Elevated plasma homocysteine (Hcy) level is considered a risk factor for vascular diseases. In recent years, many scientific reports have suggested that hyperhomocystinemia may be associated with an increased risk of retinal vascular occlusive disease (RVOD). The prevalence of elevation of homocysteine in patients with a recent retinal vascular occlusion was compared to a health control group in this study. Forty-nine consecutive patients (22 M; 27 F) (age 26-85 years, mean 69) with diagnosis of retinal vascular occlusion were compared with 71 healthy controls. These patients underwent laboratory evaluation for plasma fasting total homocysteine, activated protein C resistance, protein C, protein S, antithrombin III, and antiphospholipid and anticardiolipin antibodies. The G20210 prothrombin gene mutation (FII G20210A) and Factor V Leiden mutation (FVL) were evaluated. None of these enrolled subjects had other prothrombic risk factors. The health control group consisted of healthy subjects from the general population, with no history or clinical evidence of retinal vascular disease, recruited during the same 2-year period. High fasting homocystinemia (higher than 15 mumol/L) was detected in 24/49 subjects (48.9%) (P < .0005). There was a high prevalence of hyperhomocystinemia: these data suggest an association between RVOD and high fasting homocystinemia. Elevated homocysteine may be an independent risk factor, and its assessment may be important in the investigation, management, and follow-up of patients with RVOD. Further controlled studies are necessary to clarify the exact role of hyperhomocystinemia in RVOD and to evaluate the appropriate therapeutic approach.
Background
Immune tolerance induction (ITI) is the only proven strategy to eradicate factor VIII inhibitors in patients with haemophilia A (HA).
Aim
To identify patients and treatment options with the highest chance of inhibitor eradication by primary ITI.
Patients and methods
In the frame of the Italian ITI Registry, carried out from 1995 to 2015 (last follow‐up 2018), 137 primary ITI courses in severe HA patients (90/137 with poor prognosis) were analysed for predictors of outcome (complete/partial response or failure). Sixty‐six of them (48%) were prospectively evaluated.
Results
ITI was successful in 91/137 patients (66.4%) and 70 (51.1%) achieved complete response within 11 months (median). Historical peak titres ≤200 BU/ml (P = .033), inhibitor titres ≤5 BU/ml at ITI start (P = .001), peak titres ≤100 BU/ml during ITI (P < .001) and missense mutations and small insertions/deletions of FVIII gene (P = .027) predicted complete inhibitor eradication. A score that considers the cumulative number of these variables predicted complete response with positive predictive values up to .81 at ITI start and .91 during ITI, respectively. Patients who had no bleeding (OR, 3.45, 95% CI: 1.4–8.6) nor other adverse events (OR 2.6, 95%CI: 1.3–5.3) during ITI had higher chances of complete response. During the 120‐month follow‐up (median), 2/70 patients who had achieved complete response relapsed (2.9%).
Conclusions
This Registry, with a centralized review of outcomes, homogeneous data collection (half of which prospective) and long‐term follow‐up, provides insights for optimizing ITI, with a rationale for further studies in the currently evolving scenario of inhibitor management in HA patients.
Factor V Leiden (FVL) and G20210 prothrombin (FII G20210A) mutations are risk factors for thromboembolism. In Europe, FVL is more prevalent in the north (7%) than in the south (3%), whereas FII G20210A is more common in the south (3% to 7%) than in the north (2% to 5%). In Italy, the prevalence is 2% to 3% for both. The aim of this study was to assess if these polymorphisms could be more frequent in the south than in the rest of Italy. In 105 blood donors in southern Italy, the prevalence of FVL and FIIG20210A was 9.5% and 5.7%, respectively. These prevalence data are higher when compared with published data. The results of this study are as high as those observed in Greece and the Middle East. The diffusion of FVL and FII G20210A in the Mediterranean, consequent to Phoenician and Greek colonization, could be a reason for the high prevalence observed.
Objective: To compare the effectiveness of various phototherapy systems in lowering serum bilirubin levels in preterm infants. Methods: This randomized clinical trial enrolled 140 preterm infants with gestational age ≤30 weeks and presenting nonhemolytic hyperbilirubinemia. When total serum bilirubin level reached 6.0 mg/dl (102.6 µmol/l), eligible infants were randomly assigned to four study groups: conventional, fiberoptic Wallaby, fiberoptic Biliblanket, and combined phototherapy. Efficacy was assessed by comparing highest serum bilirubin levels, duration of treatment, and number of infants requiring exchange transfusion. Results: Our results confirm that fiberoptic phototherapy, both Wallaby and Biliblanket, had the same effectiveness of conventional phototherapy. The best results have been obtained using combined phototherapy, which allowed to reach lower serum bilirubin levels, a shorter duration of treatment and a significant reduction of exchange transfusions. Conclusion: Our data suggest that combined phototherapy should be the method of choice in treating hyperbilirubinemia in very preterm infants.
Background: Increased activity of the renin-angiotensin-aldosterone system (RAAS) has been reported in the neonatal period. Until now, it has been demonstrated that the RAAS of healthy neonates responds to acute furosemide challenge while no data concerning the responsiveness of RAAS in extremely low birth weight (ELBW) infants are available. Objective: To assess urinary aldosterone excretion (UAE) and renal function in ELBW infants who received diuretics for the purpose of reducing the incidence of chronic lung disease (CLD). Methods: Infants with birth weights ≤1,000 g, at high risk to develop CLD, were studied in a prospective observational study. UAE and renal function were investigated before and after administration of furosemide given in a single dose of 2 mg/kg. Results: UAE and renal function were evaluated in 20 ELBW infants. Diuretic administration resulted in a significant rise in UAE and urinary sodium, potassium and chloride excretion. No change occurred in creatinine clearance, while urine volume increased significantly. Conclusions: ELBW infants respond to acute furosemide challenge by increasing urine volume, urinary electrolytes and UAE.
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