A special case of congenital adrenal hypoplasia and acute bilateral infantile striatal necrosis



Isolated mineralocorticoid deficiency is described in a 5-week-old boy. The deficiency progressed to general adrenal insufficiency during the boy's first year of life. The family history suggested X-linked inheritance. At 18 months of age the patient developed acute bilateral infantile striatal necrosis, which might suggest a possible relationship between both entities.Adrenal hypoplasia, child, striatal necrosis

C. K. van der Ent, Wilhelmina Children's Hospital, University Hospital for Children and Youth, PO Box 18009, 3501 CA Utrecht, The Netherlands