Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival

OBJECTIVE: To assess the rate of progression of pulmonary hypertension (PHT) in systemic sclerosis (SSc) and its bearing on mortality. METHODS: A retrospective record review of 930 patients with SSc attending a specialist centre was carried out. Those at risk for both idiopathic and secondary PHT were assessed by serial Doppler echocardiography. Mortality data were reviewed. RESULTS: The cumulative prevalence of PHT was 13%. Pressures remained static in most cases. The mortality among those with a single pressure reading of 30 mmHg or higher was 20% at 20 months. An increased mortality risk was associated with high initial pressures and rising pressures. Rapid pressure rises occurred more frequently in limited than in diffuse SSC. CONCLUSIONS: The prevalence of PHT in SSc is high and the detection of PHT at any time in the disease course is associated with substantial mortality. These results demonstrate the value of echocardiographic screening for PHT in all patients with SSC.     

Genotyping of human parechoviruses in Iranian young children with aseptic meningitis and sepsis-like illness

Human parechoviruses (HPeV) are classified into 14 genotypes. HPeV1 and HPeV2 are the most prevalent genotypes in young children, which have been associated with mild to severe diseases. This study was conducted to investigate the involvement of HPeVs in aseptic meningitis and sepsis-like illness in Iran. Viral RNA was extracted from 148 cerebrospinal fluid samples from children <8 years old with primary diagnosis of aseptic meningitis and/or sepsis-like illness. Specific HPeV, HEV real-time PCR and HPeV typing were done to identify the infection rate of these viruses. HPeV and HEV were detected in 64 (43.24 %), 31 (20.94 %) of 148 patients with 10 (6.75 %) coinfection. VP1/VP3 junction region was successfully sequenced from 12 of the HPeV-positive specimens, and all of them were identified as HPeV1. HPeV was more prevalent than HEV in both aseptic meningitis and sepsis-like illness, so further studies are needed to understand the disease burden of HPeV infections, and clinical manifestations especially in specific illnesses of possible viral etiology. Direct detection of these viruses leads to reduce hospitalization and use of antibiotic, which are often followed by other complications in neonates and young children.     

A novel PEGylated interferon beta-1a for multiple sclerosis: safety, pharmacology, and biology

This study clinically evaluated a novel PEGylated form of interferon beta-1a (PEG-IFN beta-1a), a potential first-line treatment for relapsing multiple sclerosis, in healthy volunteers. Two randomized, blinded phase I studies were conducted: a single-dose study (n = 60) comparing subcutaneous or intramuscular PEG-IFN beta-1a (63, 125, or 188 μg) with intramuscular unmodified IFN beta-1a 30 μg and a multiple-dose study (n = 69) comparing subcutaneous PEG-IFN beta-1a dosed once every 2 or 4 weeks with placebo. Assessments included pharmacokinetic and pharmacodynamic (serum neopterin and 2',5'-OAS) measures, exploratory immune assessments, safety, and tolerability. A dose-proportional increase in PEG-IFN beta-1a exposure was observed, with a 4-fold greater exposure at 63 μg (6 million international units [MIU]) of PEG-IFN beta-1a than with 30 μg (6 MIU) intramuscular unmodified IFN beta-1a. Increases in neopterin and 2',5'-OAS levels and changes in T helper cell pathway gene expression and lymphocyte subsets were greater and more sustained with PEG-IFN beta-1a than with unmodified IFN beta-1a. PEG-IFN beta-1a was well tolerated, with only transient reductions in absolute neutrophils and some lymphocytes. Flu-like symptoms were a commonly reported adverse event. These data support the continued clinical development of PEG-IFN beta-1a as a potentially effective treatment for patients with relapsing multiple sclerosis.     

Morphological and topographical anatomy of nutrient foramina in the lower limb long bones and its clinical importance

Background

Knowledge regarding nutrient foramina of bones is useful in surgical procedures such as microvascular bone transfer in order to preserve the circulation. The objective of the present study was to study the morphology and topography of nutrient foramina and to determine the foraminal index of the lower limb long bones to provide detailed data on such features.

Method

The study comprised examination of 206 lower limb long bones which included femora, tibiae and fibulae. The nutrient foramina were identified analysed macroscopically and the foramen index calculated. Each bone was divided into five parts and topographical analysis was performed on each section.

Results

Femora had single nutrient foramen in 47.7% of the cases, double foramen in 44.2% of the cases, triple in 3.5% of the cases and an absence of foramen in 4.6%. In the case of tibiae, 98.6% showed single foramen and in 1.4% of the cases, the foramen was absent. With respect to fibulae, 90.2% had single foramen and foramen was absent in 9.8%. The mean foraminal index was 38.9 for the femora, 32.5 for tibiae and 49.2 for fibulae. The majority (51.3%) of the foramina in the femora were located at the 2/5^th part, 98.3% of the tibiae foramina at the 2/5^th part and 60% of the fibulae at the 3/5^th part.

Conclusion

The study provides information on the morphology and topography of nutrient foramina in lower limb long bones. The double foramina were more common in femur and rare in the tibia and fibula. The foramina of the femur and tibia were commonly observed at their upper part, whereas in the fibula they were present on the lower part. This knowledge of the nutrient foramina has to be kept in mind during surgical procedures.