[Treatment of haemophilia in Austria]. / Hämophilie-Behandlung in Österreich.

This guideline is intended to provide practical guidance for the diagnosis and treatment of haemophilia in Austria. Few randomized controlled interventional trials are available addressing the treatment of haemophilia, therefore recommendations are usually based on low level of evidence and represent expert consensus.This guideline is based on the WFH guideline, published in 2020, and adapted according to the national circumstances and experience.It includes recommendations and suggestions for diagnosis and follow-up visits and pharmacological therapies for treatment and prophylaxis. Further topics comprise special aspects in children and adults with severe haemophilia, outcome measurement, and management of trauma, special bleedings and interventions, including dental procedures, inhibitors, management of haemophilia carriers, and psychosocial aspects.

Associations Between Parental Occupational Balance, Subjective Health, and Clinical Characteristics of VLBW Infants.

Objective: Very low birthweight (VLBW) infants have an increased risk of mortality and frequently suffer from complications, which affects parental occupational balance. Occupational balance is the satisfaction with one's meaningful activities, which include everyday activities that people need to, want to, and are expected to do. In contrast to work-life balance, the construct of occupational balance addresses different activities equally and it applies to all persons, regardless of whether they are working or not. Parental occupational balance might be related to parents' and VLBW infants' health. Therefore, the objective of this study was to investigate associations between parental occupational balance, subjective health, and clinical characteristics of VLBW infants. Methods: A cross-sectional multicenter study was conducted in six Austrian neonatal intensive care units. Occupational balance and subjective health of parents of VLBW infants were assessed with six self-reported questionnaires. The following clinical characteristics of VLBW infants were extracted from medical records: gestational age, birthweight, Apgar scores, Clinical Risk for Babies II Score, and complications of prematurity. Spearman's rank coefficients were calculated. Results: In total, 270 parents, 168 (62%) female and their VLBW infants, 120 (44%) female, were included in this study. Parents' mean age was 33.7 (±6.0) years, mean gestational age of VLBW infants was 27 + 3 (±2) weeks. Associations between parental occupational balance, subjective health, and clinical characteristics of VLBW infants were identified (r s = 0.13 - 0.56; p ≤ 0.05), such as the correlation between occupational areas (r s = 0.22, p ≤ 0.01), occupational characteristics (r s = 0.17, p = 0.01), and occupational resilience (r s = 0.18, p ≤ 0.01) with bronchopulmonary dysplasia of VLBW infants. Conclusion: Occupational balance is associated with parents' and VLBW infants' health. Interventions to strengthen parental occupational balance might increase parental health and thereby also improve health and developmental outcomes of their VLBW infants.

Nonneutralizing FVIII-specific antibody signatures in patients with hemophilia A and in healthy donors.

Previous studies identified nonneutralizing FVIII-specific antibodies in the circulation of severe and nonsevere hemophilia A (sHA and nsHA) patients without FVIII inhibitors and also in some healthy individuals. To gain a better understanding of the nature of these nonneutralizing antibody responses, we analyzed and compared anti-FVIII antibody signatures in 3 study cohorts: previously treated sHA as well as nsHA patients without FVIII inhibitors, and healthy donors. FVIII-binding IgM, IgG1-4, and IgA antibodies were differentiated, FVIII-specificity was assessed, and associated apparent affinity constants were determined. Our results indicate that the nonneutralizing FVIII-specific antibody response in all study cohorts is dominated by IgG1 and IgA. Prevalences, titers, and affinities of these nonneutralizing antibodies were higher in the hemophilia A cohorts than in healthy donors. Stratification for the anti-hepatitis C virus (HCV) antibody status demonstrated the presence of FVIII-specific IgA with elevated titers in sHA patients with an active or past HCV infection when compared with HCV antibody-positive nsHA patients or HCV antibody-negative patients and healthy donors. Increased titers and affinities of FVIII-specific IgG1 antibodies were observed in a considerable number of hemophilia A patients as opposed to healthy subjects independently of the patients' anti-HCV antibody status. Overall, our findings support the hypothesis that the generation of nonneutralizing anti-FVIII antibodies in healthy individuals and in noninhibitor hemophilia A patients might be based on similar immune mechanisms. However, differences in prevalences, titers, and affinities of these antibodies indicate distinct differences in the antibody evolution between healthy individuals and patients.

Development and validation of a self-reported questionnaire to assess occupational balance in parents of preterm infants.

BACKGROUND: Parents' meaningful activities (occupations) and occupational balance are relevant to neonatal care. Valid and reliable self-reported measurement instruments are needed to assess parents' occupational balance and to evaluate occupational balance interventions in neonatal care. The aims of this study were to develop a self-reported questionnaire on occupational balance in informal caregivers (OBI-Care) and to examine its measurement properties including construct validity and internal consistency. METHODS AND FINDINGS: A mixed method multicenter study design was employed. Items of the OBI-Care were created with parents of preterm infants based on qualitative research methods. Measurement properties were analyzed with quantitative data of parents of preterm infants. Construct validity was assessed by determining dimensionality, overall and item fit to a Rasch model, differential item functioning and threshold ordering. Internal consistency was examined by determining inter-item and item-total correlations, Cronbach's alpha and Rasch's person separation index. Fourteen parents participated in item creation. Measurement properties were explored in data of 304 parents. Twenty-two items, summarized in three subscales were compiled to the OBI-Care. Items showed an overall fit and except one item, an item fit to the Rasch model. There was no evidence of differential item functioning and all items displayed ordered thresholds. Each subscale had good values of person separation indices and Cronbach's alpha. CONCLUSIONS: The OBI-Care demonstrates construct validity and internal consistency and is thus a suitable measurement instrument to assess occupational balance of parents of preterm infants in neonatal care. OBI-Care is generic and can be applied in various health care settings.

A severe influenza season in Austria and its impact on the paediatric population: mortality and hospital admission rates, november 2017 - march 2018.

BACKGROUND: In Austria paediatric influenza-associated hospitalisations and deaths have never been systematically monitored. We examined the influenza season 2017/18 in terms of hospitalisation and mortality in the Austrian paediatric population and put the results into perspective of the available data from the last 15 years. METHODS: Cases of influenza-associated hospitalisations and deaths for season 2017/18 in children below 18 years were retrospectively collected from 12 and 33 Austrian hospitals, respectively. Hospitalisation and mortality rates for the whole Austrian paediatric population were estimated, adjusting for the population in each catchment area. Two Austrian databases were queried for hospitalisations and deaths associated with influenza during 2002-2016. Rough estimate of the vaccination coverage was calculated from a survey on 100 inpatients. RESULTS: Influenza-related paediatric hospitalisation rate in season 2017/18 was estimated as 128 (CI: 122-135) per 100,000 children, much higher than the national average of 40 per 100,000 over the years 2002-2016. There were nine reported influenza-associated deaths among children, resulting in mortality rate of 0.67 (CI: 0.32-1.21) per 100,000 children. CONCLUSIONS: Reported influenza-associated hospitalisations and fatalities demonstrate a high burden of influenza in the Austrian paediatric population corresponding with very low vaccination coverage.

Epidemiology and Treatment of Patients with Haemophilia in Austria-Update from the Austrian Haemophilia Registry.

The Austrian Haemophilia Registry collects epidemiological data on patients with haemophilia, on treatment modalities and potential side effects. The Registry covers more than 85% of the assumed total number of haemophilia patients in Austria. This report summarizes data on 753 patients: 84.3% (635) have haemophilia A and 15.7% (118) have haemophilia B. Patients' median age is 34 years (range: 1-93 years). Of the total cohort, 39.0% (294) patients have severe haemophilia, 11.3% (85) moderate haemophilia, and 49.4% (372) mild haemophilia. Of the patients with severe haemophilia, 38.4% (113) have been infected with hepatitis C virus (HCV) and 12.6% (37) are human immunodeficiency virus (HIV) positive. Overall, 10.6% (67) of patients with haemophilia A and 1.7% (2) of those with haemophilia B have had an inhibitor in their history. Among patients with severe haemophilia, 68.4% (201) receive prophylaxis and 28.6% (84) receive on-demand therapy. There are 65.0% (191) patients with severe haemophilia who are treated with recombinant products. In conclusion, most patients with severe haemophilia receive prophylactic treatment. HCV and HIV infections are still important issues in the Austrian haemophilia population.

[Guidelines for vaccination of immunocompromised individuals]. / Impfungen bei Immundefekten/Immunsuppression - Expertenstatement und Empfehlungen.

Immunosuppression of various origins is associated with an increased risk of infection; therefore the prevention of infectious diseases by vaccination is especially important in immunocompromised patients. However, the response to vaccinations is often reduced in these risk groups and the application of live vaccines is contraindicated during immunosuppression.In the following expert statement, recommendations for vaccination were created on the basis of current evidence and theoretical/immunological considerations. A first, general part elaborates on efficacy and safety of vaccinations during immunosuppression, modes of action of immunosuppressive medications and recommended time intervals between immunosuppressive treatments and vaccinations. A core piece of this part is a graduation of immunosuppression into three stages, i. e. no relevant immunosuppression, mild to moderate and severe immunosuppression and the assignment of various medications (including biologicals) to one of those stages; this is followed by an overview of possible and necessary vaccinations in each of those stages.The second part gives detailed vaccination guidelines for common diseases and therapies associated with immunosuppression. Primary immune deficiencies, chronic kidney disease, diabetes mellitus, solid and hematological tumors, hematopoetic stem cell transplantation, transplantation of solid organs, aspenia, rheumatological-, gastroenterologic-, dermatologic-, neurologic diseases, biologicals during pregnancy and HIV infection are dealt with.These vaccination guidelines, compiled for the first time in Austria, aim to be of practical help for physicians to facilitate and improve vaccination coverage in immunocompromised patients and their household members and contact persons.

Paediatricians require more information before they routinely co-administer the meningococcal B vaccine with routine infant vaccines.

AIM: The four-component meningococcal serogroup B (4CMenB) vaccine was licensed by the European Medicines Agency in 2013. We evaluated current practice regarding multiple vaccines and the attitudes of paediatricians towards the 4CMenB before it became available in Austria in 2014. METHODS: We sent 1624 Austrian paediatricians an email invitation to participate in our nationwide web-based survey and 231 responded. RESULTS: Most participants regarded the 4CMenB vaccine as a long-needed and necessary tool against meningococcal B disease. However, most participants would not co-administer this vaccine with other routine infant vaccines. The survey showed that 58.9% of paediatricians already co-administered the hexavalent vaccine with the pneumococcal conjugate vaccine, but most of them would not add a third vaccine at the same visit. This was mainly due to lack of experience with the vaccine and also because they assumed that parents would not consent. Importantly, paediatricians said they wanted an explicit recommendation in the Austrian Immunisation Plan on the timing of the 4CMenB vaccine before they would confidently and routinely use it for infants. CONCLUSION: Paediatricians required more information for themselves and for parents before routinely co-administering the 4CMenB vaccine. They also requested a national recommendation on its timing.

Intracranial hemorrhage and other symptoms in infants associated with human parechovirus in Vienna, Austria.

The human parechovirus (HPeV), mainly genotype 3, may cause severe illness in young infants and neonates, including sepsis-like illness and central nervous system (CNS) infection. We lack data concerning the impact and symptoms of HPeV infection in infants in Austria. The aim of the study is to evaluate the spectrum of symptoms and findings in infants with the parechovirus in Vienna and its environs. Patients younger than 3 months of age, with clinically suspected sepsis-like illness or CNS infection and a positive polymerase chain reaction (PCR) for HPeV, were included in the study. Medical records were analyzed retrospectively. Twenty patients were included in the study from 2009 to 2013. The most frequent manifestations were fever and neurological symptoms (89 and 80 %, respectively). Fifty percent of the infants had white blood cell counts out of range. The most notable aspect was cerebral hemorrhage in three neonates, which has not been reported earlier in association with HPeV infection. CONCLUSION: In Austria, HPeV is a relevant pathogen in sepsis-like disease in infants. The clinical presentation is similar to that described in other studies; cerebral hemorrhage is a new aspect. WHAT IS KNOWN: • Parechovirus infection can cause severe illness in infants. • Symptoms have been described to involve all organs; sepsis-like signs, fever, and irritability are most frequent. WHAT IS NEW: • Also in Austria, HPeV plays an important role in severe illnesses in infants. • Severe intracranial hemorrhage is described as a new finding.

Blood pressure in 57,915 pediatric patients who are overweight or obese based on five reference systems.

The aim of this study was to determine the prevalence of hypertension in overweight or obese pediatric subjects using different national or international references, which are based either on the entire population or on normal weight children only: 188 centers from Germany, Austria, and Switzerland participated in the Adipositas Patienten Verlaufsbeobachtung initiative. Data from 57,915 children aged 6 to 18 years who are overweight or obese were used to determine the prevalence of prehypertension and hypertension based on Second Task Force, European pooled data, Fourth Report all and Fourth Report nonoverweight, or German Health Interview and Examination Survey for Children and Adolescents (KiGGS) references. Three references included overweight children, whereas 2 (Fourth Report nonoverweight and KiGGS) were based on nonoverweight children only. Based on KiGGS, Fourth Report nonoverweight, Fourth Report all, European pooled data, or Second Task Force, the prevalence of hypertension was 47%, 42%, 36%, 32%, and 27%, respectively. Recent references classified more children as hypertensive, whereas fewer children fell into the prehypertensive group. Only 22% of children were classified as hypertensive by each of the 5 references (8% as prehypertensive). The prevalence of normal blood pressure was independent of the reference applied. Hypertension as defined by the different reference systems was significantly correlated, and all methods were significantly associated with impaired glucose metabolism or dyslipidemia, without significant differences in methods. In conclusion, the diagnosis of elevated blood pressure depends on the reference population used. A nonoverweight reference population substantially increases the prevalence of hypertension in children and adolescents who are overweight or obese. The choice of the reference has significant implications for risk stratification and treatment decisions.

[Vaccination recommendations for health care workers in Austria]. / Impfungen für Mitarbeiter des Gesundheitswesens.

In Austria the vaccination coverage among health care workers (HCW) - particularly among hospital personnel - is not sufficient. This is of specific concern, because not only the individual protection but also the prevention of disease transmission of vaccine preventable diseases between HCW and patients needs to be guaranteed. Particularly immunosuppressed patients, who are at higher risk for morbidity and mortality due to certain infections, but cannot be vaccination themselves, must be able to rely on herd protection, i.e. not being infected by surrounding/caring persons. The following publication provides for the first time detailed guidelines for vaccination programs for HCWs in Austria, including personnel within hospitals, medical institutions and laboratories, as well as Medical Universities including students. Moreover, these guidelines are also recommended to medical personnel in outpatient clinics, social service institutions and medical practices. Additionally to the vaccination schedules this publication also includes a chapter on ethical as well as legal background underlying these recommendations.

The key role of psychosocial risk on therapeutic outcome in obese children and adolescents. Results from a longitudinal multicenter study.

OBJECTIVE: Childhood obesity is high on the global public health agenda. Although risk factors are well known, the influence of social risk on the therapeutic outcome of lifestyle intervention is poorly examined. This study aims to investigate the influence of migration background, low education, and parental unemployment. METHODS: 62,147 patients participated in multidimensional lifestyle intervention programs in 179 pediatric obesity centers. Data were collected using standardized software for longitudinal multicenter documentation. 12,305 (19.8%) attended care for 6-24 months, undergoing an intensive therapy period and subsequent follow-ups for up to 3 years. A cumulative social risk score was calculated based on different risk indicators. RESULTS: Migration background, low education, and parental employment significantly influenced the outcome of lifestyle intervention. The observed BMI-SDS reduction was significantly higher in the subgroup with low social risks factors (Δ BMI-SDS -0.19) compared to those presenting moderate (Δ BMI-SDS -0.14) and high social risk (Δ BMI-SDS -0.11). CONCLUSION: Our data underline the effect of children's social setting on the outcome of multidimensional lifestyle intervention. The presence of a high social risk burden is a negative predictor for successful weight loss. Specific therapeutic programs need to be developed for disadvantaged children and adolescents.

Effect of obesity on plasma clusterin, [corrected] a proposed modulator of leptin action.

Clusterin, a protein constituent of HDL, was recently shown to bind plasma leptin in vitro and has been proposed to modulate leptin activity. To gain insight into a possible role for plasma clusterin in human obesity, we measured plasma clusterin, leptin, soluble leptin receptor (sObR), and lipoproteins in 70 obese adolescents (12.4 ± 1.6 y; BMI-SD score (SDS-BMI) 2.35 ± 0.47) before and after 3 wk of weight reduction in a dietary camp and in 44 normal weight controls. Binding of plasma leptin to HDL or clusterin was studied using ultracentrifugation and immunoaffinity chromatography. During weight reduction, clusterin decreased from 14.6 ± 4.1 to 10.3 ± 2.9 mg/dL, p < 0.001) in obese adolescents, whereas sObR increased. However, baseline plasma clusterin in obese adolescents did not differ from controls. Clusterin did not correlate with SDS-BMI, weight loss, leptin, or lipoproteins. Only ∼ 1% of plasma leptin was associated with clusterin/apoA-I complexes or with HDL. Our results do not support a role for plasma clusterin as an important leptin-binding protein or modulator of leptin action. The decrease of plasma clusterin during weight reduction may be an effect of the hypocaloric diet rather than being directly linked to weight loss.

[FSME monitoring: monitoring of adverse events of tick-borne-encephalitis vaccines by selected paediatricians and general practitioners]. / FSME-Monitoring: Monitoring von Impfnebenwirkungen und -reaktionen auf die FSME-Impfung durch ausgewählte niedergelassene Pädiater und Allgemeinmediziner.

The Institute for Vaccine-Safety of the Austrian Green Cross, the so-called "Institut für Impfsicherheit des Osterreichischen Grünen Kreuzes", promoted a postmarketing sentinel study referring to the safety of tick-borne-encephalitis vaccines in spring 2002. Five-hundred selected paediatricians and general practitioners took part and generated data on 25,905 administered doses. They reported on 107/25,907 (0.413 %) local and systemic adverse reactions. The leading side-effect was fever, besides other adverse reactions corresponding to those known in the literature. The age-group most often affected was that of toddlers and infants. Monitoring with the help of selected paediatricians and general practitioners, carried out alongside the routine obligatory procedures of monitoring, proved to be a valid instrument for detecting vaccine adverse events of tick-borne-encephalitis vaccines.

Active hospital-based surveillance of rotavirus diarrhea in Austrian children, period 1997 to 2003.

BACKGROUND: Rotavirus is the most common pathogen causing severe dehydrating diarrhea in infants and young children worldwide. Any decision on implementation of rotavirus vaccination will be strongly influenced by the expected reduction in severe and therefore costly outcomes associated with rotavirus infection. The aim of this study was to provide data on hospitalization of young children with rotavirus infection in Austria. METHODS: The data were derived from active hospital-based sentinel surveillance for rotavirus during the period 1997 to 2003. RESULTS: During this period 25,600 children<15 years of age were hospitalized with acute laboratory-confirmed rotavirus gastroenteritis, the infection showing seasonal peaks between February and March. In 5 % of the cases first symptoms of diarrhea occurred at a minimum of 48 hours after hospital admission, indicating healthcare-associated origin of infection. The mean annual incidence of hospitalization per 100,000 population for the age group<5 years was 766 and for those<2 years 1742, the latter meaning that 1 in 60 Austrian children up to 2 years of age required hospitalization. An average peak incidence was observed between 8 and 14 months of age, with an average of 68% of the reported cases occurring in children aged

Death in two female Prader-Willi syndrome patients during the early phase of growth hormone treatment.

UNLABELLED: Reports on sudden death in Prader-Willi syndrome (PWS) patients after the start of growth hormone (GH) treatment have been published recently. We observed a 4.7-y-old girl who showed a continuous increase in pulmonary artery pressure and died of cardiorespiratory failure 7 wk after GH therapy had been initiated, and a 9.3-y-old girl with additional trisomy 21 who died during a minor respiratory infection 6 mo after GH had been started. Both patients were overweight (weight for height 127% and 224%, respectively). GH-induced fluid retention may have occurred in the younger girl. In contrast to the reported cases, our PWS patients were female. CONCLUSION: Our cases illustrate the difficulty of differentiation between possible GH side effects and the natural course of disease, in particular with respect to obesity-related comorbidity and mortality.

Influenza A virus infection and cardiac arrhythmia during the neonatal period.

Influenza virus infection during pregnancy increases the risk of spontaneous abortion, preterm contractions, fetal compromise and early neonatal mortality. We describe a case of fetal and neonatal cardiac arrhythmia coinciding with an influenza A virus infection. Viral RNA was detected in the neonate's nasopharyngeal secretions and also in its serum, indicating influenza viremia. The cardiac arrhythmia resolved spontaneously without antiarrhythmic treatment.