Hyperhomocysteinemia Independently Associated with Adult Moyamoya Disease: Hospital Based Study of 237 Patients.

AIM: To clarify the risk factors for adult moyamoya disease (MMD) in patients from South China. MATERIAL AND METHODS: We prospectively studied adult patients who were diagnosed angiographically with MMD. The demographic profiles, medical history and clinical characteristics were compared between adult MMD and non-MMD stroke patients. Logistic regression analysis was used to determine the risk factors associated with adult MMD. RESULTS: A total of 35 adult MMD patients and 202 adults patients with non-MMD stroke were included. Of the 35 MMD patients, bilateral MMD occurred in 48.6% and bypass surgery was performed in 28.6%; these figures were significantly lower than those reported in patients from Korea and the United States (p < 0.05). After adjusting for baseline demographics and potential confounders, multivariate logistic regression analysis was conducted, which showed that the plasma homocysteine level (odds ratio [OR]: 1.10; 95% confidence interval [CI]: 1.06?1.14) and occupation as a technological worker (OR: 4.23; 95% CI: 1.65?10.89) were independently associated with adult MMD. CONCLUSION: Hyperhomocysteinemia and type of occupation were found to be independent risk factors for adult MMD in patients from South China. However, there is still a need for further research to clarify the pathogenesis of MMD. Given the lack of understanding about the risk factors and prevention measures for MMD, we suggest bypass surgery be used for MMD treatment in clinical practice in China to achieve more desirable effects in the management of the disease.

Risk factors and predictors of outcomes in 243 Chinese patients with cerebral venous sinus thrombosis: A retrospective analysis.

OBJECTIVES: To investigate the risk factors and predictors of outcomes in a cohort of Chinese patients with cerebral venous sinus thrombosis (CVST), so as to provide a reference for customized clinical decision. PATIENTS AND METHODS: A total of 243 Chinese patients, diagnosed as a first CVST were enrolled in this retrospective study from March 2013 through April 2017. Risk factors and predictors of outcomes for CVST were summarized and analyzed by Chi-square test and logistic regression analysis. RESULTS: Of the 243 cases, obstetric cause (19.8%) was the leading risk factor for CVST, followed by infection (17.7%) and anemia (17.7%). Gender differences in the risk factors for CVST were analyzed, showing that obstetric cause was the top risk factor in female, while hyperhomocysteinemia (22.3%) was the top risk factor in male. In age subgroups, obstetric cause (26.3%) and anemia (17.6%) were more commonly observed in age ≤ 44 years and age > 44 years subgroup, respectively. The ratio of poor outcomes (mRS = 3-6) in this cohort was 23.0%, and central nervous system (CNS) infection was closely related to poor outcomes at discharge (p = 0.023). CONCLUSION: The predominant risk factor for CVST, in this Chinese cohort, may still be obstetric cause in female and hyperhomocysteinemia in male. In addition, CNS infection may predict poor outcomes in CVST patients.

Decrease in serum protein carbonyl groups concentration and maintained hyperhomocysteinemia in patients undergoing bariatric surgery.

BACKGROUND: Human obesity is associated with oxidative stress but the factors contributing to the increase of reactive oxygen species (ROS) production remain unknown. We evaluated the association between serum homocysteine concentration, which may increase ROS production, and serum protein carbonyl groups concentration before and after bariatric surgery. METHODS: Serum protein carbonyl groups and serum homocysteine concentrations, as well as obesity markers, were compared in 18 obese patients before and 6 months after bariatric surgery. Ten healthy individuals with normal body mass index (BMI) served as controls. RESULTS: Before bariatric surgery, obese patients displayed approximately 50% higher serum protein carbonyl groups concentration than control subjects. After surgery, serum protein carbonyl groups concentration decreased and matched values observed in controls. Serum homocysteine concentration was also elevated in obese patients, but in contrast to protein carbonyl groups, did not change after surgery. The body weight, BMI, HOMA-IR, serum leptin, triacylglycerols, LDL/HLD cholesterol ratio, insulin, and glucose concentrations were higher in obese patients as compared to controls, and decreased after bariatric surgery. CONCLUSIONS: This study demonstrates that bariatric surgery has protective effect on oxidative protein damage and improves several laboratory parameters including serum lipid concentration and insulin resistance. However, bariatric surgery does not cause a decrease in serum homocysteine concentration, a risk factor for the development of cardiovascular diseases. Collectively, the results presented in this paper suggest that serum homocysteine concentration is not directly associated with oxidative stress in obese patients after bariatric surgery.

[Recurrent transient ischemic stroke in a young patient with papillary fibroelastoma of the mitral valve and hyperhomocysteinemia. A case report]. / Accidents vasculaires cérébraux ischémiques transitoires récurrents chez une jeune patiente secondaire à un fibroélastome papillaire de la valve mitrale et une hyperhomocystéïnémie. A propos d'une observation.

We describe a case of a young patient admitted for recurrent ischemic stroke caused by a papillary fibroelastoma of the mitral valve and a hyperhomocysteinemia. A papillary fibroelastoma is a benign cardiac tumor which can be associated with serious embolic complications. A moderate plasma level of hyperhomocysteinemia is considered as a risk factor of ischemic stroke. The authors suggest that this association increases the risk of ischemic stroke in their patient. The tumor was surgically removed to avoid new embolic events associated with a vitamin B supplementation. After surgery and acid folic supplementation, no recurrence was observed.

Hyperhomocysteinemia in patients with heart failure referred for cardiac transplantation: preliminary observations.

BACKGROUND: Hyperhomocysteinemia is becoming recognized as a risk factor for cardiovascular disease, yet there are limited data on the prevalence of hyperhomocysteinemia in patients with heart failure. HYPOTHESIS: The purpose of this study was to examine the prevalence of hyperhomocysteinemia in patients with severe heart failure and to correlate serum homocysteine levels with factors that may affect homocysteine metabolism. METHODS: Serum homocysteine levels were measured at the time of cardiac transplant evaluation in 89 consecutive patients with severe heart failure. Homocysteine levels for patients with ischemic cardiomyopathy (ICM) were compared with levels obtained in patients with nonischemic cardiomyopathy (NICM), and homocysteine levels were correlated with demographic and hemodynamic parameters as well as functional status. RESULTS: The mean plasma homocysteine level was increased (14.3 +/- 5.3 micromol/l, normal <9.0 micromol/l) and was equivalent between patients with ICM versus NICM (14.7 +/- 5.8 micromol/l vs. 13.8 +/- 4.5 micromol/l, p = 0.44). Elevated homocysteine levels were seen in a large proportion (89%) of patients and were equally common to patients with NICM (94%) and ICM (85%). Serum homocysteine levels correlated with serum creatinine (r = 0.51, p < 0.001), with a history of diabetes (p = 0.028), and with a history of peripheral vascular disease (p = 0.045). Only 6% of patients were receiving folic acid therapy at the time of transplant referral. CONCLUSION: Hyperhomocysteinemia is common in patients with severe heart failure, and plasma homocysteine levels are uniformly elevated regardless of the etiology of heart failure. Elevated plasma homocysteine levels are likely a consequence of heart failure-related renal insufficiency.

Treatment of hyperhomocysteinemia in pediatric heart transplant recipients.

BACKGROUND: Graft coronary artery vasculopathy is the main cause of late morbidity and mortality in pediatric cardiac allograft recipients. Growing evidence suggests that elevated plasma homocysteine levels are associated with cardiac allograft vasculopathy following heart transplantation. The purpose of this study was to evaluate the effect of vitamin supplementation as a potential strategy for reducing homocysteine levels in pediatric heart transplant recipients and examine creatinine levels as potential determinants of plasma homocysteine concentration after transplantation. METHODS: We studied 27 pediatric heart transplant patients with homocysteine levels higher than normal. All children received vitamin supplementation (vitamin B(12), vitamin E, vitamin A and folic acid). During treatment, levels of homocysteine, vitamins and creatinine were evaluated after 3, 6, 9 and 12 months. RESULTS: We observed a significant homocysteine concentration decrease after treatment at every determination, whereas no significant change occurred for creatinine. Vitamin B(12) serum level increased markedly, whereas folic acid, vitamin E and vitamin A serum levels showed only minor increases. CONCLUSIONS: We observed a significant increase of mean levels of vitamin B(12) and a moderate increase in the other 3 vitamins. We also observed a significant reduction in homocysteine levels, which returned to normal levels for age. In our patients, there was a correlation, before and after treatment, between homocysteine and creatinine levels, but there was no a direct correlation between creatinine serum levels and homocysteine reduction. We conclude that vitamin supplementation reduces and may normalize homocysteine serum level after pediatric heart transplantation.

[Hyperhomocysteinemia and thrombosis: clinical case and literature's review]. / Iperomocisteinemia e trombosi: caso clinico e revisione della letteratura.

Patients with homocystinuria have an increased risk for both venous and artherial thrombosis but it has been found that even moderate increase in homocysteine levels are associated with heightened risk of thromboembolism. The Authors report a case of juvenile vasculopathy in a patient with hyperomocysteinemia. In this case the patient presented both venous and artherial thromboembolism in the absence of the most important risk factors. The opportunity is stressed to perform clinical tests for the correct diagnosis of hyperhomocysteinemia because it is possible, with the use of folic acid and vitamin B12, to correct the elevated levels of homocysteine.

Homocysteine-associated acute mesenteric artery occlusion treated with thrombectomy and bowel resection.

Elevated plasma homocysteine is an acknowledged risk factor for arteriosclerotic occlusive disease, but little clinical evidence is available regarding its role in acute arterial thrombosis in the absence of an underlying lesion. A 45-year old man presented with an acute abdomen. A magnetic resonance arteriogram (MRA) showed occlusion of the superior mesenteric artery. At exploration, necrotic ileum was resected and the superior mesenteric artery was thrombectomized, restoring normal mesenteric flow. The plasma homocysteine level was 98.8 mmol/L, more than eight times the normal level. No embolic source was identified and an MRA and contrast arteriogram showed no residual occlusive disease in the superior mesenteric artery. Additional studies documented pernicious anemia, which was treated with cobalamin (vitamin B12) injections. This case provides further evidence of an association between hyperhomocysteinemia and acute arterial thrombosis. Hyperhomocysteinemia can result from easily correctible vitamin B12, B6, or dietary folate deficiencies. Plasma homocysteine levels should be determined in young individuals with acute arterial thrombosis whenever a hypercoagulable state is suspected.

High prevalence of mild hyperhomocysteinemia in patients with abdominal aortic aneurysm.

PURPOSE: In vitro studies have recently demonstrated that homocysteine interacts with the aortic wall by inducing both elastolysis and endothelial perturbation. The aim of this study was to evaluate homocysteine plasma levels and their relationships with aortic diameter and endothelial damage in patients with abdominal aortic aneurysm. SUBJECTS AND METHODS: Fifty-eight consecutive male patients (mean age, 69.5 +/- 6.6 years; age range, 49-78 years) who underwent abdominal aortic aneurysm surgery were enrolled in the study. Twenty-two of 58 patients had no clinical or instrumental evidence of atherosclerosis. Sixty control subjects were age matched and sex matched with the patients. In all of the subjects, we evaluated total homocysteine and thrombomodulin plasma levels and the distribution of the C677T methylenetetrahydrofolate reductase gene mutation. RESULTS: Hyperhomocysteinemia was found in 26 (48%) of the 58 patients with abdominal aortic aneurysm, and homocysteine plasma levels were significantly higher in patients than in control subjects (15.7 +/- 6.5 micromol/L vs 9.6 +/- 3.9 micromol/L; P <. 0001). In addition, the subgroup of patients with abdominal aortic aneurysm who did not show evidence of atherosclerosis showed homocysteine plasma levels significantly higher than those in the controls (14.8 +/- 6.1 micromol/L vs 9.6 +/- 3.9 micromol/L; P <. 001). A larger aneurysmal size was detected in hyperhomocysteinemic patients than in those with normal homocysteine plasma levels (5.09 +/- 0.84 cm vs 5.79 +/- 1.5 cm; P <.05). The genotype distribution of the C677T methylenetetrahydrofolate reductase mutation was as follows: TT 21%, TC 55%, and CC 24% in the patients; TT 10%, TC 58%, and CC 32% in the controls. Moreover, in patients a significant correlation (P <.005) between homocysteine plasma level and 677TT methylenetetrahydrofolate reductase genotype was found. Thrombomodulin plasma levels were significantly higher (P <.00005) in patients (median, 30 ng/mL; range, 10-164 ng/mL) than in controls (median, 19 ng/mL; range, 13-44 ng/mL), and thrombomodulin levels were significantly higher (P <.005) in hyperhomocysteinemic patients (median, 39.5 ng/mL; range, 15-164 ng/mL) than in normohomocysteinemic patients (median, 27.5 ng/mL; range, 10-85 ng/mL). In addition, in patients with abdominal aortic aneurysm, a direct significant correlation (P <.005) was found between homocysteine and thrombomodulin. CONCLUSIONS: These data indicate an association between the presence of AAA in patients selected for surgical treatment of AAA and elevated homocysteine plasma levels and suggest that homocysteine may induce endothelial perturbation and stimulation in these patients.