Chronic granulomatous disease associated with common variable immunodeficiency - 2 clinical cases.

INTRODUCTION: Chronic granulomatous disease associated with common variable immunodeficiency (GD-CVID), although well documented, is rare. Granulomatous lesions can affect several organs and are histologically indistinguishable from sarcoidosis. CLINICAL CASES: Case 1: A 39-year-old male patient with CVID, asymptomatic although with thrombocytopenia and mediastinal-hilar adenopathies. GD-CVID was diagnosed by bone marrow biopsy. Progressive clinical and radiological improvement was obtained with corticotherapy. Case 2: A 38-year-old male patient with CVID, suffered from asthenia, anorexia, myalgia, lower limbs edemas, and dry cough. He had mediastinal and bilateral hilar adenopathies within which biopsy revealed non-necrotizing granulomatous infiltrate. A spontaneous resolution was detected after 9 months of evolution. CONCLUSION: GD-CVID is rare and can mimetize other pathologies, namely, sarcoidosis; it should therefore be publicized and discussed so that it becomes a general clinical knowledge.

Sedation with midazolam in flexible bronchoscopy: a prospective study.

INTRODUCTION: Sedatives have been increasingly used to improve patient comfort during flexible bronchoscopy (FOB). Due to its rapid-onset, anxiolytic and amnestic properties, midazolam is one of the most commonly used sedatives. OBJECTIVES: To evaluate the effect of sedation with midazolam, including patient tolerance, complications and its potential use on a daily routine basis. MATERIAL AND METHODS: A multi-centre, prospective, randomized, placebo-controlled study was made on 100 patients submitted to FOB in two Pulmonology Departments. Midazolam (0.05mg/kg) was administered to patients in Group 1 and saline solution (0,9% NaCl) to patients in Group 2, five minutes before the procedure. The Hospital Anxiety and Depression Scale (HADS-A) was used to determine patient anxiety level. Subjective questionnaires concerning main fears and complaints were answered before and after FOB. RESULTS: Mean age was 56.0 ± 14.1 years; 66% male. Most (65%) patients had low score (<7) in HADS-A scale with no difference between groups. No significant differences were seen between groups concerning FOB duration, procedures, lidocaine dosage and complications. Systolic blood pressure during and after FOB was significantly higher (p<0.003) in Group 2. Patients in Group 1 experienced less cough (32% vs 56%; p=0.03) and dyspnea (2% vs 34%; p<0.001) than in Group 2, while nausea (6% vs 18%; p>0.05) and pain (4% vs 12%; p>0.05) were not statistically different. Willingness to repeat the exam was reported in all patients in Group 1 and in 82% in Group 2 (p=0.003). CONCLUSION: Sedation with midazolam in FOB improved patient's comfort and decreased complaints, without significant haemodynamic changes. It should be offered to the patient on a routine basis.

Endobronchial myxoma--case report.

INTRODUCTION: Pulmonary myxoma is an extremely rare benign neoplasm. It is mostly parenchymal but may occasionally occur within the tracheobronchial tree. There are very few reports of endobronchial myxoma. CASE REPORT: We describe a case of endobronchial myxoma in a 40-year-old female patient with a history of asthma and repeated right-sided pneumonia. Thoracic computed tomography (CT) showed medium lobe atelectasis. Fiber optic bronchoscopy revealed a polypoid, well-circumscribed tumor, causing total obstruction of the medium lobe bronchus. Biopsy of the mass was non-diagnostic. Further study included a positron emission tomography (PET) which demonstrated low metabolic activity of the tumor and no evidence of neoplasia in other location. The patient was submitted to a medium lobectomy and microscopic examination of the tumor revealed myxoid stroma with lobulated pattern, elongated and stellate cells, compatible with myxoma. CONCLUSION: Pulmonary myxoma is extraordinary rare and endobronchial location is very few reported in medical literature.