skin
Authors: Cavill I. , Jacobs A. 
Published: March 2008

Summary. The clearance of transferrin‐bound iron from the interstitial fluid of skin has been measured in normal and iron‐deficient subjects. It is possible to interpret the results by means of a two‐compartment model and to calculate the rate of entry and exit of iron in skin cells. There is a decrease in the amount of iron entering skin cells in patients with iron‐deficiency anaemia.The demonstration of an intracellular exchangeable iron pool in the skin suggests that there may also be large parenchymal pools in other organs, which are indirectly in equilibrium with the plasma pool.
Erythrocyte Delta‐Aminolaevulic Acid Dehydrase Activity in Anaemia
Authors: Battistini V. , Morrow J. J. , Ginsburg D. , Thompson G. , Moore M. R. , Goldberg A. 
Published: March 2008

Summary. A method is described for measuring the activity of the enzyme δ‐aminolaevulic acid dehydrase (ALA‐dehydrase) in the erythrocytes of normal subjects and of patients with macrocytic anaemia due to vitamin‐B12 and folic‐acid deficiency, iron‐deficiency anaemia, lead poisoning and the secondary anaemias of rheumatoid arthritis and carcinomatosis.A significant increase in pre‐treatment enzyme activity was found in the macrocytic anaemias, whether due to a deficiency of vitamin B12 or folie acid. A further rise of ALA‐dehydrase activity occurred in close relationship with the reticulocyte response, returning to pre‐treatment levels as their numbers fell. Enzyme activity returned to normal in 2 mth.Enzyme activity was significantly diminished in lead poisoning. No significant difference from normal was found in iron‐deficiency anaemia, or in the secondary anaemias of rheumatoid arthritis and carcinomatosis.Enzyme assay in the erythrocytes of bled rabbits showed that the enzyme activity was directly related to the number of reticulocytes present.
Absorption of Vitamin B12 in the Guinea‐Pig III. THE FORMS OF VITAMIN B12 IN ILEAL MUCOSA AND PORTAL PLASMA IN THE FASTING STATE AND DURING ABSORPTION OF CYANOCOBALAMIN
Authors: Peters T. J. , Linnell J. C. , Matthews D. M. , Hoffbrand A. V. 
Published: March 2008

Summary. The forms of vitamin B12 in guinea‐pig ileum, liver and portal plasma were studied using a chromatographic‐bioautographic technique. 5’Deoxyadeno‐sylcobalamin was the principal cobalamin in each tissue although significant amounts of methylcobalamin were detected in plasma. An unidentified cobalamin was also found in all three tissues.During the absorption of labelled cyanocobalamin a proportion of this analogue was converted to 5’deoxyadenosylcobalamin in the ileum; preliminary experiments suggest that the site of inter‐conversion was the mitochondria of the ileal epithelial cells. A significant proportion of the cyanocobalamin was transferred to the portal plasma unchanged however, indicating that the mitochondrial localization of vitamin B12 during its absorption, shown in previous studies, is not solely related to the conversion of vitamin B12 analogues from one form to another.
Cytomegalovirus Infection in Blood Donors—A Prospective Study
Authors: Perham T. G. M. , Caul E. O. , Conway P. J. , Mott M. G. 
Published: March 2008

Summary. One hundred and ninety‐five blood donors for 10 patients who had open‐heart surgery and fresh blood transfusion were screened for cytomegalovirus (CMV). Complement‐fixing antibody to CMV was present in 55%, but no CMV was isolated. The 21 blood donors, for two of the 10 patients who became infected with CMV post‐operatively, were recalled, but there was no evidence of recent infection with CMV.Of the 10 patients, three died and could therefore not be studied in the post‐operative period. Of the remaining seven patients, one excreted the virus pre‐ and post‐operatively, and three acquired CMV post‐operatively, after receiving fresh blood with complement‐fixing titres to CMV ranging from 1/8 to 1/128. None of the three non‐CMV infected patients had antibody to CMV; one received CMV antibody‐free fresh blood and the other two received fresh blood with titres ranging from 1/16 to 1/64.These results are discussed and, though numbers are small, the question of using blood within 36 hr of donation should be carefully reconsidered because of the risk of cytomegalovirus transmission.