Severe dapsone hypersensitivity syndrome in a child

Korean J Pediatr > 2013 June;56(6) > Article

CASE REPORT

Korean J Pediatr 2013 June;56(6) :260-264. Published online 2012 October 25.        doi:http://dx.doi.org/10.3345/kjp.2013.56.6.260

Severe dapsone hypersensitivity syndrome in a child So Yoon Choi1, Ho Yeon Hwang1, Jung Hyun Lee1, Jae Sun Park1, Min Soo Jang2 1Departments of Pediatrics Kosin University Gospel Hospital, Busan, Korea 2Departments of Dermatology, Kosin University Gospel Hospital, Busan, Korea Corresponding Author: Jae Sun Park ,Tel: +82-51-990-6230, Fax: +82-51-990-3005, Email: pjs@ns.kosinmed.or.kr Copyright © 2013 by The Korean Pediatric Society Share :    ABSTRACT Dapsone (4,4'-diaminodiphenylsulfone, DDS), a potent anti-inflammatory agent, is widely used in the treatment of leprosy and several chronic inflammatory skin diseases. Dapsone therapy rarely results in development of dapsone hypersensitivity syndrome, which is characterized by fever, hepatitis, generalized exfoliative dermatitis, and lymphadenopathy. Here, we describe the case of an 11-year-old Korean boy who initially presented with high fever, a morbilliform skin rash, generalized lymphadenopathy, hepatosplenomegaly, and leukopenia after 6 weeks of dapsone intake. Subsequently, he exhibited cholecystitis, gingivitis, colitis, sepsis, aseptic meningitis, disseminated intravascular coagulation, syndrome of inappropriate antidiuretic hormone secretion, pneumonia, pleural effusions, peritonitis, bronchiectatic changes, exfoliative dermatitis, and acute renal failure. After 2 months of supportive therapy, and prednisolone and antibiotic administration, most of the systemic symptoms resolved, with the exception of exfoliative dermatitis and erythema, which ameliorated over the following 4 months. Agranulocytosis, atypical lymphocytosis, aseptic meningitis, and bronchiectatic changes along with prolonged systemic symptoms with exfoliative dermatitis were the most peculiar features of the present case. Keywords: Aseptic meningitis | Bronchiectasis | Dapsone | Multiple organ failure TOOLS   PDF Links   Full text via DOI   Download Citation   Supplementary Material   E-Mail

Research

Health-related quality of life, assessed with a disease-specific questionnaire, in Swedish adults suffering from well-diagnosed food allergy to staple foods

Sven-Arne Jansson, Marianne Heibert-Arnlind, Roelinde JM Middelveld, Ulf J Bengtsson, Ann-Charlotte Sundqvist,Ingrid Kallström-Bengtsson, Birgitta Marklund, Georgios Rentzos, Johanna Åkerström, Eva Östblom, Sven-Erik Dahlénand Staffan Ahlstedt

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Clinical and Translational Allergy 2013, 3:21 doi:10.1186/2045-7022-3-21

Published: 1 July 2013

Abstract (provisional)

Background

Our aim was to investigate the factors that affect health related quality of life (HRQL) in adult Swedish food allergic patients objectively diagnosed with allergy to at least one of the staple foods cow's milk, hen's egg or wheat. The number of foods involved, the type and severity of symptoms, as well as concomitant allergic disorders were assessed.

Methods

The disease-specific food allergy quality of life questionnaire (FAQLQ-AF), developed within EuroPrevall, was utilized. The questionnaire had four domains: Allergen Avoidance and Dietary Restrictions (AADR), Emotional Impact (EI), Risk of Accidental Exposure (RAE) and Food Allergy related Health (FAH). Comparisons were made with the outcome of the generic questionnaire EuroQol Health Questionnaire, 5 Dimensions (EQ-5D). The patients were recruited at an outpatient allergy clinic, based on a convincing history of food allergy supplemented by analysis of specific IgE to the foods in question. Seventy-nine patients participated (28 males, 51 females, mean-age 41 years).

Results

The domain with the most negative impact on HRQL was AADR, assessing the patients' experience of dietary restrictions. The domain with the least negative impact on HRQL was FAH, relating to health concerns due to the food allergy. One third of the patients had four concomitant allergic disorders, which had a negative impact on HRQL. Furthermore, asthma in combination with food allergy had a strong impact. Anaphylaxis, and particularly prescription of an epinephrine auto-injector, was associated with low HRQL. These effects were not seen using EQ-5D. Analyses of the symptoms revealed that oral allergy syndrome and cardiovascular symptoms had the greatest impact on HRQL. In contrast, no significant effect on HRQL was seen by the number of food allergies.

Conclusions

The FAQLQ-AF is a valid instrument, and more accurate among patients with allergy to staple foods in comparison to the commonly used generic EQ-5D. It adds important information on HRQL in food allergic adults. We found that the restrictions imposed on the patients due to the diet had the largest negative impact on HRQL. Both severity of the food allergy and the presence of concomitant allergic disorders had a profound impact on HRQL.

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Journal of Asthma and Allergy

Factors influencing asthma control: results of a real-life prospective observational asthma inhaler treatment (ASIT) study

Original Research

(37) Total Article Views

Authors: Yıldız, F

Published Date July 2013 Volume 2013:6 Pages 93 - 101

DOI: http://dx.doi.org/10.2147/JAA.S45269

Received:	15 March 2013
Accepted:	23 May 2013
Published:	01 July 2013

Füsun Yildiz, On behalf of the ASIT Study Group

Department of Pulmonary Disease, Kocaeli University School of Medicine, Kocaeli, Turkey

Background: Despite the availability of new pharmacological options and novel combinations of existing drug therapies, the rate of suboptimal asthma control is still high. Therefore, early identification of the clinical and behavioral factors responsible for poor asthma control, and interventions during routine outpatient visits to improve asthma trigger management, are strongly recommended. This study was designed to evaluate the profiles of asthmatic patients and their inhaler treatment devices in relation to asthma control in Turkey.
Methods: A total of 572 patients with persistent asthma (mean [standard deviation] age: 42.7 [12.1] years; 76% female) were included in this prospective observational study. A baseline visit (0 month, visit 1) and three follow-up visits (1, 3 and 6 months after enrolment) were conducted to collect data on demographics, past medical and asthma history, and inhaler device use.
Results: Asthma control was identified in 61.5% of patients at visit 1 and increased to 87.3% at visit 4 (P < 0.001), regardless of sociodemographics, asthma duration, body mass index or smoking status. The presence of asthma-related comorbidity had a significantly negative effect on asthma control (P = 0.004). A significant decrease was determined, in the rate of uncontrolled asthma, upon follow-up among patients who were using a variety of fixed dose combination inhalers (P < 0.001 for each). Logistic regression analysis was used to show that the presence of asthma-related comorbidity (odds ratio [OR], 0.602; 95% confidence interval [CI], 0.419; 0.863, P= 0.006) and active smoking (OR, 0.522; 95% CI, 0.330; 0.825, P = 0.005) were significant predictors of asthma control.
Conclusion: Our findings indicate that, despite ongoing treatment, asthma control rate was 61.5% at visit 1 in adult outpatients with persistent asthma. However, by the final follow-up 6 months later, this had increased to 87.3%, independent of sociodemographic and clinical characteristics. Poor asthma control was associated with asthma-related comorbid diseases, while the efficacy of fixed dose combinations was evident in the achievement of asthma control.

Keywords: persistent asthma, patient profile, asthma control, inhaler treatment, adults, Turkey



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  Arch Dis Child Educ Pract Ed 2013;98:113-118 doi:10.1136/archdischild-2012-303078
  • Pharmacy update

  Bronchodilators in wheezy under 2-year-olds: when and which (if any)?

  

  1. Will D Carroll1, 
  2. Jyothi Srinivas2

  +Author Affiliations

  1. ¹Nottingham University, Derbyshire Children's Hospital, Derby, UK
  2. ²Department of Paediatrics, Birmingham Children's Hospital, Birmingham, UK

  1. Correspondence toDr Will Carroll, Nottingham University, Derbyshire Children's Hospital, Uttoxeter Road, Derby DE22 3NE, UK; will.carroll@nhs.net

  • Received 24 September 2012
  • Revised 15 February 2013
  • Accepted 26 February 2013
  • Published Online First 9 April 2013
  Published Online First 9 April 2013

Clinical case

Amy is 13 months old and presented to the children's emergency department with a 5-week history of wheezing. Her parents explain that they have seen her general practitioner twice and attended the local walk-in centre three times over the last month. She has been prescribed antibiotics and salbutamol both of which Amy ‘hates’. On examination, she had a dry cough with mild respiratory distress. She has occasional crepitations and widespread wheeze. Parents smoke ‘outside the house’ but neither has asthma. She is their first child. Clinically, she is well but parents would like to know whether inhalers will help.

Introduction

Birth cohort studies have demonstrated that approximately one-third of the children aged between 1 and 5 years suffer recurrent episodes of respiratory symptoms including wheeze. Wheezing prevalence in UK children has increased from twofold to threefold during the past 40 years but may have stabilised or even peaked in the early 1990s. Fortunately, a majority of young children with wheeze tend to have only transient symptoms and do not have subsequently increased risk of asthma or allergy in later life. Nevertheless, childhood wheeze presents a major burden of morbidity during preschool years and there is significant progression from some childhood wheeze to adult asthma. More than 25% of an unselected birth cohort of children had wheezing that persisted from childhood into adulthood or that relapsed after remission. Despite the relative commonness of childhood wheeze, controversy and confusion exist over which treatments are effective. Doctors and nurses caring for these children face a dilemma regarding the treatment. Although bronchodilators are of clear benefit in older children with asthma, the anatomy and physiology in younger children are significantly different. In this article, we review the known physiology, the current evidence base and offer practical advice for those with a wheezy infant.

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We searched PubMed and Cochrane …

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   1. archdischild-2012-303078v1
   2. 98/3/113 most recent

Acute rhinosinusitis: New guidelines for diagnosis and treatment

Journal of the American Academy of Physician Assistants:

July 2013 - Volume 26 - Issue 7 - p 57–59

doi: 10.1097/01.JAA.0000431519.28443.5e

Special Topics in Otolaryngology

Acute rhinosinusitis: New guidelines for diagnosis and treatment

Teeters, Jennifer ATC; Boles, Michelle; Ethier, Julie; Jenkins, Ambria; Curtis, L. Gail PA-C, MPAS

Free Access

Article Outline

Author Information

Jennifer Teeters, Michelle Boles, Julie Ethier, and Ambria Jenkins are graduates of the PA program at Wake Forest School of Medicine, Winston-Salem, North Carolina. L. Gail Curtis is an associate professor and vice chair of the department of physician assistant studies at Wake Forest School of Medicine. The authors have indicated no relationships to disclose relating to the content of this article.

Roy A. Borchardt, PA-C, PhD, department editor.

Abstract

ABSTRACT: New treatment guidelines for acute rhinosinusitis outline when antibiotic therapy is appropriate, as well as describe evidence-based treatment to relieve symptoms, prevent complications, and prevent chronic disease.

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Research

The role of uPAR in epithelial-mesenchymal transition in small airway epithelium of patients with chronic obstructive pulmonary disease

Qin Wang, Yunshan Wang, Yi Zhang, Yuke Zhang and Wei Xiao

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Respiratory Research 2013, 14:67 doi:10.1186/1465-9921-14-67

Published: 28 June 2013

Abstract (provisional)

Background

Epithelial-mesenchymal transition (EMT) plays a crucial role in small airway fibrosis of patients with chronic obstructive pulmonary disease (COPD). Increasing evidence suggests that the urokinase plasminogen activator receptor (uPAR) is involved in the pathogenesis of COPD. Increased uPAR expression has been implicated in the promotion of EMT in numerous cancers; however the role of uPAR in EMT in small airway epithelial cells of patients with COPD remains unclear. In this study, we investigated the degree of EMT and uPAR expression in lung epithelium of COPD patients, and verified the effect of uPAR on cigarette smoke extract (CSE)-induced EMT in vitro.

Methods

The expression of EMT biomarkers and uPAR was assessed in lung epithelium specimens from non-smokers (n = 25), smokers (n = 25) and non-smokers with COPD (n = 10) and smokers with COPD (n = 18). The role of uPAR on CSE-induced EMT in human small airway epithelial cells (HSAEpiCs) was assessed by silencing uPAR expression in vitro.

Results

Markers of active EMT and uPAR expression were significantly increased in the small airway epithelium of patients with COPD compared with controls. We also observed a significant correlation between uPAR and vimentin expression in the small airway epithelium. In vitro, CSE-induced EMT in HSAEpiCs was associated with high expression of uPAR, and targeted silencing of uPAR using shRNA inhibited CSE-induced EMT. Finally, we demonstrate that the PI3K/Akt signaling pathway is required for uPAR-mediated EMT in HSAEpiCs.

Conclusions

A uPAR-dependent signaling pathway is required for CSE-induced EMT, which contributes to small airway fibrosis in COPD. We propose that increased uPAR expression in the small airway epithelium of patients with COPD participates in an active EMT process.

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International Journal of Chronic Obstructive Pulmonary Disease

The association between COPD and heart failure risk: a review

Review

(213) Total Article Views

Authors: de Miguel Díez J, Chancafe Morgan J, Jiménez García R

Published Date June 2013 Volume 2013:8 Pages 305 - 312

DOI: http://dx.doi.org/10.2147/COPD.S31236

Received:	28 February 2013
Accepted:	12 April 2013
Published:	28 June 2013

Javier de Miguel Díez,¹ Jorge Chancafe Morgan,¹ Rodrigo Jiménez García<sup>2

1</sup>Pulmonology Department, Gregorio Maranon University Hospital, Complutense University of Madrid, Madrid, Spain; ²Preventive Medicine and Public Health Teaching and Research Unit, Department of Health Sciences, Rey Juan Carlos University, Madrid, Spain

Abstract: Chronic obstructive pulmonary disease (COPD) is commonly associated with heart failure (HF) in clinical practice since they share the same pathogenic mechanism. Both conditions incur significant morbidity and mortality. Therefore, the prognosis of COPD and HF combined is poorer than for either disease alone. Nevertheless, usually only one of them is diagnosed. An active search for each condition using clinical examination and additional tests including plasma natriuretic peptides, lung function testing, and echocardiography should be obtained. The combination of COPD and HF presents many therapeutic challenges. The beneficial effects of selective ß1-blockers should not be denied in stable patients who have HF and coexisting COPD. Additionally, statins, angiotensin-converting enzyme inhibitors, and angiotensin-receptor blockers may reduce the morbidity and mortality of COPD patients. Moreover, caution is advised with use of inhaled ß2-agonists for the treatment of COPD in patients with HF. Finally, noninvasive ventilation, added to conventional therapy, improves the outcome of patients with acute respiratory failure due to hypercapnic exacerbation of COPD or HF in situations of acute pulmonary edema. The establishment of a combined and integrated approach to managing these comorbidities would seem an appropriate strategy. Additional studies providing new data on the pathogenesis and management of patients with COPD and HF are needed, with the purpose of trying to improve quality of life as well as survival of these patients.

Keywords: chronic obstructive pulmonary disease, heart failure



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