Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten instances of total pancreatectomy performed for chronic alcohol induced pancreatitis are reported. There was no hospital mortality, and all of the patients were free of pain. The most difficult problem was labile insulin sensitive diabetes in these patients who were chronic alcoholics. In addition, steatorrhea with weight loss, bleeding marginal ulcers and general weakness diminished working ability. The present data suggest that this procedure should be considered as the last resort in the treatment of severe instances of chronic pancreatitis.
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PMID:Total pancreatectomy for chronic pancreatitis. 388 50

Duchenne muscular dystrophy (DMD) is an X-linked inherited neuromuscular disease characterized by progressive weakness and severe muscle wasting. Alterations in carbohydrate metabolism are often associated with neuromuscular disorders. We performed oral glucose tolerance tests and insulin binding studies on erythrocytes from 17 DMD and 8 normal males. Furthermore, we measured insulin binding to erythrocytes from 12 normal males and from 11 mothers and 10 sisters of affected males. As a group, DMD patients had mild glucose intolerance and both fasting and postabsorptive marked hyperinsulinemia (insulin resistance). Levels of glucose and insulin, expressed as incremental areas under their respective curves, were significantly elevated in the wheelchair-ridden patients. Incremental areas of glucose (0-2 h) and insulin (0-5 h) were 42 +/- 5 mg/dl X h (mean +/- SEM) and 96 +/- 18 microU/ml X h, respectively, in normal subjects and 71 +/- 6 (P less than 0.05) and 206 +/- 30 (P less than 0.05), respectively, in the wheelchair-confined DMD patients. All of the ambulatory DMD males had normal oral glucose tolerance tests. Insulin binding to erythrocytes was 20-30% lower (P less than 0.01) in all DMD patients than in normal males appropriately matched for age and degree of sexual development. This difference in binding was a result of lower affinity of the insulin receptor in DMD erythrocytes. On the other hand, insulin binding to fibroblasts was the same in normal males and DMD patients, suggesting that the abnormality of erythrocyte binding in DMD is probably not genetically induced. Insulin binding to erythrocytes and monocytes was the same in all females studied, regardless of whether they were carriers of the DMD gene. Our results suggest that abnormal insulin binding in DMD erythrocytes is an acquired rather than genetic abnormality, but insulin binding is not helpful in the identification of carrier females. The defect in insulin binding in DMD is present before the development of insulin resistance, which occurs only in severely immobilized patients. Thus, the cause of the insulin resistance in DMD may reside at steps beyond the binding of insulin to its receptor.
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PMID:Dissociation of insulin resistance and decreased insulin receptor binding in Duchenne muscular dystrophy. 396 91

The metabolic response to human growth hormone (HGH) was studied in five obese subjects in the fed state and during prolonged (5-6 wk) starvation. In the fed state (three subjects), HGH induced an elevation in basal serum insulin concentration, a minimal increase in blood and urine ketone levels, and a marked reduction in urinary nitrogen and potassium excretion resulting in positive nitrogen and potassium balance. In prolonged fasting (four subjects), HGH administration resulted in a 2- to 3-fold increase in serum insulin which preceded a 50% elevation in blood glucose. Persistence of the lipolytic effects of HGH was indicated by a rise in free fatty acids and glycerol. The response differed markedly from the fed state in that blood beta-hydroxybutyrate and acetoacetate levels rose by 20-40%, resulting in total blood ketone acid concentrations of 10-12 mmoles/liter, ketonuria of 150-320 mmoles/day, and increased urinary potassium loss. The subjects complained of nausea, vomiting, weakness, and myalgias. Despite a 50% reduction in urea excretion during HGH administration, total nitrogen loss remained unchanged as urinary ammonia excretion rose by 50% and correlated directly with the degree of ketonuria. It is concluded that in prolonged starvation (a) HGH may have a direct insulinotropic effect on the beta cell independent of alterations in blood glucose concentration, (b) persistence of the lipolytic action of HGH results in severe exaggeration of starvation ketosis and interferes with its anticatabolic action by necessitating increased urinary ammonia loss, and (c) failure of HGH to reduce net protein catabolism in starvation suggests that this hormone does not have a prime regulatory role in conserving body protein stores during prolonged fasting.
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PMID:Metabolic response to human growth hormone during prolonged starvation. 554 Jan 76

Neurological involvement occurred in every one of a series of 30 patients with an insulinoma. The episodic nature of the hypoglycaemia caused symptoms and signs to fluctuate and often led to delay in diagnosis (mean length of history was 3 years). The commonest feature at first presentation was confusion (20 instances), but as the illness evolved, coma (16 instances) and convulsions (8 instances) became more frequent. Objective weakness was found in 7 patients, with 3 examples of hemiparesis and 2 each of paraparesis and monoparesis; in all, the weakness resolved over a period of 1 hr to 3 days when normoglycaemia was maintained. Other neurological features included subjective visual disturbances, headache, dysarthria and ataxia. 220 patients with an insulinoma from 7 series in the literature were reviewed. The high incidence of neurological features was confirmed, with confusion (152 cases), coma (82 cases) and convulsions (58 cases) predominating. Visual disturbances were common, though not accurately quantified in some series. Objective evidence of weakness on the other hand was reported in only 6 of the 222 patients. Other less common symptoms included headache (18 instances) and peripheral paraesthesiae (14 instances). In the 7 series reviewed, as in our own, it was found that in any one patient, each episode of hypoglycaemia was accompanied by the same symptom complex. The presence of an insulinoma should be considered in any patient with unusual, or inexplicable neurological features, particularly when they are intermittent. The diagnosis can be confirmed by demonstrating an inappropriately high circulating insulin level, for the ambient blood glucose concentration.
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PMID:Neurological aspects of insulinomas. 609 Oct 78

A ferret with clinical and laboratory signs of hypoglycemia was found at surgery to have a beta cell tumor of the pancreas. There had been recurrent episodes of weakness, ataxia, dehydration, and hypothermia. A fasting blood glucose content was 43 mg/dl and the amended insulin/glucose ratio was 362.5. The tumor was removed, yet hypoglycemia persisted postoperatively. Clinical signs related to hypoglycemia did not recur following application of medical treatment and frequent feedings. The histologic appearance of the tumor closely resembles that which has been seen in other species.
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PMID:Pancreatic beta cell tumor in a ferret. 609 38

The clinical estimation of potassium balance generally depends on the level of serum potassium. Since the extracellular fluid contains only 2 percent of the total body potassium, it must be recognized that potassium deficits are usually large before significant hypokalemia occurs, whereas smaller surfeits of potassium will cause hyperkalemia. The total body potassium is regulated by the kidney in which distal nephron secretion of potassium into the urine is enhanced by aldosterone, alkalosis, adaptation to a high potassium diet, and delivery of increased sodium and tubular fluid to the distal tubule. However, the distribution of potassium between the intracellular and extracellular fluids can markedly affect the serum potassium level without a change in total body potassium. Cellular uptake of potassium is regulated by insulin, acid-base status, aldosterone, and adrenergic activity. Hypokalemia, therefore, may be caused by redistribution of potassium into cells due to factors that increase cellular potassium uptake, in addition to total body depletion of potassium due to renal, gastrointestinal, or sweat losses. Similarly hyperkalemia may be caused by redistribution of potassium from the intracellular to the extracellular fluid due to factors that impair cellular uptake of potassium, in addition to retention of potassium due to decreased renal excretion. An understanding of the drugs that affect potassium homeostasis, either by altering the renal excretion of potassium or by modifying its distribution, is essential to the proper assessment of many clinical potassium abnormalities. Both hypokalemia and hyperkalemia may cause asymptomatic electrocardiographic changes, serious arrhythmias, muscle weakness, and death. Hypokalemia has also been associated with several other consequences, including postural hypotension, potentiation of digitalis toxicity, confusional states, glucose intolerance, polyuria, metabolic alkalosis, sodium retention, rhabdomyolysis, intestinal ileus, and decreased gastric motility and acid secretion.
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PMID:Potassium homeostasis and clinical implications. 638 26

A boy, now 22 months old, is described who presented at the age of 6 weeks with hypoglycaemic coma. The excretion pattern of organic acids in the urine was consistent with glutaric aciduria type II (GA II). A high energy diet low in fat and protein was given. Treatment with riboflavine resulted in an improvement of the metabolite profile, and the patient gained weight. However, a tendency to hypoglycaemia and severe hypotonia persisted. Due to muscle weakness, aggravated by infections, artificial ventilation was necessary during three periods. Serum carnitine level was low. Treatment with carnitine, started during the third period of artificial ventilation, led to some improvement of muscle strength, but he still could not breathe without support. Treatment with insulin, combined with further enrichment of the diet with glucose, resulted in an increase in muscular strength and in weight gain. Thirteen families with GA II have been described upto now. This is the first patient with a severe form of the disorder wo has survived the 1st year of life. Treatment and metabolic studies are presented.
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PMID:Glutaric aciduria type II: treatment with riboflavine, carnitine and insulin. 639 38

A case of hypokalemic periodic paralysis, is reported, characterized by a positive family history and by repeated attacks of transient motor weakness since the age of 14 involving one all limbs. An induction test under ECG and EMG monitoring, carried out by infusing glucose and insulin when the patient was symptom-free, provoked motor paresis comparable to the spontaneous attacks and confirmed the diagnosis.
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PMID:Familial hypokalemic paralysis: a case study. 657 50

In 1963, Klein et al. first described two girls with normokalemic periodic paralysis and cardiac arrhythmia. We have observed a 15-year-old girl with cardiac arrhythmia and normokalemia but with some features of hyperkalemic, periodic paralysis. The patient showed a waddling gait and Gower's sign. She had atrophy of the proximal muscles and deep tendon reflexes were reduced. Her ECG showed bigeminy with multifocal premature ventricular contractions. At the start of an episode of muscle weakness, the serum potassium concentration rose from 3.1 to 4.4 mEq/l. Muscle weakness was not provoked by intravenous administration of 45 g glucose or of 72 g glucose followed by 10 units of regular insulin. Muscle weakness was evoked by the ingestion of 4 g potassium chloride. During the provoked muscle weakness, the ECG showed normal sinus rhythm temporarily.
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PMID:Periodic paralysis with cardiac arrhythmia. 662 56

Fraternal twins who had fasting hypoglycemia, hypoketonemia, muscle weakness, and hepatic dysfunction are reported. The hepatic dysfunction occurred only during periods of caloric deprivation. The surviving patient developed a cardiomyopathy. In this sibling, muscle weakness and cardiomyopathy were markedly improved by a diet high in medium chain triglycerides. There was a marked deficiency of muscle total carnitine and a mild deficiency of hepatic total carnitine. Unlike patients with systemic carnitine deficiency, serum and muscle long-chain acylcarnitine were elevated and renal reabsorption of carnitine was normal. It was postulated that the defect in long-chain fatty acid oxidation in this disorder is caused by an abnormality in the mitochondrial acylcarnitine transport. Detailed studies of the cause of the hypoglycemia revealed that insulin, growth hormone, cortisol, and glucagon secretion were appropriate and that it is unlikely that there was a major deficiency of a glycolytic or gluconeogenic enzyme. Glucose production and alanine conversion to glucose were in the low normal range when compared to normal children in the postabsorptive state. The hypoglycemia in our patients was probably due to a modest increase in glucose consumption, secondary to the decreased oxidation of fatty acids and ketones, alternate fuels which spare glucose utilization, plus a modest decrease in hepatic glucose production secondary to decreased available hepatic energy substrates.
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PMID:Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet. 668 67


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