Raquitisme, una malaltia del passat? / No disponible

No disponible

Beneficios y efectos secundarios del tratamiento con análogos de la GnRH en la pubertad precoz central / No disponible

No disponible

Frecuencia aumentada de resistencia a la insulina y adrenarquía exagerada en niños con bajo peso al nacer / Increased incidence of insulin resistance and exaggerated adrenarche in low birth weight children

Objetivos: Determinar en niños prepuberales con bajo peso al nacer (peso ≤1,5 desviaciones estándar para edad gestacional), la prevalencia de hiperinsulinismo y de adrenarquía exagerada. Material y métodos: Se ha medido la insulinemia, la glucemia y el nivel de sulfato de deshidroepiandrosterona (DHEAS) en ayunas en 46 pacientes (20 niños, 26 niñas; edad: 7,5 ± 1,3 años) con bajo peso al nacer (niños: 2,3 ± 0,4 kg; niñas: 2,4 ± 0,4 kg). Los resultados obtenidos se han comparado con los valores de una población sana de similar edad y origen étnico. Se define hiperinsulinismo y adrenarquía exagerada como la existencia, respectivamente, de valores de insulina y/o de DHEAS superiores en más de 2 desviaciones estándar al promedio de una población sana (insulinemia >10 UI/mL, DHEAS >82μg/dL en niños y >53,6 μg/dL en niñas). Resultados: El índice de masa corporal (IMC) en la población estudiada es 16,3 ± 2,8 (población de referencia: 16,6 ± 2); la concentración de DHEAS, 44,2 ± 20,8 μg/dL en niños, y 49,9 ± 34,6 μg/dL en niñas; y la insulinemia, 6,6 ± 5,1 mU/L con glucemia normal. La prevalencia de adrenarquía exagerada es del 21% y de hiperinsulinismo del 11%; no se encuentra correlación entre las concentraciones de los parámetros estudiados, el peso al nacer y el IMC. Discusión: La determinación de las concentraciones de DHEAS y de insulina en ayunas ha detectado en niños con bajo peso al nacer una prevalencia del 21 % de adrenarquía exagerada y un 11% de hiperinsulinismo. Dadas las limitaciones de la metodología empleada, posiblemente la prevalencia real de estas alteraciones sea superior, por lo que es necesario realizar estudios prospectivos longitudinales con mayor número de pacientes que permitan la identificación precoz de esta enfermedad

Objectives: To determine the prevalence of hyperinsulinism and exaggerated adrenarche in prepubertal children who had a low birth weight (≤1.5 standard deviations) for their gestational age. Material and methods: We determined the fasting insulin, blood glucose and dehydroepiandrosterone sulfate (DHEAS) levels in 46 patients (20 boys and 26 girls; mean age 7.5 ± 1.3 years) with low birth weight (boys, 2.3 ± 0.4 kg; girls, 2.4 ± 0.4 kg). The results obtained were compared with those observed in a healthy population of similar age and ethnicity. Hyperinsulinism and exaggerated adrenarche were defined as the presence of insulin and DHEAS levels, respectively, more than two standard deviations higher than the mean of a healthy population (insulin >10 IU/mL; DHEAS >82 μg/dL in boys and >53.6 μg/dL in girls). Results: The mean body mass index in the study population was 16.3±2.8 (reference population, 16.6±2), the mean DHEAS concentration was 44.2 ± 20.8 (μg/dL in boys and 49.9 ± 34.6 μg/dL in girls, the mean insulin level was 6.6 ± 5.1 mU/L and the blood glucose levels were normal. The prevalence of exaggerated adrenarche was 21% and that of hyperinsulinism was 11%. No correlations were observed between the concentrations obtained and birth weight or body mass index. Discussion: The determination of the fasting DHEAS and insulin concentrations revealed an incidence of exaggerated adrenarche of 21% and of hyperinsulinism of 11%. Given the limitations of the methodology employed, the true prevalence of these disorders may be higher. Thus, prospective, longitudinal studies with a larger number of patients should be carried out as they may enable the early detection of these disorders

Influencia del peso al nacer sobre el inicio y progresión de la pubertad y la talla final en la pubarquia precoz / Influence of birthweight on the onset and progression of puberty and final height in precocious pubarche

Antecedentes: Las niñas con bajo peso al nacer (BPN), peso al nacimiento inferior a 1,5 desviaciones estándar de la media (SDS) presentan un mayor riesgo de pubertad adelantada y progresión rápida de la misma que puede ocasionar una talla final baja. Por otro lado, la pubarquia precoz idiopática no parece tener efectos adversos sobre el inicio de la pubertad y/o sobre la talla final. Objetivo: Valorar la relación entre el peso al nacer, el inicio y la progresión de la pubertad, y la talla final en las niñas con pubarquia precoz. Métodos: Estudio retrospectivo de niñas que han iniciado el desarrollo puberal y tienen el antecedente de pubarquia precoz, es decir, presencia de vello pubiano antes de los 8 años de edad. Resultados: De 124 casos estudiados, 37 son BPN; 61 han presentado menarquia y 44 han finalizado el crecimiento. La pubarquia se presentó a los 6,8 0,8 años; la pubertad se inició a los 9,5 +/- 0,9 años y la menarquia a los 11,7 +/- 0,9 años (media +/- DE). No hay correlación significativa entre el peso al nacer expresado en SDS y la edad de la pubarquia, la edad ósea al diagnóstico de pubarquia, el inicio de la pubertad y la edad de la menarquia. Existe una correlación positiva entre peso al nacer y talla en la pubarquia, inicio puberal, menarquia y talla final. Estos datos sugieren que en las niñas con BPN la pubertad progresa más rápidamente. Todas las pacientes alcanzan su talla diana familiar, pero sólo las de adecuado peso al nacimiento (ADPN) superan la materna. Conclusiones: La pubarquia precoz no parece modificar la edad de inicio ni la velocidad de progresión de la pubertad, ni la talla final en las niñas con ADPN. En cambio, en las niñas con BPN la pubarquia precoz se asocia con un curso más rápido del desarrollo puberal y una talla final más baja. Así pues, las niñas con pubarquia precoz y con BPN requieren una atención especial (AU)

Informática y endocrinología / No disponible

No disponible

[Influence of birthweight on the onset and progression of puberty and final height in precocious pubarche]. / Influencia del peso al nacer sobre el inicio y progresión de la pubertad y la talla final en la pubarquia precoz.

BACKGROUND: Girls born small for gestational age (SGA), with a birth weight < 1.5 SDS, are at increased risk of early onset and rapid progression of puberty, with reduced final height. In contrast, idiopathic precocious pubarche (PP) does not seem have negative effects on the onset of puberty or final height. OBJECTIVE: We evaluated the relationship between birthweight, the onset and progression of puberty and final height in girls with PP. METHODS: We performed a retrospective study of girls at the onset of puberty with a history of PP (pubic hair before the age of 8 years). RESULTS: A total of 124 girls (37 SGA) were investigated; 61 had presented menarche and 44 had reached final height. The mean ( SD) age was 6.8 0.8 years at pubarche, 9.5 0.9 at the onset of puberty and 11.7 6 0.9 at menarche. No significant differences were found in the correlation between birth weight expressed in SDS and age at presentation of pubarche, bone age at presentation of pubarche, age at telarche or age at menarche. A positive correlation was found between birthweight and height at pubarche, telarche, and menarche and final height. These data suggest that pubertal progression is faster in SGA girls. All patients reached the familial target height but only those with an appropriate birthweight were taller than their mothers. CONCLUSIONS: PP does not seem to modify age of onset or progression of puberty or final height in girls with adequate birth weight. In contrast, in SGA girls, PP is associated with faster puberal development and shorter final height. Therefore, SGA girls with PP require periodic monitoring.

Long-Term GH therapy: epidemiology and auxologic outcome.

OBJECTIVES: Epidemiologic and auxologic characteristics of patients treated with GH during childhood and adolescence and entered in a national registry in Catalonia were studied between 1988 and 1997. At the end of 1997, prevalence was 53.2 treatments/100,000 inhabitants aged 0-14 years. Maximum annual incidence rates were observed in 1990 and 1991 (34.0-35.6 cases/100,000 inhabitants aged 0-14 years). STUDY DESIGN: Analysis of treatments terminated in 1993 (n = 548) revealed, for the three principal reasons for cessation of treatment ('near-final height', 'adequate height but further growth potential', and 'poor growth response'), that males began and ended treatment at older ages with a better auxologic situation in SDS than girls at the beginning and end of therapy in the first two subgroups, with a similar duration of therapy. Severe GH deficiency (GHD) [both multiple pituitary hormone deficiency (MPHD) and the most severe isolated GHD (IGHD-A)] was more frequent in the group ending treatment at 'near-final height', whereas cessation of therapy because of 'poor growth response' was more frequent in the group with 'other causes of short stature' and no demonstrable GHD by routine tests. In the near-final height group, after excluding Turner's syndrome, MPHD and GHD cases secondary to brain tumors and GH deficiencies associated with malformative syndromes, positive linear correlations were observed between HSDS at the end of treatment and HSDS at the beginning, predicted adult height SDS (PAHSDS) and target height SDS (THSDS). Multiple regression analysis showed that in this group of patients, 41.4% of the variability in HSDS increment can be explained by the equation: HSDS increment = -0.33 + 0.29 THSDS - 0.68 HSDS at the beginning of treatment. RESULTS: The outcome showed a reasonable use of GH, since good-response cases generally continued treatment until final height whereas therapy was suspended in doubtful cases.

Sensitization to insulin induces ovulation in nonobese adolescents with anovulatory hyperandrogenism.

In nonobese girls with an adolescent variant of polycystic ovary syndrome, insulin-sensitizing treatment reduces hyperinsulinism, dyslipidemia, and hyperandrogenism and restores eumenorrhea; however, the effect on anovulation is unknown. We assessed whether metformin treatment is capable of inducing ovulation in nonobese adolescents with anovulatory hyperandrogenism after precocious pubarche. The study population consisted of 18 adolescents (mean age, 16 yr; body mass index, 21.4 kg/m2; 3-7 yr beyond menarche) with hyperinsulinemic hyperandrogenism. All girls received metformin for 6 months in a daily dose of 1275 mg. Before inclusion, persistent anovulation was documented by weekly serum progesterone measurements less than 4 ng/ml (months -3 and -1); the ovulation rate was assessed similarly after 2, 4 and 6 months on metformin; a premenstrual progesterone level greater than 8 ng/ml was used as ovulation marker. Regular menses were reported by 16 of 18 girls within 4 months on metformin, and all girls were eumenorrheic after 6 months on metformin. Of the 18 girls, 1 (6%) ovulated after 2 months on metformin, 7 (39%) after 4 months, and 14 (78%) after 6 months; ovulation induction failed in the girls with the lowest birth weight or most severe hyperandrogenism. Metformin treatment was well tolerated. In conclusion, sensitization to insulin was found to be an effective approach to induce ovulation in nonobese adolescents with anovulatory hyperandrogenism.

[Graves-Basedow's disease in childhood and adolescence: clinical and biologic features in 49 patients]. / Enfermedad de Graves-Basedow en la infancia y la adolescencia: aspectos clinicobiológicos en 49 casos.

INTRODUCTION: Graves' disease is rare in childhood and adolescence. PATIENTS AND METHODS: Six boys and 43 girls between the ages of 2 and 18 years were evaluated. All presented diffuse goiter and symptomatology of hyperthyroidism. Concentrations of T4, FT4, T3 and TSH were determined by RIA with various sensitivities during the study. Thyroid antibodies were determined by hemagglutination. All patients were treated with antithyroid drugs. RESULTS: In five patients, growth velocity increased. In a further five patients association with other autoimmune diseases was detected. Quetelet's index was between the 90th and 97th percentiles in five patients and between 3rd and 10th in eight. Biochemical results were as follows: T4: 282.41 +/- 1.1 nmol/1; FT4: 62.98 +/- 1.1 pmol/l; T3: 4.92 +/- 0. 07 nmol/l) and TSH: > 0.002-0.001 mU/l. Values of TSI/TRAb were 33. 23 +/- 1.95 U/l. Four total remissions and 1 partial remission were observed. Four patients underwent surgery and eight received radioiodine. CONCLUSIONS: Few remissions were obtained with medical treatment. Radioiodine seems to be a therapeutic alternative.

Enfermedad de graves-basedow en la infancia y la adolescencia: aspectos clinicobiológicos en 49 casos / Grave's disease in childhood and adolescence: clinical and biochemical aspects in 49 patients

INTRODUCCIÓN: La enfermedad de Graves-Basedow es rara en la infancia y la adolescencia. PACIENTES Y MÉTODOS: Se han evaluado 6 niños y 43 niñas, con edades comprendidas entre 2 y 18 años. Todos presentaban un bocio difuso y sintomatología de hipertiroidismo. Las concentraciones de T4, FT4, T3 y TSH se determinaron por radioinmunoanálisis con diferente sensibilidad a lo largo del tiempo y los anticuerpos, por hemaglutinación. Todos fueron tratados con fármacos antitiroideos. RESULTADOS: En 5 casos se detectó un aumento de la velocidad de crecimiento y en otros 5 había una asociación con otras autoinmunopatías. El índice de Quetelet estaba entre los percentiles 90 y 97 en 5 casos, y entre 3 y 10 en 8 casos. Los resultados bioquímicos fueron: T4 282,41 ñ 1,1 nmol/l; FT4 62,98 ñ 1,1 pmol/l; T3 4,92 ñ 0,07 nmol/l; TSH < 0,002 mU/l. Los valores de TSI/TRAb fueron 33,23 ñ 1,95 U/l. Se han obtenido 4 remisiones totales y una parcial. En 4 ocasiones se recurrió a cirugía y 8 pacientes recibieron 131I. CONCLUSIÓN: Se han obtenido pocas remisiones con el tratamiento médico y el radioyodo parece ser una terapéutica alternativa (AU)

[Acrodermatitis enteropathica: disparity between clinical features and zinc blood levels]. / Acrodermatitis enteropática: disparidad entre la clínica y los niveles plasmáticos de zinc.

A new case of acrodermatitis enteropathica in a 2.5 month old infant is presented. Oral administration of zinc sulfate (10 mg/kg/day) improved in a few days clinical and analytical pattern. In 1.5 months treatment was forsaken, reappearing after 22 days digestive and cutaneous symptoms. Serum zinc was then rather high (175 mcg/dl). Disparity between serum zinc and clinical features, suggest that in acrodermatitis enteropathica there is a lack in transport, not only in the enterocyte, but also in other cells of the body. Discerning dose and treatment duration must be assessed by clinical response rather than zinc serum level.

[Craneopharyngioma in children (author's transl)]. / Clínica del craneofaringioma en la infancia

Clinical findings in the initial and developed stages were studied in 33 cases of craneopharyngiomas in children under sixteen. A predominance of intracraneal hypertension symptoms and sight defects over endocrine symptoms was discovered; the latter appearing later on or only being detected after exhaustive studies.

[Thyroid cancer in childhood (author's transl)]. / Cáncer de tiroides en el niño

The authors present three cases of thyroid cancer in the child with comments on the etiologic, clinical and therapeutic aspects of this rare disease. The authors insist on the danger of local radiotherapy and on the need to suspect thyroid cancer in cases of persistent laterocervical adenopathies whose diagnosis can only be confirmed by means of a biopsy. It is of note that the illness took a favourable course and various therapeutic criteria are discussed.