Clinical and hematologic manifestations in patients with Diamond Blackfan anemia in Korea.

ABSTRACT:

Background

Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years.

Methods

The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study.

Results

The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptos

SUBMITTER: Kim SK 

PROVIDER: S-EPMC3389062 | biostudies-literature | 2012 Jun

REPOSITORIES: biostudies-literature