Clinical and biochemical response to neridronate treatment in a patient with osteoporosis-pseudoglioma syndrome (OPPG)

Osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal recessive syndrome characterized by juvenile-onset osteoporosis and ocular abnormalities due to a low-density lipoprotein receptor-related protein 5 (LRP5) gene mutation. Treatment with bisphosphonates, particularly with pamidronate and risedronate, has been reported to be of some efficacy in this condition. We report on a patient with OPPG due to an LRP5 gene mutation, who showed an encouraging response after a 36-month period of neridronate therapy. We report a case of a patient treated with bisphosphonates. Bisphosphonates should be administered in OPPG patients as a first-line therapy during early childhood

Laterality of Stance during Optic Flow Stimulation in Male and Female Young Adults

During self-motion, the spatial and temporal properties of the optic flow input directly influence the body sway. Men and women have anatomical and biomechanical differences that influence the postural control during visual stimulation. Given that recent findings suggest a peculiar role of each leg in the postural control of the two genders, we investigated whether the body sway during optic flow perturbances is lateralized and whether anteroposterior and mediolateral components of specific center of pressure (COP) parameters of the right and left legs differ, reexamining a previous experiment (Raffi et al. (2014)) performed with two, side-by-side, force plates. Experiments were performed on 24 right-handed and right-footed young subjects. We analyzed five measures related to the COP of each foot and global data: anteroposterior and mediolateral range of oscillation, anteroposterior and mediolateral COP velocity, and sway area. Results showed that men consistently had larger COP parameters than women. The values of the COP parameters were correlated between the two feet only in the mediolateral axis of women. These findings suggest that optic flow stimulation causes asymmetry in postural balance and different lateralization of postural controls in men and women

Serum creatine kinase isoenzymes in children with osteogenesis imperfecta

This study evaluates serum creatine kinase isoenzyme activity in children with osteogenesis imperfecta to determine its usefulness as a biochemical marker during treatment with bisphosphonate. The changes of creatine kinase (CK) isoenzyme activity during and after discontinuation therapy were observed. These results could be useful in addressing over-treatment risk prevention. Introduction The brain isoenzyme of creatine kinase (CKbb) is highly expressed in mature osteoclasts during osteoclastogenesis, thus plays an important role in bone resorption. We previously identified high serum CKbb levels in 18 children with osteogenesis imperfect (OI) type 1 treated for 1 year with bisphosphonate (neridronate). In the present study, serum CK isoenzymes were evaluated in the same children with continuous versus discontinued neridronate treatment over a further 2-year follow-up period. Methods This study included 18 children with OI type 1, 12 with continued (group A) and 6 with ceased (group B) neridronate treatment. Auxological data, serum biochemical markers of bone metabolism, bone mineral density z-score, and serum total CK and isoenzyme activities were determined in both groups. Results Serum CKbb was progressively and significantly increased in group A (p < 0.004) but rapidly decreased to undetectable levels in group B. In both groups, the cardiac muscle creatine kinase isoenzyme (CKmb) showed a marked decrease, while serum C-terminal telopeptide (CTx) levels were almost unchanged. Conclusions This study provides evidence of the cumulative effect of neridronate administration in increasing serum CKbb levels and the reversible effect after its discontinuation. This approach could be employed for verifying the usefulness of serum CKbb as a biochemical marker in patients receiving prolonged bisphosphonate treatment. Moreover, the decreased serum CKmb levels suggest a systemic effect of these drugs

Erratum to: Proximal humeral fractures in elderly patients

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Can body mass index influence the fracture zone in the fifth metatarsal base? A retrospective review

Fifth metatarsal base fracture are common in routine orthopaedic practice [1–6]. Lawrence and Botte [7] pro- posed a classification based upon the position of the fracture line (zone 1: tuberosity, zone 2: meta-diaphyseal junction, zone 3: proximal diaphysis). Pathomechani- cally, injury patterns develop in different ways: in zone 1, a traction injury caused by peroneus brevis tendon and the lateral band of the plantar fascia determine an avul- sion fracture of the tuberosity, also called “pseudo-Jones’ “fracture; in zone 2, forced foot adduction and excessive plantar flexion determine a fracture in the metaphyseal- diaphyseal junction, also called Jones’ fracture [8, 9]; in zone 3, acute over-bearing onto the area or chronic overload determine a fracture in the proximal portion of the diaphysis, distal to the intermetatarsal joint [10, 11]. To the best of the Authors’ knowledge, no study has been published to date on the relationship between the value of Body Mass Index (BMI) and the prevalence of fractures in a specific portion of the fifth metatarsal base. The aim of this study was to define the impact of BMI on fifth metatarsal base fractures location according to Lawrence and Botte classification [7]

On-line recognition of supernova neutrino bursts in the LVD detector

In this paper we show the capabilities of the Large Volume Detector (INFN Gran Sasso National Laboratory) to identify a neutrino burst associated to a supernova explosion, in the absence of an "external trigger", e.g., an optical observation. We describe how the detector trigger and event selection have been optimized for this purpose, and we detail the algorithm used for the on-line burst recognition. The on-line sensitivity of the detector is defined and discussed in terms of supernova distance and electron anti-neutrino intensity at the source.Comment: Accepted for pubblication on Astroparticle Physics. 13 pages, 10 figure

First CNGS events detected by LVD

The CERN Neutrino to Gran Sasso (CNGS) project aims to produce a high energy, wide band $\nu_{\mu}$ beam at CERN and send it toward the INFN Gran Sasso National Laboratory (LNGS), 732 km away. Its main goal is the observation of the $\nu_{\tau}$ appearance, through neutrino flavour oscillation. The beam started its operation in August 2006 for about 12 days: a total amount of $7.6~10^{17}$ protons were delivered to the target. The LVD detector, installed in hall A of the LNGS and mainly dedicated to the study of supernova neutrinos, was fully operating during the whole CNGS running time. A total number of 569 events were detected in coincidence with the beam spill time. This is in good agreement with the expected number of events from Montecarlo simulations.Comment: Accepted for publication by the European Physical Journal C ; 7 pages, 11 figure

Can muon-induced backgrounds explain the DAMA data?

We present an accurate simulation of the muon-induced background in the DAMA/LIBRA experiment. Muon sampling underground has been performed using the MUSIC/MUSUN codes and subsequent interactions in the rock around the DAMA/LIBRA detector cavern and the experimental setup including shielding, have been simulated with GEANT4.9.6. In total we simulate the equivalent of 20 years of muon data. We have calculated the total muon-induced neutron flux in the DAMA/LIBRA detector cavern as Φμn = 1.0 ×10-9 cm-2s-1, which is consistent with other simulations. After selecting events which satisfy the DAMA/LIBRA signal criteria, our simulation predicts 3.49 ×10-5 cpd/kg/keV which accounts for less than 0.3% of the DAMA/LIBRA modulation amplitude. We conclude from our work that muon-induced backgrounds are unable to contribute to the observed signal modulation

Enhancing the pharmacodynamic profile of a class of selective COX-2 inhibiting nitric oxide donors

We report herein the development, synthesis, physicochemical and pharmacological characterization of a novel class of pharmacodynamic hybrids that selectively inhibit cyclooxygenase-2 (COX-2) isoform and present suitable nitric oxide releasing properties. The replacement of the ester moiety with the amide group gave access to in vivo more stable and active derivatives that highlighted outstanding pharmacological properties. In particular, the glycine derivative proved to be extremely active in suppressing hyperalgesia and edema. © 2013 Elsevier Ltd. All rights reserved