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L de Leval

Publications and source records attributed to L de Leval.

31 records · Page 2Linked to original sources
PubMed2001

[Alpha-1-antitrypsin deficiency. An indication for pediatric liver transplantation].

Alpha-1-antitrypsin deficiency is the most common inborn error of metabolism leading to liver transplantation, and the second cause of liver transplantation in children after biliary atresia. The authors report the case of a 6-year-old girl, who was suffering from end-stage liver disease secondary to alpha-1-antitrypsin deficiency. She was successfully treated by whole liver transplantation, the hepatic graft coming from a 3-year-old donor. Three months later she went back to school. The authors discuss the pathogenesis and the natural history of this frequent cause of liver transplantation in children.

Child↗
PubMed1999

Infantile and juvenile presentations of Alexander's disease: a report of two cases.

We describe 2 new cases of Alexander's disease, the first to be reported in Belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco-lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. Brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico-pathological conditions reported as Alexander's disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.

Adolescent↗
PubMed1998

CD28-B7 costimulatory blockade by CTLA4Ig delays the development of retrovirus-induced murine AIDS.

Mouse AIDS (MAIDS) induced in C57BL/6 mice by infection with a replication-defective retrovirus (Du5H) combines extensive lymphoproliferation and profound immunodeficiency. Although B cells are the main target of viral infection, recent research has focused on CD4(+) T cells, the activation of which is a key event in MAIDS induction and progression. A preliminary observation of increased expression of B7 molecules on B cells in MAIDS prompted us to address the possible involvement of the CD28/B7 costimulatory pathway in MAIDS. Mice infected with the MAIDS-inducing viral preparation were treated with murine fusion protein CTLA4Ig (3 x 50 microg/week given intraperitoneally), a competitive inhibitor of physiological CD28-B7 interactions. In CTLA4Ig-treated animals, the onset of the disease was delayed, lymphoproliferation progressed at a much slower rate than in untreated mice, and the loss of in vitro responsiveness to mitogens was reduced. Relative expression of Du5H did not differ between treated and untreated animals. These results suggest that the CD28/B7 costimulatory pathway contributes to MAIDS development.

Abatacept↗
PubMed1998

[Anatomo-clinical correlation. A case of invasive pulmonary aspergillosis].

This report describes a case of invasive pulmonary aspergillosis affecting a young female patient who had benefited from a liver transplantation for a fulminant hepatic failure. Infections following liver transplantation can happen but in this case the short delay to the fatal outcome is unusual (6 days). Corticotherapy given prior to transplantation could have impaired the immune system. The authors discuss the infections due to Aspergillus species and particularly invasive pulmonary aspergillosis.

Adult↗
PubMed1997

Peyer's patches in murine AIDS: dissociation between lymphoproliferation and anergy.

RadLV-Rs infection induces a murine immunodeficiency syndrome associated with a dramatic enlargement of spleen and lymph nodes. Surprisingly, the lymphoproliferation excludes thymus and Peyer's patches (PP). To understand the cellular interactions underlying lymphoproliferation further, the authors investigated the fate of PP in RadLV-Rs infected mice. The atrophy of PP was mostly due to the depletion of B cells, while the proportion of CD4+ and CD8+ T cells was increased. Nevertheless, B cell phenotype was modified with the emergence of lymphocytes with a low expression of B220 in infected PP. T cells characterized by a memory/activated phenotype in control PP did not undergo phenotypical changes after viral infection (i.e. regarding Thy-1 and CD44 expression). Despite the absence of lymphoproliferation, PP T and B cells displayed altered responses to mitogens in vitro. Finally, alterations of the expression of adhesion molecules and vascular addressins could not explain the atrophy of PP by a reduced homing to this lymphoid site. B cells and T cells from normal PP are clearly different from lymph nodes (LN) lymphocytes. The authors propose that the particular functional state which characterizes PP lymphocytes influences the B cell/T cell crosstalk necessary for RadLV-Rs-induced lymphoproliferation.

Animals↗
PubMed1995

Morphological changes of thymus in retrovirus-induced murine acquired immunodeficiency syndrome (MAIDS).

The possible contribution of the thymus in the setting of acquired immunodeficiencies is still questioned. Here we report some new findings regarding a potential involvement of the thymus in mice infected with RadLV-Rs, a viral mixture inducing murine acquired immunodeficiency syndrome (MAIDS). Thymi were sequentially removed, weighted and morphologically analyzed at different time intervals post-infection. Infection with RadLV-Rs led to a decrease in thymus weight mostly apparent from the fourth week. The first changes were seen at the third week as perivascular clusters of B-cells at the cortico-medullary junction. The ensuing process of atrophy mainly involved the cortex, while a mixed population of large T- and B-cells filled the medulla. These observations are discussed with regard to the pathological changes occurring in other lymphoid and non-lymphoid organs, in the context of the lymphoproliferation and immunodeficiency characterizing the disease, and by comparison with other models of retrovirus-induced immunodeficiencies.

Animals↗
PubMed1994

[Benign testicular and paratesticular tumors].

Five cases of benign testicular and paratesticular tumors are reported which have been treated by conservative methods. A literature review is presented about the most frequent benign testicular and paratesticular neoplasms. The preoperative diagnosis and the choice of treatment are discussed.

Adenomatoid Tumor↗
PubMed1994

[Rupture of a superficial femoral aneurysm in the context of neurofibromatosis. Report of a case].

The authors observed the spontaneous rupture of a dysplastic superficial femoral artery in a 47 years old patient with Von Recklinghausen disease. The patient presents a gigantism of his right lower limb. On angiography, the femoropopliteal axis is polyaneurysmal and there is evidence of rupture at mid-thigh. The diseased artery is excluded by interposition of a dacron prosthesis, with reimplantation of the deep femoral artery. Histology shows fibrohyalin thickening of the intima. Dysplasia of large limb arteries is excessively rare in Von Recklinghausen disease.

Aneurysm, Ruptured↗
PubMed1994

Flow cytometry in prostate cancer: histological and clinical correlation.

DNA histograms were obtained by flow cytometry for 39 human prostate carcinomas (27 total prostatectomy specimens, 5 biopsies and 7 transuretral resections). The study was performed on formalin-fixed and paraffin-embedded material. In this report, ploidy index did not seem to be a good marker of prognosis as no significant variation in ploidy was found neither among the different stages nor among the different Gleason categories. Proliferative index of the tumors seemed to be a more sensitive parameter; a significant relation was observed between proliferative index and stage of the tumor. The authors discuss these results under the light of previously reported observations.

Aged↗
PubMedDate not supplied

[Anatomo-clinical confrontation: idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic diseases are the most frequent diffuse interstitial pulmonary diseases. They slowly but irrevocably progress towards terminal respiratory failure. They can also be complicated by severe acute respiratory failure and admission in the intensive care unit can be discussed. Despite invasive mechanical ventilation, anti-infectious and immunosuppressive treatments, the disease carries a high mortality rate. We report and discuss the case of a patient with idiopathic, pulmonary fibrosis (UIP) who underwent rapid clinical deterioration.

Humans↗
PubMedDate not supplied

[Molecular characterization of Epstein-Barr virus associated with classical Hodgkin's lymphoma in Tunisia: prevalence of the LMP1 oncogene deletions and A and B viruses strains].

Epstein-Barr virus (EBV) is detected in Hodgkin's lymphoma (HL) at variable frequencies, depending on various factors including the geographic location. The prevalence of EBV is high among HL diagnosed in the region of the center of Tunisia. The aim of the current work was to define the genotypic features of EBV in a series of EBV-positive HL in this area, in comparison to those of EBV found in association with benign lymphoadenopathies. A 30 bp and a 69 bp deletion within the LMP1 gene were detected in 41% and 11% of HL cases, respectively. In reactive lymph nodes, the 30 bp deletion was found in 43% of the cases. Type A EBV was detected in 84% of HL cases and in 96% of reactive lymphoadenopathies, and type B EBV was detected in others. No significant association was found between histological type of HL, age of the patients, or stage at diagnosis, and the molecular configuration of LMP1. However, LMP1 deletions were found more frequently among male patients. The significance of these results is discussed.

Adolescent↗
PubMedDate not supplied

Fatal massive liver steatosis--a clinicopathological case report.

Hepatic fatty change is a common lesion. Two forms are recognized: micro- and macrovesicular steatosis, the former being much less frequent and more serious than the latter. The case of an alcoholic woman under anticonvulsivant therapy and with medications for a syndrome of inappropriate secretion of antidiuretic hormone (SIADH) who presented with rapidly progressive cholestasis and hepatocellular failure is reported. Massive macro- and micro-vesicular hepatic steatosis was diagnosed at autopsy. The authors review the clinico-pathological features associated with this condition, and causal factors possibly implicated in this case are discussed in regard with currently considered pathophysiological mechanisms.

Anti-Bacterial Agents↗