Deficiencia somatotrófica en niños: características clínicas y bioquímicas según la etiología en 75 casos / Somatotrophic deficiency in children: report of 75 cases

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.

Treatment of central precocious puberty with triptorelin 11.25 mg depot formulation.

A new triptorelin 11.25 mg long depot formulation is now available for the treatment of central precocious puberty (CPP). The aim of our study was to evaluate the efficacy of triptorelin 11.25 mg administered every 90 days to suppress gonadotropin and sex steroid secretion and pubertal signs in children with CPP during 2 years of treatment. Inclusion criteria were clinical pubertal development before the age of 8 years in girls or 9 years in boys, advanced bone age and a pubertal LH response (peak >5 mIU/ml) to GnRH. We studied 20 patients (19 girls and 1 boy), with a median age at entry into the study of 7.5 +/- 0.2 years for girls, and 9 years for the boy. The basal and GnRH-stimulated serum levels of LH and FSH decreased significantly from baseline to 3 months of therapy (p <0.0001). All patients had a GnRH-stimulated peak below 3 mIU/ml between 6 and 24 months of treatment. The pituitary-gonadal axis recovered adequately after discontinuation of therapy. These results suggest that 3-month depot triptorelin is a satisfactory alternative for the therapy of children with CPP. The longer interval between injections may increase acceptability and compliance with treatment.

GH-IGF axis during catch up growth in small for gestational age (SGA) infants.

The hormonal profile in 47 small for gestational age (SGA) term newborns during their first year of life was studied. The newborns had a mean birth weight of 2290 +/- 230 g and a length of 45.5 +/- 2.0 cm, and they were followed up every month. Serum IGF-I, IGF-II, and urinary growth hormone (u-GH) concentrations were measured at 3 days of age and every 3 months during one year. Serum IGFBP-3 levels were measured at 3 and 6 months of age. Catch up growth (CUG) was defined as an increase in length z score greater than 1 SD between birth and 6 months of age. According to this definition, 27 infants (57.4%) experienced CUG. We compared the hormonal profile of the infants who demonstrated evidence of CUG [CUG(+)] with those who did not [CUG(-)]. Serum IGF-II levels were significantly higher in CUG(+) infants compared to CUG(-) infants at 3 months of age. We did not find any differences in serum IGF-I, IGFBP-3 and urinary GH between CUG(+) and CUG(-) infants at any time during the study.

Isolation of a bovine uterotropic placental factor and development of an enzyme immunoassay in peripheral serum.

Bovine uterotropic placental factor (bUTPF) was obtained from bovine term placentae that had been homogenized and extracted in acidic medium. The extracts were chromatographed on Sephadex G-75, a fraction named native bUTPF was obtained, and an antiserum was generated. The native bUTPF was chromatographed on Sephacryl S-300 HR, and a 230-kDa fraction was obtained. A competitive enzyme immunoassay (EIA) was developed to determine bUTPF levels during gestation. This EIA was linear between 0 and 80 micrograms/ml (with a detection limit of 0.2 microgram/ml) and showed intraassay and interassay coefficients of variation of 11.2% and 15.5%, respectively. Bovine UTPF serum levels during gestation were determined by means of a cross-sectional study between the first month of gestation and delivery. Maximal serum levels of bUTPF were found during the first month of gestation: 7.1 +/- 1.9 micrograms/ml (p < 0.05). In another cross-sectional study, blood samples were collected from 105 cows between 8 and 25 days postinsemination (day of insemination = Day 0). Levels of bUTPF were found to be significantly higher (p < 0.05) by Day 17 compared to Day 0 (5.8 +/- 2.2 micrograms/ml vs. 1.2 +/- 0.7 micrograms/ml). We conclude that bUTPF is detected by EIA in maternal peripheral serum early during gestation in the bovine species.

[Delayed constitutional puberty or hypogonadotrophic hypogonadism?]. / Pubertad retrasada constitucional o hipogonadismo hipogonadotrófico?

This review is focused on the diagnosis, clinical and general therapeutic approach of constitutional growth and puberty delay and hypogonadotrophic hypogonadism in males, two entities that are difficult to distinguish. Clinical history and physical examination must be carefully performed. Delayed puberty is due to constitutional growth and puberty delay in the vast majority of children. These must be distinguished from a small fraction of boys with hypogonadism, a pathological condition. A number of laboratory test allow the prediction of puberty onset and progression. Nevertheless, the advent of highly sensitive immunoassay and radiometric immunoassay systems for LH, FSH and testosterone has not entirely solved the problems, since their values may overlap between normal and pathological conditions.

Chromatographic, immunochemical and electrophoretic studies of human uterotrophic placental factor (hUTPF).

The human uterotrophic placental factor (hUTPF) is a protein obtained from human term placentae and acts on uterine growth, mammary gland, and blastocyst development and implantation. In the present work, we further define some molecular characteristics of hUTPF using chromatographic, electrophoretic and immunochemical methods. It is concluded that in human term placenta a high molecular weight hUTPF is present, bound to albumin and immunoglobulins, which could represent a storage or transport form of this factor. hUTPF presents several molecular forms, one of them of 270 kDa and others of approximately 90 kDa and 27 kDa.

Chromatographic, immunochemical and electrophoretic studies of human uterotrophic placental factor (hUTPF)

The human uterotrophic placental factor (hUTPF) is a protein obtained from human term placentae and acts on uterine growth, mammary gland, and blastocyst development and implantation. In the present work, we further define some molecular characteristics of hUTPF using chromatographic, electrophoretic and immunochemical methods. It is concluded that in human term placenta a high molecular weight hUTPF is present, bound to albumin and immunoglobulins, which could represent a storage or transport form of this factor. hUTPF presents several molecular forms, one of them of 270 kDa and others of approximately 90 kDa and 27 kDa

Bocio y tiroiditis linfocitaria crónica / Goiter and chronic lymphocitic thyroiditis

Se estudiaron 60 pacientes con bocio, de 3 a 16 años de edad, 58 eran mujeres, 38 (63%) de los cuales presentaba dos o más elementos sugerentes de tiroiditis linfocitaria crónica (TLC) (criterio de Fisher) destacando en ellos el compromiso de la función tiroidea 18/38 (47,4%) con hipotiroidismo y 7/38 (18,4%) con hipertiroidismo. Los demás pacientes con bocio difuso, que no cumplían los criterios de Fisher, fueron denominados "bocio no tiroiditis" (37%) y eran eutiroideos en proporción de 95%. En todos los pacientes con probable TLC se detectaron anticuerpos antimicrosomales en títulos relativamente altos. Sólo 32% tenían simultáneamente anticuerpos antimicrosomales y antitiroglobulinas positivos. Entre los 22 pacientes con "bocios no tiroiditis", 6 (28%) tenían anticuerpos antimicrosomales positivos, en títulos bajos (1 x 100 y 1 x 40, respectivamente). En un grupo control de 28 niños de edades similares a los propósitos, sin enfermedades endocrinas ni antecedentes familiares de afecciones tiroideas, sólo 3 (11%) presentaron anticuerpos positivos, en títulos bajos

[Goiter and chronic lymphocytic thyroiditis]. / Bocio y tiroiditis linfocitaria crónica.

Sixty patients with goiter, aged 3 to 16 years, 58 girls, were studied for evidence of chronic lymphocytic thyroiditis (CLT). Thirty eight patients, 63%, presented two or more diagnostic elements of TLC, according to Fisher's criteria, with a high frequency of thyroid function involvement (47.4% had hypothyroidism and 18.4% had hyperthyroidism). The rest of the patients with diffuse goiter (37%) did not meet Fisher's criteria, they were mostly euthyroid (95%) and they were designated "non thyroiditis goiter". All patients with probable CLT had positive antimicrosomal antibodies at relatively high titer (greater than 1 x 600 in 71% of the cases) and 32% of them had both antimicrosomal and antithyroglobulin antibodies. In the "non thyroiditis goiter" group we found 28% of children with positive antimicrosomal antibodies at low titers (1 x 100 and 1 x 400, respectively). In a control group of 28 children of similar ages, without endocrine diseases neither familiar history of thyroid diseases only 3 (11%) cases showed positive antimicrosomal antibodies, always at low serum titers.

[Breast feeding and socioeconomic level of Chilean infants under 1 year of age]. / Lactancia materna y nivel socioeconómico en lactantes chilenos menores de un año.

PIP: 500 mothers of infants under 1 year old who were registered in 2 health service centers in Chile were interviewed between January and July 1982 as part of a collaborative study on breast feeding in Latin America conducted by the Latin American Association of Pediatrics. 5% of the infants were never breast fed, at 3 months approximately 50% were breastfed, and by 6 months only about 28% were breastfed. Classification of households according to ownership of automobiles and other household equipment, education of the household head, and household domestic help showed 6.0% in class A, the highest, 18.2% in class B, 40.4% in class C, 30.2% in class D and 5.2% in class E. 79.9% of infants in all social classes were breastfed at least in the 1st month, but the proportion was negatively related to class. 59.4% in class A were breastfed, compared to 69.1% in B, 84.8% in C, 82.6% in D, and 92.0% in E. Educational level and marital status of the mother were not related to breastfeeding practice. The relationship of employment outside the home could not be determined since 440 of the women were not in the labor force at the time of interview. 41.6% of mothers not breastfeeding cited lack of milk and 19.4% illness as the reason. 81.3% of mothers receiving prenatal care breastfed their babies compared to 52.4% who did not. 43.1% of the study children were aged 5 months or under. 79% of the mothers were aged 20-29 years. 33% discontinued breastfeeding because of declining milk supply, .48% for esthetic reasons, 3.3% for medical reasons, and 7.2% because of rejection by the infant. The introduction of other foods was cited by 24.3% as the reason for discontinuing breastfeeding.^ieng