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Pulmonary arteries aneurysms are rare and the rarely described bilateral aneurysms. A 45-year-old patient carrier of mitral stenosis was admitted for dyspnoea class III of the NYHA, chest pain and a not infectious cough. The clinical examination found semiology of mitral stenosis, tricuspid incapacity and pulmonary arterial hypertension. The electrocardiogram showed atrial fibrillation and right ventricle hypertrophy. Chest X-ray found a cardiomegaly, an aspect of double outline of the inferior right bow, a prominent aspect of the left average bow reminding an aneurysm of the left pulmonary artery. The echocardiography Doppler found a pure tight mitral stenosis (mitral surface=0.6 cm(2)), a dilation of the trunk of the pulmonary artery (diameter=74 mm) and of its branches (diameter of the right pulmonary artery=28 mm, diameter of the left pulmonary artery=36 mm) seat of a spontaneous contrast. The left atrium and right cardiac cavities were also dilated. There was an important tricuspid incapacity with a major pulmonary hypertension (systolic pulmonary arterial=109 mmHg). The thoracic angioscan showed a pseudoaneurysm of the trunk of pulmonary artery and its branches to their distal parts. Under diuretic, anticoagulating and analgesic treatment the clinical signs improved however the spontaneous contrast persisted. The patient was rejected by the surgery for exceeded clinical board. She is at present followed in our service for 5 months.
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PMID:[Aneurysmal dilatation of pulmonary artery and its branches on mitral stenosis: a case report]. 2129 35

Infection with HIV increases the risk for lung diseases, including noninfectious pulmonary hypertension (PH). HIV-associated PH (HIV-PH) is an important lung disease in HIV-infected persons who live longer with antiretrovirals. The early stages of HIV-PH may be overlooked by healthcare providers due to nonspecific symptoms, including progressive dyspnea and nonproductive cough. HIV-PH may be detected via chest radiographs, CT scans, or electrocardiograms, but Doppler echocardiography is the most useful screening test to identify candidates for right heart catheterization. HIV-PH has a poor prognosis with high mortality; improved biomarkers to identify earlier stages of PH would benefit clinical care. The HIV-PH mechanism remains unknown, but HIV proteins such as Tat and Nef may play a role. HIV-1 Nef is a broad-spectrum adaptor protein that may affect HIV-infected and uninfected pulmonary vascular cells. Studies in macaques suggest that Nef is important in HIV-PH pathogenesis because monkeys infected with a chimeric simian immunodeficiency virus (SIV) expressing HIV-nef (SHIVnef) alleles, but not monkeys infected with the native SIV, develop pulmonary vascular remodeling. Four consistent amino acid mutations arose spontaneously in Nef passaged in the monkeys. To translate these findings to humans, one research endeavor of the Lung HIV Study focuses on the identification of HIV nef mutations in HIV-infected individuals with PH compared with HIV-infected normotensive patients. We present some of the preliminary evidence. Ongoing longitudinal studies will establish the connection between Nef mutations and the propensity for HIV-PH.
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PMID:Pathogenesis of HIV-associated pulmonary hypertension: potential role of HIV-1 Nef. 2165 33

Sarcoidosis affects the lungs in more than 90% of cases. Symptoms include cough, dyspnea, and chest pain. The entire respiratory tract can be involved. The most common areas of involvement are the airways and interstitium. Airway disease can lead to airway obstruction while interstitial lung disease can lead to restrictive disease. Patients may have a mix of these areas of involvement. For the symptomatic patient, the identification of disease involvement can usually be determined by pulmonary function testing and chest imaging. The chest X-ray staging system has been widely used in sarcoidosis, high-resolution computer tomography (HRCT) can provide detailed information regarding lung involvement. Unfortunately the various patterns seen on HRCT have limited the ability to develop a simple scoring system. Special studies such as bronchoscopy can be useful for detecting large airway disease. Other chest manifestations include adenopathy, pulmonary hypertension, and pulmonary muscle weakness. Fibrotic lung disease can lead to bronchiectasis, which can become infected.
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PMID:Pulmonary manifestations of sarcoidosis. 2257 34

Combined pulmonary fibrosis and emphysema (CPFE) syndrome is an uncommon entity characterised by emphysema of the upper lobes and diffuse fibrosis of the lower lobes and carries a bad prognosis with the onset of pulmonary hypertension. Lung involvement due to exposures suffered by welders is generally considered benign though, rarely, a diffuse interstitial fibrotic disease has been reported. CPFE syndrome has however never been reported in welders. A 65-year-old man, welder by occupation and an ex-smoker, presented with progressive exertional dyspnoea associated with dry cough noticed for the last four months. On examination, there was mild tachypnea, clubbing and bilateral basal velcro crepitations on chest auscultation. Lung function test revealed mild mixed ventilatory impairment with severe diffusion defect. HRCT chest showed bilateral upper lobe emphysema and diffuse interstitial fibrosis in the lower lobes. Transbronchial lung biopsy revealed interstitial fibrosis, chronic inflammation and iron deposits. A diagnosis of combined pulmonary fibrosis with emphysema (CPFE) with interstitial pulmonary siderofibrosis (IPS) was established. A review of literature did not show any other report of a similar nature.
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PMID:Combined pulmonary fibrosis and emphysema in a welder. 2266 43

A 70-year-old Caucasian male presented 8 months postcadaveric renal transplant with slowly progressive shortness of breath, abdominal distention, and cough for a duration of a few days. Thorough evaluation found him to have severe pulmonary hypertension (PH) on echocardiogram with decompensated high-output congestive heart failure. A right heart catheterization was done, which confirmed elevated right-sided pressures and high cardiac output. The mean pulmonary artery pressure, on a Swan-Ganz catheter, improved from 37 to 30 mmHg on partial manual occlusion of his still functioning hemodialysis arteriovenous fistula. Subsequently, the patient underwent ligation of the fistula and this led to gradual improvement in his symptoms. Follow-up right heart catheterization and echocardiogram showed marked improvement and normalization of right heart pressures. We recommend that patients with arteriovenous fistula should undergo close monitoring for development of early signs and symptoms of congestive heart failure and screening for PH by echocardiography post-kidney transplant. Right heart catheterization should be considered if screening is positive. Risk and benefit of fistula closure should be weighed in face of reduced survival from PH in dialysis patients and closure should be considered in post-transplant patients.
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PMID:Decompensated high-output congestive heart failure in a patient with AVF and the role of right heart catheterization: a case study. 2303 39

A 29-year-old man developed a persistent dry cough. Chest high-resolution computed tomography (HRCT) revealed centrilobular ultrafine granular shadows scattered in all lung fields. A lung biopsy with video-assisted thoracoscopic surgery revealed findings compatible with pulmonary tumor thrombotic microangiopathy (PTTM). However, the primary tumor was not identified. Combination chemotherapy with S-1 and cisplatin decreased his cough and improved the chest HRCT findings. The illness, however, gradually became difficult to control. He eventually developed pulmonary hypertension and died. Typically, an antemortem diagnosis of PTTM cannot be made. In this case, the diagnosis of PTTM and combination chemotherapy improved the chest HRCT findings, respiratory symptoms, and prognosis.
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PMID:Pulmonary tumor thrombotic microangiopathy diagnosed antemortem and treated with combination chemotherapy. 2303 71

Pertussis, or whooping cough, caused by Bordetella pertussis, still occurs despite vaccination. Most of the cases occurring in adolescents and adults are mild or have a subclinical course, but these patients can be a source of transmission to unvaccinated or partially vaccinated infants. Symptoms of infant pertussis are often not specific, but pertussis can be fatal. In this article, we present one case of unvaccinated twins who each presented with initial signs of a viral respiratory disease. Within a few days, each developed rapidly progressive respiratory failure complicated by refractory pulmonary hypertension due to malignant pertussis. Both patients died eventually. It is important for paediatricians, general practitioners, midwives and gynaecologists to be alert to coughing in their patients. More efficient vaccination strategies should be discussed to prevent both the transmission of B. pertussis and the occurrence of severe and fatal pertussis in young infants.
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PMID:[Pertussis in young infants: a dangerous disease with non-specific signs]. 2334 39

Pulmonary veno-occlusive disease (PVOD) is a rare and challenging cause of pulmonary hypertension. Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. Our case involves a healthy 21-year-old man who developed dyspnoea on exertion at an elevated altitude during deployment to Afghanistan. His work-up included an echocardiogram, a high-resolution CT scan, V/Q scan, pulmonary function tests with diffusion capacity, and a cardiac catheterisation with vasodilator challenge. Initially diagnosed with vasodilator responsive pulmonary arterial hypertension, an oral vasodilator was given with subsequent development of non-cardiogenic pulmonary oedema, thus confirming a clinical diagnosis of PVOD. He was medically stabilised with diuretic therapy, but developed progressive right-ventricular failure. For definitive treatment, he underwent a successful bilateral lung transplant. Explanted lung histology confirmed the diagnosis of PVOD.
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PMID:Pulmonary veno-occlusive disease: an uncommon cause of pulmonary hypertension. 2337 46

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure > or = 25 mmHg at rest as assessed invasively by right heart catheterisation. It can affect patients at any age and presents with non-specific symptoms. Accurate diagnosis is important as while PH is a potentially lethal disease it is treatable. Identification of the cause of PH is crucial to ensure that the patient receives appropriate management. The most common causes are heart and lung disease; specialist drug therapies for PH are contraindicated in these patients. However, PH is also associated with a diverse group of diseases. The most common symptom of PH is breathlessness on exertion. Syncope may be the presenting symptom especially in children. Other PH symptoms include: angina pectoris, palpitations, dry cough, exertional nausea and vomiting. Lack of response to conventional treatment for dyspnoea should alert the GP to search for another cause of the patient's symptoms, one of which could be PH. Patients should have an ECG, a chest radiograph and a full blood count, renal and liver function as well as thyroid function tests. When PH is suspected, referral to the local cardiology or respiratory department is recommended. Referral should be urgent if the patient is syncopal, has rapidly progressing symptoms or is in heart failure. Echocardiogram is the investigation of choice for detecting elevated pulmonary pressures. If the echocardiogram shows signs of PH then the patient should be referred to a designated PH centre if pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension are suspected or if PH is of uncertain cause.
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PMID:Diagnosing and managing pulmonary hypertension. 2347 17

The present report describes a 60-year-old Caucasian woman who presented with progressive dyspnea, cough and wheeze. A computed tomography scan of the chest showed innumerable bilateral inflammatory pulmonary nodules with bronchovascular distribution and a mediastinal and hilar infiltrative process with calcified lymphadenopathy leading to narrowing of lobar bronchi and pulmonary arteries. An echocardiogram revealed pulmonary hypertension. Bronchoscopy showed left vocal cord paralysis and significant narrowing of the bilateral bronchi with mucosal thickening and multiple nodules. Transbronchial biopsy was compatible with sarcoidosis. Despite balloon angioplasty of the left lower lobe and pulmonary artery, and medical therapy with oral corticosteroids, her symptoms did not significantly improve. To the authors' knowledge, the present report describes the first case of pulmonary sarcoidosis resulting in major airway, vascular and nerve compromise due to compressive lymphadenopathy and suspected concurrent granulomatous infiltration. Its presentation mimicked idiopathic mediastinal fibrosis.
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PMID:Sarcoidosis with major airway, vascular and nerve compromise. 2371 17


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