Assessment of respiratory involvement in children with mucoplysaccharidosis using pulmonary function tests

Background: Mucopolysaccharidosis (MPS) are classified into seven clinical types based on eleven known lysosomal enzyme deficiencies of glycosaminoglycan (GAG) metabolism. Respiratory involvement seen in most MPS types includes recurrent respiratory infections, upper and lower airway obstruction, tracheomalacia, restrictive lung disease, and sleep disturbances.Aim of the study: To delineate the pattern of respiratory compromise and pulmonary function abnormalities in MPS patients.Methods: This is a cross section observational study conducted on 30 patients recruited from the Neurometabolic Clinic, Children’s Hospital, Cairo University over a period of 18 months. All patients were screened first by the quantitative determination of GAGs in urine, and diagnosis was confirmed by unidimensional electrophoresis for GAGs in urine and/or specific enzymatic assay in blood leucocytes. Infant pulmonary functions (IPFT) were done in twenty-two patients (<3 years of age), while 8 cases performed impulse oscillometry (IOS) test (3–6 years of age).Results: Ages at diagnosis ranged from 1 to 9 years with a median of 2.3 years. Male to female ratio was 4:1. Consanguinity was observed in 53.3% whereas similar family condition was present in 40% of cases. Lumbar kyphosis was detected in 60% of cases, while scoliosis was detected in 46.7%. Results of pulmonary functions were mainly obstructive in 20 (66.6%) cases; however, combined obstructive and restrictive were detected in only 6 (20%) of cases. Data showed no association between the presence of scoliosis or the presence of organomegaly and the pattern of pulmonary function abnormalities.Conclusions: Evaluation and follow up of patients with MPS using pulmonary function tests are essential to detect early involvement of respiratory system and hence start treatment for respiratory complications early in the course of the disease

The femoral insertions of the anteromedial and posterolateral bundles of the anterior cruciate ligament: a radiographic evaluation

The aim of this radiographic study was to visualize the femoral insertion sites of the anteromedial (AM) and posterolateral (PL) bundle of the anterior cruciate ligament (ACL) on lateral radiographs in different angles of knee flexion to gain better understanding for arthroscopic femoral tunnel placement in ACL double bundle reconstruction. Four fresh cadaveric knees with an intact ACL were dissected to isolate the AM and PL bundle of the ACL. We obtained lateral radiographs of each knee over the range of 0°–90° flexion in 30° increments after painting the bundles with a radiopaque tantalum powder. The center of the radiographically marked femoral insertion was defined for each bundle on the lateral roentgenogram. We analyzed the relationship of knee flexion and the projection of the relative position of the femoral insertion sites of both bundles of the ACL on the lateral roentgenogram. The centre of the PL bundle visualized more anterior and distal than the centre of the AM bundle with the knee held in 90° flexion. The centers of the AM and PL bundle were horizontally aligned when the knee was flexed over 90°. The resulting images allow a radiographic description of the femoral insertion sites of both bundles in different angles of knee flexion. It is essential to be aware of the degree of knee flexion when drilling the femoral tunnels

A hierarchical model for the cash transfer system design problem

This paper presents a hierarchical model that incorporates strategic, tactical, and operational decisions of cash transfer management system of a bank. The aim of the model is to decide on the location of cash management centers, the number and routes of vehicles, and the cash inventory management policies to minimize the cost of owning and operating a cash transfer system while maintaining a pre-defined service level. Owing to the difficulty of finding optimal decisions in such integrated models, an iterative solution approach is proposed in which strategic, tactical, and operational problems are solved separately via a feedback mechanism. Numerical results show that such an approach is quite effective in reaching at greatly improved solutions with just a few iterations, making it a very promising approach for similar models

Description of the attachment geometry of the anteromedial and posterolateral bundles of the ACL from arthroscopic perspective for anatomical tunnel placement

The anterior cruciate ligament (ACL) consists of an anteromedial bundle (AMB) and a posterolateral bundle (PLB). A reconstruction restoring the functional two-bundled nature should be able to approximate normal ACL function better than the most commonly used single-bundle reconstructions. Accurate tunnel positioning is important, but difficult. The purpose of this study was to provide a geometric description of the centre of the attachments relative to arthroscopically visible landmarks. The AMB and PLB attachment sites in 35 dissected cadaver knees were measured with a 3D system, as were anatomical landmarks of femur and tibia. At the femur, the mean ACL centre is positioned 7.9 ± 1.4 mm (mean ± 1 SD) shallow, along the notch roof, from the most lateral over-the-top position at the posterior edge of the intercondylar notch and from that point 4.0 ± 1.3 mm from the notch roof, low on the surface of the lateral condyle wall. The mean AMB centre is at 7.2 ± 1.8 and 1.4 ± 1.7 mm, and the mean PLB centre at 8.8 ± 1.6 and 6.7 ± 2.0 mm. At the tibia, the mean ACL centre is positioned 5.1 ± 1.7 mm lateral of the medial tibial spine and from that point 9.8 ± 2.1 mm anterior. The mean AMB centre is at 3.0 ± 1.6 and 9.4 ± 2.2 mm, and the mean PLB centre at 7.2 ± 1.8 and 10.1 ± 2.1 mm. The ACL attachment geometry is well defined relative to arthroscopically visible landmarks with respect to the AMB and PLB. With simple guidelines for the surgeon, the attachments centres can be found during arthroscopic single-bundle or double-bundle reconstructions

Compressed representation of a partially defined integer function over multiple arguments

In OLAP (OnLine Analitical Processing) data are analysed in an n-dimensional cube. The cube may be represented as a partially defined function over n arguments. Considering that often the function is not defined everywhere, we ask: is there a known way of representing the function or the points in which it is defined, in a more compact manner than the trivial one

Performance of the CMS Cathode Strip Chambers with Cosmic Rays

The Cathode Strip Chambers (CSCs) constitute the primary muon tracking device in the CMS endcaps. Their performance has been evaluated using data taken during a cosmic ray run in fall 2008. Measured noise levels are low, with the number of noisy channels well below 1%. Coordinate resolution was measured for all types of chambers, and fall in the range 47 microns to 243 microns. The efficiencies for local charged track triggers, for hit and for segments reconstruction were measured, and are above 99%. The timing resolution per layer is approximately 5 ns

A review of bronchiolitis obliterans syndrome and therapeutic strategies

Lung transplantation is an important treatment option for patients with advanced lung disease. Survival rates for lung transplant recipients have improved; however, the major obstacle limiting better survival is bronchiolitis obliterans syndrome (BOS). In the last decade, survival after lung retransplantation has improved for transplant recipients with BOS. This manuscript reviews BOS along with the current therapeutic strategies, including recent outcomes for lung retransplantation