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Query: UMLS:C0011854 (type 1 diabetes)
 20,749 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Schmidt's syndrome (thyroid and adrenal insufficiency) and concurrent diabetes mellitus represent an intriguing multiple endocrinopathy in children. This report describes an eleven-year-old girl with diabetes of eight years' duration presenting in adrenal crisis. Serum thyroxine was undetectable, and antibodies to both thyroglobulin and adrenal tissue were found in high titer. The child's condition stabilized with hormonal replacement therapy, except for persistent growth failure. Approximately two years later she succumbed during a rapidly fulminant episode of ketoacidosis. The natural history of her illness supports recent speculation based on serologic data that juvenile diabetes mellitus may be an immunologic disorder in some children.
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PMID:Schmidt's syndrome in a child with diabetes mellitus. 55 85

We reported a 29-year-old woman with autoimmune polyglandular deficiency (APGD) type 1 accompanied by progressive myopathy. She had chronic mucocutaneous candidiasis at the age of 3, primary hypothyroidism at 12, insulin dependent diabetes mellitus at 27, and adrenal insufficiency at 29 years. Laboratory findings indicated an underlying defect in cell mediated immunity. Meanwhile, she had progressive muscular weakness and wasting at the age of 22 years which brought her to our hospital at 29 years. On admission, she could not walk without support and raise her arms up to the level of shoulders. Moderate to severe muscle wasting as well as weakness was observed in the limb girdle muscles. Serum CK levels were mildly elevated. A needle EMG examination disclosed short-duration and low-amplitude polyphasic motor units at voluntary contraction with few fibrillations and positive sharp waves at rest. On muscle CT examination, decreased density was detected in the neck extensor, paravertebral, rectus femoris, vastus intermedius, biceps femoris and soleus muscles. Muscle biopsy was performed on the biceps brachii and rectus femoris muscles. The former showed chronic dystrophic changes including marked variation in fiber size with necrotic and degenerating process, interstitial fibrosis, and lobulated and right fibers. In the latter, in addition to variation in fiber size with some necrotic fibers and occasional multi-core structures, nemaline bodies were seen in approximately 30% of muscle fibers. The progressive muscle involvement in our patient might be induced from 1) endocrine abnormality, 2) autoimmune disorder, and/or 3) coincidental complication of nemaline myopathy or limb girdle muscular dystrophy. The clinical and laboratory examinations, however, failed to support any of them.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of autoimmune polyglandular deficiency associated with progressive myopathy]. 145 27

We describe a patient with type I diabetes mellitus and hypothyroidism who developed frank adrenocortical insufficiency while receiving a high-dose ketoconazole therapy for keratitis caused by Acanthamoeba species. While impaired cortisol responses to corticotropin and mildly symptomatic hypoadrenalism have been described previously with ketoconazole therapy, to our knowledge, this case represents the first documented article of an actual adrenal crisis associated with this drug. Two reasons are postulated for the development of this complication in our patient: high-dose ketoconazole therapy given in divided doses during the day, and a possibly impaired central response to stress because of hypothyroidism. Our article points to the need to monitor patients treated with high-dose ketoconazole for adrenal insufficiency, particularly if associated illnesses are present that may impair an adequate stress response.
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PMID:Adrenal crisis in the setting of high-dose ketoconazole therapy. 270 31

Twenty patients with autoimmune endocrinopathies experienced 45 episodes of pleural and/or pericardial serositis. Seventeen of these patients were women and 15 had clinical or serologic evidence of 2 or more endocrinopathies. Idiopathic primary hypoadrenalism (10 cases), Graves' disease (8 cases), Hashimoto's disease (4 cases), atrophic thyroiditis with hypothyroidism (3 cases), idiopathic primary hypogonadism (3 cases), transient thyroiditides (2 cases), and type I diabetes mellitus (1 case) were diagnosed at a mean age of 24 years. Serositis recurred after asymptomatic intervals of months to years even in patients treated for endocrine dysfunction. Fourteen of 16 Caucasians had circulating immune complexes, including all 9 patients with a C4AQ0 (C4A null) phenotype and including all 12 patients with HLA antigens B8 and DR3, antigens associated with systemic lupus and with autoimmune endocrinopathies. Serositides associated with autoimmune endocrinopathies can occur with chest pain, fever, and exudative effusions in young Caucasian women with the HLA B8 DR3 C4AQ0 phenotype. These serositides may have a common pathophysiologic mechanism.
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PMID:Serositis with autoimmune endocrinopathy: clinical and immunogenetic features. 349 14

A 57-year-old white man manifested adrenal insufficiency, insulin-dependent diabetes mellitus reflected by diabetic ketosis, primary hypothyroidism, and primary hypogonadism on rapid withdrawal of glucocorticoid therapy of several years' duration for rheumatoid arthritis. Resumption and continuation of glucocorticoid therapy for six months resulted in remission of type I diabetes mellitus and hypogonadism, and a euthyroid state was achieved by replacement therapy with L-thyroxine.
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PMID:Onset of type I diabetes mellitus, hypothyroidism, and hypogonadism on withdrawal of glucocorticoid therapy and remission on its resumption in a patient with rheumatoid arthritis. 394 47

Thirty-four insulin-dependent diabetics with a coexistent organ-specific autoimmune disease (Graves' disease, primary myxedema, adrenal insufficiency, generalized vitiligo, primary biliary cirrhosis) were compared to 100 insulin-dependent patients in whom no obvious etiology was detectable. The autoimmune group was characterized by a predominance of females, a family history of autoimmune disease, a later age at onset, better glycemic control, low insulin requirement, persistence of ICA, and greater frequency of HLA B8 but not of B18. However, there was a large overlap between the two groups for all these criteria. In addition, a family history of IDD in first degree relatives and the frequency of serum positive for neutralizing anti-Coxsackie B antibodies were identical in the two groups. These results do not justify the separation of this group of patients as having purely autoimmune diabetes, to the exclusion of other etiological factors, whether genetic or viral.
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PMID:Clinical characteristics and etiological markers in insulin-dependent diabetes associated with an organ-specific autoimmune disease. 631 21

Studies on erythrocyte insulin receptors were performed in a boy with type I diabetes mellitus and superimposed adrenocortical insufficiency before and during treatment with hydrocortisone and 9-alpha-fluorohydrocortisone. The development of the adrenal insufficiency was associated with a progressively increased sensitivity to insulin which reverted after therapy. The maximum specific insulin binding of the erythrocytes was low during threatening addisonian crisis (6.9%) but normalized during hydrocortisone treatment (12.0%). These findings suggest that the increased insulin sensitivity characteristic for adrenocortical insufficiency is not an effect of an increased insulin receptor concentration and that hypocortisolaemia is associated with a down-regulation of the insulin receptors.
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PMID:Erythrocyte insulin receptors and insulin sensitivity in adrenocortical insufficiency. Report of a case of diabetes mellitus type I with superimposed Addison's disease. 633 70

Polyglandular autoimmune (PGA) syndrome is caused by autoimmune process in multiple endocrine glands. This usually results in endocrine gland hypofunction, except for the thyroid gland, in which both hyper or hypofunction may occur. The syndrome can be classified into two types, type I and type II, each with distinct clinical characteristics. We report three cases of PGA syndromes. The first patient had type I PGA syndrome, characterized by hypoparathyrodism, primary adrenal insufficiency and primary ovarian failure. She also had chronic mucocutaneous candidiasis, which is the distinct feature in this syndrome. The second patient had type II PGA syndrome, with primary adrenal insufficiency, Hashimoto's thyroiditis and primary ovarian failure. She also had widespread vitilgo. The last patient also had type II PGA syndrome. She had insulin dependent diabetes mellitus, Graves' disease and alopecia areata.
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PMID:Polyglandular autoimmune (PGA) syndromes: report of three cases and review of the literature. 786 20

The polyglandular autoimmune syndromes (PGA) are well known and are distinguished into type I, type II and type III. PGAI, also called APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy), is an autosomal recessive disorder, appearing in childhood and typically characterized by hypoparathyroidism (unusual in PGAII and PGAIII) and adrenal insufficiency. In APECED, autoimmune destruction of the pancreatic beta cells with development of insulin-dependent type 1 diabetes is possible, but less frequent than in the other PGAs, especially PGAII. The pathogenesis of this unique autoimmune disease is unknown. No HLA association seems to exist and genetic studies have assigned the autosomal APECED locus to chromosome 21. The case of a 28-years-old female suggesting the diagnosis of APECED, is presented, characterized by psycho-somatic abnormal development, teeth alterations, post-puberal gonadal failure with dystrophic hypoplasia of external genitalia, previous vaginal candidiasis, a slowly developing juvenile brittle diabetes. Intestinal malabsorption induced by Giardia lamblia occurred (probably resulting, like candidiasis, from immunological anergy). A strong familiarity linked to female sex was noticed (the mother, a sister, the little nice and some maternal female cousins being affected) while the father and a brother were healthy. Diabetes seems to be characterized by early onset and severe complications. In this patient no organo-specific antibodies were detected and the only immunologic disorder was a small decrease of CD3 and CD4/CD8 ratio, both CD4 and CD8 being at the lower normal range. This patient (and her female maternal relatives) needs a long-term follow-up in order to evaluate the function of endocrine glands and to initiate early treatment for hormonal deficits, as well as to detect the non-endocrine components of disease.
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PMID:[A rare case of juvenile diabetes mellitus associated with APECED (autoimmune poly-endocrinopathy, candidiasis and ectodermal dystrophy) with strong X-linked familial inheritance]. 930 48

Polyglandular autoimmune (PGA) syndromes (types I and II) may affect various endocrine and non-endocrine organs in the body. In the commoner PGA type II, primary adrenal insufficiency, autoimmune thyroid disease and type I diabetes mellitus are the most frequent manifestations. Serositis with pericardial or pleural involvement is not a well known component of the disease. Here, we report a 21-year-old man who first presented with a pleuropericardial effusion and Graves' disease, and who then developed type I diabetes mellitus.
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PMID:Polyserositis as a rare component of polyglandular autoimmune syndrome type II. 1140 17


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