SUN-457 Congenital Growth Hormone Deficiency Associated with Hip Disorders in Children and Adolescents

Abstract

Growth hormone deficiency (GHD) is the commonest pituitary hormone deficiency and may require treatment with recombinant human GH (rhGH) for a variable period, ranging from a few years to lifelong. In this respect, surveillance for the long-term side-effects of rhGH treatment is important. Orthopedic complications may occur with rhGH, but some patients may develop hip disorders before rhGH is started, suggesting that untreated GHD/hypopituitarism may be associated with hip pathology. We aimed to: 1. describe clinical data of patients with congenital GHD receiving rhGH associated with hip dysplasia or Legg-Calve-Perthes disease (LCPD) 2. identify those patients with GHD who presented with hip dysplasia before rhGH was initiated in an attempt to determine whether GHD and hip dysplasia occur coincidentally, or if the hip dysplasia could be a manifestation of the underlying neurodevelopmental disorder; 3. determine whether patients receiving rhGH develop LCPD as an adverse effect or not. We performed a retrospective study of pediatric and adolescent patients seen between 1992 to 2017 with congenital GHD and hip disorders. Data were collected through a review of the patients’ medical records. From over 300 eligible patients with congenital GHD followed-up by a single physician in a single tertiary center, 12 patients were included in this study. Ten patients had septo-optic dysplasia (SOD) and 2 had multiple pituitary deficiencies with pituitary stalk interruption syndrome. Hip dysplasia was present in 9 patients and was diagnosed at a mean age of 5.52 years (range 0.86-13.54). These patients had no traumatic births. Five (56%) patients had acetabular dysplasia, 1 (11%) patient had subluxated hips and 3 (33%) had dislocated hips. rhGH was started at an average age of 5.60 years (range 1.94-13.16). Four patients (44%) had developed hip dysplasia on average 4.53 years (0.53-10.15) prior to initiating rhGH treatment. All 4 had bilateral hip dysplasia and isolated GHD. LCPD was present in 3 patients (25%) and was diagnosed at 3, 15 and 18 years old. rhGH was started at age 4, 10 and 14 years, respectively. One patient had developed LCPD one year before rhGH was commenced and did not progress. In 2 patients, LCPD was diagnosed after rhGH was started and did temporarily progress in one of them, but rhGH was not discontinued because LCPD did not seem to be related to rhGH treatment. In conclusion, this study suggests that hip dysplasia could be a manifestation of an underlying GH deficiency, either isolated or in combination with other pituitary hormone deficiencies and complex midline disorders. Additionally, rhGH may not necessarily be causative in patients with hip dysplasia or LCPD receiving rhGH. Nevertheless, we recommend monitoring for development of hip dysplasia in children on rhGH treatment.