- A Endotracheal Intubation in a Child with Mocopolysaccharidoses Using the Fiberoptic Laryngoscopy
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Park CH, Lee SH, Cho SK, Kim BI, Rho WS, Choi BK
- KMID: 2155265
- Korean J Anesthesiol.
- 2000 Jun;38(6):1089-1091.
- doi: 10.4097/kjae.2000.38.6.1089
Mucopolysaccharidoses are a group of inherited disorders of metabolism resulting in the deposition of mucopolysaccharide in various tissues. This leads to organ dysfunction and anatomical abnormalities which can be important... -
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- Experience of endotracheal intubation using video styletscope in a patient with mucopolysaccharidoses: A case report
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Joo HS, Kim MH, Lee AR, Park HG
- KMID: 1971081
- Anesth Pain Med.
- 2013 Jan;8(1):64-67.
Mucopolysaccharidoses (MPSs) are a group of inherited heterogenous metabolic disorders, caused by deficiency of an enzyme involved in the degradation of mucopolysaccharides, and thus deposition of mucopolysaccharides in all connective... -
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- Nerve Conduction Studies in the Patients with Mucopolysaccharidoses
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Kim SY, Kim HS, Lee KW, Hwang JH, Lee YT, Jung ST, Lee MH, Jin DK
- KMID: 2323673
- J Korean Acad Rehabil Med.
- 2001 Oct;25(5):791-794.
OBJECTIVE: To evaluate the characteristics of peripheral nervous system involvement in patients with mucopolysaccharidoses (MPS). METHOD: Electrophysiologic studies were performed in 26 children with MPS confirmed by semiquantitative MPS study,... -
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- A Case of Pulmonary Edema which Developed after Difficult Endotracheal Intubation of Hunter Syndrome: A Case Report
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Kim HJ, Yoon SH, Kim YH, Yoon HS
- KMID: 2228508
- Korean J Crit Care Med.
- 2005 Dec;20(2):187-191.
Hunter syndrome is one of the mucopolysaccharidoses, characterized by abnormal accumulation and deposition of mucopolysaccharides in the tissues of several organs which are known to complicate anaesthetic and airway management.... -
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- Clinical and Laboratory Features of Korean Mucopolysaccharidoses (MPSs)
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Sohn WY, Lee JH, Paik KH, Kwon EK, Kim AH, Jin DK
- KMID: 2279075
- Korean J Pediatr.
- 2005 Oct;48(10):1132-1138.
PURPOSE: The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is... -
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- A case of Hunter's Syndrome: Mucopolysaccharidosis type II
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Cheong HI, Yand HW, Lee KD, Moon HR
- KMID: 1667808
- J Korean Pediatr Soc.
- 1979 Dec;22(12):1074-1079.
We experienced a case of Hunter's syndrome (mucopolysaccharidesis type II) in 9 year old male poatient, whose diagnosis was established by typical findings in physical examination, family history, and radiologic... -
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- Cases of Macular Corneal Dystrophy with a Family History
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Kim IS, Myong YW
- KMID: 2123360
- J Korean Ophthalmol Soc.
- 1995 May;36(5):874-878.
Macular corneal dystrophy is a heredo-familial disease inherited as an autosomal recessive trait and characterized by corneal opacities due to localized mucopolysaccharidosis. One case of macular corneal dystrophy was reported... -
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- A Case of Mucopolysaccharidosis Type I with Spinal Cord Compression
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Cheon SM, Park MJ, Cha JK
- KMID: 2342957
- J Korean Neurol Assoc.
- 2002 Mar;20(2):199-201.
A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan... -
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- Carpal Tunnel Syndrome Caused by Persistent Median Artery and Bifid Median Nerve in an Adolescent
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Lee SU, Lee HW, Joo SY
- KMID: 2461236
- J Korean Orthop Assoc.
- 2019 Oct;54(5):452-456.
- doi: 10.4055/jkoa.2019.54.5.452
Carpal tunnel syndrome is rare in children. When it does occur in children, the most common causes reported are mucopolysaccharidosis and mucolipidosis. The median artery is a transitory vessel that... -
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- Joys and Sorrows in Mothers of Children with Mucopolysaccharidosis
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Park HS, Kim KB
- KMID: 2291806
- J Korean Acad Child Health Nurs.
- 2011 Jan;17(1):58-67.
PURPOSE: This study was done to explore the meaning of joys and sorrows in mothers of children with Mucopolysaccharidosis (MPS). METHODS: The participants were 9 mothers who each had a child... -
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- Mucopolysaccharidosis and mucolipidosis
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Chang SH, Song SM, Sohn WY, Jin DK
- KMID: 2168070
- Hanyang Med Rev.
- 2005 Aug;25(3):27-36.
Mucopolysaccharidosis (MPS) and mucolipidosis(ML) belong to a group of rare genetic disorders of lysosomal enzymes and share some clinical manifestations. MPS is characterized by the accumulation of glycosaminoglycans (GAG) and... -
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- Ocular Manifestations of Pediatric Systemic Disease
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Lim HW, Oh SY
- KMID: 2350369
- Hanyang Med Rev.
- 2016 Aug;36(3):182-185.
- doi: 10.7599/hmr.2016.36.3.182
Pediatric diseases are important because diagnosis and care for these can be complex. Among them, specific diseases have been associated with ocular involvement. This review presents the ocular manifestations of... -
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- Bilateral Maxillary Dentigerous Cysts in a Non-Syndromic Patient
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Jung JH, Kang IG, Cha HE, Kim ST
- KMID: 2277131
- Korean J Otorhinolaryngol-Head Neck Surg.
- 2010 Jan;53(1):57-59.
- doi: 10.3342/kjorl-hns.2010.53.1.57
Dentigerous cysts are the most common type of developmental odontogenic cysts and the second most common odontogenic cyst of the jaw after radicular cysts. Dentigerous cysts are usually solitary, but... -
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- A Large Dentigerous Cyst Found in the Mandible
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Kim YH, Choi JS, Kim BH, Kang SH, Lim DJ, Yu MS
- KMID: 2297566
- J Rhinol.
- 2013 May;20(1):46-49.
Dentigerous cysts are benign odontogenic cysts that are associated with the crowns of permanent teeth. They are developmental epithelial-lined lesions which are formed from fluid accumulation in the space between... -
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- Extensive Mongolian Spots Associated with Hunter Syndrome
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Chun EY, Kim HS, Lee JS, Lee MG
- KMID: 2250789
- Korean J Dermatol.
- 2002 Jul;40(7):820-824.
We report a case of Hunter syndrome with extensive Mongolian spots. A 4-year-old male baby presented with asymptomatic, firm, raised, ivory-colored papules and nodules which coalesced to form ridges in... -
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- Inborn Errors of Metabolism in Korea
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Lee HJ
- KMID: 2185707
- J Korean Neurol Assoc.
- 2004 Feb;22(1):1-10.
Diseases of inborn errors of metabolism (IEMs) are very rare but the overall prevalence of IEMs is not low, and in the United States, about 5~10% of admitted patients have... -
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- Joint Contracture and Radiologic Features in Mucopolysaccharidosis
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Shim JS, Jin DK, Park SJ, Kim SM, Ryu BD
- KMID: 1901418
- J Korean Orthop Assoc.
- 2004 Dec;39(7):825-829.
PURPOSE: To evaluate the clinical manifestations of contracted joints and the radiological features in mucopolysaccharidosis(MPS). MATERIALS AND METHODS: From January 1996 to March 2002, 15 children from a pool of... -
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- Oral manifestation and root canal therapy of the patient with mucopolysaccharidosis
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Yoon JH, Lee HI, Jang JH, Choi SH, Chang HS, Hwang YC, Hwang IN, Lee BN, Oh WM
- KMID: 2446662
- Restor Dent Endod.
- 2019 May;44(2):e14.
- doi: 10.5395/rde.2019.44.e14
Mucopolysaccharidosis (MPS) is an inherited metabolic disorder caused by a deficiency in enzymes that participate in the degradation of glycosaminoglycans (GAGs) such as heparin sulfate and dermatan sulfate. Left untreated,... -
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- Clinical Experience of the Anesthetic Management of Mucopolysaccharidosis
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Lee S, Kim CS, Yang MK, Choi SJ, Lee BD, Park YO, Heo BY
- KMID: 1935962
- Korean J Anesthesiol.
- 2003 Nov;45(5):672-676.
- doi: 10.4097/kjae.2003.45.5.672
Mucopolysaccharidosis is characterized by the progressive accumulation of glycosaminoglycans in multiple organs. Valve and coronary involvement, upper airway obstructive disease, joint stiffness, and mental retardation are associated perioperative anesthetic risks.... -
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- Birth of a healthy baby after preimplantation genetic diagnosis in a carrier of mucopolysaccharidosis type II: The first case in Korea
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Ko DS, Lee SH, Park CW, Lim CK
- KMID: 2469598
- Clin Exp Reprod Med.
- 2019 Dec;46(4):206-210.
- doi: 10.5653/cerm.2019.00094
Mucopolysaccharidosis type II (MPS II) is a rare X-linked recessive lysosomal storage disease caused by mutation of the iduronate-2-sulfatase gene. The mutation results in iduronate-2-sulfatase deficiency, which causes the progressive... -
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