ESPE2022 Meet the Expert Pediatric Cushing Disease (1 abstracts)
By far, the most common type of CS is due to exogenous causes, i.e. medication-induced CS. Endogenous causes are rare by they are all associated with one form of another of a tumor. We call Cushing Disease (CD) what is caused by pituitary adenomas only. It is common for children with CD to show an increase in rate of weight gain accompanied by a decrease in growth rate (linear height) due to decreased growth hormone secretion. This is often easily detected in growth charts of children with CS. Other symptoms include: rounded face, reddened cheeks, acne, headache, excess hair growth (fine downy hair on cheeks, arms, and legs), purplish-pink stretch marks (more common in older children), darkened skin around neck and armpit areas, easy bruising, development of pubic hair at a younger age than usual, irregular or absent menstrual periods, and high blood pressure. Compared to adults with CS, symptoms that are less commonly seen in children include: sleep disruption, mental changes, and muscle weakness. A review of the child’s growth chart is essential: if the growth chart shows an increase in the rate of weight gain and decrease in the rate of linear growth (height velocity) during the same time period, then further evaluation and monitoring is needed to determine the cause. Other diagnostic testing in children with CD is similar to that in adults. Pituitary adenomas are usually treated by surgical removal (transsphenoidal surgery or TSS). In certain patients, treatment with medication(s) that control or block cortisol production, may be done on a short-term basis. In some patients, in those that surgery has failed (or if they were not good candidates for surgery, which is very rare in children but more frequent in adults), radiation therapy is indicated. Various studies report that one year after surgical cure of pediatric CS most children had lost weight and body mass and their height and growth velocity had increased; however final adult height is often impaired (by at least an inch or more). Many children and adolescents recovering from CD experience changes in cognitive performance that can be stressful for both the child and the parents. We have identified several hereditary diseases associated with genetic predisposition to the development of CD (MEN1 and MEN4, Carney complex, succinate-dehydrogenase mutations and others); more genes genetic factors are expected to be identified that may even lead to better medical treatments.
15 Sep 2022 - 17 Sep 2022