[Werner's syndrome and endocrine disorders]. / Syndrome de Werner et atteintes endocriniennes.

Werner's syndrome is a rare autosomal recessive disease caused by the mutation of DNA helicase gene (WRN), characterized by the premature onset of multiple age-related disorders and skin changes similar to those observed in scleroderma. Some endocrinologic and metabolic disorders have been described in patients with Werner's syndrome. We report one case in a 41-year-old man issuing from consanguineous parents, who presented for exploration of hypoglycemic episodes and sexual impotence. Werner's syndrome was diagnosed on the basis of his characteristic clinical appearance. Metabolic disorders were insulin-requiring diabetes and hypertriglyceridemia. Endocrinologic investigation revealed nodular goiter, sub clinical primary hypothyroidism, hypergonadotrophic hypogonadism,adrenal cortical hypofunction and GH deficiency. Pathology examination of the skin biopsy showed a scleroderma-like aspect. Finally, osteoporosis, atherosclerosis and sub-capsular cataract were associated. Thus, in Werner's syndrome metabolic and endocrinologic investigation is necessary in order to treat these disorders and improve the patient's prognosis and life.

Primary amenorrhea-galactorrhea with hyperprolactinemia and huge pituitary enlargement in juvenile primary hypothyroidism.

We report a girl with juvenile primary hypothyroidism revealed by growth retardation and a syndrome of primary amenorrhea-galactorrhea with hyperprolactinemia and suprasellar pituitary enlargement. Resolution of the pituitary enlargement and the amenorrhea-galactorrhea syndrome occurred after thyroid hormone replacement. No similar observation has been reported earlier in juvenile hypothyroidism.

[Antithyroid antibodies. Prevalence in primary hypothyroidism in Central Tunisia]. / Les anticorps antithyroïdiens. Prévalence dans l'hypothyroïdie primaire en Tunisie Centrale.

Thyroid microsomal (AAM) and thyroglobulin antibodies (AAT) were studied in sera of 112 patients with overt primary hypothyroidism in Central Tunisia. Thyroid antibodies were detected by an agglutination method. AAM and AAT were found respectively in 78.6% and 42%. The data confirm that there is a high prevalence of antibodies in primary hypothyroidism of short duration (AAM: 90%, AAT: 56.7%). The study reflect the prevalence of Hashimoto's thyroiditis and point to the usefulness of thyroid auto-antibodies in early screening of symptomless autoimmune thyroiditis.

[Primary hypothyroidism and glomerular involvement]. / Hypothyroïdie primitive et atteinte glomérulaire.

Association between auto-immune dysthyroidism and glomerulonephritis is quite rare. The authors report 3 cases with proteinuria varying from 2.72 to 6.06 g/day and hypothyroidism. Nephrotic syndrome was found in 2 cases and thyroid auto-antibodies (microsomal and thyroglobulin) and circulating immun complexes (C.I.C.) in 1 case, complement fractions C3 and C4 were normal in all cases. Renal biopsy showed membranous glomerulonephritis in 2 cases and minimal lesions in the third case. Proteinuria disappeared with glucocorticoids and thyroxine in 1 case, with thyroxine alone in an other case and persisted in the third case with normal thyroid function.