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

Primary sclerosing cholangitis (PSC) leads to a progressive destruction of the intra- and extrahepatic bile ducts. The cause is unknown but genetic and immunological mechanisms may play a role. The median survival time from diagnosis to death is about 12 years. MRCP is almost equal to ERCP for diagnosing PSC and shows the typical localised or multifocal strictures and interfering segments of ectatic bile ducts. Liver histology can be helpful in making the diagnosis but is often unspecific and there is a large sampling variability. The treatment of PSC is disappointing. The combination of ursodeoxycholic acid with endoscopic dilatation is probably the best treatment. Patients with cirrhosis and/or recurrent cholangitis should be evaluated for liver transplantation as the outcome after liver transplantation is good, especially if there is no cholangio-carcinoma present and if the Child-Pugh score is not too high. There is also a need to treat the complication of PSC such as osteoporosis, cholangitis and the development of cholangiocarcinoma.
 ...
PMID:Diagnosis and therapeutic problems of primary sclerosing cholangitis. 1289 25

We report two cases of hepatitis C virus (HCV) associated autoimmune haematological disorders successfully treated with an unusual protocol (mycophenolate mofetil: MMF). The first case was a male patient with chronic HCV infection who developed, during interferon (IFN)/ribavirin therapy, severe autoimmune thrombocytopenia unresponsive to steroids. MMF was then administered and, simultaneously, the steroid dose was gradually reduced until withdrawal. Following this strategy, a progressive increase in platelet count and complete negativity of anti-PLT antibodies were achieved without changes in HCV-RNA quantitative determination. The second case was a woman with HCV liver cirrhosis with severe anaemia and Coombs test positivity partially responsive to continuous administration of steroid high doses. However, this treatment unmasked a severely painful vertebral osteoporosis. For this reason we introduced MMF and simultaneously steroid therapy was progressively reduced until withdrawal. Haemoglobin reached a normal value and the Coombs test became negative within 60 days. These case reports suggest that MMF may represent an interesting therapeutic approach for autoimmune HCV associated haematological disorders.
 ...
PMID:Mycophenolate mofetil in the treatment of autoimmune HCV-associated haematological disorders showing steroid resistance or dependence. 1296 91

This study aimed to assess the clinical, biochemical and hormonal factors contributing to low bone density in a large ambulatory group of patients with cirrhosis of diverse aetiology. Bone density of the lumbar spine, neck of femur, total hip, total body, as well as total body fat, was measured by dual X-ray (DEXA) absorptiometry in 81 men and 32 women (average age 50.3 years). Morning blood and urine samples were taken for hormonal and biochemical analysis. Viral hepatitis was the most common cause of cirrhosis (54%) and the severity of cirrhosis ranged from Child-Pugh A5-C14. Osteoporosis was most common in the lumbar spine but was present at any site in 31% of women and 22% of men, with osteopenia present in another 40% of both genders. Urinary deoxypyridinoline, a marker of bone resorption, was elevated in 56% of patients and was associated with increasing severity of cirrhosis and a higher prevalence of osteoporosis, particularly of the lumbar spine. Hip-bone density was primarily affected by low 25-hydroxyvitamin D levels and was associated with secondary hyperparathyroidism in one third of these patients. Additional important predictors for low bone density at all sites were age in women and testosterone in men. These findings indicate that, although the pathophysiology of osteoporosis in chronic liver disease is heterogeneous, high bone turnover may be the underlying pathophysiological mechanism in a significant subgroup of cirrhotic patients and may reflect metabolic effects of hypogonadism or secondary hyperparathyroidism on bone.
 ...
PMID:The heterogeneity of bone disease in cirrhosis: a multivariate analysis. 1450 96

Although best characterized in chronic cholestatic liver disease, osteopenic bone disease and fracturing are well-recognized complications of cirrhosis, particularly after liver transplantation. We sought to compare the prevalence of osteopenia and osteoporosis, to assess the effect of orthotopic liver transplantation (OLT) on bone density, and to determine fracture rates before and after OLT in three groups of patients with advanced cirrhosis: patients with cirrhosis from hepatitis C virus (HCV) alone, from alcohol abuse (ALD), and from HCV in conjunction with alcohol abuse (HCV+ALD). Between 1991 and 2001, 207 consecutive patients who underwent OLT for HCV (68 patients), ALD (66), and HCV+ALD (73) were assessed clinically, biochemically, radiologically, and by bone densitometry. The baseline mean T score was lower in the HCV group than in the ALD group (-1.43 versus -0.87, P =.048) despite ALD patients having more advanced liver disease; patients with HCV+ALD had intermediate T scores. The pattern of significant bone loss at 4 months and 12 months was similar for all three groups. The baseline fracture rate was lowest in the HCV group and highest in the ALD group, despite the latter having the highest bone density. Fractures occurred in 17% of patients in the first year after transplantation, the majority being vertebral compression fractures. Patients with cirrhosis caused by HCV, ALD, or a combination of both should be screened for osteopenia, especially before OLT.
 ...
PMID:Osteopenia and osteoporosis in patients with end-stage liver disease caused by hepatitis C and alcoholic liver disease: not just a cholestatic problem. 1458 77

Osteoporosis is an important and common complication in patients with chronic liver disease. The goal of this study was to determine the bone mineral density (BMD) in different subgroups among pretransplant cirrhotic patients. BMD of the lumbar vertebrae (L) and femoral neck (F) were obtained in 104 consecutive cirrhotic patients. Descriptive and inferential statistics were used to compare the BMD among various groups. The mean BMD in males (n = 54) and females (n = 50) at L were 1.28 +/- 0.25 g/cm(2) and 1.13 +/- 0.20 g/cm(2), respectively (P =.001); at F they were 1.03 +/- 0.14 and 0.91 +/- 0.17, respectively (P <.0001). Among males, BMD at L in Child-Turcotte-Pugh class B and C were 1.40 +/- 0.21 and 1.13 +/- 0.20, respectively (P =.001); at F they were 1.11 +/- 0.10 and 0.93 +/- 0.13, respectively (P <.0001). Among females, BMD at L in Child-Turcotte-Pugh class B and C were 1.27 +/- 0.18 and 1.05 +/- 0.16, respectively (P =.0003); at F they were 1.02 +/- 0.16 and 0.83 +/- 0.12, respectively (P =.001). The BMD in premenopausal females (n = 15) and postmenopausal females (n = 35) at L were 1.20 +/- 0.19 and 1.11 +/- 0.20, respectively (P =.15); at F they were 0.97 +/- 0.17 and 0.88 +/- 0.16, respectively (P =.12). The BMD in postmenopausal females on hormone replacement therapy (n = 19) and on no hormone replacement therapy (n = 16) at L were 1.07 +/- 0.17 and 1.14 +/- 0.23, respectively (P =.29); at F they were 0.85 +/- 0.15 and 0.91 +/- 0.18, respectively (P =.33). The BMD values between etiologic groups were not significantly different. The overall prevalence of osteopenia and osteoporosis were 34.6% and 11.5%, respectively, being significantly higher in females than in males. In conclusion, significant difference in BMD values exists between males and females, as well as between Child-Turcotte-Pugh class B and C patients with cirrhosis. In addition, there is no significant influence of menopausal status, hormone replacement therapy, and etiology of cirrhosis on BMD.
 ...
PMID:Bone mineral density among cirrhotic patients awaiting liver transplantation. 1510 56

Liver cirrhosis is a risk factor for osteoporosis. Nevertheless, little is known about the mechanisms of bone mass loss in patients with viral cirrhosis. TNFalpha is a potent bone-resorbing agent. Serum concentrations of soluble TNF receptor p55 (sTNFR-55) correlate with clinical activity in liver cirrhosis. Our aim was to evaluate the possible role of sTNFR-55 in the pathogenesis of osteoporosis in patients with viral cirrhosis and its relationship with bone turnover markers. We studied 40 consecutive patients with viral cirrhosis and no history of alcohol intake and 26 healthy volunteers. Bone mineral density (BMD) was measured by dual x-ray absorptiometry in the lumbar spine (LS) and femoral neck (FN). Patients with viral cirrhosis had reduced BMD (expressed as the z-score) in all sites [LS, -1.5 +/- 0.22 (P < 0.001); FN, -0.37 +/- 0.15 (P < 0.01)]. Serum concentrations of sTNFR-55 and urinary deoxypyridinoline, a biochemical marker of bone resorption, were significantly higher in patients with osteoporosis than in patients without osteoporosis (P < 0.001 and P < 0.05, respectively). Serum levels of sTNFR-55 correlated inversely with BMD in LS (r = -0.62; P < 0.005) and FN (r = -0.47; P < 0.05) and positively with urinary deoxypyridinoline (r = 0.72, P < 0.001). Our findings show that high serum concentrations of sTNFR-55 play a role in the pathogenesis of viral cirrhosis-associated bone mass loss and provide evidence of increased bone resorption related to the high serum sTNFR-55 levels.
 ...
PMID:Osteoporosis, mineral metabolism, and serum soluble tumor necrosis factor receptor p55 in viral cirrhosis. 1535 28

This article covers in vitro, in vivo, and human data on the positive effect of vitamin K2 on osteoporosis. Data is available on vitamin K2 for osteoporosis caused by a number of conditions, including postmenopausal osteoporosis, Parkinson's disease, biliary cirrhosis, stroke, and drug-induced osteoporosis. The activity of vitamin K2 involves both an increase in the bone-building process and a separate decrease in the bone-loss process. Vitamin K2 exerts a more powerful influence on bone than vitamin K1, and should be considered for prevention or treatment in those conditions known to contribute to osteoporosis.
 ...
PMID:Vitamin K2 in bone metabolism and osteoporosis. 1577 60

Genetic hemochromatosis (GH) is an iron overload disorder mainly due to the C282Y mutation of the HFE gene. The possibility of bone involvement was only recently recognized. The aims of this study were to assess bone mineral density (BMD) and bone remodeling in men with GH, and to examine the influence of iron overload. Thirty-eight men (mean age 47.2+/-9.4 years) with well-defined HFE-related GH were studied. They had an important iron overload with liver iron concentration to age ratio >2.5, no previous venesection therapy and were C282Y homozygotes (n=37) or compound C282Y/H63D heterozygote (n=1). BMD measured by DXA was 0.925+/-0.15 g/cm2 at the lumbar spine (LS) and 0.778+/-0.13 g/cm2 at the femoral neck (FN). Osteopenia (T-score<-1 SD) was observed in 78.9% of patients and osteoporosis (T-score<-2.5 SD) in 34.2%. Vitamin D levels were normal, and no 1-84 parathyroid hormone dysfunction was found. Hypogonadism was found in only 13.2% of patients. Patients with hypogonadism had lower LS BMD than eugonadal patients (0.788+/-0.16 and 0.954+/-0.14 g/cm2). Bone remodeling and parathyroid hormone levels were lower in patients with cirrhosis, but BMD values were similar to those in patients without cirrhosis. FN BMD appeared to fall with rising hepatic iron concentrations (r=-0.399). We conclude that there is significant bone loss in HFE-related hemochromatosis that cannot solely be explained by hypogonadism or cirrhosis. Further investigations are needed to determine the role of iron overload itself.
 ...
PMID:Bone mineral density in men with genetic hemochromatosis and HFE gene mutation. 1592

Dyskeratosis congenita (DC) is a rare inherited bone marrow failure syndrome associated with abnormalities of the skin, fingernails, and tongue. Other clinical manifestations may include epiphora, lung fibrosis, liver cirrhosis, osteoporosis, and a predisposition to develop a variety of malignancies. The clinical picture often resembles that of a premature aging syndrome and tissues affected are those with a high cell turnover. DC has been linked to mutations in at least four distinct genes, three of which have been identified. The product of these genes, dyskerin, the telomerase RNA (TERC), and the catalytic unit of telomerase (TERT) are part of a ribonucleoprotein complex, the telomerase enzyme, that is essential for the elongation and maintenance of chromosome ends or telomeres. All patients with DC have excessively short telomeres, indicating that the underlying defect in these individuals is an inability to maintain the telomeres. The purpose of the current review is to highlight recent insights into the molecular pathogenesis of DC. We discuss the impact these findings have on our current understanding of telomere function and maintenance, and on the diagnosis, management, and treatment of patients with conditions caused by dysfunctional telomeres.
 ...
PMID:Dyskeratosis congenita -- a disease of dysfunctional telomere maintenance. 1597 69

Etidronate is an oral bisphosphonate compound that is known to reduce bone resorption through the inhibition of osteoclastic activity. The efficacy of etidronate for involutional (postmenopausal and senile) and glucocorticoid-induced osteoporosis, as well as that for other skeletal diseases, was reviewed in Japanese patients. Cyclical etidronate treatment (200 mg or 400mg/day for 2 weeks about every 3 months) increases the lumbar bone mineral density (BMD) in patients with involutional osteoporosis and prevents incident vertebral fractures in patients with glucocorticoid-induced osteoporosis. The losses of the lumbar BMD in patients with liver cirrhosis and the metacarpal BMD in hemiplegic patients after stroke are prevented, and the lumbar BMD is possibly increased, preventing fragile fractures in adult patients with osteogenesis imperfecta type I. Furthermore, proximal bone resorption around the femoral stem is reduced and some complications may be prevented in patients who undergo cementless total hip arthroplasty. Oral etidronate treatment may also help to transiently relieve metastatic cancer bone pain followed by a decrease in abnormally raised bone resorption in patients with painful bone metastases from primary cancer sites, such as the lung, breast and prostate. Thus, oral etidronate treatment is suggested to be efficacious for osteoporosis, as well as other skeletal diseases associated with increased bone resorption, in Japanese patients. Randomized controlled trials needed to be conducted on a large number of patients to confirm these effects.
 ...
PMID:Efficacy of oral etidronate for skeletal diseases in Japan. 1598 1


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>