MORTALITY IN CANADIAN CHILDREN WITH GROWTH-HORMONE (GH) DEFICIENCY RECEIVING GH THERAPY 1967-1992

The objective of this study was to determine the risk of death and pot ential for prevention of mortality in a large population of children w ith growth hormone deficiency (GHD). The Canadian GH Advisory Committe e registry was initiated in 1967 to include all persons in Canada trea ted with pituitary-derived GH (1967-1985). Since 1985, the registry ha s been maintained for continuous surveillance of those treated with bi osynthetic GH. Thirty-seven children have died out of a total of 1366 children treated for GHD in the 25 years up to December 31, 1992. Indi vidual cases were reviewed for circumstances before death and autopsy information. The likelihood of individual deaths being caused by poten tially preventable endocrine causes was graded on a scale of 1-5. Surv ival curves were analyzed for the children with idiopathic GHD and cra niopharyngioma. Age- and sex-specific mortality rates for children wit h idiopathic GHD were compared with those of the general population. T he overall elude mortality rate was 2.7%. The most frequent cause of m ortality was tumor recurrence (11/37). A surprisingly high proportion of deaths (9/37) were caused by the preventable endocrine complication s of adrenal crisis and hypoglycemia. Children with idiopathic GHD rec eiving GH therapy had similar age- and sex-specific mortality rates co mpared with general population rates, except in a high-risk subgroup o f males diagnosed with GHD before 2 yr of age. The highest mortality o ccurred in children with GHD secondary to craniopharyngioma. We conclu ded that preventable sudden deaths caused by adrenal crisis continue t o occur in children with hypopituitarism. A high level of vigilance mu st be maintained in this population.