Coexistence of the POEMS syndrome with hepatitis B: a rare case report.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a rare multisystemic disease of unknown pathogenesis. Proinflammatory and proangiogenic cytokines play important roles in its pathogenesis. POEMS syndrome is a rare cause of ascites. Until now, the coexistence of POEMS syndrome and hepatitis B has not been reported. In this case report, we present a 48-year-old male patient who presented with malaise, fatigue, diarrhea, and abdominal swelling. Organomegaly, endocrinopathy, ascites, skin changes, and polyneuropathy were identified, and we arrived at a diagnosis of POEMS syndrome. The patient was administered methylprednisolone 64 mg/day, lamivudine 100 mg/day, calcium 1.5 g/day, and calcitriol 0.5 µg/day. The patient's clinical manifestations had moderately resolved at the follow-up visits. At the end of 6 months of follow-up, his ascites was minimally reduced, and his neurologic manifestations had not lessened. The present case shows that accurate diagnosis is required for the management of patients with coexisting POEMS syndrome and hepatitis B.

Serum paraoxonase activity, total thiols levels, and oxidative status in patients with acute brucellosis.

It is well known that paraoxonase-1 (PON1) activity may decrease during the course of infection and inflammation. The aim of this study was to investigate serum PON1 activity, oxidative status, and thiols levels in patients with acute brucellosis. In addition, we investigated the PON1 phenotype in patients with acute brucellosis. Thirty patients with acute brucellosis and 35 healthy controls were enrolled. Serum paraoxonase and arylesterase activities, thiols levels, lipid hydroperoxide levels, total antioxidant capacity (TAC), total oxidant status (TOS) and oxidative stress index (OSI) were determined. Serum basal and salt-stimulated paraoxonase-arylesterase activities, TAC levels and thiols levels were significantly lower in patients with acute brucellosis than controls (for all, p < 0.05), while LOOH levels, TOS levels, and OSI values were significantly higher (for all, p < 0.05). We concluded that oxidative stress is increased, while serum PON1 activity is decreased in patients with acute brucellosis. These results indicate that lower PON1 activity is associated with oxidant-antioxidant imbalance.

Abnormal liver function test in hydatidiform moles: a retrospective study comparing the hyperthyroid state and the euthyroid state.

INTRODUCTION: The effect of a hyperthyroid or euthyroid state on liver function tests in patients with hydatidiform moles (HM) is not known. The aim of this study was to determine the effect of hyperthyroidism on liver transaminases in HM. PATIENTS AND METHODS: We retrospectively reviewed aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels in 80 patients with HM (23 complete moles and 57 partial moles). RESULTS: Of the 80 HM patients, 52 (65%) were euthyroid and 28 (35%) were hyperthyroid. The number of gravida and the levels of serum ß-human chorionic gonadotropin (ß-HCG), AST, and ALT were significantly higher in the hyperthyroid state than in the euthyroid state (p = 0.033, p = 0.001, p = 0.001 and p = 0.001; respectively). Number of gravida, serum TSH and total T4 were significantly higher in complete HM than partial HM (p < 0.05, p < 0.001, p < 0.05; respectively). CONCLUSIONS: Our results demonstrated that HM-related ß-HCG may activate thyroid cells via TSH-related signalling, resulting in the release of high levels of FT4, FT3, TT3 and TT4, and a subsequent decrease in TSH.

Serum prolidase enzyme activity and oxidative stress levels in patients with diabetic neuropathy.

Previous studies have suggested that prolidase and nitric oxide (NO) regulate many processes, such as collagen synthesis and matrix remodeling. Oxidative stress plays an important role in the development of microvascular complications in diabetic patients. Data on serum prolidase activity in patients with diabetes mellitus or diabetic neuropathy (DN) are limited and conflicting. The aim of this study was to measure serum prolidase activity, NO, total antioxidant status (TAS), and malondialdehyde (MDA) levels in patients with DN. Forty-five patients with DN and 40 healthy controls were enrolled. Serum prolidase activity, TAS, MDA, and NO levels were determined. Serum MDA and NO levels were significantly higher in DN patients than controls (p = 0.002, p = 0.001, respectively), while prolidase activity and TAS levels were lower (p = 0.003, p = 0.001, respectively). Prolidase activity was negatively correlated with NO and MDA (r = -0.911, p < 0.001; r = -0.905, p < 0.001, respectively), while positively correlated with TAS (r = 0.981, p < 0.001) in DN patients. The current study is the first showing the decreased serum prolidase enzyme activity. Our results suggest that decreased collagen turnover may occur in DN patients, who have increased oxidative stress and increased NO levels. Decreased prolidase activity seems to be associated with increased NO levels and oxidative stress along with decreased antioxidant levels in DN. Therefore, decreased prolidase activity may play a role in pathogenesis of DN. Prospective clinical studies are necessary to confirm these findings.

A rare cause of massive ascites: familial Mediterranean fever.

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent fever and peritoneal and pleural inflammation. It is an inherited disorder commonly found in Armenians, Turks, Arabs, Balkans, and Jews originating from North African countries. A small amount of peritoneal fluid collection can be observed during peritoneal attacks in patients with Familial Mediterranean fever, but chronic ascites has been described rarely in these patients. A 42-year-old female patient was admitted to our clinic in June 2010 with fever, severe abdominal pain and abdominal distention that had continued for one month. There was no family history of periodic fevers or abdominal pain. We could not find any cause for ascites, including tuberculosis. A diagnosis of Familial Mediterranean fever was suspected based on the clinical findings and her family history. She was screened for mutations causing Familial Mediterranean fever, and when found to be homozygous for M694V, treatment with colchicine was initiated. After treatment, the amount of ascites decreased, and relief of symptoms was confirmed during a follow-up. In conclusion, because Familial Mediterranean fever is common in our country, it should be considered in the differential diagnosis of patients with ascites of unknown etiology in populations where hereditary inflammatory disease is endemic.

Serum myeloperoxidase activity and oxidative stress in patients with acute brucellosis.

OBJECTIVES: The role of infection in the pathogenesis of atherosclerosis has been increasingly discussed. Previous studies have suggested that increased myeloperoxidase activity plays an important role in the pathogenesis of atherosclerosis. The aim of this study was to investigate the serum myeloperoxidase activity and catalase activity along with lipid hydroperoxide (LOOH) levels in patients with acute brucellosis. DESIGN AND METHODS: Thirty-two patients with brucellosis and 33 healthy controls were enrolled. Serum myeloperoxidase activity, catalase activity and LOOH levels were determined. RESULTS: Serum myeloperoxidase activity and LOOH levels were significantly higher in patients with brucellosis than controls (p<0.05, p<0.001), while catalase activity were significantly lower (p<0.001). LOOH levels were found to be significantly positively correlated with MPO activity (r=0.297, p=0.016) in patients. CONCLUSIONS: These results indicate that increased myeloperoxidase activity and decreased catalase activity is associated with increased oxidative stress, which may have a role in atherosclerotic processes in brucellosis patients.

Skin bleeding time for the evaluation of uremic platelet dysfunction and effect of dialysis.

INTRODUCTION: In patients with chronic kidney disease (CKD) predisposition to bleeding is frequently seen due to disturbances in platelet adhesion and aggregation. Various tests have been utilized to evaluate the disturbance of hemostasis in end-stage renal disease patients. In this trial; we evaluated skin bleeding time in patients admitted to our hospital with uremic symptoms and having hemodialysis (HD) for the first time. We also examined the effects of HD and uremia on this test and investigated its effectiveness in predicting the hemorrhagic complications before implementation of invasive procedures in uremic patients. MATERIAL-METHOD: Twenty nine patients (13 men,16 women; mean age 59.7 ± 18.1) with CKD who presented with symptoms of uremia and treated with HD for the first time were enrolled in this trial. The skin bleeding time were measured before initiation of first hemodialysis and after the second hemodialysis session. RESULTS: The skin bleeding time after the second dialysis was significantly shorter when compared to pre-dialysis values (p < 0.05). Correlation analysis between the skin bleeding time and urea, creatinine, hemoglobin, platelet, and bicarbonate showed no correlation. CONCLUSIONS: Skin bleeding time could reveal the uremic platelet dysfunction and beneficial effect of dialysis in the patients who presented with uremic symptoms and treated with HD for the first time. We suggest that skin bleeding time may be an appropriate test for the evaluation of hemostasis disturbance in uremic patients and prediction of the bleeding risk before invasive procedures.

Relationship between visfatin and some clinical and biochemical parametres in peritoneal dialysis patients.

OBJECTIVE: To characterise the relationship between visfatin levels and various clinical and biochemical parameters in peritoneal dialysis patients. METHODS: The case-control study was conducted at the Medical Faculty Hospital, Yuzuncu Yil University, Van, Turkey, between May 2007 and December 2008, and involving 41 patients on peritoneal dialysis, 20 haemodialysis patients and 20 healthy controls. Fasting visfatin level was measured with enzyme-linked immunosorpent assay (ELISA) method, and patients on peritoneal dialysis were separated into two groups according to the visfatin levels - high and low. The groups were compared in terms of some clinical (height, weight, body mass index, waist circumference, hip circumference, waist/hip ratio, heart rate, systolic and diastolic blood pressure and the kt/V and CrCI (creatanine clearance) parameters which are indicative of the dialysis adequacy) and biochemical parameters (glucose, triglycerides, cholesterol, low density lipoprotein, high density lipoprotein, aspartate aminotransferase, alanine transminase, blood urea nitrogen, creatinine, total protein, albumin, globulin, sodium, potassium, magnesium, calcium, phosphorus, ferritin, venous blood gas, parathyroid hormone and insulin). SPSS 15 was used for statistcal analysis. RESULTS: No statistically significant difference in the visfatin levels was found between the patients and controls (7.71 +/- 4.04, 7.36 +/- 3.71, 7.70 +/- 1.61, respectively, p = 0.63). The triglyceride level of the high-visfatin group was significantly higher than that of the low-visfatin group (243.8 +/- 133.2, 150.8 +/- 65.8, respectively, p<0.05). However, there was no correlation between visfatin and triglyceride levels. No difference in the other clinical and biochemical parametres was observed between the two groups of peritoneal dialysis patients. CONCLUSIONS: No significant difference in the serum visfatin levels of peritoneal dialysis patients compared to haemodialysis patients or healthy individuals was noticed. Further studies are needed to confirm the effect of visfatin on triglyceride levels, and, if confirmed, the mechanism of this relation.

Evaluation of oxidative status in patients with hyperthyroidism.

Data on the antioxidant levels enzyme in patients with hyperthyroidism are limited and conflicting. Therefore, the objective of this study was to evaluate the oxidative status using an automated method in patients with hyperthyroidism. Thirty-six subjects with hyperthyroidism and 30 healthy controls were enrolled in this study. Serum oxidative status was determined via measurement of total antioxidant capacity (TAC) and total oxidant status (TOS) and calculation of oxidative stress index (OSI). Serum TAC levels were significantly lower in patients with hyperthyroidism than controls (P=0.002), while serum TOS levels and OSI values were significantly higher (P=0.008, 0.004; respectively). Serum TAC levels were correlated with TSH levels (rho=0.223, P=0.032), FT3 levels (rho=-0.434, P=0.002) and FT4 levels (rho=-0.363, P=0.003) in patients. Further, TOS levels and OSI values were correlated with TSH levels (rho=-0.245, P=0.037; rho=-0.312, P=0.011, respectively), FT3 levels (rho=0.293, P=0.017, rho=0.505, P=0.002, respectively), and FT4 levels (rho=0.302, P=0.006, rho=0.321, P=0.008, respectively) in patients. Duration of disease was significantly correlated with OSI values in patients (rho=0.420, P=0.011), while no correlation with serum TAC levels and TOS levels (P>0.05). Oxidants are increased and antioxidants are decreased in patients with hyperthyroidism; as a result, the oxidative-antioxidative balance is shifted to the oxidative side. Increased oxidative stress may play a role in the pathogenesis of hyperthyroidism. It is believed that supplementation of antioxidant vitamins such as vitamins C and E may be helpful for these patients.

[Case report: Budd-Chiari syndrome and esophageal variceal bleeding due to alveolar echinococcosis]. / Olgu sunumu: alveoler ekinokoka bagli budd-chiari sendromu ve özofagus varis kanamasi

Alveolar echinococcosis of the liver is a rare larval cestode disease which is due to the intrahepatic growth of the tapeworm Echinococcus multilocularis. This cestode naturally evolves as a larval stage within cysts in the body of carnivores. Humans are accidental intermediate hosts and become infected, either by eating food contaminated with carnivore-originated eggs or by touching foxes. It behaves as malignant liver tumour and rarely causes Budd-Chiari syndrome and variceal bleeding. Budd-Chiari syndrome is a hepatic venous outflow tract obstruction and may be present abdominal pain, hepatomegaly and ascites. Parasitic cysts may cause compression and thrombosis of the hepatic venous outflow tract. It may present as portal hypertension and variceal upper gastrointestinal bleeding. We here in report a 47-year-old woman without a prior history of liver disease presented with Budd-Chiari syndrome and variceal bleeding due to Alveolar echinococcosis. The course of this rare disease is demonstrated by means of the most important laboratory, serologic and radiologic parameters.

Syndrome of inappropriate secretion of antidiuretic hormone cholestasis and pericardial effusion due to brucellosis infection: a case report.

Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) is an extremely rare complication of infectious diseases. A rare case of brucellosis complicated by syndrome of inappropriate secretion of antidiuretic hormone (SIADH) cholestasis and pericardial involvement is reported. A 27-year-old woman was admitted for fever, abdominal pain, and scleral icterus. Her medical history revealed no recent use of diuretic agents. In addition to cholestasis and elevated liver enzymes, euvolemic hyponatremia, hypouricemia, low plasma osmolality, and high urinary osmolality were also detected. Surrenal and thyroid tests were also within normal range. Echocardiography revealed minimal pericardial effusion with normal cardiac functions. The final diagnosis was SIADH due to Brucellosis. Hyponatremia, cholestasis, and pericardial disease were resolved with effective antibrucellar treatment with streptomycine and doxycycline. After completing treatment of brucellosis, there was not any more evidence of cholestasis and pericardial fluid.

Serum cystatin C measurement in differential diagnosis of intra and extrahepatic cholestatic diseases.

OBJECTIVE: Cystatin C is a very potent inhibitor of cysteine proteinases and, it has been clinically applied as a sensitive marker in monitoring of renal and liver functions. The aim of this study was to reveal whether cystatin C may be a useful marker for distinguishing intra- versus extrahepatic cholestasis. MATERIALS AND METHODS: Serum cystatin C concentrations were determined by nephelometric immunoassay using N latex cystatin C kit in 53 patients with cholestatic disorder that included 18 patients with intrahepatic cholestasis , 17 patients with malignant extrahepatic cholestasis , 18 patients with benign extrahepatic cholestasis. Serum cystatin C concentration was also determined in 20 healthy volunteers. RESULTS: Mean serum cystatin C concentration was 2.82 +/- 0.24 mg/l (SD) in patients with intrahepatic cholestasis, 2.05 +/- 0.15 mg/l in patients with extrahepatic malignant cholestasis, 1.37 +/- 0.13 mg/l in extrahepatic benign cholestatic patients and 0.93 +/- 0.24 mg/l in control group. Serum cystatin C concentrations in patients with cholestatic disease were significantly higher than those in the healthy controls (p < 0.001). Moreover, mean serum cystatin C concentration in patients with intrahepatic cholestasis was higher than those in extrahepatic cholestasis groups (p < 0.001). Serum cystatin C concentrations were significantly higher in patients with malignant xtrahepatic cholestasis than in patients with benign extrahepatic cholestasis p < 0.001). There were no correlations patients among serum cystatin C concentrations and serum levels of AST, ALT, ALP, GGT, total and conjugated bilirubin. CONCLUSION: Our results suggested that serum cystatin C level may be a potential biochemical marker both to point out an intrahepatic origin by excluding an extrahepatic source of cholestasis in patients with jaundice and to possibly differentiate bening and malignant extrahepatic cholestatic disorders.