Plasma Cysteine and Sulphate Levels in Patients with Cirrhosis of the Liver

1994 ◽  
Vol 87 (3) ◽  
pp. 357-362 ◽  
Author(s):  
M. H. Davies ◽  
L. Klovrza ◽  
R. H. Waring ◽  
E. Elias

1. Fasting levels of plasma cysteine, plasma sulphate and the plasma cysteine/sulphate ratio were measured in patients with primary biliary cirrhosis and compared with those in patients with other liver disease, general intensive therapy unit patients and healthy subjects. 2. Plasma cysteine was significantly elevated in patients with primary biliary cirrhosis (median 0.364 nmol/mg of protein, P < 0.0001) and patients with other liver disease (median 0.445 nmol/mg of protein, P < 0.0001), compared with healthy control subjects (median 0.125 nmol/mg of protein) and increased progressively with the severity of liver disease. Plasma cysteine was also elevated in intensive therapy unit patients (median 1.564 nmol/mg of protein) compared with healthy control subjects (P < 0.0001) and patients with other liver disease (P < 0.0001). 3. Plasma sulphate was reduced significantly only in patients with primary biliary cirrhosis (median 0.822 nmol/mg of protein) compared with healthy control subjects (median 1.37 nmol/mg of protein, P < 0.05). There was no significant difference in plasma sulphate between disease groups. 4. The plasma cysteine/sulphate ratio was significantly elevated in patients with primary biliary cirrhosis (median 0.448, P < 0.0001) and patients with other liver diseases (median 0.394, P < 0.0001) compared with healthy control subjects (median 0.095). The ratio was also elevated in intensive therapy unit patients (median 1.650, P < 0.0001) compared with healthy control subjects and liver disease groups (P < 0.0001). 5. In conclusion, plasma cysteine rises in primary biliary cirrhosis and other forms of liver disease. This effect is not specific to liver disease, since cysteine is elevated in an heterogeneous group receiving intensive care. Impairment of trans-methylation and trans-sulphuration pathways does not explain the finding of increased plasma cysteine. Since cysteine is elevated in non-hepatic disease, it may reflect the effect of muscle breakdown and the catabolic state. Impaired activity of cysteine dioxygenase and impaired mitochondrial function may be contributory, but this requires further study. These metabolic changes may reflect progressively diminished detoxification capacity within the liver and other tissues.

1996 ◽  
Vol 91 (5) ◽  
pp. 551-558 ◽  
Author(s):  
David E. J. Jones

1. Primary biliary cirrhosis is a chronic cholestatic liver disease with an autoimmune aetiology. The classical histopathological lesion, of portal tract biliary epithelial cell damage, is accompanied by a T-cell-rich mononuclear cell infiltrate and upregulation of cell surface markers suggestive of local T-cell activation and cytokine release. This suggests that T-cell mediated mechanisms play an important role in tissue damage in primary biliary cirrhosis. 2. CD4+ T-cells specific for the E2 component of human pyruvate dehydrogenase complex (PDC-E2), a highly conserved enzyme which plays a critical role in intermediate metabolism, are present in the peripheral repertoire of the majority of patients with primary biliary cirrhosis. These cells are almost entirely absent from normal and chronic liver disease control subjects. The observations that peripheral blood PDC-E2-specific cells are most commonly seen in early stage disease, when active bile duct damage is occurring, and that PDC-E2-specific cells can be found in the portal tract infiltrate at times when this damage is occurring, suggest that these autoreactive cells may have a role to play in the aetiology of primary biliary cirrhosis. 3. T-cells specific for the whole PDC and its E1 component are seen in significant numbers of normal control subjects as well as patients with primary biliary cirrhosis. Retention of potentially autoreactive cells in the normal T-cell repertoire has been reported for a number of other autoantigens. 4. T-cell epitopes appear to be widely distributed throughout PDC-E2. This is in contrast to the B-cell epitopes which are highly restricted to the inner lipoyl binding domain of the protein.


2020 ◽  
pp. 4-6

The present study was carried out in the Labs of collage of education for pure science, during period from January 2017 to endDecember of the same year. The immune status investigates for CLpatients by measuring the levels of cytokines (IL6and IL10) in sera using a technique enzyme-linked immune Sorbent adsorptive (ELISA). The study included 120 subjects with (60 CLpatientsL.majar and 60 CLpatients L. tropica with and (30) were healthy control. Increased mean Serum level of IL6 was in the observed in the total patients as compared to control Subjects (224.53pg/ml,70.70pg/ml), the result indicate there was significant difference at (p<0.05) ,such observation was consistent in the patient infected with L.majar and L. tropica (104 .90 pg/ml and 112.78 pg/ml) respectively. The results of the IL10 showed significant difference at (p<0.05)increased of mean Serum level in the total CL patients as compared to control Subjects(226.90 pg/ml 46.77pg/ml,).Ahighly significant difference at (p<0.05) increased observed in patients group infected with L.majar and followed by patients group infected L. tropica (112.78pg/ml and 114.12pg/ml) respectively.These results revealed that the excessive presence of cytokines might play a role in CL patients.


1985 ◽  
Vol 69 (5) ◽  
pp. 561-570 ◽  
Author(s):  
E. Barbara Mawer ◽  
H. J. Klass ◽  
T. W. Warnes ◽  
Jacqueline L. Berry

1. The metabolism of isotopically labelled vitamin D2 and D3 has been investigated in eight patients with primary biliary cirrhosis and in five controls. The concentration of labelled vitamin D2 was lower than that of vitamin D3 in serum of patients with primary biliary cirrhosis on days 1 and 2 after intravenous injection (P < 0.005 and P < 0.05, respectively) but no difference was seen in controls. 2. Similar amounts of labelled 25-hydroxyvitamin D2 and D3 were seen in serum of the control group; the same pattern was observed in the primary biliary cirrhosis group, and no significant differences were observed between the two groups. 3. In both control and primary biliary cirrhosis groups, the serum concentration of labelled 24,25-dihydroxyvitamin D2 exceeded that of 24,25-dihydroxyvitamin D3 (significant for controls on day 2, P < 0.02) but concentrations in the two groups were not different. 4. Concentrations of labelled 25,26-dihydroxyvitamin D3 were significantly higher than those of 25,26-dihydroxyvitamin D2 in the primary biliary cirrhosis group at all times and in the control group on days 2 and 3. Both 25,26-dihydroxyvitamin D2 and D3 were higher in the serum of patients with primary biliary cirrhosis than in controls (significant on day 1, P < 0.05). 5. Urinary excretion over days 0–3 of radioactivity from both vitamins D2 and D3 was significantly higher in the primary biliary cirrhosis group than in controls: 12.03 vs 1.80% for vitamin D2 and 8.98 vs 1.76% for vitamin D3(P < 0.005). Vitamin D2-derived urinary radioactivity in primary biliary cirrhosis correlated strongly with serum bilirubin (P = 0.005). 6. The metabolism of labelled vitamin D3 was studied in seven patients with alcoholic liver disease, three of whom showed low serum concentrations of labelled 25-hydroxyvitamin D3 suggesting impaired hepatic synthesis. The 25-hydroxylation response was quantified as the relative index of 25-hydroxylation and was significantly related to two other indices of liver function. It is concluded that impaired 25-hydroxylation of vitamin D may occur in alcoholic liver disease and results from hepatocellular dysfunction. 7. Less than the predicted amounts of 1,25-dihydroxyvitamin D3 were produced in four of the seven patients with alcoholic liver disease; this defect may be attributable in part to decreased precursor 25-hydroxyvitamin D and to poor renal function.


2015 ◽  
Author(s):  
Daniel S. Pratt ◽  
Lindsay Y. King

Primary biliary cirrhosis (PBC) is a progressive autoimmune disease of the liver. It is the most common cause of chronic intrahepatic cholestatic liver disease in adults. This review addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of PBC. Figures show the pathogenesis and natural history of PBC and histologic features of the four stages of PBC. Tables list diagnostic criteria for PBC via the American Association for the Study of Liver Diseases, differential diagnosis for PBC, medications used to treat PBC, secondary therapy for PBC, and follow-up of patients with PBC. This review contains 2 highly rendered figures, 5 tables, and 45 references.


2011 ◽  
Vol 2011 ◽  
pp. 1-12 ◽  
Author(s):  
Cristina Rigamonti ◽  
Dimitrios P. Bogdanos ◽  
Maria G. Mytilinaiou ◽  
Daniel S. Smyk ◽  
Eirini I. Rigopoulou ◽  
...  

Patients with primary biliary cirrhosis (PBC) often have concurrent limited systemic sclerosis (SSc). Conversely, up to one-fourth of SSc patients are positive for PBC-specific antimitochondrial antibodies (AMA). The mechanisms responsible for the co-occurrence of these diseases are largely unknown. Genetic, epigenetic, environmental, and infectious factors appear to be important for the pathogenesis of the disease, but the hierarchy of events are not well defined. Patients with SSc and PBC have an increased morbidity and mortality compared with the general population, but whether the presence of both diseases in an affected individual worsens the prognosis and/or outcome of either disease is not clear. Some case reports suggested that the presence of SSc in PBC patents is associated with a more favorable prognosis of the liver disease, whereas others report an increased mortality in patients with PBC and SSc compared to patients with PBC alone. This paper discusses the features of patients with PBC-associated SSc. Our aims are to clarify some of the pathogenetic, diagnostic, and clinical challenges that are currently faced in the routine management of these patients. We also intend to provide some practical hints for practitioners that will assist in the early identification of patients with PBC-associated SSc.


Author(s):  
Mahmut Atum ◽  
Gürsoy Alagöz

Purpose: This study aimed to compare the neutrophil-to-lymphocyte (NLR) and plateletto- lymphocyte (PLR) ratios in patients with retinal artery occlusion (RAO) with those from a healthy control population and to identify the relationship between them. Methods: Forty-six patients with RAO and fifty-one healthy control subjects were included in this retrospective case-control study. RAO was diagnosed following an ophthalmic examination and fluorescein angiography (FA). Blood neutrophil, lymphocyte, and platelet counts were recorded for each of the 97 subjects, from which NLR and PLR values were calculated. Results: There were 46 patients (28 male [M], 18 female [F]) in the RAO group and 51 patients (27 M, 24 F) in the control group. No significant differences were found between patients with RAO and the control subjects in terms of gender and age (P > 0.05). Patients with RAO had significantly increased NLR values (2.85 ± 1.70) than the control subjects (1.63 ± 0.59, P < 0.001). The mean PLR in patients with RAO was 123.69 ± 64.98, while that in control subjects was 103.08 ± 36.95; there was no significant difference between the two groups (P = 0.055). A logistic regression analysis revealed that NLRs were 3.8 times higher in patients with RAO than in control subjects (odds ratio = 3.880; 95% confidence interval = 1.94 to 7.74; P < 0.001). Conclusion: NLRs were significantly increased in patients with RAO compared to the control subjects.


2003 ◽  
Vol 38 (1) ◽  
pp. 52-59 ◽  
Author(s):  
Kazuhide Yamamoto ◽  
Ryo Terada ◽  
Ryoichi Okamoto ◽  
Youichi Hiasa ◽  
Masanori Abe ◽  
...  

1999 ◽  
Vol 54 (5) ◽  
pp. 165-168 ◽  
Author(s):  
Bruno Cupertino Migueletto ◽  
Abrahão Elias Hallack Neto ◽  
Elaine Zamora Domingues ◽  
Pedro Paulo Neves de Castro ◽  
Hartmut Stocker ◽  
...  

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.


Critical Care ◽  
10.1186/cc555 ◽  
1999 ◽  
Vol 3 (Suppl 1) ◽  
pp. P182
Author(s):  
A Verma ◽  
M Phillips ◽  
J Wendon

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