Results 151 to 160 of about 3,016 (187)
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1996
Morquio’s syndrome is one of the mucopolysaccharide abnormalities. The mucopolysaccharides are complex sugars which require a variety of enzymes for their correct metabolism. There is a group of diseases, known as the mucopolysaccharides, each of which has a specific enzyme defect.
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Morquio’s syndrome is one of the mucopolysaccharide abnormalities. The mucopolysaccharides are complex sugars which require a variety of enzymes for their correct metabolism. There is a group of diseases, known as the mucopolysaccharides, each of which has a specific enzyme defect.
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Oral manifestations of Morquio's syndrome
Oral Surgery, Oral Medicine, Oral Pathology, 1975The clinical and laboratory findings in two siblings affected with Morquio's syndrome are described. The oral findings, consisting of disturbances in the structure of the enamel in both deciduous and permanent teeth, resemble those of amelogenesis imperfecta, Type 1.
M, Sela, E, Eidelman, S, Yatziv
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Coronary intimal sclerosis in Morquio's syndrome
Virchows Archiv A Pathological Anatomy and Histology, 1978Mitral valve, coronary arteries, cartilage, and liver were studied by light and electron microscopy in a 15 year old boy with Morquio's syndrome, a genetic mucopolysaccharidosis, in which a deficiency of lysosomal hexosamine sulfatase is associated with accumulations of keratan sulfate in various organs.
S M, Factor, L, Biempica, S, Goldfischer
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The Lower Extremity in Morquio Syndrome
Journal of Pediatric Orthopaedics, 2012The modalities and results of surgical intervention in the lower extremity in children with Morquio syndrome type A [mucopolysaccharidosis-IV (MPS-IVA)] have not been well described. The aims of this study are to define the lower extremity deformities, and describe the results of intervention in MPS-IVA patients.Retrospective chart and radiograph ...
Arjun A, Dhawale +5 more
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The morquio syndrome: Neuropathologyxs and biochemistry
Annals of Neurology, 1978AbstractThe activity of N‐acetyl galactosamine‐6‐sulfate sulfatase was studied for the first time in the liver and brain of a patient with a clinically typical case of Morquio syndrome with keratosulfaturia. As has been demonstrated in the fibroblasts of patients with this syndrome, this enzymatic activity was markedly decreased in both organs ...
A, Koto +4 more
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The American journal of otology, 1987
Treatment of hereditary syndromes is still hampered by a scarcity of information. Morquio's syndrome is a mucopolysaccharidosis associated with chronic middle ear disease and with mixed or sensorineural hearing loss, although some patients with this syndrome have had normal hearing. This case is presented to report the occurrence of hearing loss and to
R T, Sataloff +2 more
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Treatment of hereditary syndromes is still hampered by a scarcity of information. Morquio's syndrome is a mucopolysaccharidosis associated with chronic middle ear disease and with mixed or sensorineural hearing loss, although some patients with this syndrome have had normal hearing. This case is presented to report the occurrence of hearing loss and to
R T, Sataloff +2 more
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Morquio syndrome: Diagnosis in an adult
Joint Bone Spine, 2008Morquio syndrome or mucopolysaccharidosis (MPS) type IV is a rare autosomal recessive disease in which keratan sulfate builds up in cells. There are two variants, A and B, corresponding to deficiencies of two different enzymes. Type A is usually severe, although considerable clinical variability occurs due to the existence of attenuated phenotypes ...
Catherine, Prat +4 more
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CUTANEOUS CHANGES IN THE MORQUIO SYNDROME
British Journal of Dermatology, 1969SUMMARY.— Two cases of the Morquio syndrome, with associated mucopolysacchariduria, are described. Striking cutaneous abnormalities were found in each, the skin being loose, thickened, tough and inelastic particularly at the extremities. Generalized telangiectasia was present especially on the face and limbs.
M W, Greaves, P M, Inman
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Morquio's syndrome and its anaesthetic considerations
Pediatric Anesthesia, 2002Summary Morquio's syndrome is an inherited disorder characterized by excessive excretion of keratan sulphate in the urine. The anaesthetic care of these patients should take into consideration respiratory, craniofacial, cardiac, skeletal, ocular and hepatic abnormalities.
Kathleen A, Morgan +2 more
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