bluuebunny/crossref_metadata_2025_split huggingface dataset:
Erythrocytosis in Emphysema
Authors: FREEDMAN B. J. , PENINGTON D. G. 
Published: March 2008

Marked erythrocytosis is frequently seen in hypoxia due to high altitudes and cyanotic congenital heart disease (Hurtado, Merino and Delgado, 1945) but is relatively uncommon in association with chronic respiratory failure (Baldwin, Cournand and Richards, 1949). This discrepancy has exercised a number of writers. Wilson, Borden and Ebert (1951) have proposed that bronchial infection may depress the erythropoietic response of the bone marrow in patients with chronic bronchitis and emphysema and Ratto, Briscoe, Morton and Comroe (1955) have postulated depression of erythropoiesis by carbon‐dioxide retention. Simpson (1957) found evidence of bleeding in a number of his emphysematous subjects and suggested that concealed haemorrhage may be of importance in modifying haematological changes in respiratory hypoxia.More recently, Shaw and Simpson (1961) and Lertzman, Israels and Cherniack (1962) have proposed that expansion of plasma volume accompanying respiratory failure may account for the apparent infrequency of erythrocytosis in emphysema, but the view that the erythropoietic response to hypoxia is normal in emphysema is based on evidence which is not entirely satisfactory. Thus Shaw and Simpson compared the red‐cell mass in English subjects with emphysema with that of native Peruvian Indians living at high altitudes, a comparison which assumes similarity of nutrition and incidence of conditions such as intestinal infestation. Lertzman and associates compared the red‐cell mass in respiratory failure with that in patients with hypoxia due to other causes. In the majority of these latter patients, the hypoxia was due to obesity but the red‐cell mass was expressed per kilogram of body weight rather than as a function of height or lean‐body mass. In each of these studies the red‐cell mass in emphysema varied from within the normal range to more than double this figure in patients with hypoxia of comparable severity. The fact that many chronically cyanosed emphysematous patients show no significant increase in red‐cell mass remains unexplained and warrants further attention.In the present investigation a small group of severely disabled patients was chosen for study. All suffered from chronic bronchitis and emphysema with persistent cyanosis during remission from acute pulmonary infection. Four exhibited the conventional haematological changes of secondary polycythaemia, whilst another four were similarly disabled but showed no such changes in the peripheral blood. The two groups were compared in an attempt to account for the apparent failure of erythropoietic response to hypoxia in the latter. The investigation was designed to ascertain whether the defect lay in the elaboration of erythropoietin, the humoral mediator of the hypoxic stimulus to the bone marrow, or in the response of the bone marrow to this humoral stimulus.
The Relation of Alkali‐Resistant Haemoglobin in Thalassaemia and Abnormal Haemoglobin Syndromes to Foetal Haemoglobin
Authors: Sturgeon P. , Schroeder W. A. , Jones R. T. , Bergren W. R. 
Published: March 2008

Alkali‐resistant haemoglobin (haemoglobin F or foetal haemoglobin) normally constitutes the major haemoglobin component of the foetus; it is also present in gradually decreasing amounts through the first year of life. In the older individual, less than I per cent of the haemoglobin is of the foetal type; normally, most of the remaining haemoglobin is the adult variety—haemoglobin A.Alkali‐resistant haemoglobin is found in greater than normal concentrations in several blood dyscrasias. In patients with thalassaemia major, it is invariably increased and frequently constitutes the major haemoglobin component (Fessas, 1959; Beaven, Ellis and White, 1961). On the other hand, in those who are homozygous for an abnormal haemoglobin, 20 per cent or less of the haemoglobin, as a rule, is alkali‐resistant and increased percentages are not invariably found (Beaven et al., 1961; Rucknagel and Neel, 1961). The abnormal haemoglobin syndromes are most characterized by the presence of electrophoretically abnormal haemoglobin(S), although alkali‐resistant haemoglobin may be present. Neither in thalassaemia major nor in thalassaemia minor, despite the frequently increased quantity of haemoglobin A2 (Fessas, 1959), have electrophoretically abnormal components been found. Both the thalassaemia and abnormal haemoglobin syndromes, have common clinical, haematological, biochemical, genetic and anthropological features. It has not seemed unlikely that thalassaemia is based on a genetically determined abnormality similar to that resulting in the abnormal haemoglobin syndromes; it is assumed, however, that a structurally modified haemoglobin is electrophoretically undetectable (Itano and Pauling, 1961).Because an electrophoretically abnormal haemoglobin is absent in thalassaemia, one might consider that the alkali‐resistant haemoglobin is, in fact, the abnormal haemoglobin. This subject has been reviewed by Beaven and Gratzer (1959) who cite abundant biochemical and immunological evidence to the contrary. Nevertheless, recent publications report biochemical and immunological differences in the foetal haemoglobin of thalassaemia compared to that from the normal newborn (Derrien, 1959; Diacono and Castay, 1959). Studies on the structure of alkali resistant haemoglobin in thalassaemia and abnormal haemoglobin syndromes, however, have not been available.Structurally, haemoglobin A is composed of a pair of α chains and a pair of β chains. In haemoglobin F, γ chains occur instead of β chains. From genetic evidence, the production of the three types of chain appears to be under the control of sets of allelic genes at separate loci; this subject has been reviewed recently (Rucknagel and Neel, 1961). The abnormal haemoglobins are products of mutant genes at the α and β chain loci. A single amino acid
Platelet Clumping in vitro
Authors: Mitchell J. R. A. , Sharp A. A. 
Published: March 2008

Osler (1874), Hayem (1878) and Eberth and Schimmelbusch (1886) showed that the process of blood clotting initiated platelet clumping in shed blood, and later workers (Roskam, 1922; Zucker and Borrelli, 1955; Bergsagel, 1956; Bounameaux, 1957) have shown that various coagulation factors released during the clotting process can produce this platelet clumping. There has, however, been a growing body of evidence to suggest that other mechanisms, not directly related to clotting, could also initiate platelet clumping (Sharp, 1958, 1961), and the existence of one such mechanism was confirmed by the observation that a protein‐free, heat‐stable, dialysable extract of human red cells could increase platelet adhesiveness (Hellem, 1960). Hellem called the active principle of his extract ‘Factor R’ fractionation of the extract showed that the active agent was adenosine diphosphate (ADP) and it was found that when this substance was added to platelet‐rich citrated plasma in concentrations as low as 0.02 μg./ml., platelet clumping was produced (Gaarder, Jonsen, Laland, Hellem and Owren, 1961).We have studied this clumping phenomenon in vitro, tested other substances for clumping activity and searched for materials which would modify the process. This paper reports the results of these studies.
Haemophilia, Christmas Disease and the Xg Blood Groups
Authors: Harrison J. F. 
Published: March 2008

In1954 the Medical Research Council and the Ministry of Health jointly asked various clinics and laboratories to act as reference centres for patients with hereditary bleeding diseases. In the following study, the register at one such centre has been used to trace families who might help determine the degree of linkage between haemophilia and Christmas disease and the X‐borne Xg blood groups. Information has also been obtained on the incidence of the two diseases.Mann, Cahan, Gelb, Fisher, Hamper, Tippett, Sanger and Race (1962) reported the discovery of the Xg system. Two alleles, Xgaand Xg, are so far known; the antigen Xgais a dominant character and occurs in about 66 per cent of the male and 88 per cent of the female white population. Accounts have now appeared of attempts to measure linkage between Xg and colour blindness (Jackson, Symon and Mann, 1962), glucose‐6‐phosphate dehydrogenase (G6PD) deficiency (Adam, Sheba, Sanger, Race, Tippett, Hamper, Gavin and Finney, 1963), Duchenne muscular dystrophy (Clark, Puite, Marczynski and Mann, 1963), and haemophilia and Christmas disease (Davieset al., 1963). Lindsten, Fraccaro, Polani, Hamerton, Sanger and Race (1963) used the Xg blood groups to study 14 patients with an isochromosome for the long arm of the X, and suggested, with certain reservations, that the deutan colour blindness and Xg loci both lie on the short arm of the X chromosome.
The Marrow Reserve of Eosinophils
Authors: Hudson G. 
Published: March 2008

The quantitative methods developed in this laboratory (Yoffey, 1960) have already been used to demonstrate the large ‘reserve’of mature and almost mature eosinophils in normal bone marrow and to study changes which it undergoes in the foreign protein response (Hudson, 1960, 1963). These studies indicated that large numbers of eosinophils are discharged from the marrow reserve during the first 24–30 hours following the re‐exposure of a sensitized guinea‐pig to foreign protein. Further experiments showed that the marked increase in the level of circulating eosinophils, which is usually observed in these circumstances, can be prevented by the administration of hydrocortisone, and the question arose of the mechanism by which cortical hormones could exert such an effect. Quantitative studies have been carried out in an attempt to throw light on this, and the results are the basis of the present communication.It was noted that Samter, Erickson and Kofoed (1952) and Speirs (1958) had observed that ACTH and cortisone may depress or inhibit the peripheral eosinophilia in animals during the foreign protein response, and that Essellier and Wagner (1952) had reported no changes in the percentage of marrow eosinophils in patients in whom a high‐grade peripheral eosinophilia had been abolished by ACTH. There appeared, however, to have been no previous quantitative studies of the bone marrow in such circumstances.
Familial Pyridoxine‐Responsive Anaemia
Authors: Bourne M. S. , Elves M. W. , Israëls M. C. G. 
Published: March 2008

Pyridoxine‐Responsive anaemia as a naturally occurring disease in man was first described by Harris, Whittington, Weisman and Horrigan in 1956. Since then 26 further cases have been described in the literature. Most reports concern single cases and suggest that pyridoxine‐responsive anaemia is not a single disease entity but rather a syndrome with variable aetiology. In only three instances could the disease have been inherited.We report here details of a family in which six members suffered from pyridoxine‐responsive anaemia. We have investigated 83 members of the family and studied in detail the six affected members.
Studies on Desferrioxamine in Relation to the Absorption of Iron
Authors: Bannerman R. M. , Malpas J. S. 
Published: March 2008

In Conditions of iron overload, and particularly primary haemochromatosis, any agent which would prevent or diminish the absorption of iron in the diet might play a useful part in therapy.When desferrioxamine was first introduced it was suggested that this chelating agent, which has a very strong affinity for iron, might prevent the absorption of iron from the diet.The present studies were designed to investigate the effect of desferrioxamine on the absorption of inorganic iron and haemoglobin iron labelled with 59Fe. In addition the fate of labelled ferrioxamine given by mouth was studied.
Red‐Cell Enzymes and Co‐Enzymes in Non‐Spherocytic Congenital Haemolytic Anaemias
Authors: Loder P. Bronwen , De Gruchy G. C. 
Published: March 2008

The non‐spherocytic congenital haemolytic anaemias were classified on haematological grounds by Selwyn and Dacie (1954) into two main types—Type I and Type II. It now seems that Type I cases probably form a heterogeneous group while the Type II cases are probably a homogeneous group (de Gruchy, Santamaria, Parsons and Crawford, 1960). Robinson, Loder and de Gruchy (1961) presented evidence of impairment of cell metabolism in both Type I and Type II cases and suggested that in Type II cases there was an enzymatic defect in glycolysis beyond the stage of 2,3‐diphosphoglycerate (2,3‐DPG) synthesis. This was confirmed by the demonstration by Tanaka, Valentine and Miwa (1962) of a pyruvate kinase (PK) deficiency in Type II cases.This paper reports observations in a number of patients with Type I or Type II non‐spherocytic haemolytic anaemia on (a) the activities of the enzymes of the Embden–Meyerhof glycolytic pathway, (b) the activities of the enzymes glucose‐6‐phosphate dehydrogenase and glutathione reductase, (c) the values of the co‐enzymes diphosphopyridine nucleotide (DPN) and reduced diphosphopyridine nucleotide (DPNH) and (d) the values of reduced glutathione. The significance of the findings is discussed together with their relevance to the classification of these anaemias.
Lymphography with Radioactive Contrast Medium in Lymphosarcoma and Reticulosarcoma
Authors: Chiappa S. , Galli G. , Palmia C. , Severini A. 
Published: March 2008

Since 1961 we have been using a method of lymphography based on the intralymphatic injection of an oily contrast medium (Lipiodol Fluide) rendered radioactive by replacement of the inactive iodine by its isotope 131I (Chiappa, Galli, Barbaini and Ravasi, 1962; Ratti, 1962; Buraggi, D'Amico and Fava, 1963). The method has recently been taken up by other workers (Seitzman, Wright, Halaby and Freeman, 1963).The method combines lymphadenographic investigation with elective radiotherapy of tumours located in the lymph nodes. The most promising results have been achieved in the treatment of systemic lymph node diseases, such as Hodgkin's disease, lymphosarcoma and reticulosarcoma (Chiappa et al., 1962; Chiappa, Galli, Guarino, Barbaini and Ravasi, 1963a; Chiappa and Galli, 1963). In a preliminary review of the case material treated at the Institute of Radiology (Chiappa, Galli and Severini, 1964), we reported the diagnostic and therapeutic results obtained in Hodgkin's disease.The purpose of the present paper is to summarize our experience of lymphadenography with radioactive Lipiodol in lymphosarcoma and reticulosarcoma. We have treated 14 cases of lymphosarcoma, 18 cases of reticulosarcoma and three cases in which the histological diagnosis was lymphoreticulosarcoma. Five cases of lymphatic leukaemia, leukaemic and non‐leukaemic, have been included with the lymphosarcoma patients, because of the resemblance between the two in morbid anatomy, clinical course and lymphadenographic findings. The series thus totals 40 patients.Although the various diseases are histologically and clinically different, they have certain lymphographic features in common: (a) the topographic distribution of the lymph node tumours within the area studied and, even more important, (b) the similarity of their response to irradiation with Lipiodol F131I.
Pyridoxine‐Responsive Anaemias
Authors: Gehrmann G. 
Published: March 2008

Patients with sideroblastic anaemia do not respond to iron or other common haematinics. Since the first report by Harris, Whittington, Weisman and Horrigan (1956) on ‘Pyridoxine‐Responsive Anemia in the Human Adult’, 30 additional cases have been reported in the literature (Gehrmann, 1963).The problem presented by these pyridoxine‐responsive anaemias is underlined by the fact that not all patients with sideroblastic anaemia respond to the administration of pyridoxine, and that in some patients with pyridoxine‐responsive anaemia the effect of treatment is incomplete whereas in others full regression of all abnormalities follows.These different responses to the administration of pyridoxine and certain distinguishing clinical and laboratory features suggest that at least two miscellaneous groups of pyridoxine‐responsive anaemias exist. The available data suggest that some of these patients are actually deficient in pyridoxine and this allows us to distinguish between ‘pyridoxine‐deficiency anaemia’ and so‐called pyridoxine‐responsive anaemia. All the reported cases in the literature can be classified into these two groups. Furthermore, it seems justified to distinguish within the group of pyridoxine‐responsive anaemias cases of either hereditary (‘anaemia sideroblastica hereditaria’) or acquired (‘anaemia refractoria sideroblastica’) origin.Features common to both pyridoxine‐deficiency and pyridoxine‐responsive anaemia are: (1) severe hypochromic anaemia with a dimorphic blood picture and a low reticulocyte count; (2) hyperferraemia and haemosiderosis; (3) maturation arrest of the erythroblasts with sideroblastosis (‘ring’ sideroblasts); (4) an increased iron disappearance rate but a decreased red‐cell uptake; (5) a slightly decreased or a normal red‐cell survival time; (6) absence of an abnormal haemoglobin, and (7) at least some correction of anaemia after the administration of pyridoxine.Present knowledge of these two groups will be discussed in the present report and supplemented by personal observations. Patients with pyridoxine‐responsive anaemia which develops during the course of isoniazid treatment will be excluded, since this drug can induce true pyridoxine deficiency by forming a water‐soluble iso‐nicotinyl hydrazone complex (Williams and Abdulian, 1956).
Neutrophil (Leucocyte) Alkaline Phosphatase in Paroxysmal Nocturnal Haemoglobinuria
Authors: Lewis S. M. , Dacie J. V. 
Published: March 2008

The alkaline phosphatase activity of the peripheral blood neutrophils has been studied extensively during the past 10 years, especially in leukaemia and allied disorders. A deficiency in paroxysmal nocturnal haemoglobinuria (PNH) was noted by Beck and Valentine (1951). This observation has been confirmed and it has been suggested that it is a characteristic feature of the disease (Hartmann and Auditore, 1959; Tanaka, Valentine and Fredricks, 1960; Lewis and Dacie, 1962).In the present study neutrophil (leucocyte) alkaline phosphatase has been determined in a relatively large number of patients with PNH in order to assess the constancy of the defect and the relationship between the neutrophil alkaline phosphatase activity and the severity of the disease.PNH is frequently associated with impaired marrow function, manifested by moderate pancytopenia. In a few cases severe, and possibly fatal, marrow failure may occur during the course of PNH. More commonly aplastic anaemia is the dominant feature at the onset while PNH becomes manifest only at a later stage (Dacie and Lewis, 1961). It is convenient to consider this ‘aplastic‐PNH’ syndrome separately from typical PNH, although during the PNH phase the disease is no different. Six patients with this syndrome have been studied and the neutrophil alkaline phosphatase results have been compared with those in typical PNH and in aplastic anaemia.
The Measurement of Iron Absorption by the Double Isotope Technique
Authors: Pitcher C. S. , Williams H. S. , Parsonson A. , Williams R. 
Published: March 2008

Theprinciple of the double isotope technique for measuring iron absorption is to inject one isotope,59Fe, intravenously and give the other,55Fe, orally at the same time (Saylor and Finch, 1953). The59Fe is used to determine the percentage of plasma iron that is used for haemoglobin synthesis and it is assumed that the percentage utilization of the absorbed55Fe will be the same. From the relative amounts of the two isotopes present in the red cells at 10 days, it is possible to calculate the percentage of the oral55Fe absorbed. For the counting of the two isotopes in blood samples, the iron must be separated from the blood by digestion and precipitation followed by electroplating onto metal planchettes. The technique as originally described and as used by subsequent workers had a number of disadvantages. The various types of electroplating apparatus have proved unreliable in use or are complex in construction and the calculations have depended on the accurate recovery of known amounts of haemoglobin iron from blood samples (Hallberg and Brise, 1960; Brise and Hallberg, 1962). This is not easy to achieve in practice. The counting methods devised, including the use of gas flow counters and filters, have in our experience variable efficiencies.The present report describes a relatively simple and reliable technique which has proved suitable for both single and serial measurements of iron absorption. The normal range of absorption by this method has been defined using a group of control subjects carefully screened to exclude mild unsuspected iron deficiency. Also reported is a comparison of the results obtained by this technique in a number of patients with widely differing absorption rates in whom iron absorption was also determined simultaneously by stool counting and by whole body counting.
A Study of the Coagulant Properties of Malayan Pit‐Viper Venom
Authors: Chan K. E. , Rizza C. R. , Henderson Marie P. 
Published: March 2008

The commonest cause of snake‐bite in Northern Malaya is the Malayan pit‐viper (Ancistrodon rhodostoma), more than 300 cases being recorded each year. In a study carried out by Reid, Thean, Chan and Baharom (1963), 39 per cent of the victims suffered systemic poisoning, the outstanding feature of which was a prolonged defect of blood coagulation due to complete or partial disappearance of fibrinogen from the blood (Reid, Chan and Thean, 1963).This paper describes in vitro experiments carried out to learn more about the action of the Malayan pit‐viper venom on the blood coagulation process and on the blood coagulation factors.
Fibrinolysis in Haemophilia: The Effect of ε‐Amino‐Caproic Acid
Authors: Mainwaring Dorothy , Keidan S. E. 
Published: March 2008

In 1962 Abe, Sato, Kazama and Matsumura reported that two boys suffering from haemophilia had improved during treatment with ε‐amino‐caproic acid (ε‐A.C.A.) as shown by a diminished number of haemorrhages and decreased need for blood transfusions and that this improvement was associated with reduced fibrinolytic activity.In order to assess the effect of ε‐A.C.A. in haemophilia, a trial has been conducted in six boys over a period of 6 months. They were all suffering from haemophilia A and their previous histories were available. During the trial they were seen at fortnightly intervals and on each visit a full record of all haemorrhagic episodes since the last visit was made, together with any subjective comments from the patient or his parents about pain, mobility, etc. At each visit a specimen of venous blood was taken for estimation of fibrinolytic activity, the patients attending at the same time of day in order to minimize physiological diurnal variation (Fearnley, 1960). Any changes in diet, activity or habits during the preceding 24 hours were also noted. Fibrinolytic activity was measured by the method of Lackner and Goosen (1959) (which is a modification of that originally described by Fearnley, Balmforth and Fearnley, 1957). During the first 3 months half of the patients were on treatment with ε‐A.C.A. and during the second 3 months the other half. The dosage was calculated by the percentage method (Catzel, 1962) based on the daily adult dose of 10 g. per day. No treatment was given during the control period.