Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatolenticular degeneration (Wilson's disease) is a hereditary disease in which metabolic disorder of copper leads to its accumulation in the liver, brain, cornea and kidneys with consequent pathologic changes in those organs. Hereditary mechanism of the disease is autosomal recessive with prevalence of 30-100 per 1,000,000 inhabitants. Etiology of this disease is not yet explained. There are two hypotheses. The first one is that it is the disorder of ceruloplasmine metabolism caused by insufficient synthesis of normal ceruloplasmine, or synthesis of functionally abnormal ceruloplasmine. The second one is: the block of copper biliar excretion which is the consequence of the liver lysosomes functional defect. Pathogenetic mechanism of disease is firstly long-term accumulation of copper in the liver, and later, when the liver depo is full, its releasing in circulation and accumulation in the brain, cornea, kidneys and bones, which causes adequate pathologic changes. Toxic activity of copper is the consequence of its activity on enzymes, particularly on those with -SH group. There are two basic clinical forms of the disease: liver disease or neurologic disease. Before puberty the liver damage is more frequent, while in adolescents and young adults neurologic form of the disease is usual. The liver disease is nonspecific and characterized by symptoms of cirrhosis and chronic aggressive hepatitis. The only specificity is hemolytic anemia which, in combination with previous symptoms, is important for diagnosis of the disease. Neurologic symptoms are the most frequent consequence of pathologic changes in the basal ganglia. In our patients the most frequent symptoms were tremor (63%); dysarthria, choreoathetosis and rigor (38%); ataxia and mental disorders (31%); dysphagia and dystonia (12%), diplopia, hypersalivation, nystagmus and Babinski's sign (6%). Among pathologic changes in other tissues and organs the most important is the finding of Kayser-Fleischer ring in the cornea as a result of copper accumulation. Its importance for precise diagnosis is great. The diagnosis of the disease is based on anamnesis, clinical examination, specific and nonspecific laboratory tests. The therapy of choice is penicillamine. If we use it early, the result will be good remission in the majority of patients. Late diagnosis or delay in treatment cause death which is the result of bleeding from esophageal varices or basal ganglia disease. Immunologic damages caused by penicillamine demand interruption of therapy and substitution by three-ethyl-tetra-amine (TETA). We also use zinc salts and tetratiomolibdate in therapy of this disease. Pathogenesis, clinical picture and therapy of the disease are based on our own results.
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PMID:[Hepatolenticular degeneration]. 226 49

1. An endoscopic survey was done in Linxian, Henan Province, Northern China on 527 people in May 1980 and histological evidence of oesophageal lesions was obtained. 2. Blood samples were collected from 58 men and 53 women aged 22-71 yr. Measurements of riboflavin, vitamin A and zinc status were made. 3. Most (97 per cent) had biochemical ariboflavinosis on the basis of erythrocyte glutathione reductase activation coefficients equal to or greater than 1.30. Five per cent of subjects had deficient vitamin A status (plasma retinol less than 10 micrograms/dl and one of these subjects reported night blindness) and 37 per cent had low vitamin A status (10-20 micrograms/dl). Approximately 24 per cent of the Chinese had low plasma zinc by comparison with values for subjects in Denver. Plasma retinol binding protein concentration was also low but was only measured in 56 subjects. 4. Concentrations of plasma carotene were relatively high and only one person had a value less than 20 micrograms/dl. The value of plasma carotene as a measure of vitamin A status in this community is discussed. 5. There was no difference between the sexes for any of the measurements made. In addition there was no difference in the biochemical measurements between those with and without oesophageal lesions (oesophagitis 66 per cent, clear cell acanthosis (CCA) 88 per cent) or those with intermittent dysphagia (26 per cent). 6. Multiple linear regression showed no relationship between the biochemical data and either oesophagitis or CCA. There were, however, inverse relationships between oesophagitis and CCA in both sexes and in addition, plasma carotene and riboflavin status tended to fall while plasma retinol tended to increase with age. 7. The role which these nutritional deficiencies may play in facilitating neoplastic processes is discussed.
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PMID:Riboflavin, vitamin A and zinc status in Chinese subjects in a high-risk area for oesophageal cancer in China. 717 57

Twelve patients (10.4%) had a history of partial gastrectomy among the 115 patients with squamous cell carcinoma of the esophagus. The clinical characteristics, nutritional parameters, and incidences of esophagitis of the resected specimens were investigated between 11 patients with esophageal carcinoma who had partial gastrectomy for peptic ulcer diseases (Group A) and 103 patients with esophageal carcinoma without any previous history of gastrectomy (Group B). Age, sex, tumor location, clinical stages, and cigarette and alcohol consumption were not different between the two groups. Hemoglobin, mean corpuscular volume, and mean corpuscular hemoglobin level in Group A were lower than those in Group B (p < 0.05). Serum zinc level in Group A was lower than that in Group B, but this difference was not significant. There was no difference in the incidences of esophagitis between the two groups. In conclusion, there is an association between partial gastrectomy and later development of squamous esophageal carcinoma. The role of malnutritional factors and gastroesophageal reflux, however, remain unclear. The long-term follow-up of patients after partial gastrectomy is warranted.
Dysphagia 1995
PMID:Squamous cell carcinoma of the esophagus after partial gastrectomy. 785 34

Establishing reliable nutrient requirements for individuals over the age of 65 years is a difficult task. Research on nutrient requirements in the aged is sparse and often contradictory. However, there are important clues in the literature suggesting that requirements for certain nutrients are altered with age and that preventable nutrient deficiencies exist. Energy expenditure and caloric intake typically decline with age. Unless nutrient density of the diet improves, a parallel decline in vitamin and mineral intake is inevitable. Deficiencies or suboptimal intakes of water-soluble vitamins, vitamin D, calcium, zinc, copper, chromium, and water are reported in groups of older adults. Marginal nutrient deficiencies in this population may easily go undetected and contribute to morbidity.
Dysphagia 1993
PMID:Nutrition and nutritional requirements for the older adult. 843 22

An 86-year-old man presented ambulatory with acute dysphagia. Radiologic examination and endoscopy revealed a swallowed postauricular hearing aid. The earmold of the hearing aid became visible in the hypopharynx after mucus and saliva were removed. It could be extracted without effort once the connecting tube was disconnected from the coupling device lodged in the upper esophageal sphincter. The hearing aid itself was impacted in the proximal esophagus and was extracted without any problems. The postoperative phase was uneventful with normal swallowing and discharge. Technical inspection revealed that the hearing aid no longer worked. Diffusion of toxic substances (zinc, mercury) from the impacted batteries is not to be expected.
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PMID:[Case report: swallowed hearing aid]. 856 24

Liver transplantation (LTX) is an approved method to treat patients with end-stage liver cirrhosis and acute liver failure due to Wilson's disease. Initially, there was some consideration about the indication for LTX in the case of Wilson's disease with severe neurological impairment but normal liver function. From 1988 until 1995, 13 out of 700 LTX (1.9%) were performed for Wilson's disease. Indications for LTX were (I) intractable neurological impairment with normal liver function (n = 4; including one patient with Child A cirrhosis), (II) fulminant hepatic failure (n = 3), and (III) end-stage liver cirrhosis (n = 6) (Child B, n = 1; Child C, n = 5). There were 8 females and 5 males with a mean age of 27 yr (range 15-34 yr). All patients of group I required continuous nursing care before LTX, in spite of pretreatment with d-penicillamine and zinc. The most frequent symptoms in these patients were dysphagia (n = 4), dysarthria (n = 4), tremor (n = 4), sialorrhea (n = 3), ataxia (n = 3), dystonia (n = 3) and handwriting difficulties (n = 3). All patients of group II presented with hemolytic anemia. The survival rate was 100%, and all patients were doing well after a mean follow-up period of 32.8 months (range 8-68 months). The postoperative course was without severe infectious and other complications. All patients of group I revealed the first signs of improvement for all types of neurological symptoms 4-6 wk after LTX. One patient has been without any symptoms from 18 months until 5.5 yr after LTX. Two patients with short-term follow-up also had noticeable improvement of neurological impairment, but residual symptoms are still present. One patient showed only slight improvement. We conclude that Wilson's disease may be a good indication for LTX for both neurological manifestation with stable liver function and hepatic manifestation with cirrhosis or acute liver failure.
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PMID:Liver transplantation: treatment of choice for hepatic and neurological manifestation of Wilson's disease. 919 46

To evaluate the current nutrition therapy for the patients of amyotrophic lateral sclerosis (ALS) in Japan, we sent questionnaires to 53 Japanese national sanatoriums (hospitals) and were able to collect replies from 42 institutes. In these replies, information on 466 patients (262 inpatients and 204 outpatients) was reported, accounting for about 10% of all Japanese ALS patients. Patients who ate orally were more frequent (p < 0.001, chi 2 test) in the outpatients (76.0%) than in the inpatients (39.7%). When the subjects were limited to non-orally nourished cases, gastrostomy was more frequently performed (p < 0.05, chi 2 test) in the outpatients (49%) than in the inpatients (29.1%), but no significant differences were observed between the above two groups as to the quantity of daily caloric intake; the mode was 1000-1200 Cal. About a quarter of non-orally nourished patients received supplemental sodium chloride, and fewer were supplemented copper and/or zinc. The consent of the patients as well as the clinical findings weighed heavily in the determination of the feeding route. Most physicians were disinclined to encourage tube feeding in the early stage of dysphagia, but were supportive of the operation of percutaneous endoscopic gastrostomy when non-oral nutrition therapy became necessary. Although the efficacy of vitamins for ALS has not been proved, 45 of 58 physicians agreed to subscribe vitamins to their patients, especially vitamin B12, E and C.
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PMID:[A survey of current nutrition therapy for the ALS patients in Japanese national sanatoriums]. 1133 87

Gastric cancer continues to be the second cause of cancer-related mortality in the world. Surgery is the only potentially curative therapy, although the adverse effects of surgery are considerable and include digestive symptoms, loss of appetite and malnutrition. Our study included 45 patients subjected to gastrectomy who were under treatment at our unit during 2000. The data given here refer to their first visit following surgery. The most frequent complications were diarrhoea (31%), pain (29%) and early dumping (24%). Other complications found were late dumping, nausea/vomiting and dysphagia. Anorexia appeared in 49% and 29% presented a negative attitude towards food. These complications give rise to insufficient food intake, leading to malnutrition, mainly marasmic in nature. Only 7% of the patients were normonourished, with 86% presenting slight or moderate malnutrition and 7% severe malnutrition. The mean Body Mass Index (BMI) of these patients was 20 +/- 3 kg/m2. The most frequent analytical alterations were anaemia with ferropenia and b12 deficit, and a reduction in the levels of zinc and retinol transporting protein. Many patients had impaired quality of life; 43% did not leave home and only 13% were able to work. Three groups were established depending on the time that had passed since the gastrectomy was performed before the first nutritional assessment (less than 3 months, from three months to a year, and over one year), without significant differences being found in any of the parameters studied. In this article we include recommendations for the nutritional handling and treatment of patients following gastrectomy.
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PMID:[Nutritional evaluation in patients with total gastrectomy]. 1242 99

Sixty patients (age 73 +/- 14 years; 22 women, 38 men) with dysphagia (67% neurological diseases, 33% tumors) were followed up for 1 year after placement of a percutaneous endoscopic gastrostomy (PEG). Before PEG placement and at six appointments thereafter, the patients' nutritional status was measured using bioelectric impedance analysis (BIA) and hematological nutritional parameters. A validated questionnaire was used to assess quality of life (the Gastrointestinal Quality of Life Index, GIQLI). The overall mortality rate was 65%, and mortality during the hospitalization period was 22%. Depending on the duration of the dysphagia, marked nutritional deficits were observed at the start of the study (deficiencies in albumin in 49% of the patients, calcium in 15%, magnesium in 18%, retinol in 78%, alpha-tocopherol in 16%, folic acid in 16%, vitamin B12 in 8%, vitamin D in 40%, and zinc in 46%). With the exception of vitamin E, all parameters returned to normal during the follow-up period. At the start of the study, BIA indicated nutritional deficiency in 90% of the patients, with no overall improvement being observed during the follow-up period. The GIQLI scores, on average, reached a figure of 61% of an unrestricted quality of life. In conclusion, long-term nutrition via the PEG tube maintained the patients' quality of life. For BIA most patients were malnourished during the follow-up period, but nevertheless PEG feeding was enough to compensate for gross nutritional deficiencies. Not infrequently, the indication for PEG placement is established too late.
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PMID:Nutritional status and quality of life in patients with percutaneous endoscopic gastrostomy (PEG) in practice: prospective one-year follow-up. 1462 55

Traumatic brain injury (TBI) is a common injury among children. Most TBIs are mild and do not require hospitalization. However, whether or not the patients require acute hospitalization, TBIs may have long-lasting consequences. There is little research on the nutrient needs of these patients, and recommendations are frequently based on data from adults with TBI. It is clear that calorie and protein needs are elevated with acute TBI. However, calorie needs are also decreased by therapies such as sedation, chemical paralysis, and barbiturate coma. Long-term calorie needs may be lower for "comatose" patients. Enteral feeding is preferred and possible for patients with TBI, though gastric feeding may be problematic in some patients. In the acute phase, patients with TBI can also have dysregulation of fluid and electrolyte balance, which may require alterations in nutrition care. Dysphagia is common after moderately severe TBI and requires a multidisciplinary approach for treatment. Future opportunities for research on pediatric TBI are numerous and may include ongoing clarification of macronutrient needs, as well as investigation into the roles of specific nutrients such as zinc, antioxidants, and anti-inflammatory compounds.
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PMID:Traumatic brain injury in the pediatric population. 1699 44


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