Sneddon's syndrome is characterized by livedo reticularis and cerebrovascular lesions. We report the cases of women (mean age, 36.2 +/- 8.1 years) diagnosed with Sneddon's syndrome based on the presence of livedo reticularis and characteristic cerebrovascular findings. Seven of these patients had cerebral infarcts on cranial computed tomography scan. Antiphospholipid antibodies were positive in 6 of these cases. Three cases had abnormal levels of antithrombin III. Analyses of chromosome 6 revealed no abnormalities. In 3 of the cases, investigation of the pedigrees revealed autosomal dominant traits. Two cases had epilepsy, and 3 had migraine. One case with migraine also had myasthenia gravis. In addition, we detected inferior altudinal hemianopia in 2 cases, cognitive functional disorder in 3 and depression in 2. Based on these findings, the entire vascular, haematologic, neurologic, and dermatologic systems should be evaluated in patients diagnosed with Sneddon's syndrome. 相似文献
Background/AimsTo investigate the presence of seronegative celiac disease in patients with isolated refractory dyspepsia and gastroesophageal reflux disease (GERD)-related complaints.MethodsThis was a single-center, prospective study performed at a tertiary care referral hospital. Among 968 consecutive patients, 129 seronegative patients with tissue damage consistent with Marsh IIIa classification or above were included. The patients were divided into two groups dyspepsia (n=78) and GERD (n=51). Biopsies were taken from the duodenum regardless of endoscopic appearance, and patients with Marsh IIIa or above damage were advised to consume a gluten-free diet. The Glasgow Dyspepsia Severity (GDS) score, Reflux Symptom Index (RSI), and Biagi score were calculated at baseline and every 3 months. Control endoscopy was performed every 6 months during follow-up.ResultsThe median follow-up time was 19.9 months (range, 6 to 24 months) in the dyspepsia group and 19.2 months (range, 6 to 24 months) in the GERD group. All the patients were positive for the HLA-DQ2 and DQ8 haplotypes. The differences between the mean GDS scores (14.3±2.1 vs 1.1±0.2, respectively, p<0.05), RSI scores (6.3±0.8 vs 0.7±0.1, respectively, p<0.05), and Biagi scores (3.1±0.4 vs 0.7±0.3 in the dyspepsia group and 2.5±0.4 vs 0.5±0.2 in GERD group) before and after implementation of the gluten-free diet were statistically significant. The decreases in the scores were consistent with improvements in the histological findings. There was no significant correlation between endoscopic appearance and histological examination results (p=0.487).ConclusionsSeronegative celiac disease may be considered in this group of patients. Even if a patient is seronegative and has normal endoscopic findings, duodenal biopsy should be considered. 相似文献
Transient left ventricular apical ballooning syndrome is characterized by reversible left ventricular wall motion abnormalities, chest pain or dyspnea, ST-segment elevation, and mild elevation of cardiac enzyme levels in the absence of obstructive coronary artery disease. The pathophysiology of the syndrome is still unknown. The probable mechanism is supposed to be a catecholamine discharge. We report the case of a 66-year-old woman with recently diagnosed pheochromocytoma who presented with chest pain and ST-segment elevation. Coronary angiography revealed normal coronaries and apical dyskinesia at ventriculography. A similar episode of chest pain occurred 4 years ago with same angiographic findings and reversible inferobasal akinesia. In-hospital course was uneventful and the patient was discharged from the hospital 4 days later with treatment of aspirin 1 × 100 mg, metoprolol 1 × 50 mg, lisinopril 1 × 10 mg, and atorvastatin 1 × 20 mg. At 2 years follow-up after the event, the patient remained asymptomatic. 相似文献
Background: Prediction of preeclampsia and adverse maternal and perinatal outcomes with biomarkers has been proposed previously. Anti-mullerian hormone (AMH) is a growth factor, which is primarily responsible of the regression of the mullerian duct, but also used to predict ovarian reserve and decreases with age similar to the fertility.
Aim: To evaluate the predictive role of maternal anti-mullerian hormone (mAMH) in adverse maternal and perinatal outcomes in preeclampsia.
Methods: This prospective case-control study was conducted at current high-risk pregnancy department in a tertiary research hospital and 45 cases with preeclampsia classified as study group and 42 as control group. Data collected and evaluated were; age, body mass index (BMI), marriage duration (MD), gestational weeks (GW), gravidity, parity, mode of delivery, birth weight, newborn Apgar score, newborn gender, maternal complication, perinatal outcome, some laboratory parameters and mAMH. The association between mAMH levels and maternal and fetal outcomes were evaluated.
Results: There were no statistically significant differences between groups in terms of age, BMI, MD, gravidity, parity and newborn gender (p?>?0.05). GW, vaginal delivery, birth weight, newborn Apgar score, were statistically significantly lower in preeclamptic patients when compared with non-preeclamptic patients (p?<?0.001). Adverse maternal and perinatal outcomes were statistically significantly higher in the study group (p?<?0.001). The laboratory values [alanine transaminase (ALT), aspartate transaminase (AST), blood urea nitrogen (BUN), creatinine, lactic dehydrogenase (LDH), uric acid and fibrinogen) were statistically significantly lower in the control group (p?<?0.001). The mAMH level was significantly lower in the preeclamptic group (p: 0.035). There was no correlation between mAMH levels and demographic and clinical parameters. The area under the ROC curve (AUC) was 0.590 and the cut-off value was 0.365?ng/ml with sensitivity of 67.4% and specificity of 47.1% for mAMH. Logistic regression analysis showed a statistically insignificance between mAMH and maternal complication and perinatal outcome (p: 0.149).
Conclusion: According to this study, mAMH level was lower in preeclamptic patients than in normal pregnants, and is found to be a discriminative factor with low sensitivity and specificity. There was no relationship between mAMH and adverse maternal and perinatal outcomes. Further randomized controlled studies with more participants are needed to evaluate the accurate effects of mAMH levels on preeclampsia and should increase the power of mAMH levels in predicting the preeclampsia. 相似文献
Elevated concentrations of asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide synthase, are associated with endothelial dysfunction. Metabolic syndrome (MetS) is also a condition associated with impaired endothelial function. To test the hypothesis that lipid lowering treatment with a statin lowers ADMA levels, we investigated the effect of fluvastatin treatment on serum ADMA levels in patients with MetS. A total of 85 hypercholesterolemic MetS patients (53 females, 32 males; mean age, 55.8+/-9.1 years) were included in this prospective, randomized, controlled study. Patients were randomly assigned to either the treatment (n=42) or control group (n=43). Recommendations for lifestyle modification were provided to both groups. In addition, the patients in the treatment group received fluvastatin, extended release tablets, 80 mg/day, orally for 6 weeks. Serum levels of ADMA and lipids were assessed at baseline and at the completion of treatment. High performance liquid chromatography was used to measure serum ADMA concentrations. In the fluvastatin group, there was a significant reduction in serum ADMA levels compared to baseline (from 1.57+/-1.07 micromol/L to 1.17+/-1.41 micromol/L, P<0.05), whereas in the control group no significant change was observed (from 1.06+/-0.46 micromol/L to 1.24+/-1.38 micromol/L, P>0.05). There was a statistically significant difference between the groups in terms of mean percent change from baseline (P=0.047). Fluvastatin treatment for hypercholesterolemia in patients with MetS is associated with a decrease in serum ADMA levels at 6 weeks. This finding is consistent with known beneficial effects of statin treatment on endothelial function in hypercholesterolemic patients. 相似文献
Journal of Clinical Immunology - Severe combined immunodeficiency is an inborn error of immunity characterized by impairments in the numbers and functions of T and B lymphocytes due to various... 相似文献
Increased deposition of the extracellular matrix components, particularly collagen, is a central phenomenon in liver fibrosis. Stellate cells, the central mediators in the pathogenesis of fibrosis are activated by free radicals, and synthesize collagen. Melatonin is a potent physiological scavenger of hydroxyl radicals. Melatonin has also been shown to be involved in the inhibitory regulation of collagen content in tissues. At present, no effective treatment of liver fibrosis is available for clinical use. We aimed to test the effects of melatonin on dimethylnitrosamine (DMN)-induced liver damage in rats. Wistar albino rats were injected with DMN intraperitoneally. Following a single dose of 40 mg/kg DMN, either saline (DMN) or 100 mg/kg daily melatonin was administered for 14 days. In other rats, physiologic saline or melatonin were injected for 14 days, following a single injection of saline as control. Hepatic fibrotic changes were evaluated biochemically by measuring tissue hydroxyproline levels and histopathogical examination. Malondialdehyde (MDA), an end product of lipid peroxidation, and glutathione (GSH) and superoxide dismutase (SOD) levels were evaluated in blood and tissue homogenates. DMN caused hepatic fibrotic changes, whereas melatonin suppressed these changes in five of 14 rats (P < 0.05). DMN administration resulted in increased hydroxyproline and MDA levels, and decreased GSH and SOD levels, whereas melatonin reversed these effects. When melatonin was administered alone, no significant changes in biochemical parameters were noted. In conclusion, the present study suggests that melatonin functions as a potent fibrosuppressant and antioxidant, and may be a therapeutic choice. 相似文献
[Purpose] Paget-Schroetter syndrome (PSS) is an uncommon deep vein thrombosis of the
axillary and subclavian veins which may occur spontaneously, but is usually caused by
excessive upper limb activity. PSS is clinically similar to other upper limb
musculoskeletal disorders and soft tissue infections, and this may lead to delay in
correct diagnosis in its early stages. The aim of our case report is to discuss this rare
condition with reference to the available literature. [Subjects and Methods] Here we
report the case of a doner kebab chef who complained of swelling and pain in his right arm
around the biceps muscle. The initial diagnosis was biceps tendon rupture, for which the
patient underwent magnetic resonance imaging (MRI) of the right arm and shoulder. Since
the MRI revealed no pathological findings, right upper limb venous Doppler ultrasound
analysis was performed. Subacute thrombosis materials were detected in the subclavian,
axillary, and brachial veins. [Results] With rapid anticoagulant therapy, the patient’s
symptoms quickly improved. [Conclusion] Early diagnosis and treatment of PSS is critical
for preventing potentially fatal complications such as pulmonary embolism. Prophylaxis is
important for preventing recurrent thrombosis and for avoiding the development of
post-thrombotic syndrome. PSS should be considered a possible cause of painful swelling of
the upper limbs, especially in young, active patients who use their arms excessively.Key words: Doner kebab chef, Effort thrombosis, Paget-Schroetter syndrome相似文献