Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011849 (diabetes)
277,896 document(s) hit in 31,850,051 MEDLINE articles (0.01 seconds)

The female patient initially showed the acquired type of total lipoatrophy at about 8 years of age. At 12 years of age, the onset of diabetes mellitus was speculated from advanced pyodermia and dedentition. At 29 years of age, glucosuria was found, and she developed proteinuria, ascites, and pretibial edema. The physical examination revealed: hepatosplenomegaly, complete absence of subcutanous fat, cutaneous xanthomas, and emaciated facies with pronounced zygomatic arches. Diabetic retinopathy was revealed in the ophthalmological examination, and nephropathy was evident in renal biopsy specimens. She also had peripheral diabetic neuropathy. No adipose tissue was found in the mesenterium under peritoneoscopy. The hepatic biopsy specimen revealed advanced portal liver cirrhosis. Laboratory findings included: hyperlipidemia, elevation of BMR without evidence of hyperthyroidism, impaired renal function, and undetected anti-insulin antibodies and anti-insulin antibodies. Endocrinological examinations revealed normal value, except for an impaired hGH response in the arginine test. C-peptide immunoreactivity was high. Her condition was fairly well controlled by 140 units of insulin injection daily.
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PMID:Lipoatrophic diabetes. Report of a case. 15 92

A 21 years old female patient with lipoatrophic diabetes, a distinct syndrome of insulin resistant diabetes mellitus, loss of subcutaneous fat, hepatosplenomegaly, hyperlipidemia, increased basal metabolic rate, subvalvular aortic stenosis and cystic bone lesions is described. She exhibited all clinical signs of diabetic microangiopathy. Quantitative estimation revealed severely thickened basement membranes of muscle capillaries. It is concluded that the extent of her microangiopathy is a consequence of her excessive metabolic abnormalities.
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PMID:Muscle capillary basement membrane thickness in lipoatrophic diabetes. 59 1

Lipoatrophic diabetes is characterized by: complete lack of subcutaneous fattissue (generalized lipodystrophy), insulin resistant diabetes mellitus, hepatosplenomegaly, excessive hyperthyroidism, elevated basal metabolic note without hyperthyroidism. Recently, "cystic" alterations in the bone have been described as a possible further characteristic. The case of a young woman, now aged 21, who has had generalized lipodystrophy for 17 years and overt diabetes mellitus for 6 years is reported.
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PMID:[Lipoatrophic diabetes. Report of a case (author's transl)]. 80 20

Histoplasma capsulatum is not endemic in Britain. We report a case of disseminated histoplasmosis in an English man who had not ventured out of northern Europe for 30 years. The disease presented as painful mouth ulcers and hepatosplenomegaly six months after he had developed maturity-onset diabetes. The origin of the infecting fungus may have been from within the United Kingdom or alternatively it may have existed as an intraoral saprophyte for over 30 years.
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PMID:Disseminated histoplasmosis in an English patient with diabetes mellitus. 85 18

Disseminated histoplasmosis is a rare and potentially fatal disease caused by the dimorphic soil fungus Histoplasma capsulatum. We describe a 67-year-old man with diabetes who presented with a 6-month history of fever, weight loss, and mental deterioration; using computed tomography (CT), we found hepatosplenomegaly and bilateral adrenal masses. CT-directed adrenal biopsy showed yeast forms consistent with H capsulatum. Therapy with ketoconazole was accompanied by adrenal insufficiency (treated successfully with prednisone and fludrocortisone) and produced an excellent therapeutic response. We address the differential diagnosis of bilateral adrenal enlargement, and we discuss the clinical features, diagnosis, and treatment of disseminated histoplasmosis, a disease that includes parts of the Southwestern United States in its endemic region.
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PMID:Disseminated histoplasmosis with bilateral adrenal enlargement: diagnosis by computed tomography-directed needle biopsy. 203 63

A women presented at the age of 22 with anemia, hepatosplenomegaly, polyclonal hypergammaglobulinemia and a mediastinal shadow. At thoracotomy she had enlarged mediastinal lymph nodes which displayed histological features typical of angiofollicular hyperplasia. Marrow plasmacytosis was present. She developed diabetes mellitus at the age of 29 yrs after she had received oral steroid treatment for one year. The nephrotic syndrome supervened another year later. Her kidneys were enlarged before the onset of diabetes. The glomerular changes included a marked increase of mesangial matrix and segmental hypercellularity. The association of the nephrotic syndrome and angiofollicular lymph node hyperplasia is extremely rare and their interrelation remains enigmatic.
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PMID:Nephrotic syndrome associated with angiofollicular lymph node hyperplasia. 344 67

A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity known as POEMS syndrome.
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PMID:An unusual case of POEMS syndrome. 357 Jul 37

Type V hyperlipoproteinemia is an unusual entity in children. Only 6 cases have been described so far to our knowledge. Authors present a 9 year old male that came for diagnosis of a hepatosplenomegaly. There was no evidence of abdominal pain, xanthomas or pancreatitis. Secondary disorders such as uncontrolled insulinopenic diabetes mellitus, glycogen storage disease, administration of estrogen compounds, nephrotic syndrome or uremia, and dysglobulinemias were excluded. His father presented the same lipoprotein pattern suggesting a dominant mode of inheritance. The administration of heparin showed a good response of serum proteinlipase.
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PMID:[Primary hyperlipoproteinemia in childhood (author's transl)]. 728 88

A new syndrome has been described comprising polyneuropathy, oedema, hyperpigmentation and thickening of the skin, gynaecomastia in males and amenorrhoea in females, monoclonal gammopathy, papilloedema and diabetes. There is frequent osteosclerosis with or without plasmacytoma, hepatosplenomegaly and polycythaemia. There is a good response to corticosteroids, immunosuppressive drugs and occasionally to excision or irradiation of the plasmacytoma. This syndrome was first described in Japan, and is still seen predominantly there, and only occasionally in other areas. An example in a 51-year-old Spanish female is described: she had a spectacular response to prednisone and melphalan. The aetiopathogenic possibilities are discussed.
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PMID:Plasma cell dyscrasia with polyneuritis and dermato-endocrine alterations. Report of a new case outside Japan. 741 45

We describe a 52-year-old male manifesting severe insulin resistance associated with hypereosinophilic syndrome (HES). Diabetes mellitus was initially well-controlled by an oral hypoglycemic agent, and thereafter by human insulin. Due to the progression of hypereosinophilia, hepatosplenomegaly and peripheral lymphoadenopathy, severe insulin resistance associated with diabetic ketoacidosis occurred repeatedly, despite intravenous administration of over 1,000 U per day of human insulin. A high plasma insulin-binding capacity as determined by Scatchard analysis was consistent with insulin antibody-mediated resistance. The diagnosis of HES was made due to the persistent elevation of eosinophil count and associated liver and cardiac damage. Glucocorticoid therapy successfully achieved both reducing clinical symptoms and improving glycemic control.
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PMID:Severe insulin-resistant diabetes mellitus associated with hypereosinophilic syndrome. 782 82


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