Andersen’s disease [Dorothy Hansine Andersen, American pediatrician and pathologist, 1901–1963] , a rare glycogen storage disease characterized by a genetic deficiency of branching enzyme (alpha-1:4, alpha 1:6 transglucosidase), causing the deposition in tissues of abnormal glycogen with long inner and outer chains. Also called amylopectinosis, brancher glycogen storage disease, glycogen storage disease, type IV. ▪ OBSERVATIONS: Infants with the disease are not affected at birth but fail to thrive and soon show hepatomegaly, splenomegaly, and hypotonia of muscle associated with the progressive development of cirrhosis or heart failure of unknown mechanisms. Diagnosis is by enzyme assays of leukocytes and fibroblasts. ▪ INTERVENTIONS: There is no specific therapy for the disease; treatment is directed toward control of symptoms. Liver transplantation may be an option for some individuals. Dietary measures to maintain normal blood sugar levels and to improve liver function and general overall nutrition should be employed. ▪ PATIENT CARE CONSIDERATIONS: The coordinated effort of a team of health professionals, based on symptoms, is important to assist the patient and family to cope with the progressive nature of the disease. Genetic counseling should be considered.
