Familial extensive idiopathic bilateral pleural fibrosis.

The authors report three sisters with bilateral isolated apical pleural fibrosis of unknown origin, which did not respond to empirical antituberculosis therapy and oral corticosteroids. The disease evolved in an unrelenting fashion producing pleural fibrosis at the lung bases and leading to the death of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity. The particular features of these cases and the differences from other reports of apparently cryptogenic pleural fibrosis are outlined.     

111In-pentetreotide scintigraphy in patients with Langerhans' cell histiocytosis.

Langerhans' cell histiocytosis is a granulomatous disease that may involve multiple organs and the prognosis of which is highly variable. Because the prognosis depends particularly on the number of tissues involved, the accurate identification of the organs involved by granulomatous lesions is of critical importance. We hypothesized that 111In-pentetreotide scintigraphy would be useful for evaluation of patients with Langerhans' cells histiocytosis. METHODS: Thirteen patients (38.3+/-10.4 y) with Langerhans' cell histiocytosis (8 patients with unifocal lung disease, 5 with multifocal disease) received intravenous 111In-pentetreotide (111-222 MBq), and planar images were obtained at 24 h after injection. Pulmonary uptake was quantified using a lung-to-background ratio (L/B) and compared with a population of 10 normal scintigrams. For the other sites, uptake of radioactivity in disease-related areas was visually assessed. RESULTS: Ten of 12 patients with lung involvement had increased lung uptake (UB, 2.23+/-0.49 versus 1.34+/-0.07; P < 0.001). In the patients with multifocal disease, increased 111In-pentetreotide uptake was found in disease-related areas such as the salivary glands, the skin, the soft tissues, and the bones. However, somatostatin receptor imaging was insensitive for detecting central nervous system and liver involvement and most skin lesions. CONCLUSION: 111In-pentetreotide imaging may be useful in Langerhans' cell histiocytosis. Further study will indicate whether 111In-pentetreotide is a relevant tracer in the management of histiocytosis.     

Déficit en sphingomyélinase acide (maladie de Niemann-Pick B) : une étude rétrospective multicentrique de 28 patients adultes

Introduction

Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease with a clinical spectrum ranging from a neurovisceral infantile form (Niemann-Pick disease type A) to a chronic visceral form also encountered in adults (Niemann-Pick disease type B, NP-B).

Hodgkin's disease revealed by a nephrotic syndrome: a case report with literature review

The association between paraneoplasic nephrotic syndrome and Hodgkin's disease is rare. We report a case of Hodgkin's lymphoma in a young female patient with nephrotic syndrome. A 40-year-old woman presented with the clinical symptoms of nephrotic syndrome, kidney biopsy revealed minimal change glomerulonephritis. A treatment with prednisone was started but the response was partial. Twenty-four months after the diagnostic of nephrotic syndrome was made, she presented prolonged fever and weight loss, the physical examination showed cervical lymphadenopathy, which lymph node biopsy revealed Hodgkin lymphoma of the nodular sclerosing type. The patient was treated for Hodgkin disease stage IVB with ABVD regimen, and was in complete remission of both Hodgkin's disease and nephrotic syndrome.     

Approche épidémiologique de la boulimie et du comportement alimentaire inhabituel en milieu universitaire à Marrakech (Maroc)

Bulimia is a public health problem. The aim of this study is to determine characteristics of bulimia and unusual eating behavior among female's students. We carried out an investigation among 480 female students in four universities and one higher school of management. The prevalence of bulimia is 4 % and an unusual eating behavior is 32.2 %. Severe bulimia was found in 21.05 %. The appetite suppressant was the most used way to lose weight in the two groups of students. Medical school had most of bulimia and unusual eating behavior. Bulimia was predominant in the middle of university studies. Bulimic female students were more overweight and those with unusual eating behavior were with stoutness that is more normal. In conclusion, the lifestyle had no influence on bulimia and unusual eating behavior, and depression is common in both of the groups.     

Systemic therapy for advanced endometrial cancer

Endometrial cancer has generally a good prognosis when it is diagnosed at an early stage but remains incurable at an advanced stage (recurrent or metastatic) with only few therapeutic options. Hormone therapy is the treatment of choice in case of slowly progressive disease with a tumor expressing hormonal receptors due to its favorable safety profile. Taxanes, anthracyclines and platinum compounds are the most active chemotherapy agents with greater response rates when they are combined at the price of a significant toxicity. Targeted therapies based on a better understanding of tumor biology are being evaluated with some promising results.     

Profil cytologique des leucémies aigu?s à Casablanca

The diagnosis of AML is based on the clinical and biological arguments. The purpose of this study is to describe cytological characteristics of 814 cases of acute leukemia collected at the Laboratory of Haematology, University Hospital Ibn-Rochd of Casablanca between 1^st January 2004 and 31 July 2007. Morphological examination of bone marrow and the reaction to myeloperoxidase were used to classify acute leukemias: 1) 64% of acute leukemias are the myeloid type (AML); 2) 30% of acute leukemias are the lymphoblastic type (ALL); 3) 6% of acute leukemias are difficult to classify according to criteria of the FAB group. In this series, 81.6% of AML cases are seen in adults with a sex ratio M/F of 1.05, for ALL, 67.3% of cases are diagnosed in children with a sex ratio M/F of 1.2. According to FAB classification, and the LAM1 and LAM2 are most frequent, respectively 31% and 28 %, for lymphoblastic form, type 2 is predominant with 75%.