Severe influenza cases in paediatric intensive care units in Germany during the pre-pandemic seasons 2005 to 2008

<p>Abstract</p> <p>Background</p> <p>Data on complications in children with seasonal influenza virus infection are limited. We initiated a nation-wide three-year surveillance of children who were admitted to a paediatric intensive care unit (PICU) with severe seasonal influenza.</p> <p>Methods</p> <p>From October 2005 to July 2008, active surveillance was performed using an established reporting system for rare diseases (ESPED) including all paediatric hospitals in Germany. Cases to be reported were hospitalized children < 17 years of age with laboratory-confirmed influenza treated in a PICU or dying in hospital.</p> <p>Results</p> <p>Twenty severe influenza-associated cases were reported from 14 PICUs during three pre-pandemic influenza seasons (2005-2008). The median age of the patients (12 males/8 females) was 7.5 years (range 0.1-15 years). None had received vaccination against influenza. In 14 (70%) patients, the infection had been caused by influenza A and in five (25%) by influenza B; in one child (5%) the influenza type was not reported. Patients spent a median of 19 (IQR 12-38) days in the hospital and a median of 11 days (IQR 6-18 days) in the PICU; 10 (50%) needed mechanical ventilation. Most frequent diagnoses were influenza-associated pneumonia (60%), bronchitis/bronchiolitis (30%), encephalitis/encephalopathy (25%), secondary bacterial pneumonia (25%), and ARDS (25%). Eleven (55%) children had chronic underlying medical conditions, including 8 (40%) with chronic pulmonary diseases. Two influenza A- associated deaths were reported: <it>i) </it>an 8-year old boy with pneumococcal encephalopathy following influenza infection died from cerebral edema, <it>ii) </it>a 14-year-old boy with asthma bronchiale, cardiac malformation and Addison's disease died from cardiac and respiratory failure. For nine (45%) patients, possibly permanent sequelae were reported (3 neurological, 3 pulmonary, 3 other sequelae).</p> <p>Conclusions</p> <p>Influenza-associated pneumonia and secondary bacterial infections are relevant complications of seasonal influenza in Germany. The incidence of severe influenza cases in PICUs was relatively low. This may be either due to the weak to moderate seasonal influenza activity during the years 2005 to 2008 or due to under-diagnosis of influenza by physicians. Fifty% of the observed severe cases might have been prevented by following the recommendations for vaccination of risk groups in Germany.</p

The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort.

PURPOSE: It has been argued that rare diseases should be recognized as a public health priority. However, there is a shortage of epidemiological data describing the true burden of rare diseases. This study investigated hospital service use to provide a better understanding of the collective health and economic impacts of rare diseases. METHODS: Novel methodology was developed using a carefully constructed set of diagnostic codes, a selection of rare disease cohorts from hospital administrative data, and advanced data-linkage technologies. Outcomes included health-service use and hospital admission costs. RESULTS: In 2010, cohort members who were alive represented approximately 2.0% of the Western Australian population. The cohort accounted for 4.6% of people discharged from hospital and 9.9% of hospital discharges, and it had a greater average length of stay than the general population. The total cost of hospital discharges for the cohort represented 10.5% of 2010 state inpatient hospital costs. CONCLUSIONS: This population-based cohort study provides strong new evidence of a marked disparity between the proportion of the population with rare diseases and their combined health-system costs. The methodology will inform future rare-disease studies, and the evidence will guide government strategies for managing the service needs of people living with rare diseases.Genet Med advance online publication 22 September 2016Genetics in Medicine (2016); doi:10.1038/gim.2016.143

Child and family experiences with inborn errors of metabolism: a qualitative interview study with representatives of patient groups

© 2015, The Author(s). Background: Patient-centered health care for children with inborn errors of metabolism (IEM) and their families is important and requires an understanding of patient experiences, needs, and priorities. IEM-specific patient groups have emerged as important voices within these rare disease communities and are uniquely positioned to contribute to this understanding. We conducted qualitative interviews with IEM patient group representatives to increase understanding of patient and family experiences, needs, and priorities and inform patient-centered research and care. Methods: We developed a sampling frame of patient groups representing IEM disease communities from Canada, the United States, and United Kingdom. With consent, we interviewed participants to explore their views on experiences, needs, and outcomes that are most important to children with IEM and their families. We analyzed the data using a qualitative descriptive approach to identify key themes and sub-themes. Results: We interviewed 18 organizational representatives between February 28 and September 17, 2014, representing 16 IEMs and/or disease categories. Twelve participants voluntarily self-identified as parents and/or were themselves patients. Three key themes emerged from the coded data: managing the uncertainty associated with raising and caring for a child with a rare disease; challenges associated with the affected child’s life transitions, and; the collective struggle for improved outcomes and interventions that rare disease communities navigate. Conclusion: Health care providers can support children with IEM and their families by acknowledging and reducing uncertainty, supporting families through children’s life transitions, and contributing to rare disease communities’ progress toward improved interventions, experiences, and outcomes.The study was partially funded by the Rare Disease Foundation (RDF). In-kind support was provided by the Canadian Inherited Metabolic Diseases Research Network (CIMDRN) which is funded by the Canadian Institutes of Health Research (CIHR, grant TR3-119197) and administered by the University of Ottawa

Control of vaccine preventable diseases in Australian infants: reviewing a decade of experience with DTPa-HBV-IPV/Hib vaccine

The combined vaccine against diphtheria, tetanus, pertussis, hepatitis B, poliomyelitis, and Haemophilus influenzae b (DTPa-HBV-IPV/Hib, Infanrix Hexa, GSK) has been used for childhood immunization in Australia according to a two-, four-, six-month schedule since 2009. We reviewed data available in the Australian National Notifiable Diseases Surveillance System, annual vaccination coverage reports, the Database of Adverse Event Notifications, and peer-reviewed literature to assess vaccine coverage rates, incidence of all six vaccine preventable diseases, and the safety profile of DTPa-HBV-IPV/Hib vaccine in Australian infants over a period of ten years of exclusive use. Between 2009 and 2018 vaccine coverage for infants aged 12 months increased from 91.7% to 94.0% and from 84.9% to 92.6% for all and for Indigenous infants, respectively. Over the same time period, there were no reports of poliomyelitis, diphtheria or tetanus in infants <12 months of age. The incidence of hepatitis B among Australian infants <12 months of age remains 10 to 20-fold lower than the national average. Control of Haemophilus influenzae b (Hib) and pertussis disease has continued to be challenging. Timely administration of the primary series, as well as increasing coverage rates, particularly among Indigenous children, has contributed to improvements in Hib and pertussis disease control. The incorporation of additional strategies such as adjustment of the first vaccination encounter to six weeks of age, parental cocooning, and most recently maternal vaccination has further reduced the burden of pertussis, particularly during the first six months of life. The frequency of the ten most common adverse events related to the DTPa-HBV-IPV/Hib vaccine demonstrates an acceptable safety profile. Data collected over ten years of consistent, exclusive use of the DTPa-HBV-IPV/Hib vaccine in Australia highlights combination vaccination as a cornerstone in maintaining infant health

Applications of transcranial Doppler in the ICU: a review

Objective: Transcranial Doppler (TCD) ultrasonography is a technique that uses a hand-held Doppler transducer (placed on the surface of the cranial skin) to measure the velocity and pulsatility of blood flow within the intracranial and the extracranial arteries. This review critically evaluates the evidence for the use of TCD in the critical care population. Discussion: TCD has been frequently employed for the clinical evaluation of cerebral vasospasm following subarachnoid haemorrhage (SAH). To a lesser degree, TCD has also been used to evaluate cerebral autoregulatory capacity, monitor cerebral circulation during cardiopulmonary bypass and carotid endarterectomies and to diagnose brain death. Technological advances such as M mode, colour Doppler and three-dimensional power Doppler ultrasonography have extended the scope of TCD to include other non-critical care applications including assessment of cerebral emboli, functional TCD and the management of sickle cell disease. Conclusions: Despite publications suggesting concordance between TCD velocity measurements and cerebral blood flow there are few randomized controlled studies demonstrating an improved outcome with the use of TCD monitoring in neurocritical care. Newer developments in this technology include venous Doppler, functional Doppler and use of ultrasound contrast agents