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1.
Pediatr Radiol ; 2024 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-38772925

RESUMO

BACKGROUND: Non-invasive imaging modalities are warranted for diagnosing and monitoring veno-occlusive disease because early diagnosis and treatment improve the prognosis. OBJECTIVE: To evaluate the usefulness of liver shear wave elastography (SWE) and shear wave dispersion (SWD) imaging in diagnosing and monitoring veno-occlusive disease in pediatric patients. MATERIALS AND METHODS: We conducted a prospective cohort study at a single tertiary hospital from March 2021 to April 2022. The study protocol included four ultrasound (US) sessions: a baseline US and three follow-up US after hematopoietic stem cell transplantation. Clinical criteria, including the European Society for Blood and Marrow Transplantation criteria, were used to diagnose veno-occlusive disease. We compared clinical factors and US parameters between the veno-occlusive disease and non-veno-occlusive disease groups. The diagnostic performance of US parameters for veno-occlusive disease was assessed by plotting receiver operating characteristic (ROC) curves. We describe temporal changes in US parameters before and after veno-occlusive disease diagnosis. RESULTS: Among the 38 participants (mean age 10.7 years), eight developed veno-occlusive disease occurring 17.0 ± 5.2 days after hematopoietic stem cell transplantation. Liver stiffness, as measured by SWE (15.0 ± 6.2 kPa vs. 5.8 ± 1.8 kPa; P<0.001), and viscosity, as assessed with SWD (17.7 ± 3.1 m/s/kHz vs. 14.3 ± 2.8 m/s/kHz; P=0.015), were significantly higher in the veno-occlusive disease group compared to the non-veno-occlusive disease group at the time of diagnosis. Liver stiffness demonstrated the highest area under the ROC (AUROC) curves at 0.960, with an optimal predictive value of >6.5 kPa, resulting in sensitivity and specificity of 100% and 83.3%, respectively. Viscosity demonstrated an AUROC of 0.783, with an optimal cutoff value of 13.9 m/s/kHz for predicting veno-occlusive disease, with a sensitivity of 100% and specificity of 53.3%, respectively. Liver stiffness increased with disease severity and decreased during post-treatment follow-up. CONCLUSION: SWE may be a promising technique for early diagnosis and severity prediction of veno-occlusive disease. Furthermore, liver viscosity assessed by SWD may serve as an additional marker of veno-occlusive disease.

3.
World Neurosurg ; 139: e286-e292, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32294567

RESUMO

OBJECTIVE: To compare the radiologic union rates between autogenous iliac crest bone graft (ICBG) and local bone graft in 1- to 3-level lumbar fusion. METHODS: We reviewed 178 consecutive patients who underwent 1- to 3-level lumbar fusion surgery because of lumbar spinal stenosis. Fusion status of the anterior or posterior column was evaluated by plain radiographs obtained at 24 months postoperatively. If at least either the anterior or posterior column was fused, that segment was regarded as having achieved fusion and was termed segment union. The definition of overall union was achieving union of all segments in a single patient. RESULTS: For each ICBG group and local bone graft group, fusion rate of the anterior and posterior column, and rate of the segments and overall union at postoperative 2 years were not different between the groups, regardless of surgery level. In the overall union rate according to the fusion level, the ICBG group showed constant overall fusion rate according to the fusion level (i.e., 96.9%, 96.9%, and 93.1% for 1-, 2-, and 3-level fusion), but tended to decrease with increasing level in the local bone graft group (100%, 95.8%, and 85.7% for 1-, 2-, and 3-level fusion, respectively) without statistically significant differences. CONCLUSIONS: The union rate of 3-level fusion was not inferior to those of 1- or 2-level fusion in both ICBG and local bone graft patients. Local bone graft could be regarded as an adequate option for not only 1- or 2-level lumbar fusion but also 3-level lumbar fusion surgery.


Assuntos
Transplante Ósseo/métodos , Fusão Vertebral/métodos , Estenose Espinal/cirurgia , Idoso , Autoenxertos , Feminino , Humanos , Ílio/transplante , Vértebras Lombares , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Coluna Vertebral , Transplante Autólogo/métodos , Resultado do Tratamento
4.
J Bone Metab ; 26(4): 263-270, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31832392

RESUMO

BACKGROUND: To investigate the characteristics of patients with osteoporotic fractures (OF) who visited the emergency room, we analyzed the frequency and distribution of primary and secondary OF. METHODS: From March 2015 to April 2017, 406 patients with OF were assigned to wrist (W; n=132), spine (S; n=78), and hip (H; n=196) according to the site. All subjects were classified as having primary fracture or secondary fracture. Age, fracture site, the risk of future fracture using Fracture Risk Assessment Tool (FRAX) were compared. RESULTS: The mean age at fracture site was significantly different among the 3 groups, groups W (66.57±10.03), S (73.50±9.07), and H (78.50±7.72). The most common site of OF were in the order of wrist, spine, and hip with the increase of age. The FRAX scores significantly increased (P<0.001) in the order of W (8.35±5.67), S (10.74±6.99), and H (13.88±7.98) in total patient, and same in primary fracture group (W, 7.50±4.18; S, 9.76±5.91; H, 11.93±6.61; P<0.001). The main site of prior fractures in secondary fracture was same lesion, which means that the prior fracture of secondary wrist fracture was wrist fracture such as spine for spine, hip for hip. CONCLUSIONS: We determined that as age increasing, the major sites of OF were different and FRAX scores increased. The most common site for secondary fracture was the same one. Hence, the risk of subsequent fracture in same site should be noted after patient suffered OF.

5.
Int J Mol Sci ; 18(12)2017 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-29194391

RESUMO

Cardiofaciocutaneous (CFC) syndrome is a rare genetic disorder caused by mutations in the extracellular signal-regulated kinase (ERK) signaling. However, little is known about how aberrant ERK signaling is associated with the defective bone development manifested in most CFC syndrome patients. In this study, induced pluripotent stem cells (iPSCs) were generated from dermal fibroblasts of a CFC syndrome patient having rapidly accelerated fibrosarcoma kinase B (BRAF) gain-of-function mutation. CFC-iPSCs were differentiated into mesenchymal stem cells (CFC-MSCs) and further induced to osteoblasts in vitro. The osteogenic defects of CFC-MSCs were revealed by alkaline phosphatase activity assay, mineralization assay, quantitative real-time polymerase chain reaction (qRT-PCR), and western blotting. Osteogenesis of CFC-MSCs was attenuated compared to wild-type (WT)-MSCs. In addition to activated ERK signaling, increased p-SMAD2 and decreased p-SMAD1 were observed in CFC-MSCs during osteogenesis. The defective osteogenesis of CFC-MSCs was rescued by inhibition of ERK signaling and SMAD2 signaling or activation of SMAD1 signaling. Importantly, activation of ERK signaling and SMAD2 signaling or inhibition of SMAD1 signaling recapitulated the impaired osteogenesis in WT-MSCs. Our findings indicate that SMAD2 signaling and SMAD1 signaling as well as ERK signaling are responsible for defective early bone development in CFC syndrome, providing a novel insight on the pathological mechanism and therapeutic targets.


Assuntos
Displasia Ectodérmica/patologia , Insuficiência de Crescimento/patologia , Cardiopatias Congênitas/patologia , Células-Tronco Pluripotentes Induzidas/citologia , Células-Tronco Mesenquimais/citologia , Osteoblastos/citologia , Proteínas Proto-Oncogênicas B-raf/genética , Animais , Diferenciação Celular , Linhagem Celular , Displasia Ectodérmica/genética , Displasia Ectodérmica/metabolismo , MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Fácies , Insuficiência de Crescimento/genética , Insuficiência de Crescimento/metabolismo , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/metabolismo , Humanos , Células-Tronco Pluripotentes Induzidas/metabolismo , Células-Tronco Mesenquimais/metabolismo , Camundongos , Mutação , Osteoblastos/metabolismo , Osteogênese , Fosforilação , Transdução de Sinais , Proteína Smad1/metabolismo , Proteína Smad2/metabolismo
6.
J Adv Prosthodont ; 9(2): 130-137, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28435623

RESUMO

PURPOSE: The purpose of this study was to evaluate the effect of nano-structured alumina surface coating on shear bond strength between Y-TZP ceramic and various dual-cured resin cements. MATERIALS AND METHODS: A total of 90 disk-shaped zirconia specimens (HASS CO., Gangneung, Korea) were divided into three groups by surface treatment method: (1) airborne particle abrasion, (2) tribochemicalsilica coating, and (3) nano-structured alumina coating. Each group was categorized into three subgroups of ten specimens and bonded with three different types of dual-cured resin cements. After thermocycling, shear bond strength was measured and failure modes were observed through FE-SEM. Two-way ANOVA and the Tukey's HSD test were performed to determine the effects of surface treatment method and type of cement on bond strength (P<.05). To confirm the correlation of surface treatment and failure mode, the Chi-square test was used. RESULTS: Groups treated with the nanostructured alumina coating showed significantly higher shear bond strength compared to other groups treated with airborne particle abrasion or tribochemical silica coating. Clearfil SA Luting showed a significantly higher shear bond strength compared to RelyX ARC and RelyX Unicem. The cohesive failure mode was observed to be dominant in the groups treated with nano-structured alumina coating, while the adhesive failure mode was prevalent in the groups treated with either airborne particle abrasion or tribochemical silica coating. CONCLUSION: Nano-structured alumina coating is an effective zirconia surface treatment method for enhancing the bond strength between Y-TZP ceramic and various dual-cured resin cements.

7.
Sci Rep ; 7: 45693, 2017 04 10.
Artigo em Inglês | MEDLINE | ID: mdl-28393871

RESUMO

Overconsumption of sugar-sweetened beverages (SSBs) is known to be a key contributor to the obesity epidemic; however, its effects on behavioral changes are yet to be fully studied. In the present study, we examined the long-term effects of SSB on social aggression in mice. Three-week-old weaned mice started to drink either a 30 w/v% sucrose solution (S30), plain water (CT), or an aspartame solution with sweetness equivalent to the sucrose solution (A30) and continued to drink until they were 11-week-old adults. Aggressive behaviors were assessed by the resident-intruder test. We found that SSB significantly promoted social aggression, accompanied by heightened serum corticosterone and reduced body weight. To understand the underlying mechanism, we performed transcriptome analyses of brain. The profiles of mice on S30 were dramatically different from those on CT or A30. Transcriptional networks related to immunological function were significantly dysregulated by SSB. FACS analysis of mice on S30 revealed increased numbers of inflammatory cells in peripheral blood. Interestingly, the artificial sweetener failed to mimic the effects of sugar on social aggression and inflammatory responses. These results demonstrate that SSB promotes aggressive behaviors and provide evidence that sugar reduction strategies may be useful in efforts to prevent social aggression.


Assuntos
Agressão , Bebidas/efeitos adversos , Encéfalo/patologia , Dieta/métodos , Açúcares/efeitos adversos , Edulcorantes/efeitos adversos , Animais , Comportamento Animal , Peso Corporal , Corticosterona/sangue , Perfilação da Expressão Gênica , Camundongos , Açúcares/administração & dosagem , Edulcorantes/administração & dosagem
8.
J Cheminform ; 9: 2, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28184254

RESUMO

BACKGROUND: Natural products have increasingly attracted much attention as a valuable resource for the development of anticancer medicines due to the structural novelty and good bioavailability. This necessitates a comprehensive database for the natural products and the fractional extracts whose anticancer activities have been verified. DESCRIPTION: NPCARE (http://silver.sejong.ac.kr/npcare) is a publicly accessible online database of natural products and fractional extracts for cancer regulation. At NPCARE, one can explore 6578 natural compounds and 2566 fractional extracts isolated from 1952 distinct biological species including plants, marine organisms, fungi, and bacteria whose anticancer activities were validated with 1107 cell lines for 34 cancer types. Each entry in NPCARE is annotated with the cancer type, genus and species names of the biological resource, the cell line used for demonstrating the anticancer activity, PubChem ID, and a wealth of information about the target gene or protein. Besides the augmentation of plant entries up to 743 genus and 197 families, NPCARE is further enriched with the natural products and the fractional extracts of diverse non-traditional biological resources. CONCLUSIONS: NPCARE is anticipated to serve as a dominant gateway for the discovery of new anticancer medicines due to the inclusion of a large number of the fractional extracts as well as the natural compounds isolated from a variety of biological resources.

9.
Pediatr Int ; 59(3): 271-279, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27589674

RESUMO

BACKGROUND: Management of congenital heart disease (CHD) in developing countries is challenging because of limited access to health-care facilities and socioeconomic limitations. The aim of this study was to describe the recent experience with CHD at Children's Hospital, Vientiane, Laos, the only pediatric referral hospital in the country. METHODS: From July 2013 to November 2015, 1009 echocardiograms were carried out in 797 individuals who visited Children's Hospital, in whom CHD was identified in 213. Demographic characteristics, echocardiography and age at first CHD diagnosis on echocardiogram were compared by residential area. RESULTS: Among the 213 patients, the most frequent anomalies, in descending order, were ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Moderate or severe CHD requiring surgery or intervention was detected in 137 patients; median age at initial diagnosis was 6.0 months (IQR, 1.5-29.8). Among those with moderate or severe CHD, 89 patients were from rural areas and 48 from the capital area. The proportion of patients from rural areas older than 1 and 3 years at diagnosis was 46.1% and 32.6%, respectively. In contrast, patients from the capital area were diagnosed at a significantly earlier age: 16.7% at age >1 year and 6.2% at age >3 years (both P < 0.001 vs rural areas). CONCLUSIONS: Diagnosis of CHD requiring surgery is delayed in Laos, especially in rural areas. Better education and training of local medical staff, and greater access to medical advice are required to improve CHD care in Laos.


Assuntos
Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Disparidades em Assistência à Saúde/estatística & dados numéricos , Cardiopatias Congênitas , Hospitais Pediátricos , Criança , Pré-Escolar , Diagnóstico Tardio/estatística & dados numéricos , Países em Desenvolvimento , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Laos/epidemiologia , Masculino , Encaminhamento e Consulta/estatística & dados numéricos , Características de Residência , Saúde da População Rural/estatística & dados numéricos , Resultado do Tratamento , Saúde da População Urbana/estatística & dados numéricos
10.
Korean J Food Sci Anim Resour ; 36(3): 319-25, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27433102

RESUMO

This study was conducted to determine the residual amount of veterinary drugs such as meloxicam, flunixin, and tulathromycin in animal products (beef, pork, horsemeat, and milk). Veterinary drugs have been widely used in the rearing of livestock to prevent and treat diseases. A total of 152 samples were purchased from markets located in major Korean cities (Seoul, Busan, Incheon, Daegu, Daejeon, Gwangju, Ulsan and Jeju), including Jeju. Veterinary drugs were analyzed by liquid chromatography-tandem mass spectrometry according to the Korean Food Standards Code. The resulting data, which are located within 70-120% of recovery range and less than 20% of relative standard deviations, are in compliance with the criteria of CODEX. A total of five veterinary drugs were detected in 152 samples, giving a detection rate of approximately 3.3%; and no food source violated the guideline values. Our result indicated that most of the veterinary drug residues in animal products were below the maximum residue limits specified in Korea.

11.
Stem Cells Dev ; 25(8): 636-47, 2016 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-26914390

RESUMO

Citrin deficiency (CD) is a recessive genetic disorder caused by mutations in the citrin gene SLC25A13. CD causes various symptoms related to nutrient metabolism such as urea cycle failure, abnormal amino acid levels, and fatty liver. To understand the pathophysiology of CD, the molecular phenotypes were investigated using induced pluripotent stem cells derived from fibroblasts of CD patient (CD-iPSCs). In this study, we demonstrate that aberrant mitochondrial ß-oxidation may lead to fatty liver in CD patients. CD-iPSCs normally differentiated into hepatocytes, similar to wild-type iPSCs (WT-iPSCs). However, hepatocytes derived from CD-iPSCs (CD-HLCs) did not exhibit ureogenesis. Cellular triglyceride and lipid granule levels were significantly increased in CD-HLCs compared with WT-HLCs. Peroxisome proliferator-activated receptor-α (PPAR-α) and its target genes which are involved in mitochondrial ß-oxidation were downregulated in CD-HLCs, and treatment with a PPAR-α agonist partially reduced the lipid accumulation in CD-HLCs. In addition, the mitochondria in CD-HLCs exhibited abnormal morphologies. Based on these observations, we conclude that the lipid accumulation in CD-HLCs results from dysfunctional mitochondrial ß-oxidation and abnormal mitochondrial structure.


Assuntos
Proteínas de Ligação ao Cálcio/metabolismo , Hepatócitos/metabolismo , Células-Tronco Pluripotentes Induzidas/fisiologia , Mitocôndrias Hepáticas/metabolismo , Transportadores de Ânions Orgânicos/metabolismo , Animais , Diferenciação Celular , Células Cultivadas , Humanos , Metabolismo dos Lipídeos , Camundongos Nus , Oxirredução
12.
Hip Pelvis ; 28(4): 208-216, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28097110

RESUMO

PURPOSE: To compare and analyze clinical and radiologic outcomes of cemented versus cementless bipolar hemiarthroplasty for treatment of femur neck fractures. MATERIALS AND METHODS: A total of 180 patients aged 65 years and over older who underwent bipolar hemiarthroplasty for treatment of displaced femur neck fractures (Garden stage III, IV) from March 2009 to February 2014 were included in this study. Among the 180 patients, 115 were treated with cemented stems and 65 patients with cementless stems. Clinical outcomes assessed were: i) postoperative ambulatory status, ii) inguinal and thigh pain, and iii) complications. The radiologic outcome was femoral stem subsidence measured using postoperative simple X-ray. RESULTS: The cemented group had significantly lower occurrence of complications (postoperative infection, P=0.04) compared to the cementless group. There was no significant difference in postoperative ambulatory status, inguinal and thigh pain, and femoral stem subsidence. CONCLUSION: For patients undergoing bipolar hemiarthroplasty, other than complications, there was no statistically significant difference in clinical or radiologic outcomes in our study. Selective use of cemented stem in bipolar hemiarthroplasty may be a desirable treatment method for patients with poor bone quality and higher risk of infections.

13.
J Adv Prosthodont ; 7(2): 172-7, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25932317

RESUMO

PURPOSE: The purpose of this study was to evaluate cell toxicity due to ion release caused by galvanic corrosion as a result of contact between base metal and titanium. MATERIALS AND METHODS: It was hypothesized that Nickel (Ni)-Chromium (Cr) alloys with different compositions possess different corrosion resistances when contacted with titanium abutment, and therefore in this study, specimens (10×10×1.5 mm) were fabricated using commercial pure titanium and 3 different types of Ni-Cr alloys (T3, Tilite, Bella bond plus) commonly used for metal ceramic restorations. The specimens were divided into 6 groups according to the composition of Ni-Cr alloy and contact with titanium. The experimental groups were in direct contact with titanium and the control groups were not. After the samples were immersed in the culture medium - Dulbecco's modified Eagle's medium[DMEM] for 48 hours, the released metal ions were detected using inductively coupled plasma mass spectrometer (ICP-MS) and analyzed by the Kruskal-Wallis and Mann-Whitney test (P<.05). Mouse L-929 fibroblast cells were used for cell toxicity evaluation. The cell toxicity of specimens was measured by the 3-{4,5-dimethylthiazol-2yl}-2,5-diphenyltetrazolium bromide (MTT) test. Results of MTT assay were statistically analyzed by the two-way ANOVA test (P<.05). Post-hoc multiple comparisons were conducted using Tukey's tests. RESULTS: The amount of metal ions released by galvanic corrosion due to contact between the base metal alloy and titanium was increased in all of the specimens. In the cytotoxicity test, the two-way ANOVA showed a significant effect of the alloy type and galvanic corrosion for cytotoxicity (P<.001). The relative cell growth rate (RGR) was decreased further on the groups in contact with titanium (P<.05). CONCLUSION: The release of metal ions was increased by galvanic corrosion due to contact between base metal and titanium, and it can cause adverse effects on the tissue around the implant by inducing cytotoxicity.

14.
Int J Cardiol ; 187: 157-63, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25828344

RESUMO

BACKGROUND: Atrial tachyarrhythmia is a major late complication in adult Fontan patients. This study examined the clinical features and risk factors of late intra-atrial reentrant tachyarrhythmia (IART) in adult patients after Fontan surgery and the mid-term outcome of Fontan conversion with or without antiarrhythmic surgery in these patients. METHODS: We conducted a retrospective study on adult patients who were born before 1994 and survived at least 3 months after a Fontan operation at Seoul National University Children's Hospital. RESULTS: We followed 160 patients over 20.9 ± 4.1 years. Sustained atrial tachycardia was identified in 51 patients, and IART was found in 41, appearing a mean 13.6 years after surgery. By the 25 year follow-up, 40% had developed IART. The incidence of IART significantly increased over time. Patients with an atriopulmonary connection (APC) (n=65) had significantly longer follow-up duration and higher incidence of IART than patients with a lateral tunnel (n=86) or extracardiac conduit Fontan (n=9). On multivariate analysis, APC, sinus node dysfunction, and nonsustained atrial tachycardia were found to be significantly associated with IART. Twenty-four patients with IART underwent Fontan conversion. Over the follow-up period, IART severity scores in the 22 patients who survived after Fontan conversion decreased significantly, and New York Heart Association functional class significantly improved. On multivariate analysis, protein losing enteropathy and ventricular dysfunction were found to be significant risk factors for mortality. CONCLUSIONS: IART was common in adult Fontan patients, and Fontan conversion with or without antiarrhythmic surgery and pacemaker placement helped to control it.


Assuntos
Eletrocardiografia , Técnica de Fontan/efeitos adversos , Previsões , Cardiopatias Congênitas/cirurgia , Taquicardia por Reentrada no Nó Sinoatrial/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Átrios do Coração/fisiopatologia , Sistema de Condução Cardíaco/fisiopatologia , Humanos , Incidência , Lactente , Masculino , República da Coreia/epidemiologia , Estudos Retrospectivos , Taquicardia por Reentrada no Nó Sinoatrial/diagnóstico , Taquicardia por Reentrada no Nó Sinoatrial/epidemiologia , Adulto Jovem
15.
Stem Cells ; 33(5): 1447-55, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25639853

RESUMO

Cardio-facio-cutaneous (CFC) syndrome is a developmental disorder caused by constitutively active ERK signaling manifesting mainly from BRAF mutations. Little is known about the role of elevated ERK signaling in CFC syndrome during early development. Here, we show that both SMAD1 and ERK signaling pathways may contribute to the developmental defects in CFC syndrome. Induced pluripotent stem cells (iPSCs) derived from dermal fibroblasts of a CFC syndrome patient (CFC-iPSCs) revealed early developmental defects in embryoid body (EB) development, ß-catenin localization, and neuronal differentiation. Both SMAD1 and ERK signalings were significantly activated in CFC-iPSCs during EB formation. Most of the ß-catenin was dissociated from the membrane and preferentially localized into the nucleus in CFC-EBs. Furthermore, activation of SMAD1 signaling recapitulated early developmental defects in wild-type iPSCs. Intriguingly, inhibition of SMAD1 signaling in CFC-iPSCs rescued aberrant EB morphology, impaired neuronal differentiation, and altered ß-catenin localization. These results suggest that SMAD1 signaling may be a key pathway contributing the pathogenesis of CFC syndrome during early development.


Assuntos
Displasia Ectodérmica/metabolismo , Displasia Ectodérmica/patologia , Insuficiência de Crescimento/metabolismo , Insuficiência de Crescimento/patologia , Cardiopatias Congênitas/metabolismo , Cardiopatias Congênitas/patologia , Células-Tronco Pluripotentes Induzidas/metabolismo , Transdução de Sinais , Proteínas Smad/metabolismo , Diferenciação Celular , Núcleo Celular/metabolismo , Corpos Embrioides/metabolismo , Fácies , Humanos , Masculino , Neurônios/patologia , Transporte Proteico , beta Catenina/metabolismo
16.
Artigo em Inglês | MEDLINE | ID: mdl-26737484

RESUMO

An innovative single-port surgical robot has recently been developed by the Samsung Advanced Institute of Technology (SAIT). The robot can reach various surgical sites inside the abdominal cavity from a single incision on the body. It has two 7-DOF surgical tools, a 3-DOF endoscope, a flexible hyper-redundant 6-DOF guide tube, and a 6-DOF manipulator. This paper primarily focuses on the manipulator, called a slave arm, which is capable of setting the location of a Remote Center Motion (RCM) point. Because the surgical tools can explore the abdominal area through a small incision point when the RCM point is aligned with the incision area, the RCM mechanism is an integral part of the manipulator for single-port surgery. The mechanical feature, operational principle, control method, and the system architecture of the slave arm are introduced in this paper. In addition, manipulation experiments conducted validate its efficacy.


Assuntos
Abdome/cirurgia , Procedimentos Cirúrgicos Robóticos/instrumentação , Procedimentos Cirúrgicos Robóticos/métodos , Desenho de Equipamento , Humanos , Movimento (Física)
17.
J Korean Med Sci ; 29(11): 1544-9, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25408587

RESUMO

Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.


Assuntos
Síndrome de Down/complicações , Cardiopatias Congênitas/epidemiologia , Adulto , Povo Asiático , Peso ao Nascer , Aberrações Cromossômicas , Bases de Dados Factuais , Permeabilidade do Canal Arterial/epidemiologia , Feminino , Idade Gestacional , Cardiopatias Congênitas/etiologia , Defeitos dos Septos Cardíacos/epidemiologia , Comunicação Interatrial/epidemiologia , Comunicação Interventricular/epidemiologia , Humanos , Masculino , Prevalência , República da Coreia/epidemiologia
18.
Knee ; 21(5): 906-10, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24998912

RESUMO

BACKGROUND: To compare the ceiling effect of the Lysholm and IKDC subjective scores for assessing functional outcome after ACL reconstruction and evaluated the correlation with the one-leg hop test. METHODS: A total of 134 patients who underwent ACL reconstruction between 2007 and 2011 were enrolled in this study. All patients fulfilled the postoperative 6- and 12-month evaluations. The ceiling effect of the Lysholm and IKDC subjective scores was assessed, and the correlations between two scales and one-leg hop test were analysed. RESULTS: For the entire sample, the ceiling effect for the Lysholm score was 14.9% and 30.6% at 6 and 12 months postoperatively. The values for the IKDC subjective score were 5.2% and 17.2%, respectively. In all subjects, the correlation coefficients [95% confidence intervals] between the IKDC subjective score and one-leg hop test at 6 and 12months (r=0.492, [0.34 to 0.62]; r=0.296, [0.12 to 0.46]) were higher than those for the Lysholm score (r=0.355, [0.18 to 0.51]; r=0.241, [0.06 to 0.41]), respectively.(p<0.05). CONCLUSION: With regard to evaluating ACL reconstruction outcomes in patients, no significant difference between the IKDC subjective and the Lysholm scores exists in terms of the amount of ceiling effect and the correlation with the LSI. However, the concern that the ceiling effect of the Lysholm score was greater than the IKDC subjective score, should be addressed in assessing the patient's functional status postoperatively. LEVEL OF EVIDENCE: III, retrospective comparative study.


Assuntos
Lesões do Ligamento Cruzado Anterior , Reconstrução do Ligamento Cruzado Anterior , Traumatismos do Joelho/fisiopatologia , Traumatismos do Joelho/cirurgia , Escore de Lysholm para Joelho , Recuperação de Função Fisiológica , Adolescente , Adulto , Ligamento Cruzado Anterior/fisiopatologia , Ligamento Cruzado Anterior/cirurgia , Criança , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Resultado do Tratamento , Suporte de Carga , Adulto Jovem
19.
BMC Pediatr ; 14: 51, 2014 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-24548331

RESUMO

BACKGROUND: Kawasaki disease (KD) is an acute febrile vasculitis that causes coronary artery abnormality (CAA) as a complication. In some patients, an association has been noted between elevated liver enzymes or an abnormal gallbladder (GB) and hepatobiliary involvement in KD. In this study, we aimed to evaluate clinical, laboratory, and ultrasonographic (USG) risk factors of hepatobiliary involvement for the intravenous immunoglobulin (IVIG) resistance and the development of CAA in children with KD. METHODS: From March 2004 through January 2013, clinical features, laboratory data, echocardiographic findings, and USG findings were retrospectively reviewed regarding the response to IVIG treatment and coronary artery complications in 67 children with KD. Acute acalculous cholecystitis (AAC) was diagnosed based on USG criteria. RESULTS: Among all factors, only the prothrombin time international normalized ratio was significantly different between the IVIG-response and IVIG-resistance groups (p = 0.024). CAA was statistically more frequent in the AAC group (n = 24) than in the non-AAC group (n = 43) (23.3% vs. 58.3%, p = 0.019). Among the laboratory factors, segmented neutrophil percentage, total bilirubin level, and C-reactive protein were significant in children with CAA (p = 0.014, p = 0.009, and p = 0.010). Abnormal GB findings on USG were significantly more frequent in children with CAA than in those without CAA (p = 0.007; OR = 4.620; 95% confidence interval [CI]: 1.574-13.558). GB distension on USG was the only significant risk factor for CAA (p = 0.001; OR = 7.288; 95% CI: 2.243-23.681) by using multiple logistic regression analysis. CONCLUSION: For children in the acute phase of KD, USG findings of the GB, especially GB distension, may be an important risk factor for CAA as a complication.


Assuntos
Doenças Biliares/etiologia , Doença da Artéria Coronariana/etiologia , Hepatopatias/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Doenças Biliares/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Hepatopatias/epidemiologia , Masculino , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Estudos Retrospectivos , Fatores de Risco
20.
Korean Circ J ; 43(11): 774-81, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24363755

RESUMO

The ductus arteriosus is a normal and essential structure in fetal circulation. Since the introduction of fetal echocardiography, there have been reports of ductal constriction, many of which were related to maternal use of some medications. However, there have been some reports of idiopathic ductal constriction, which usually present in later gestation. Recently we experienced a case, which initially showed an S-shaped ductus with mild narrowing at 23 weeks and 27 weeks gestation and developed severe ductal constriction at 33 weeks. Soon after birth, ductus was searched for but no ductus was found in 2-D and color images. The neonate required mechanical ventilation with supplemental oxygen for 3 days. All echocardiographic abnormalities were normalized in 7 months. We report progressive ductal constriction in an S-shaped ductus and emphasize the importance of continuous follow up extending to the third trimester and even immediately after birth.

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