• Journal of Yeungnam Medical Science
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• v.6 no.1
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• pp.171-178
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• 1989

Eaton Lambert Syndrome(ELS)is a disorder of neuromuscular transmission. The defect of neuromuscular transmission is due to decrease in the release of acetylcholine guanta from nerve terminal. This syndrome is frequently associated with bronchogenic carcinoma. The diagnosis is established by electromyography, which characteristically shows 1) low amplitude of evoked compound muscle potential to a single supramaximal stimulus on nerve, 2) significant decremental response at low rates of stimulation 3) marked incremental response at high rates of stimulation. Our patient is 52year old man with dyspnea, coughing and muscle weakness of proximal lower limbs. He has small cell lung cancer and associated with ELS, Superior venacava syndrome and has metastatic lesion on right supraclavicular lymphnode confirmed by pathology. Metastatic mass and SVC syndrome are marked improved following chemotherapy and radiotherapy, however follow up EMG finding does not improved. We are here reporting one case which considered compatible for ELS, with a few elementary reviewed literatures.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.2
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• pp.237-244
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• 2021

Dentinogenesis imperfecta (DI) is a hereditary disorder of dentinal defect. It is generally inherited as a single autosomal dominant trait. DI usually affects both the primary and permanent dentition. Affected teeth have various types of discolorations, rapid destruction of the dentin, and severe attrition. In radiologic view, the affected teeth have bulbous crowns, short roots and narrow or closed pulp chambers. The treatment objective is to prevent additional attrition and recover the vertical dimension of occlusion. The aim of this report was to present the long-term prognosis in 15 years in a pair of siblings. Both the patients had DI with tooth attrition and discoloration. Different treatment procedures were used, depending on the difference in the timing of intervention. The first patient saved most of his teeth. The second patient had all of her teeth extracted. This report could be helpful for early diagnosis and overall treatment of DI.

• Annals of Coloproctology
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• v.34 no.6
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• pp.317-321
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• 2018

Purpose: We evaluate the role of transanal tube drainage (TD) as a conservative treatment for patients with anastomotic leakage (AL). Methods: Patients treated for AL who had undergone a low or an ultralow anterior resection with colorectal or coloanal anastomosis for the treatment of rectal cancer between January 2013 and January 2017 were enrolled in this study. The data were collected prospectively and analyzed retrospectively. The primary outcomes were the diagnosis and the management of AL. Results: Two hundred thirteen consecutive patients, 122 males and 91 females, were included. The mean age was $66.91{\pm}11.15years$, and the median body mass index was $24kg/m^2$ (range, $20-35kg/m^2$). The median tumor distance from the anal verge was 8 cm (range, 4-12 cm). Ninety-three patients (44%) received neoadjuvant therapy for nodal disease and/or locally advanced rectal cancer. Only 13 patients (6%) developed AL. Six patients developed subclinical AL as they had a defunctioning ileostomy at the time of the initial procedure. They were treated conservatively with TD under endoscopic guidance in the endoscopy unit and received intravenous antibiotics. Six weeks after discharge, these 6 patients underwent follow-up flexible sigmoidoscopy which showed a completely healed anastomotic defect with no residual stenosis. Seven patients developed a clinically significant AL and required reoperation with pelvic abscess drainage and Hartmann colostomy formation. Conclusion: These results suggest that TD for management of patients with AL is safe, cheap, and effective. Salvaging the anastomosis will help decrease the need for Hartmann colostomy formation. Proper patient selection is important.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.30 no.1
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• pp.42-44
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• 2019

Hashimoto's encephalopathy (HE) is a rare and underdiagnosed neuropsychiatric illness. We present the case of a 17-year-old girl who was admitted to a tertiary-care psychiatric center with acute onset psychosis and fever. Her psychotic symptoms were characterized by persecutory and referential delusions, as well as tactile and visual hallucinations. Her acute behavioral disturbance warranted admission and treatment in a psychiatric setting (risperidone tablets, 3 mg/day). She had experienced an episode of fever with a unilateral visual acuity defect approximately 3 years before admission, which was resolved with treatment. Focused clinical examination revealed an enlarged thyroid, and baseline blood investigations, including thyroid function test results were normal. Abnormal laboratory investigations revealed elevated anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) levels (anti-TPO of 480 IU/mL; anti-TG of 287 IU/mL). Results of other investigations for infection, including cerebrospinal fluid examination, electroencephalography, and brain magnetic resonance imaging were normal. She was diagnosed with HE and was treated with intravenous corticosteroids (methylprednisolone up to 1 g/day; tapered and discontinued after a month). The patient achieved complete remission of psychotic symptoms and normalization of the anti-thyroid antibody titers. Currently, at the seventh month of follow-up, the patient is doing well. This case highlights the fact that in the absence of well-defined clinical diagnostic criteria, a high index of suspicion is required for early diagnosis of HE. Psychiatrists need to explore for organic etiologies when dealing with acute psychiatric symptoms in a younger age group.

• Neonatal Medicine
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• v.25 no.4
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• pp.186-190
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• 2018

Esophageal atresia (EA) with proximal tracheoesophageal fistula (TEF; gross type B) is a rare defect. Although most patients have long-gap EA, there are still no established surgical guidelines. A premature male infant with symmetric intrauterine growth retardation (birth weight, 1,616 g) was born at 35 weeks and 5 days of gestation. The initial diagnosis was pure EA (gross type A) based on failure to pass an orogastric tube and the absence of stomach gas. A "feed and grow" approach was implemented, with gastrostomy performed on postnatal day 2. A fistula was detected during bronchoscopy for recurrent pneumonia; thus, we confirmed type B EA and performed TEF excision and cervical end esophagostomy. As the infant's stomach volume was insufficient for bolus feeding after reaching a body weight of 2.5 kg, continuous tube feeding was provided through a gastrojejunal tube. On the basis of these findings, esophageal reconstruction with gastric pull-up was performed on postnatal day 141 (infant weight, 4.7 kg), and he was discharged 21 days postoperatively. At 12 months after birth, there was no catch-up growth; however, he is currently receiving a baby food diet without any complications. In patients with EA, bronchoscopy is useful for confirming TEF, whereas for those with long-gap EA with a small stomach volume, esophageal reconstruction with gastric pull-up after continuous feeding through a gastrojejunal tube is worth considering.

• Journal of Trauma and Injury
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• v.34 no.4
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• pp.299-304
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• 2021

Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, life-threatening condition of thrombotic microangiopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and renal impairment. The mechanisms underlying aHUS remain unclear. Herein, we present the first case in the literature of aHUS after a traumatic injury. A 55-year-old male visited the emergency department after a traumatic injury caused by a tree limb. Abdominal computed tomography revealed a rectal wall defect with significant air density in the perirectal space and preperitoneum, implying rectal perforation. Due to the absence of intraperitoneal intestinal perforation, we performed diverting sigmoid loop colostomy. An additional intermittent simple repair was performed due to perianal and anal injuries. One day postoperatively, his urine output abruptly decreased and serum creatinine level increased. His platelet level decreased, and a spiking fever occurred after 2 days. The patient was diagnosed with acute renal failure secondary to aHUS and was treated with fresh frozen plasma replacement. Continuous renal replacement therapy (CRRT) was also started for oliguria and uremic symptoms. The patient received CRRT for 3 days and intermittent hemodialysis thereafter. After hemodialysis and subsequent supportive treatment, his urine output and renal function improved. The hemolytic anemia and thrombocytopenia also gradually improved. Dialysis was terminated on day 22 of admission and the patient was discharged after recovery. This case suggests that that a traumatic event can trigger aHUS, which should be considered in patients who have thrombocytopenia and acute renal failure with microangiopathic hemolytic anemia. Early diagnosis and appropriate management are critical for favorable outcomes.

• 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.

• Journal of the Korean Orthopaedic Association
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• v.55 no.4
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• pp.348-353
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• 2020

A 68-year-old man presented with a bed sore with pus discharge on lower back. Radiographs showed extensive destruction of the L4 vertebral body. Magnetic resonance imaging (MRI) showed fluid collection with an enhanced wall at the defect of the L4 vertebral body extending into both psoas muscles. The primary diagnosis was neuropathic spondylopathy, but infective spondylitis was not ruled out. Initially, he was treated with antibiotics for two weeks. A follow-up MRI showed no improvement of the abscess, so surgical exploration was done. Charcot spinal arthropathy resulted in extensive vertebral body destruction that may be similar to infectious spondylitis, particularly in the case with fluid accumulation due to rupture of dura.

• The Journal of Korean Academy of Prosthodontics
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• v.60 no.4
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• pp.382-394
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• 2022

The treatment of patients with severe periodontitis should be proceeded step-bystep through an accurate diagnosis of each patients' individual tooth and with a strategic treatment plan. Implant-supported fixed prosthetic restoration has the advantage of high patient satisfaction and stable vertical dimension compared to the removable partial denture. However, multiple teeth defect areas lacking hard tissue may be disadvantageous in aesthetic failure and longer treatment time. In addition, it takes a certain period of time to manufacture and install a conventional fixed prosthesis, and during this process, the provisional prosthesis must satisfy the mechanical, biological, and aesthetic requirements of teeth. The purpose of this article is to describe the fabrication of implant-supported fixed prosthesis through a step-by-step approach in a partially edentulous patient.

• KSCE Journal of Civil and Environmental Engineering Research
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• v.43 no.2
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• pp.165-173
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• 2023

The data management and the evaluation of defects in the bridge are generally conducted based on inspection and diagnosis data, including the exterior damage map and defect quantity table prepared by periodic inspection. Since most of these data are written in 2D-based documents and are difficult to digitize in a standardized manner, it is challenging to utilize them beyond the defined functionality. This study proposed methods to efficiently build a BIM (Building Information Modeling)-based bridge damage model from raw data of inspection report and to manage and utilize the damage information linking to bridge model through the spread sheet data generated by COBie (Construction Operations Building Information Exchange). In addition, a method to conduct the condition assessment of defects in bridge was proposed based on an automatic evaluation process using digitized bridge member and damage information. The proposed methods were tested using superstructure of PSC-I girder concrete bridge, and the efficiency and effectiveness of the methods were verified.