1/12. Inherited abnormal thyroid hormone-binding protein causing selective increase of total serum thyroxine.A 9-yr-old boy is described in whom increased serum T4 concentration, increased T3 uptake, and increased free T4 index were associated with a euthyroid clinical state with normal total serum T3. T4-binding globulin (TBG), measured by RIA, was decreased. Reverse flow paper electrophoresis of serum proteins after reaction with radioactively labeled T4 demonstrated increased binding of T4 to a protein with electrophoretic mobility corresponding to albumin. Displacement of serum protein-bo-nd [125I]T4 activity by increasing concentrations of T4 revealed the presence of a low affinity, high binding capacity system with an association constant similar to that of T4-binding prealbumin. This low affinity binding protein cochromatographed with TBG on a DEAE-Sephadex column which normally separates TBG from T4-binding prealbumin. At free T4 concentrations equivalent to those present in the plasma of normal individuals, the T4 bound to free ratio is higher in the patient than in normals and the total serum T4 level is increased in the presence of normal free T4 concentrations. The relative affinity of this abnormal T4-binding protein for T3 is low compared to that of TBG. The patient's father had the same abnormal binding protein, which was not found in his mother or fraternal twin brother. These data suggest an autosomal dominant mode of inheritance of an aberration leading to synthesis of a new protein instead of normal TBG. The new protein is different from TBG in electrophoretic mobility, T4 and T3 binding, and antigenic properties.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/12. Sudden sensorineural hearing loss: a case report supporting the immunologic theory.Sudden sensorineural hearing loss (SSNHL) is one of the autoimmune diseases of the inner ear (AIED), which is characterized by a hearing loss of above 30 decibels in at least three contiguous audiometric frequencies over a time course of 72 hours or shorter. Its cause can be found in only 10% to 15% of patients. Histopathologic findings have reported retrograde neuronal degeneration and atrophy of Corti's organ and of the vascular stria. This paper describes a case of a middle-aged female patient undergoing a treatment for hyperthyroidism who developed bilateral SSNHL. The patient was treated with methylprednisone (1 mg/kg/day) for three days with considerable hearing improvement. This treatment was followed by lung and kidney tuberculosis. The immune mechanism of this entity and the possibility of interconnected participation of the antigen type, of an autoimmune disease and of bacterial infection are discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/12. An informative case of Graves' disease with implications for schizophrenia.The aetiology of schizophrenia and the other psychoses is not yet established. The Knight model, based on genetic and other evidence, proposes that schizophrenia is an autoimmune disease, caused by the development of forbidden clones of B lymphocytes that secrete autoantibodies that accidentally stimulate cell surface receptors on certain neurons, affecting the limbic system of the brain. An unusual defect in a Maori man with Graves' disease rendered him unresponsive to the usually effective antithyroid drugs, prompting his being treated with prednisone, a non-specific immunosuppressant agent. This was highly successful, reducing the blood level of the causative thyroid-stimulating autoantibodies with reduction of thyroid hormone levels and thyroid gland size. Unfortunately, high dosage prednisone can be used for only a month, because of steroid toxicity. A research pathway to effective therapy of receptor-mediated autoimmune diseases, which probably include the psychoses, is now apparent. It involves finding the autoantibodies, then cloning of their antigenic targets, as has been done for Graves' disease. This will provide knowledge of the peptide sequences necessary for constructing therapeutic agents for selectively destroying the pathogenic forbidden clones. Meanwhile, usage of short-term therapy with prednisone could be helpful in the management of schizophrenia and should be explored.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/12. hyperthyroidism associated with primary cirrhosis. Two case reports.Only a single case of Graves' disease has been reported so far in Primary Biliary Cirrhosis (PBC), whereas hypothyroidism is a rather common association. We report two cases of hyperthyroidism associated with PBC. A common pathogenic mechanism involving HLA II class antigens is suggested.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/12. graves disease presenting as painful thyroiditis.hyperthyroidism associated with subacute (painful, viral) thyroiditis is well-recognized as a clinical entity; the thyroid gland in graves disease is minimally, if ever, tender and painful. We describe a 10-year-old girl with hyperthyroidism whose initial clinical presentation was predominantly a painful, tender goiter. graves disease was established by high uptake of 131I with a diffuse pattern of distribution of radioactivity on scan and the presence of thyroid-stimulating antibody. thyrotropin-binding inhibiting IgG and antibody to thyroid microsomal antigen were both positive. She responded well to treatment with propylthiouracil and had spontaneous regression of her thyroid pain. The cause of the severe pain and tenderness remains speculative.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/12. Evidence of a new antigen-antibody system (anti-Mic-1) in patients with systemic lupus erythematosus and hyperthyroidism.Autoimmune thyroid diseases may occur in association with systemic rheumatic disorders, and usually they show a high prevalence of antithyroglobulin and antimicrosomal antibodies. We report 6 patients with the clinical association of systemic lupus erythematosus (SLE) and hyperthyroidism. Of interest, in 5 of the 6 patients (83%), we found an antibody directed against a microsomal extract of human thyroid gland which was different than previous microsomal antibodies in that it was a precipitating antibody; we have called it anti-Mic-1 antibody. We investigated the prevalence of this specific autoimmune reaction in 58 patients with idiopathic SLE, 30 with hyperthyroidism, 15 with Hashimoto's thyroiditis, 25 with insulin dependent diabetes mellitus, 45 with rheumatoid arthritis, and 25 healthy controls. No control had anti-Mic-1 antibody. In addition, this antibody was shown to be organ specific. We suggest that patients with the combined association of SLE and hyperthyroidism may represent a different subset in the spectrum of SLE. The high prevalence of this antigen-antibody reaction in these cases may serve as a serological marker of this association.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
7/12. Thyroid antigen-antibody nephritis: possible involvement of fucosyl-GM1 as the antigen.hyperthyroidism, microscopic hematuria, and proteinuria developed in an 11-year-old girl. proteinuria decreased during treatment of hyperthyroidism with an antithyroid drug. On admission, serum anti-thyroglobulin antibody, antimicrosomal antibody, and immune complex were present. The thyrotropin binding inhibitory immunoglobulin (TBII) level was low. On the other hand, an antibody to the ganglioside component (fucosyl-GM1) was detected by an enzyme linked immunosolvent assay (ELISA). A thyroid biopsy specimen showed massive lymphocytic infiltration and interstitial fibrosis. A renal biopsy specimen showed marked proliferation of mesangial cells and increased mesangial matrix with focal segmental capillary wall abnormality. Electron microscopec studies demonstrated mild paramesangial dense deposits. By indirect immunofluorescence, granular glomerular basement membrane and mesangial staining were not detected with rabbit antibody to thyroglobulin, but were detected with rabbit antibody to fucosyl GM1. Fucosyl GM1 was also seen along the basilar aspect of the thyroid follicular epithelial cells. These observation suggests the development of glomerulonephritis mediated by thyroid antigen, particularly ganglioside component.- - - - - - - - - - ranking = 9keywords = antigen (Clic here for more details about this article) |
8/12. Thyrotoxic periodic paralysis.A case of thyrotoxic periodic paralysis is reported in a Hispanic man with an unusual recurrence six weeks after radioactive iodine treatment. Thyrotoxic periodic paralysis has now been well characterized in the literature: it occurs primarily in Orientals with an overwhelming male preponderance and a higher association of specific hla antigens. Clinical manifestations include onset after high carbohydrate ingestion or heavy exertion, with progressive symmetric weakness leading to flaccid paralysis of the extremities and other muscle groups, lasting several hours. If hypokalemia is present, potassium administration may help abort the attack. Although propranolol can be efficacious in preventing further episodes, the only definitive treatment is establishing a euthyroid state. The pathophysiology is still controversial, but reflects altered potassium and calcium dynamics as well as certain morphologic characteristics within the muscle unit itself.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/12. Immune response in fulminant viral hepatitis, type B.Serial serum samples from the time of exposure until fatal outcome in 3 patients with fulminant viral hepatitis, type B, were examined for the presence of the antigens associated with hepatitis, type B, and their corresponding antibodies. The titers of hepatitis b surface antigen (HBsAg) were found to decrease by greater than 50% before death. Antibody to surface antigen (anti-HBs) was not detectable in any sample. Patterns of antibody to core antigen (anti-HBc)), HBsAg subtype "e" antigen, and anti- "e" were unremarkable, and could not be distinguished from those that might occur in many self-limited cases of hepatitis, type B. A rise in alpha-fetoprotein before demise suggests that late but inadequate liver regeneration occurred in these patients.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
10/12. hyperthyroidism with normal serum total T4, T3, and thyroid binding protein concentration.A 60-year-old woman, free of other disease, with severe clinical hyperthyroidism had normal total serum T4 with elevated free T4 concentration and T3 resin uptake characteristic of hyperthyroidism, with thyroid binding globulin (TBG) deficiency. However, serum TBG concentration as determined by immunoassay was normal, suggesting that the biochemical defect affecting the T4 binding sites did not affect is antigenicity. Her total and free serum T3 levels were low-normal with a markedly elevated reverse T3 concentration. These findings, in the presence of clinical hyperthyroidism, are consistent with an impairment in peripheral conversion of T4 to T3, apparently, in this case, due only to long-standing severe hyperthyroidism.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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