Multiple relapses of hyperthyroidism after thyroid surgeries in a patient with long term follow-up of sporadic non-autoimmune hyperthyroidism.

Constitutively activating thyrotropin receptor (TSHR) germline mutations have been identified as a molecular cause of congenital hyperthyroidism. Patients with relapsing hyperthyroidism were previously treated with surgery and radioiodine. We report on a 22-year-old male patient who was treated for his multiple relapses of hyperthyroidism by repeated subtotal thyroidectomies (STE). During the 22 years of follow-up, the patient developed several relapses of hyperthyroidism, four of them after thyroid surgeries. Sequencing of the TSHR gene revealed a gain-of-function mutation with an amino acid exchange of aspartate to tyrosine in codon 633 which is located in the sixth transmembrane domain of the TSH receptor. The absence of the mutation in all other family members identifies the patient's TSHR mutation as a sporadic germline mutation. In this patient, thyroid tissue growth and hyperthyroidism could repeatedly be controlled only for limited periods by near total thyroidectomy. Therefore, this case confirms that early combined treatment with near-total thyroidectomy plus radioiodine therapy seems to be the treatment of choice for patients with sporadic non-autoimmune hyperthyroidism.

Studies of 9-fluorenyl carbocations. intramolecular hydride migration in a substituted 9-fluorenyl carbocation.

The substituted fluorenyl cation, 9-(diphenylmethyl)fluoren-9-yl cation (4), is formed under stable ion conditions (low temperature/strong acid) from its corresponding alcohol 3. This ion is transformed to a substituted diphenyl methyl cation 8 at ambient temperature via an apparent 1,2-hydrogen shift. Irradiation of 9-(diphenylmethyl)fluoren-9-ol in methanol gives products derived from the corresponding cation along with radical-derived products from C-C and C-O homolysis processes. The laser flash photolysis of this alcohol gave a transient corresponding to cation 4. All of the photoproducts are derived from cation 4 or radical pathways. High level MO calculations point to a high barrier (23.8 kcal x mol(-1)) for the 1,2-hydride shift. This barrier is the consequence of the minimum energy conformation of this fluorenyl cation which is less than ideal for the periplanar geometry necessary for this process.

Autoimmune thyroid disorders in juvenile chronic arthritis and systemic lupus erythematosus.

The appearance of autoimmune thyroiditis in the course of other autoimmune diseases, which do not affect specific organs (systemic lupus erythematosus, Sjögren syndrome, rheumatoid arthritis and others), is more frequent than is usually believed. Nevertheless, it is scarcely studied, especially in children. The purpose of this study was to look for autoimmune lesions of the thyroid gland in children suffering from juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE). Twenty seven children having JCA and twelve children with SLE, aged 5 to 18 years, were enrolled into study. In all of them the disease was in an active phase. The serum levels of total thyroid hormones (T3, T4) and TSH, thyroid antibodies (TAB and MAB) and antinuclear antibodies (ANAB) were analyzed using respectively fluoroimmunologic, microhemagglutination and indirect immunofluorescention tests. According to our results, autoimmune thyroiditis was found in 12 out of 27 children with JCA (44.4%); 85.2% of them were euthyroid, 11.1% had a compensated hypothyroidism, and 3.7% had Hashi-toxicosis. From a clinical point of view, very interesting was the combination of JCA, autoimmune thyroiditis and pseudoxanthoma elasticum in a 13-year old girl. Positive thyroglobulin antibodies (1:80-1:5120) were found in 17 out of 27 cases of JCA (63%). The microsomal antibodies were elevated (1:100-1:1600) in 7 out of 27 (25.9%); antinuclear antibodies (1:80-1:640) were detected in 15 out of 27 cases of JCA (55.5%). A simultaneous elevation of all three kinds of antibodies was found in 14.8% of children with JCA, and of TAB and MAB--in 18.5%. Thyroid gland disorders were detected also in children suffering from SLE. Thyroglobulin antibodies were positive (1:80-1:5120) in 7 out of 12 cases. Antinuclear antibodies (1:320-1:2560) were detected in 8 out of 12 cases (66.7%). The serum levels of T3, T4 and TSH were in the reference limits in all children with SLE. The present study suggests that involvement of the thyroid gland is not uncommon in autoimmune disease in Autoimmune thyroiditis can occur in association with other autoimmune diseases, affecting some organs or systems, such as the insulin-dependent diabetes mellitus, pernicious anaemia, thrombocytopenia, vitiligo, as well as some chromosomal aberrations--Turner's syndrome, Noonan's syndrome and Down's disease [1]. The appearance of autoimmune thyroiditis together with other autoimmune diseases which do not affect specific organs (such as systemic lupus erythematosus, Sjögren syndrome) is the reason to classify them in a common subgroup of the autoimmune polyendocrine syndromes--type IIID [2]. The rheumatic diseases are--more frequently than suspected--associated with autoimmune thyroiditis, but this connection is not well studied. The literature offers very scarce information on the problem, especially for the childhood. The purpose of this study was to look for autoimmune lesions of the thyroid gland in children suffering from juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE).