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From the viewpoint of internal medicine, the author deals with risks encountered in the dental treatment of certain patients. Special attention is paid to local anaesthetics and certain risk factors such as chronic ischaemic disease, pacemaker, hypertension, and circulatory lability. Furthermore, haemorrhages are treated with special regard to haemophilia. The group of the diabetics requires consideration. The significance of endocrine diseases, allergies and vegetative disorders should not be underrated.
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PMID:[Internistic risk patients in dental treatment]. 31 May 90

Classical sex-linked hemophilia (Hemophilia A) has been described as due to deficiency in the synthesis of Factor VIII procoagulant activity (VIII:C). The availability of immunological techniques provided the means of identifying Factor VIII-Related Antigen(VI-IIR:Ag) detectable by rabbit antibodies to F VIII, which is distinct from VIII:C detected by human anti-F VIII available from multitransfused patients. Hemophilia A is lacking in VIII:C but not VIIIR:Ag. Recently, a third function of the F VIII "complex" was discovered with the help of ristocetin (von Willebrand's Factor, VIIIR: RCo). This activity is reduced in von Willebrand's syndrome. Estimation of the titers of VIII:C and VIIIR:Ag provides a method for more accurate detection of hemophilic carriers. Newly available chromogenic substrates perhaps will give rise to more simplified assays of VIII:C. The development of cryoprecipitates and stable lyophilized concentrates of F VIII has greatly simplified and intensified maintenance therapy, and has opened a new era in treatment. Prophylactic therapy has been shown to be very helpful in certain "high risk" cases. The impact and benefits of home care and self-administration has been tremendous. However, the varying quality of cryoprecipitates and the high cost of more purified concentrates are still stumbling blocks in treatment regimes. Other problems exist. Spontaneous bleeding, especially central nervous system bleeding, account for the majority deaths by haemorrhage. Inhibitor kinetics have been well characterized. It is clear that there exists "low" and "high" responders. For the "high" responders, plasmapheresis, immunosuppressives and the infusion of Factor IX concentrates have been utilized with varying success. The prevention of hemophilic arthropathy and its progression by maintenance therapy seems to be still inadequate. The results of trials with more vigorous regimes are awaited. The complications of therapy still remain to be solved. Apart from the well-known complications wuch as hepatitis, haemolytic disease and F VIII inhibitors, the existence of previously unnoticed complications as splenomegaly, hypertension, renal disease and paradoxal bleeding have been recently realized. The role of altered fibrinogen, fibrin degradation products (FDP) and unclassified fibrinogen derivatives (UFD) present in cryoprecipitates and F VIII concentrates in the above complications needs to be further clarified. In conclusion, tremendous progress in various aspects of hemophilia has been achieved in developed countries. Comprehensive care can now be carried out in various centers. On the other hand, developing countries still face a number of basic problems. The concept that hemophilia is a "manageable" disease and that chronic crippling and death from exsanguination can be prevented, should be disseminated widely by various means...
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PMID:Recent advances in hemophilia. 52 46

Percutaneous transcatheter arterial embolization was performed in a case of severe haemophilia A to control haemorrhage secondary to renal trauma. The treatment proved to be life-saving. Eighteen months follow-up revealed no evidence of hypertension, renal failure or infection.
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PMID:Transcatheter embolization in a haemophiliac with post-traumatic renal haemorrhage. Report of a case. 52 2

One hundred hemophiliacs were examined at a formal comprehensive health care clinic. Sixty-eight percent had abnormal results of liver function tests, and 26% had spleens that were palpable. Measurement of range of motion of knees, ankles, hips, shoulders, and elbows showed a high incidence of hemophilic arthropathy and established a precise baseline by which to judge efficacy of therapy. Results of dental examination disclosed a 14% incidence of multiple severe caries, which is an incidence lower than that of the population as a whole. Examples of inadequate dosage of replacement therapy (16%) and chronic delay in application of self-therapy (14%) were discovered. An 8% incidence of hypertension was noted; prior experience suggests that the combination of hypertension and hemophilia may be lethal. Other clinical and laboratory data also illustrate the importance of a periodic, formally structured, comprehensive examination of hemophiliacs.
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PMID:Comprehensive health care clinic for hemophiliacs. 93 70

Sixty four cases of retinal and vitreous hemorrhages are reported during a 15 months prospective study in Bamako. Main diseases associated with hemorrhages are high blood pressure (56% of cases), hemoglobinopathies (33%) and diabetes mellitus (23%). In 28% of cases several aetiologies are connected. SC hemoglobin is a frequent aetiology of vitreous hemorrhage (40%). Hemoglobin AS and AC, generally asymptomatic, are also liable to hemorrhages. Terson and Eales syndromes, Werlhof disease, hemophilia and AIDS are most uncommon. In 8% of cases there is not any aetiology.
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PMID:[General causes of retinal and vitreous hemorrhages in Mali]. 181 88

By means of the Central Card Index of Haemophilia, a working material of the Section of Haemophilia in the Society of Haematology and Blood Transfusion of the GDR, 52 patients with heriditary haemorrhagic diatheses could be registered. These persons had died between 1962 and 1984. The average age of death amounted to 33.8 years. With 23 cases intracranial bleedings were the dominating cause of death in all haemophiliacs. From this fact the absolute necessity arises to initiate a substitutive therapy with a sufficiently high dosage, if there is an assumption of a craniocerebral trauma and, in addition, to treat hypertension in such a way that normotonic values are achieved. Even retrospectively no signs of an acquired immunodeficiency syndrome could be found in any of the patients. As haemophilic bleedings apparently represented the life limiting factors in the majority of the deceased, the importance of an early adequate substitutive therapy is stressed, the principles of which should be known to all general practioners.
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PMID:[Causes of death in hemophilia patients in East Germany]. 241 12

Eight hundred and fifty two cases, who were introduced from the Dental Hospital to Department of Internal medicine, Medical Hospital at Niigata, The Nippon Dental University, were examined for medical complications. About one hundred and fifty cases were introduced per year from the Dental Hospital, which consisted of about 15% of the total outpatients in the Department of Internal medicine. The age distribution showed a high incidence of patients in their 50's. The number of patients over 60 years old was 220 (25.8%), and the number of patients over 65 years old was 160 (18.8%). There was then a high percentage of elder patients. The reason for the initial medical examinations was the need for a pre-operative medical examination in 42.6% of the total cases. Concerning medical complications among the patients, the highest incidence was hypertension in 116 cases (13.6%); the second highest was hepato-biliary and pancreatic diseases in 114 cases (13.4%); and heart diseases in 81 cases (9.5%). Eighteen cases of HBs antigen positive (2.1%) were seen among the patients with liver diseases. Among those with blood diseases, anemia showed the highest incidence, and a small number had leukemia and hemophilia. Diabetes mellitus showed up in 39 cases (4.6%), most of them being more than 40 years of age. Mentally and physically handicapped patients accounted for 30 cases. Half of them were from the pre-operative examination area, like tooth extraction.
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PMID:[Clinico-statistical study of medical complication in dental patients]. 253 46

The modern, comprehensive care of patients with hemophilia requires an awareness that complications other than those caused by acute hemorrhage can occur. The use of newer, more potent plasma concentrates has been accompanied by an increased incidence of liver disease in transfusion-requiring hemophiliacs. The progression to chronic active hepatitis and cirrhosis are particularly ominous developments in these patients. There is also a high incidence of urinary tract abnormalities in hemophiliacs, though the long-term consequences of these abnormalities are unknown. Furthermore, it must be remembered that urinary tract disorders unrelated to hemorrhage, such as nephrolithiasis, tumors, and nephritis, can occur in patients with hemophilia and may be mistaken for hemorrhage. Finally, hypertension occurs more frequently in patients with hemophilia than in the general population and may in part contribute to the occurrence of bleeding within the central nervous system. Methods for evaluating and treating these various disorders are discussed. Greater awareness of these potentially treatable medical complications will improve further the quality of care in hemophilia.
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PMID:Medical complications of hemophilia. 676 70

Until recently, infectious diseases and malnutrition-related disorders constituted the major cause of ill health and mortality in the world population. However, advances in treatment of such disorders and increased understanding of the molecular basis of heredity have led to genetically transmitted conditions becoming a major cause of morbidity and mortality. Several disorders, including chromosomal (Down syndrome, Turner syndrome), single-gene (sickle-cell disease, thalassaemia, glucose-6-phosphate dehydrogenase deficiency, haemophilia, inborn errors of metabolism) and multifactorial disorders (coronary artery disease, arteriosclerosis, diabetes mellitus, hypertension, obesity) are common and becoming increasingly important. As there is no agreed-upon definitive cure with acceptable risk, these disorders are a significant burden on the health care delivery system. This is because the chronic nature of genetic diseases requires lifelong medical attention, expensive supportive and symptomatic therapy and specialist care. This review outlines the genetic disorders, their impact on health care delivery systems and the general framework required to prevent and control these disorders.
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PMID:Spectrum of genetic disorders and the impact on health care delivery: an introduction. 1192 97

A 47-year old patient who had been suffering from hypertension and chronic renal failure for many years developed progressive extensive haemorrhagic erosions of the mouth within 3 months and less severe erosions of the genital and nasal mucosa. Additionally, subcutaneous haematomas developed spontaneously. Laboratory investigations demonstrated circulating antibodies against factor VIII while direct and indirect immunofluorescent microscopy showed discrete tissue-bound and circulating IgG reactive with the epidermal basement membrane in a pemphigoid-like fashion. Immunoblot analysis of the patient's serum revealed an "atypical" IgG reactivity against a central portion of the extracellular domain of the BP180 antigen. These findings were unexpected, since the clinical aspect showed striking resemblance to (paraneoplastic) pemphigus. The patient developed life-threatening complications. Eventually, reduction of circulating autoantibodies by a combination of plasmapheresis and subsequent immunosuppressive therapy led to a stable remission of both autoimmune bullous skin disorder and acquired haemophilia.
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PMID:[Simultaneous onset of pemphigoid and factor VIII antibody hemophilia]. 1213 99


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