Respiratory Medicine Extra最新文献

A 78-year-old man was admitted with a tumor in the left anterior mediastinum that invaded the trachea and protruded into the lumen. Despite debulking with endobronchial laser followed by a tracheal stent and external radiotherapy, the tumor spread rapidly and extensively. Autopsy findings of the tumor indicated three distinct components: epithelial foci characterized by papillo-tubular structure, sarcoma-like spindle cells, and undifferentiated carcinoma. Immunohistochemical examination revealed that the epithelial foci were positive for thyroglobulin and thyroid transcription factor-1 whereas the spindle cells and undifferentiated carcinoma reacted with antibodies to α-smooth muscle actin and vimentin. Since the tumor was entirely separated from the cervical thyroid, we diagnosed it as undifferentiated carcinoma (spindle cell pattern) arising from ectopic mediastinal thyroid tissue. To our knowledge, this is the first report of undifferentiated carcinoma arising from ectopic thyroid tissue underscoring the need for correct diagnosis and early intervention of the highly malignant disease.

Background

Hairdressers, performing hair bleaching treatments and using hair spray, are exposed to risk of asthma. The precise underlying mechanisms, however are unclear, and little is known about the use of biomarkers in the clinical setting to predict treatment outcome and subsequent disease evolution.

Objective

To describe a case of asthma onset in an atopic hairdresser, after years of occupational exposure, and to evaluate the relationship between clinical symptoms and several markers of eosinophil and mast cell activation.

Methods

We studied retrospectively: symptoms, diagnostic tests and laboratory data collected at each visit.

Results

The causative role of occupational exposure on the onset of asthma was demonstrated by the case history. High values of eosinophil cationic protein (ECP) and tryptase in serum were consistent with a prominent role of eosinophils and mastcells in the active inflammatory process; expression of IL-5 cytokine was also enhanced at the time of exacerbations.

Conclusion

The relationship between clinical manifestations and mediators of cellular activation suggest their possible role as biomarkers to predict a response to steroid therapy or disease progression.

Diaphragm paralysis may be the presenting feature of motor neuron disease (MND), but signs attributable to the disease are usually found at that time or within the following month.

We present two patients who developed shortness of breath due to isolated diaphragm paralysis, which was confirmed by transdiaphragmatic pressure measurements and cervical magnetic stimulation of the phrenic nerves.

A 65-year-old man developed hypercapnia and progressive muscle wasting 6 years after first symptoms of diaphragm paralysis. Dysarthria, common peroneal nerve palsy on the right and fasciculations of the tongue were found a year later and MND was diagnosed.

A 47-year-old-man first presented with hypercapnic respiratory failure (PaCO₂ 18 kPa) and was intubated and ventilated. He had a 2-year history of dyspnoea and isolated bilateral diaphragm paralysis was confirmed. Other symptoms of MND developed 9 months later.

These cases serve as a reminder that MND may be the underlying diagnosis of diaphragm paralysis and may occasionally take years to develop more typical features.

Sarcoidosis is a multisystemic disorder, which mainly affects the respiratory system. Diagnosis is based on the exclusion of other infectious, interstitial and neoplastic diseases and a typical pathology. Presentation as an endobronchial mass is very rare. We describe a patient who presented with dry cough, mild fever, night sweats, asthenia and thoracic pain. Chest radiograph showed an increase of the right hilum. A Computed Tomography showed right parahiliar adenopathies with mediastinal involvement. Physical examination and routine serological and biochemistry tests were normal. Bronchoscopy showed a mass that occluded the entrance to the right segment IX. Its biopsy showed epitelial granulomatosis without necrosis. No malignancy was observed. The biological samples cultures were negative for mycobacterium. A diagnosis of sarcoidosis was made. The symptoms disappeared. No treatment was prescribed, remaining the patient asymptomatic. A tumoral behaviour of sarcoidosis is not usual, however, a mass presentation is very rare. Bronchial biopsy is mandatory for a confirmation diagnosis.

The acute respiratory distress syndrome (ARDS) secondary to tuberculosis in adults carries a high mortality. Its presentation in children is exceptional.

We report the case of a pregnant 15-year-old adolescent with no immunodeficiency who developed ARDS secondary to tuberculosis, requiring mechanical ventilation and inhaled nitric oxide treatment and presenting a favourable clinical course.

We conclude that, although very infrequent, tuberculosis must be excluded in any child with ARDS of unknown cause as early diagnosis and treatment significantly improves the prognosis.

Hepatopulmonary syndrome and portapulmonary hypertension are two distinct and not commonly encountered lung diseases occurring in patients with portal hypertension. These disorders are characterized by opposite pathophysiologic pictures and have been considered mutually exclusive. Although the sequential development of these syndromes in the same patient has been described, the simultaneous presence of features of hepatopulmonary syndrome and portapulmonary hypertension is very rare. In this report, we discuss the pathophysiological paradox and therapeutic dilemma associated with such coexistence.

Background

Celiac disease is a common disorder characterised by an immune response to ingested wheat gluten. It may be associated with pulmonary or cardiac manifestations.

Methods

Case report.

Results

A 52-year old woman with a history of chronic anemia presented with diffuse alveolar hemorrhage and atrioventricular heart block. The diagnosis of celiac disease was based on positive anti-transglutaminase, anti-endomysial, and anti-gliadin antibodies, and on jejunal biopsies showing complete villous atrophy.

Conclusion

Screening for celiac disease using anti-transglutaminase antibodies should be performed in patients with diffuse alveolar hemorrhage. Electrocardiogram is warranted in patients with celiac disease or alveolar hemorrhage.

Mycobacterium avium intracellulare complex (MAC) infection is a common cause of significant morbidity and mortality among human immunodeficiency virus (HIV)-infected patients. It usually presents as a systemic disease process. Endobronchial disease secondary to MAC is distinctly rare and can be a manifestation of the immune reconstitution in response to the highly active antiretroviral therapy. The purpose of this report is to document a case of MAC-related pulmonary mass associated causing endobronchial lesion simulating lung cancer in a patient with immune reconstitution with AIDS along with review of the relevant literature.

Thrombolysis is indicated for patients with massive pulmonary embolism who are haemodynamically unstable. [ACCP Consensus Committee on Pulmonary Embolism. Opinions regarding the diagnosis and management of venous thrombo embolic disease. Chest 1996; 109:233–7; Goldhaber SZ, Haire WD, Feldstein ML, Miller M, Toltsiz R, Smith JL, et al. Alteplase versus heparin in acute pulmonary embolism: randomised trial assessing right ventricular function and pulmonary perfusion. Lancet 1993; 341:507–11] Its use in the management of patients who have proximal life threatening thrombus in the pulmonary vasculature without systemic hypotension is controversial [ACCP Consensus Committee on Pulmonary Embolism. Opinions regarding the diagnosis and management of venous thrombo embolic disease. Chest 1996; 109:233–7; Goldhaber SZ, Haire WD, Feldstein ML, Miller M, Toltsiz R, Smith JL, et al. Alteplase versus heparin in acute pulmonary embolism: randomised trial assessing right ventricular function and pulmonary perfusion. Lancet 1993; 341:507–11]. We describe three patients (two males aged 45 and 58 years) and one female (aged 44 years who was 25 weeks pregnant) with acute proximal pulmonary arterial embolism in the absence of systemic hypotension. Surgical embolectomy was considered but our clinical assessment was that the location and extent of the thrombus in each case justified thrombolytic therapy and subsequently heparinisation even though no patient had evidence of systemic hypotension. Each patient was successfully treated with streptokinase (250,000 iu intravenously over 30 min followed by 100,000 iu per h intravenously for 12–24 h). Warfarin was commenced in two patients to achieve a target INR of 3–4 and low molecular weight heparin was continued throughout the remainder of her pregnancy in the third patient. Follow-up pulmonary angiography showed complete resolution of thrombus and there was improvement in 2D echocardiographic appearance. The pregnant lady delivered a healthy baby, normally, at term. In one patient who is now 6 months post-thrombolysis, repeat estimate of pulmonary vascular resistance and 2D echocardiography was normal at 6 months. We suggest that patients with potentially fatal pulmonary emboli should be considered for thrombolytic therapy even if they do not demonstrate haemodynamic instability.

The case of 57-year-old man with chronic ventilatory insufficiency due to asbestos pleurisy and sequelae of pulmonary tuberculosis is described. Non-invasive positive pressure ventilation therapy appeared to improve the pulmonary hypertension. Ultrasonic cardiography was valuable in the serial assessment of pulmonary artery pressure.