Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the case of a patient with Rett syndrome, a syndrome characterized by progressive infant encephalopathy, developmental delay, dementia, autism, ataxia, microcephaly, spastic paraparesis, and autonomic neuropathy with constipation. At colonoscopy, multiple foci of tiny white, sessile, polypoid lesions were seen throughout the colon and rectum, mimicking the appearances of small hyperplastic or adenomatous polyps, associated with generalized melanosis coli. This is the first case to our knowledge describing melanosis coli in a patient with Rett syndrome. As melanosis pigment deposition is characteristically not seen in lymphoid tissue, the lymphoid tissue was identifiable at endoscopy as multiple white nodules mimicking generalized colonic polyposis throughout the colon. We discuss the likely mechanisms of lymphoid hyperplasia and coexistent melanosis coli in Rett syndrome.
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PMID:Colonic lymphoid hyperplasia in melanosis coli. 1147 72

Lactulose has profound health benefits by way of increasing bifidobacterial flora in the intestine of infants thereby protecting them against enteric infection, constipation and systemic encephalopathy. In the present study to assess the sub chronic toxicity of lactulose syrup, the rats were fed on a basal feed supplemented with lactulose syrup at 0.5, 1.0, 2.0 and 5.0% for a period of 21 weeks. Monitoring of food consumption, gain in body weight and physical observations did not reveal any treatment-related toxicity in any of the group of rats. Terminal autopsy also did not reveal any signs of toxicity. Further, no significant alterations in relative organ weight, serum biochemistry and urinalysis were observed up to 1% lactulose supplementation level. The results suggest that supplementation of lactulose in the diet does not produce any toxicity at the doses tested.
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PMID:Sub chronic toxicity studies of lactulose in rats. 1151 Jan 27

The undigestable disaccharide lactulose has been in medical use for over 40 years, mainly in the treatment of portosystemic encephalopathy and of constipation. Pharmacodynamics of lactulose make it an efficacious and safe drug in these indications. But the reason for its numerous potential benefits are under research now. The major principle of action is the promotion of growth and activity of lactic acid bacteria in the gut which counteract detrimental species such as clostridia or salmonellae. This shows that prebiotic action, if used accordingly, can have medically significant effects. The mechanism of action, medical and prebiotic effects, veterinary uses, and technological properties of lactulose, e. g. in yoghurt production are reviewed.
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PMID:Medical, nutritional and technological properties of lactulose. An update. 1242 Jan 12

Mitochondrial neurogastrointestinal encephalomyopathy is a rare disorder affecting the pediatric age group with a heterogeneous multisystem involvement. We happen to manage a young child with symptoms of constipation since infancy along with cachexia, seizures and peripheral neuropathy. The child later went into encephalopathy preterminally. This clinical syndrome fitted very well with mitochondrial neurogastrointestinal encephalomyopathy. The child had elevated lactate levels and electron microscopy of the rectal biopsy was suggestive of a mitochondrial disorder To the best of our knowledge there is no case report of this syndrome from India and since this presents with diagnostic difficulties so is being reported.
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PMID:Mitochondrial neuro-gastrointestinal encephalopathy syndrome. 1720 42

A 62-year-old man had been treated with combined androgen blockade due to cT2bN1M0 prostate cancer, and his serum prostate-specific antigen (PSA) levels decreased and remained under the level of 0.5 ng/mL during therapy. Approximately 40 months after the initial therapy, difficulty on urination and constipation developed gradually, and serum carcinoembryonic antigen (CEA) and pro-gastrin-releasing peptide (ProGRP) levels were high at this point. He underwent transrectal and transurethral biopsy of the prostate, which revealed adenocarcinoma positive for CEA and chromogranin A. He received palliative pelvic irradiation, and oral estramustine phosphate and etoposide combined therapy. Tumor markers decreased and clinical symptoms improved for several months. The patient died of encephalopathy of unknown etiology approximately 11 months after the relapse.
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PMID:Relapsed prostate cancer with neuroendocrine differentiation and high serum levels of carcinoembryonic antigen without elevation of prostrate-specific antigen: a case report. 1730 72

(1) In dialysis patients with chronic renal failure, hyperphosphataemia can cause osteorenal dystrophy, leading to bone pain, fractures and excess cardiovascular mortality. In addition to a low-phosphorus diet and dialysis, phosphorus chelators are usually needed to control blood phosphorus levels. The first choice is calcium carbonate, and sevelamer is an alternative. (2) Lanthanum carbonate, a phosphorus chelator, is now also licensed for the treatment of hyperphosphataemia in dialysis patients with chronic renal failure. (3) In addition to three dose-finding placebo-controlled studies, clinical evaluation includes 2 comparative randomised unblinded trials: one 6-month trial versus calcium carbonate and a 2-year trial versus other phosphorus chelators. During these trials, lanthanum was no more effective than the comparators in terms of effects on the mortality rate, incidence of fractures, or blood phosphorus level. (4) During these trials, adverse events attributed to treatment were more frequent with lanthanum than with the other phosphorus chelators. The main problems were gastrointestinal disorders (nausea, vomiting, diarrhoea, constipation and abdominal pain), headaches, seizures, and encephalopathy. (5) The accumulation of lanthanum in the bones and brain is troubling. The known long-term adverse effects of aluminium, another trivalent cation with weak gastrointestinal absorption, suggest that caution is also required with lanthanum. (6) In practice, when a phosphorus chelator is needed to treat hyperphosphataemia in dialysis patients with chronic renal failure, calcium carbonate is the first choice and sevelamer remains the best alternative.
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PMID:Lanthanum: new drug. Hyperphosphataemia in dialysis patients: more potential problems than benefits. 1745 39

Mitochondrial encephalomyopathy, lactic acidosis, and stroke (MELAS) is a mitochondrial genetic disorder caused by a point mutation, resulting in the substitution of guanine for adenine at nucleotide 3243 (A3243G). It is a multisystem disorder with variable manifestations and typically presents between the first and third decades of life. It should be suspected if a patient exhibits stroke-like episodes before age 40, encephalopathy characterized by seizures, dementia, or both, and lactic acidosis, ragged-red fibers in muscle, or both. We present the case of a 26-year-old white man suspected with primary central nervous system vasculitis admitted to our facility with profound constipation from severe intestinal dysmotility. Although his gastrointestinal and neurologic symptoms did not meet criteria for a specific vasculitic syndrome, his symptoms and blood test abnormalities were concerning for such a process. MELAS was included in our differential diagnosis because his symptoms failed to fit a defined vasculitic process. When genetic testing documented the presence of the point mutation A3243G, his diagnosis was changed. This case illustrates the importance of considering a mitochondrial genetic disorder in the differential diagnosis of patients who present to Rheumatologists with suspected unusual or atypical vasculitic symptoms.
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PMID:MELAS masquerading as a systemic vasculitis. 1817 43

Occupational and environmental exposures to lead (Pb), one of the toxic metal pollutants, is of global concern. Health risks are increasingly associated with environmental exposures to Pb emissions from, for example, the widespread use of leaded gasoline in developing countries. Exposure occurs mainly through the respiratory and gastrointestinal systems, and the ingested and absorbed Pb is stored primarily in soft tissues and bone. Autopsy studies of Pb-exposed patients have shown a large amount (approximately 33%) of the absorbed Pb in soft tissue stored in liver. In addition to neuronal encephalopathy observed in persons after exposure to very high concentrations of Pb, gastrointestinal colic (abdominal pain, constipation, intestinal paralysis) is a consistent early symptom of Pb poisoning in humans. Such severe gastrointestinal effects are consistently observed in patients with a blood Pb range of 30 to 80 microg/dl. Ingestion of Pb is one of the primary causes of its hepatotoxic effects. Hepatocarcinogenic effects of Pb reported in animal toxicology studies have led to new research into the biochemical and molecular aspects of Pb toxicology. Gains in the molecular understanding of Pb effects on hepatic drug metabolizing enzymes, cholesterol metabolism, oxidative stress, and hepatic hyperplasia suggest a potential role for Pb in damaging extrahepatic systems, including the cardiovascular system. This review also discusses the therapeutic potential of chelation therapy in treating Pb-induced hepatotoxicity in animals.
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PMID:Lead hepatotoxicity & potential health effects. 1821 78

A 63-year-old man with liver cirrhosis and hepatocellular carcinoma presented with abrupt encephalopathy with markedly elevated blood ammonia levels. He was found lying down in front of the hospital 2 days after treatment of right hypochondrial pain with sustained-release oral morphine sulfate. He tended to be constipated before receiving morphine sulfate. The excess production of ammonia due to his constitutional constipation exacerbated by the use of oral morphine was thought to be the causal association with transient hepatic encephalopathy. He regained consciousness by receiving aminoleban intravenously and anticonstipating suppository. We realized that much care should be taken to avoid such opioid-related constipation at the time of pain management for quality of life improvement in patients with cancer pain.
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PMID:[A case of hepatic encephalopathy induced by adverse effect of morphine sulfate]. 1863 39

Acute intermittent porphyria (AIP) is an inherited metabolic disease that can affect the autonomic, peripheral and central nervous systems. Pancreatic diseases assocated with AIP is rarely reported. We report here a 60-year-old non-alcoholic male who had typical manifestations of AIP, including abdominal pain, constipation, tachycardia, hypertension, mental disturbances, psychiatric manifestations, seizures, peripheral neuropathy, and excessive excretion of porphyrin precursors in urine. Increases of serum amylase and lipase, as well as mild pancreatic edema on ultrasonography, were noted during the acute attack of AIP, suggesting concomitant acute pancreatitis. In this patient, brain magnetic resonance imaging revealed reversible multifocal cerebral lesions resembling a posterior reversible encephalopathy syndrome (PRES) during the acute attack of AIP. Because the clinical manifestations of acute pancreatitis could be present with an acute attack of AIP, early confirmation of diagnosis is mandatory to effectively manage the attack and avoid inappropriate treatment.
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PMID:Acute intermittent porphyria presenting as acute pancreatitis and posterior reversible encephalopathy syndrome. 1897 24


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