Role Of Puberty Research Articles

The role of puberty in red-cell production in hereditary haemolytic anaemias.

Summary. Long‐term follow‐up of two male siblings with hereditary non‐spherocytic haemolytic anaemia revealed marked haematological improvement leading to normal haemoglobin concentration in the older boy undergoing puberty, whereas the younger brother who was prepubescent remained anaemic. While the erythrocyte life‐span was the same in both patients, the total nucleated erythron, red‐cell volume, and consequently the rate of red‐cell production, was significantly higher in the older sibling. This finding suggested that puberty in males may play an important role in the compensation of haemolytic process. This hypothesis was further substantiated by analysis of data in 119 patients with hereditary spherocytic and non‐spherocytic haemolytic anaemia. In comparing the haemoglobin concentration it was found that affected adult males had significantly higher haemoglobin concentration than affected children. This haematological discrepancy is thought to be the result of erythroid stimulation by androgens produced endogenously.