[Accuracy of three methods of height prediction in a group of variant short stature children]. / Fiabilidad de tres métodos de predicción de talla adulta en un grupo de tallas bajas variantes de la normalidad.

OBJECTIVE: The objective of this study was to determine how accurately three methods of height prediction estimate adult height in a group of 27 boys and 16 girls with untreated short stature who have reached adult height. Twenty of the patients were considered to have familial short stature, 8 constitutional delay of growth and puberty, 12 a combination of both conditions and 3 idiopathic short stature. PATIENTS AND METHODS: Height predictions at various chronological ages were retrospectively compared to final adult height according to the Tanner-Whitehouse Mark I, Bayley-Pinneau and Roche-Wainer-Thisen methods. Heights were expressed as centimeters and standard deviation scores (SDS) and related to Tanner standardized curves. RESULTS: The final heights reached were significantly below the target height [-2.0 (0.5) SDS versus -1.6 (0.6), p < 0.01]. The three methods over-estimated adult height. The Bayley-Pinneau method was especially accurate in girls and in familial short stature. The Tanner and Bayley-Pinneau predictions were similar in constitutional delay of growth and puberty. The Roche method gave the greatest errors in all groups. CONCLUSIONS: 1) Thee groups of normal variant short stature patients reached an adult height in the lower normal range. 2) The three method of height prediction over-estimated final height. 3) As in some pathological conditions, the Bayley-Pinneau was the most reliable method of height prediction in children with short stature. 4) These children may be considered as a historical group to evaluate the effect of growth-promoting therapies.

[Turner's syndrome. Relationship between the karyotypes and malformations and associated diseases in 23 patients]. / Síndrome de Turner. Relación entre sus cariotipos y las malformaciones y algunos procesos asociados en 23 pacientes.

UNLABELLED: In this study we have assessed the frequency of karyotypes, phenotypes and some associated diseases 23 girls affected with turner's syndrome. Moreover, we have analyzed their relationships. RESULTS: The most important findings the following: 1) The mean age at diagnosis was 7.37 +/- 5.65 (0, 16) years. 2) The most frequent karyotype was monosomy 45XO, which was found in fourteen patients (60.9%), followed by isochromosome of the long arm of chromosome 46 XiXq in five cases (21.7%), two mosaics, one 45 XO/46 XiXq and one 45XO/46XX, and two deletions of the short arm of chromosome X (46 XX Xp-). 3) The classical phenotype was found in 87% of the cases. 4) Bone malformations were found in nine patients (39.1%). The most frequent were, short metacarpals in five cases, knee anomalies (Kosowicz's sign) in four, one Madelung deformity and one alata scapula. 5) Renal malformations were detected in five patients (21.8%), two rotational abnormalities, two horseshoe kidneys and one double collecting system. 6) Cardiovascular malformations were found in four cases (17.3%). Three bicuspid aortic valves and one aortic coarctation were diagnosed. 7) Otitis media was discovered in seven girls (30.5%). 8) Other processes found were, congenital lymphedema in four cases, one Klipell-Trenaunay syndrome, one Dandy-Walker anomaly, one congenital glaucoma, one colesteatoma, one congenital torticolis, one hit luxation and one essential arterial hypertension. A significant correlation was found between karyotype and phenotype, such that all of the patients with monosomies and with mosaics, 66% of those with X isochromosomes and one of the patients with a deletion had a classical phenotype. We found no correlation between the karyotype and the different malformations and associated diseases.

[Etiological, clinical and hormonal characteristics of a group of patients with permanent hypogonadism]. / Características etiológicas, clínicas y hormonales de un grupo de pacientes con hipogonadismos permanentes.

A retrospective study analyzing etiological, clinical and hormonal aspects in a population of 45 patients (14 males and 31 females) with permanent hypogonadism was performed, the most important findings were: 1) The most common cause of hypogonadism was gonadal failure (60% of all patients). This included-twenty-three females and four males. Eighteen patients had XO, two XY and two more XX gonadal dysgenesis. In the remaining cases, three patients had bilateral agonadism and two had testicular atrophy secondary to radiochemotherapy. 2) Eighteen patients had hypogonadotropic hypogonadism (40% of the cases). Ten were males and eight females. Eleven patients had gonadotropin deficiency associated with other pituitary dysfunctions. Deficiency of GH was found in all cases. TSH in ten, ACTH in nine and ADH in five. An increase in prolactin was observed in seven patients. The etiology of the hypopituitarism was intracranial tumors in five cases, idiopathic in three, perinatal hypoxemia in two and hypoplastic pituitary in one. In the remaining seven cases, isolated gonadotropin deficiency was found. Four cases were idiopathic, two cases had demyelinating diseases and one beta-thalassaemia. 3) Mean levels of testosterone were 4.20 +/- 6.5 (0, 20) pg/ml. Meal levels of estradiol of the total group, gonadal failure patients and those with hypogonadotropic hypogonadism were 8.51 +/- 14.7 (0, 50), 9 +/- 16 (0, 50) and 7.12 +/- 10.98 (0, 29) pg/ml, respectively. 4) Mean basal levels of LH and FSH in patients with gonadal failure were 35.57 +/- 60.66 (5, 320) and 53.19 +/- 53.92 (4, 230) mUi/ml, respectively. In hypogonadotropic hypogonadism patients, mean basal and peak levels of LH were 0.98 +/- 1.24 (0, 5) and 3.45 +/- 3.94 (0, 12) mUi/ml, respectively. Mean basal and peak levels of FSH after LHRH stimulation were 1.43 +/- 1.88 (0, 6) and 3.85 +/- 4.85 (0, 17) mUi/ml, respectively.

[Accuracy of the methods for height prediction in patients treated for congenital primary hypothyroidism]. / Fiabilidad de los métodos de predicción de la talla adulta en pacientes con hipotiroidismo congénito primario.

OBJECTIVE: The objective of this study was to determine how accurately three methods of height prediction (the Bayley-Pinneau, Tanner-Whitehouse Mark 1 and Roche-Wainer-Thissen methods) estimate adult height in a group of 17 girls and 7 boys treated for congenital primary hypothyroidism. PATIENTS AND METHODS: The patients were diagnosed at a mean chronological age of 1.2 years. Their thyroxine treatment dose ranged between 3.1 and 8.6 micrograms/kg/day. Height predictions at various chronological ages were retrospectively compared to final adult height. The first prediction was made at a chronological age of 7.3(1.1) years, corresponding to the sixth year of treatment. Heights were expressed in centimeters and standard deviation scores (SDS) and related to Tanner standardized curves. RESULTS: The final heights reached were significantly higher than the target heights (-0.5 (0.7) SDS vs -1.1 (0.9) SDS, p < 0.01). The three methods accurately estimated adult height. Tanner and Bayley-Pinneau height predictions were similar. The Roche method over-predicted height at all chronological ages with a mean error which ranged between +2 (3.2) centimeters and +2.8 (2.1) centimeters. CONCLUSIONS: 1) The patients who were diagnosed and treated early for congenital primary hypothyroidism reach an adult height in the normal range, which is probably related to the total recovery of their retarded bone age. 2) In this pathological condition, the Bayley-Pinneau and Tanner-Whitehouse Mark 1 are the most reliable methods of height prediction.

[Vitreous fluorophotometry and nocturnal GH secretion in young insulin-dependent diabetics treated with oral pirenzepine]. / Fluorofotometría vítrea y secreción nocturna de G.H. en jóvenes diabéticos insulino-dependientes tratados con pirenzepina oral.

With the aim of finding a correlation between the blocking of G.H. secretion and the irregularities in the permeability of the hematoretinal barrier, we have studied the average nocturnal G.H. levels (NAGHL) and the vitreous penetration rate (VPR) in five young diabetic patients. These patients, 3 males and 2 females, were between the ages of 12 and 17 years with a mean age of 16.0. They were studies both before and after receiving treatment for one month with an oral nocturnal dose of 0.6 mg/kg of pirenzepine (gastrozepin) and 1 mg/kg during the subsequent five months. We also tried to find a relationship between the VPR post-treatment and the evolution time of their illness and with their BA1C. The most important results found in this study were: 1) the nocturnal oral pirenzepine modified the NAGHL in the study population (10.48 +/- 4.94 vs; 4.34 +/- 2.53 ng/ml; p < 0.05). 2) Ingestion of the aforementioned drug did not affect the VPR (4.84 +/- 2.08 vs 4.53 +/- 2.54 x 10(-6)/min; p > 0.05). We have not found a relationship between the VPR after treatment with either the HBA1C levels or with the evolution time of the illness. Therefore, we conclude that the dose of oral pirenzepine used for 6 months, although it definitely decreases G.H. secretion, does not modify the permeability of the B.H.R. within this group of young diabetics. Hence, we can infer that the G.H. hypersecretion does not seem to have a relationship, at least exclusively, with the development of diabetic retinopathy.

[Nocturnal use of pirenzepine in young insulin dependent diabetics. Results of a 6-months treatment period]. / Uso de pirenzepina oral nocturna en jóvenes diabéticos insulino-dependientes. Resultados tras 6 meses de tratamiento.

Previous studies have demonstrated an elevation in GH in adult insulin-dependent diabetics which can be modified by administration of pirenzepina either IV or orally. In this study we have evaluated the mean nocturnal GH levels (MNGH) and HbA1-C levels in a group of young insulin-dependent diabetics, both before and after treatment with pirenzepina (Gastrozepin). The study population included 8 patients, 6 males and 2 females, between the ages of 12 and 17 years, with a mean of 15.6 years. Pirenzepina was administered during one month at a nightly oral dose of 0.6 mg/kg followed by 5 months of treatment with 1 mg/kg. The most important results obtained in the study are the following: 1) Nocturnal administration of pirenzepina did not significantly modify the MNGH in the study population (10.88 +/- 3.81 ng/ml vs 9.57 +/- 8.25 ng/ml, p > 0.05). 2) This pharmaceutical did not alter the plasma levels of HbA1-C (9.57 +/- 8.25 vs 10.01 +/- 2.30, p > 0.05). However, 5 out of 8 patients had a decrease in their nocturnal GH secretion after pirenzepina treatment. If only the 5 patients that responded to this treatment are considered, the differences in MNGH are significant (10.48 +/- 4.94 before treatment vs 4.35 +/- 2.53 following treatment, p < 0.05). Therefore, we conclude that oral pirenzepina treatment for 6 months, at the doses described, do not consistently decrease GH secretion in young diabetics nor does it decrease HbA1-C values in this group. However, further studies are necessary to establish the possible value of this treatment.

[Nocturnal use of pirenzepine by a group of insulin dependent juvenile diabetics]. / Uso de pirenzepina oral nocturna en jóvenes diabéticos insulino-dependientes.

Different studies have shown an elevation of GH levels in insulin-dependent diabetic adults and its modification after pirenzepine administration. We have studied nocturnal GH secretion (NIGHS) and fructosamine levels before and after one month of administering a nocturnal dose of pirenzepine [Gastrozepin (R.) 0.6 mg/kg] in a group of eight young insulin-dependent diabetics, 6 males and 2 females. The ages of the subjects ranged between 12 and 17 years with a mean age of 15.28 years. The most important findings were: 1) The NIGHS was not modified by pirenzepine administration, 10.88 +/- 3.81 ng/ml/min vs 11.25 +/- 7.90 ng/ml/min, p greater than 0.05. However, two patients showed a clear decrease in their G.H. levels. 2) Plasma fructosamine levels were also unaffected, 409 +/- 101 mmol/l vs 361 +/- 127 mmol/l, p greater than 0.05. However, the levels of five individual patients decreased after pirenzepine administration. We conclude that oral pirenzepine administration at this dose does not modify GH secretion nor improve short term metabolic control of the disease in young insulin-dependent diabetics. Further investigation, using higher doses and for a more prolonged period of time, is necessary to know the long term effects of this treatment.

[Spontaneous and GHRH-induced nocturnal GH secretion in a child population with insulin dependent diabetes mellitus]. / Secreción de G. H. nocturna espontánea y tras GHRH en una población de niños con diabetes mellitus insulino-dependiente.

It has been studied integrated nocturnal secretion of GH after GHRH test in 15 diabetic children and 10 short stature children with normal GH secretion. The most important findings are: 1) The integrated nocturnal secretion of GH was significantly higher in diabetics than in controls (6.27 +/- 3.11 ngrs/ml/min versus 3.06 +/- 1.41 ngrs/ml/min P [symbol; see text] 0.01). 2) After an acute stimulous with GHRH, diabetic population shows an exaggerated secretion of G. H. during the first 90 minutes compared with control population (33.20 +/- 12.41 ngrs/ml/min versus 18.18 +/- 11.09 P [symbol; see text] 0.01). 3) Both higher spontaneous secretion of G. H., and after GHRH test, are independent of metabolic control (mean nocturnal glycemies and HBA1) and evolution time of disease.