Steatohepatitis and liver cirrhosis in Chanarin-Dorfman syndrome with a new ABDH5 mutation.

Chanarin-Dorfman syndrome (CDS) is an autosomal recessive neutral lipid storage disease. It is very rare and characterized by ichtiosis, intracellular fat droplets in leucocytes (Jordan anomaly) and involvement of multiple tissues (skeletal muscle, central nervous system, bone marrow, eye and ear) mainly the liver. Our patients were diagnosed as CDS because they had ichtiosis, Jordon anomaly of leucocytes in peripheral blood smear, liver involvement and presence of homozygous 88 insertion C frame shift mutation on exon 4 of ABHD5/CGI-58 gene in genetic analysis. Our cases were two sisters. One of them developed severe steatohepatitis on age 19 and the other one was diagnosed as decompensated cirrhosis when she was 26 years old. We report here a new mutation in comparative gene identification-58 (CGI-58) gene causing syndactyly and steatohepatitis induced early cirrhosis.

Prevalence of hepatitis B and C virus infection in barbers in the Sivas region of Turkey.

Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections are among the most devastating health problems in the world, including Turkey. The route of transmission of HBV and HCV is mainly parenteral, a small number of epidemiological studies demonstrating that perinatal, sexual, household and occupational transmission occurs. Contact of a patient's blood or bodily fluids with non-intact skin is another mode of HBV and HCV transmission. Barbers in Turkey may often be exposed accidentally to the blood and bodily fluids of their customers. The aim of this study was to determine the prevalence of HBV and HCV infection in barbers. We conducted a study to determine the prevalence of antibodies against HBV and HCV among 176 barbers and 180 control subjects in the Sivas region of Turkey. The prevalence of HBV and HCV was found to be higher in barbers (39.8 and 2.8%, respectively) than in a comparison group (28.3 and 1.1%, respectively). No significant relationship was found with the duration of occupation. Among the seropositive subjects, it was found that most had been exposed to needle pricks or scissor cuts. Our data suggest that both HBV and HCV infections may constitute occupational hazards for barbers. The sources of infection could be not only such personal risk factors as 'sharps' injuries and scissor cuts, but may also include other unknown factors.

The effect of carnitine on random-pattern flap survival in rats.

Carnitine is an endogenous cofactor involved in the transport of long-chain fatty acids into the mitochondria where they undergo beta-oxidation. Through another reaction, carnitine produces free coenzyme A and reduces the ratio of acetyl-coenzyme A to coenzyme A, thereby enhancing oxidative use of glucose, augmenting adenosine triphosphate synthesis, and reducing lactate production and acidosis. Because of its regulatory action on the energy flow from the different oxidative sources, especially under ischemic conditions, carnitine has been used in cardiovascular diseases such as coronary heart disease, congestive heart failure, peripheral vascular disease, dyslipidemia, diabetes, and chronic renal diseases with satisfactory results. A flap is also a relatively ischemic tissue and may obtain benefit from carnitine. To investigate this, 30 rats were divided into three groups of 10 animals: a control group and two carnitine-treated groups. Random dorsal skin flaps were elevated on the rats. In the control group, no pharmacologic agents were used. Of the two treated groups, group 1 was treated with 50 mg/kg/day carnitine for 1 week and group 2 was treated with 100 mg/kg/day carnitine for 1 week. The areas of flap necrosis were measured in each group. The median areas of flap necrosis of the groups were 12.55, 9.23, and 4.9 cm2, respectively. There was a statistically significant improvement of flap necrosis in carnitine-treated groups compared with the control group (group 2, p = 0.001; group 3, p = 0.000). Furthermore, there was less necrosis in the high-dose carnitine-treated group than the low-dose carnitine-treated group. As a conclusion, carnitine may have a dose-dependent effect to increase flap survival in random skin flaps.

Captopril inhibits the pulmonary toxicity of paraquat in rats.

Paraquat (PQ) is a herbicide that is very toxic to all living organisms. It generates free radicals and leads to acute or chronic lung injury. Free radicals are often associated with fibrogenesis, which occurs in various disease states. The purpose of this study was to determine whether captopril prevents paraquat toxicity in lung tissue. Paraquat alone increased the level of lipid peroxidation (LPO) and the activity of superoxide dismutase (SOD) after 4, 12, 24 and 72 h of administration. Also, the level of hydroxyproline showed an increase after 24 h of paraquat administration. However, paraquat also decreased the level of glutathione (GSH) and the activity of glutathione peroxidase (GSH-Px). Captopril (50 mg/kg i.p.) and paraquat were simultaneously injected (40 mg/kg i.p.), and the captopril injection 1 h after paraquat ameliorated the biochemical toxicity induced by paraquat. This was evidenced by a significant reduction in LPO and balancing the endogenous antioxidant capacity by normalizing the activities of SOD and GSH-Px and the GSH content in the lung tissue. Moreover, captopril injection prevented the increase of hydroxyproline content as an index of lung fibrosis. From these results, the beneficial effects of captopril on paraquat toxicity appear to be through enhancement of the endogenous antioxidant system preventing the lung fibrosis.

Lipid and lipoprotein patterns in type 2 non-obese diabetic patients. Do Lp(a) levels decrease with improved glycemic control in these patients?

BACKGROUND AND AIM: In this study, we investigated the levels of apolipopotein-AI (apo-AI), apolipoprotein (apo-B), triglyceride (TG), high-density-lipoprotein-cholesterol (HDL-C), low-density-lipoprotein-cholesterol (LDL-C), total cholesterol, lipoprotein(a) in a group of non-obese, type 2 diabetes mellitus patients with different types of treatment and a control group of non-obese, non-diabetic subjects. METHODS AND RESULTS: Patients were divided into three groups according to their treatment types: insulin, sulphonylurea and untreated groups. All groups were similar in sex, weights, known duration of diabetes and habits. Each group consisted of 30 subjects. There were no differences in apo-AI, apo-B and TG levels (p > 0.05), whereas HDL-C levels in the untreated group were significantly lower than those of the other groups (p < 0.05). Lp(a) levels in the untreated group were higher than in the other (p < 0.05). CONCLUSIONS: Gaining metabolic control in diabetes mellitus is crucial in pulling back lipid, lipoprotein and apolipoprotein levels to a desired level and in attenuating CAD (coronary artery disease) risk factors, and also in preventing CAD. Lp(a) levels in particular are decreased by insulin or sulfonylurea in non-obese patients with type 2 diabetes mellitus.

The effects of angiotensin-converting enzyme inhibitors on the clinical and biochemical parameters in diabetic nephropathy.

Captopril's short-term effects on clinical and biochemical parameters were studied in 21 diabetic nephropathic patients. Their mean age was 57.50 +/- 2.28 years; 16 of them were women and 5 were men. Eleven patients had been regulated with insulin and 10 of them had been regulated with oral antidiabetics. Fifteen patients were microalbuminuric (200 mg/daily and below albuminuria) and their mean diabetes mellitus history was 14.86 +/- 1.44 years. Six patients had advanced diabetic nephropathy (400 mg/daily and above albuminuria). Their mean diabetes mellitus history was 4.50 +/- 2.87 years. Captopril in a low dose (37.5 mg/daily p.o., three separated doses) was given during 20 days. In the microalbuminuria group there were insignificant alterations in renal function, blood glucose levels, and systolic blood pressure. Diastolic blood pressure decreased significantly in this group (p < .05). Microalbuminuria increased significantly after the therapy in this group (p < .05). In the advanced diabetic nephropathy group, blood glucose and systemic blood pressure levels did not change significantly (p > .05), while serum BUN and creatinine levels increased significantly (p < .05), and GFR decreased significantly in this group (p < .05). Albuminuria decreased after the therapy in this group (p < .05). In all study groups, serum potassium levels increased significantly while serum total protein and albumin levels did not change significantly.We concluded that in the microalbuminuria group, increasing microalbuminuria may be related to a captopril-induced increase in renal plasma flow rate and single nephron glomerular filtration rate. This increase in microalbuminuria cannot be related with blood glucose levels, renal functions, and systemic blood pressure alterations.(ABSTRACT TRUNCATED AT 250 WORDS)