[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.

Cardiac substructures exposure in left-sided breast cancer radiotherapy: Is the mean heart dose a reliable predictor of cardiac toxicity?

PURPOSE: This study aimed to assess radiation dose distribution to cardiac subvolumes in left-sided breast cancer radiotherapy (LBCRT) and to clarify whether the mean heart dose (MHD) reliably reflects cardiac substructures exposure. MATERIALS AND METHODS: Fifty women referred for adjuvant LBCRT were prospectively evaluated. All patients received 3D-conformal hypofractionated radiotherapy (40Gy delivered in 15 fractions of 2.67Gy±boost of 13.35Gy). Cardiac substructures were contoured using the F. Duane's cardiac atlas. Dose distribution to cardiac chambers, left main (LM), left anterior descending (LAD), left circumflex (LCx) and right coronary artery (RCA)) was assessed. Dosimetric associations were analysed. RESULTS: The mean MHD was 3.08Gy (EQD2=3.67Gy). The mean Dmean/Dmax LAD was 11.45Gy (EQD2=13.64Gy)/29.5Gy (EQD2=35.15Gy). Low doses were delivered to LM, LCx, and RCA (Dmean≤1.3Gy). The left ventricle (LV) was the most exposed cardiac chamber with Dmean/Dmax of 4.78Gy/37Gy. The strongest correlation with MHD was found for Dmean LAD (r=0.81). For every 1Gy increase in MHD, Dmean LAD rose by 3.4Gy. However, the proportion of variance in Dmean LAD predictable from MHD was moderate (R2=0.65). For all other cardiac substructures, R2 values were<0.7. CONCLUSION: Our study showed high exposure of LAD and LV in LBCRT. With poor predictive value, MHD may underestimate doses to cardiac substructures. For optimal heart sparing radiotherapy, we recommend to consider LV and LAD as separate organ at risk.

[Prevalence and risk factors for fatty liver in Tunisian adults with obstructive sleep apnea]. / Prévalence et facteurs de risque de stéatose hépatique chez les adultes tunisiens atteints de syndrome d'apnées hypopnées obstructives du sommeil.

INTRODUCTION: Obstructive sleep apnoea (OSA) is commonly associated with non-alcoholic fatty liver disease (NAFLD). Early identification of NAFLD in OSA patients is important in order to try to prevent its evolution to advanced stages. The objective of this study was to determine the prevalence and risk factors for the occurrence of NAFLD in OSA patients. METHOD: A cross-sectional analysis including 124 OSA patients examined in the pulmonology department of Abderahmane Mami Hospital between January 2017 and March 2018 was undertaken. NAFLD was diagnosed using an abdominal ultrasonography. Data were analysed in a univariate and multivariate fashion in order to determine the characteristics of OSA patients with and without NAFLD. RESULTS: NAFLD was found in 62.9 % patients, with a frequency according to OSA severity of 51.3 %, 56.5 % and 72.6 % in mild, moderate and severe OSA, respectively. Severe OSA multiplies by 2.32 the risk of having NAFLD. The comparison between groups with and without NAFLD reveals that patients with the disease were younger, more obese, had more severe OSA, lower nocturnal oxygen saturation during sleep, and higher ALAT levels. Multivariate analysis showed a statistically significant link between NAFLD and serum ALAT elevation and the oxygen desaturation index. CONCLUSIONS: NAFLD is a frequent comorbidity in OSA, correlated to the severity of the disease. Thus, early screening of the disease in OSA patients especially in younger obese patients with high ALAT serum levels and a high oxygen desaturation index is proposed.

Leukemia in Patients with Klinefelter Syndrome: A Report of Two Cases.

Klinefelter syndrome (KS) is a chromosome abnormality characterized by a 47, XXY karyotype associated with hypogonadism and infertility. We present two cases of leukemia in patients with KS. The first patient presented with acute promyelocytic leukemia. He relapsed after the end of treatment. The second patient was diagnosed with chronic myeloid leukemia. Treatment with imatinib failed and the patient presented with myeloid blast crisis.

Solid tumors after chronic lymphocytic leukemia patients: Report of six cases and review of the literature.

INTRODUCTION: Malignancies have been reported to occur with increased frequency in chronic lymphocytic Leukemia (CLL) patients. The aim of this study was to describe which second malignancies occur in patients with CLL, whether these malignancies are related to CLL, its treatment, or both. We also attempt to study factors predicting the development of other malignancies. PATIENTS AND METHODS: Between 1995 and 2009, six cases of CLL associated with solid tumor were diagnosed in Hematology Department of Military Hospital of Tunis. The diagnosis of CLL was made by immunophenotyping of peripheral blood circulating B cells, and the diagnosis of solid tumors was made by biopsy with anatomopathological exam and immunohistochemical study. RESULTS: The mean age of patients was 71 years. Five patients were male. The CLL was classified Stage A in one case, Stage B in three cases and Stage C in two cases. Two patients had abnormal karyotype. Three patients have not received specific treatment for their CLL. Solid tumors were represented by skin cancer in three cases, lung cancer in two cases and breast cancer in one case. The median time between diagnosis of CLL and that of solid tumor was 53 months. CONCLUSION: Patients with CLL have an increased risk of developing a second cancer. Awareness of risk factors could permit early detection.

[The role of imaging in thoracic tuberculosis]. / Apport de l'imagerie dans la tuberculose thoracique.

Tuberculosis is an infectious disease mostly due to Mycobacterium tuberculosis. It is frequent in developing countries and its incidence is rising in developed countries. Lungs are the most involved organs of the chest but other structures can be affected. Imaging is fundamental in the management of the disease. Confirmation of diagnosis can be made only by bacteriologic and/or histologic exams. The first approach of diagnosis is based on clinical symptoms and chest X-ray signs. Radiologic signs depend on patient's age, his immune status and his previous contact with M. tuberculosis. Conventional chest X-ray remains the first-line exam to realize. It can suggest the diagnosis on the appearance and location of the lesions. CT scan is recommended for the positive diagnosis in case of discrepancy between clinical and radiographic signs, as for the diagnosis of parenchymal, vascular, lymph nodes, pleural, parietal or mediastinal complications. It is also essential for the evaluation of parenchyma sequelae. MRI and PET-scan have limited indications. The purpose of this article is to illustrate different radiological forms of chest tuberculosis, its sequelae and complications and to highlight the role of each imaging technique in the patient's management.

[Imaging of pulmonary artery involvement in Takayasu disease]. / Imagerie de l'atteinte artérielle pulmonaire au cours de la maladie de Takayasu.

OBJECTIVES: Illustrate imaging aspects of pulmonary artery involvement in Takayasu's arteritis. PATIENTS AND METHODS: Retrospective study of six patients among 28 patients with Takayasu arteritis whose disease involved the pulmonary arteries and to review their clinical and computed tomography data. RESULTS: Mean patient age among those with pulmonary artery involvement was 34 years. All patients exhibited extensive lesions of systemic arteries. The most common computed tomography angiography sign was wall thickening. Dilatation of the pulmonary artery trunk was observed in one-third of cases. CONCLUSION: Pulmonary arterial involvement in Takayasu's disease is not uncommon. Computed tomography is a reliable imaging technique to establish the diagnosis.

[Mediastinal mature teratoma with spontaneous malignant transformation]. / Tératome médiastinal mature avec transformation maligne spontanée.

INTRODUCTION: Malignant teratomas can be either immature teratomas or they can develop after treatment of other malignant germ cell tumors with chemotherapy or radiotherapy. Spontaneous malignant transformation of mature teratomas is very rare. OBSERVATION: We report the case of a patient complaining of atypical chest pain. Chest plain radiography showed a right anterior mediastinal mass. Computed tomography revealed a heterogeneous mediastinal mass with fat areas and calcification. There were hilar and mediastinal lymph nodes as well as lung parenchymal nodules. CT-guided and then surgical biopsies confirmed the diagnosis of a mature teratoma, which had degenerated into a digestive adenocarcinoma. CONCLUSION: Spontaneous malignant transformation of mature mediastinal teratoma is very rare. The diagnosis of malignant transformation is based on radiological examinations, laboratory tests and histological studies.

Allogeneic hematopoietic stem cell transplantation for acquired aplastic anemia using cyclophosphamide and antithymocyte globulin: a single center experience.

Between February 1998 and October 2007, 97 (69 male, 28 female) patients with acquired aplastic anemia and a median age of 18 years (range, 2-39) received related allogeneic hematopoietic stem cell transplantation. Ninety-five patients received bone marrow grafts and two patients G-CSF primed peripheral blood stem cell transplantation. The donors were genotypically HLA-identical siblings in 94 cases, HLA-matched parents in 2 cases and a syngeneic twin in 1 case. Median time from diagnosis to transplantation was 2 months (range, 1-15). Conditioning regimen consisted of cyclophosphamide combined with antithymocyte globulin in all patients. For graft versus host disease (GVHD) prophylaxis, all patients received methotrexate and cyclosporine. Eighty-six patients showed evidence of hematopoietic engraftment. Eight patients died before engraftment. Rejection rate was 14.8% with three primary graft failures and eight secondary graft rejections occurring between 2 and 27 months post transplantation. Of the 11 rejecting patients, 3 died from infection and 8 proceeded to a second transplantation. Among the eight patients re-transplanted, seven are alive with successful second engraftments and one died from acute grade III GVHD. Acute GVHD occurred in 15.5% and extensive chronic GVHD in only 5.3% of patients. The 4-year overall probability of survival was 76.8%. Infection was the cause of 81.1% of deaths. The major factor affecting survival was onset of infection before transplantation. Major ABO donor-recipient incompatibility, disease severity and acute GVHD had also negative impact on survival. These results could be improved by reducing the time to transplant and by a more efficient supportive care policy.Bone Marrow Transplantation advance online publication, 27 July 2009; doi:10.1038/bmt.2009.175.

Carbachol effect on carotid body dopamine in vitro release in response to hypoxia in adult and pup rabbit.

Dopamine (DA) release from the adult carotid body (CB) is dependent, in part, upon CB cholinergic receptor stimulation. The aim of the present study was to determine the role of cholinergic stimulation on DA release from rabbit pup CB with reference to adult's. CBs sampled from adult (n = 52) and 10-day-old (n = 49) rabbits were incubated in vitro for 1 h in a surviving medium bubbled with either 100 or 8% O2 in N2, without (control) or in the presence of the cholinergic agonist carbachol 1 microM. In adults, DA released (DAr) in the medium was significantly larger with 1 microM carbachol compared with control in either 100 or 8% O(2) (P < 0.01). In pups, carbachol 1 microM had no effect in 100% O2 but significantly increased DAr compared with control in 8% O2 (P < 0.01). The data suggest that cholinergic mechanisms regulating DAr are not fully expressed in pup rabbit CBs, in contrast with adults and thus, exhibit maturation-related functional differences.

Cholinergic dopamine release from the in vitro rabbit carotid body.

The aim of this study was to test whether cholinergic mechanisms regulate dopamine (DA) release from the carotid body (CB) and interact with DA D(2) autoreceptors. One hundred forty-two CBs from adult rabbits were infused in vitro in a surviving medium bubbled with O(2) (Bairam A, Marchal F, Cottet-Emard JM, Basson H, Pequignot JM, Hascoet JM, and Lahiri S. J Appl Physiol 80: 20-24, 1996). CB DA content and release were measured after 1 h of exposure to various treatments: control, cholinergic agonist (0.1-50 microM carbachol), full muscarinic antagonist (1 and 10 microM atropine), antagonists of M(1) and M(2) muscarinic receptors (1 and 10 microM pirenzepine and 10 microM AFDX-116, respectively), and the DA D(2) receptor antagonist domperidone (1 microM), alone and with carbachol (1 microM). Compared with control, the release of DA was significantly increased by carbachol (1-50 microM), AFDX-116, and domperidone and decreased by atropine (10 microM) and pirenzepine (10 microM). The effects of domperidone and carbachol were not significantly different but were clearly additive. It is concluded that, in the rabbit CB, M(1) and M(2) muscarinic receptor subtypes may be involved in the control of DA release, in addition to the DA D(2) autoreceptors.

Autoreceptor mechanism regulating carotid body dopamine release from adult and 10-day-old rabbits.

Dopamine (DA) release (r) from the carotid body (CB) is thought to be modulated by feedback inhibition mediated by DA D2 autoreceptors. We tested the hypothesis that CB DAr is autoregulated in a concentration and age dependent manner. Using an in vitro CB infusion model [Bairam, A., Marchal. F., Cottet-Emard, J.M., Basson, H., Pequignot, J.M., Hascoet, J.M., Lahiri, S., 1996b. Effects of hypoxia on carotid body dopamine content and release in developing rabbits. J. Appl. Physiol. 80, 20-24.], we evaluated under unstimulated conditions the effects of 0.001, 0.01, 0.1, 1.0 and 10.0 microM of the specific DA D2 receptor antagonist domperidone on CB DAr in adult rabbits. In 10-day-old rabbit pups, concentrations of 0.01, 0.1, 1.0 microM were studied. In adult CBs, domperidone increased DAr in a concentration-dependent manner. DAr (pmol/h) was significantly greater compared to control (without domperidone) starting at a domperidone concentration of 0.1 microM (P<0.01). In 10-day-old pup CBs, 1.0 microM domperidone was required to produce a significant increase of DAr (pmol/h) compared to control (P<0.005). However, control DAr (as % of total catecholamine) was about 40%; significantly higher than 24% observed in adult CBs (P<0.001). We conclude that in rabbit CB, DAr is controlled by an autoreceptor mechanism in a concentration-dependent manner and this mechanism is less developed in pups than in adults.