Pediatric patients with leukocyte adhesion deficiency type-I (LAD-I) experience severe and recurrent lifethreatening bacterial infections with failure of pus formation and delayed wound healing. LAD-I is a rare inherited disease caused by mutation in the leukocyte CD18 integrin expression, resulting in defective adherence and migration of leukocytes, in particular neutrophilic granulocytes through the intravascular space. Hematopoietic SCT is the only curative treatment option available to patients with LAD-I. Since 2007, in a prospective trial, reduced-intensity conditioning regimen have been developed for 10 consecutive patients with LAD-I who were referred to our center. Based upon available data, it is the first time that such a number of patients affected by LAD-I have been treated with this regimen. This study attempts to show that reduced-intensity regimen leads to a favorable result in LAD-I patients even in those who have experienced comorbid complications. Following transplantation, some patients develop mixed chimerism, however, in our study mixed chimerism was not followed by transplant rejection.

Background The Zimmer Trabecular Metal Total Ankle Replacement (Zimmer TM TAR) is a recent ankle arthroplasty approved for use in the United States and Europe. Many of the studies reporting the results of this implant are provided by surgeons involved at least in the initial design of the implant under study. The aim of this study is to describe the early clinical and radiological outcomes in patients who underwent this procedure performed by non-designer surgeons. Methods A total of thirty consecutive patients underwent total ankle replacement with a Zimmer TM TAR surgery between July 2013 to January 2016.All clinical assessments were collected pre- and post-operatively with minimum follow-up of 12 months for each patient using the American Orthopedic Foot and Ankle (AOFAS) score, the Foot Function Index (FFI) and a visual analogue scale (VAS) for pain. Radiographic outcomes included ankle orientation assessed with angle “α”,“β” and “γ” according to Wood. Furthermore, the anteroposterior offset ratio was measured in weight-bearing lateral ankle radiographs at the last follow-up. Results The mean preoperative FFI-pain (FFI-P) value was 53.67, the FFI-disability (FFI-D) was 64.19. At the last follow-up visit, the FFI-P and FFI-D was 16.95 and 20.76 respectively ( p <0.01 for the both scales). Preoperatively, the mean VAS for pain and AOFAS score was 7.81 and 40.95 respectively, and at the last follow-up 2.29 and 86.38 ( p <0.01 for the both scales). The mean angle calculated using Wood and Deakin’s method were “α”= 89.02°, “β”= 85.11 and “γ”= 27.54 post-operatively. At the last follow-up the same values were respectively 89.43, 85.18 and 29.94. At the last follow-up, the mean offset ratio was 0.06 (range 0.003/-0.17). Conclusions These early results show high levels of patient satisfaction, and we are encouraged to continue with lateral approach total ankle arthroplasty.

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This paper proposes an efficient numerical method in the context of variational formulation and on the basis of Rayleigh-Ritz technique to address the free vibration problem of laminated composite conical shells. To this end, the energy functional of Hamilton's principle is written in a quadratic form using matrix relations first. Displacements are then approximated via a linear combination of base functions, by which the number of final unknowns reduces. Afterthat, the strain tensor is discretized by means of matrix differential quadrature (DQ) operators. In the next step, using Taylor series and DQ rules, a matrix integral operator is constructed which is embedded into the stiffness matrix so as to discretize the quadratic representation of energy functional. Finally, the reduced form of mass and stiffness matrices are readily obtained from the aforementioned discretized functional. To obtain the natural frequencies of conical shell, hybrid harmonic-beam base functions are employed as modal displacement functions. The accuracy of the present numerical method is examined by comparing its results with those from the published literature. It is revealed that the method is capable of accurately solving the problem with a little computational effort and ease of implementation.