A Constellation of Rare Findings in a Case of Goldenhar Syndrome

An 18-month-old child presented with right macrostomia, bilateral preauricular skin tags, bilateral CTEV, squint in bilateral eyes, thoracic vertebral anomalies, right sided aortic arch, and associated left pulmonary agenesis. The patient did not have any associated respiratory symptoms. Ipsilateral pulmonary agenesis is considered as a rare association with Goldenhar syndrome and a case of contralateral pulmonary aplasia has been described as an even rarer association.

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Outcomes of external septorhinoplasty in a Turkish male population

Publication date: Available online 20 May 2017
Source:Brazilian Journal of Otorhinolaryngology
Author(s): Mustafa Çelik, Gamze Didem Kilci, Engin Başer, Ayşegül Verim, Ömer Faruk Çalim, Bayram Veyseller, Orhan Özturan, Ahmet Altintaş
IntroductionThe first and one of the most important steps in facial plastic surgery is accurate preoperative facial analysis and recording of data that may help the surgeon to check the outcomes of his/her techniques, promoting a surgeon's professional development.ObjectiveTo evaluate the esthetic outcomes of external septorhinoplasty (SRP) relevant to ethnic facial harmony and to investigate the relationship of the columellar incision scar with the type of skin and columellar incision type in a Turkish population.MethodsIn total, 28 consecutive adult male patients with a mean age of 32.14±10.66 years (range: 18–61 years) were included the study. Primary outcomes were preoperative and postoperative photogrammetric facial analyses of the patients including measurement of nasofrontal angle, nasolabial angle and nasal projection ratios (Gode) assessed according to the data derived from the Rhinobase program. Results were compared to facial proportions of the Turkish population. Columellar incision scar scores related to the Fitzpatrick skin type classification of the patients and columellar incision types used for the external approach were secondary outcomes of the study.ResultsMean preoperative and postoperative nasofrontal angles were 148.04°±8.18° and 144.50°±7.15°, respectively, while mean preoperative and postoperative nasolabial angles were 87.59°±14.01° and 98.50°±9.71°, respectively. Mean preoperative and postoperative nasal tip projection ratios were 0.56±0.05 and 0.60±0.06, respectively. The differences between pre- and postoperative measurements were all significantly different and were in accordance with Turkish nasal harmony. Columellar inverted "V" incisions were performed in 15 (53.6%) patients while "V" incisions were used in 13 (46.4%) patients. Fitzpatrick skin Type 4 was seen in 46.42% of the patients, Fitzpatrick Type 3 in 46.42% and Fitzpatrick Type 2 in 7.14% of the patients. No significant difference was seen between columellar scar scores according to skin type and columellar incision type used for external septorhinoplasty.ConclusionsThis study demonstrated that outcomes for nasofrontal angle, nasolabial angle and nasal tip projection ratios analyzed using the Rhinobase program in patients who underwent external septorhinoplasty were similar to reference values for the Turkish population.



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Effect of vitamin D replacement on immunological biomarkers in patients with multiple sclerosis

Publication date: Available online 20 May 2017
Source:Clinical Immunology
Author(s): May F. Mrad, Nabil K. El Ayoubi, Maria O. Esmerian, Jalal M. Kazan, Samia J. Khoury
We aimed to investigate the immunologic effects of vitamin D replacement in RRMS patients. In a controlled single center study, patients deficient in 25-hydroxyvitamin D (serum level<25ng/ml) received 10,000IU/week cholecalciferol for 3months. Sufficient vitamin D patients (serum level>35ng/ml) were followed for the same period. Assessments were performed at baseline and at 3months. 25-hydroxyvitamin D levels increased significantly from baseline to month-3 in the deficient group after treatment and remained stable in the sufficient group. We observed a decreased interferon-γ (IFNγ) secretion by CD4⁺ T cells in vitamin D deficient group but not in the sufficient group, and a negative correlation between baseline serum vitamin D and IFNγ production. There was no change in the frequency of T helper or regulatory T cell subsets in either group. Increasing serum levels of 25-hydroxyvitamin D are associated with decreased production of IFNγ by CD4⁺ T cells.



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Income is an independent risk factor for worse asthma outcomes

Publication date: Available online 20 May 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Juan Carlos Cardet, Margee Louisias, Tonya S. King, Mario Castro, Christopher D. Codispoti, Ryan Dunn, Linda Engle, B. Louise Giles, Fernando Holguin, John J. Lima, Dayna Long, Njira Lugogo, Sharmilee Nyenhuis, Victor E. Ortega, Sima Ramratnam, Michael E. Wechsler, Elliot Israel, Wanda Phipatanakul
BackgroundSocioeconomic status (SES) is associated with asthma morbidity in observational studies but the factors underlying this association are uncertain.ObjectiveWe investigated whether three SES correlates—low income, low education, and high perceived stress— were independent risk factors for treatment failure and asthma exacerbations in the context of a randomized controlled trial (RCT).MethodsThe effect of low SES [household income (defined as <$50,000/year), household educational level (defined as less than a bachelor's degree) and high perceived stress (defined as a score of >20 on a perceived stress scale)] on asthma morbidity was analyzed in 381 participants utilizing Poisson regression models. The primary outcome was treatment failure (defined in the trial protocol as a significant clinical or airflow deterioration) and the secondary outcome was asthma exacerbations requiring systemic corticosteroids.Results54% of participants had a low income, 40% had a low educational level, and 17% had high perceived stress levels. Even after adjusting for race and other important confounders, participants with lower income had higher rates of both treatment failures [RR=1.6, 95%CI 1.1-2.3, p=0.03] and exacerbations [RR=1.9, 95%CI 1.1-3.3, p=0.02]. Adherence with inhaled corticosteroids was similarly high for both income categories. Education and perceived stress were not significantly associated with either outcome.ConclusionsIn the context of a RCT, participants with lower income were more likely to experience adverse asthma outcomes independent of education, perceived stress, race, and medication adherence.

Teaser

Asthma outcomes are worse in low income groups, regardless of race, BMI, perceived stress, inhaled corticosteroid dose or adherence, baseline asthma control, and second-hand smoke exposure. This suggests that clinicians and researchers should consider income when managing patients and designing interventions.


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Frequency of untreated hypogammaglobulinemia in bronchiectasis

Publication date: Available online 20 May 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Melanie A. Ruffner, Timothy R. Aksamit, Byron Thomashow, Radmila Choate, Angela DiMango, Gerard M. Turino, Anne E. O'Donnell, Margaret M. Johnson, Kenneth N. Olivier, Kevin Fennelly, Charles L. Daley, Kevin L. Winthrop, Mark L. Metersky, Matthias A. Salathe, Michael R. Knowles, M. Leigh Anne Daniels, Peadar G. Noone, Gregory Tino, David E. Griffith, Kathleen E. Sullivan




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A survey of inpatient practitioner knowledge of penicillin allergy at 2 community teaching hospitals

Publication date: Available online 20 May 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Mary L. Staicu, Dipekka Soni, Kelly M. Conn, Allison Ramsey
BackgroundThe negative effect of the penicillin allergy label on antibiotic use and patient outcomes has brought to light the need for thorough penicillin allergy assessments and heightened practitioner education.ObjectiveTo evaluate practitioner knowledge of penicillin allergy and the clinical approach to the patients with penicillin allergy.MethodsAn electronic survey was distributed to attending physicians, residents, pharmacists, nurse practitioners, and physician assistants practicing adult inpatient medicine at 2 community-based teaching hospitals from February to April 2016.ResultsA total of 276 (39%) of 716 practitioners completed surveys were analyzed. Most respondents were attending physicians (45%) with more than 10 years of experience (53%). Approximately half of the respondents indicated that they were unfamiliar with the rate of cross-reactivity between penicillin and cephalosporin (46%), carbapenem (42%), and monobactam (48%) antibiotics. When evaluating the role of penicillin skin testing and temporary induction of drug tolerance in the case vignettes, only 41% and 19% of respondents appropriately considered these options as the leading antibiotic management plan, respectively. Despite acknowledging the need for allergy/immunology consultation in clinical scenarios, 86% of respondents indicated that they never consult an allergist or immunologist or do so only once per year. Overall, pharmacists had a better understanding of the natural history of penicillin allergy and antibiotic cross-reactivity (P < .05).ConclusionThere is an overall limited understanding of the management of patients with a history of penicillin allergy in the hospital setting, where collaborative efforts between allergy and nonallergy health care practitioners are sparse. The expansion of a multidisciplinary approach may optimize antimicrobial prescribing in this subset of patients.



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The interplay between neuroendocrine activity and psychological stress-induced exacerbation of allergic asthma

Publication date: Available online 20 May 2017
Source:Allergology International
Author(s): Tomomitsu Miyasaka, Kaori Dobashi-Okuyama, Tomoko Takahashi, Motoaki Takayanagi, Isao Ohno
Psychological stress is recognized as a key factor in the exacerbation of allergic asthma, whereby brain responses to stress act as immunomodulators for asthma. In particular, stress-induced enhanced type 2 T-helper (Th2)-type lung inflammation is strongly associated with asthma pathogenesis. Psychological stress leads to eosinophilic airway inflammation through activation of the hypothalamic-pituitary-adrenal pathway and autonomic nervous system. This is followed by the secretion of stress hormones into the blood, including glucocorticoids, epinephrine, and norepinephrine, which enhance Th2 and type 17 T-helper (Th17)-type asthma profiles in humans and rodents. Recent evidence has shown that a defect of the μ-opioid receptor in the brain along with a defect of the peripheral glucocorticoid receptor signaling completely disrupted stress-induced airway inflammation in mice. This suggests that the stress response facilitates events in the central nervous and endocrine systems, thus exacerbating asthma. In this review, we outline the recent findings on the interplay between stress and neuroendocrine activities followed by stress-induced enhanced Th2 and Th17 immune responses and attenuated regulatory T (Treg) cell responses that are closely linked with asthma exacerbation. We will place a special focus on our own data that has emphasized the continuity from central sensing of psychological stress to enhanced eosinophilic airway inflammation. The mechanism that modulates psychological stress-induced exacerbation of allergic asthma through neuroendocrine activities is thought to involve a series of consecutive pathological events from the brain to the lung, which implies there to be a "neuropsychiatry phenotype" in asthma.



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