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Lung squamous carcinoma with two paraneoplastic syndromes: dermatomyositis and Lambert –Eaton myasthenic syndrome
ConclusionRecognition of paraneoplastic syndrome is crucial for early diagnosis of lung cancer because up to 80% of patients may present paraneoplastic syndromes before any other direct indication of malignancy. Early diagnosis and treatment of paraneoplastic syndrome is also important because delayed diagnosis may result in rapid disease progression and irreversible neurological damage.
Source: The Clinical Respiratory Journal - November 25, 2014 Category: Respiratory Medicine Authors: Yuanrong Dai, Ping Li, Sunshun Yan, Xiaodong Xia, Zhixiong Li, Mengling Xia Tags: CASE REPORT Source Type: research

Lung squamous carcinoma with two paraneoplastic syndromes ‐Dermatomyositis and Lambert‐Eaton myasthenic syndrome
We reported a case of a 60‐year‐old male smoker who presented multiple erythematous lesion for six months with progressive proximal muscular weakness, and developed to dysphonia in two months. His chest computed tomography showed a spiculate pulmonary nodule near the hilar in the right upper lobe and mediastinal lymph nodes enlargement. Lung biopsy showed squamous cell carcinoma. The patients’blood test revealed elevated creatine kinase levels. Electromyography results was consistent with moderate myopathy accompanied with a pattern suggestive of Lambert‐Eaton myasthenic syndrome. So we made the diagnosis of an ove...
Source: The Clinical Respiratory Journal - October 21, 2014 Category: Respiratory Medicine Authors: Yuanrong Dai, Ping Li, Sunshun Yan, Xiaodong Xia, Zhixiong Li, Mengling Xia Tags: Other Source Type: research

Retrospective Review Of Combined Sirolimus And Simvastatin Therapy In Lymphangioleiomyomatosis.
Conclusions.Therapy with sirolimus and simvastatin does not increase the prevalence of drug adverse events or alter the therapeutic effects of sirolimus. Background.: Combined simvastatin and sirolimus therapy reduce TSC2-null lesions and alveolar destruction in a mouse model of lymphangioleiomyomatosis (LAM), suggesting that therapy with both drugs may benefit LAM patients. Methods.: To determine whether simvastatin changed the prevalence of adverse events or altered the therapeutic effects of sirolimus, we recorded adverse events and changes in lung function in LAM patients treated with simvastatin plus sirolim...
Source: Chest - August 28, 2014 Category: Respiratory Medicine Authors: Taveira-DaSilva AM, Jones AM, Julien-Williams P, Stylianou M, Moss J Tags: Chest Source Type: research

The value of respiratory muscle testing in a child with congenital muscular dystrophy
We report the case of a 12‐year‐old boy with congenital muscular dystrophy (CMD) in whom invasive and non‐volitional respiratory muscle tests showed an elective diaphragmatic dysfunction with the preservation of expiratory muscle strength. This finding, coupled with a clinical phenotype associating diffuse muscle atrophy with finger hyperlaxity and proximal contractures, strengthened the suspicion of Ullrich CMD. Skin‐cultured fibroblasts showed intracellular retention of collagen 6 (COL6), muscle magnetic resonance imaging was typical of COL6 myopathy, and molecular studies identified a COL6 gene mutation (COL6A2 ...
Source: Respirology Case Reports - June 30, 2014 Category: Respiratory Medicine Authors: Sonia Khirani, Ivana Dabaj, Alessandro Amaddeo, Adriana Ramirez, Susana Quijano‐Roy, Brigitte Fauroux Tags: Case Report Source Type: research

The lung is involved in juvenile dermatomyositis
ConclusionThe frequency of lung involvement in children with JDM justifies systematic respiratory assessment with PFT including measures of respiratory muscle strength. We suggest that a chest HRCT scan is indicated in cases of respiratory symptoms and/or PFT abnormalities. Longitudinal studies are needed to assess pediatric characteristics, long‐term outcomes, and responses to treatment taking into account the risk–benefit ratio. Pediatr Pulmonol. © 2012 Wiley Periodicals, Inc.
Source: Pediatric Pulmonology - December 31, 2012 Category: Respiratory Medicine Authors: Guillaume Pouessel, Antoine Deschildre, Muriel Le Bourgeois, Jean‐Marie Cuisset, Benoit Catteau, Chantal Karila, Véronique Nève, Caroline Thumerelle, Pierre Quartier, Isabelle Tillie‐Leblond Tags: Original Article: Other Source Type: research

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