Abstract：
:Gaucher's disease is a not exceptional lysosomial disease in Tunisia. Type 1 is by far the most common one. Pulmonary involvement is considered to be rare in type 1 Gaucher's disease. Pulmonary hypertension, infiltration of the lungs with Gaucher cells, and severe hypoxemia due to intrapulmonary arterial-venous shunts, have been described in case reports and small case series. We reported the case of hepatopulmonary syndrome in a 14-year-old boy with type 1 Gaucher disease. The diagnosis of Gaucher disease was established, at 2 years age, by enzyme assay of leucocyte β-glucosidase. The patient presented dyspnoea, digital clubbing and cyanosis of the lips. The arterial blood gas found severe hypoxaemia with PaO(2) at 56.9 mmHg. The diagnosis of hepatopulmonary syndrome, in our patient, was confirmed by demonstration of the intrapulmonary shunting using contrast-enhanced echocardiography and the technetium-99m-labeled macroaggregated albumin. The patient was treated by symptomatic measure, long term oxygen therapy because the insufficiency of the enzyme replacement therapy. Screening for hypoxemia in children with liver disease should be considered.
journal_name
Rev Pneumol Clinjournal_title
Revue de pneumologie cliniqueauthors
Bouguila J,Rouatbi H,Tej A,Chabchoub I,Trimech B,El Ajmi S,Essoussi AS,Boughammoura Ldoi
10.1016/j.pneumo.2011.07.002subject
Has Abstractpub_date
2012-02-01 00:00:00pages
58-62issue
1eissn
0761-8417issn
1776-2561pii
S0761-8417(11)00093-9journal_volume
68pub_type
杂志文章abstract：UNLABELLED:Exacerbations of chronic obstructive pulmonary disease (COPD) have an inflammatory component in addition to the possible infectious component. The antiinflammatory properties of fenspiride (Pneumorel(R) 80 mg) should be evaluated in this frequent clinical situation. OBJECTIVES:Assess the supplementary thera...
journal_title：Revue de pneumologie clinique
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：
更新日期：2000-02-01 00:00:00
abstract：:Acute chest syndrome is a frequent complication of sickle cell disease. This syndrome is characterized by recent infiltrate on chest X-ray with chest pain or fever or dyspnea. We report the case of a 26-year-old man in whom an acute chest syndrome with fat embolism was the inaugural sign of sickle cell disease. This r...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/s0761-8417(06)75439-6
更新日期：2006-06-01 00:00:00
abstract：:Over a 6 years' period (1980-1986), 272 patients with chronic respiratory disease were admitted for respiratory failure to the same intensive care unit. The series was characterized by the high mean age of the patients (69.3 years) and by the high proportion of those who were ventilated (75%); 33.7% of the patients di...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1987-01-01 00:00:00
abstract：:Tracheobronchomegaly is defined as a dilatation of the trachea and the large bronchi. It may occur as a familial condition or in association with a connective tissue disease, e.g. Ehlers-Danlos syndrome. Tracheobronchomegaly occurs late in adults. The predominant symptoms are bronchial irritation and recurrent broncho...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：2002-02-01 00:00:00
abstract：:Inflammation aggravates respiratory impairment in patients with cystic fibrosis. It plays an essential role in the genesis of bronchial hyperreactivity. Corticosteroids are the predominant therapeutic agents used. The importance of bronchial hyperreactivity in cystic fibrosis is correlated with disease severity. Treat...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1995-01-01 00:00:00
abstract：:Medical Images are components of the so-called "Medical Imaging Folder". This folder is a subset of the so-called "Medical Folder", part of the "Patient Folder. The G8 promotes the concept of a "Global Information Society for Health. Within this society, the Patient Folder is seen either from a healthcare organization...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：
更新日期：2000-04-01 00:00:00
abstract：:Constrictive pericarditis was diagnosed in four patients who presented respiratory impairment and pleural effusion, associated with right ventricular failure in two. The cause was probable tuberculosis in two cases, post-irradiation lesions in one and unknown in one. The diagnosis was suspected due to the normal heart...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1998-02-01 00:00:00
abstract：INTRODUCTION:Primary bronchial cancer (PBC) is a major public health problem. The diagnosis is often late resulting in a poor prognosis. PURPOSE:To determine the factors leading to a late diagnosis. PATIENTS AND METHODS:All PBCs diagnosed between 01 January and 31 December were included. The factors studied were: "ag...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2010.02.004
更新日期：2010-12-01 00:00:00
abstract：:The presence of non-caseating epitheloid cell granulomas in biopsy material is still one of the most important factors in establishing the diagnosis of sarcoidosis. Since Carlens introduced mediastinoscopy as a diagnostic procedure in these cases the use of liver biopsy or bronchoscopic biopsy has decreased because of...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1984-01-01 00:00:00
abstract：:We report 3 cases of recurrent idiopathic pneumothorax treated first by video-surgery then by classical surgery. These cases illustrate the difference in hospital stay and patient satisfaction, which is not always in favor of video-surgery. We also examine our personal experience with a series of 480 cases of pneumoth...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：2000-09-01 00:00:00
abstract：:Pulmonary invasive aspergillosis is a frequent and poor prognosis complication of immunodeficiency and prolonged neutropenia. Its treatment is usually based on amphotericin given as intravenous infusion at 1 to 1.25 mg/kg/d. Use of lipid carriers give the opportunity to administrated higher dose, 5 mg/kg/d, with respe...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：
更新日期：1995-01-01 00:00:00
abstract：:The diagnosis of pulmonary infection due to Mycobacterium xenopi was retained in a 48-year-old immunocompetent patient. The lesion was isolated in a peripheral region and was well limited. Exclusive surgical treatment was undertaken. Cure was complete without recurrence at three years. This case illustrates that in ce...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/s0761-8417(05)84822-9
更新日期：2005-09-01 00:00:00
abstract：INTRODUCTION:Post-traumatic diaphragmatic hernia is a particular lesion in traumatology that may be neglected. Thus, the diagnosis may be delayed for a few days to several months and only be made following a complication. The left diaphragmatic cupola is the most touched. Tension fecopneumothorax following diaphragmati...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2008.10.004
更新日期：2009-02-01 00:00:00
abstract：:The authors report a case of AIDS revealed by pleuropulmonary cryptococcosis. The infection was diagnosed by culture of the pleural fluid and confirmed by microscopic examination of the cerebrospinal fluid, using Indian ink as stain. Apyrexia and return to normal of chest radiography were obtained with oral fluconazol...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：
更新日期：1991-01-01 00:00:00
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journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1993-01-01 00:00:00
abstract：:The aim of this article is to give practicing physicians a practical approach to the treatment of latent and active tuberculosis. Most patients follow TB standard treatment recommended by WHO that depend on category of patient. It is a combination of four essential tuberculosis drugs of the first group: isoniazid, rif...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：10.1016/j.pneumo.2014.09.001
更新日期：2015-04-01 00:00:00
abstract：:The authors report a case of febrile pancytopenia of tuberculous origin with a normal chest X-ray at the outset. The diagnosis was virtually confirmed by bone marrow biopsy, even before the result of AAFB cultures. The course was rapidly favorable under the influence of triple anti-tuberculous therapy, with normalisat...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：:Perthes syndrome, or traumatic asphyxia syndrome, is a rare clinical entity, associating cyanosis, cervicofacial petechiae and subconjunctival hemorrhage. It is usually secondary to chest trauma, but can occur in any situation of abrupt rise in intrathoracic pressure with closed glottis. In this paper, we present a ca...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2016.08.002
更新日期：2016-12-01 00:00:00
abstract：:We report the case of a patient aged 23, admitted for bilateral intrathoracic tumor, including a giant right. Surgery was performed by right sternothoracotomy. After 7 days, she presented an irreversible cardiac arrest. The malignant peripheral nerve sheath tumors are rare and aggressive. Their incidence is 0.001% in ...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2013.08.006
更新日期：2014-06-01 00:00:00
abstract：:The authors describe a case of catamenial pneumothorax with diaphragmatic lacerations and partial liver herniation. It's a rare presentation of catamenial pneunmothorax. We analyse the diagnostic and surgical therapeutic. ...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2010.07.005
更新日期：2010-09-01 00:00:00
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journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1998-05-01 00:00:00
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journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2008.06.012
更新日期：2008-10-01 00:00:00
abstract：BACKGROUND:Asthma guidelines recommend combining inhaled corticosteroids and long-acting inhaled B2-agonists when inhaled corticosteroids alone are insufficient to achieve good control of the disease. OBJECTIVES:To determine in routine practice whether the fixed-dose fluticasone-salmeterol combination (FP/s) is as lea...
journal_title：Revue de pneumologie clinique
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：2003-06-01 00:00:00
abstract：:Asthma is a very common disease and may be encountered in pregnant women. The physiological changes which accompany pregnancy, as well as the risks of drug teratogenicity and drug interactions make it necessary to take certain therapeutic precautions. Following a review of the specific respiratory physiological change...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1987-01-01 00:00:00
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journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/s0761-8417(05)84870-9
更新日期：2005-12-01 00:00:00
abstract：:Assessment of daily physical activity (DPA) is a major element in the development of respiratory rehabilitation. The aim of this study was to evaluate the DPA and the daily energy expenditure (DEE) in adult patients with cystic fibrosis. Thirty adult patients (16 women, 14 men, mean age 27.1±8.4 years) with cystic fib...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2012.11.006
更新日期：2013-02-01 00:00:00
abstract：:The lymphatic vascular system is widely developed among vertebrates. Lymphatic vessels provide the interstitial fluid (20% of the body weight) drainage through interstitial prelymphatic channels, capillaries, precollectors and collectors flowing into the venous blood. Endothelial cells of capillaries are overlapped an...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：10.1016/j.pneumo.2013.01.005
更新日期：2013-04-01 00:00:00
abstract：:Although on the increase overall, the actual prevalence of occupational asthma is difficult to assess because of variations seen according to periods, countries, jobs, and the agents responsible. In the authors' experience, this prevalence is slightly less than 10%. Three principal mechanisms, sometimes present togeth...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：
更新日期：1984-01-01 00:00:00
abstract：INTRODUCTION:Dermatofibrosarcoma protuberans (Darrier-Ferrand sarcoma, DFSP) is an uncommon tumor. This sarcoma has a tendency to local recurrence, requiring a wide surgical resection. OBSERVATION:We report herein two cases of patients presenting with recurrent dermatofibrosarcoma protuberans after several surgical re...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章
doi：10.1016/j.pneumo.2016.09.005
更新日期：2017-04-01 00:00:00
abstract：:Complications observed after lung transplantation are well described for the different indications. Certain complications are specifically observed in patients with cystic fibrosis who have reached the stage of major respiratory failure. The most specific complications result from digestive tract manifestations of the...
journal_title：Revue de pneumologie clinique
pub_type： 杂志文章,评审
doi：
更新日期：2002-12-01 00:00:00
