Psychometric validation of the needs assessment tool: progressive disease in interstitial lung disease.

The inter-rater/test-retest reliability and construct validity of a palliative care needs assessment tool in interstitial lung disease (NAT:PD-ILD) were tested using NAT:PD-ILD-guided video-recorded consultations, and NAT:PD-ILD-guided consultations, and patient and carer-report outcomes (St George's Respiratory Questionnaire (SGRQ)-ILD, Carer Strain Index (CSI)/Carer Support Needs Assessment Tool (CSNAT)). 11/16 items reached at least fair inter-rater agreement; 5 items reached at least moderate test-retest agreement. 4/6 patient constructs demonstrated agreement with SGRQ-I scores (Kendall's tau-b, 0.24-20.36; P<0.05). 4/7 carer constructs agreed with the CSI/CSNAT items (kappa, 0.23-20.53). The NAT:PD-ILD is reliable and valid. Clinical effectiveness and implementation are to be evaluated.

Emphysema and bronchiectasis secondary to alpha-1 antitrypsin deficiency.

A 47-year-old Caucasian male presented to the chest clinic with a 4-week history of exertional dyspnea. A chest radiograph showed mild hyperinflation without any focal pathology and spirometry showed a mild obstructive defect. In view of symptoms being disproportionate to spirometric and radiologic abnormalities, a thoracic CT scan was obtained. It revealed that there was evidence of bronchiectasis and mild emphysema in basal distribution. Subsequently, he was confirmed to have severe α1-Antitrypsin deficiency. This case illustrates the importance of considering α1-Antitrypsin deficiency in patients with combination of emphysema and bronchiectasis in a basal distribution. Although basal emphysema is well-recognized pulmonary manifestation of α1-Antitrypsin deficiency, it is extremely unusual to have bronchiectasis with very mild degree of emphysema.

Pins and needles and unilateral foot drop: a presentation of sarcoidosis.

We describe a case of sarcoidosis in a 57-year-old man who presented with neurological symptoms of pins and needles in both of his hands, left leg weakness and left foot drop. Neurophysiological examination revealed asymmetric motor and sensory polyneuropathy. Common peroneal nerve involvement accounted for the left foot drop. Thoracic CT scan revealed bilateral hilar and mediastinal lymphadenopathy. He had hypercalcaemia and raised serum ACE level. Histological examination of a mediastinal lymph node showed non-caseating epithelioid cell granulomas consistent with the diagnosis of sarcoidosis. There was no evidence of acid-fast bacilli or fungi on special stains. This case highlights the importance of considering sarcoidosis as a potential diagnosis in patients presenting with peripheral neuropathy. Although response to corticosteroids and immunosuppressive therapy may be seen, in our case the patient's neurological deficit remained persistent despite treatment.

Bronchiolitis obliterans organising pneumonia: a consequence of breast radiotherapy.

The authors describe a case of 51-year-old woman who presented with breathlessness following radiotherapy for breast carcinoma. A chest radiograph and thoracic CT scan revealed extensive airspace consolidation affecting right upper and lower lobes. A trans-bronchial biopsy revealed evidence of foamy macrophages and fibroblastic plugs within alveoli, consistent with organising pneumonia. Indirect immunofluorescence microscopy revealed evidence of antiepithelial antibodies. Gradual but complete resolution occurred without any specific treatment. This case highlights the importance of considering radiation induced bronchiolitis obliterans organising pneumonia in the context of parenchymal shadowing following radiotherapy. Although corticosteroids are widely recommended for treatment, this case illustrates that organising pneumonia may resolve spontaneously.

Stable bronchiectasis is associated with low serum L-ficolin concentrations.

BACKGROUND AND AIMS: Bronchiectasis is a common chronic respiratory condition with recurrent cough and sputum production and recurrent chest infections. It is characterised by pathological dilatation of the bronchi thought to result from infection and inflammation. It was hypothesised that impaired innate immunity might influence susceptibility to this disease process. The aim of the present study was to look for an association between bronchiectasis and insufficiency of either mannan-binding lectin (MBL) or L-ficolin. MATERIALS AND METHODS: MBL and L-ficolin were measured by Enzyme-linked Immunosorbent Assay (ELISA) in sera from 119 clinically stable bronchiectasis patients, and compared with 43 age-matched disease controls admitted to hospital with community-acquired pneumonia, as well as healthy blood donors (168 for L-ficolin and 564 for MBL). RESULTS: Average (mean and median) serum L-ficolin concentrations were lower in the bronchiectasis patients (P < 0.04), but average MBL values did not differ significantly between the three groups. Relative L-ficolin deficiency was more frequent in bronchiectasis patients compared with blood donors (P