Amyloidosis in Behçet's disease.
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Biomedical subjects
Publications and source records attributed to S Dündar.
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Erythrocyte half-life (Et-1/2) has been measured in 11 patients with poorly controlled blood sugar levels and compared with a normal control group to determine whether decreased red cell deformability, which occurs in diabetic patients, causes shortening of erythrocyte life or not. No difference was seen (Et-1/2 = 28.1 days in diabetic patients and 28.5 days in controls). There was also no correlation between Et-1/2 and glycosylated hemoglobin (HbA1) levels. It has been concluded that poorly controlled diabetes mellitus has no effect upon erythrocyte life.
The proteins beta-thromboglobulin, platelet factor 4 and thrombospondin are stored in platelet alpha-granules and released from the platelet by the release reaction. The assays of these proteins were studied in patients with type I diabetes mellitus (n = 30) and a healthy control group (n = 15). Platelet factor 4 and beta-thromboglobulin levels were not significantly different in both groups but thrombospondin concentrations in diabetic patients were significantly higher than those of the control group (136.6 +/- 14.2 ng/ml vs 91.2 +/- 14.3 ng/ml, P < 0.05). When the diabetic patients were divided into those with or without complications, the diabetic patients with complications (n = 11) had significantly elevated plasma thrombospondin concentrations compared with the control group (150.4 +/- 23.7 ng/ml vs 91.2 +/- 14.3 ng/ml, P < 0.05), while thrombospondin concentrations in the control group were not statistically different from the diabetic patients without complications. Plasma beta-thromboglobulin and platelet factor 4 levels were not significantly different between the diabetic and the control group. It is suggested that thrombospondin may be a convenient marker of in vivo platelet release reaction.
INTRODUCTION: Central retinal vein occlusion (CRVO) tends to be a disease of older individuals, with some known major risk factors, and activated protein C resistance seems to be one of the causes in younger patients. In this study, we reviewed risk factors including activated protein C resistance in a series of patients with CRVO. METHODS: Twenty-four patients with a diagnosis of CRVO presenting either acutely or for a follow-up visit during the study period, were enrolled. Ages ranged between 31 and 83 years (mean 59.7). The risk factors, presence of ischemia, the results of biochemical serological and hematological tests, especially activated protein C resistance (APC-R), were analysed. RESULTS: In 17 patients (71%) one or more of the following risk factors existed: hypertension, diabetes, oral contraceptive usage, family history of thrombosis, and previous operation. In 40% of the eyes, there were ischemic changes. Serum triglycerides were high in 4 of 21 patients (19%) and cholesterol and urea were high in 10 of 21 patients (19%). APC-R was found in 6 out of 24 patients (25%). CONCLUSIONS: Besides known predisposing factors like hypertension, diabetes, glaucoma and hyperlipidemia, APC-R seems to be a risk factor for CRVO at all ages.
The vasoactive products of uncontrolled protease activity can play an important role in the pathogenesis of respiratory distress syndrome in newborn infants. In this study alpha-2-macroglobulin, which has antiproteolytic activity, was studied in the first few hours of life in preterm infants with and without respiratory distress syndrome. Mean plasma alpha-2-macroglobulin levels were found to be similar in both groups. These findings implicated that alpha-2-macroglobulin has no significant role in the pathogenesis of respiratory distress syndrome.
The serum plasminogen status in 35 preterm infants with or without respiratory distress syndrome (RDS) was determined in the first few hours of life. Blood samples for plasminogen testing (Synthetic chromogenic substrate method; Stachorm PLG. Diagnostica Stago) were obtained from a peripheral vein within six hours after birth. Among 35 infants, 20 infants who were in stable clinical condition served as the control group. Fifteen developed RDS with clinical, laboratory and radiological findings. The mean plasma plasminogen levels were found to be similar in the two groups (p > 0.05 by Mann-Whitney U test). These findings are discussed along with our previous findings showing normal tissue plasminogen activator (tPA) but lower D-dimer and higher plasminogen activator inhibitor (PAI) levels within the first few hours of life in preterm infants who later developed RDS compared to the control group.
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A 39-year-old woman was admitted with fatigue, weight loss, and fever. Nothing, except skin pallor could be found on physical examination. Her haemoglobin (Hb) was 6.3 g/dl. The blood picture showed dimorphic red cell changes and there were dyserythropoiesis and ring sideroblasts in the bone marrow. After detailed investigations, she was diagnosed with tuberculosis, and anaemia was assigned to chronic disease. With anti-tuberculosis therapy (including isoniazid), her Hb and bone marrow findings returned to normal. After cessation of therapy, Hb fell to 8.9 g/dl. Bone marrow examination again showed dyserythropoietic morphologic abnormalities and ring sideroblasts. No reason could be identified to explain the recurrence of anaemia. When we realised that preparations of isoniazid included vitamin B6 to prevent the development of sideroblastic anaemia, we challenged with pyridoxin 200 mg daily. Her Hb rose to 14.6 g/dl. We suggest that in any cases with sideroblastic anaemia, if no cause can be identified, or anaemia persists or recurs despite therapy, pyridoxine therapy should be instituted.
BACKGROUND/AIMS: Congenital hepatic fibrosis (CHF), which is one of the fibropolycystic diseases, occurs in various forms. Portal hypertension, a very common clinical feature of this condition, has been attributed to the compression of portal vein radicles in the fibrous bands. We investigated whether there are any other contributing factors in the development of portal hypertension in patients with CHF. METHODOLOGY: A total of 1285 patients with portal hypertension of different etiologies were studied using ultrasonography as the screening test. Forty-seven (including portal vein involvement and/or CHF) of these 1285 patients were prospectively studied to evaluate the etiology of the portal hypertension by portography, abdominal computed tomography, exploratory laparotomy, peritonoscopy, liver biopsy and laboratory tests. The patients with CHF were divided into two groups, according to whether or not they had portal vein involvement. RESULTS: Eleven (0.8%) of the 1285 patients with portal hypertension had CHF, and 41 (3.2%) had cavernous transformation of the portal vein (CTPV), resulting from different or unknown etiologies. Five patients had both pathologies (CTPV and CHF). In the 11 patients with CHF, there was CTPV in 5 patients, Caroli's disease in 2 patients, cholangiocarcinoma in 1 patient, inferior vena caval obstruction in 1 patient, and CHF in only 2 patients. There were statistically significant differences in the age of the CHF patients at clinical onset, the incidence of bleeding from esophageal varices, and laboratory findings between the 2 groups with and without CTPV. Despite a thorough investigation, we could not distinguish any predisposing factor in 25 of the 41 patients with CTPV. The incidence of CTPV was 48% in patients with CHF and 3.2% in patients with portal hypertension. CONCLUSIONS: These results suggest that the association of CTPV with CHF is not coincidental, but that CTPV may be associated with CHF and a new possible factor in portal hypertension, and that it can be a major factor in the manifestation of esophageal bleeding from varices.
Infants with respiratory distress syndrome (RDS) undergo hemodynamic alterations in both the pulmonary and systemic vascular beds that predispose these infants to microvascular damage. In addition, disseminated intravascular coagulation (DIC) is frequently encountered in preterm infants with advanced RDS. In preterm infants with RDS, the plasma status is not known. Therefore, we studied plasma thrombomodulin levels in 25 preterm infants who did or did not develop RDS within the first few hours of life. The mean plasma thrombomodulin levels were found to be similar in the two groups (8.12 +/- 10.20 ng/ml vs. 18.30 +/- 22.77 ng/ml, respectively, p > 0.05 by Mann-Whitney U Test). It seems that plasma thrombomodulin levels are normal in the earlier stages of RDS before the occurrence of severe vascular damage and DIC due to hypoxemia.