Magnetic resonance imaging of CNS in 15 043 children with GH deficiency in KIGS (Pfizer International Growth Database)

Abstract

Objectives: Neuroimaging has become an essential part of the diagnostic process in children with GH deficiency (GHD). The aim of the study was to document the frequency of neuroanatomical abnormalities in a very large cohort of children with GHD and to relate these findings to patient clinical characteristics. Design and methods: Results of magnetic resonance imaging (MRI) were reported in 15 043 of 43 725 children with non-acquired GHD (idiopathic, neurosecretory dysfunction (NSD) and known congenital cause) who were enrolled in KIGS (Pfizer International Growth Database) between 1987 and 2011. Clinical characteristics of patients before GH treatment with normal MRI (idiopathic GHD (IGHD) and NSD) were compared with those of patients with abnormal pituitaries (hypoplasia, empty sella (ES), HME (hypoplastic anterior pituitary, missing pituitary stalk and ectopic posterior pituitary)). Results: Abnormal MRIs were found in 4032 (26.8%) children, within which ES (nZ1178 (7.8%)) and HME (nZ1019 (6.8%)) were the most frequent findings. In 2361 children diagnosed as IGHD or NSD before MRI examination, anatomical abnormalities ((pituitary hypoplasia: nZ974); (HME: nZ459)) were documented. Patients with anatomical abnormalities had more severe characteristics of GHD: normal MRI ! pituitary hypoplasia ! ES ! HME. Conclusions: GHD is associated with a great variety of neuroanatomical abnormalities as identified by MRI. The investigation and evaluation of MRI need to be conducted in a structured mode. There is an association between anatomical and functional abnormalities of the pituitary.

Auxologic parameters and response to 2-year therapy with recombinant human growth hormone in growth hormone deficient children with an ectopic posterior pituitary

“…Bone age delay was another differential factor, which was significantly greater in the EPP group as compared to the nP group, similarly to the studies by Maghnie et al 5 Moreover, significant bone delay was maintained in the following months of the rhGH therapy in the EPP group, but not in the nP group. This could be explained in part by slower or no pubertal progress in the EPP patients, who may manifest combined pituitary hormonal deficiency, including gonadotropins.…”

“…Maghnie et al proposed that the final height depends on the prepubertal height velocity and on the extent of the height deficit at the start of the rhGH therapy. 5 Significant improvement of height in the first year of treatment in both groups in our study may be considered as a favorable prognostic factor of adult stature.…”

“…4 Moreover, Maghnie et al found bone age to be more delayed in this group of patients. 5 By contrast, Zenaty et al reported that auxological indices were comparable in the prepubertal period, regardless of the pituitary image. 20 in our study, both chronological age and the pubertal stage were similar in the two analyzed groups, while in addition no significant differences between the groups were observed in terms of height velocity in the pre-treatment period.…”

“…12-14 among auxiliary diagnostics methods, MRi has been gaining an apparent role as an indicator of hormonal deficiencies of the pituitary. 4,5,[15][16][17] according to the guidelines of several endocrine societies, GH stimulation tests are not necessary in the event of co-existence of clinical signs of GHD and structural anomalies of the hypothalamic-pituitary area in the MRi. 2 Pituitary stalk interruption syndrome with ectopia of the posterior pituitary (PSiS), characterized by the hyperintensive T1 signal, is one of the typical qualitative alternations in the MRi related to GHD.…”

“…an increasing number of reports suggest that magnetic resonance imaging (MRi) of the hypothalamic-pituitary area could be a more accurate marker of the deficiency of tropic hormones as compared with laboratory findings. [3][4][5] Pituitary stalk interruption syndrome (PSiS), visualised by MRi, is one of several specific congenital midline defects and is considered to be indicative of combined pituitary deficiency. it is defined as a triad consisting of a very thin or interrupted pituitary stalk, an ectopic posterior pituitary (EPP) and hypoplasia or aplasia of the anterior pituitary.…”