• Title/Summary/Keyword: Multiple Defect

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Incontinentia Pigmenti with Multiple Missing Teeth : Case Reports (다수의 치아결손을 동반한 색소실조증 환아의 치험례)

  • Choi, Shinae;Kim, Youngjin;Nam, Soonhyeun;Kim, Hyunjung
    • Journal of the korean academy of Pediatric Dentistry
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    • v.42 no.2
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    • pp.180-187
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    • 2015
  • Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

COMPARISON OF MARGINAL LEAKAGE OF WEDGE-SHAPED CLASS V CAVITY ACCORDING TO RESTORATIVE MATERIALS (쐐기 형태의 5급와동에서 수복재료에 따른 변연 미세누출의 비교)

  • Jang, Hyun-Joo;Lee, Hee-Joo;Hur, Bock
    • Restorative Dentistry and Endodontics
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    • v.25 no.1
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    • pp.56-62
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    • 2000
  • The purpose of this study was to assess the effects of restorative materials on the marginal leakage of wedge-shaped class V cavity. The study was performed in vitro in 25 defect-free permanent, extracted teeth. Wedge-shaped class V cavities were prepared and then the teeth were randomly selected and restored according to the following. Group A : restoration with Tetric Ceram(composite resin) Group B : restoration with Tetric flow(flowable resin) Group C : restoration with Compoglass after acid etching(compomer) Group D : restoration with Compoglass(compomer) Group E : restoration with Fuji II LC improved(resin-modified GIC) After thermocycling, the specimens were immersed in 5% basic fuchsin solution for 6 hours and sectioned longitudinally through the center of the restoration. The degree of marginal leakage was measured as the extent of dye penetration under the stereomicroscope. The data were analysed using one-way ANOVA. When significant differences found, multiple comparisons were made using Duncan's Multiple Range Test. The results were as follows: 1. The occlusal margins of all groups except for Fuji II LC improved showed lesser leakage than gingival margins and there was statistically significant difference(p<0.05). 2. At the occlusal margins, group A, B showed same marginal leakage scores, and others were decreased as group C, D, E in that order. There were statistically significant difference between group A, Band group D, E, group C and group E(p<0.05). 3. At the gingival margins, group B, C showed same marginal leakage scores, and others were decreased as group A, D, E in that order. But there was statistically significant difference between group B, C and group E(p<0.05). 4. In the Compoglass restoration, acid-etching technique was beneficial for marginal sealing ability at all of margins. But there was no statistically significant difference (p>0.05). In the restorations for wedge-shaped class V cavities, resin restoration with acid etching technique is recommended.

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Development of the Splint Manufacturing Process Using Indirect Coating and Roll Bonding (간접 코팅과 롤 접합을 이용한 의료용 스플린트 제작 및 공정기술 개발)

  • Ha, Kyoung-Ho;Kang, Dae-Min;Lee, Jung-Hee;Kwak, Jae-Seob
    • Journal of the Korean Society of Manufacturing Process Engineers
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    • v.18 no.2
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    • pp.1-6
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    • 2019
  • With the increase in number of the athletic population and elderly demographic, the demand for orthopedic splints, which are used to support a damaged body, has rapidly increased. Current splints mainly consist of inner and outer parts, which are multiple fabrics covered with polyurethane and nonwoven fabrics, respectively. However, the laminated materials with directly applied pre-polymer coating lead to a high defect rate because of the uneven thickness on the surface. Thus, this study proposes an indirect coating method using a precise clearance controller, which enables the even application of the coating material on multiple inner parts while maintaining a constant thickness. In addition, a roll-to-roll (R2R) technique is applied instead of the sewing mechanism to bond the inner and outer materials together and enhance the productivity in the final stage. In the advanced methods, there is a storage tank that contains polyurethane, a clearance controller, and pairs of rollers in the upper and lower rows. To improve the quality of the products and optimize the equipment, three controllable factors are determined: the viscosity of polyurethane, angle of the gap controller and number of pairs of rollers in the R2R system.

A patient with multiple arterial stenosis diagnosed with Alagille syndrome: A case report

  • Lee, Yoon Ha;Jeon, Yong Hyuk;Lim, Seon Hee;Ahn, Yo Han;Lee, Sang-Yun;Ko, Jung min;Ha, II-Soo;Kang, Hee Gyung
    • Journal of Genetic Medicine
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    • v.18 no.2
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    • pp.142-146
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    • 2021
  • Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

The first Korean case of a newborn with 3p26 microdeletion and 5q35 microduplication inherited from paternal balanced translocation

  • Jang, Jin A;Sohn, Young Bae;Lee, Jang Hoon;Park, Moon Sung
    • Journal of Genetic Medicine
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    • v.18 no.1
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    • pp.48-54
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    • 2021
  • Genetic imbalances are a major cause of congenital and developmental abnormalities. We report the first case of a 3p26 microdeletion and 5q35.2q35.3 microduplication in a newborn with multiple congenital anomalies evaluated using chromosomal microarray analysis (CMA) and fluorescence in situ hybridization (FISH). The patient was born at 30 weeks and 2 days of gestation with a body weight of 890 g. He had symmetric intrauterine growth restriction, microcephaly, facial dysmorphism (hypertelorism, blepharophimosis, mild low-set ears, high-arched palate, and micrognathia), and right thumb polydactyly. Echocardiography revealed an atrial septal defect and patent ductus arteriosus. Furthermore, CMA revealed a concurrent microdeletion in 3p26 and a microduplication in 5q35.2q35.3. FISH analysis showed that these genetic changes resulted from a translocation mutation between chromosomes 3 and 5. The patient's mother had mild intellectual disability, short stature, and facial dysmorphism, while his father had a normal phenotype. However, parental FISH analysis revealed that the asymptomatic father carried a balanced translocation of chromosomes 3p26 and 5q35. CMA and FISH tests are useful for diagnosing neonates with multiple congenital abnormalities. Further parental genetic investigation and proper genetic counseling are necessary in cases of chromosomal abnormalities inherited from parental balanced translocations.

Role of the Gastrocnemius Musculocutaneous with a Propeller Style Skin Flap in Knee Region Reconstruction: Indications and Pitfalls

  • Gianluca Sapino;Rik Osinga;Michele Maruccia;Martino Guiotto;Martin Clauss;Olivier Borens;David Guillier;Pietro Giovanni di Summa
    • Archives of Plastic Surgery
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    • v.50 no.6
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    • pp.593-600
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    • 2023
  • Background Soft tissue reconstruction around the knee area is still an open question, particularly in persistent infections and multiple reoperations scenario. Flap coverage should guarantee jointmobility and protection, even when foreign materials are implanted. The chimeric harvesting of the musculocutaneous gastrocnemius flap, based on the sural artery perforators, can extend its applicability in soft tissue reconstruction of the upper leg, overcoming the drawbacks of the alternative pedicled flaps. Methods A multicenter retrospective study was conducted enrolling patients who underwent to a pedicled, chimeric gastrocnemius musculocutaneous-medial sural artery perforator (GM-MSAP) or lateral sural artery perforator (GM-LSAP) flap for knee coverage in total knee arthroplasty (TKA) recurrent infections and oncological or traumatic defects of the upper leg from 2018 to 2021. Outcomes evaluated were the successful soft tissue reconstruction and flap complications. Surgical timing, reconstruction planning, technique, and rehabilitation protocols were discussed. Results Twenty-one patients were included in the study. Nineteen GM-MSAPs and 2 GM-LSAPs were performed (soft tissue reconstruction in infected TKA [12], in infected hardware [4], and in oncological patients [5]). Donor site was closed primarily in 9 cases, whereas a skin graft was required in 12. Flap wound dehiscence (1), distal flap necrosis (1), distal necrosis of the skin paddle (1), and donor site infection (1) were the encountered complications. Flap reraise associated to implant exchange or extensive debridement was successful without requiring any further flap surgery. Conclusion The propeller-perforator GM-MSAP offers qualitative defect coverage and easiness of multiple flap reraise due to skin availability and its laxity.

Interdisciplinary approach on oral rehabilitation of an adolescent patient with multiple missing teeth: a clinical report (타과와의 협진을 통한 다수치 결손을 동반한 성장기 환자의 보철 수복 증례)

  • Kwon, Kung-Rock;Paek, Janghyun;Lee, Hyun-Woo
    • The Journal of Korean Academy of Prosthodontics
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    • v.51 no.4
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    • pp.339-346
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    • 2013
  • In case of missing of permanent teeth by trauma or innate defect, the decision of treatment modalities and application timing have an important effect on the prognosis of oral rehabilitation. In this case report, interdisciplinary approach between the orthodontic and prosthodontic treatment, the way to re-establish the collapsed occlusal vertical dimension, and implant prosthetic considerations will be discussed. Proper diagnosis on teeth and craniofacial skeleton was made prior to treatment and provisional restorations were used in regard of growth patterns of the patient. Finally, the edentulous areas were restored with fixed implant prostheses. Diagnosis, treatment rationale and prognosis will be discussed thoroughly.

A Korean Girl with Campomelic Dysplasia caused by a Novel Nonsense Mutation within the SOX9 Gene

  • Ko, Jung Min;Hah, J.-Hun;Kim, Suk-Wha;Cho, Tae-Joon;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of Genetic Medicine
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    • v.9 no.2
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    • pp.89-92
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    • 2012
  • Campomelic dysplasia (CMD) is a rare, often lethal, genetic disorder characterized by multiple congenital anomalies and abnormal development of the reproductive organs in males. Mutations in the SOX9 gene are known to cause CMD. We present a Korean CMD girl with a normal 46,XX karyotype and a female reproductive organ phenotype. She was born at 2.35 kg at 38 weeks of gestation and showed characteristic phenotypes, including cleft palate, micrognathia, hypertelorism, flat nasal bridge, congenital bowing of limbs, hypoplastic scapulae, deformed pelvis, and 11 pairs of ribs. She also had an atrioseptal defect of the heart and marked laryngotracheomalacia requiring tracheostomy and tracheopexy. SOX9 mutation analysis revealed the presence of a novel nonsense mutation, $p.Gln369^*$, and the patient was genetically confirmed to have CMD. Although she showed marked failure to thrive and neurodevelopmental delay, she is now 40 months of age and is the only surviving patient with CMD in Korea.

Dynamic Load Balancing Algorithm using Execution Time Prediction on Cluster Systems

  • Yoon, Wan-Oh;Jung, Jin-Ha;Park, Sang-Bang
    • Proceedings of the IEEK Conference
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    • 2002.07a
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    • pp.176-179
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    • 2002
  • In recent years, an increasing amount of computer network research has focused on the problem of cluster system in order to achieve higher performance and lower cost. The load unbalance is the major defect that reduces performance of a cluster system that uses parallel program in a form of SPMD (Single Program Multiple Data). Also, the load unbalance is a problem of MPP (Massive Parallel Processors), and distributed system. The cluster system is a loosely-coupled distributed system, therefore, it has higher communication overhead than MPP. Dynamic load balancing can solve the load unbalance problem of cluster system and reduce its communication cost. The cluster systems considered in this paper consist of P heterogeneous nodes connected by a switch-based network. The master node can predict the average execution time of tasks for each slave node based on the information from the corresponding slave node. Then, the master node redistributes remaining tasks to each node considering the predicted execution time and the communication overhead for task migration. The proposed dynamic load balancing uses execution time prediction to optimize the task redistribution. The various performance factors such as node number, task number, and communication cost are considered to improve the performance of cluster system. From the simulation results, we verified the effectiveness of the proposed dynamic load balancing algorithm.

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Sensor Fusion System for Improving the Recognition Performance of 3D Object (3차원 물체의 인식 성능 향상을 위한 감각 융합 시스템)

  • Kim, Ji-Kyoung;Oh, Yeong-Jae;Chong, Kab-Sung;Wee, Jae-Woo;Lee, Chong-Ho
    • Proceedings of the KIEE Conference
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    • 2004.11c
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    • pp.107-109
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    • 2004
  • In this paper, authors propose the sensor fusion system that can recognize multiple 3D objects from 2D projection images and tactile information. The proposed system focuses on improving recognition performance of 3D object. Unlike the conventional object recognition system that uses image sensor alone, the proposed method uses tactual sensors in addition to visual sensor. Neural network is used to fuse these informations. Tactual signals are obtained from the reaction force by the pressure sensors at the fingertips when unknown objects are grasped by four-fingered robot hand. The experiment evaluates the recognition rate and the number of teaming iterations of various objects. The merits of the proposed systems are not only the high performance of the learning ability but also the reliability of the system with tactual information for recognizing various objects even though visual information has a defect. The experimental results show that the proposed system can improve recognition rate and reduce learning time. These results verify the effectiveness of the proposed sensor fusion system as recognition scheme of 3D object.

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