ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02542.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

I should like to present this review in memory of the late Professor Erik Jorpes. Born on Kökar in the Åland archipelago, Erik Jorpes was our mentor when we began working on von Willebrand's disease; and his interest, his support and enthusiasm, were a splendid encouragement to u

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02543.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02544.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Since chronic disease compounds the difficulties of child rearing and increases the incidence of psychological problems in all age groups, patient care emphasizing technology alone is inadequate. Instead, a biopsychosocial approach is necessary to correlate medical care with vital information about the patient's psychological and social state. Hemophilia acts as a chronic recurrent stress on the individual and his family requiring the development of coping behaviors for adaptation. While many hemophiliacs achieve normal psychological development, some are overwhelmed by the illness contributing to maladaptation and a variety of psychological disorders. Accordingly, medical staff efforts and attitudes that promote effective coping are a vital part of comprehensive care. The goals of coping include carrying out acceptable life goals despite illness handicaps, keeping uncomfortable feelings within manageable limits and the maintenance of a positive self‐image. Efforts to support effective coping begin with effective medical care including adequate relief for acute pain, educational programs that promote optimal child rearing and self‐care and the opportunity for the patient and family to talk about concerns and feelings. Comprehensive care also provides for the identification and specific treatment of psychiatric disorders that may deve

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02545.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

To live with hemophilia presents many long‐term psycho‐social stresses, in addition to the painful and often immobilizing physical symptoms. Among the psychological stress factors are the impact on the family of raising a hemophilic boy, the patient's coping with frequent hospitalizations often involving infusions and orthopedic care, patient and his family learning the aspects of home care, and the common uncertainties relating to the hemophiliac's social adjustment and educational and professional future. These aspects of the life of hemophiliacs will be presented by the use of an open systems model which allows for delineation of the many interrelationships of the hemophiliac which have to be considered by the comprehensive care team. The maturation of the mind of a young hemophiliac, that is his cognitive functions, and the existence of stable family functioning and open communication within the family and with the health care team are essential allies in promoting a healthy psychosocial development of the hemophil

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02546.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

The sexual consequences of chronic pediatric disease are only now beginning to be studied. Most clinicians do not yet have the conceptual tools to thoroughly assess the sexual life of young adult hemophiliacs. This paper presents five major landmarks of sexual development: gender identity, orientation, intention, function (desire, arousal, orgasm, emotional satisfaction) and the enabling relationship. Several hypotheses about the impact of hemophilia are described. First, the illness identity of the person with chronic disease threatens to supercede the masculine gender identity. Second, an unappreciated high prevalence of orientation and intention problems lurk behind the high frequency of heterosexual “shyness”. Suggestions to maximize the sexual developmental potential of young hemophiliacs are provided based on our current concepts of child development and sexual development in particu

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02547.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

The possibility of preventing some major familial maladjustment following the diagnosis of severe haemophilia in the frame of free discussion group was explored. Five couples, 3 with a familial history of haemophilia and 2 without met 5 times over a 2 year period with 3 staff members for a full day. Transcripts of tape recorded sessions were analyzed. Parents with no familial history were apparently more affected but had a more dynamic approach towards hemophilia than the others. They benefited more from the program. Four of the five families were able to treat their child at home before 4 years of age. This new approach seems to help parents to adjust to this severe chronic disease and staff members to reconsider their interaction with parents.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02548.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

The aim of this research study was firstly to investigate the implications of the psycho‐social aspects of haemophiliacs/carriers in Greece and secondly to understand human nature and thus be able to handle haemophiliacs' and carriers' anxieties and fears.The design and methodology of the study was an exploratory and descriptive one. The total membership was a random sample of hundred haemophiliacs and hundred carriers. Only 89 and 75 respectively answered. Two questionnaires were administered with a veiw to obtain the groups' reactions and attitudes to various psycho‐social aspects affecting their personal life and environment. The questionnaires consisted of open‐ended and closed questions. The findings from this study suggested that haemophiliacs experience pain, parental anxiety, stress in the family and interference with family plans in a more realistic and objective way. This attitude could be a result either from a genuine awareness of their disease or from the psychology of defending oneself through denial. With regard to carriers it was found that similar psycho‐social aspects had significant signs of exaggeration in terms of fear and anxiety. This could be attributed to the inappropriateness of their defensive reaction. The evaluation of the responses in the light of the actual situation, confirms the above results with an even greater emphasis on ten

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02549.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Blood was drawn after the intranasal administration of 325 μg DDAVP from altogether 420 blood donors at 5 blood banks in Sweden. No significant side‐effects were observed. The DDAVP plasma was pooled in two separate batches. Matched pools of control plasma from the same donors were also prepared. From each pool a separate batch of high purity factor VIII concentrate (Octonativ) was produced.The yield of VIII:c in the DDAVP batches was 2.6 and 1.5 times higher than in the corresponding control batches. The specific activity was 2.8 for the two DDAVP batches and 2.0 IU/mg protein for the control batches. Thein vivorecovery was about 100 per cent for all the batches when examined in 5 patients with severe haemophilia a. The individual half‐lives (late phase 5–48 hours) varied between 7 and 17 hours. No significant differences between the batches derived from the DDAVP and the control plasma could be detected.Thus, intranasal DDAVP seems to be a safe and practicable means of improving the production of factor vIII concen

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02550.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Although a number of different processes are used to prepare factor VIII concentrates from human plasma the recovery of coagulant activity is almost always very low.A number of loss mechanisms can be identified and these tend to be associated with large‐scale manufacture, where pools of hundreds or thousands of litres of plasma are processed. Many of the procedures which result in loss are common to virtually all of the different manufacturing processes and considerable improvement in yield is possible, particularly if attention is given to careful process engineering design.For the preparation of an intermediate‐purity concentrate the most important points are considered to be:• Provision of a good quality plasma feedstock.• Rapid processing, with no delays from initiation of plasma thawing to freezing of the final concentrate.• Continuous thawing of plasma to allow rapid thawing with precise control of temperature.• Effective centrifugation for cryoprecipitate collection.• The use of low shear agitators and careful control of pH adjustment.• In‐process control of ionised calcium concentration.Further processing to prepare concentrates of increased purity also results in significant loss of factor VIII and new higher yielding methods are needed if high

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb02551.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY