Fatal Mycobacterium colombiense/cytomegalovirus coinfection associated with acquired immunodeficiency due to autoantibodies against interferon gamma: a case report.

BACKGROUND: Reports of acquired immunodeficiency due to autoantibodies against interferon gamma in the adult population are increasing. The interleukin-12-dependent interferon-gamma axis is a major regulatory pathway of cell-mediated immunity and is critical for protection against a few intracellular organisms, including non-tuberculous mycobacteria and Salmonella spp. We report the first case of a fatal disseminated Mycobacterium colombiense/cytomegalovirus coinfection in an adult woman associated with the acquisition of autoantibodies against interferon-gamma. CASE PRESENTATION: A 49-year-old woman, born to nonconsanguineous parents in Laos, but who had lived in Canada for the past 30 years, presented with a 1-month history of weight loss, fatigue, cough, and intermittent low-grade fever. A thoracic computed tomography scan revealed an 8 × 7 cm irregular mass impacting the right superior lobar bronchus along with multiple mediastinal and hilar adenopathies. On the fourth day of admission, the patient developed fever with purulent expectorations. Treatment for a post-obstructive bacterial pneumonia was initiated while other investigations were being pursued. Almost every culture performed during the patient's hospitalization was positive for M. colombiense. Given the late presentation of symptoms - at the age of 49 years - and the absence of significant family or personal medical history, we suspected an acquired immunodeficiency due to the presence of anti-interferon-gamma autoantibodies. This was confirmed by their detection at high levels in the plasma and a STAT1 phosphorylation assay on human monocytes. The final diagnosis was immunodeficiency secondary to the production of autoantibodies against interferon-gamma, which resulted in a post-obstructive pneumonia and disseminated infection of M. colombiense. The clinical course was complicated by the presence of a multiresistant Pseudomonas aeruginosa post-endobronchial ultrasound mediastinitis, cytomegalovirus pneumonitis with dissemination, and finally, susceptible P. aeruginosa ventilator-associated pneumonia with septic shock and multiple organ failure, leading to death despite appropriate antibacterial and anti-mycobacterial treatment. CONCLUSIONS: Although rare, acquired immunodeficiency syndromes should be considered in the differential diagnosis of patients with severe, persistent, or recurrent infections. Specifically, severe non-tuberculous mycobacteria or Salmonella infections in adults without any other known risk factors may warrant examination of autoantibodies against interferon-gamma because of their increasing recognition in the literature.

[General practitioners in private practice in the city of Quebec and the application of population responsibility]. / Les médecins omnipraticiens de cabinet privé de la ville de Québec et l'application de la responsabilité populationnelle.

OBJECTIVES: The aim of this study is to examine the way in which general practitioners (GPs) in private practice view the idea of population responsibility, proposed by the Quebec Health and Social Services Ministry in 2004. We then look at how these views impact primary health care practice in Quebec City. METHOD: A qualitative exploratory approach was used; 18 semi-directed interviews were performed with private practice GPs, administrators and health professionals from community health and social services centres (CSSS). A content thematic analysis of the data was performed based on St-Pierre et al.'s model and grounded on Giddens' structuring theory. RESULTS: Because neither the population meant to be served nor the underlying responsibility are perceived the same way by the GPs and the CSSS health professionals, the respective practices do not always converge. Consequently, methods of communication, offers of services and management of resources impact on the operationalization of the concept of population responsibility, which has to be negotiated. DISCUSSION: In these negotiations, because physicians are the ones solicited by the other partners, the application of population responsibility increasingly becomes an opportunity to develop a medically oriented primary care organization.

Selecting process quality indicators for the integrated care of vulnerable older adults affected by cognitive impairment or dementia.

BACKGROUND: This study aimed at evaluating face and content validity, feasibility and reliability of process quality indicators developed previously in the United States or other countries. The indicators can be used to evaluate care and services for vulnerable older adults affected by cognitive impairment or dementia within an integrated service system in Quebec, Canada. METHODS: A total of 33 clinical experts from three major urban centres in Quebec formed a panel representing two medical specialties (family medicine, geriatrics) and seven health or social services specialties (nursing, occupational therapy, psychology, neuropsychology, pharmacy, nutrition, social work), from primary or secondary levels of care, including long-term care. A modified version of the RAND(R)/University of California at Los Angeles (UCLA) appropriateness method, a two-round Delphi panel, was used to assess face and content validity of process quality indicators. The appropriateness of indicators was evaluated according to a) agreement of the panel with three criteria, defined as a median rating of 7-9 on a nine-point rating scale, and b) agreement among panellists, judged by the statistical measure of the interpercentile range adjusted for symmetry. Feasibility of quality assessment and reliability of appropriate indicators were then evaluated within a pilot study on 29 patients affected by cognitive impairment or dementia. For measurable indicators the inter-observer reliability was calculated with the Kappa statistic. RESULTS: Initially, 82 indicators for care of vulnerable older adults with cognitive impairment or dementia were submitted to the panellists. Of those, 72 (88%) were accepted after two rounds. Among 29 patients for whom medical files of the preceding two years were evaluated, 63 (88%) of these indicators were considered applicable at least once, for at least one patient. Only 22 indicators were considered applicable at least once for ten or more out of 29 patients. Four indicators could be measured with the help of a validated questionnaire on patient satisfaction. Inter-observer reliability was moderate (Kappa = 0.57). CONCLUSION: A multidisciplinary panel of experts judged a large majority of the initial indicators valid for use in integrated care systems for vulnerable older adults in Quebec, Canada. Most of these indicators can be measured using patient files or patient or caregiver interviews and reliability of assessment from patient-files is moderate.

Field testing of two electronic mobility aid devices for persons who are deaf-blind.

PURPOSE: To test a methodology for assessing the effects of electronic mobility aid devices (EMAD) on the mobility of persons who are deaf-blind in real-life situations. METHOD: A single-subject desing was done with four users followed in a program for persons who are deaf-blind. Participants were trained to use two commercial EMADs: the Miniguide and the Breeze. The Canadian Measure of Occupational Performance (CMOP) was administered before training (T1), after training (T2) and three months later (T3). The Quebec User Evaluation of Satisfaction with assistive Technology (QUEST) was administered at T2 and T3. A follow-up interview was conducted with the participants and clinicians at T3 to evaluate the benefits of the EMAD and any problems. RESULTS: CMOP suggests that both performance and satisfaction improved following the use of both EMADs in four occupations (functional mobility, active leisure, community life and socialization). QUEST indicates high satisfaction with eight items except for one participant. Follow-up interviews revealed a wide variety of perceptions regarding EMAD use and seven problems although CMOP and QUEST indicated good satisfaction. CONCLUSION: A high satisfaction score on QUEST does not necessarily imply that an assistive device is efficient in all circumstances; follow-up interviews provided important complementary information. IMPLICATIONS FOR REHABILITATION: For people who are deaf-blind The Miniguide is reliable for detecting obstacles (vibrations are sent as a warning when approaching an obstacle). It is often used to know if taking the right route. It identifies entrances or openings (indicated by the vibrations stopping when pointing at a wall), which can replace the echo-location for blind persons with hearing impairments. The Miniguide helped to locate overhanging objects which are not detectable with a long cane. The Breeze can record landmarks for orientation and it possible to use those landmarks to go back alone later after taking a certain route for the first time with someone else. It helps to familiarize the person with new places. It can tell you where you are at any time. It helps with orientation and the participant also liked this feature because it helped with learning street names. The Miniguide and the Breeze were not efficient in all circumstances; there were some problems with the ergonomic (both), detecting snow banks (Miniguide), sensitiveness to the surroundings in crowded places (Miniguide) and exactitude for geo-localization (Breeze).