Summary
Patients with MPS IV have a clinical disorder quite different from other MPS conditions. The major treatment issue revolves around the prevention of cervical myelopathy, although the other aspects of this multisystem disease should not be forgotten. Management is inevitably multidisciplinary and the paediatrician should play a lead role in the coordination of services for affected patients.
The timing of occipito-cervical fusion and the selection of patients for this procedure remain unclear. A prophylactic approach to surgery is suggested in this review, although it is by no means clear that all patients will invariably develop cervical myelopathy if left untreated.
Parents of affected children need considerable support in dealing with the affected child and the children themselves need help to try to come to terms with their severe physical disabilities. The Society for Mucopolysaccharide Diseases (55 Hill Avenue, Amersham, Bucks HP6 5BX, UK) has offered many parents and affected individuals considerable help as well as raising significant sums for research.
Finally, it is important to remember the genetic nature of the disease and the possibility of prenatal diagnosis in subsequent pregnancies.
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References
Cahane M, Treister G, Abraham FA, Melamed S (1990) Glaucoma in siblings with Morquio syndrome.Br J Ophthalmol 74: 382–383.
Dangel ME, Tsou BH-P (1985) Retinal involvement in Morquio's syndrome (MPS IV).Ann Ophthalmol 17: 349–354.
Diaz JH, Belani KG (1993) Perioperative management of children with mucopolysaccharidoses.Anesth Analg 77: 1261–1270.
Factor SM, Biempica L, Goldfischer S (1978) Coronary intimal sclerosis in Morquio's syndrome.Virchows Arch A: Pathol Anat Histol 379: 1–10.
John RM, Hunter D, Swanton RH (1990) Echocardiographic abnormalities in type IV mucopolysaccharidosis.Arch Dis Child 65: 746–749.
Kopits SE (1976) Orthopedic complications of dwarfism.Clin Orthop Relat Res 114: 153–179.
Lipson SJ (1977) Dysplasia of the odontoid process in Morquio's syndrome causing quadriparesis.J Bone Joint Surg Am 59-A: 340–344.
Nelson J, Kinirons M (1988) Clinical findings in 12 patients with MPS IV A (Morquio's disease). Further evidence for heterogeneity. Part II: Dental findings.Clin. Genet 33: 121–125.
Nelson J, Thomas PS (1988) Clinical findings in 12 patients with MPS IV A (Morquio's disease). Further evidence for heterogeneity. Part III: Odontoid dysplasia.Clin Genet 33: 126–130.
Ogawa T, Tomatsu S, Fukuda S, et al (1995) Mucopolysaccharidosis IVA: screening and identification of mutations of theN-acetylgalactosamine-6-sulfate sulfatase gene.Hum Mol Genet 4: 341–349.
Stevens JM, Kendall BE, Crockard HA, et al (1991) The odontoid process in Morquio-Brailsford disease.J Bone Joint Surg (Br) 73-B: 851–858.
Tomatsu S, Fukuda S, Masue M, et al (1992) Mucopolysaccharidosis type IVA: characterisation and chromosomal localization ofN-acetylgalactosamine-6-sulfate sulfatase gene and genetic heterogeneity.Am J Hum Genet 51 (supplement): A178.
Wraith JE (1995) The mucopolysaccharidoses: a clinical review and guide to management.Arch Dis Child 72: 263–267.
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Northover, H., Cowie, R.A. & Wraith, J.E. Mucopolysaccharidosis type IVA (morquio syndrome): A clinical review. J Inherit Metab Dis 19, 357–365 (1996). https://doi.org/10.1007/BF01799267
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DOI: https://doi.org/10.1007/BF01799267