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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine cases have been presented in detail to illustrate some of the varied causes of sudden neurological deficit in childhood: arteriovenous malformation, cryptic hamartoma, berry aneurysm, mycotic aneurysm, intraspinal arteriovenous malformation, brain tumor, migraine, arteritis, and multiple sclerosis. The Boston Children's Hospital experience with aneurysms and intracranial arteriovenous malformation has been summarized. It is noteworthy that a cutaneous hemangioma overlay one cranial and one intraspinal arteriovenous malformation. One small but deep cerebral arteriovenous malformation apparently destroyed itself after its second hemorrhage. Not only have multiple sclerosis and a brain tumor mimicked a vascular lesion, but a series of vascular accidents was misdiagnosed first as multiple sclerosis then as a thalamic tumor. The many possible causes of childhood strokes has been thoroughly cataloged in the Report of the Joint Committee for Stroke Facilities in 1973 (11). Children may be more susceptible to strokes because of congenital abnormalities such as congenital heart disease, hemophilia, and sickle cell anemia, or by diseases which more commonly occur in this age group, such as leukemia. The likelihood of brain abscess in cyanotic congenital heart disease is stressed. Arteriographic studies in our series have been safe; however, there have been reports of probable worsening of symptoms in children with multiple cerebral occlusive lesions in the presence of homocystinuria.
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PMID:Strokes in children. 98 45

The Baltimore-Washington Infant Study, an epidemiologic investigation of congenital heart disease, searches for genetic and environmental risk factors. Among 2,102 infants with heart disease, 17.5% had a noncardiac abnormality of chromosomal or genetic origin, whereas among 2,328 control infants, only 0.7% had a genetic abnormality. Familial cardiovascular malformations encountered can be grouped into five distinct etiologic mechanisms. Single gene effects may be responsible for the specific histologic and biochemical changes in familial atrial septal defect with conduction disturbance and also in idiopathic ventricular hypertrophy. Left heart lesions showed familial concordance by the presumed morphogenetic mechanism of abnormal embryonic blood flow with phenotypes of varying severity. Pulmonary stenosis appeared with familial heritable disorders, as well as a partially concordant lesion with tetralogy of Fallot. Ventricular septal defect with transposition of the great arteries (one sibling pair) and with truncus arteriosus (two sibling pairs) indicate forme fruste expression of conotruncal defects. Endocardial cushion defect occurred with and without Down's syndrome in members of three families, suggesting inheritance of a defect affecting cellular migration. Heritable blood coagulopathies occurred in case families and not in control families. The associated of hemophilia and transposition, observed also by others, is extremely unlikely by chance and suggests genetic errors of endothelial cell function. The description of specific families from a population-based study emphasizes biologic questions on the nature of the inheritance of cardiovascular malformations.
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PMID:Congenital cardiovascular malformations: questions on inheritance. Baltimore-Washington Infant Study Group. 276 23

Over a period of nine years we observed 52 children with acute neurological symptoms which were caused by a cerebrovascular disease. Fourteen patients had congenital vascular malformations, most frequently AV-angiomas (9 patients). A Sturge-Weber-Syndrome and a venous angioma were found in two cases and one patient had an aneurysm of the middle cerebral artery. Thirty-eight patients had acquired cerebrovascular diseases such as ischaemic infarctions (22), intracranial haemorrhages without vascular malformations (14) and thromboses of the dural sinus (2). The cerebral infraction was a complication of a congenital heart disease in 8 children, two others suffered from chronic renal insufficiency and were on haemodialysis. Two children had a trauma of the internal carotid artery and in one patient a large haemorrhagic infarct was caused by hypernatremic dehydration. In 9 patients (6 females, 3 males) no obvious aetiology of the infarct could be found. However, in most of these cases a nonspecific febrile illness preceded the neurological manifestations. The thrombosis of the dural sinus occurred in a 6-week old previously healthy infant and in a 3-year old boy as a complication of a nephrotic syndrome. Intracranial haemorrhages (without cerebrovascular malformations) occurred in 14 patients, mainly as a complication of haematological diseases (acute lymphatic leukaemia, severe aplastic anaemia, haemophilia A, lupus erythematodes). Four children had spontaneous intracerebral haemorrhages without obvious causes. The prognosis for survival was good in children with infarcts, but persisting neurological deficits were more severe than in children with haemorrhages. At the acute stage the lethality was higher in children with intracranial haemorrhages.
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PMID:[Cerebrovascular diseases in childhood--etiology, clinical aspects and prognosis]. 395 16

A survey of 875 disabled children in Norway aged 0-19, representing ten different disabling conditions, was carried out between January 1976 and December 1978. Parents of the disabled children were interviewed, medical records studied and the children examined. Mother's age, level of education, presence of disabled siblings, spouse's education and profession as well as emergency situations related to the disabled child's condition appeared to be factors influencing the mother's health and therefore inevitably the family's ability to cope with the situation. Social insurance seemed to have been granted in a rather haphazard way; only families of children suffering from hemophilia, mental retardation, spina bifida and cerebral palsy seemed to have received fairly adequate social insurance benefits. Families of children suffering from juvenile rheumatoid arthritis, asthma, congenital heart disease and epilepsy had received less social insurance assistance than those in the other groups. One-parent families had received more social insurance than others. Families with children who were totally dependent on their parents, who had several diagnoses or had spent much time in hospital, had also been granted more social insurance. Welfare benefits distributed by local authorities had mainly been given to families who were also receiving social insurance benefits and to families of children with brain damage. Almost half of all families expressed needs for welfare benefits which had not been met. Thus, there seemed to be an underconsumption of both social insurance and welfare benefits, particularly among some diagnostic groups.
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PMID:Aspects of living conditions among groups of disabled children and their families in Norway: family situation, mothers' health, financial assistance. 622 33

This paper describes a successful cardiac operation in a young boy with hemophilia, congenital heart disease, severe factor VIII deficiency, and an acquired high titer antibody to factor VIII. To our knowledge, there have been no published cases of elective cardiac operations in a person with severe hemophilia and an accompanying complex problem. Utilizing the team approach, we administered a megadose bolus of factor VIII concentrate preoperatively (eight times the calculated dose), followed by a continuous intravenous infusion at 500 units/hr throughout the procedure and at a reduced dose for the first 5 postoperative days. With the anamnestic rise in factor VIII antibody on day 5, activated prothrombin complex concentrates were substituted for factor VIII and provided continued adequate hemostasis during the remaining 9 postoperative days. The rapid infusion of large quantities of factor VIII was effective in neutralizing the low titer inhibitor and providing normal hemostasis during the procedure. In addition, activated prothrombin complex concentrates were substituted for factor VIII coagulant without recurrent bleeding or thromboembolic phenomena.
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PMID:Elective cardiac operation in a patient with severe hemophilia and acquired factor VIII antibodies. 642 12

A 52 year-old man, with rheumatic cardiopathy and hemophilia was admitted to the hospital for aortic valve replacement and mitral valve commisurotomy. He had a history of allergic reaction to cryoprecipitate, and to undergo to the cardiac surgery, the ideal level of factor VIII was estimated to be 100%. To reach this level he first received 2,500UI of the factor VIII:C increasing the factor VIII:C level from 20 to 58%. During the surgery he received an additional dose of 1,500UI of factor VIII plus 3,500,000UI of aprotinin (Trasylol), which was started at the beginning of the anaesthesia and maintained during the surgical procedure, increasing the factor VIII level to 220%. The patient was discharged 11 days after the surgery without any hemorrhagic complication.
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PMID:[Chronic rheumatic cardiopathy in a patient with of hemophilia successfully treated with surgery]. 761 17

At the Department of Acquired Cardiac Diseases (Head is Prof. G. I. Tsukerman), A. N. Bakulev Institute of Cardiovascular Surgery, 145 patients underwent operation for ascending aortic aneurysms, out of them the Bentalle-De Bono and the Cabrol operations were performed in 81 and 25 patients, respectively, the remaining 39 patients had palliative interventions. From February 1991 to April 1993, the clinic performed in 11 patients prosthesis of the ascending aorta with a xeno-pericardial conduit containing a bioprosthesis of the same tissue. The paper describes the technical features of bio-conduit implantation. The use of xeno-pericardial conduits facilitates the technique of applying anastomoses, lowers hemophilia and reduces the length of an operation. Hemorrhagic and thromboembolic events are expected to be reduced in late periods. At the hospital stage 1 patient died. The cause of his death was peri-coronary hematoma. Ten patients are still under observation. Repeated examinations have not detected no specific complications typical of those observed in the application of synthetic conduits. The first experience has proved to be quite promising. However, the final assessment is possible only when sufficient clinical materials are accumulated and late outcomes are studied.
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PMID:[Clinical applications of xenopericardial conduits in surgery of ascending aortic aneurysms]. 814 70

The aim of this study was to investigate the views of General Dental Practitioners (GDPs) regarding their provision of dental treatment for medically compromised children. A questionnaire to assess confidence, experience and willingness to treat eight specific groups of medically compromised children was sent to 524 GDPs. Information is based on 271 completed questionnaires. The median number of children treated by GDPs in each of the eight groups over the previous 5 years was 0-2. Eighty percent of respondents stated that they would value further training in the provision of dental care for medically compromised children. Confidence was highest in providing dental treatment for children with three conditions: congenital heart disease (37% very confident), diabetes (39% very confident) and epilepsy (41% very confident). These were also the conditions that the GDPs reported as presenting most frequently in the dental surgery. GDPs reported least confidence in providing dental care for children with haemophilia (12% very confident) and organ transplants (14% very confident). Only 30% of GDPs wanted to be routinely involved with the provision of dental care for medically compromised children. The results indicate that medically compromised children may have problems accessing dental care and expertise. A system of integrated medical and specialised dental care is suggested.
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PMID:Provision of dental care for medically compromised children in the UK by General Dental Practitioners. 1131 Feb 46

Physical activity is a key component of a healthy lifestyle. Exercise and physical activity have been shown to help maintain a healthy body weight, reduce stress, increase self-esteem and feelings of wellbeing, control blood pressure, and prevent heart disease and diabetes. Children with haemophilia may feel restricted from competing in sports through parental concern or pain and difficulty in moving, or they may rebel against such restrictions, thus leaving themselves open to serious injury. Several groups have attempted to classify sports activities with regard to the level of risk involved; however, these are not consistent. It is important to match the child's abilities with the sport in which they want to take part, and suggest alternatives if this is not possible. Prevention of injury should not depend solely on use of factor concentrates.
Haemophilia 2004 Oct
PMID:Risks and benefits of sports and fitness activities for people with haemophilia. 1547 91

There have been conflicting reports in the literature about the protective effect of hemophilia on the occurrence of ischemic heart disease (IHD). Circulatory disease has been reported as the second most common cause of death in persons with hemophilia in the United States. In addition to diabetes and hypertension, high levels of FVIII, as may occur during factor concentrate infusions, may increase IHD risk in this population. To estimate the prevalence of heart disease and examine factors associated with IHD and other heart diseases among persons with hemophilia, we analyzed data collected from the medical records of 3,422 males with hemophilia living in six U.S. states from 1993 to 1998. Heart disease cases were ascertained from among 2,075 persons who were hospitalized at least once during the 6-year period. Of these, 48 were diagnosed with IHD and 106, with other types of heart disease. The age-specific prevalence of IHD ranged from 0.05% in those under 30 years to 15.2% in those 60 years or older. Hospital discharge rates in males with hemophilia with IHD and other types of heart disease were lower compared to rates in age-matched U.S. males. In our cohort, as in the general population, IHD was independently associated with age, hypertension, diabetes, and hyperlipidemia. Other heart diseases were associated with HIV infection, hypertension, hemophilia B, and diabetes. In summary, persons with hemophilia have unique risk factors such as infusion of factor concentrates and infection with HIV that may predispose them to heart disease as their life expectancy increases.
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PMID:Prevalence and risk factors for heart disease among males with hemophilia. 1584 61


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