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In the case of a postoperative joint infection, the orthopaedic surgeon is frequently blamed. Certain intrinsic disease processes, however, make a joint more susceptible to infection. In these three reports of septic polyarthritis, all patients had underlying systemic disorders, including rheumatoid arthritis, diabetes mellitus, and hemophilia A. Two of the three patients had had no recent surgical procedures. This suggests that the development of a pyarthrosis may depend at least as much on the patient's underlying systemic condition as on the surgeon and his or her technique.
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PMID:Septic polyarthritis and its relation to systemic disease processes. A report of three cases. 324 Nov 75

A 17-year-old boy with hemophilia A and strong inhibitors for factor VIII suffered a myocardial infarction on the sixth day of treatment with large amounts of human factor IX complex (Konyne). The patient also had had diabetes for one year, for which he was receiving insulin injections, but there was no evidence of vascular disease. Thromboembolic complications that occur after the administration of factor IX concentrates have been reported in patients with hemophilia B or liver disease, but these complications are extremely rare in patients with classic hemophilia.
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PMID:Myocardial infarction after factor IX therapy. 678 56

Medical investigation in Chile is beginning to apply molecular biology methods as diagnostic tools and in studies establishing the prevalence of pathologies with Mendelian or multifactorial genetic origin. In this way, we are getting nearer to the strategies used worldwide for these purposes. Two studies published in this journal, one about hemophilia A where an analysis of intron-18 and intron-7 of the Factor VIII gene was applied as strategy for the diagnosis of carrier state in a Chilean population, and the other one about insulin-dependent diabetes mellitus in Santiago, Chile, with an analysis from immunogenetics to some environmental risk factors, constitute good examples about this integration.
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PMID:[Use of molecular biology techniques in clinical research, in Chile]. 923 14

Atherosclerosis is a multifactorial disease caused by genetic and environmental factors with important clinical sequelae. The aim of this study was to evaluate the degree of carotid atherosclerosis by echo-color Doppler scan in a group of patients affected by hemophilia A and von Willebrand disease versus a group of normal subjects apparently free of atherosclerotic risk factors. All coagulopathics and normal patients who came to our Internal Medicine Department (Padua Hospital) underwent physical exam, blood analysis, standard electrocardiogram, chest x-ray, echo-color Doppler scan, and a thorough history. We examined 156 subjects, 76 coagulopathics (46 men, 30 women) and 77 normals (37 men, 40 women). Coagulopathics were affected by hypertension in 28.9% of cases, diabetes mellitus in 6.5%, dislipidemia in 17.1%, smoke in 39.4%, and obesity in 36.8% (p < .05). Echo-color Doppler scan revealed carotid plaques in 27.2% of control patients versus 13.1% of coagulopathics (p < .05). Hemophilics and subjects with von Willebrand disease with a more serious illness had fewer plaques than those with lighter defects. Coagulopathics showed 23.6% of the plaques we revealed on the whole, versus 76.3% of control subjects (p < .01), with a lighter degree of stenosis (p < .01). Our data demonstrate that patients with hemophilia A and von Willebrand disease have fewer carotid plaques and a smaller degree of carotid stenosis than normal subjects of the same sex and age. These data seem to strengthen the hypothesis that blood coagulation defects may allow protection against carotid atherosclerosis and its sequelae.
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PMID:Do hemophilia A and von Willebrand disease protect against carotid atherosclerosis? A comparative study between coagulopathics and normal subjects by means of carotid echo-color Doppler scan. 1072 19

Specific features of dilatation tracheostomy (DTS) were studied in 45 patients with hemorrhagic syndrome; 37 of them had thrombocytopenia; 6 patients had thrombocytopenia and disseminated intravascular coagulation (DIC); and 2 patients had congenital coagulopathy. Besides, there were patients, among the examined ones, with hemoblastosis (36), with idiopathic thrombocytopenic purpura (1), with aplastic anemia (1), with HELLP-syndrome (1), with diabetes mellitus (1), with rheumatoid arthritis, with stomach cancer (2) and with hemophilia A (2). Commercial sets were made use of for DTS ("Portex"). DTS can be safely made provided the blood platelets' level is not below 40-50 x 10(9), and the time of bleeding is below 3 min; in hemophilia, the procedure is possible after the administration factor's preparations; and in DIC--after plasma transfusion and after ensuring the normal coagulation. Obturation of lumens of the trachea and bronchi with blood grumes is an often complication in DTS, which makes it necessary to make bronchoscopy immediately after DTS.
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PMID:[Dilatation tracheostomy in patients with hemorrhagic syndrome]. 1291 1

Haematuria is common among persons with haemophilia (PWH), but its long-term effects on the kidney and renal function are not well defined. In addition, infection with human immunodeficiency virus (HIV) or hepatitis C, or exposure to nephrotoxic agents as therapy for these infections may place PWH at increased risk for renal disease. To examine factors associated with chronic renal disease (CRD) and acute renal disease (ARD) in PWH, we analysed data collected from the medical records of 3422 males with haemophilia living in six US states from 1993 to 1998. Renal disease cases were ascertained from among 2075 persons who were hospitalized at least once over the 6-year period. Of these, 60 (2.9%) were diagnosed during one or more hospitalizations with either ARD (29/60) or CRD (31/60). In multivariate analyses, we examined associations between renal disease and demographic and clinical factors including age, race, haemophilia type and severity, hypertension, diabetes, history of recent renal bleeds, presence of an inhibitor, and infection with hepatitis C or HIV. HIV infection and hypertension were strongly associated with both ARD and CRD. PWH who had ARD were also more likely to have an inhibitor than those without this diagnosis. PWH who had CRD were more likely to be older and non-white and to have had a recent admission for a kidney bleed than those without diagnosed CRD. In summary, we found that HIV infection and haemophilia-related factors including inhibitors and kidney bleeds were associated with renal disease in a cohort of males with haemophilia.
Haemophilia 2003 Nov
PMID:Renal disease among males with haemophilia. 1475 Sep 36

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

Prophylaxis with von Willebrand factor (VWF)-containing concentrates is considered to be a potential approach for patients with von Willebrand disease (VWD) and severe bleeding tendency. We report the case of a 57-year-old man with type 3 VWD and a history of recurrent melaena. Bleeding frequency and severity had progressively increased and the patient showed chronic anaemia and persistent haemoglobin in the stools. Endoscopic examinations revealed multiple vascular mucosal abnormalities (MVA) of the stomach and large bowel. Photocoagulation of some actively bleeding lesions and octreotide did not significantly affect his clinical conditions: six red cell transfusions and >400 000 IU of intermediate-purity factor VIII (FVIII) concentrate (Haemate P) on-demand were needed during 2002. Prophylaxis with Haemate P 40 IU kg(-1) (102 IU kg(-1) VWF:RCo) thrice weekly was associated with improvement of his mean haemoglobin levels, cessation of clear-cut melaena and red cell transfusions and reduction of total Haemate P requirements (-20% over 2003-04). Prophylaxis with Haemate P is still ongoing without any adverse event over a 30-month period. Clinical course and pharmacokinetic evaluations led to administer Haemate P each 72-96 h. Possible vascular complications were excluded by a careful clinical follow up, as the patient suffered from arterial hypertension and diabetes mellitus; thrombophilic abnormalities were previously excluded and no signs of abnormal coagulation activation or FVIII:C levels >150% were detected thereafter. Long-term prophylaxis with Haemate P has been shown to be safe, effective (also in terms of quality of life) and cost saving in this patient with severe gastrointestinal bleeding due to MVA and VWD.
Haemophilia 2006 Jan
PMID:Long-term prophylaxis with intermediate-purity factor VIII concentrate (Haemate P) in a patient with type 3 von Willebrand disease and recurrent gastrointestinal bleeding. 1640 82

The major focus of care for patients with haemophilia is to ensure health with minimal joint dysfunction. As this population ages, additional coexisting conditions can develop including rare instances of nephrotic syndrome in haemophilia B inhibitor patients undergoing immune tolerance, hypertension, diabetes, and coronary artery disease, all of which can adversely affect the renal system over time. In haemophilia patients, co-infected with HIV and hepatitis C, these conditions can also increase the risk of renal problems resulting in the need for dialysis. This article provides a practical approach for the haemophilia patient who requires dialysis and outlines the decision making process to ensure a positive outcome. The goal of care is to optimize dialysis treatment without increasing the bleeding risk.
Haemophilia 2009 Jan
PMID:Dialysis in the haemophilia patient: a practical approach to care. 1878 42

The older generation of patients with haemophilia still has musculoskeletal problems which limit activities and participation. One important aspect of male aging is the changes in sexuality. Sexual desire can be disturbed by fatigue, low testosterone or pain. Sexual excitement (erection) may be influenced by diabetes mellitus, arteriosclerosis, hypertension and side effects of antihypertensive and antiviral medication. Sexual response problems can be caused by antidepressant medication. In aging haemophiliacs arthropathy, iliopsoas muscle bleeding, chronic hepatitis C and HIV medications influence sexuality. The haemophilia care professionals should communicate proactively, give information on various practical aspects of sexuality (suggest suitable positions, recommend painkillers, reflect on prescribing erection-enhancing medication, refer to a sexology expert).
Haemophilia 2009 Jan
PMID:Haemophilia, aging and sexuality. 1914 47


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