[Werdnig-Hoffman disease (apropos of 3 cases)].
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Biomedical subjects
Publications and source records attributed to C Giordano.
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The latency of pattern-reversal VEPs was studied in type I insulin-dependent diabetics without retinal and extraocular involvement. One hundred eyes of 50 diabetics (mean age 19.8 +/- 7.2 years) formed the study group; the duration of the disease ranged between 1 month and 15 years. The latencies of VEPs were progressively delayed in relation to the duration of the disease, becoming more and more evident and stabilizing after about 6 years from the onset of diabetes. The VEP alterations probably indicate alteration of membrane balance or demyelinization.
Patients with type I (insulin-dependent) diabetes mellitus maintain B-cell function for a varying period of time after onset. This is commonly held to account for post-initial remission. To estimate residual B-cell function we measured plasma and 24-h urinary C-peptide in 68 type I diabetic patients (age range 4-35 years, within 10-180 days of the onset of symptoms, typed for HLA-A, -B, -C and DR loci. A positive correlation (r = 0.26; p less than 0.05) was found between urinary C-peptide levels and the age of the patient. The analysis of variance of urinary C-peptide values on the basis of the presence or absence of DR3 and DR4 antigens revealed that the DR3-positive patients had reduced excretion (15.2 +/- 9.2 SD micrograms/24h) with respect to the others (22.7 +/- 15.5 SD micrograms/24h) (F = 6.35; p less than 0.05). No interaction effect was found in DR3/4 positive patients. Hence, late onset patients appear to have higher residual C-peptide secretion. In the light of these findings, the assessment of B-cell function and genetic profile may be useful in predicting which patients are likely to have remission periods and identifying the metabolic consequences of even minimal endogenous insulin secretion.
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The response of circulating leukocytes to thyroid subcellular fractions was investigated in 19 patients with Graves' disease, 15 patients with Hashimoto's thyroiditis, 7 patients with toxic adenoma, 19 patients with nontoxic goiter and in 10 healthy students as control subjects. For this purpose, the leukocyte migration test of Soborg and Bendixen was performed against human crude thyroid extract (CTE), cell plasma membranes, nuclei, ribosomes, mitochondria and microsomes. Our results show positive LMT against: 1) CTE in patients with Graves' disease (61 +/- 13, p less than 0.001) and Hashimoto's thyroiditis (65 +/- 11, p less than 0.001) compared to controls (90 +/- 11); 2) cell plasma membranes in patients with Graves' disease (41 +/- 14, p less than 0.001) and Hashimoto's thyroiditis (64 +/- 21, p less than 0.05) compared to controls (88 +/- 19); 3) nuclei in patients with Graves' disease (53 +/- 25, p less than 0.001) and Hashimoto's thyroiditis (53 +/- 23, p less than 0.001) compared to controls (83 +/- 11). Our findings of circulating leukocytes sensitized to cell plasma membranes and nuclear fraction in patients with Graves' disease and Hashimoto's thyroiditis provide the additional information that these patients have a specific defect in immune-surveillance.
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