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Lam S.,Alberta Childrens Hospital | Kavadas F.D.,Section of Allergy and Immunology | Haider S.,Alberta Childrens Hospital | Noseworthy M.E.,Section of Respiratory Medicine
Canadian Respiratory Journal | Year: 2014

Severe combined immunodeficiency (SCID) is considered to be a pediatric emergency, with respiratory distress being the most common presenting symptom. The authors present two cases of SCID in children <4 months of age with respiratory distress at a tertiary care centre due to a recently described homozygous CD3 delta mutation found only in the Mexican Mennonite population. Failure to respond to broad-spectrum antibiotics prompted investigation for possible SCID. Bronchial alveolar lavage fluid from both patients grew Pneumocystis jiroveci, and flow cytometry revealed absent T cells. The CD3 delta gene is believed to be important in T cell differentiation and maturation. The present article reminds pediatricians and pediatric respirologists that the key to diagnosing SCID is to have a high index of suspicion if there is poor response to conventional therapies. ©2014 Pulsus Group Inc. All rights reserved.

Chalhoub N.,New York Medical College | Kamel H.,Section of Radiology | Ehlayel M.,Section of Allergy and Immunology | Ibrahim N.,National Research Center of Egypt | And 6 more authors.
Clinical Genetics | Year: 2015

We present a Qatari family with two children who displayed a characteristic phenotype of congenital marked pain insensitivity with hypohidrosis and progressive aseptic destruction of joints and vertebrae resembling that of hereditary sensory and autonomic neuropathies (HSANs). The patients, aged 10 and 14, remained of uncertain genetic diagnosis until whole genome sequencing was pursued. Genome sequencing identified a novel homozygous C65S mutation in the LIFR gene that is predicted to markedly destabilize and alter the structure of a particular domain and consequently to affect the functionality of the whole multi-domain LIFR protein. The C65S mutant LIFR showed altered glycosylation and an elevated expression level that might be attributed to a slow turnover of the mutant form. LIFR mutations have been reported in Stüve-Wiedemann syndrome (SWS), a severe autosomal recessive skeletal dysplasia often resulting in early death. Our patients share some clinical features of rare cases of SWS long-term survivors; however, they also phenocopy HSAN due to the marked pain insensitivity phenotype and progressive bone destruction. Screening for LIFR mutations might be warranted in genetically unresolved HSAN phenotypes. © 2015 John Wiley & Sons A/S.

Yang C.-T.,Tunghai University | Liao C.-J.,Tunghai University | Liu J.-C.,Tunghai University | Den W.,Tunghai University | And 2 more authors.
Journal of Medical Systems | Year: 2014

Indoor air quality monitoring in healthcare environment has become a critical part of hospital management and policy. Manual air sampling and analysis are cost-inhibitive and do not provide real-time air quality data and response measures. In this month-long study over 14 sampling locations in a public hospital in Taiwan, we observed a positive correlation between CO 2 concentration and population, total bacteria, and particulate matter concentrations, thus monitoring CO2 concentration as a general indicator for air quality could be a viable option. Consequently, an intelligent environmental monitoring system consisting of a CO 2/temperature/humidity sensor, a digital plug, and a ZigBee Router and Coordinator was developed and tested. The system also included a backend server that received and analyzed data, as well as activating ventilation and air purifiers when CO2 concentration exceeded a pre-set value. Alert messages can also be delivered to offsite users through mobile devices. © 2014 Springer Science+Business Media New York.

Huang P.-Y.,Tunghai University | Shi Z.-Y.,Section of Infectious Diseases | Chen C.-H.,Tunghai University | Den W.,Tunghai University | And 2 more authors.
Aerosol and Air Quality Research | Year: 2013

Samples of airborne and surface-bound microbial contamination were taken in two intensive care units of a large-scale medical center. Microbial analyses included total bacterial and fungal loads, as well as the four bacterial species of Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa and Acinetobacter baumannii. Among the 114 surface samples taken from seven designated surface locations of room and equipment for each patient, P. aeruginosa was the most frequently detected (19.3%) and most abundant (mean count: 4.18 cfu/plate) bacterium, whereas the respirator represented the most heavily contaminated surface location in both total pathogenic bacteria colony counts (272 cfu) and frequency of positive detection (38.2%). P. aeruginosa also represented the most frequently detected (39.1%, n = 46) and abundant (11.52 ± 17.16 cfu/m3) bacterium in the air samples (n = 46), and was the only bacterium exhibiting a positive correlation of the mean counts between air and surface samples. The data analysis results further suggested a higher value of relative risk among the infected patients in the presence of the pathogens as compared to those in the absence of them, although the evidence of a of correlation for the individual bacterial species between the environmental samples and infected patients was inconclusive. This study also found that the mean airborne counts and the detection frequencies of these bacteria after patient visitation periods were higher than those before visitation, and that the installation of local air ionizers did not lead to any discernible differences in total bacterial and fungal concentrations. © Taiwan Association for Aerosol Research.

Shaker M.S.,Dartmouth Hitchcock Medical Center | Gruenberg D.A.,Section of Allergy and Immunology
Current Opinion in Pediatrics | Year: 2013

Purpose of review Allergic reactions to stinging insects may be unexpected, frightening, and severe. A clear understanding of recent advances in the field facilitates appropriate care of children who experience severe reactions to hymenoptera stings. Recent findings Recent investigations have underscored the importance of appropriate patient selection for potentially lifesaving venom immunotherapy. Venom immunotherapy is effective in preventing future anaphylaxis from hymenoptera stings. Immunotherapy is indicated for patients with a history of anaphylaxis. Children who develop large local swelling or strictly cutaneous systemic reactions generally do not require immunotherapy. Component resolved diagnostic testing has been investigated to clarify the possibility of multiple venom allergies in patients with sensitization to multiple venoms. Summary Rapid recognition and treatment of anaphylaxis are critical. Subsequent education about avoiding future stings and attention to emergency preparedness with appropriate prescription of self-injectable epinephrine is important. Referral of patients who have experienced venom-associated anaphylaxis for possible venom immunotherapy can prevent future severe episodes of anaphylaxis resulting from stings. © 2013 Wolters Kluwer Health / Lippincott Williams & Wilkins.

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