Biomedical subjects

M Godard

Publications and source records attributed to M Godard.

10 recordsLinked to original sources

PubMed1998

Familial lipoprotein lipase deficiency in infancy: clinical, biochemical, and molecular study.

OBJECTIVES: To describe the characteristics of lipoprotein lipase (LPL)-deficient patients seen in infancy and to evaluate the safety and efficacy of severe fat restriction. METHODS: Children <1 year old presenting with chylomicronemia between 1972 and 1995 were identified, and their clinical courses were reviewed retrospectively. RESULTS: LPL deficiency was demonstrated in 16 infants who presented with irritability (n = 7), lower intestinal bleeding (n = 2), pallor, anemia, or splenomegaly (n = 5), and a family history or fortuitous discovery (n = 2). All plasma samples were lactescent at presentation. Chylomicronemia responded rapidly to dietary fat restriction, and it was possible to maintain satisfactory metabolic control for a prolonged period of time. Only 1 adolescent girl had an episode of pancreatitis associated with the use of oral contraceptives. No persistent adverse effects on growth were seen. We obtained abnormal values for serum iron, alkaline phosphatase, and total calcium. CONCLUSIONS: The presentation of LPL deficiency is heterogeneous during infancy. Close dietary monitoring is required to avoid nutritional deficiencies. Estrogen therapy should be avoided in LPL-deficient patients.

Adolescent↗

PubMed1995

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PubMed1994

Improvement of hematologic recovery after high-dose intensification using peripheral blood progenitor cells (PBPC) mobilized by chemotherapy and GM-CSF.

We have tested the efficiency of GM-CSF to mobilize peripheral blood progenitor cells (PBPC) and evaluated the hematological reconstitution after GM-CSF primed-PBPC infusion following myeloablative therapy. Twenty three patients suffering from hematological malignancies were included in this study. Starting 24 hours after completion of a standard dose chemotherapy including vindesine, cyclophosphamide, adriblastine, prednisone, (VCAP), 5 micrograms/kg sub-cutaneous daily dose GM-CSF was given for a median time of 14 days followed by three consecutives cycles of leukapheresis. Fifteen of these 23 patients underwent GM-CSF primed-PBPC autotransplantation following high dosed intensification regimen. PBPC collection and hematopoietic recovery were compared with a 15 patients control group who did not receive GM-CSF. No marrow or growth factors were administered after PBPC reinfusion in the two groups. VCAP/GM-CSF mobilization induced significantly higher yields of CFU-GM (3.8 fold) than did VCAP mobilization alone, 19 x 10(4)/kg (2-73) vs 5 x 10(4)/kg (2-27), (p < 0.005). The median number of days to achieve 1.10(9)/l neutrophils, platelet count > 20.10(9)/l and > 50.10(9)/l was significantly lower in the GM-CSF group than in the control group, respectively 13 vs 19 days (p = 0.04), 15.5 vs 27 days (p < 0.02), 19 vs 51 days (p < 0.01). When compared with the control group, transfusion requirements and median of hospital stay were both significantly decreased for the patients receiving GM-CSF primed-PBPC. Our study confirms that infusion of GM-CSF primed-PBPC as a sole source of hematopoietic support improves hematopoietic reconstitution following myeloablative therapy.

Adult↗

PubMed1993

Beneficial effects of fish-oil supplements on lipids, lipoproteins, and lipoprotein lipase in patients with glycogen storage disease type I.

Glycogen storage disease type I (GSD-I) is frequently complicated by severe hyperlipoproteinemia and the increased potential risk of premature atherosclerosis. The effects of fish-oil supplementation [MaxEPA, 10 g.(1.73 m2)-1 for 3 mo] were investigated prospectively in seven hyperlipoproteinemic patients with GSD-I. Hypertriglyceridemia and hypercholesterolemia improved after 3 mo of fish-oil treatment, decreasing 49% (P < 0.005) and 23%, respectively. This was accompanied by a reduction in both low-density-lipoprotein (LDL) cholesterol (25%, P < 0.03) and apolipoprotein B (40%) and by increased high-density-lipoprotein increased (HDL) cholesterol (30%, P < 0.002) and apolipoprotein A-I (31%, P < 0.05). Low pretreatment ratios of HDL to total cholesterol and HDL to LDL, indicators of elevated atherosclerosis risk, increased significantly (P < 0.05). Plasma lipoprotein profile as well as lipoprotein composition [triglyceride (TG) enrichment and cholesteryl depletion] improved. Reduced TG concentrations were due to enhanced fat catabolism, as evidenced by the significantly increased hepatic and extrahepatic lipoprotein lipase activity (P < 0.05). Withdrawal of fish oil for 3 mo was associated with a return to pretreatment abnormalities in plasma lipids and lipoproteins. Fish-oil supplementation thus improves the hyperlipoproteinemia in GSD-I and may significantly reduce the risk of premature atherosclerotic cardiovascular disease.

Adolescent↗

PubMed1989