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

Summary.  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.     

Variability in bleeding phenotype in Amish carriers of haemophilia B with the 31008 C→T mutation

Summary.  The aim of this study was to characterize the variability of bleeding phenotype and its association with plasma factor IX coagulant activity (FIX:C) in haemophilia B carriers in a large Amish pedigree with a unifying genetic mutation, C-to-T transition at base 31008 of the factor IX gene ( Xq27.1–27.2) . A cross-sectional survey of haemophilia B carriers included a multiple choice questionnaire evaluating symptoms of mucocutaneous bleeding, joint bleeding and bleeding after haemostatic stress [menstruation, postpartum haemorrhage (PPH), dental extractions and invasive surgeries]. Severity of bleeding was graded as 0 to 4, 0 being no bleeding whereas 4 being severe bleeding. Association between total bleeding scores and the FIX:C was evaluated. Sixty-four haemophilia B carriers participated in this study. Median age: 18 years (range 1–70 years); median bleeding score: 1 (range 0–8). Besides PPH, isolated symptoms of bruising, epistaxis, menorrhagia and postsurgical bleeding including dental extraction were not associated with lower FIX:C. Bleeding score ≥3 was associated with involvement of at least two bleeding sites and a lower mean FIX:C of 42 ± 10.3% (95% CI 36.4–47.7) while a score >3 had involvement of ≤2 sites and higher mean FIX:C of 54.9 ± 21.5% (95% CI 49–61), P  = 0.005. Subcutaneous haematoma formation and bleeding after haemostatic stress requiring treatment were associated with bleeding scores ≥3. Phenotypic variability existed among the carriers of haemophilia B who belonged to a single pedigree carrying a single unifying mutation. The utility of bleeding scores to define bleeding phenotype precisely in haemophilia B carriers needs further evaluation.     

An investigation of the frequency of bacteraemia following dental extraction,tooth brushing and chewing

Abstract

We conducted a study to determine the frequency of bacteraemias following dental extraction and common oral procedures, namely tooth brushing and chewing, and the relationship between bacteraemia and oral health in black patients. Positive blood cultures were detected in 29.6% of patients after dental extraction, in 10.8% of patients after tooth brushing and in no patients after chewing. No relationship between the state of oral health, which was assessed using the plaque and gingival indices, and the incidence of bacteraemia was found. The duration of bacteraemia was less than 15 minutes. One patient had a positive blood culture prior to dental extraction; his oral health status was poor. Our study confirmed that bacteraemia occurs after tooth brushing.     

Factors associated with oral health-related quality of life in community-dwelling elderly persons with disabilities

OBJECTIVES: To examine, in community‐dwelling elderly persons with disabilities, the association between oral health—related quality of life (OHRQOL) as measured using the 14‐item Oral Health Impact Profile (OHIP‐14) and specific oral health, health, and disability status variables; life satisfaction; living alone; and low income. DESIGN: Observational cross‐sectional. SETTING: A Medicare demonstration conducted in 19 counties in three states. PARTICIPANTS: Six hundred forty‐one disabled, cognitively intact, community‐dwelling individuals aged 65 and older. MEASUREMENTS: The subjects' OHRQOL was assessed using the OHIP‐14, which was scored using three different methods. Data on oral health, health and functional status, life satisfaction, prior health services use, and sociodemographics were collected using interviewer‐administered questionnaires. RESULTS: The participants' mean age was 79.1, and they were dependent in an average of 1.8 activities of daily living (ADLs); 43.1% were edentulous, 77.4% wore a denture, 40.4% felt that they were currently in need of dental treatment, and 64.7% had not had a dental examination in the previous 6 months. Seven of the 16 variables of interest had significant bivariate relationships using three OHIP scoring methods. Logistic regression analysis found that poor OHRQOL was significantly associated with perceived need for dental treatment (odds ratio (OR)=2.61), poor self‐rated health (OR=2.29), poor (OR=2.00) and fair (OR=1.73) mental health, fewer than 17 teeth (OR=1.74), and relatively poor cognitive functioning (OR=1.52). CONCLUSION: OHRQOL is associated with some (perceived need for dental treatment, poor self‐rated health, worse mental health, fewer teeth, and relatively poor cognitive status) but not all (e.g., ADL and instrumental ADL dependence) measures of oral health, health, and disability status and not with life satisfaction, living alone, or low income.     

The association between demographic and oral health‐related quality of life factors and dental care attendance among underprivileged older people

Aim: In order to identify whether demographic and oral health‐related quality of life factors are associated with dental care attendance among an underprivileged older population, a comparison was performed between people who have and have not attended dental care. Methods: A cross‐sectional purposive sample of 344 older underprivileged people comprised the study population. The dependent variable was dental care attendance. The 14‐item version of the Oral Health Impact Profile index (OHIP‐14) was used as the independent variable, together with other social and general variables, using a structured interview. Results: The variables that were significantly associated with dental care attendance were family status (not married, the highest attendance), dwelling location (living at home, the highest attendance), caregiver (family member, the highest attendance), place of birth (Western countries, the highest attendance) and income (pension, the highest attendance). Sex, welfare support, functional ability, education, age and OHIP‐14 were not associated with dental care attendance. Conclusions: Attending dental care was not associated with oral health‐related quality of life measured by OHIP‐14. Several socioeconomic variables were strongly associated.     

Survival in a cohort of patients with haemophilia at the haemophilia care center in Vienna, Austria, from 1983 to 2006

Summary.  Survival of patients with haemophilia is still a relevant issue of great interest. A survival analysis was conducted among 226 patients with haemophilia A and B (128 severe haemophiliacs), who were treated at the haemophilia care centre in Vienna. Information on mortality in our patient cohort was obtained from the Austrian Central Death Register. Overall, 96 of a total of 226 patients (42.5%) died between 1983 and 2006; 37 patients (38.5%) died due to HIV-infection, 15 due to HCV infection, 15 due to bleeding (15.6%, respectively) and 29 (30.2%) due to various other causes. The mortality of HIV-positive patients was 74.3% ( n  = 55) and that of HCV-positive patients was 40.4% ( n  = 55) in the analysed period. The patient mortality rates were compared with those of the general Austrian male population following adjustment for age and calendar period. We found that the cumulative relative survival of all patients was 0.694 (95% CI 0.614–0.767). The cumulative relative survival of patients with severe haemophilia (FVIII or IX level ≤1%) was 0.489 (0.394–0.579), but was normal (0.986; 95% CI 0.858–1.082) for patients with mild or moderate haemophilia (FVIII or IX level 2–50%). The survival rate was lowest in HIV-positive patients (0.287; 95% CI 0.186–0.398), but was also decreased to 0.874 (0.776–0.951) in HIV-negative patients. It can, therefore, be concluded that the survival of patients with severe haemophilia is still decreased compared to those with non-severe haemophilia and the general male population, regardless of HIV-infection.     

General and oral health‐related quality of life among caregivers of Parkinson's disease patients

Aim: The aim of the present study was to evaluate the general and oral health‐related quality of life among caregivers of patients with Parkinson's disease. Methods: A representative sample (n = 80, 19 men and 61 women) of caregivers from São José dos Campos, Brazil, answered Oral Health Impact Profile (OHIP‐14) and Medical Outcomes Study 36‐Item Short Form (SF‐36) questionnaires, as well as another questionnaire regarding caregivers' characteristics and caring activities. Results: The OHIP‐14 average score was 2.56. The SF‐36 average score for the domain of physical function was 87.4; for role‐physical function, 85.0; for bodily pain, 62.9; for general health, 76.6; for vitality, 80.0; for social function, 88.4; for role‐emotional function, 83.8; and for mental health, 85.3. All domains of the SF‐36 also showed negative correlations with OHIP‐14 in the entire sample, but this correlation could not be observed among lower educated caregivers. There were significant differences between formal and informal caregivers for OHIP‐14 and for the majority of SF‐36 domains. Conclusions: Most caregivers were female, the mean scores for SF‐36 and OHIP‐14 were adequate, and formal caregivers had better results for the SF‐36 and OHIP‐14 when compared with informal caregivers. Geriatr Gerontol Int 2013; 13: 429–436 .     

Comorbidities and bleeding pattern in elderly haemophilia A patients

Summary.  The increasing numbers of comorbidities related to higher age and their treatment constitute a challenge in the treatment of haemophiliacs. Comparing prevalences of morbidities in the elderly haemophilia A population ( n  = 29) and the general elderly population of Germany reveals some differences. HCV infections are more frequent in the elderly haemophilia population (69% vs. 0.6%). Prevalence of cancer was five times higher than in the age matched general population (28% vs. 5.2%). Cardiac diseases seem to be less frequent although the prevalences of cardiovascular risk factors like hypertension, diabetes, and body mass index (BMI) >25 do not differ in comparison to the general population. A reduction of bleeding symptoms or dosage of FVIII could not be observed. There is a tendency of increasing bleeding symptoms with increasing age of the patients due to more frequent spontaneous joint bleedings, malignancies or treatment with phenprocoumon or ASA. In consequence, FVIII dosage had to be increased in eight patients (28%). Our patient population at the age >60 years is very small and no statistical evidence can be shown, therefore appropriate treatment of elderly haemophiliacs needs further evaluation in multicentre studies with sufficient patient numbers.     

Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations

Summary.  Although most surgical and invasive procedures can be performed safely in patients with haemophilia, the optimal level and duration of replacement therapy required to prevent bleeding complications have not been established conclusively. For providing more insight into optimal therapy during invasive procedures, a literature review of surgical procedures in patients with haemophilia was conducted. Concomitantly, current practice was surveyed in 26 European Haemophilia Comprehensive Care Centres, representing 15 different countries. The review identified 110 original papers published between 1965 and 2007. Of these, only two studies were randomized controlled trials. Target levels and the duration of replacement therapy in the published studies were as follows. For major orthopaedic surgery: preoperative targets were 80–90%; postoperative targets showed a high degree of variation, with trough levels ranging from 20% to 80%, duration 10–14 days; for liver biopsy, 70–100%, 1–7 days; tonsillectomy: 90–100%, 5–11 days; indwelling venous access device insertion: 100%, 3–10 days; circumcision: 50–60%, 2–4 days; dental surgery: 30–50%, single treatment. With the exception of dental surgery, current practice in Europe, as assessed by the survey, was largely in agreement with published data. In conclusion, this study provides both a comprehensive review and a large survey of replacement therapy in patients with haemophilia undergoing invasive procedures; these data have informed the consensus practical treatment recommendations made in this paper. This study highlights the need for better-designed studies in order to better define minimal haemostatic levels of replacement therapy and optimal treatment duration.     

Impact of mandibular two-implant retained overdentures on life quality in a group of elderly Turkish edentulous patients

The aim of the study was to examine the influence of mandibular two-implant retained overdentures on life quality of elderly Turkish patients. The study population comprised 78 edentulous complete denture wearers (aged 65-82) seeking implant-retained mandibular overdentures at Istanbul University, Turkey. Every patient received 2 dental implants in the interforaminal region of the mandible and maxillary conventional dentures and mandibular implant retained overdentures 6 weeks after surgery. Turkish versions of oral health impact profile (OHIP-14) and the United Kingdom oral health-related quality of life (OHQoL-UK) were used for the evaluation of life quality before and after treatment. Compared to pretreatment scores, subjects had statistically significant improvements in life quality after receiving new dentures (p < 0.05). Based on these results, it can be concluded that mandibular two-implant retained overdentures improve the life quality of elderly edentulous Turkish patients.     

Illicit drug use and oral health

People with drug use disorders (PWDUD) have elevated prevalence of oral diseases, in particular dental caries (tooth decay), periodontal (gum) disease and xerostomia (dry mouth). When left untreated, these oral health conditions may progress and lead to tooth ache, abscesses and tooth loss, and in turn, to poor chewing functioning and digestion, dental aesthetic problems and reduced wellbeing. Illicit drug use may, per se, cause xerostomia, which in turn increases vulnerability for dental caries. However, the other main drivers of oral diseases and their progression—poor oral hygiene, frequent sugar intake and infrequent dental visits—can mainly be ascribed to the irregular lifestyle, poor economy and mental health problems that often accompany illicit drug use. Establishment of good oral health habits is essential in the dental care for PWDUD. Dental treatment is often comprehensive and challenging; because the patients may have extensive treatment needs but also difficulties adhering to preventive measures and dental appointments. An integrated care approach for PWDUD would likely benefit both their oral and general health.     

Effect of the Japanese preventive-care version of the Minimum Data Set – Home Care on the health-related behaviors of community-dwelling, frail older adults and skills of preventive-care managers: A quasi-experimental study conducted in Japan

Aim:   To determine whether the Japanese preventive-care version of the Minimum Data Set-Home Care improves the health-related behaviors of older adults and the skills of preventive-care managers.
Methods:   Municipal preventive-care managers were instructed on the use of the Japanese preventive-care version of the Minimum Data Set – Home Care and asked to employ it in their interactions with clients during the intervention period (intervention group). The health-related behaviors of older adults (maintenance of self-care and consumption of a balanced diet) were assessed by self-rating methods. The skills of the preventive-care managers were assessed by considering the number of and variations in the needs of the clients, as reflected in the care plans formulated by the managers.
Results:   The clients' self-care levels were higher in the intervention group than in the control group ( P  < 0.05). A greater number of needs, as reflected in the care plans, were noted in the intervention group than in the control group ( P  < 0.05), and the variation in the assessed needs was greater in the former than in the latter.
Conclusion:   This study suggests that the Japanese preventive-care version of the Minimum Data Set – Home Care may improve the skills of preventive-care managers, and consequently, the health-related behaviors of frail older clients.     

大学新生口腔健康状况调查分析

目的：调查大学新生口腔健康状况,为针对性开展口腔疾病防治工作提供信息支持及依据。方法参考全国口腔流行病学调查方案,自行设计大学新生口腔健康状况调查问卷,对该校2009年入学的新生960名进行调查分析。结果大学新生龋齿、牙龈炎、牙结石、口腔溃疡、牙体缺损患病率分别为62.1％、55.0％、48.1％、18.1％和14.2％;女生龋齿率达71.3％,显著高于男生的56.2％（ P＜0.01）,男生牙龈炎、牙结石、口腔溃疡、牙体缺损患病率均显著高于女生（P＜0.05）;性别、是否早晚刷牙、刷牙持续时间是否＜2 min、睡前是否习惯吃零食、是否定期口腔检查、城乡来源是大学新生口腔健康的主要影响因素（ P＜0.01）。结论大学新生口腔健康状况不容乐观,应对大学新生开展口腔健康教育,普及口腔健康保健知识,践行口腔卫生行为,提高大学生口腔健康水平。     

Association of the oral health impact profile with malnutrition risk in Spanish elders

The objective of this study was to determine any relationship between oral health-related quality of life (OHR-QoL) and malnutrition risk in the elderly using the oral health impact profile (OHIP). We studied 250 institutionalized elderly people, 162 females and 88 males, with and without teeth. Data were gathered on: general health; oral health; malnutrition risk, measured with the Mini Nutritional Assessment (MNA); and OHR-QoL, evaluated with the OHIP. A multivariate binary logistic regression model was constructed with malnutrition presence/risk as dependent variable. Mean age was 82.7 ± 8.2 years. Malnutrition or malnutrition risk was shown by 36.8% of the sample. OHIP was associated with malnutrition/risk after adjustment for age, sex, functional status, and mild dementia diagnosis. Malnutrition/risk was 3.43-fold more likely in participants with OHIP-reported “problems” than in those with none. The conclusion of the study was that OHIP-measured OHR-QoL is associated with malnutrition risk.     

Association of the oral health impact profile with malnutrition risk in Spanish elders

The objective of this study was to determine any relationship between oral health-related quality of life (OHR-QoL) and malnutrition risk in the elderly using the oral health impact profile (OHIP). We studied 250 institutionalized elderly people, 162 females and 88 males, with and without teeth. Data were gathered on: general health; oral health; malnutrition risk, measured with the Mini Nutritional Assessment (MNA); and OHR-QoL, evaluated with the OHIP. A multivariate binary logistic regression model was constructed with malnutrition presence/risk as dependent variable. Mean age was 82.7 ± 8.2 years. Malnutrition or malnutrition risk was shown by 36.8% of the sample. OHIP was associated with malnutrition/risk after adjustment for age, sex, functional status, and mild dementia diagnosis. Malnutrition/risk was 3.43-fold more likely in participants with OHIP-reported “problems” than in those with none. The conclusion of the study was that OHIP-measured OHR-QoL is associated with malnutrition risk.     

Dental health and disease in patients with haemophilia – a case‐control study

Management of patients with hereditary bleeding disorders in dentistry causes considerable problems. This study examined different aspects of dental health or disease of Lithuanian children and adults with haemophilia and compared them with the general population. Two study groups of cases and controls were formed. Cases were recruited through census sampling and controls were randomly chosen from the general population matched for gender, age and place of residence. Dental health of permanent and deciduous dentitions was assessed by one examiner employing the WHO Criteria for Oral Health Surveys. The following aspects of dental health/disease were considered: overall caries experience, treatment experience, unmet dental treatment needs and the presence of functional dentition. Data were collected from 76 patients with haemophilia among which 27 were children and 49 were adults and a control group of 76 subjects comprising 30 children and 46 adults. Children with haemophilia had a significantly lower overall caries experience and less unmet dental treatment needs in deciduous teeth as compared to healthy children. In permanent dentitions, overall caries experience, unmet dental treatment needs or treatment experience did not differ between cases and controls either in older children or adult cohorts. There were no differences between the study groups regarding the functional dentition‐related indices. Healthier deciduous teeth were observed in children with haemophilia than in children without haemophilia, but other dental health or disease‐related outcomes did not differ between cases and controls.     

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.     

Oral health status in children and adolescents with haemophilia

This case‐controlled study aimed to evaluate the existing oral health status in children and adolescents with haemophilia. A total of 50 haemophilia patients and 50 matched controls aged seven to 16 years were recruited into the study. Clinical examination was carried out to determine dental caries experience, oral hygiene status and gingival condition in these two groups. Information regarding previous dental history, oral hygiene practices and dietary habits were also obtained. No significant difference was found in mean caries experience in primary and secondary dentitions (P = 0.86 and 0.32) and in Simplified Oral Hygiene Index (OHI‐S, P = 0.20) between both groups. However, a significantly higher proportion of haemophilia patients (24%) had better oral hygiene status as compared to the controls (2%, P = 0.004). Furthermore, there was a significant difference in Modified Gingival Index (MGI, P = 0.02) between the two groups with the study group having less gingival inflammation. A total of 88% (n = 44) of the haemophilia patients were registered and received dental treatment in specialist dental clinics. More than half (56%, n = 28) had frequent dental visits and only one‐third of the haemophilia patients had history of hospitalization due to oral problems. There was no significant difference in oral hygiene practices and dietary habits between both groups. In general, haemophilia children and adolescents in this study had similar caries experience, a significantly better oral hygiene status and gingival health as compared to healthy controls. The main reason for this is the multidisciplinary approach implemented by medical health‐care professionals as primary care provider and the dental team.     

Functional tooth number and 15-year mortality in a cohort of community-residing older people

Background:   To study how dental status can become a predictor of overall mortality risk.
Methods:   Community residents ( n  = 5730) over 40 years old in the Miyako Islands, Okinawa Prefecture, Japan were followed up for 15 years, 1987–2002. Functional tooth numbers were examined by dentists and overall mortalities of subjects with functional tooth numbers of <10 and ≥10 were compared in the age groups 40–49, 50–59, 60–69, 70–79 and 80 years or more in both males and females.
Results:   Groups of 80 years or more showed a significantly higher rate of overall mortality in subjects with functional tooth numbers of less than 10 than 10 or more, and there was no significant difference in the other age groups.
Conclusion:   The present study suggests that systemic attention to dental status should be recommended in older males.