• The Journal of Korean Medicine
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• v.34 no.1
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• pp.179-191
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• 2013

Kleine-Levin syndrome is a rare disorder characterized by recurrent episodes of hypersomnia, hyperphagia, hypersexuality and delusion and hallucination with spontaneous recovery. A 17-year-old female suddenly showed hypersomnia, severe fatigue, and compulsive hyperphagia. She was diagnosed with suspected Kleine-Levin Syndrome. The precipitating factor was an upper respiratory tract infection that the patient had undergone 2 weeks prior. Past psychiatric history included somnambulism and ADHD; her symptoms of ADHD included attention deficit, impulsiveness, hyperactivity, and irritability. This was her third episode. Her hypersomnia was longer in duration but lower in frequency compared with usual KLS. Her low LF/HF ratio in heart rate variability measurement showed high parasympathetic nerve activity. A MMPI report showed some psychological problem. The patient was diagnosed of gastric fever by pattern identification. Her hypersomnia episode improved 6 weeks after onset. This is the first reported case of suspected Kleine-Levin Syndrome in the field of Oriental Medicine in Korea.

• Archives of Craniofacial Surgery
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• v.20 no.5
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• pp.341-344
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• 2019

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.922-929
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• 1997

Relapsing polychondritis is a systemic disorder characterized by recurrent inflammation and degeneration of cartilaginous tissue throughout the body. The association with HLA-DR4 and the occurrence of antibodies to type II collagen and other autoantibodies suggest that an immunologic mechanism is involved in its pathogenesis. The eyes, ears, nose, larynx, trachea and articular areas are commonly involved. Airway narrowing or collapse from respiratory tract involvement occurs in up to 50% of patients with relapsing polychondritis. Treatment consists of administration of corticosteroids and other anti-inflammatory and immunosuppresive drugs. We experienced a case of relapsing polychondritis involving the tracheobronchial tree, nose and ears in a 49-year-old woman. The patient was clinically and histologically diagnosed as relapsing polychondritis according to McAdam's and Damiani's criteria. We report this case with a review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.61 no.4
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• pp.403-406
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• 2006

A 66-years-old man was refered to our hospital because of cough, sputum, chill and fever. Enlargement of the trachea and main bronchi on radiography and bronchoscopy is compatible with Mounier-Kuhn syndrome. Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disorder of uncertain etiology, characterized by marked dilatation of the trachea and major bronchi. This syndrome is associated with tracheal diverticulosis, bronchiectasis and recurrent respiratory tract infection. We report a rare case of Mounier-Kuhn syndrome with pneumonia and literature reviews.

• BMB Reports
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• v.45 no.8
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• pp.427-432
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• 2012

Primary cilia, single hair-like appendage on the surface of the most mammalian cells, were once considered to be vestigial cellular organelles for a past century because of their tiny structure and unknown function. Although they lack ancestral motility function of cilia or flagella, they share common ground with multiciliated motile cilia and flagella on internal structure such as microtubule based nine outer doublets nucleated from the base of mother centrioles called basal body. Making cilia, ciliogenesis, in cells depends on the cell cycle stage due to reuse of centrioles for cell division forming mitotic spindle pole (M phase) and assembling cilia from basal body (starting G1 phase and maintaining most of interphase). Ciliary assembly required two conflicting processes such as assembly and disassembly and balance between these two processes determines the length of cilia. Both process required highly conserved transport system to supply needed substance to grow tip of cilia and bring ciliary turnover product back to the base of cilia using motor protein, kinesin and dynein, and transport protein complex, IFT particles. Disruption of ciliary structure or function causes multiple human disorder called ciliopathies affecting disease of diverse ciliated tissues ranging from eye, kidney, respiratory tract and brain. Recent explosion of research on the primary cilia and their involvement on animal development and disease attracts scientific interest on how extensively the function of cilia related to specific cell physiology and signaling pathway. In this review, I introduce general features of primary cilia and recent progress in understanding of the ciliary length control and signaling pathways transduced through primary cilia in vertebrates.

• Journal of Pharmacopuncture
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• v.23 no.3
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• pp.91-123
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• 2020

The application of antique medical instructions, practices, skills and knowledge has been considered as the most affordable treatment in many developing countries. The use of these preparations and prescriptions over generations has made a useful and valuable guide for drug discovery in modern medicine. Medical herbs have been of a high importance for this purpose. The genus Dorema, of Apiaceae family (Umbelliferae) has a wide use in ethnobotany and traditional medicine around the world. It has been used as a treatment for CNS disease, convulsion, upper respiratory tract problems, gastrointestinal disorder and high blood sugar. Furthermore, phytochemical investigations have reported Dorema species to contain a wide range of constituents including terpenes, coumarins and phenolic compounds. The current review summarizes comprehensive information regarding botany, phytochemistry and pharmacological aspects of Dorema spp.

• Molecules and Cells
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• v.44 no.5
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• pp.292-300
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• 2021

Macrophages residing in various tissue types are unique in terms of their anatomical locations, ontogenies, developmental pathways, gene expression patterns, and immunological functions. Alveolar macrophages (AMs) reside in the alveolar lumen of the lungs and serve as the first line of defense for the respiratory tract. The immunological functions of AMs are implicated in the pathogenesis of various pulmonary diseases such as allergic asthma, chronic obstructive pulmonary disorder (COPD), pulmonary alveolar proteinosis (PAP), viral infection, and bacterial infection. Thus, the molecular mechanisms driving the development and function of AMs have been extensively investigated. In this review article, we discuss the roles of granulocyte-macrophage colony-stimulating factor (GM-CSF) and transforming growth factor (TGF)-β in AM development, and provide an overview of the anti-inflammatory and pro-inflammatory functions of AMs in various contexts. Notably, we examine the relationships between the metabolic status of AMs and their development processes and functions. We hope that this review will provide new information and insight into AM development and function.

• Journal of The Korean Society of Integrative Medicine
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• v.9 no.2
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• pp.105-108
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• 2021

Background : Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare, autosomal recessive metabolic disorder which is caused by genetic mutations that disrupt the urea cycle. It is characterized by variable clinical presentation and the age of onset. Patients may present with gait disturbance and progressive paraplegia and muscle tightness in the lower extremities. The use of botulinum toxin in metabolic disease has rarely been discussed. We describe a case of a 14-year-old-boy with HHH syndrome, who presented with a several - month history of gait disturbance and lower extremity weakness. Case presentation : A 14-year old male had a history of recurrent upper respiratory tract infections, occasional vomiting, loss of appetite, and general weakness, all of which started since he was 10 months old. He was diagnosed with HHH syndrome at one year of age. At the age of 14, he was referred for the assessment and treatment of his gait disturbance and aggravated weakness of the lower extremities. Brain MRI, electrodiagnostic study and blood test were performed to exclude any lesions related to neurologic dysfunction. Botulinum toxin type A were injected into muscles of adductor longus, adductor magnus, lateral and medial hamstring, and lateral and medial gastrocnemius muscle heads under needle electromyography guidance to reduce lower limb spasticity. Intensive physical therapy including gait training and stretching exercise of adductor and calf muscles were also provided. After intensive physical therapy and botulinum toxin injection to reduce lower limb spasticity, he was able to ambulate for 20 meters independently without any walking aids. There were no adverse events after the injection. Conclusion : Botulinum toxin injection is a safe and effective therapy for patients with HHH syndrome who suffer from gait disturbance.

• Tuberculosis and Respiratory Diseases
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• v.57 no.5
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• pp.470-475
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• 2004

Idiopathic hypereosinophilic syndrome (HES) is a disorder characterized by prolonged eosinophilia without an identifiable cause and eosinophil related tissue damage in multiple organs including heart, lung, skin, gastrointestinal tract, liver, and the nervous systems. Pulmonary involvement occurs in about 40% of HES cases, but pleural effusion due to pleuritis and bilateral pneumothoraces are very rare manifestations. We report a case of hypereosinophilic syndrome presented with bilateral pleural effusions and recurrent bilateral pneumothoraces in a 44 year-old male with brief review of the literature.

• Journal of Oral Medicine and Pain
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• v.35 no.2
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• pp.155-163
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• 2010

Dermatomyositis (DM) is an idiopathic inflammatory connective tissue disorder and a systemic autonomic immune disease which shows a progressive muscle weakness and characteristic rash. It is identified by a characteristic rash accompanying, or more often preceding muscle weakness. Pathognomonic skin lesions are a blue-purple discoloration on the upper eyelids with edema (heliotropic rash), a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron's papule). The myopathy represents inflammatory and degenerative changes primarily affecting proximal muscles. DM often involves GI tract and respiratory system with as risk of 15-25% internal malignancy. It's managed with sun protection since muscle weakness as well as a rash could be aggravated by sun exposure. Systemic corticosteroid is an initial therapy and other immunosuppressive agent has been used as alternatives. Facial muscles are unaffected and masticatory muscles are rarely affected in DM. We present trismus close to muscle contracture in a patient with DM. Therefore, it needs continuous mouth-opening exercise to prevent progressive muscle contracture and to ensure normal mouth opening.