1/16. Three patients with adrenal tumors having been treated simply for diabetes mellitus.Three patients with functional adrenal tumors, Cushing's syndrome, primary aldosteronism and pheochromocytoma, who underwent adrenalectomy and were subsequently cured, were studied. All these patients had been treated for diabetes for several years before the diagnosis of adrenal tumors. In each case the state of diabetes before and after surgery, including parameters of insulin secretion and insulin resistance, was compared to demonstrate how the adrenal disorder influenced the nature of diabetes. In the case of Cushing's syndrome the hypercortisolemia caused insulin resistance in the peripheral tissues. In the case of primary aldosteronism, excessive production of aldosterone diminished insulin secretion possibly through hypokalemia. pheochromocytoma affected both insulin secretion and insulin sensitivity through hypersecretion of catecholamines. In all these patients the adrenal tumors were found in clinical contexts other than management of diabetes itself. By careful retrospective review of these three patients' history, several important points that might have drawn the physician's attention to the underlying adrenal disorders were pointed out. These included past history of acute myocardial infarction with onset at unexpectedly young age in the case of Cushing's syndrome and unexpectedly high insulin resistance for the patient's body mass index in the case of pheochromocytoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/16. A unique case of central diabetes insipidus (DI) associated with transient pituitary stalk enlargement: close observation over several years using magnetic resonance imaging (MRI) and hypophysial endocrine tests.We had the opportunity to closely observe a unique case of central diabetes insipidus (DI), in which dramatic changes in both radiological findings and hypophysial functions were seen. A 63-year-old female developed central DI, and magnetic resonance imaging (MRI) revealed a mild thickening of the pituitary stalk and lack of hyperintense signal associated with normal neurohypophysis on T1-weighted images. About three months later, the stalk was found to be remarkably expanded like neoplasm; however, anterior pituitary functions were almost normal on that occasion, except for the absence of GH response to an insulin tolerance test. About nine months after the onset of DI, secondary hypoadrenalism and hypothyroidism, which required replacement therapy, developed transiently, but recovered about one year later. Results of hypophysial endocrine tests during this period showed that the dysfunction was predominantly suprapituitary in nature. As time passed, the stalk lesion began to shrink spontaneously and another MRI, obtained five years after the onset of DI, disclosed normal findings for the infundibulo-hypophysial system, except for lack of the hyperintense signal of the neurohypophysis. The patient has since been healthy, except for the DI, which has been controlled by treatment with vasopressin. We report here a unique case of central DI associated with transient pituitary stalk enlargement.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/16. wolfram syndrome.The wolfram syndrome is a rare dysmorphogenetic disease of autosomic recessive hereditary nature. The pathogenesis of the disease is still not well known. It is characterised by the presence of diabetes insipidus, diabetes mellitus, optic atrophy and deafness. Other anomalies, such as renal outflow tracts and multiple neurological disorders may develop later. In our case report the diabetes mellitus appeared at the age of 4; the hearing loss and renal disturbances at the age of 11; the optic atrophy at the age of 16. No signs of ataxia, diabetes insipidus and neurologic anomalies were found. The diagnosis of wolfram syndrome is not always easy in the first stages of the disease. The suspect may come from the presence of a juvenile diabetes mellitus asssociated with optic atrophy. For the diagnosis a valid clue can be given from the results of some clinical tests such as the positivity of the visual evoked potentials and the retinogram reliefs and the exclusion of the autoimmune origin of the diabetes mellitus. Other signs such as the progressive sensorineural hearing loss, the presence of nystagmus and of urodynamic disturbances and renal complications makes the diagnosis of this syndrome easier.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/16. Clinical aspects of mitochondrial disorders.Mitochondrial disorders have long been regarded as neuromuscular diseases only. In fact, owing to the ubiquitous nature of the oxidative phosphorylation, a broad spectrum of clinical features should be expected in mitochondrial disorders. Here, we present eight puzzling observations which give support to the view that a disorder of oxidative phosphorylation can give rise to any symptom in any organ or tissue with any apparent mode of inheritance. Consequently, we suggest giving consideration to the diagnosis of a mitochondrial disorder when dealing with an unexplained association of symptoms, with an early onset and a rapidly progressive course involving seemingly unrelated organs. Determination of lactate/pyruvate and ketone body molar ratios in plasma can help to select patients at risk for this condition.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/16. The glucagonoma syndrome and its management.The glucagonoma syndrome occurs in some but not all patients with a benign or malignant islet cell tumor and hyperglucagonemia. Manifestations may include anemia, diabetes mellitus, pruritic skin rash, glossitis, stomatitis, weight loss, diarrhea, flexible fingernails, venous thromboses, low plasma amino acid levels, and coarse folds of the jejunum and ileum. Most patients are postmenopausal women, but men and women ages 40 to 65 have been affected. The course is variable depending upon the nature of the underlying tumor. Twenty-two cases of probable glucagonoma syndrome have been reported; twelve documented with glucagon levels. The hyperglucagonemia results from elevation of the proglucagon and true glucagon immunoreactive fractions of pancreatic glucagon. Management of the rash can be accomplished rarely with topical or systemic antibiotics or corticosteroids. If the tumor is resectable, surgery reverses the syndrome. patients with metastatic disease have responded to streptozotocin and DTIC.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/16. Clinical and etiological heterogeneity of idiopathic diabetes mellitus. The banting memorial lecture.We have presented and reviewed evidence for the heterogeneous nature of diabetes mellitus in terms of genetics, environmental factors, insulin responses to glucose and vascular disease. We have reviewed evidence for heterogeneity between juvenile-onset diabetes (JOD) and maturity-onset diabetes (MOD) and maturity-onset diabetes of young (MODY) and for heterogeneity within groups of JOD and MOD and MODY patients. Although much remains to be learned, a beginning has been made and suggests that primary diabetes mellitus is not a single specific disease but a syndrome comprised of a variety of diseases all characterized by hyperglycemia and tissue changes that result from heterogeneous etiologic and pathogenetic factors. Future classifications of primary diabetes mellitus will undoubtedly be lengthy, as are for other diseases and syndromes also caused by a variety of etiologic and pathogenetic mechanisms.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/16. Undetected diabetes and the plastic surgeon.Plastic surgery candidates are generally healthy. Therefore, major postoperative complications are rare. Should they happen, the surgeon should search for possible causes, one of which is undetected diabetes mellitus. Six patients are presented who, based on the individual or family history or the unusual nature of their complications, were suspected of having diabetic tendencies. This experience necessitated our in-depth search into the role of silent or undetected diabetes. This report emphasizes the importance of positive family history of diabetes and the role of glucose tolerance tests on suspected cases. Even with normal glucose tolerance tests, however, some of these patients with a positive family history of diabetes and history of previous infections suffer from deficiencies in the chemotactic immune system. We recommend full discussion of the increased risk of infection and delayed healing with these patients, conservatism during surgical procedures, and prophylactic use of antibiotics perioperatively.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/16. Severe persistent biphasic local (immediate and late) skin reactions to insulin.A patient with adult-onset insulin-requirging diabetes mellitus had persistent severe local reactions to all available insulins of animal origin. skin reactions were biphasic in nature with both immediate and late characteristics. An extensive immunologic investigation of this problem was undertaken, revealing evidence of reaginic antibody involvement in the reactions. Routine histologic studies suggested the possibility that Arthus-type mechanisms played a part, although this impression was not confirmed by immunofluorescent microscopy. A program of medical management provided some relief of symptoms.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/16. A new case of abnormal insulinemia with diabetes. Reduced insulin values determined by radioreceptor assay.A 44-yr-old Japanese woman was found to have diabetes with marked fasting hyperinsulinemia. Her fasting plasma glucose, serum insulin, and c-peptide levels were 137 mg/dl, 204 microU/ml, and 1.13 pmol/ml, respectively, and the c-peptide-to-insulin molar ratio was markedly reduced. insulin antibodies and insulin-receptor antibodies were negative. fasting levels of counter-insulin hormones were normal. She had normal hypoglycemic response to exogenous insulin injection. Binding of 125I-labeled insulin to erythrocytes was normal. Oral glucose-tolerance tests in eight members of her first-degree relatives revealed four members (mother, sister, brother, and daughter) with fasting hyperinsulinemia (111-314 microU/ml), and two of them (mother and sister) were overtly diabetic. Thus, the abnormality was thought to be an autosomal dominant trait. Reverse-phase high-performance liquid chromatograph analysis of immunopurified insulin obtained from her serum revealed two peaks of insulin immunoreactivity. The amount of the abnormal insulin peak was seven times greater than that of normal insulin. The abnormal insulin was eluted after bovine, human, and porcine insulins, indicating it has a more hydrophobic nature than normal human insulin. Radioreceptor assay (RRA) for serum insulin with guinea pig kidney membrane revealed that the binding activity of their serum insulin was markedly decreased. Discrepancies between the values measured by RRA and those measured by radioimmunoassay were also found in her family members with hyperinsulinemia but not in her family members without hyperinsulinemia and other hyperinsulinemic patients.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/16. Parental attitudes toward children with a chronic medical disorder: special reference to diabetes mellitus.Children who suffer from chronic medical disorders requiring continuous therapy create threatening situations for parents, which are often acute at diagnosis and may continue over the years. Many parental reactions and consequent emotional interactions with the children are common to all medical handicaps, but also vary with the nature of the particular disease and its treatment. Attitudes of parents to the diagnosis of a chronic handicap or medical disorder will always include some apprehension. This is usually more acute in mothers than in fathers, both by their nature and position of authority in the care of the child in most homes. Some anxiety is normal at diagnosis of a chronic ongoing illness in a child and may even help in making the mother more aware of the extra responsibility that has been thrust on her and give her the desire to manage the treatment well.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
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