Hereditary gingival fibromatosis and growth retardation.
OBJECTIVE
To describe two patients with hereditary gingival fibromatosis (HGF) and growth hormone deficiency and to review the literature on HGF and related endocrine abnormalities.


METHODS
We present case reports of two patients (first cousins)-an 8-year-old girl and a 13-year-old boy-with an existing diagnosis of HGF, who were assessed because of presumed growth failure. Both patients underwent growth hormone stimulation testing and more in-depth endocrine evaluation, including measurement of morning cortisol, adrenocorticotropic hormone (ACTH), and prolactin levels as well as thyroid function tests. An ACTH stimulation test was also performed. Radiologic evaluation included assessment of bone age and magnetic resonance imaging of the brain.


RESULTS
In addition to HGF, both patients had short stature, subnormal growth velocity, and delayed bone age but no abnormalities on magnetic resonance imaging of the brain. Serum prolactin levels and results of thyroid function tests were normal. Subnormal growth hormone response was noted during hypoglycemia and pharmacologic stimuli with clonidine and levodopa. The female patient, who also had recurrent hypoglycemic episodes, had a suboptimal cortisol and ACTH response during hypoglycemia. On the ACTH stimulation test, she showed an inadequate cortisol response at 30 minutes but a normal response at 60 minutes. The male patient had normal morning cortisol and ACTH levels plus a normal response to ACTH stimulation. Both patients are responding well to treatment with growth hormone. The girl is also receiving cortisol replacement and has had no further episodes of hypoglycemia.


CONCLUSIONS
Although HGF has been described as an isolated finding, it can occur as part of a syndrome, including infrequent endocrine abnormalities such as growth hormone insufficiency. The cause of the growth hormone deficiency remains unclear in these two patients. We believe that patients with HGF should be monitored carefully for a prolonged period for growth as well as other endocrine abnormalities.
Bhowmick
S K
SK
Department of Pediatrics and Adolescent Medicine, Division of Endocrinology and Metabolism, University of South Alabama, 2451 Fillingim Street, MSTN 212, Mobile, AL 36617-2297, USA.
Gidvani
V K
VK
Rettig
K R
KR
D000293:Adolescent; D001827:Body Height; D002648:Child; D005260:Female; D005351:Fibromatosis, Gingival / Q000150:complications / Q000235:genetics* / Q000601:surgery; D005890:Gingivectomy; D019382:Human Growth Hormone / Q000172:deficiency* / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007003:Hypoglycemia / Q000150:complications; D007659:Ketones / Q000652:urine; D008297:Male
D007659:Ketones; D019382:Human Growth Hormone; D006854:Hydrocortisone