[Lung manifestations of sickle-cell disease]. / Manifestations respiratoires de la drépanocytose.

Sickle-cell disease is an autosomal recessive genetic disorder of hemoglobin that causes systemic damage. Hypoxia is the main actor of sickle-cell disease. It initiates acutely the pathogenic cascade leading to tissue damages that in turn induce chronic hypoxia. Lung lesions represent the major risk of morbidity and mortality. Management of sickle-cell disease requires a tight collaboration between hematologists, intensivists and chest physicians. Recurrent episodes of thrombosis and hemolysis characterize the disease. New therapeutic protocols, associating hydroxyurea, transfusion program and stem cell transplantation in severe cases allow a prolonged survival until the fifth decade. However, recurrent pain, crisis, frequent hospital admissions due to infection, anemia or acute chest syndrome and chronic complications leading to organ deficiencies degrade the patients' quality of life. In low-income countries where the majority of sickle-cell patients are living, the disease is still associated with a high mortality in childhood. This paper focuses on acute chest syndrome and chronic lung manifestations.

[A case of pulmonary arteriovenous malformation treated surgically]. / Bisegmentectomie pour une malformation artérioveineuse pulmonaire.

Pulmonary arteriovenous malformation is a rare abnormality consisting of a direct connection between the arteries and the pulmonary veins. Most of the malformations are related to hereditary hemorrhagic telangiectasia, although 10 to 20% cases are idiopathic. Clinical manifestations are due to right-to-left shunting. Embolization is the treatment of choice, when it is possible and accessible. Surgery continues to be appropriate in certain cases. We report the case of a woman who presented with an isolated complex arteriovenous malformation fed by two afferent arteries, a lingular one and an antero-basal one. Surgical treatment by lingual and antero-basal bisegmentectomy was undertaken with a good outcome.

[Diagnostic and therapeutic management of operable bronchopulmonary carcinoid tumours]. / Prise en charge diagnostique et thérapeutique des tumeurs carcinoïdes broncho-pulmonaires opérables.

INTRODUCTION: Bronchial carcinoid tumours (CT), divided into typical carcinoid (TC) or atypical carcinoid (AC), are rare tumours whose therapeutic management remains unspecified. METHODS: Retrospective study collecting cases of bronchial CT operated at the thoracic surgery department of Abderrahmane-Mami hospital of Ariana and recruited from the pneumology departments of Northern Tunisia, during a 12-year period. RESULTS: Ninety patients were collected (74 cases of TC and 16 cases of AC). The mean age was 45 years and the sex ratio H/F=0.5. The chest X-ray was normal in 11 cases, as well as flexible bronchoscopy in seven cases. The tumour was classified: stage IA (10 cases), IIA (28 cases), IIB (31 cases), IIIA (15 cases) and IIIB (six cases). Surgery resulted in a complete resection in 78 patients, an extensive resection in six patients, and a conservative resection in six patients. Adjuvant chemotherapy was given in 10 patients. The survival was 84% at five years and 42% at 10 years. CONCLUSION: The prognosis of CT depends directly on the histological subtype. It is excellent for TC after complete resection, unlike ACs that are similar to well-differentiated bronchial carcinomas.

[Woman's asthma throughout life: Towards a personalized management?] / Asthme de la femme au fil du temps : vers une prise en charge personnalisée ?

In a woman's life, asthma can affect her in a variety of ways, with the onset of premenstrual asthma currently under-diagnosed. It is estimated that about 20% of women with asthma have premenstrual asthma, which is more common in patients with severe asthma. Women with asthma are at high risk of exacerbations and of severe asthma. Asthma is the most common chronic disease during pregnancy with potential maternal and foetal complications. Asthma medications are safe for the foetus and it is essential to continue pre-existing treatment and adapt it to the progress of asthma during the pregnancy. Sex steroids modulate the structure and function of bronchial and immune cells. Understanding their role in asthma pathogenesis is complicated by the ambivalent effects of bronchodilating and pro-inflammatory oestrogens as well as the diversity of response to their association with progesterone. Menopausal asthma is a clinical entity and is part of one of the phenotypes of severe non-allergic and low steroid-sensitive asthma. Targeted assessment of the domestic and professional environment allows optimization of asthma management.

[Prevalence and role of IgG anti-Chlamydia trachomatis in a population of infertile men in Morocco]. / Prévalence et rôle des IgG anti-Chlamydia trachomatis chez une population d'hommes infertiles au Maroc.

INTRODUCTION: Infertility is a global public health problem that affects 15% of couples of childbearing age. Male infertility is involved in 20 to 50% of cases. These figures are sharply increasing around the world. Several factors may be responsible for this infertility with especially hormonal, genetic, toxic or infectious factors. The latter are dominated mainly by Chlamydia infection. Among the most serious complications of this infection are infertility related to urethritis, epididymitis and irreversible total azoospermia in men and tubal obstructions and ectopic pregnancies in women. STUDY OBJECTIVE: To determine the prevalence of IgG anti-Chlamydia trachomatis in men consulting for infertility and the association between previous contact with this bacterium and the impairment of sperm quality and sperm function. MATERIAL AND METHODS: Prospective study over 26months of 143 patients referred to the service for infertility assessment of the couple. Demographic data, primary or secondary character of infertility, risk factors (tobacco, inguinal hernia, varicocele and history of urogenital infections), semen parameters (volume, mobility, pH, vitality and morphological abnormalities) were studied as well as the determination of the anti-C. trachomatis IgG titer. The prevalence of Chlamydia infection and the association of the infection and alteration of the various parameters of the semen were analyzed. RESULTS: The average age of patients was 38.5±8.55. Infertility was primary in 72% of patients. Among the patients, 54.5% had an abnormal spermogram. Chlamydia IgG antibodies were positive in 37.1% of patients whose 58.5% had abnormal spermogram. Analysis of sperm parameters of patients with and without IgG C. trachomatis showed an altered vitality in Chlamydia positive patients with an OR at 2.41, P=0.02, (95% CI: 1.15-5.06). CONCLUSION: The prevalence of Chlamydia infection is high in infertile male. C. trachomatis IgG antibodies may be associated with an alteration of spermatozoa vitality without significant impairment of other semen parameters. LEVEL OF EVIDENCE: 3.

[Tuberculous pneumothorax: Diagnosis and treatment]. / Pneumothorax tuberculeux : prise en charge diagnostique et thérapeutique.

BACKGROUND: Pneumothorax is a serious complication of cavitary pulmonary tuberculosis. The aim of this study was to describe clinical futures, to highlight challenges of its management. METHODS: A retrospective multicentric and descriptive study including 65 patients treated for PT (1999-2015) was conducted to figure out clinical futures and its work-up. RESULTS: The mean age was 37.8 years. The sex ratio was 3.6. Smoking history and incarceration were noted respectively in 67.6 and 15.3% of cases. Acute respiratory failure and cachexia were reported in 26.1 and 10.7% of cases. The PT was inaugural in 41.5% of cases. Pyo-pneumothorax was noted in 69.2% of cases. The duration of antituberculous treatment ranged from 6 to 15 months for susceptible TB and was at least 12 months for resistant TB (4 cases). Thoracic drainage was performed in 90.7% patients. Its average length was 47 days. The drain drop was noted in 20% of cases. Bronchopleural fistula was diagnosed in 6 cases and pleural infection in 5 of cases. Surgery treatment was necessary in 6 cases. Mean time to surgery was 171 days. Six patients had pleural decortication associated with pulmonary resection in 4 cases. Persistent chronic PT was noted in 12.6% and chronic respiratory failure in 3% of cases and death in 15.3% of cases. CONCLUSION: The diagnosis of the PT is often easy. Its treatment encounters multiples difficulties. Duration of thoracic drainage and anti-TB treatment are usually long. Surgery is proposed lately.

Update on Asthma Genetics: Results From Meta-Analyses of Candidate Gene Association Studies.

Several studies have investigated the risk factors associated with asthma. Both genetic and environmental factors are considered to contribute to asthma susceptibility. Individual genetic association studies usually suffer from small sample size leading to biased results. Meta-analysis is a powerful tool that has the potential to resolve this limitation by increasing the statistical power of analyses. The current review summarizes the recent knowledge concerning genetic factors involved in asthma predisposition based on meta-analyses. Using the keywords: asthma, meta-analysis, polymorphism, we searched Pubmed, Medline, Embase and Google Scholar databases for the associated articles. Genetic polymorphisms in twenty-three genes are associated with asthma risk in meta-analyses. However, polymorphisms in nine genes showed none significant association. These findings are used to assess the genetic risk factors and to understand the molecular pathways related to asthma.

Successful treatment of refractory adult onset Still's disease with rituximab.

Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome.

[Results of tuberculosis screening in children with household contact]. / Résultats du dépistage de la tuberculose chez les enfants contacts de parents tuberculeux.

INTRODUCTION: Tuberculosis screening in children is important to identify and treat latent tuberculosis infection and thus avoid progression to disease. METHODS: It is a prospective study realized in 83 children between November 2009 and January 2013 who consulted after a household contact for tuberculosis in the pediatric department B of Abderrahmen Mami hospital of Ariana. RESULTS: The mean age of the children was 4.8 years (3 months-15 years). A latent tuberculosis infection was diagnosed in 31.3% of children, 10.8% had tuberculosis and 57.8% were considered healthy. Fifty-seven children (68.7%) had a close daytime contact with the index and 57.8% slept in the same bedroom of the contaminator. The identified risk factors were the diagnosis delay of the index of more than 30 days (P=0.023), presence of cavitations on the chest X-ray of the index (P=0.029) and a close daytime contact (P=0.004). CONCLUSION: Our study showed a high rate of contamination in children with a household contact. Efforts are needed to shorten the time to diagnosis of adults' tuberculosis.

[Surgery of grown up congenital heart disease. About 540 cases]. / Chirurgie des cardiopathies congénitales à l'âge adulte. À propos de 540 cas.

INTRODUCTION: With advances in recent decades in the field of congenital heart disease both for imaging in medical therapy, a large number of heart disease is diagnosed before birth. Many of them benefit from surgery and reach adulthood, they do not require further action. Some of them develop later in their lives other problems requiring reoperation in adulthood. This sparked the birth of a subspecialty within the department of congenital heart disease: GUCH Unit "grown up congenital heart disease". In developing countries, little heart are detected in childhood, a minority of them are operated and very few reach adulthood or with minor heart disease or become advanced enough then inoperable. Only part may still take advantage of surgery at this age. The aim of our study is to describe the spectrum and characteristics of congenital heart disease in adulthood in Algiers a center of cardiovascular surgery. POPULATION AND METHODS: A retrospective descriptive study of patients aged 15 and above operated for congenital heart defects between 1995 and 2011. RESULTS: Five hundred and forty patients aged 15 to 76years (29±10 years), including 314 women and 226 men are operated congenital heart defects between 1995 and 2011. The left-right shunts represent two thirds of heart disease, represented mainly (50%) by the atrial septal defect. Barriers to the ejection of the left heart represent one forth of cases with a predominance of subvalvular aortic stenosis. We find the native heart whose survival is considered exceptional in adulthood in the absence of surgery, such as tetralogy of Fallot, aortopulmonary windows wide, double outlet right ventricle and atrioventricular canal that take advantage of always surgery. The results are encouraging with low perioperative mortality (2%). CONCLUSION: The approach of congenital heart disease in developing countries is different from that of developed countries. Efforts need to be made in early detection and monitoring of congenital heart disease and improve access to surgery centers in close collaboration with pediatricians, cardiologists and obstetricians.

Microscopic polyangiitis associated with primary biliary cirrhosis, Sjogren's syndrome and Hashimoto's thyroiditis.

The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.

[Childhood tuberculosis]. / La tuberculose de l'enfant.

Childhood TB is an indication of failing TB control in the community. It allows disease persistence in the population. Mortality and morbidity due to TB is high in children. Moreover, HIV co-infection and multidrug-resistant diseases are as frequent in children as in adults. Infection is more frequent in younger children. Disease risk after primary infection is greatest in infants younger than 2 years. In case of exposure, evidence of infection can be obtained using the tuberculin skin test (TST) or an interferon-gamma assay (IGRA). There is no evidence to support the use of IGRA over TST in young children. TB suspicion should be confirmed whenever possible, using new available tools, particularly in case of pulmonary and lymph node TB. Induced sputum, nasopharyngeal aspiration and fine needle aspiration biopsy provide a rapid and definitive diagnosis of mycobacterial infection in a large proportion of patients. Analysis of paediatric samples revealed higher sensitivity and specificity values of molecular techniques in comparison with the ones originated from adults. Children require higher drugs dosages than adults. Short courses of steroids are associated with TB treatment in case of respiratory distress, bronchoscopic desobstruction is proposed for severe airways involvement and antiretroviral therapy is mandatory in case of HIV infection. Post-exposure prophylaxis in children is a highly effective strategy to reduce the risk of TB disease. The optimal therapy for treatment of latent infection with a presumably multidrug-resistant Mycobacterium tuberculosis strain is currently not known.

Inflammatory optic neuropathy in Behçet's disease.

Inflammatory optic neuropathy (ON) is a rare event in Behçet's disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.

[Pulmonary zygomycosis in a diabetic child complicated with thrombus of the left atrial auricle]. / Mucormycose pulmonaire chez une enfant diabétique, compliquée d'un thrombus intra-auriculaire gauche.

Mucormycosis is a rare but fatal, opportunistic fungal infection caused by fungi of the order of mucorales in the class of Zygomycetes. Isolated pulmonary mucormycosis is rare and occurs principally in particular conditions, especially in patients with uncontrolled diabetes. The fungi invades the blood vessels and causes distal ischemic necrosis. We report a case in a 13-year-old girl with diabetes mellitus who developed pulmonary zygomycosis complicated with thrombus of the left atrial auricle. The diagnosis of mucormycosis was confirmed by histologic examination of endobronchial biopsies. Combined treatment with systemic amphotericin B and surgery resulted in a favorable outcome. This difficult diagnosis must be raised in diabetic patients with unresponsive lung infections in order to apply early aggressive therapy. Successful management continues to be early diagnosis, followed by systemic antifungal therapy and surgical resection combined with control of the underlying disease.

[Cardiac tamponade and myocarditis in Churg-Strauss syndrome]. / Tamponnade et myocardite aiguë au cours d'un syndrome de Churg-Strauss.

INTRODUCTION: The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome. CASE REPORT: A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment. CONCLUSION: Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment.

Primary mediastinal angiosarcoma: a rare observation in a patient with 8-year-survival.

BACKGROUND: Vascular tumours of the mediastinum are rare, accounting for 1-2% of all mediastinal tumours in this location. Angiosarcomas are most often encountered as sporadic lesions, typically in the scalp or face of elderly patients. However, they can occur in any anatomic site. Mediastinal angiosarcomas (MA) are very rare with less than 50 cases reported. CASE REPORT: The authors describe the case of a 38-year-old woman whose past medical history was consistent for a MA that was diagnosed in 2003. This tumour was treated by complete surgical resection followed by radiation therapy and chemotherapy. Diagnosis was based on histologic examination. In 2011, the patient presented a pleural localisation of the angiosarcoma and died one month after admission, 8 years after diagnosis of the MA. CONCLUSION: MA is a very rare tumour causing a diagnostic dilemma. Clinical and radiologic findings are non-specific, and final diagnosis is based on histologic examination. The case described is unusual considering the long period of survival, which may be explained by the treatment modalities associating complete surgical resection, chemotherapy and radiation therapy.