Incidence of the mucopolysaccharidoses in Northern Ireland
作者： J. Nelson
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摘要: An epidemiological study of the mucopolysaccharidoses (MPS) in Northern Ireland using multiple ascertainment sources was carried out and incidence rate for period 1958–1985 estimated. approximately 1 76 000 live births obtained MPS 1H (Hurler phenotype); 280 000 H/S (Hurler/Scheie 140 000 (1 72 000 male births) II (Hunter syndrome); III (Sanfilippo syndrome) IV A (Morquio syndrome type A). No cases IS (Scheie phenotype), B B) or VI (Maroteaux–Lamy were ascertained during period. Three non-immune hydrops fetalis born to consanguineous parents thought be due β-glucuronidase deficiency (MPS VII) on basis placental histology enzyme studies both but no living VII ascertained. The overall all types mucopolysaccharidosis 25 000 births. comparison is made with estimates from other published studies.
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