Long-term outcomes of enzyme replacement therapy from a large cohort of Korean patients with mucopolysaccharidosis IVA (Morquio A syndrome).

Mucopolysaccharidosis (MPS) IVA (Morquio A syndrome) is an autosomal recessive lysosomal storage disorder caused by a mutation affecting the enzyme -acetylgalactosamine-6-sulfatase (EC 3.1.6.

Risk and clinical characteristics of spinal cord compression across different mucopolysaccharidosis types: A retrospective cohort study.

In patients with mucopolysaccharidosis (MPS), the accumulation of glycosaminoglycans leads to various complications, including spinal cord compression (SCC). Although SCC is a well-known complication in MPS, data comparing its clinical features across different MPS types remain limited. This study aimed to investigate the timing, location, and underlying causes of SCC in MPS, as well as to compare the risk and clinical characteristics by MPS type.

Six-year clinical outcomes of enzyme replacement therapy for perinatal lethal and infantile hypophosphatasia in Korea: Two case reports.

Introduction: Hypophosphatasia (HPP) is a genetic disease caused by loss-of-function mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (ALP). Early diagnosis and treatment of perinatal and infantile HPP are important because of their high mortality rates. Enzyme replacement therapy (ERT) using human recombinant tissue-nonspecific ALP asfotase alfa was introduced in Korea in 2016.

Lead-Free AE Sensor Based on BZT-BCT Ceramics.

In this study, an acoustic emission (AE) sensor was fabricated using lead-free Ba(ZrTi)O-0.5(BaCa)TiO (BZT-BCT) ceramics. The acoustic and electromechanical properties of the AE sensor were determined by the shapes of the piezoelectric ceramics.

Long-Term Antithyroid Drug Treatment of Graves' Disease in Children and Adolescents: A 20-Year Single-Center Experience.

Background/purpose: Graves' disease (GD) is the most common cause of thyrotoxicosis in children and adolescents. There is some debate regarding the optimal treatment and predicting factors of remission or relapse in children and adolescents with GD. In this study, we report a retrospective study of 195 children and adolescents with GD treated at a single tertiary institution in Korea.

Effect of Mass Drug Administration with a Single Dose of Albendazole on Ascaris lumbricoides and Trichuris trichiura Infection among Schoolchildren in Yangon Region, Myanmar.

Soil-transmitted helminths, including Ascaris lumbricoides and Trichuris trichiura, are important intestinal parasites mostly affecting younger people in developing countries. In 2014-2015, we performed mass fecal examinations targeting a total of 2,227 schoolchildren in 3 districts (South Dagon, North Dagon, and Hlaing-thar-yar) of Yangon Region, Myanmar, using the Kato-Katz thick smear technique. The egg positive children were subjected to a mass drug administration (MDA) using a single oral dose of 400 mg albendazole.

Exposure to cold airflow alters skin pH and epidermal filaggrin degradation products in children with atopic dermatitis.

Background: We aimed to evaluate the influence of cold airflow from the air conditioner on skin barrier function and filaggrin degradation products (FDPs) in children with atopic deramtitis (AD).

Methods: In a case-control study, 28 children with AD and 12 normal children without AD were exposed to one of two air conditioner modes (conventional or wind-free) for 2 h. Skin temperature, transepidermal water loss (TEWL), and skin pH were measured on right cheek and forearm at pre- and post-exposure time points.

Revealing of Core Shell Effect on Frequency-Dependent Properties of Bi-based Relaxor/Ferroelectric Ceramic Composites.

In this study, electromechanical characteristics of (1-x) BiNaTiO-xSrTiO (ST26, x = 0.26)/(1-y) BiNaTiO-ySrTiO (ST10, y = 0.1) (matrix/seed) composites were studied.

Midterm outcome of fourth-generation ceramic-on-ceramic bearing surfaces in revision total hip arthroplasty.

Purpose: The purpose of this study is to evaluate the clinical and radiologic outcomes after revision total hip arthroplasty (THA) using fourth-generation ceramic-on-ceramic (CoC) bearing surfaces.

Methods: A total of 52 revision THAs (28 men and 19 women) using the fourth-generation CoC bearing surfaces were retrospectively evaluated. Both acetabular cup and femoral stem were revised in all cases.

Effects of the Brown Seaweed Supplementation on Serum Concentrations of IgG, Triglycerides, and Cholesterol, and Intestinal Microbiota Composition in Rats.

The intestinal microbial communities play critical roles in various aspects of body function of the host. Prebiotics, such as dietary fiber, can affect health of the host by altering the composition of intestinal microbiota. Although brown seaweed is rich in dietary fiber, studies on its prebiotic potential are quite rare.

Fixation Strength of Pedicle and Cortical Lumbar Vertebral Screws after Laminectomy: A Cadaver Study.

Background And Study Aim:  Cortical screws were proposed as an alternative to the traditional pedicle screws. Diverse experimental results support the biomechanical superiority of cortical screws compared to pedicle screws. Laminectomy is often part of multilevel lumbar surgeries.

Diagnosis and Treatment of Inflammatory Joint Disease.

Arthritis damages the cartilage within joints, resulting in degenerative changes, including loss of function and joint instability. Ankylosing spondylitis (AS) is a chronic inflammatory condition affecting the spine and bone-to-tendon attachment area within the sacroiliac joint leading to back pain and progressive spinal stiffness. In the final stages, AS causes hyperkyphosis-a condition closely tied to the human leukocyte antigen-B27 gene.

Management of Blood Loss in Hip Arthroplasty: Korean Hip Society Current Consensus.

The volume of hip arthroplasty is stiffly increasing because of excellent clinical outcomes, however it has not been shown to decrease the incidence of transfusions due to bleeding related to this surgery. This is an important consideration since there are concerns about the side effects and social costs of transfusions. First, anemia should be assessed at least 30 days before elective hip arthroplasty, and if the subject is diagnosed as having anemia, an additional examination of the cause of the anemia should be carried and steps taken to address the anemia.

Acute Delayed or Late Infection of Revision Total Hip Arthroplasty Treated with Debridement/Antibiotic-loaded Cement Beads and Retention of the Prosthesis.

Purpose: The treatment of infected revision total hip arthroplasty (THA) is very challenging due to retained revision prosthesis, poor bone stock and soft tissue condition derived from previous revision surgeries, and comorbidities. The purpose of this study was to investigate the effectiveness and short-term outcomes of aggressive debridement and use of antibiotic-loaded cement beads with retention of the prosthesis for acute delayed or late infection of revision THAs.

Materials And Methods: Ten consecutive patients with symptoms or signs of less than one-week evolution and well-fixed prostheses, were treated with this procedure and a postoperative course of organism-specific antibiotics for a minimum of 6 weeks.

The Evolution of Computer-Assisted Total Hip Arthroplasty and Relevant Applications.

In total hip arthroplasty (THA), the accurate positioning of implants is the key to achieve a good clinical outcome. Computer-assisted orthopaedic surgery (CAOS) has been developed for more accurate positioning of implants during the THA. There are passive, semi-active, and active systems in CAOS for THA.

Greater Trochanteric Reattachment Using the Third-Generation Cable Plate System in Revision Total Hip Arthroplasty.

Background: The purpose of the study is to evaluate the radiologic and functional results of greater trochanteric reattachment using the third-generation cable plate system in revision total hip arthroplasty (THA).

Methods: A total of 47 trochanteric fixations (27 men and 18 women; mean age of 60.2 years) using the third-generation cable plate system in revision THA were retrospectively evaluated.

Revision Total Hip Arthroplasty Using Imageless Navigation With the Concept of Combined Anteversion.

Background: The purpose of the study was to evaluate the implant positions and clinical results of revision total hip arthroplasty (THA) using an imageless navigation with the concept of combined anteversion.

Methods: A total of 40 cementless revision THAs (24 men and 16 women) using an imageless navigation with the concept of combined anteversion were retrospectively evaluated. The concept of combined anteversion was applied in cup positioning based on Widmer's equation (cup anteversion + 0.

Efficacy and Usefulness of New Single-Needle Intensive Granulocyte and Monocyte Adsorptive Apheresis in Active Ulcerative Colitis Patients Without Corticosteroids and Biologics.

Intensive granulocyte and monocyte adsorptive apheresis (GMA) twice weekly is effective and safe for patients with active ulcerative colitis (UC), but the requirement for maintaining two blood access routes is problematic. Here we compared the efficacy and safety of one-route blood access intensive GMA using a single-needle (SN) and conventional two-route blood access intensive GMA using a double-needle (DN) in patients with active UC not undergoing corticosteroid therapy. Among 80 active UC patients, 38 patients received SN intensive GMA and 42 patients received DN intensive GMA.

In vitro and in silico evaluation of transactivation potencies of avian AHR1 and AHR2 by endogenous ligands: Implications for the physiological role of avian AHR2.

Aryl hydrocarbon receptor (AHR) is well conserved from invertebrates to vertebrates, and it mediates the toxic effects of exogenous ligands, including dioxins. Recent studies reported that AHRs activated by endogenous ligands play critical roles in mammalian physiological homeostasis. Avian species possess at least two AHR isoforms (AHR1 and AHR2), which exhibit species- and isoform-specific transactivation potencies to exogenous ligands, whereas mammals possess a single AHR.

Factors Influencing the Spatial Variation of Microplastics on High-Tidal Coastal Beaches in Korea.

The presence and distribution characteristics of microplastics become a big issue due to the adverse effects on marine organisms caused by not only microplastics but any incorporated and/or adsorbed pollutants. Distribution of microplastics (50- to 5000-μm size) was determined for three sandy beaches on an isolated island in a high-tidal costal region to elucidate spatial distributions in relation to beach locations. The abundances of microplastics (n = 21) measured were 56-285,673 (46,334 ± 71,291) particles/m(2) corresponding to the highest level globally.

Delayed infrarenal aortic pseudoaneurysm treated by endovascular stent graft in pyogenic spondylitis.

A 61-year-old male patient with pyogenic spondylodiscitis and epidural and psoas abscesses underwent posterior decompression, debridement, and instrumented fusion, followed by anterior debridement and reconstruction. Sudden onset flank pain was diagnosed 7 weeks postoperatively and was determined to be a pseudoaneurysm located at the aorta inferior to the renal artery and superior to the aortic bifurcation area. An endovascular stent graft was applied to successfully treat the pseudoaneurysm.

Cervical foraminal and discal height after dynamic rotational plating in the cervical discectomy and fusion.

Study Design: This is a retrospective study.

Purpose: To evaluate the effect of the dynamic rotational plate to the intervertebral foraminal and discal height after anterior cervical discectomy and fusion.

Overview Of Literature: There is no report regarding the changes of foraminal and discal height following cervical dynamic rotational plating.