Avaliação cefalométrica em pacientes adultos com deficiência isolada do hormônio do crescimento tratados com terapia de reposição hormonal

Abstract

In Itabaianinha - Sergipe we identified a large family that has an inactivating mutation in releasing hormone receptor gene of growth hormone, causing isolated deficiency of GH (IGHD). These severe IGHD patients has no deficiency of other pituitary hormones, which causes severe low and proportionate stature. Some of these patients had done during adolescence replacement therapy with GH, resulting in height increased and a study in 2007 observed that these patients clinically presented vertical rise of the face, thereby modifying the proportions and facial profile of these compared to untreated patients, but little is known about its effects on the craniofacial complex. This work, pioneer, aims to characterize the influence of GH on craniofacial growth in eight subjects who underwent hormonal treatment. The measurements were performed by cephalometric analysis of a homogeneous group with genetic and isolated and severe growth hormone deficiency treatment with reposicional comparing them with dwarfism group without replacement, where linear and angular measurements were evaluated reasons. With supplementation of GH, the extent of the measures reduced in this way, all cephalometric measurements were close to normal. The most affected measures with hormone replacement were jaw length and gonial angle. Hormonal therapy with GH increases the linear measurements, but there was no change in angular measurements, and trends presented the normalization of cephalometric measurements.