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Biomedical subjects

J L Dupond

Publications and source records attributed to J L Dupond.

At least 19 recordsLinked to original sources
PubMed1992

[Bone and visceral manifestations of lipoatrophic diabetes. Apropos of a case].

Lipoatrophic diabetes, known by pediatricians as Lawrence-Seip disease or Berardinelli lipodystrophy syndrome, is an infrequent condition of which approximately one hundred cases have been published to date. A case in a 24-year-old female with a fifteen-year follow-up is reported. Manifestations included acanthosis nigricans, generalized lipoatrophy, hirsutism, muscle hypertrophy, and intellectual impairment. Biologic tests revealed insulin-resistant diabetes mellitus with major diet-dependent type V hypertriglyceridemia. The patient had nephrotic syndrome (focal and segmental endocapillary proliferative glomerulonephritis without dense deposits). Phosphorus and calcium determinations were normal, as were the endocrinologic tests. Roentgenograms of the bones disclosed increased density of axial bones and large epiphyseal defects with increased bone density as determined by osteodensitometric studies. The bone manifestations of this syndrome have been documented but are often overshadowed by the severe metabolic alterations.

Abnormalities, Multiple↗
PubMed1992

[Silent exercise-induced enzymatic myopathies at rest in adults. A cause of confusion with fibromyalgia].

Exercise-induced enzymatic myopathies include carnitine palmityl transferase deficiency and, among muscular glycogenoses, Mac Ardle's and Tarui diseases. These diseases are usually recognized when exercise-induced myalgias, myoglobinuria and raised creatinine kinase (CK) levels are present. However, myoglobinuria may be absent in 10 to 50 percent of the cases, and CK levels are often normal at rest; thus, the diagnosis is often delayed for several years, with a risk of acute renal failure in 10 to 30 percent of the patients. We report 6 cases of exercise-induced enzymatic myopathies with normal CK levels and with electromyographic studies at rest. The main clinical features of these cases and those of similar conditions reported in the literature are male sex, onset of the disease before the age of 15 years, episodes of severe exercise-induced myalgias, cramps and muscle weakness and myogenic hyperuricaemia at rest in muscular glycogenosis.

Adolescent↗
PubMed1992

Risk factors associated with hepatitis B or C markers or elevated alanine aminotransferase level among blood donors on a tropical island: the Guadeloupe experience.

Donated blood is currently screened for hepatitis B surface antigen (HBsAg), antibody to hepatitis B core antigen (anti-HBc), antibody to hepatitis C virus (anti-HCV), and alanine aminotransferase (ALT) levels to prevent posttransfusion hepatitis. A prospective study of 2368 blood donors was carried out in Guadeloupe (French West Indies) with a view to determining the risk factors associated with serologic abnormalities. Blood donors included in the study had to complete a questionnaire. Statistical analysis was performed on the data thus obtained: 571 donations (24%) were positive for at least one of the four analyzed markers. The results were that 3.2 percent were positive for HBsAg, 22 percent for anti-HBc, and 0.8 percent for anti-HCV, and 1.4 percent had ALT > or = 45 IU per L. A good correlation was found between anti-HCV and elevated ALT. Transfusion history and two socioeconomic categories (working class, military personnel) were found to be risk factors. Other risk factors were lifelong residence in Guadeloupe (with risk increasing with the number of years), birthplace and current residence in the southern part of the island, and the existence of gastrointestinal discomfort unrelated to viral hepatitis (odds ratio = 2.98). The results of this study illustrate the difficulty of implementing a preventive policy against posttransfusion hepatitis in a tropical area. The unique epidemiologic situation of Guadeloupe as regards hepatitis B virus has led to more restrictive criteria for the acceptance of blood donors.

Adolescent↗
PubMed1992

[Evidence that excessive sedimentation rate is predictive of traffic accidents in systemic diseases].

OBJECTIVE: To identify the factors accelerating the sedimentation rate and to determine whether an excessive quickness may be responsible of the circulatory accident in systemic diseases. METHODS: The sedimentation rate has been enregistred in 100 patients with systemic diseases known for having a major quickness. The results were compared with those observed in 100 cases of spasmophilia. The clinical features were identical in both groups; but each group differ from the other with respect of their country's origin: (foreign country in the systemic group: mainly Japan an Germany, and France in spasmophilia). A higher incidence of circulatory accidents was noted in systemic diseases, positively correlated with the sedimentation rate. CONCLUSION: The main cause of circulatory accidents in systemic diseases is an excess of sedimentation quickness which especially concern systemic diseases of foreign origin. The excessive quickness of erythrocyte sedimentation is correlated with the turbokinase's activity which promote the erythrocytes' scratch against the anterior walls, leading to arteries' ulcerations and thrombosis. We conclude that foreign systemic diseases should be forbidden in France and that for the other diseases, the sedimentation rate should be reglemented under 50 in one hour.

Accidents, Traffic↗
PubMed1991

Adult onset Still's disease and related renal amyloidosis.

A 57 year old woman presented with clinical and biological features of adult onset Still's disease. A kidney biopsy was performed at the onset of the condition and was found to be normal. Four years later, in view of a nephrotic syndrome, histopathological examination of a new kidney biopsy specimen was made and showed typical amyloid deposition. Renal amyloidosis was suspected in five previously reported cases but demonstrated by kidney biopsy in only two of them. In the present case renal amyloidosis was recorded after disease of four years' duration. The normality of the first renal biopsy specimen suggests the possibility of a direct relation between amyloidosis and adult onset Still's disease.

Amyloidosis↗
PubMed1990

Localized scleroderma--response to 1,25-dihydroxyvitamin D3.

1,25-Dihydroxyvitamin D3 [1,25(OH)2 D3] may be an immunomodulatory drug which could have a role in controlling collagen deposition, and inducing reversal of fibrosis in some tissues. These observations prompted a study of the possible use of this hormone for the treatment of scleroderma. A 35-year-old woman, who had been suffering from localized scleroderma for 2 years, was given oral 1,25(OH)2 D3 for 6 months. The effects of the treatment were evaluated using clinical and physical measurements (skin thickness, extensibility properties of the skin). The evolution of the patient's condition during the 6-month therapy suggests that 1,25(OH)2 D3 is beneficial in localized scleroderma. The mechanisms of action are discussed in relation to the literature, which suggests both immunoregulatory and inhibitory effects on fibroblast growth.

Adult↗
PubMed1989

[The kidney in Still's disease in adults].

Renal involvement during Still's disease in the adult is rarely mentioned in the literature. Proteinuria and hematuria are frequently reported during systemic involvement in the disease but, conversely, observations including an anatomical account of the kidney are rare: amyloidosis is mentioned the most often (5 compatible cases), while other cases are more disparate: non specific glomerulitis (4 cases), glomerulonephritis with mesangial deposition of IgA (2 cases), tubulo-interstitial nephritis; these different non specific aspects may correspond to an immune complex disease. The apparent rarity of renal investigations is a factor in marking out Still's disease in the adult from other systemic diseases.

Adult↗
PubMed1989

Malignant angioendotheliomatosis. Reclassification as an angiotropic lymphoma.

A 70-year-old woman with chronic edema of the lower limbs was diagnosed as having a "malignant angio-endotheliomatosis," because histologic examination showed a strict intravascular location of a malignant proliferation. Immunochemical studies actually disclosed the lymphomatous origin of malignant cells. The recent literature regarding the nosology of this rare condition is reviewed.

Aged↗
PubMed1989

Polyneuropathy: an unusual extraintestinal manifestation of Crohn's disease.

Among the extraintestinal complications of Crohn's disease, neuropathy seems to be rare, and is only exceptionally reported in the literature. The authors present the report of a patient followed over a 12-year period who developed polyneuropathy with a parallel course to Crohn's disease. There was no evidence of any other underlying condition. On one occasion, the patient developed a cutaneous vasculitis with fibrinoid deposits in the vessels. The search for circulating immune complexes was positive. The authors demonstrated increased intestinal permeability using the 51chromium-labeled ethylenediaminetetra-acetate (51Cr-EDTA) test. Ultrastructural study of a biopsy of the superficial peroneal nerve disclosed a marked denervation process. Therapeutic plasmapheresis were performed, improving the neurologic symptoms. Vasculitis with circulating immune complexes is suggested as a possible mechanism in view of the occurrence of cutaneous vasculitis, increased intestinal permeability, and plasma exchange efficiency.

Biopsy↗