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Cystic fibrosis (CF) is of particular importance in Ireland as the Irish population has both the highest incidence (2.98/10,000) and the highest carrier rate (1 in 19) in the world. Primary immunodeficiency has not been previously reported as co-existing with CF.We report a unique case of CF associated with a primary immunodeficiency syndrome--common variable immunodeficiency (CVID).Our patient has CF, CVID and the additional comorbidity of Aspergers syndrome. The challenges inherent in diagnosing and treating such a case are outlined herein and the successful management of this case is evidenced by the well-preserved lung function of our patient.

The provision of life-sustaining ventilation, such as tracheostomy to critically ill patients, is commonly performed. However, the utilization of tracheostomy or extubation after a withdrawal of treatment decision is debated. There is a dearth of practical information available to aid clinical decision making because withdrawal of treatment is a challenging scenario for all concerned. This is further complicated by medicolegal and ethical considerations. Care of the "hopelessly ill" patient should be based on daily evaluation and comfort making it impossible to fit into general algorithms. Although respect for autonomy is important in healthcare, it is limited for patients in an unconscious state. Beneficence remains the basis for withdrawing treatment in futile cases and underpins the "doctrine of double effect." This article presents a relevant clinical case of hypoxic brain injury where a question of withdrawal of treatment arose and examines the ethical, clinical, and medicolegal considerations inherent in such cases, including beneficence, nonmaleficence, and the "sanctity of life doctrine." In addition, the considerations of prognosis for recovery, patient autonomy, patient quality of life, and patient family involvement, which are central to decision making, are addressed. The varying legal frameworks that exist internationally regarding treatment withdrawal are also described. Good ethics needs sound facts, and despite the lack of legal foundation in several countries, withdrawal of treatment remains practiced, and the principles described within this article aim to aid clinician decision making during such complex and multifaceted end-of-life decisions.

Background Colonization by Aspergillus fumigatus in patients with cystic fibrosis (CF) can cause A fumigatus sensitization and/or allergic bronchopulmonary aspergillosis (ABPA), which affects pulmonary function and clinical outcomes. Recent studies show that specific allergens upregulate the surface-expressed basophil marker CD203c in sensitized subjects, a response that can be readily measured by using flow cytometry. Objective We sought to identify A fumigatus sensitization in patients with CF by using the basophil activation test (BAT). Methods Patients with CF attending Beaumont Hospital were screened for study inclusion. BAT was used to identify A fumigatus sensitization. Serologic (total and A fumigatus –specific IgE), pulmonary function, and body mass index measurements were performed. Results The BAT discriminates A fumigatus –sensitized from nonsensitized patients with CF. Persistent isolation of A fumigatus in sputum is a significant risk factor for A fumigatus sensitization. Levels of the A fumigatus –stimulated basophil activation marker CD203c inversely correlated with pulmonary function and body mass index in A fumigatus –sensitized but not nonsensitized patients with CF. Total and A fumigatus –specific IgE, but not IgG, levels are increased in A fumigatus –sensitized patients with CF and ABPA when compared with those in A fumigatus –sensitized and nonsensitized patients with CF without ABPA. Itraconazole treatment did not affect A fumigatus sensitization. Conclusion Combining the BAT with routine serologic testing allows classification of patients with CF into 3 groups: nonsensitized, A fumigatus –sensitized, and ABPA. Accurate and prompt identification of A fumigatus –associated clinical status might allow early and targeted therapeutic intervention, potentially improving clinical outcomes.

To determine if sputum colonization with Aspergillus species in patients with cystic fibrosis (PWCF) correlates with radiological abnormalities and/or a reduction in pulmonary function (FEV1).We prospectively evaluated 32 PWCF utilizing high resolution computed tomography (HRCT) of the thorax and pulmonary function testing (PFT). The cohort was assessed as two groups: Aspergillus positive (n=16) and Aspergillus negative (n=16) based on sputum culture for Aspergillus species. A modified Bhalla scoring system was applied to each HRCT scan by two blinded radiologists.Aspergillus positive patients had more severe and significant bronchiectasis compared to those Aspergillus negative (p0.05). This was most marked in the right upper and lower lobes (RUL, RLL). Total Bhalla score was clinically significant in both groups and approached statistical significance between groups (p=0.063). No difference in pulmonary function between the groups was detected.PWCF colonized by Aspergillus species have greater radiological abnormalities undetectable by PFTs. Early radiological evaluation of Aspergillus colonized PWCF is therefore warranted.

Introduction: Cystic fibrosis related diabetes (CFRD) is increasing. We aim to determine HbA1C ranges and incidence of complications in CFRD pts when age/sex matched to non-CF diabetic controls. Methods: 11 CFRD patients (mean age 23.5 SE 1.49, 7M/4F) were age/sex matched to non-CF, type I diabetics (mean age 23.6 SE 1.52, 7M/4F). The HbA1C range (over 3 y), length from dx and all complications of DM recorded. Results: CFRD had comparable ranges of HbA1C than age/sex matched controls (range 4.8 15.1%, mean 7.90% SE 0.41, and 5.0 14.6%, mean 8.73% SE 0.41, respectively) [p-value 0.05]. Duration of DM differed b/w the gps; CFRD (mean 3.56 yrs/median 3 yrs) and non-CF diabetics (mean 9.61 yrs/median 8 yrs) [p-value

An 87-year-old woman presented with a 2-day history of dyspnoea, productive cough and right-sided chest discomfort. Having become increasingly lethargic over the preceding fortnight, she also noted unquantifiable weight loss over the prior 3-month period. Physical examination and initial radiological and haematological assessment was consistent with a community-acquired pneumonia and associated parapneumonic effusion confirmed by pleural aspiration. She was appropriately treated with a full course of intravenous antibiotic therapy however failed to completely resolve. Subsequent bronchoscopy did not reveal an obstructing tumour and associated specimens …

To the Editor.— We read with interest the recent article by Grey et al,1 which illustrated admirably the extent of suboptimal bone health and vitamin D and K status among a pediatric population with cystic fibrosis (CF) with relatively well preserved lung function. They appropriately suggested that the vitamin deficiencies observed may be causative and explain the low bone mass and turnover detected as reflected by the measured bone biomarkers. At our tertiary referral center, we previously examined similar relationships between fat-soluble vitamin deficiencies, bone fragility, and pulmonary function in …