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)

The Bardet-Biedl syndrome is a rare autosomal recessive disorder characterized by pigmentary retinopathy, obesity, polydactyly, hypogonadism, and mental retardation. Renal abnormalities, hypertension, acquired heart disease, and hepatic fibrosis also occur in homozygotes. Two adult Bardet-Biedl sibs, a man with hypertension and cardiomegaly and a woman with biliary cirrhosis, and 75 relatives in 5 generations of the extended family were identified. Hospital records for major illnesses, death certificates, and autopsy reports were examined. The frequent observation of obesity, hypertension, diabetes mellitus, and renal disease in first-degree relatives, obligate gene carriers, and other blood relatives raise the possibility that Bardet-Biedl heterozygotes are also predisposed to these disorders.
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PMID:Obesity, hypertension, and renal disease in relatives of Bardet-Biedl syndrome sibs. 187 34

This article deals with the use of oral contraceptives and IUDs by chronically ill adolescent females. Results of controlled studies of contraceptive choices and problems are reviewed for teenagers with cardiac disease, epilepsy, multiple sclerosis, migraine headaches, asthma, cystic fibrosis, inflammatory bowel disease, hepatitis, diabetes mellitus, thyroid disease, oligomenorrhea and amenorrhea. If oral contraceptives (OC) are prescribed for use in teens with cardiac disease, a contraceptive with 35ug or less of estrogen and the equivalent of 1 mg or less of norethindrone should be used. The low-dose progestin only pill can be prescribed, but should be used in conjunction with a back-up barrier method. Reports to date have failed to reveal increased seizure activity in epileptic pattients on OCs, and there is no significant evidence to date that OCs alter the course of multiple sclerosis. Although the evidence is inconclusive, the physician should use extreme caution in prescribing OCs for teens with prior migraines. Regarding asthmatic patients, no problems have been reported with IUD use except in regard to steroid therapy and its possible effect on reducing IUD effectiveness. No adverse effects 2ndary to the use of OCs in asthmatic patients have been reported. OCs should be avoided or used with extreme caution in the cystic fibrosis patient. Teens with active inflammatory bowel disease should be advised that OCs may be ineffective or dangerous; there are no reports available on the effects of the IUD on the disease. The pill is contraindicated during active liver disease or cirrhosis. The IUD is not highly recommended for contraception in diabetic teenagers, whereas a low-dose combined OC can be used with extreme caution. However, OCs should be avoided in the diabetic patient with nephropathy, vascular complications or retinopathy. There is at present no contraindication for contraceptive use by women with thyroid disease. Finally, patients with prolonged post pill amenorrhea and infertility are generally females with amenorrhea or oligomenorrhea before pill use.
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PMID:Contraceptive use in the chronically ill adolescent female: Part I. 351 58

A clinical and electrophysiological study of neuropathy in 215 unselected Ethiopian diabetics and 100 healthy controls was carried out at the Tikur Ambassa Teaching Hospital, Addis Ababa. The prevalence in diabetics was 54%, in controls 8%. The prevalence was significantly related to the duration of diabetes: 42%, 60% and 80% for a duration of respectively less than 5 years, 5 to 14 years, and 15 years and longer. There was no relationship of the prevalence to age. Diabetics with retinopathy (15%), nephropathy (12%) and liver cirrhosis (13%) had prevalences of respectively 70%, 76% and 71%; much higher figures than in patients without these complications. A higher prevalence of neuropathy was detected by nerve conduction studies than by clinical methods. The mean conduction velocity diminished in direct relation to the duration of the diabetes. The prevalence of neuropathy in our patients is similar to the prevalence reported from other countries in Africa. Poor nutritional background, inadequate control of the diabetes and the high prevalence of associated diseases such as liver cirrhosis may be partly responsible for the high prevalences of neuropathy in African diabetics.
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PMID:Neuropathy in Ethiopian diabetics: a correlation of clinical and nerve conduction studies. 401 53

In spite of the high prevalence of diabetes mellitus (DM) in patients with liver cirrhosis (LC) few studies have focused on the clinical implications of this association. We investigated the clinical and pancreatic-endocrine features of 34 patients who developed DM after LC (Group I). Results were compared with 34 carefully matched patients with only Type II DM (Group II). A standard meal test was performed in 26 patients with normal renal function from each group to assess beta-cell function. Group I patients, less frequently had retinopathy (14.7% vs. 45.5%, P < 0.05) and a family history of diabetes (23.5% vs. 58.8%, P < 0.01). Group I patients also showed signs of enhanced insulin resistance, reflected by higher insulin dose requirements in insulin-treated patients (0.87 +/- 0.10 vs. 0.62 +/- 0.05 IU/kg/day, P < 0.01) and increased basal C-peptide values (0.88 +/- 0.06 vs. 0.68 +/- 0.07 pmol/l, P < 0.05, respectively) than those in Group II. These results suggest that several clinical features, probably related to the hepatopathy, define DM occurring in patients with LC.
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PMID:Diabetes mellitus in patients with liver cirrhosis. 783 8

Gastroparesis, constipation, diarrhea, and fecal incontinence occur frequently in diabetics with long-standing and often poorly controlled insulin-dependent diabetes. These motor abnormalities of the gastrointestinal tract tend to be associated in these patients with evidence of autonomic neuropathy and other diabetes-related complications such as peripheral neuropathy, nephropathy, and retinopathy. The management of these derangements of motility is generally frustrating and very difficult. The prokinetic agents currently available have fewer side effects than previously used drugs, and have expanded the treatment options for diabetics with motility disorders of the gastrointestinal tract. The treatment of diabetic diarrhea remains aimed at the symptom because the cause is often unknown. The diagnosis of diabetic diarrhea depends on a careful and judicious assessment, which allows for the distinction of this condition from other causes of diarrhea. For example, celiac disease can occur in insulin-dependent diabetics, but it is specifically treated by the elimination of gluten from the diet. In recent years, we have also gained a better understanding of the liver and biliary tree abnormalities that occur in the diabetic. The most common hepatobiliary lesions found in these patients include excessive glycogen deposition, fatty liver, and gallstones. Cirrhosis of the liver can develop in diabetics as a result of progressive fatty steatosis, pericentral hepatic fibrosis, and, at times, central hyaline sclerosis. Future study of the underlying pathogenesis of diabetes may one day allow us to find common threads in the seemingly disparate gastrointestinal and hepatic complications of this disease.
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PMID:The intestinal and liver complications of diabetes mellitus. 843 40

Therapy with disease modifying antirheumatic agents (DMARDs) is often complicated by the occurrence of adverse effects. Although risk factors for several DMARDs have been reported, the prediction of adverse drug reactions is not yet possible. Therefore regular monitoring remains mandatory. Monitoring for adverse effects to DMARDs usually includes one or more of the following: blood count, liver, kidney, urine or ophthalmologic tests. Since most adverse reactions occur during the first few months of treatment, monitoring should be more intense and frequent in this initial phase. Some adverse effects are dose-dependent, and therefore dosage reduction may help alleviate these. Others are idiosyncratic, and often necessitate drug withdrawal. Except for (hydroxy)chloroquine-induced retinopathy and methotrexate-induced liver cirrhosis, most adverse reactions to DMARDs are fortunately reversible.
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PMID:Management of adverse effects of disease-modifying antirheumatic drugs. 857 95

The morbidity rate for retinopathy of unknown origin was significantly higher (P < 0.001) in the chronic hepatitis C group, 27 (31.8%) of 85 cases, than in the control group, 6 (6.0%) of 100 cases. Among hepatitis C virus (HCV)-associated retinopathy patients, 6 cases (22.2%) were aware of ocular subjective symptoms, retinopathy recurred in 8 (29.6%) cases, binocular retinopathy occurred in 14 (51.9%) cases, and retinopathy related to liver dysfunction, in 13 (81.3%) of 16 tested cases. HCV-associated retinopathy involved hemorrhage at the posterior pole retina in 21 (77.8%), cotton-wool patches in 9 (33.3%), and hemorrhage at the peripheral retina in 7 (25.9%) cases. Sequelae occurred in only one case. Retinopathy worsened or recurred in all 7 cases treated with interferon. The risk factors for HCV-associated retinopathy were mild thrombocytopenia (P < 0.001), long-term hepatitis illness (P < 0.005), advanced age (P < 0.02), concurrence with liver cirrhosis (P < 0.02), history of systemic hypertension (P < 0.05), and female gender (P < 0.05).
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PMID:Clinical characteristics of hepatitis C virus-associated retinopathy. 892 49

Because hepatocellular carcinoma often recurs after surgical resection or ethanol injection therapy, we conducted a prospective randomized controlled trial of interferon (IFN) in patients with chronic liver disease caused by hepatitis C virus (HCV). Twenty eligible patients with cirrhosis were randomized into two groups: 10 patients treated with 6 million units of natural IFN-beta twice a week for 36 months and 10 patients without IFN therapy. One patient within the treatment group discontinued interferon therapy after 19 months of treatment because of a mild degree of retinopathy. None of the patients in either group lost HCV-RNA until the end of the observation. Although 7 (70.0%) of 10 patients in the nontreatment group showed tumor recurrence, only 1 (10.0%) of 10 patients with IFN therapy developed tumor recurrence during a median observation period of 25.0 months. Cumulative recurrence rates of the treated and untreated groups were 0% and 62.5% at the end of the first year, and 0% and 100% at the second year, respectively (log-rank test, P =.0004). In conclusion, intermittent administration of IFN suppressed tumor recurrence after treatment with surgery or ethanol injection in patients with HCV-related chronic liver disease.
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PMID:Interferon beta prevents recurrence of hepatocellular carcinoma after complete resection or ablation of the primary tumor-A prospective randomized study of hepatitis C virus-related liver cancer. 1117 57

Among 457 elderly patients of 65 years or older with chronic hepatitis or cirrhosis caused by hepatitis C virus, 117 patients underwent interferon therapy for the elimination of hepatitis C virus. A total of 87 patients could be analyzed for the interferon effect, since the remaining 20 patients had still been receiving or just finished the therapy. Thirty-six patients(41.4%) achieved complete elimination of HCV-RNA with interferon therapy. Although those patients with a milder hepatitis stage and better virological condition(low viral concentration or group 2 subtype) were preferentially enrolled in the therapy, 13 patients(11.1%) discontinued the administration with varied side effects: severe general malaise in 6 patients, depression in 3, pneumonia/pneumonitis in 2, and retinopathy in 2. Crude hepatocellular carcinogenesis rates in the subgroup of F1 + F2 and the subgroup of F3 + F4 were 1.8%, 21.2% at the end of 5th year, and 14.3% and 53.7% at the tenth year, respectively.
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PMID:[Hepatocellular carcinogenesis and prognosis of elderly patients with chronic hepatitis type C]. 1149 48

We report the case of a girl with Hardikar syndrome who underwent living-donor liver transplantation at 2 years of age. This disease, described in 1992, includes a constellation of abnormalities, such as cleft lip and palate, pigmentary retinopathy, and multiple tubular stenoses (e.g., bile ducts, ureters). Other system involvement is variable. Rotation anomalies of the gut and cardiac abnormalities are frequently present. Pathogenesis remains obscure. Our patient was delivered at 33 weeks of gestation by cesarean section, and was jaundiced, with low birth weight and height. On day 5 after birth, the patient underwent Ladd's surgery for intestinal malrotation. One month later, she developed pyelonephritis and urosepsis. She remained jaundiced and a liver biopsy revealed cirrhosis with regenerating nodules, portal chronic inflammation with bile duct proliferation, and lobular cholestasis. The patient underwent several corrective operations, and at 12 months of age she was diagnosed with Hardikar syndrome. She failed to thrive and had progressive cholestasis and jaundice, coagulation disorders, bilateral ureterostomies, repetitive urinary tract infections, bilateral cleft lip and palate, retinopathy, and gut malrotation. She received a liver transplant at 24 months of age from a living donor. She has had an excellent clinical outcome in liver function without further decline of growth and development.
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PMID:Hardikar syndrome: a case requiring liver transplantation. 1239 56


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