Search PubMed⌕ Search

Biomedical subjects

W Schramm

Publications and source records attributed to W Schramm.

At least 37 records · Page 2Linked to original sources
PubMed2003

[Gathering and evaluation of phenotype data of haemophilia A patients for correlation with genotype data].

Haemophilia A is caused by a genetic defect of the factor VIII gene resulting in complete or considerable functional loss of factor VIII molecule within blood. The high bleeding risk of patients can be prevented by intravenous injections of factor VIII protein. However, 25% of patients affected with severe haemophilia, develop factor VIII antibodies against the concentrate substituted. Within this study we try to comprise the phenotypic parameters (e. g. detailed documentation of disease course, basic laboratory values) and the therapy-associated data (e. g. applicated type and amount of factor VIII, number of substitutions, factor VIII recovery, inhibitor development and inhibitor elimination). We hope to identify differences of variable therapeutic treatments on course of disease as already identified for the factor VIII gene defects. At least we expect that certain mutations and mutation types, respectively, can be referred to typical phenotypes and similar course of treatment protocols.

Databases, Factual↗
PubMed2002

Clinical outcomes and resource utilization associated with haemophilia care in Europe.

We conducted a multicentre, cross- sectional study of 1042 haemophilia subjects across Europe to compare various health outcomes associated with on-demand vs. prophylactic factor-substitution therapy. Demographic, medical history, and healthcare resource utilization data were analysed along with the number of bleeding events over the past 6 months. Treatment-cost data were also examined to provide preliminary information for future economic studies. A logistic regression analysis, controlling for other statistically significant covariates, showed that patients treated on demand were 3.4 times more likely to have had a joint bleed over the previous 6 months than those treated with prophylaxis. Multiple regression analyses further confirmed these findings, because on-demand subjects had, on average, 5.15 more joint bleeds over the reporting period than patients treated with prophylaxis. Notably, these findings were even more dramatic for younger haemophilia patients when our study sample was stratified by age. Due to the high cost of factor replacement, healthcare costs were significantly higher for subjects treated prophylactically. While hospital costs for prophylaxis subjects were, on average, lower, statistically significant cost savings for prophylactic subjects were not noted. These results suggest that clinicians and health policy decision-makers should consider the advantages of prophylactic therapy for haemophilia patients in formulating treatment protocols and allocating health resources.

Age Factors↗
PubMed2002

Quality-of-life differences between prophylactic and on-demand factor replacement therapy in European haemophilia patients.

The European Study on the Clinical Outcomes and Resource Utilization associated with Haemophilia Care was designed to compare various health outcomes associated with on-demand and prophylactic factor substitution methods in European haemophilia patients. While the primary objective of this research is to conduct an economic analysis, an important component of this study is to evaluate quality-of-life differences that may exist between patients who utilize these two styles of therapy. Quality-of-life research has emerged as a primary measure of health outcomes because it allows the augmentation of traditional clinical indicators of health with data gathered from the patient's perspective. A total of 1033 haemophilia patients from 16 European haemophilia treatment centres were enrolled in this study. The SF-36, a multidimensional quality-of-life instrument, was administered to all participants. This instrument measures eight health-related quality-of-life dimensions: physical functioning, physical role limitations, bodily pain, general health, vitality, social functioning, emotional role limitations, and mental health. All haemophilia subjects enrolled in the study scored significantly lower than the population normative means in the three physical dimensions and in the general health dimension. HIV-negative haemophiliac subjects differed significantly by factor substitution type in a multivariate analysis examining all eight health dimensions. Univariate analyses testing each dimension separately indicated that patients treated prophylactically reported significantly less bodily pain, better general health, and scored significantly higher in the physical functioning, mental health, and social functioning dimensions. While these results suggest that health-related quality-of-life may be better for haemophilia patients treated prophylactically, future prospective studies that gather periodic quality-of-life data over time should be conducted.

Acquired Immunodeficiency Syndrome↗
PubMed2002

Linking medicine and economics: health economics and quality of life in haemophilia care.

Scarce resources in healthcare are a fact of life everywhere. Providing optimal healthcare for haemophilia means delivering the best possible clinical outcomes at an acceptable total cost, however that is defined by individual societies. Health economics helps us to do this. So far, economic evaluation has enhanced our understanding of some of the economic implications of haemophilia and has provided some guidance on the way forward to optimize efficient allocation of resources for this condition. Further advances and consensus on evaluation methods and standards for the economics of haemophilia are required, however. This need will continue in the face of new, more sophisticated and therefore more costly haemophilia therapies.

Economics, Medical↗
PubMed2001

Impact of early high caloric duodenal feeding on the oxygen balance of the splanchnic region after severe burn injury.

Early enteral nutrition is recommended in burned patients. Depending on the amount administered, enteral feeding causes an increase of intestinal oxygen-demand. Although early moderate enteral nutrition has been shown to be beneficial, early high calorie enteral nutrition might lead to an imbalance of the O(2)-balance of the gut since intestinal perfusion is decreased after major burns. In 20 severely burned patients during the first 48 h of early high caloric duodenal feeding an assessment of the CO(2)-gap between the arterial and the gastric CO(2), as parameter for the intestinal O(2)-balance, was performed. Time points were prior to starting the enteral nutrition (BASE) subsequently every 30 min after increasing the amount of nutrition administered and from the 11th to the 48th h after beginning of nutrition in intervals of 6 h. In none of the patients was the CO(2)-gap increased during the rapid increase of enteral nutrition. On the contrary the CO(2)-gap decreased significantly. We conclude that high caloric duodenal feeding in the early hypodynamic postburn phase does not have adverse effects on the oxygen balance of the intestine.

Adult↗
PubMed2001

Proprioception and isometric muscular strength in haemophilic subjects.

Haemophilia is characterized by intra-articular bleeding, often requiring immobilization, which may result in muscle atrophy and impaired proprioception. The aim of the study was to investigate differences in proprioceptive performance and isometric muscular strength of the lower limbs in haemophilic subjects compared with control subjects. Twelve subjects with severe haemophilia (11 haemophilia A; one haemophilia B) vs. 12 control subjects were matched for anthropometric data and tested for differences of proprioception (one-leg-stand, posturomed, angle-reproduction, and tuning fork tests) and isometric strength (leg press, knee extensor). The static proprioceptive performance of the haemophilic group, as measured by the one-leg-stand test (on hard or soft ground, with open or closed eyes; P < 0.05) was demonstrably impaired (by 41-363%). In contrast, the dynamic proprioceptive performance measured by the posturomed test did not show any difference between the groups. The local proprioceptive performance (angle-reproduction test) of the knee, (the most commonly affected joint in haemophiliacs) showed a trend to impaired function but was not distinctly different from that of controls. The quantitative sensory function (tuning fork) showed significant (P < 0.05) impairment of 9-10% in the haemophilic subjects. Additionally, the isometric muscular strength of the leg extensor was weaker (32-38%) in the haemophilic group when the limbs were tested individually as well as bilaterally (P < 0.05). In conclusion, the results suggest that global proprioceptive performance is impaired and that the isometric strength of the leg extensors is weaker in the haemophilic subjects. Therefore, specialized training for global proprioception would be helpful in order to compensate for proprioceptive deficits. This exercise regimen should also include safe strength-training for an optimal stabilization of the joints, but must be adapted to the individual needs and situations of the haemophilic subjects.p

Hemophilia A↗
PubMed2000

[The Bavarian Thromboembolic Risk Cohort Study (BATER). Study protocol, state of the investigation and first results].

OBJECTIVE: Familiar venous thromboembolic disease (VTE) is known to be related with factor V Leiden mutation (FVL), but also with other genetic markers. It is the objective to investigate of the BATER-study in a representative Bavarian cohort, and to assess whether they could predict VTE events. This paper shortly describes the study protocol, gives an overview of planned sub-studies, and provides first results of the historic cohort analysis. PATIENTS AND METHODS: The baseline survey of the cohort study of Bavarian women aged 18-49 years (random sample from the population) was performed in two samples in 1996 and 1997. It was planned to estimate a prevalence and predictive value of potential markers of VTE in a historic--prospective as well as concurrent approach with annual follow-up of the cohort. This representative cohort should build a basis for nested case-control studies and serve as a reference group for other analytical epidemiological studies in young women. 1685 women were ascertained (response rate 61%), underwent an inquiry, and provided blood samples for a blood bank; for this paper, complete data are available from 1650 women. Laboratory parameters were measured to determine APC resistance, FVL-mutation, antithrombin-, protein C and S deficiency, and were correlated to the results of a detailed, life-time history of thrombembolic events. RESULTS: The prevalence of FVL mutation in the sample was 5.7% (95% confidence interval 4.6-6.6%). Other genetic VTE risk markers were observed to be less frequent than 1%. The positive predictive value (pPV) of FVL mutation for a VTE event is about 7%, but for a positive family history of VTE (first grade relatives) 3% only. CONCLUSIONS: VTE events are rare in the German population of young women, even in cases of FVL mutation. A positive family history is rarely associated with the occurrence of VTE in women under 50 years of age, and the predictive value of FVL mutation is low. Therefore, a screening for FVL mutation is not justified unless there is suspicion of a high VTE risk for other reasons.

Adolescent↗
PubMed2000

Twin birth weight discordance and risk of preterm birth.

OBJECTIVES: Our purpose was to determine whether birth weight discordance is a risk factor for preterm birth of twins, and to further characterize the relationships involved. STUDY DESIGN: Maternally linked 1978-1990 Missouri birth certificates were used to analyze gestations resulting in live twins. We used contingency tables and multiple logistic regression. RESULTS: The degree of discordance correlated strongly with risk for live preterm birth but only for discordances >30% and preterm birth at <32 weeks' gestation. Among 9479 pregnancies with discordance <30%, 9.5% ended in birth at <32 weeks' gestation, versus 13.7% of 326 with discordance of 30% to 40% (P =.03) and versus 34.1% of 126 with discordance > or =40% (P <. 001). There were 42 preterm twin births at <32 weeks' gestation with discordances > or =40%. Of these, 51% were attributable to fetal growth restriction and 16% to large size for gestational age in one infant; in 72% the smaller twin was the second born, and in 86% the twins were like sex. The relative association between > or =40% discordance and preterm birth at <32 weeks' gestation was strengthened (final odds ratio, 9.54; P <.0001) in a multivariate model containing other risk factors for delivery at <32 weeks' gestation: black race, either twin small for gestational age, unmarried, teenage mother, number of male fetuses, like fetal sex, education <12 years, nulliparity, and cigarette smoking. CONCLUSIONS: Twin birth weight discordance has now clearly been demonstrated to be a risk factor for preterm birth. The effect was found particularly with discordances > or =40% before 32 weeks' gestation. Discordance was usually attributable to fetal growth restriction, most often in the second-born twin.

Birth Order↗
PubMed2000

Are factor V Leiden carriers who use oral contraceptives at extreme risk for venous thromboembolism?

BACKGROUND: Major concern was raised by an earlier study regarding oral contraceptive use in women with the factor V Leiden mutation. A more than 30-fold increase in relative risk for venous thromboembolism was reported; for homozygotes, the relative risk was as much as 100-fold or more. OBJECTIVE: To replicate the reported risk estimates with a new population-based case-control study. METHODS: Eighty women with a diagnosis of venous thromboembolism were consecutively identified and compared with population-based controls (n = 406). Factor V Leiden mutation was identified by genotype analysis. The evaluation was performed with conditional logistic regression (matched for 5-year age group). RESULTS: Matched, adjusted odds ratios (OR) for idiopathic venous thromboembolism in women without and with the factor V Leiden mutation who used oral contraceptives were 4.1 (95% confidence interval (CI) 2.1-7.8) and 10.2 (95% CI 1.2-88.4), respectively. The adjusted OR for factor V Leiden carriers was 2.0 (95% CI 1.0-4.4). The OR for women with the factor V Leiden mutation and oral contraceptive use versus no factor V Leiden mutation and no oral contraceptive use was 10.2 (95% CI 3.8-27.6). CONCLUSION: The results confirm the increased relative risk of idiopathic venous thromboembolism for users of oral contraceptives and factor V Leiden carriers. However, we suspect that the true risk for women who are factor V Leiden carriers may be increased two- to four-fold rather than seven-fold or more, and that the risk for the combination of factor V Leiden and oral contraceptive use may be increased in the order often- to 15-fold rather than over 30-fold.

Adolescent↗
PubMed2000

Propofol without muscle relaxants for conventional or fiberoptic nasotracheal intubation: a dose-finding study.

UNLABELLED: Endotracheal intubation has been performed during the administration of propofol anesthesia without neuromuscular blockade. In this study, we determined the propofol dose required for conventional nasotracheal or for fiberoptic nasotracheal intubation of all patients. Thirty-two patients undergoing maxillofacial surgery were randomly assigned to the conventional (n = 16) or to the fiberoptic (n = 16) intubation group. In both groups, anesthesia was induced by using IV fentanyl and IV titrated propofol according to clinical need (spontaneous respiration rate, verbal response). An endotracheal tube was placed nasally in the pharynx and the vocal cords visualized by using a fiberscope inserted via the tube. In the conventional group, the larynx was visualized additionally with a laryngoscope blade (Miller). In both groups propofol was titrated until the vocal cords opened. Patients were tracheally intubated, and the propofol dose was recorded. In all patients, the trachea could be intubated without the use of muscle relaxants. Considerable interindividual differences of dose requirements were observed. The amount of propofol required in the conventional group was significantly (P < 0.0001) larger (median +/- SD: 2.74 +/- 1.59 mg/kg; range 1.95-7.07 mg/kg) than in the fiberoptic group (1.37 +/- 0.59 mg/kg; 0.72-2.86 mg/kg). Hemodynamics remained stable in all patients. Postintubational hoarseness occurred in three patients of each group. Fiberoptic nasal intubation without a muscle relaxant can be facilitated with significantly smaller and more predictable dosages of propofol than conventional nasal endotracheal intubation. The possibility of titrating the propofol dose under assisted ventilation until the vocal cords open during fiberoptic nasotracheal intubation and the better predictability of the required dose favors the fiberoptic approach. IMPLICATIONS: In this study, contrary to all preceding studies using predefined doses of propofol and opioids, we determined the minimal required propofol dose in combination with fentanyl for conventional or fiberoptic nasotracheal intubation without muscle relaxants.

Adult↗