Assessment of haemophilia treatment practice pattern in Japan.

The current status of replacement therapy for haemophilia and the role played by nurses in Japan were investigated at 16 facilities (17 specialties) each providing care to 20 or more haemophilia A or B patients without inhibitor. The questionnaire was mailed to the nurse or physician in charge of haemophilia at each facility in August 2006, asking the nurse to fill in the questionnaire. Responses were collected on 1318 patients (haemophilia A: 1078 patients; haemophilia B: 240 patients). About 70% of these patients were reported to be severe haemophilia A or B. Overall, 26% were receiving regular prophylaxis while 74% received on-demand therapy with or without temporary prophylaxis before special events. The percentage of patients receiving primary prophylaxis was only 2%. The percentage of adherence to prophylaxis decreased with age (lowest at age 19-29) but this percentage for each age group in Japan was higher than that in the western countries. Of the nurses working at the facilities surveyed, 88% considered prophylaxis as an optimal therapy for severe haemophilia patients, although the percentage of patients receiving prophylaxis for the entire population surveyed was lower than that in the western countries. The main factor precluding introduction of prophylaxis was 'difficulty in venous access' for infants and small children. On the other hand, 'unwillingness of family members' and 'poor adherence' were the main factors precluding introduction of this therapy for those aged over 6 years.

Comprehensive care for hemophiliacs--activities of North Kyushu Hemophilia Center at the University Hospital of Occupational and Environmental Health, Japan.

One of our objectives in establishing the Hemophilia Center was to create a model of a total care system for chronic diseases which develop in the childhood and continue through adulthood. We also believe that the comprehensive medical care system should be introduced not only for the care of hemophiliacs but also for those with every chronic refractory disease. It may be a surprise that in a university hospital, which is likely to lack cooperation between departments, this kind of cross sectional support system, even extending to activities outside the hospital, has been continued. This success owes much to the understanding and consideration by successive Directors of the Hospital and the Professors (Directors) of every department, the enthusiastic efforts by members of the Center who have continued their volunteer work for the purpose of improving the QOL of patients, and the cooperation by students of the Volunteer Study Club. We also have to mention the advice and guidance by Dr. Minoru Inagaki (now at the National Children's Hospital) who established the first substantial hemophilia center in Ogikubo Hospital, and Dr. Kaneo Yamada, present Visiting Professor in the Faculty of Medicine, St. Marianna University. We would like to thank every one who has supported the activities of the Center. Finally, we strongly hope that this total care system will become popular among those responsible for caring of patients with other chronic refractory diseases.

[Benefits and problems of home infusion therapy].

A survey was performed to clarify the benefits and problems of home infusion therapy because 8 years had passed since this type of treatment was officially approved in Japan. Questionnaires were sent to hemophiliacs and the physicians in charge of hemophiliacs in the Kyushu district. The main results obtained from analysis of responses concerning 197 patients were as follows. 1) Out of 197, 140 patients were on home therapy programs. 2) The number of bleeding episodes was increased in 6.8%, decreased in 51.1% and unchanged in 42.1% of patients after the start of home infusion therapy programs. 3) The severity of bleeding symptoms was reduced in 92.9% of patients after the start of these programs. 4) After the start of home infusion therapy programs, the amount of blood products administered increased in 24.4%, decreased in 20.6% and was unchanged in 55.0%. 5) Complications such as abdominal pain, headache, ulticaria, itching, shivering, fever and discomfort were reported from 19 hemophiliacs. Only one of them visited the hospital due to severe abdominal pain which appeared immediately after home infusion of blood product. 6) It was indicated that better education or re-education of home infusion therapy is necessary for hemophiliacs and/or their families who are on home infusion therapy programs, because half of them had not received proper education concerning home infusion.

[A case of long-standing isolated traumatic tricuspid regurgitation with remarkably dilated right cardiac chambers and pancytopenia].

We report a rare case of tricuspid regurgitation due to nonpenetrating chest trauma 33 years previously. A 79-year-old man suffered a blunt trauma due to a piece of wood at work in 1958. He suffered multiple rib fractures on the right side and was admitted. Since then, he began having shortness of breath on exertion and was treated with medication. The patient was transferred to the Division of Cardiology, Hakodate National Hospital in 1984. A chest x-ray film revealed a marked cardiomegaly. Cardiac catheterization showed severe tricuspid regurgitation. Hepatomegaly and pancytopenia was observed. He was readmitted because of general fatigue in July 1991. Two-dimensional echocardiography demonstrated systolic excursion of septal and posterior tricuspid leaflets with ruptured chordae tendineae into the right atrium, and a remarkably enlarged right ventricule, right atrium and vena cava interior. Cardiac catheterization was performed. The right atrial pressure-wave form resembled the right ventricular pressure recording (ventricularization of the atrial pressure). Right ventricular cineangiography revealed severe tricuspid regurgitation, grade 4. Laboratory data showed pancytopenia. Thrombocytopenia progressed (3 x 10(4)/mm3), and a hemorrhagic tendency developed. The liver edge was palpable 4 finger breadths below the right costal margin. Pancytopenia due to congestive hepatomegaly and hypersplenism would have complicated this case.

Counselling for HIV-positive haemophiliacs in Japan.

This paper describes the current status of counselling for HIV-positive haemophiliacs at a haemophilia centre in Japan. Clinical experience with 23 HIV-infected haemophiliacs (all male, age range 16-56, mean 29.9 years), 22 family members (eight couples of parents, one grandmother, five siblings) and nine sexual partners (five wives and four girlfriends) between April 1990 and February 1994 is detailed. Methods of counselling consisted of individual counselling in 32, couple counselling in eight and family counselling in three cases. Indications for counselling consisted of fear of or shame of HIV infection in 37, concern for the family in 21, risk of transmitting HIV to sexual partners in 12, fear of death and dying in 12, future career and family life in seven, interest in treatment options in five, daily life style in 10, parents' feeling of guilt over the infection in five cases, lack of knowledge of haemophilia, HIV infection and AIDS in seven, and bereavement in two cases. A total of 200 counselling hours were provided. Consequently, pending disclosure of HIV infection was successfully completed in five, disclosure of HIV infection to the sexual partner by the patient was facilitated in two, the patient-family relationship improved in nine, and smooth transition of care to the adult in-hospital unit was achieved in two patients. Our experience in Japan clearly verifies the importance of psychoeducational counselling for HIV-positive haemophiliacs and their family as well as sexual partners.

Flow effects on cultured vascular endothelial and smooth muscle cell functions.

Cultured vascular endothelial cells were exposed to fluid shear stress by means of a rotary-disc shear-loading device, and the physiological effects of the conditioned medium (CM) and the homogenate (HM) of the cells on migration, adhesion and growth of endothelial cells (EC) or smooth muscle cells (SMC) were studied. Effects of shear stress on the production and secretion of collagen, one of the extracellular matrices of EC, were also studied. CM stimulated the adhesion and growth of SMC, but not of EC themselves. The ability to stimulate SMC adhesion and growth was similar in CM obtained from the static and shear-loaded cells. HM of the shear-loaded EC stimulated SMC migration. Further, HM of the shear-loaded EC contained increased amounts of collagen compared with the static EC. These results suggest that: 1) EC produce and secrete accelerators for the adhesion and growth of SMC, 2) EC react to the physical stimulus of fluid shear stress to produce stimulators of SMC migration, and 3) EC produce collagen, the production of which is enhanced by fluid shear stress.

[A case of spontaneous regression of malignant lymphoma, complicated with miliary tuberculosis, liver dysfunction and pancytopenia].

An 82-year-old woman was admitted to Iwamizawa Rosai Hospital, Cardiovascular Medicine, for detailed examination of an aneurysm of abdominal aorta. CT scan revealed marked swelling of para-aortic lymph nodes which regressed spontaneously in three months. She was re-admitted to our hospital because of general malaise and gait disturbance. Her chest X-ray on the second admission revealed miliary disseminated shadows, which were confirmed to be tuberculous granuloma by lung biopsy. She was initially well controlled with anti-tuberculosis drugs, followed by severe liver dysfunction and pancytopenia, and died of respiratory and cardiac failure. At autopsy, wide-spread malignant lymphoma and miliary tuberculosis of the lung were found. The cause of liver dysfunction was strongly suspected to be due to infiltration of the lymphoma cells to portal triads of the liver. Hemophagocytosis found in the bone marrow, spleen and liver might be consistent with clinically so-called "hemophagocytic syndrome", causing pancytopenia, associated with infection.