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Introducción: La diabetes mellitus tipo 1 es una de las enfermedades crónicas que se producen con más frecuencia en la infancia. La mortalidad global de la cetoacidosis diabética en pediatría se ha estimado en un 1-2 por ciento. En este trabajo se presenta, de manera retrospectiva, nuestra experiencia en el trata-miento de dicha entidad. Pacientes y métodos: Durante los años comprendidos entre 1982 y 1996 se han estudiado 40 pacientes diabéticos, cuyo inicio fue en forma de cetoacidosis. Se ha analizado sexo, edad, cifras plasmáticas de glucosa, BUN (mg/dL), Na, K, bicarbonato, hiato aniónico en miliequivalentes por litro (mEq/L), osmolalidad en miliosmoles por kilogramo (mOsm/kg), pH y tasas de HbA ( por ciento) en el diagnóstico. Además, la cantidad que se aporto de insulina (Ul/kg), líquidos intravenosos (ml/kg), Na, K y bicarbo-nato (mEq/kg) durante las primeras 24 horas. También, el tiempo (horas) en que la glucemia descendió hasta 200 mg/dL, la cetonuria se hizo negativa, el pH ascendió a 7,30 y el bicarbonato alcanzó cifras de 15 mEq/L. El análisis estadístico de las variables cuantitativas se presenta, como media y desviación típica. Resultados: De los 40 pacientes, 24 eran niñas (60 por ciento) y 16 niños (40 por ciento). La edad en el diagnóstico fue de 9,17 ñ 4,18 años. El pH fue de 7,11 ñ 0,13. Los valores de bicarbonato, Na, K, glucosa, BUN, la osmolalidad y el hiato aniónico fueron de 7,8 ñ 4,5; 139,6 ñ 6,4; 4,2 ñ 0,8; 448,6 ñ 134,5; 14,4 ñ 5,3; 297,1 ñ 12,3 y 28,9 ñ 8,9, respectivamente. Las tasas de HbA 1c fueron de 11,8 ñ 2,8.Los aportes de insulina, líquidos, Na y K fueron de 1,52 ñ 0,7; 107,2 ñ 58,51; 7,1 ñ 4,2 y 2,6 ñ 1,6, respectivamente. Veintisiete pacientes recibieron bicarbonato intravenoso a 3,9 ñ 2,2. El tiempo de recuperación de los valores de pH, de bicarbonato y de glucosa fueron de 10,9 ñ 8,7; 10 ñ 7,6, y 11,9 ñ 9,9 horas, respectivamente. La negatividad de la cetonuria se alcanzó en 56,2 ñ 35,8.Comentarios: Con el tratamiento aplicado no hubo ningún fallecimiento. Como complicaciones se observaron 5 hiponatremias, 5 hipocaliemias y una hipernatremia. Tan sólo una paciente presentó edema cerebral (AU)

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OBJECTIVE: To determine bone mineral density (BMD) at axial and appendicular sites in patients with type 1 diabetes mellitus and evaluate its relationship with metabolic control and disease duration. PATIENTS AND METHODS: BMD was measured by dual-energy X-ray absorptiometry (Hologic QDR-1000) in the lumbar spine (L1-L4) and at the distal third forearm in 246 healthy non-diabetic children and adolescents (111 boys, 135 girls, aged 2.8-20.8 years) and in 45 diabetic patients (18 boys, 27 girls, aged 5.2-19.4 years). The results were expressed as the mean and standard deviation. The differences were tested by analysis of variance or Students t-test, as appropriate. The relationship between BMD and the remaining variables was studied by simple Pearsons coefficient and partial correlation coefficient. Significance was defined as p < 0.05. RESULTS: BMD Increased progressively from infancy to adulthood In both populations. Lumbar spine and forearm BMD were significantly lower in diabetic patients than in the healthy non-diabetic children, mainly during pubertal spurt. The greatest differences were found in males and in the trabecular bone. No relationship was found between metabolic control (mean glycosylated hemoglobin, insulin requirement) and duration of diabetes and the BMD in any region studied (p > 0.05). CONCLUSIONS: Pediatric patients with type 1 diabetes mellitus appear to constitute a population at risk of osteoporosis in adult-hood. Diagnosis of osteopenia diagnosis should be established according to densitometric criteria. In this study, metabolic control parameters and duration of diabetes did not enable predetermination of diabetic patients at risk of osteoporosis in adulthood.

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The evolution of congenital primary hypothyroidism has extremely capitalized on early L-T4 substitutive therapy, as a result of neonatal screening techniques, that allow early diagnosis. However, IQ and neurodevelopment of patients diagnosed and treated in this manner are yet quite different from those normal controls. Latest efforts to achieve precocious neonatal diagnosis have led to earlier initiation of treatments. These circumstances, in addition to the use of higher L-T4 doses (between 10 and 15 micrograms/Kg/day) have permitted to improve clinical responses, in terms of QI and neurodevelopment. Still concern remains about the potential late side effects of high L-T4 doses, that could promote conduct and behavior alterations in these patients in the coming years; in this regard, some suspicions have been shown.

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The secular changes in growth of the Spanish male population throughout the current century are presented. Data are adult mean height: 163.4 cm in 1910 and 174.5 cm in 1995. The increase was produced after 1950 and specially after 1965. From this last date, the increase by decade was between 2 and 2.9 cm. Lately the increase is gone on but more slowly. It was possible establish a direct relationship between height and culture and income of population. Maturation of female was studied using the menarcheal age. In 1998 it was 12.5 years. The menarcheal ag is now 0.3 years before than 15 years ago.

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OBJECTIVE: In order to establish the normal patterns of forearm bone mineral density (BMD), BMD in the cortical and trabecular parts of the distal forearm were studied in a normal pediatric population. PATIENTS AND METHODS: BMD was measured by dual-energy X-ray absorptiometry (HOLOGIC QDR-1000) in the distal third forearm of 246 normal children and adolescents (111 boys and 135 girls) ranging from 2.8 to 20.8 years of age. BMD was correlated by multiple regression analysis with age, weight, body mass index (BMI), sex and pubertal Tanner stage. RESULTS: Forearm BMD increased progressively with age, weight, height, BMI and maturity, with the maximal increase in all forearm bone sites occurring at the onset of puberty in girls and boys. A statistically significant correlation was found between forearm BMD and all of these variables (r = 0.65 to 0.92). Mean BMD was higher in boys than in girls in cortical, trabecular and cortical-trabecular sites of the distal forearm. Maximal differences in BMD between boys and girls occurred at 17-18 years of age, especially the trabecular-dominated (ultradistal) part of the distal forearm (0.446 vs 0.384 g/cm2). CONCLUSIONS: Forearm BMD studies permit information of both cortical and trabecular bone mineralization to be obtained at the same time. This study reports normative data for forearm BMD in a healthy pediatric population. The values obtained may be used as a reference of normality when evaluating bone density in situations where skeletal mineralization may be compromised.

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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.

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OBJECTIVE: Our objective was to study children with familial short stature (FSS) to observe whether they develop bone mineralization similar to that seen in healthy children with an adequate height for their age and sex. PATIENTS AND METHODS: The study included 70 FSS patients (39 boys and 31 girls) between 6 and 20 years of age and 246 control patients with the same mean age and sex of the study group. Bone mineral density was measured in the lumbar spine and forearm by performing dual energy X-ray absorptiometry using a Hologic ADR-1000. RESULTS: The main difference between the FSS population and the control group was in the final adult bone mass, which was 20% less in the lumbar spine and 15% less in the forearm in the FSS group. CONCLUSIONS: A large difference in bone mineralization was observed among the FSS population compared to the control group during infancy and adolescence and this becomes accentuated with age or growth resulting in bone mineralization that is not optimum for facing the losses which occur during adulthood.

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A boy with severe symptoms of biotinidase deficiency diagnosed at the age of 12 years showed a remarkable improvement of his neurological picture and normalization of brain magnetic resonance imaging abnormalities when prescribed oral biotin.

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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.

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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.

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Thyroid hormones are fundamental for growth and bone maturity. Retarded physical and osseous development signals congenital hypothyroidism. This study assessed the evolution of height and bone age and final height after hormone replacement treatment in 25 patients with primary congenital hypothyroidism. Bone ages, measured periodically for 12 years after treatment began, were expressed as standard deviation scores (SDS) corresponding to chronologic age. Heights were expressed as SDS and related to standardized curves and genetic height. All patients experienced height recovery during the first year. Of 19 patients who reached their final height, 16 surpassed the expected mean for genetic height. Bone age accelerated progressively, with total recovery toward the third year, and remained accelerated, reaching +1.43 +/- 1.27 in relation to chronologic age 12 years after hormone replacement began. Thus, early diagnosis and adequate treatment of congenital hypothyroidism improved growth and osseous development, although progressive acceleration of bone age may have limited final height in some children.

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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.

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