Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred twenty-five infants underwent surgical intervention for necrotizing enterocolitis between 1972 and 1984. Sixty-three infants, who survived more than 30 days postoperatively, were evaluated for long-term complications. There were 28 girls and 35 boys (mean birth weight 1,725 +/- 890 g; gestational age 32 +/- 4 weeks). Associated problems included hyaline membrane disease (43), cardiac anomalies (25), and trisomy 21(2). Thirty-six survivors required long-term ventilatory support. Fifty-nine infants underwent bowel resection and enterostomy, 3 decompressing enterostomies without resection, and 1, exploratory laparotomy only. Enterostomies were closed at four months. Twenty four had short bowel syndrome. Fifteen infants subsequently died for a late mortality rate of 23%. Mortality was related to sepsis (3), respiratory failure (5), cardiac anomalies (3), cardio-respiratory arrest (2), and TPN related liver failure (2), and was common with gestational age less than 31 weeks and birth weight less than 1,000 g. Medical problems included cholestasis (17), TPN induced cirrhosis (3), meningitis (3), seizures (8), and nutritional rickets (6). Significant developmental and intellectual delays were observed.
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PMID:Long-term follow-up after surgical management of necrotizing enterocolitis: sixty-three cases. 372 6

We carried out a study on 63 patients suffering from alcoholism in order to determine the frequency of 27 HLA antigens. In comparison to healthy blood donors no significant deviation of HLA distributions in alcoholics was found. The data on alcoholic patients with physical consequences such as cerebral seizures, liver cirrhosis and polyneuropathy failed to identify an association with HLA.
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PMID:Genetic markers in alcoholism: no association with HLA. 657 88

Hepatic function tests were performed on 48 dogs that had been given primidone, phenytoin, or a combination of anticonvulsant drugs for 6 months or longer. Except for histories of seizures, 44 of the dogs were healthy at the time the tests were performed. Abnormal test results were observed most frequently in dogs given only primidone and in dogs given combinations of anticonvulsant drugs. The test results that were abnormal most often were those for alanine transaminase and alkaline phosphatase activities, and sulfobromophthalein excretion. The dosage of anticonvulsant drug was found to modify certain test results. Statistically significant positive correlations were found between the dosage of primidone and serum alanine transaminase activity and between the dosage of phenytoin and serum alkaline phosphatase activity. Four of the dogs were examined because of signs of weakness and anorexia and 2 also had ascites. Three of the 4 dogs were euthanatized 2 to 49 days after admission with clinical signs compatible with hepatic failure, and cirrhosis of the liver was confirmed at necropsy. The fourth dog died at home and was not necropsied. Four of the remaining 44 dogs that apparently were healthy at the time of examination had abnormalities in hepatic biochemical test results that were comparable with those in the 4 dogs with clinical illness. We concluded that, although results of hepatic biochemical tests frequently may be abnormal in dogs given anticonvulsant drugs long-term, severe hepatic injury is observed less often.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Compromised hepatic function in dogs treated with anticonvulsant drugs. 669 76

The physiology of the release of antidiuretic hormone (ADH) from the posterior pituitary is briefly reviewed. The importance of both osmolar and non-osmolar stimuli is emphasised. Osmolar and non-osmolar factors usually reinforce each other; for example, hydropenia leads to hyperosmolality and hypovolaemia, both promoting ADH release, while hydration has the opposite effect. In disease, osmolar and non-osmolar factors may become dissociated leading to baroreceptor-mediated ADH release in the presence of hyponatraemia and hypo-osmolality. Examples include heart failure, glucocorticoid or thyroxine deficiency, hepatic cirrhosis and nephrotic syndrome with or without the superimposed effect of diuretics, i.e. conditions in which circulatory, and in particular effective arterial, volume is reduced. It is dangerous to label such conditions as 'inappropriate' secretion of ADH since the maintenance of circulating volume is at least as important a physiological requirement as the defence of tonicity. The syndrome of inappropriate secretion of ADH (SIADH) is uncommon in childhood and should only be diagnosed when physiological release of ADH in response to non-osmolar as well as osmolar factors has been excluded. Criteria for the correct identification of SIADH are discussed; the presence of continuing urinary sodium excretion in the presence of hyponatraemia and hypo-osmolality is essential to the diagnosis. SIADH in children is usually due to intracranial disease or injury. The mainstay of treatment is water restriction which reverses all the physiological abnormalities of the condition. Hypertonic saline is rarely indicated for the short-term control of neurological manifestations such as seizures. Drugs have little or no place in the treatment of SIADH in children.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The syndrome of inappropriate secretion of antidiuretic hormone. 861 39

delta-Aminolevulinic acid dehydratase (ALAD) is the second enzyme in the heme biosynthetic pathway and catalyzes two molecules of delta-aminolevulinate (ALA), which is a potent agonist for GABA autoreceptors. ALAD has two common alleles and thus consists of three distinct isozymes, designated 1-1, 1-2, and 2-2. It has been shown recently that ALAD1 allele is associated with alcoholic liver injury. This association was ascribed to possible differences among isozymes in sensitivity to oxidized glutathione (GSSG), and this sensitivity is increased in erythrocytes of alcoholic patients. In the present study we measured erythrocyte ALAD activity from subjects with different ALAD genotype and found ALAD-1 is most sensitive to GSSG. We then investigated allele frequencies of ALAD in alcoholics (n = 126) and healthy controls (n = 115). For the control group, the frequencies were 0.94 (ALAD1) and 0.06 (ALAD2) and for the overall alcoholic group, 0.91 (ALAD1) and 0.09 (ALAD2). There were no significant differences in allele frequencies at the ALAD locus between the two groups. Subtyping the alcoholics according to the presence or absence of delirium tremens, hallucinosis, withdrawal seizure or liver cirrhosis failed to show statistically significant differences in the allele frequencies. We conclude that our data do not support the evidence of an allelic association between the ALAD1 and alcoholism.
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PMID:[Lack of association between alcoholism and alleles in the delta-aminolevulinic acid dehydratase (ALAD) gene]. 808 Apr

The authors present and discuss the care of a nine-month-old with neonatal adrenoleukodystrophy who required general anaesthesia for gastrointestinal endoscopy. Neonatal adrenoleukodystrophy is an inherited disorder of peroxisomal enzymes. Anaesthetic care may be affected by the presence of hypotonia, liver function abnormalities, gastroesophageal reflux, and impaired adrenocortical function. Preoperative sedation is contraindicated because of the risk of precipitating airway obstruction due to pre-existing hypotonia. Anaesthetic induction and tracheal intubation should be performed to minimize the risk for aspiration of gastric contents. The choice of muscle relaxant should take into account the pre-existing hypotonia as well as the possibility of hyperkalaemia in response to succinylcholine. Anaesthetic agents known to decrease the seizure threshold should be avoided in patients with a seizure disorder. In addition, anaesthetic agents that rely on the liver for metabolism should be used with caution in patients with cirrhosis. When time permits, these patients should be screened for adrenocortical insufficiency before surgery, and perioperative steroid coverage is advisable when preoperative testing of adrenocortical function is not feasible. While these patients eventually die after progressive deterioration, full recovery from the effects of anaesthesia and surgery can be achieved with attention to neurological, metabolic, and physical problems.
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PMID:Anaesthesia for the patient with neonatal adrenoleukodystrophy. 811 45

In this paper, three cases of grand mal seizures are reported as a complication of pefloxacin at usual doses (400 mg twice a day) in patients with cirrhosis. Grand mal seizures occurred from 12 h to 8 days after the onset of pefloxacin treatment. In 1 case, seizures recurred after inadvertent rechallenge with the drug. Elevated pefloxacin serum levels were demonstrated in 2 cases. Brain computed tomography in all 3 cases and cerebrospinal fluid examination showed normal results. No etiology other than pefloxacin overdose was found. After pefloxacin withdrawal, no recurrence of seizures were observed. Therefore, when pefloxacin treatment is indicated for cirrhotic patients, a reduced dosage and/or careful monitoring of pefloxacin serum levels should be recommended.
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PMID:Grand mal seizures as a complication of treatment with pefloxacin in patients with cirrhosis. A report of three cases. 815 Oct 98

To determine the prevalence and spectrum of alcohol-related diseases in a general hospital inpatient population, data of 1288 patients newly admitted to a city general hospital, who had been examined with regards to alcoholism, were surveyed. The sample consisted of 625 medical and 663 surgical patients aged 18-64 years. In 21% (29.3% of the men and 9.4% of the women), inpatient treatment was due to an alcohol-related disorder. The highest occurrence was found in the 35-55 year-olds. Frequently diagnosed disorders in alcohol-dependent patients included delirium tremens (12.8%), seizures (11.4%), head injuries (9.4%) and cirrhosis of the liver (8.1%), whereas alcohol abusers had often been injured. Excluding patients with alcohol-related diseases decreases the proportion of men in the sample by 6.2%. The prevalence of physically-damaged alcoholic patients in general hospitals suggests that preventative measures, such as consultation services, could be applied efficiently in this setting.
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PMID:Alcohol-related diseases in general hospital patients. 910 12

Alpers disease is a neurodegenerative disorder of childhood characterized by early developmental delay, intractable seizures, and death in childhood. Neuropathologic changes are most severe in the gray matter and consist of diffuse neuronal loss, spongiform changes, and astrocytosis. We report 2 siblings with Alpers disease who were discordant for exposure to valproate (VPA). Both had developmental delay, and a progressive seizure disorder beginning at 5 years of age. The proband died at age 8 years of complications of ongoing seizures, including epilepsia partialis continua, with only minimal liver abnormalities. Her younger brother was treated with VPA for new-onset seizures and developed fulminant liver failure 6 months later, which led to his death at 5 years of age. Neuropathologic abnormalities of both siblings were consistent with Alpers disease. These observations support classification of Alpers disease and Alpers disease with liver cirrhosis as a single disease. They also confirm previous reports indicating that VPA may accelerate fulminant liver failure in Alpers disease. We recommend that a diagnosis of Alpers disease be considered in children with unexplained early developmental delay, cerebellar signs, or partial seizures, especially epilepsia partialis continua. When Alpers disease is strongly suspected, use of VPA should be avoided.
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PMID:Valproate-induced liver failure in one of two siblings with Alpers disease. 925 71

Information on GH in relation to epilepsy is sparse, and to our knowledge there is no information on GH levels during status epilepticus in man. We studied GH in serum in six patients during status epilepticus, and in a control group of six seizure-free patients with epilepsy, before and after injection of TRH. The baseline GH values before TRH administration were within the normal range in all patients. After injection of TRH all patients with status epilepticus showed a paradoxical peak-shaped increase of GH to at least twice their baseline levels within 45 min after the injection (median basal GH value 1.5 mU/l and median peak GH value 6. 5 mU/l, mean increase 330%). No uniform reaction to TRH was observed in the control group (median basal GH value 2.7 U/l and median of the highest value within 45 min 5.2mU/l). A paradoxical peak reaction of GH to TRH was significantly more frequent in the status epilepticus group compared with the control group (P=0.008, Fisher exact probability test). TRH is not considered a GH-releasing hormone in humans during normal conditions, but a paradoxical response of GH to TRH, similar to that observed during status epilepticus, has been reported in various other pathological conditions, such as acromegaly, liver cirrhosis, mental depression and hypothyroidism. Our results of GH release after TRH administration in patients with status epilepticus suggest an altered regulation of GH as a result of the long-standing epileptic activity.
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PMID:Paradoxical GH response to TRH during status epilepticus in man. 1009 49


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