Epidemiology, Evaluation and Management of Wilson Disease: Review Article

Wilson disease (hepatolenticular degeneration) is a rare autosomal recessive ailment characterized by aberrant copper buildup in the body, with the brain, liver, and cornea being notably affected. Wilson illness is caused by a mutation in the ATP7B gene on chromosome 13, which regulates the protein transporter that excretes excess copper into the bile and out of the body. So far, over 500 mutations have been discovered. The most common treatment for WD is D-penicillamine (D-PCA). Patients with severe spasms, deformities, or dysphonia, as well as those who are allergic to D-PCA, should avoid it. Early Diagnosis is a key factor in saving patient’s live, and thus prober investigation should be done as soon as possible. Family screening is a must when a patient is diagnosed to role out any other patients in the family with the disease and because of the strong genetic factor impacting the disease. early detection is critical for initiating therapy in the early, asymptomatic stages of the disease, rather than when liver decompensation or extensive neurological irreversible harm has already occurred. In this circumstance, the optimum technique is to finish copper investigations in the index patient's first- and second-degree relatives. In the present article we’ll be discussing disease prevalence, etiology and more importantly diagnosis and management.

Red Seaweeds as a Source of Nutrients and Bioactive Compounds: Optimization of the Extraction

The present work aimed to determine the nutritional composition (ash, protein, fat, carbohydrate content and energy value), phenolic compounds, pigments and organic acids content of three typical red algae from the Northwest of Spain: Chondrus crispus, Mastocarpus stellatus, and Gigartina pistillata; as well as their antioxidant and antimicrobial activities. Furthermore, the present work compared two extraction techniques: conventional heat assisted extraction (HAE) and high pressure assisted extraction (HPAE) to maximize the yield and the concentration of target compounds. Different independent variables were considered for the response study. Time (t) and percentage of ethanol of the solvent (S) were chosen for both techniques and temperature (T) and pressure (P) were used for HAE and HPAE, respectively. The experiments were designed following a response surface methodology (RSM) approach. The obtained results showed a similar nutritional composition between algae samples: low-fat content and high content of proteins, carbohydrates and energy. All tested algae showed good antioxidant and antimicrobial properties. Finally, HEA demonstrated to be the most efficient extraction technique. This study confirms the potential of red algae to be part of the human diet as a source of non-animal protein, due to its nutritional content, phenolic profile, pigments concentration and bioactive properties, which proves that HAE is the optimum technique for the extraction maximization.

Optimum technique of carotid artery reconstruction in her atherosclerotic lesion

Purpose of the study. Was to compare the early and long-term results of different variants of the classical KEAE.Materials and methods. In a retrospective study, we collected data on 1.242 patients who trans- ferred KEA from January 2011 to December 2019, using a patch was performed in 239 cases in 1003 — primary suture closure. We used 3 methods of arteriotomy closure with the use of a patch. There were 848 men and 394 women, the average age was 63.16 years, all of them were sympto- matic. Most patients had a transient disorder (49.2%) or ischemic stroke (50.8%). All patients had significant (moderate to severe) carotid stenosis. After the operation 5 (0.4%) patients had ischemic stroke, 1 (0.08%) had n. hypo-glossus damage, 3 (0.24%) had postoperative hematoma. The usual endarterectomy was performed without a shunt in all cases. All arteriotomies were mostly open. Early and late results of this procedure with a period of observation of at least 36 months were ana-lyzed: technical aspects of arteriotomy, features of restoration of the integrity of the vessel, changes in the "geometry" of the vessel, the use of zaplat. We studied the state of hemodynamics and complications in the early (30 days) and late postoperative period, the development of restenosis was compared between groups during the 36 months of observation. Results. When assessing the geometrical changes in the group with the patching, it was noted that the worst results were obtained using the technique with a U-shaped fixation suture on the distal part of the patch and the use of a wide patch. When using a narrow patch geometric changes in the width of the patch are the smallest. The main reason of the frequent geometrical changes in the dis-tal part of the patch was the patch wrapping inside the artery with the formation of a duplicate. The application of the modified technique of patch closure allowed to reduce the number of distortions in the operation area, to avoid the formation of artery stenoses in the place of patch imposition, to prevent changes in the bifurcation geometry of the internal carotid artery. The best results in the application of the primary edge suture were obtained by the method of closing the arteriotomy with the counter edge suture with the precision control of the application. The application of the U-shaped suture along the edges of the arteriotomy allowed avoiding deformations in this place. Con-trol of needle injection by means of straightening and lifting of the wall edge ensured absence of deformations in the suture.Conclusions. The post-CEAE closure technique affects the hemodynamic profile. Sewing patch-ing does not seem to create favorable flow dynamics. Consideration should be given to the elective use of the patch to improve disturbed flows. However, the linear precision suture remains the method of choice for suturing the arteriotomy.

Optimum Techniques for the Conversion of Space Rectangular and Curvilinear Coordinates

Conversion between space rectangular (X, Y, Z) and curvilinear (φ, λ, h) coordinates is an important task in the field of Surveying, geodesy, positioning, navigation, mapping etc. Different techniques which include iterative methods, non-iterative techniques and closed form algebraic methods have been applied over the years to carry out the coordinate conversion. However, the results obtained using these techniques are deficient in one way or the other due to the inherent limitations such as inability to produce results for curvilinear coordinates when the values of X, Y and Z are subsequently or simultaneously equal to zero. Therefore, this study attempts to put forth an optimum coordinate conversion technique between space rectangular and curvilinear coordinates. The data used are coordinates of points which include the space rectangular coordinates and their equivalent curvilinear coordinates. They were observed and processed in Nigeria using Doppler 9 software by African Doppler Survey (ADOS) and they were confirmed to be of first order accuracy and hence of high quality. The data processing involved the design of the optimum techniques equations, coding of the algorithms and necessary computations to obtain results. Analyzing the results obtained, it can be inferred that the designed optimum model has successfully carried out the conversion between space rectangular and curvilinear coordinates. Therefore, the optimum technique model is recommended for use for the conversions from Space rectangular coordinates to Geocentric, Geodetic, Reduced coordinates and vice versa.

In search of sports biomechanics’ holy grail: Can athlete-specific optimum sports techniques be identified?

The development of methods that can identify athlete-specific optimum sports techniques—arguably the holy grail of sports biomechanics—is one of the greatest challenges for researchers in the field. This ‘perspectives article’ critically examines, from a dynamical systems theoretical standpoint, the claim that athlete-specific optimum sports techniques can be identified through biomechanical optimisation modelling. To identify athlete-specific optimum sports techniques, dynamical systems theory suggests that a representative set of organismic constraints, along with their non-linear characteristics, needs to be identified and incorporated into the mathematical model of the athlete. However, whether the athlete will be able to adopt, and reliably reproduce, his/her predicted optimum technique will largely be dependent on his/her intrinsic dynamics. If the attractor valley corresponding to the existing technique is deep, or if the attractor valleys corresponding to the existing technique and the predicted optimum technique are in different topographical regions of the dynamic landscape, technical modifications may be challenging or impossible to reliably implement even after extended practice. The attractor layout defining the intrinsic dynamics of the athlete, therefore, needs to be determined to establish the likelihood of the predicted optimum technique being reliably attainable by the athlete. Given the limited set of organismic constraints typically used in mathematical models of athletes, combined with the methodological challenges associated with mapping the attractor layout of an athlete, it seems unlikely that athlete-specific optimum sports techniques will be identifiable through biomechanical optimisation modelling for the majority of sports skills in the near future.

PECULIARITIES OF INTRAMEDULLARY NAILING IN TREATMENT OF EXTRAARTICULAR PROXIMAL TIBIAL FRACTURES

From the biological and biochemical points of view closed blocking intramedullary osteosynthesis is an optimum technique for the treatment of extraarticular proximal tibial fractures. The disadvantage of the technique is the complexity of reposition achievement and maintenance. Great number of surgical methods and implants with different efficacy and safety has been proposed to solve these problems. The review presents the analysis of literature dedicated to different methods of reposition achievement and maintenance in intramedullary osteosynthesis of extraarticular proximal tibial fractures.

Percutaneous Image-Guided Biopsy of the Spleen

Percutaneous spleen biopsy has a small but important role in the diagnostic approach to splenic lesions. Nonetheless, there remain concerns about the safety of the procedure, limiting its use despite evidence that splenic biopsy performed with optimum technique has comparable diagnostic yield and safety as other solid organ biopsies. To assure appropriate use of percutaneous image-guided spleen biopsy, we discuss the rationale, technique, and outcomes of the procedure.

Peculiarities of intramedullary nailing in treatment of extraarticular proximal tibial fractures

From the biological and biochemical points of view closed blocking intramedullary osteosynthesis is an optimum technique for the treatment of extraarticular proximal tibial fractures. The disadvantage of the technique is the complexity of reposition achievement and maintenance. Great number of surgical methods and implants with different efficacy and safety has been proposed to solve these problems. The review presents the analysis of literature dedicated to different methods of reposition achievement and maintenance in intramedullary osteosynthesis of extraarticular proximal tibial fractures.