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R Lagarde

Publications and source records attributed to R Lagarde.

17 recordsLinked to original sources

Metachromatic leukodystrophy. Ultrastructural and enzymatic study of a case of variant O form.

A variant of metachromatic leukodystrophy (MLD), Austin disease, is characterized by a multiple isozyme deficiency of arylsulfatase. A 3 1/2-year-old girl with progressive mental and physical deterioration had decreased activities of arylsulfatases A and B in the leukocytes, shown by acylamide gel electrophoresis. Under the electron microscope, biopsy specimens of the brain and the peripheral nerve showed lamellar structures with socalled zebra bodies in the cytoplasmic processes of glial cells, granulo-membranous inclusions with fingerprint configurations in neurons, and myelinlike material in Schwann cells. Results from our study suggest an intricate nature of this dysmetabolic disorder, which shows ultrastructural changes usually seen in classic MLD, a deficiency of arylsulfatase A only, concomitant with those seen in mucopolysaccharidoses such as Hurler and Sanfilippo syndromes.

Cerebroside-Sulfatase↗

[Tuberculosis and human immunodeficiency virus infection in the Central African Republic].

In order to determine the proportion of tuberculosis (TB) patients in Bangui (Central African Republic) infected with human immunodeficiency virus (HIV), we collected prospectively serum samples from all new TB patients seen at the University Hospital in Bangui during a three-month period (nov. 87-Jan. 88). 220 serum samples were tested for antibodies to HIV, by an Elisa assay and confirmed by Western-Blot. The general HIV seropositivity rate among TB patients was 27.7% (31.2% in adults (N = 183), and 10.8% in children (N = 37)). Within the adult population, there was non association between HIV infection and sex or previous history of TB. Seropositivity was more common in patients with extrapulmonary TB, especially lymphadenitis, and in patients with mediastinal adenopathy, or extensive pulmonary involvement without cavitation. Seropositivity was also strongly associated with negative tuberculin skin tests, chronic diarrhoea, generalized lymphadenopathy, and thrush. It is concluded that HIV infection is responsible for the increasing incidence of tuberculosis in Central African Republic, and that investigations for TB should be carried out in every symptomatic African patient infected with HIV.

AIDS Serodiagnosis↗