info:eu-repo/semantics/article
Granulações de Alder-Reilly em Pacientes com Mucopolissacaridose VI
Alder-Reilly Anomaly in Patients with Mucopolysaccharidosis VI
Autor
Vieira, Taiane Alves
Schwartz, Ida Vanessa
Souza, Carolina Moura
Azevedo, Ana Cecília
Pinto, Louise Lapagesse
Bitar, Cristina
Giugliani, Roberto
Resumen
Several authors have described the occurrence of coarse granular cytoplasmic inclusions (the “AlderReilly Bodies”; ARB) in leukocytes of patients with Mucopolysaccharidosis VI (MPS VI; OMIM +253200). Aiming at estimating the frequency of ARB in MPS VI, we analyzed the reports of the peripheral blood counts routinely performed in the MPS VI patients at our hospital from 2002 to 2003 (35 reports of 17 patients; 9/17 patients had two or more evaluations performed in this period). No patient was on enzyme replacement therapy at the time of the blood sampling. The analysis of blood cells was first performed with automation in haematology (Pentra 120-ABX).