The impact of severe haemophilia on the social status and quality of life among Austrian haemophiliacs.

Although many studies of the impact of haemophilia on the quality of life were conducted, there is hardly any data on the social status of haemophiliacs. It was the aim of our study to obtain data on the social status of Austrian haemophiliacs and to compare these with an age- and sex-matched reference population. Furthermore, we collected data on the quality of life of haemophilia patients. We conducted a case-control study in two Austrian haemophilia centres with 53 patients (mean age 36.7 +/- 10.6 years) and 104 male controls (mean age 36.7 +/- 11.1 years). Socio-demographic data were collected using a standardized questionnaire and quality-of-life data using the SF-36. More patients (56.6%) than controls (37.5%) were married (P = 0.023), whereas more controls (17.3%) than patients (3.8%) had a partner with whom they were not married (P = 0.016). The percentage having children was equivalent in both groups (47% and 41% respectively), but controls had more children (mean number 1.5 in patients and 2.1 in controls, P < 0.007). A greater number of patients was unemployed (34% of patients, 9% of controls, P < 0.001) as well as retired (23% and 4% respectively; P < 0.001). Patients had worse scores regarding physical functioning, role-physical, bodily pain and general health (P < 0.001), whereas vitality, social functioning, role-emotional and mental health were similar in both groups. Despite their disability, most of the Austrian haemophiliacs share a sound family environment. This suggests that they are highly capable of coping with their chronic disease and is indicated by good scores for role-emotional and mental health.

Assessment of anti-inflammatory activity of Poria cocos in sodium lauryl sulphate-induced irritant contact dermatitis.

OBJECTIVE: In the present study, we evaluated the anti-inflammatory activity of Poria cocos (PoCo) on experimentally induced irritant contact dermatitis (ICD) in a repeated sodium lauryl sulphate (SLS) irritation model. METHODS: The anti-irritative effect of PoCo was evaluated with a visual score and quantified by non-invasive bioengineering methods, namely chromametry and transepidermal water loss. Three concentrations of PoCo in base cream DAC (amphiphilic emollient; German pharmacopoeia) were tested in a 4-day repetitive irritation test using SLS. RESULTS: A statistically significant anti-inflammatory activity was observed for PoCo by all three methods when applied in parallel to the induction period of ICD. Application of PoCo after induction of ICD once a day for 5 days, starting just at the end of 4 days, was without any effect. CONCLUSION: An anti-inflammatory efficacy of PoCo on the elicitation phase of the ICD induced by repeated SLS test could be observed and quantified by three independent, non-invasive biophysical assessment parameters. This effect can be explained by its influence on pro-inflammatory enzymes, namely phospholipase A2.

Pure myopathy associated with a novel mitochondrial tRNA gene mutation.

The authors describe a 47-year-old man who presented with proximal muscle weakness, myalgia, elevated creatine kinase, and features of a pure myopathic syndrome in whom they have identified a novel mutation in the mitochondrial tRNA(Ala) gene. This 5591G>A transition is heteroplasmic, segregates with cytochrome c oxidase deficiency in single muscle fibers, and fulfills recognized criteria for pathogenicity. This case exemplifies the wide-ranging clinical spectrum of mitochondrial disease presentations.

[AIDS prevention in austrian schools]. / Aids-Prävention an österreichischen Schulen. Aufklärungsprojekte zu HIV und Aids in Wien.

Prevention campaigns and new antiretroviral therapies caused a decline in HIV-infections as well as mortality from HIV/AIDS in industrialised countries. Despite this development AIDS is one of the ten mean causes of death worldwide, with 3,1 million deaths in 2002. Hence there is an urgent need for prevention and information campaigns, which ideally should start in early childhood or at school age. It is well known that preventive strategies start at a time when the risk or the possibility of a risky behaviour does not yet exist. A WHO-report in 1993 showed, that sex education often comes too late, as sexual activity of adolescents is already on its way. Persons who are already sexually active can be influenced to have fewer sexual partners and to increase safer sex methods. There is no evidence for causing a higher risk by sex education of adolescents. In Austrian schools numerous experts and organisations cooperate in the sphere of sex education and STD-prevention. School children in Vienna are confronted with this topic by the schools they attend according to the Sex Education-Act of 1970 on a voluntary basis. Various examples of effective teaching models prove intensive efforts and successful interdisciplinary cooperation. This study analyses common risk-reduction-methods and describes the Austrian way of HIV/AIDS/STD prevention in school children, although there is no obligation for sex education in schools, according to the School Teaching-Act of 1986, which leads to better knowledge and subsequent risk behaviour of adolescents in the same geographical area.

Outcome of osteotomies for the treatment of haemophilic arthropathy of the knee.

BACKGROUND AND AIMS: The knee is one of the most commonly affected joints in haemophilic arthropathy leading to stiffness and disability. It is the aim of this study to investigate the outcome of corrective osteotomies around the knee. PATIENTS AND METHODS: We report on the long-term results of ten osteotomies around the knee for severe haemophilic arthropathy and axial deviation at an average of 7.25+/-1.8 years postoperatively. Seven high tibial (preoperatively 7.2+/-2 degrees varus) and three supracondylar osteotomies (preoperatively 7+/-3 degrees valgus) were performed on seven patients (three of them bilateral). RESULTS: The clinical score of the Advisory Board of the World Federation of Haemophilia (average 7.4 points preoperatively) remained unchanged in two patients, improved in three patients and deteriorated in five patients. The radiological Pettersson score (average 8.2 points preoperatively) showed a worsening of 2.5 points over the time. Patients reported a subjective improvement for seven osteotomies, with sports activity in three patients, although the range of motion did not change significantly. Total knee arthroplasty was considered to be a failure, i.e. endpoint of follow-up. Six knees were replaced in four patients by total arthroplasty after a mean of 6.6 years. CONCLUSION: Although survival of osteotomies around the knee in haemophilic arthropathy is lower than in non-haemophilic patients, we think that it is a choice of treatment, which, at least, postpones the indication for total knee arthroplasty in this young patient group.

Synthesis and characterization of the first examples of perfluoroalkyl-substituted trialkyloxonium salts, [(CH3)2OCF3]+[Sb2F11]- and [(CH3)2OCF(CF3)2]+[Sb2F11]-.

In the superacidic HF/SbF(5) system, methyl trifluoromethyl ether forms at -78 degrees C the new tertiary oxonium salt [(CH(3))(2)OCF(3)](+)[Sb(2)F(11)](-), which was characterized by Raman and multinuclear NMR spectroscopy and its crystal structure. The same oxonium salt was also obtained by methylation of CH(3)OCF(3) with CH(3)F and SbF(5) in HF solution at -30 to -10 degrees C. Replacement of one methyl group in the trimethyloxonium cation by the bulkier and more electronegative trifluoromethyl group increases the remaining O-CH(3) bond lengths by 0.037(1) A and the sum of the C-O-C bond angles by about 4.5 degrees. Methylation of CH(3)OCF(CF(3))(2) with CH(3)F in HF/SbF(5) solution at -30 degrees C produces [(CH(3))(2)OCF(CF(3))(2)](+)[Sb(2)F(11)](-). The observed structure and vibrational and NMR spectra were confirmed by theoretical studies at the B3LYP/6-311++G(2d,2p) and the MP2/6-311++G(2d,p) levels.

[Treatment of coronary risk factors by general practitioners in Austria]. / Praxis der Behandlung koronarer Risikofaktoren bei niedergelassenen Arzten in Osterreich.

Aim of the study was to investigate attitudes and practice of coronary prevention in offices of general practitioners and internists. 67 Austrian physicians took part in a mail survey focussing on hyperlipidemia. 96.6% of participating physicians recommend dietary intervention at least at total cholesterol levels of 250 mg/dl, 98.0% prescribe lipid-lowering drugs at least at total cholesterol levels of 300 mg/dl. At corresponding levels of total/HDL cholesterol ratios and especially at corresponding levels of LDL-cholesterol the proportion was lower. On average 37.9% of physicians spend up to 5 minutes for patients with hyperlipidemia, 10.3% spend more than 15 minutes. The time frame is similar in overweight patients, and bigger in patients with hypertension and diabetes. Dietary therapy is estimated similarly successful in patients with hyperlipidemia and overweight, but estimated more successful in patients with hypertension and diabetes. Drug therapy of hyperlipidemia is estimated more successful than in overweight and diabetes, and worse compared with hypertension. Main measures for improving prevention are more time and specific postgraduate education. The majority of physicians feel that within the last five years quality has improved both in the outpatient and the inpatient care.

[Influenza pandemic: does Austria need an emergency plan?]. / Influenza-Pandemie: Braucht Osterreich einen Vorsorgeplan?

Even in present times and given the current state of medical science, an influenza pandemic may be expected to occur in addition to the usual annual epidemic of the disease. A pandemic is defined as a concentrated worldwide occurrence of a disease within a short period. Such a pandemic has been registered three times in this century. The influenza epidemic of 1918 to 1919 ("Spanish flu") killed 20 to 40 million people. A similar pandemic occurred in 1957 to 1958 (Hong Kong) and in 1968 (Asia). Although the current state of medicine is such that the high morbidity and mortality of 1918 is unlikely to recur, adequate preventive measures are warranted in health care as well as in social and political fields. In the event of such a pandemic, a large number of persons in Austria may be expected to be affected by the disease. In contrast to other countries, a detailed plan of prevention does not exist in Austria at the present time. A sudden influenza pandemic would give rise to a number of grave problems in the medical field (increase in mortality rates) as well as in social and political areas (maintaining the infrastructure of the country). In light of this fact it appears necessary to investigate the possibility of an influenza pandemic and to take suitable precautionary measures.

Acute cardiac inflammatory responses to postischemic reperfusion during cardiopulmonary bypass.

OBJECTIVES: The investigation centers on whether there is a reperfusion-induced specific cardiac inflammatory reaction after bypass surgery. BACKGROUND: Cardiopulmonary bypass (CPB) leads to systemic inflammation. Additionally, cardiac inflammation due to reperfusion could occur. Knowledge about nature and time course of this reaction might help to develop cardioprotective interventions. METHODS: In 12 patients receiving coronary bypass grafts, arterial and coronary venous blood was obtained before onset of CPB, and 1, 5, 10, 25, 35 and 75 min after cardiac reperfusion. Plasma levels of IL6 and IL8 were measured by immunoassay. CD11b, CD41, and CD62 on blood cells were quantified by flow cytometry. Measurement of CD41, a platelet marker, on neutrophils and monocytes allowed detection of leukocyte-platelet microaggregates. RESULTS: Transcardiac veno-arterial difference of IL6 rose in the 10th and 25th min of reperfusion (from 0 to 7 pg/ml; p < 0.05), and after 75 min (15 pg/ml). IL8 did not change. CD11b on neutrophils (PMN) decreased transcardially to 95, 88 and 82% of the initial level in the 5th, 10th, and 75th min, respectively, suggesting sequestration of activated neutrophils. CD62 on platelets rose about 30% in the 75th min. Initially, leukocyte-platelet microaggregates were formed during coronary passage (+31% of the arterial level for PMN, +23% for monocytes). During reperfusion, coaggregates were retained (PMN: -1% and -7% in the 5th and 10th min, monocytes: -22%, -13% and -12% in the 1st, 5th and 10th min. CONCLUSIONS: During early reperfusion after aortic declamping, the coronary bed is already a source of proinflammatory stimuli and target for activated leukocytes, partly in conjunction with platelets. Mitigation of these phenomena might help to improve cardiac function after CPB especially in patients at risk.

Azidothymidine (AZT) in the treatment of symptomatic HIV-1-infected hemophiliacs.

Twenty-one immunodeficiency virus 1 (HIV 1)-positive hemophilic patients were treated with Azidothymidine (AZT) for symptomatic HIV infection. The median observation period was 20.5 months. At 25 months the probability of survival was 82%, the probability of progression of disease from CDC III or IV C2 to IV C1 (AIDS) was 20% in patients on continuous AZT treatment and 50% in patients with interruption of treatment. Three patients developed severe leukopenia and 3 patients severe anemia during AZT treatment. In 1 patient a dose-dependent striking increase of transaminases during AZT treatment was observed. In 7 patients treatment was interrupted, in 1 patient because of anemia, in 1 because of pruritus and in 5 patients because of non-compliance. No significant changes in the consumption of clotting factor concentrates and number of bleeding episodes before and during AZT treatment were noted. We conclude, that both hematological and non-hematological side effects of AZT in HIV 1-infected hemophilic patients are comparable to those seen in other risk groups. AZT does not increase the bleeding tendency in this patient group.

High risk of acquired immune deficiency syndrome (AIDS) and of AIDS related complex (ARC) in hemophiliacs seropositive for more than 5 years.

A group of 90 hemophiliacs who had been regularly treated with non virus-inactivated factor VIII or IX concentrates were studied in 1983. At that time 50 patients were HIV-1-antibody positive, 6 additional seroconversions occurred until 1985. 26 of the 50 patients seropositive in 1983 are currently asymptomatic. 4 patients have developed the lymphadenopathy syndrome, 9 patients AIDS and 11 patients ARC (CDC IV C 2). 6/9 cases of AIDS and 10/11 cases of ARC have occurred only after 1985. Patients, who subsequently became symptomatic, had significantly higher IgG levels in 1983, otherwise no predictive laboratory tests were identified. Patients with T4 counts above 500/microliters became symptomatic later, but after 5 years the incidence of AIDS was comparable in patients with original T4 counts of more than or below 500/microliters.

Anticardiolipin antibodies are elevated in HIV-1 infected haemophiliacs but do not predict for disease progression.

Levels of anticardiolipin antibodies (ACA) were measured in 55 patients with haemophilia A in serum samples obtained in 1983 and in 1987. Twenty-one patients were negative for anti HIV-1 antibodies in 1983 and remained negative in 1987; 34 patients had anti HIV-1 antibodies in 1983; 17 of these latter patients remained asymptomatic, whereas 17 patients developed ARC or AIDS during the 4 years follow-up. Thirteen anti HIV-1 negative patients had elevated ACA levels in 1983; subsequently, a significant decrease was observed in all these subjects (p less than 0.001). All anti HIV-1 positive patients had elevated ACA levels in 1983; normal values were found in 9 patients in 1987. Yet, these changes were not significant (p greater than 0.05). ACA levels were significantly higher in HIV-1 infected patients than in those without anti HIV-1 antibodies (p less than 0.05). There was no difference of ACA levels between the two anti HIV-1 positive patient groups, be it in 1983 or be it in 1987 (p greater than 0.05). There was no correlation of ACA levels with serum IgG concentrations, CD4+ lymphocytes, or the consumption of factor VIII concentrates.

Treatment of tracheal stenoses by resection in infancy and early childhood.

An inquiry of Austrian paediatric and thoracic surgeons revealed six children aged 4 months to 5 years who had undergone circumferential resection of the trachea for congenital or acquired tracheal stenoses. Clinical data relating to these six Austrian cases are briefly presented. Experience shows that circumferential resection of localized tracheal stenoses can also be recommended for infants and young children.

[Early diagnosis and early treatment of a patient with intralobular pulmonary sequestration]. / Frühdiagnose und Frühbehandlung eines Patienten mit intralobärer Lungensequestration.

A report is given on a case of intralobar pulmonary sequestration (I. L. S), which was hospitalized with the suspect diagnosis of a cavernous tuberculous infection. The 3 6/12-year-old boy was suffering of recurrent respiratory infections. These led finally to a thoracic X-ray control. X-rays showed a cystic malformation of the left lower lobe. This localisation drew our attention especially to I. L. S. after exclusion of various other diagnostic possibilities. For this reason a retrograde aortography was carried out and showed an abnormal blood supply of the cyst from the thoracic aorta. So this procedure established the diagnosis. Because of the high pulmonary and cardiac complication rate, a surgical treatment, as early as possible, is the therapy of choice. In our case the segmental resection was sufficient.

[The chronically altered appendix in children. Cooperative work of the Austrian Society for Pediatric Surgery]. / Die "chronisch veränderte" appendix beim kind. Gemeinschaftsarbeit der Osterreichischen Gesellschaft für Kinderchirurgie.

In this multicentric study the anamnestic data and parents questionaires of 2-29 children were evaluated, whose appendix vermiformis at the time of operation showed no signs of inflammation. 31,1% macroscopically showed cicatrisation or fasciation, in 13,7% other enteral or gynaecological (1%) findings were present. In 1194 of the cases of patho-histological examination of the appendix had been made of which 36.4% were negative, 37.7% showed cicatrisation, 15,0% oxyuriasis and 10,4% coproliths. In the discussion of the significance of the chronically altered non-inflamed appendix we compared patients with and without macroscopical or microscopical alterations. We came to the conclusion that the probability-index as to sex, duration of symptoms, complexity of symptoms, incidence of postoperative well-being, proved the chronically altered appendix not to be an illness per se, but the result of spontaneously arrested inflammation. Since the rate of children admitted with a perforated appendix is high (15-20%) in comparison with the rate of complications after removal of non-inflamed appendices (2,9%), we believe that according to the diagnostical problems the principle can be maintained: in dubio pro operatione.

[Treatment of Wilms' tumor (author's transl)]. / Therapie des Wilms-Tumors.

Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.