• Title/Summary/Keyword: slowly progressive

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Actinomycotic Osteomyelitis of the Mandible: A Case Report

  • Lee, Young-Cheol;Lim, Lee-Rang;Lee, Kyu-Hoon;Seo, Dong-Jun;Yun, Na-Ra;Oh, Ji-Su;You, Jae-Seek;Choi, Hae-In
    • Journal of Oral Medicine and Pain
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    • v.44 no.3
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    • pp.140-144
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    • 2019
  • Actinomycosis is rare, chronic, slowly progressive disease caused by gram-positive anaerobic organisms from the Actinomycosis family that normally colonizes the oral cavity. Actinomycotic osteomyelitis is even more rare and refractory disease because diagnosis by bacterial culture is not easy. In our case, 80-year-old man visited our clinic with a complaint of swelling and severe sinus tracts without teeth evolvement on anterior mandible. Computed tomography (CT) scan demonstrated erosive bone destruction on anterior mandible, clinically suspected actinomycotic osteomyelitis. The patient also had thoracic actinomycosis on Lt. lower lung. We could diagnosis actinomycosis by histopathologic examination. He treated by conservative surgery and long term antibiotics. After 2 year, no recurrence was seen in CT scan.

Three-dimensional cone-beam computed tomographic sialography in the diagnosis and management of primary Sjögren syndrome: Report of 3 cases

  • Thomas, Nithin;Kaur, Aninditya;Reddy, Sujatha S.;Nagaraju, Rakesh;Nagi, Ravleen;Shankar, Vidya Gurram
    • Imaging Science in Dentistry
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    • v.51 no.2
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    • pp.209-216
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    • 2021
  • Sjögren syndrome is a chronic autoimmune inflammatory disease characterized by lymphocytic infiltration of exocrine glands, predominantly the parotid and lacrimal glands, thereby resulting in oral and ocular dryness. It has been reported to occur most frequently in women between 40 and 50 years of age. Sjögren syndrome has an insidious onset, is slowly progressive, and presents a wide range of clinical manifestations, leading to delays or challenges in the diagnosis. Early diagnosis of this condition is essential to prevent the associated complications that affect patients' quality of life. This report presents 3 cases of Sjögren syndrome in female patients aged between 40 and 75 years who presented with complaints of persistent dry mouth and burning sensation. The cases highlight the diagnostic value of 3-dimensional cone-beam computed tomographic sialography in the detection of salivary gland pathologies at an early stage.

The Effect of Seminal Plasma on Chilling and Freezing of Canine Spermatozoa (개 정액의 정장이 개정자의 냉각과 동결에 미치는 영향)

  • You, Myung-Jo;Lee, John-Hwa;Kim, In-Shik;Park, Jin-Ho;Kwon, Jung-Kee;Kim, Jong-Hoon;Kim, Bum-Seok;Yu, Il-Jeoung
    • Journal of Veterinary Clinics
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    • v.24 no.4
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    • pp.486-492
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    • 2007
  • Seminal plasma(SP) is usually removed from semen that is to be cryopreserved. However, some reports indicate that SP has beneficial effects on spermatozoa during chilling and freezing. The purpose of this study was to determine the effect of SP on sperm survival by adding SP to the extender before cooling and freezing canine spermatozoa. In replicate experiments, ejaculates obtained from four healthy dogs(1-4 years old) of various breeds were pooled, centrifuged at $300{\times}g$ for 10 min at $25^{\circ}C$, and the supernatant of seminal plasma was decanted. Spermatozoa were suspended in egg yolk-Tris(EYT) buffer. The study comprised two experiments: [Exp 1] Sperm were suspended in EYT extender containing either 0, 20, 40, 80 or 100% SP and were slowly cooled to $4^{\circ}C$ for 2h or held at $25^{\circ}C$ as controls. Sperm concentration was adjusted to $2{\times}10^8/ml$. [Exp II] Sperm samples, each of which contained $1{\times}10^8/ml$, were assigned to nine groups to be frozen. In the first four groups, sperm in EYT containing either 20, 40, 80 or 100% SP were cooled to $4^{\circ}C$, then diluted to contain final concentrations of EYT+0.6M glycerol and then were frozen. The final concentrations of SP were 10, 20, 40 or 50%. In the other four groups, sperm in EYT alone were first cooled slowly to $4^{\circ}C$, then diluted to contain final concentrations of EYT+0.6M glycerol plus 10, 20, 40 or 50% SP and then were frozen. Spermatozoa, which chilled in EYT alone and diluted to contain final concentrations of EYT+0.6M glycerol without seminal plasma, and then frozen, was regarded as control. Spermatozoa were frozen at $25^{\circ}C/min$ of cooling rate in plastic straws that were suspended above liquid nitrogen and thawed in water at $38^{\circ}C$ for 1 min. Sperm survival was assayed by determining progressive motility and integrity of plasma and acrosome membranes. Progressive motility was determined by microscopic examination at $200{\times}$ magnification. Membrane integrity was assessed by use of a double fluorescent dye, and acrosome integrity by staining sperm with Pisum sativum agglutinin. The results of the first experiment showed that adding SP did not improve motility of spermatozoa compared to those incubated without SP regardless of temperature. The results of the second experiment showed that spermatozoa suspended in EYT+0.6M glycerol containing SP exhibited the higher progressive motility before being frozen(P<0.05). However, frozen-thawed spermatozoa that had suspended in EYT+0.6M glycerol containing SP showed the similar or lower viability(P<0.05). In summary, although seminal plasma did not affect spermatozoa that were chilled in EYT without cryoprotectant(CPA), addition of seminal plasma to EYT containing CPA did significantly improved progressive motility of canine spermatozoa that were chilled.

Clinical and Biochemical Diagnosis in Children with Leigh Syndrome (Leigh 증후군 환자의 임상적 생화학적 진단)

  • Lee, Sun Ho;Jeon, Mina;Lee, Hyun Joo;Park, Dae Young;Kim, Se Hoon;Lee, Young-Mock
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.2
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    • pp.72-77
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    • 2015
  • Purpose: Deficits of the respiratory chain are reported to be the major cause of Leigh syndrome is said to be the underlying causes. The need for biochemical diagnosis to draw more accurate diagnosis or prognosis to support treatments is rapidly increasing. This study tried to analyze the aspects of clinical characteristics and biochemical diagnosis of mitochondrial respiratory chain complex (MRC) defect in Leigh syndrome, using methods of biochemical enzyme assay. Methods: We included total number of 47 patients who satisfied the clinical criteria of Leigh syndrome and confirmed by biochemical diagnosis. All those patients went through muscle biopsy to perform biochemical enzyme assay to analyze MRC enzyme in order to find the underlying cause of Leigh syndrome. Results: MRC I defect was seen in 23 (48.9%) cases taking the first place and MRC IV defect in 15 (31.9%) following it. There were 9 (19.2%) cases of combined MRC defect. Combined cases of type I and IV were detected in 7 (14.9%) patients while type I and V in 2 (4.3%). The onset age of symptom was less than 1 year old in 28 (59.6%). The most common early symptom, observed in 23 (48.9%), was delayed development, but there were other various neurological symptoms observed as well. In regard with the disease progression, 35 (74.5%) patients showed slowly progressive course, the one that progressed continuously but slowly over 2 years of period. As for Maximum motor development, 22 (46.8%) were bed-ridden state, most of them suffering serious delayed development. Patients showed various symptoms with different organs involved, though neuromuscular involvement was most prominent. Delayed development was seen in all cases. Multifocal lesion in brain MRI study was seen in 36 (76.6 %) cases, taking a greater percentage than 11 (23.4%) cases with single lesion. In MR spectroscopy study, the characteristic lactate peak of mitochondrial disease was identified in 20 (42.6%) patients. Conclusions: Further analysis of clinical and biochemical diagnosis on more extended group of patients with Leigh syndrome will enable us to improve diagnostic precision and to understand the natural course of mitochondrial disease.

A Case of Spindle Cell Hemangioendothelioma Involving the Lung, Mediastinum and Brain (폐, 종격동 및 뇌를 침범한 방추세포형 혈관내피종 1예)

  • Kim, Hwan-Tae;Kim, In-Ho;Lee, Bong-Choon;Kang, Chang-Il;Yoon, Hye-Kyoung
    • Tuberculosis and Respiratory Diseases
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    • v.40 no.3
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    • pp.301-307
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    • 1993
  • Intravascular bronchioloalveolar tumor is now recognized as a pulmonary form of hemangioendothelioma(HE). HE is an unusual tumor of adult life which is characterized by proliferation of an "epithelioid" or "spindle" endothelial cell. In the lung it usually presents as multiple bilateral slowly growing nodules less than 2 cm in diameter. The aetiology and pathogenesis of this disease are unknown. Spindle cell HE occurs at any age, but approximately one half of patient are 25 years of age or younger and males are affected twice more frequently than females. On light microscopic examination, the tumor show mild cellular atypia, nearly absent mitoses and electron-microscopic studies reveal evidence of endothelial cell differentiation. Intracytoplasmic localization of Factor VIII-related antigen is demonstrated on immunohistochemical study, which confirmed the endothelial origin of the tumor. No effective therapy is yet known for HE, but survival of this tumor can be quite long. However, one half of the patient have died, usually of progressive pulmonary insufficiency. This 19-yr-old male complained of Rt. chest pain and intermittent hemoptysis. Simple chest film and chest CT scan showed the Rt. pleural effusion, variable sized bilateral pulmonary nodules, irregular large heterogenous tumor with well enhancement and extensive necrosis in the anterior mediastinum. The mediastinal mass was biopsied and diagnosed as spindle cell HE by light microscopic finding and immunohistochemical studies.

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Multiple Symmetric Lipomatosis (다발성 대칭성 지방종증 -1례 보고-)

  • Lee, Jo-Han;Hong, Jong-Myeon;An, Jae-Ho
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.244-247
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    • 1996
  • Multiple symmetric lipomatosis is a rare disease characterized by progressive growth of subcutaneous fat masses which are located symmetrically at neck, shoulders, chest, abdomen and groin. Recent surveys revealed a high incidence of combined somatic and autonomic neuropathy. The exact cause of the disease is not known. We have experienced one case of multiple symmetric lipomatosis with mediastinal involvement with symptomatic compression of trachea. The patient was a 55-year-male, complaining of dyspnea and slowly enlarging multiple symmetric masses at the neck, shoulders, chest, abdomen, flank and groin over a period of 10 years. He had a habit of excessive alcohol intake for many years. The fatty masses in the neck and the upper mediastinum including peritracheal region were excised through transverse cervical incision. But, because of the incomplete excision of peritracheal fatty tissue, we performed reoperation for the relief of residual tracheal compression at the 15th postoperative day. Two days later emergent tracheostomy was performed due to postoperative pneumomediastinum and subcutaneous emphysema. He could discharge with permanant tracheostomy.

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Study on the Sampling of Distributors : Relating Olfactory Cues and Social Density (유통점의 샘플링에 관한 연구 : 후각적 자극과 매장 밀집도를 중심으로)

  • Hwang, Hee-Joong;Youn, Myoung-Kil
    • Journal of Distribution Science
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    • v.16 no.9
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    • pp.59-63
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    • 2018
  • Purpose - It has already been proved that 'mood' as the physical environment of shopping affects consumers' main sensory channels such as sight, hearing, smell, touch. However, there is no consensus on how the olfactory cue influences the customers in the shopping environment. In this study, we examine the previous studies on how the olfactory cue affects the customers in the shopping environment and present a clear direction as a suggestion for progressive research. Research design, data, and methodology - It is not important to use a lot of unconditional fragrance, but it should be exposed to the environment that suits the proper fragrance. In recent years, meaningful research on store fragrance has been slowly increasing. As a result, studies on the fragrance effects of retail stores have been conducted to verify the relevance of fragrance suitability in stores and consumer spending scale. Results - The fragrance appropriate for each store can not be uniformly specified as any fragrance. This is because external variables such as time, season, temperature, lighting, density of shoppers, and music in the store also affect customer evaluation. For example, using an unsuitable fragrance may encourage customers to leave the store quickly by restraining impulsive purchases or by disturbing concentration. The store manager should also be interested in using fragrances that are proven and effective in the store environment, but they should also have the ability to easily manipulate and manage the fragrances very appropriately according to changes in the store environment. Store managers should observe consumer preferences and responses according to their goals and strategies, and then systematically manage and store information about the fragrance appropriate to the store. Conclusions - In the future, the fragrance marketing researcher needs to consider the spatial form and density of the customer. In practice, managers operating a retail store should check the most appropriate store density(congestion) according to the size and spatial characteristics of the store and maintain the ideal conditions. To do this, it is necessary to pay attention to how to select and control sensory elements such as fragrance(olfactory), music(auditory), and lighting(visual).

BAG3 mutation in a patient with atypical phenotypes of myofibrillar myopathy and Charcot-Marie-Tooth disease

  • Kim, Seung Ju;Nam, Soo Hyun;Kanwal, Sumaira;Nam, Da Eun;Yoo, Da Hye;Chae, Jong?Hee;Suh, Yeon?Lim;Chung, Ki Wha;Choi, Byung?Ok
    • Genes and Genomics
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    • v.40 no.12
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    • pp.1269-1277
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    • 2018
  • Bcl2-associated athanogene 3 (BAG3) mutations have been reported to cause the myofibrillar myopathy (MFM) which shows progressive limb muscle weakness, respiratory failure, and cardiomyopathy. Myopathy patients with BAG3 mutation are very rare. We described a patient showing atypical phenotypes. We aimed to find the genetic cause of Korean patients with sensory motor polyneuropathy, myopathy and rigid spine. We performed whole exome sequencing (WES) with 423 patients with sensory motor polyneuropathy. We found BAG3 mutation in one patient with neuropathy, myopathy and rigid spine syndrome, and performed electrophysiological study, whole body MRI and muscle biopsy on the patient. A de novo heterozygous p.Pro209Leu (c.626C>T) mutation in BAG3 was identified in a female myopathy. She first noticed a gait disturbance and spinal rigidity at the age of 11, and serum creatine kinase levels were elevated ninefolds than normal. She showed an axonal sensory-motor polyneuropathy like Charcot-Marie-Tooth disease (CMT), myopathy, rigid spine and respiratory dysfunction; however, she did not show any cardiomyopathy, which is a common symptom in BAG3 mutation. Lower limb MRI and whole spine MRI showed bilateral symmetric fatty atrophy of muscles at the lower limb and paraspinal muscles. When we track traceable MRI 1 year later, the muscle damage progressed slowly. As far as our knowledge, this is the first Korean patient with BAG3 mutation. We described a BAG3 mutation patient with atypical phenotype of CMT and myopathy, and those are expected to broaden the clinical spectrum of the disease and help to diagnose it.

Transcriptome Analysis of Long-Term Exposure to Blue Light in Retinal Pigment Epithelial Cells

  • Jin, Hong Lan;Jeong, Kwang Won
    • Biomolecules & Therapeutics
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    • v.30 no.3
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    • pp.291-297
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    • 2022
  • Dry age-related macular degeneration (AMD) is a type of progressive blindness that is primarily due to dysfunction and the loss of retinal pigment epithelium (RPE). The accumulation of N-retinylidene-N-retinylethanolamine (A2E), a by-product of the visual cycle, causes RPE and photoreceptor degeneration that impairs vision. Genes associated with dry AMD have been identified using a blue light model of A2E accumulation in the retinal pigment epithelium and transcriptomic studies of retinal tissue from patients with AMD. However, dry macular degeneration progresses slowly, and current approaches cannot reveal changes in gene transcription according to stages of AMD progression. Thus, they are limited in terms of identifying genes responsible for pathogenesis. Here, we created a model of long-term exposure to identify temporally-dependent changes in gene expression induced in human retinal pigment epithelial cells (ARPE-19) exposed to blue light and a non-cytotoxic dose of A2E for 120 days. We identified stage-specific genes at 40, 100, and 120 days, respectively. The expression of genes corresponding to epithelial-mesenchymal transition (EMT) during the early stage, glycolysis and angiogenesis during the middle stage, and apoptosis and inflammation pathways during the late stage was significantly altered by A2E and blue light. Changes in the expression of genes at the late stages of the EMT were similar to those found in human eyes with late-stage AMD. Our results provide further insight into the pathogenesis of dry AMD induced by blue light and a novel model in vitro with which relevant genes can be identified in the future.

Leigh Syndrome Mimicking Wernicke's Encephalopathy: A Case Report (베르니케 뇌병증으로 오인된 리 증후군: 증례 보고)

  • Jisoo Oh;Jinok Choi;Soojung Kim;Eun-ae Yoo
    • Journal of the Korean Society of Radiology
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    • v.81 no.6
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    • pp.1478-1485
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    • 2020
  • Leigh syndrome or subacute necrotizing encephalomyelopathy is a rare, rapidly progressive neurodegenerative disorder. In general, symptoms such as shortness of breath and decreased cardiac function usually occur within 1 year of life. It is a serious disease with a mortality rate of 75% in 2-3 years. The cause of Leigh syndrome is DNA mutation. Approximately 75% of patients have nuclear DNA mutations while 25% have mitochondrial DNA mutations. Clinical symptoms vary depending on the affected brain area. Neuroimaging plays an important role in diagnosing patients with Leigh syndrome. Late-onset Leigh syndrome is rarer and progresses more slowly compared to the classic form. Here, we report a case of late-onset Leigh's syndrome mimicking Wernicke's encephalopathy.