Etiologies and Clinical Presentation of Gigantism in Algeria

Abstract

Background/Aims: True gigantism is an exceptional and fascinating pediatric disease. Our aim in this study was to describe the different etiologies of a large group of children with gigantism and the natural history of their growth. Methods: In this multicenter study, we considered as giant children, adolescents and adults whose heights were ≥3 SD compared to their target stature or to our population average lengths. Isolated hypogonadism and Klinefelter syndrome were excluded from this series. All underwent clinical exam, and hormonal and neurological investigations. Results: From 1980 to 2010, we observed 30 giants: 26 males (86.6%) and 4 females (mean age 19.8 ± 11 years). Among the 13 patients (40.3%) who consulted before the age of 16 years, 9 had acromegaly and 6 had mental retardation and body malformations. Based on growth hormone (GH) secretion evaluation, 2 groups were observed: pituitary gigantism (n = 16): GH = 150 ± 252 ng/ml (n ≤ 5), and other causes with normal GH (0.7 ± 0.6 ng/ml): 6 Sotos syndrome and 8 idiopathic cases. Only the first group had neurological, ophthalmological, metabolic and cardiovascular complications and received treatment. The result was not optimal as GH normalization was not observed. Reduction of tumor size and decreased GH plasma values were not observed. Conclusion: Gigantism predominates in males. The main cause is GH excess. The diagnosis was very late except for cerebral gigantism. Complications were observed in pituitary gigantism only.

Gigantism: Microsurgical treatment by transsphenoidal approach and prognostic factors

“…These facts are also present in this series. A preference of the disease for the male gender has been reported [2,8,9], although others report greater frequency in women [7].…”

“…The clinical picture of gigantism is well known and is caused by an excess of growth hormone (GH) during childhood before epiphyseal closure, causing disproportionate linear growth. The clinical and genetic characteristics of this disease have been thoroughly analyzed [1,2,3].…”

Gigantism: microsurgical treatment by transsphenoidal approach and prognostic factors

“…Gigantism is considered a rare disease and there is evidence in the literature that supports (likely other pituitary adenomas in childhood) that adenomas in giants are usually large and have invasive characteristics [ 7 ]. The appearance of visual disturbances [ 8 ] is not uncommon and a predominance of the disease in males has been published [ 2 , 8 , 9 ], although other articles report higher frequency in women [ 7 ]. These facts are also present in this series.…”

“…The clinical picture of gigantism is well known, and it is caused by an excess of growth hormone (GH) during childhood, before epiphyseal closure, causing disproportionate linear growth. The clinical and genetic characteristics of this disease have been thoroughly analyzed [1][2][3].…”

The Giant of Tripoli: The Case of Late Recognition and Management of an Extreme Acromegalic Gigantism in Resource-Poor Settings

“…However, a few studies are from the Middle East and North Africa. 6,7 The present case illustrates the challenges of managing young patients with GH excess who tend to have a genetic mutation associated with aggressive somatotroph adenoma and suffer from early mortality due to GH-related comorbidities. This case of acromegalic gigantism is likely a result of an AIP gene mutation.…”