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At a glance

Gigantism caused by excessive growth hormone (GH) secretion usually because of a pituitary adenoma that normally occurs before the epiphyses are fused in comparison with acromegaly that occurs after fusion of the epiphyses. The onset of the disease is usually prior to puberty and is often associated to muscle weakness, headache, visual defects, and articulation pain. Males are more commonly affected.

Synonyms

Acromegaloid Gigantism; Acromegalic Gigantism; Fractional Hypopituitarism-Gigantism Syndrome; Neurath-Cushing Syndrome; Pituitary Gigantism Syndrome.

History

This medical condition was first described by Pierre-Emile Launois in 1899. Dr Launois was a Professor of histology in 1898 and worked exclusively in Paris at the Hôpital Lariboisière. He dedicated his career to the study of the pituitary gland.

Incidence

The exact incidence remains unknown. Must be suspected in children with very tall stature. Reports are limited to sporadic cases. Peak incidence in teenagers, although it has been reported in a child as young as 21 months.

Genetic inheritance

It is caused by a pituitary adenoma. In a recent international, retrospective, multicenter study 208 patients (163 males; 78.4%) with GH excess and a current/previous abnormal growth velocity for age or final height greater than 2 standard deviation (s.d.) above country normal means were reviewed. The median onset of rapid growth was 13 years and occurred significantly earlier in females. The pituitary adenomas were diagnosed earlier in females than males (15.8 vs. 21.5 years, respectively). Final height was greater when it affected younger patients at the time of the onset. Larger tumors and higher GH levels were also reported. AIP mutations and X-LAG explain many cases.

Pathophysiology

Almost always caused by pituitary adenoma producing excessive GH. Some tumors are mixed and produce both prolactin and GH. Very rarely, it is caused by excessive secretion of GH-releasing factor from an ectopic source.

Clinical aspects

Rapid linear growth, tall stature, large hands and feet, coarsening of facial features, prognathism, macroglossia widely spaced teeth, and husky voice. Other features include greasy thick skin, slipped epiphyses with joint pain, kyphoscoliosis myopathy, nerve entrapment syndromes; headaches and visual field defects, usually hemianopsia, pale optic discs and optic atrophy. Patients may have behavioral problems and mental retardation early pubertal signs, galactorrhea, glucose intolerance, obesity, or diabetes mellitus.

Diagnosis

Clinical features; biochemical (elevated serum levels of GHs not suppressed by oral glucose loading, elevated levels of insulin-like growth factor-1 [IGF-1], high prolactin and gonadotropin levels); radiology (advanced bone age, enlarged sella turcica on skull radiograph, CT scan demonstrates pituitary tumor).

Anesthetic considerations

May be anxious and uncooperative in the presence of visual defects, mental retardation, or behavioral problems. Airway problems should be anticipated, but, unlike acromegaly, ...

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