Deficiencia somatotrófica en niños: características clínicas y bioquímicas según la etiología en 75 casos / Somatotrophic deficiency in children: report of 75 cases
Rev. chil. endocrinol. diabetes
;
1(1): 7-16, ene. 2008. ilus, tab
Article
in Spanish
| LILACS
| ID: lil-612498
ABSTRACT
Somatotrophic deficiency (SDMT) can be due to a deficiency of growth hormone releasing hormone(GHRH), growth hormone (GH) or insulin like growth factor I (IGF-I). Although its clinical features have been thoroughly described, the diagnosis is still controversial. Now there is an effective treatment with GH or IGF-I for these patients. AIM:
To analyze the main clinical, etiological and laboratory characteristics of 75 SD patients (44 males), aged 9.4 + 4.5 years, with severe growth retardation. The diagnosis was confirmed by the lack of response to two GH stimulation tests (Clonidine, Glugagon or Insulin) and low levels of IGF-I or insulin-like growth factor binding protein- 3 (IGFBP-3).RESULTS:
In 34 patients (46 percent), the cause of DSMT was considered idiopathic (DSMT-I), in 31 (41 percent) there was an organic cause (DSMT-O), most commonly caused by malformations or pituitary tumors and in 10 (13 percent), it was genetic (DSMT-G) (three patients with Laron's Syndrome, five with mutations of GH gene and 2 with probable mutations of Prop-1 and Pit-1 genes). IGF-1 levels, were significantly lower in DSMT-O and DSMT-G thanin DSMT-I (21.2 +/- 46.1, 23.4 +/-30.3 ng/mL and 50.2 +/- 48.3 ng/mL, respectively). The lowest height score corresponded to DSMT-G, compared to DSMT-O and DSMT (5.7 +/- 0.9, -4.0 +/- 1.6 and 4.3 +/- 1.2 DS, respectively)CONCLUSIONS:
The high percentage of organic and genetic etiologies in our patients can be due to the systematic search of these diseases. DSMT-G (Laron, mutations in GH and Pit-1 genes) had the most severe growth retardation.
Full text:
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Index:
LILACS (Americas)
Main subject:
Body Height
/
Growth Hormone
/
Growth Disorders
Type of study:
Etiology study
/
Observational study
Limits:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Country/Region as subject:
South America
/
Chile
Language:
Spanish
Journal:
Rev. chil. endocrinol. diabetes
Journal subject:
Endocrinology
Year:
2008
Type:
Article
Affiliation country:
Chile
Institution/Affiliation country:
Clínica Santa María/CL
/
Hospital Exequiel González Cortés/CL
/
Instituto de Investigaciones Materno Infantil/CL
/
Pontificia Universidad Católica de Chile/CL
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