Morquio's Disease

  • Hariarti S. Pramuljo Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
  • L. A. Tamaela Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
  • W.T. Karyomanggow Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
DOI: https://doi.org/10.14238/pi20.5-6.1980.130-8
Keywords: Morquio's disease, mucopolysaccharidosis, deformities, radiological examination

Abstract

Morquio's disease belongs to the group of mucopolysaccharidosis which causes deformities of the bones. The diagnosis is made by radiological examinations and others such as biochemical examination.

Four cases of Morquio's disease are presented, which are the first reported in the Indonesian literature. All cases are of Chinese origin.

Cervical fusion had been done in one of the cases to prevent the complication of neurologic sequelae and sudden death. The symptoms, diagnosis and therapeutic measures to prevent sequelae have been discussed.

References

1. HORTON, W.A. and SCIMKE, R.N. : A new mucopolysaccharidosis. J. Pediatr. 77 : 252-258 (1970).
2. KOZLOWSKI, K. : Platyspondyly in children. Paediatr. Radio!. 2:81-88 (1974). Bone dysplasia in Radiographic diagnosis Paediatr. Radio!. 4 : 66 - 68 (1976).
3. LIPSON, S.J. : Dysplasia of the Odontoid process in Morquio's Syndrome causing quadriparesis. J. Bone. Joint Surgery 59a ; 340 (1977).
4. PENNOCK, C.A. : A review and selection of simple laboratory methods used for the study of glycosaminoglycan excretion and the diagnosis of the mucopolysaccharidosis. J. Clin. Path. 29 : 111-123 (1976).
5. SPRANGER, J. und GERMAN, R.V. : Mucopolisacehaxide - suchtest. Pediatr. Prax. 6 : 659 - 660 (1967).
6. SPRANGER, J.; LANGER, L.O. and WIEDEMAN, H.R. : Bone dysplasia. An atlas of constitutional disorders of skeletal development. (Saunders, Philadelphia/Toronio 1974).
7. TACHDJIAN, M.O. : Pediatric Orthopaedics, p. 287 - 301. (Saunders. Philadelpilia/London/Toron1o 1972).
Published
2021-12-16
How to Cite
1.
Pramuljo HS, Tamaela LA, Karyomanggow W. Morquio’s Disease. PI [Internet]. 16Dec.2021 [cited 23Nov.2024];20(5-6):130-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/2873
Issue
Vol 20 No 5-6 (1980): May - June 1980
Section
Case Report

Copyright (c) 2021 Hariarti S. Pramuljo, L. A. Tamaela, W.T. Karyomanggow

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Received 2021-12-16
Published 2021-12-16