Endocrinological evaluation of GH deficient patient with acromegaloidism showing excessive growth.

In this report we describe the first case of a girl with acromegaloidism in Japan. She had large and coarse facial features with acral enlargement accompanying height overgrowth; these resemble the manifestations of acromegaly and gigantism due to growth hormone (GH) overproduction. However, pituitary function studies revealed a dysfunction of her GH secretion. Moreover, markedly decreased serum somatomedin C (SM-C) levels also indicated impairment of GH secretion. Therefore, GH and SM-C cannot have been responsible for promoting somatic growth. However, serum alkaline-phosphatase (Al-P) and osteocalcin, were increased, indicating that stimulation of bone metabolism was increased without GH and SM-C effects. The patient is a typical case showing growth without GH, and these data suggest the existence of an unidentified growth promoting factor that is independent of GH and SM-C.

[1]  A. Olney Overgrowth syndromes. , 1990, Pediatric annals.

[2]  N. Katsumata,et al.  Serum insulin-like growth factor I (somatomedin-C) level in normal subjects from infancy to adulthood, pituitary dwarfs and normal variant short children. , 1988, Endocrinologia japonica.

[3]  E. Canalis,et al.  Growth factors and the regulation of bone remodeling. , 1988, The Journal of clinical investigation.

[4]  N. Bersch,et al.  GROWTH WITHOUT GROWTH HORMONE: EVIDENCE FOR A POTENT CIRCULATING HUMAN GROWTH FACTOR , 1986, The Lancet.

[5]  H. Bucher,et al.  Insulin-like growth factors I and II, prolactin, and insulin in 19 growth hormone-deficient children with excessive, normal, or decreased longitudinal growth after operation for craniopharyngioma. , 1983, The New England journal of medicine.

[6]  N. Bersch,et al.  A unique growth factor in patients with acromegaloidism. , 1983, The Journal of clinical endocrinology and metabolism.

[7]  R. Mims Pituitary function and growth hormone dynamics in acromegaloidism. , 1978, Journal of the National Medical Association.

[8]  David W. Smith,et al.  A new overgrowth syndrome with accelerated skeletal maturation, unusual facies, and camptodactyly. , 1974, The Journal of pediatrics.

[9]  H. Friesen,et al.  Prolactin and somatomedin in hypopituitary patients with "catch up" growth following operations for craniopharyngioma. , 1973, The Journal of clinical endocrinology and metabolism.

[10]  J. Beckwith Macroglossia, omphalocele, adrenal cytomegaly, gigantism, and hyperplastic visceromegaly , 1969 .

[11]  J. Crawford,et al.  CEREBRAL GIGANTISM IN CHILDHOOD. A SYNDROME OF EXCESSIVELY RAPID GROWTH AND ACROMEGALIC FEATURES AND A NONPROGRESSIVE NEUROLOGIC DISORDER. , 1964, The New England journal of medicine.

[12]  W. Berardinelli An undiagnosed endocrinometabolic syndrome: report of 2 cases. , 1954, The Journal of clinical endocrinology and metabolism.