Pubertad precoz completa isosexual: hallazgos clínicos, de laboratorio y ecografía pélvica / Complete isosexual precocious puberty: clinical, pelvic ultrasound and laboratory features

Objetivo: El objetivo de este estudio ha sido determinar si la forma de presentación inicial de la pubertad precoz central (PPC) varía en relación con la etiología y permite el diagnóstico diferencial entre formas idiopáticas y orgánicas (neurogénicas), lo que haría innecesarias las pruebas de imagen del sistema nervioso central (SNC) en determinados pacientes. Pacientes y métodos: Los niños con PPC evaluados fueron incluidos de forma consecutiva en un estudio prospectivo observacional. Se recogieron los hallazgos clínicos, de laboratorio y ecográficos. Se compararon los hallazgos de PPC idiopática (3 niños y 49 niñas) y orgánica (2 niños y 8 niñas). Resultados: No hubo diferencias en cuanto al estadio puberal, edad de inicio puberal (7,0 [5,8-7,5] frente a 7,3 [5,1-8,3] años), cociente edad ósea/edad cronológica (1,26 [1,2-1,3] frente a 1,23 [1,1-1,3]) y menarquia materna (11,7 ± 0,2 frente a 11,7 ± 0,6 años) entre PPC idiopática y orgánica, respectivamente. Los pacientes con PPC orgánica presentaron una menor desviación estándar (DE) de la talla (0,35 ± 0,4 frente a 1,6 ± 0,1; p < 0,01), predicción de talla adulta y DE de la velocidad de crecimiento (0,8 ± 0,9 frente a 3,7 ± 0,7). Las niñas con PPC orgánica presentaban de forma significativa unas mayores concentraciones plasmáticas de estradiol (47,5 [25-68] frente a 27 [14-43] pg/ml) que las niñas con PPC idiopática. La ecografía pélvica realizada en el momento del diagnóstico reveló la presencia de cambios puberales en genitales internos en el 43,9 % de las niñas (el 37,2 % en la subpoblación con PPC idiopática frente al 62,5 % en el grupo de PPC orgánica; p = 0,18). Conclusiones: Existe un solapamiento clínico-ecográfico entre PPC idiopática y orgánica. Las pruebas de imagen del SNC siguen siendo necesarias en todos los casos de PPC y los estudios ecográficos no pueden sustituir a otras investigaciones diagnósticas (AU)

Objective: To determine whether initial presentation varies according to aetiology, whether such differences allow differential diagnosis between idiopathic and organic forms, and whether CNS imaging can be avoided in some patients with central precocious puberty (CPP). Patients and methods: Children referred for evaluation of precocious puberty were evaluated, and the subpopulation of children with CPP was enrolled in this prospective observational study. Clinical, laboratory and ultrasound features of 62 consecutive patients with CPP (5 boys and 57 girls) were recorded. We compared the characteristics of idiopathic (3 boys, 49 girls) and organic (2 boys, 8 girls) CPP. Results: There were no differences in pubertal staging, age at puberty onset (7.0 [5.8-7.5] vs. 7.3 [5.1-8.3] years), bone age/chronological age ratio (1.26 [1.2-1.3] vs. 1.23 [1.1-1.3]), maternal menarche (11.7 ± 0.2 vs. 11.7 ± 0.6 years) between idiopathic and organic CPP, respectively. Organic CPP patients had a poorer height SD (0.35 ± 0.4 vs. 1.6 ± 0.1; p < 0.01), predicted adult height, growth rate and growth rate SD (0.8 ± 0.9 vs. 3.7 ± 0.7). Girls with organic CPP had significantly higher oestradiol levels (47.5 [25-68] vs. 27 [14-43] pg/ml) than girls with idiopathic CPP. Pelvic ultrasound at the time of diagnosis revealed the presence of pubertal changes in internal genitalia in 43.9 % of girls (37.2 % idiopathic versus 62.5 % organic CPP subpopulation; p=0.18). Conclusions: There is a clinical-ultrasound overlap between idiopathic and organic CPP. Imaging remains necessary in all cases of central precocious puberty, and ultrasound data should not be replaced by other diagnostic investigations (AU)

[Complete isosexual precocious puberty: clinical, pelvic ultrasound and laboratory features]. / Pubertad precoz completa isosexual: hallazgos clínicos, de laboratorio y ecografía pélvica.

OBJECTIVE: To determine whether initial presentation varies according to aetiology, whether such differences allow differential diagnosis between idiopathic and organic forms, and whether CNS imaging can be avoided in some patients with central precocious puberty (CPP). PATIENTS AND METHODS: Children referred for evaluation of precocious puberty were evaluated, and the subpopulation of children with CPP was enrolled in this prospective observational study. Clinical, laboratory and ultrasound features of 62 consecutive patients with CPP (5 boys and 57 girls) were recorded. We compared the characteristics of idiopathic (3 boys, 49 girls) and organic (2 boys, 8 girls) CPP. RESULTS: There were no differences in pubertal staging, age at puberty onset (7.0 [5.8-7.5] vs. 7.3 [5.1-8.3] years), bone age/chronological age ratio (1.26 [1.2-1.3] vs. 1.23 [1.1-1.3]), maternal menarche (11.7+/-0.2 vs. 11.7+/-0.6 years) between idiopathic and organic CPP, respectively. Organic CPP patients had a poorer height SD (0.35+/-0.4 vs. 1.6+/-0.1; p<0.01), predicted adult height, growth rate and growth rate SD (0.8+/-0.9 vs. 3.7+/-0.7). Girls with organic CPP had significantly higher oestradiol levels (47.5 [25-68] vs. 27 [14-43] pg/ml) than girls with idiopathic CPP. Pelvic ultrasound at the time of diagnosis revealed the presence of pubertal changes in internal genitalia in 43.9% of girls (37.2% idiopathic versus 62.5% organic CPP subpopulation; p=0.18). CONCLUSIONS: There is a clinical-ultrasound overlap between idiopathic and organic CPP. Imaging remains necessary in all cases of central precocious puberty, and ultrasound data should not be replaced by other diagnostic investigations.

[A comparative study of the weight-height development of the long-term survivors of acute leukemia and solid tumors]. / Estudio comparativo del desarrollo pondoestatural de los supervivientes a largo plazo de leucemia aguda y tumores sólidos.

OBJECTIVE: Our objectives were to analyze the final height and nutritional status in survivors of childhood cancer, their evolution since diagnosis and to identify neoplasm- and/or therapy-related differences. PATIENTS AND METHODS: A survey of long-term survivors of childhood cancer (acute leukemia, Wilms' tumor, sympathetic nervous system tumors) diagnosed before 15 years of age and between 1971 and 1985 in a single tertiary care center was performed. Final height, target height and body mass index were measured at evaluation. Height and body mass index, at diagnosis and at the end of treatment, was retrieved from their clinical records. All parameters are expressed as standard deviation scores of the mean of the population reference. Survivors were grouped according to diagnosis and type of treatment. Comparisons between groups and within each group were made at the time of diagnosis, at the end of treatment and at the time of evaluation. RESULTS: Sixty-one survivors of acute leukemia and 62 of solid tumors were included (32 Wilms' tumors, 20 neuroblastomas, 4 ganglioneuroblastomas and 6 ganglioneuromas). Eighty survivors had attained final height at the time of evaluation and their target height was available. Fourteen had at least one relapse. The mean height standard deviation score was positive at diagnosis and negative at the time of evaluation in all groups. Mean height loss ranged from -0.84 for the non-irradiated acute leukemia group to -1.34 for the non-irradiated solid tumor group. Adjusted final height for target height showed stature loss only in irradiated groups. Height loss was equivalent in cranially irradiated survivors (-0.32 after 18 Gy, -0.34 after 24-25.5 Gy). The age at menarche correlated negatively with the dose of cranial radiotherapy (r = -0.6, p = 0.002) and positively with stature loss (r = 0.5, p = 0.006). The mean body mass index standard deviation score was negative at diagnosis and positive at the time of evaluation in all groups. Twenty percent of solid tumor survivors and 12.5% of acute leukemia survivors were malnourished at diagnosis. Nutritional status improved in all groups at the time of evaluation. Obesity was more frequent in those who received cranial radiotherapy (14%) or intensified therapy (21%) compared with those non-cranially irradiated (none) or whose therapy was less intense (9%). CONCLUSIONS: Most survivors of childhood cancer attained their target height. Stature loss was related to cranial radiotherapy in acute leukemia survivors and to spinal irradiation in solid tumor survivors. At diagnosis, malnourishment was more frequent in solid tumor patients, while at the time of evaluation obesity was associated with a more intensified therapy.