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An increased risk of developing precocious puberty has been reported in children with myelomeningocele. In order to evaluate this further and to study factors associated with early or precocious puberty the medical records of all girls with myelomeningocele, born from 1970 onwards (n = 64), who were admitted to our unit were reviewed. Early/precocious puberty (E/PP) was defined as breast development or pubic hair corresponding to Tanner stage 2 before the age of 9.2 years. In 32 out of 62 cases data were sufficient for evaluation of the timing of puberty. Twenty girls had E/PP and 12 girls normal timing of puberty. In the girls who had reached the age of 9.2 years the incidence of E/PP was at least 52%. Girls with E/PP had a higher incidence of hydrocephalus, were treated with intraventricular shunts more often, and had significantly higher frequency of increased intracranial pressure during the perinatal period (p < 0.05, p < 0.01, and p < 0.001, respectively). The group of girls developing E/PP was also more severely disabled with respect to motor and urological function and had more shunt revisions. In conclusion, E/PP in girls with myelomeningocele is strongly associated with increased intracranial pressure particularly during the perinatal period. [ABSTRACT FROM AUTHOR]

Background Children with myelomeningocele (MMC) run an increased risk of developing early or precocious puberty (E/PP). In a previous study on girls with MMC we found increased intracranial pressure during the perinatal period to be strongly associated with E/PP. We also found the incidence of E/PP among girls to be as high as at least 52%. In a number of studies PP in boys also has been reported, but most studies have been based on selected groups of children. The aims of the present study was to investigate the incidence of E/PP in boys with MMC treated at a regional habilitation centre, and to identify possible risk factors associated with the development of early or precocious puberty. In addition we analyzed the clinical course of pubertal development. Materials and methods The study population comprised all boys born between 1970 and 1992 admitted to the Folke Bernadottehemmet at least at one occasion (n = 59). The medical records were examined and those who had reached 10.2 years (n = 47) were evaluated regarding pubertal status, growth charts, perinatal problems and medical problems and problems regarding other areas of function. Results Of the 47 boys who had reached the age of 10.2 years or above, 43 boys were possible to evaluate regarding onset of puberty. Nine of these boys had had an early puberty and one a precocious puberty. Thus the incidence of E/PP in this group is at least 21% (10/47). The boys with E/PP had significantly higher incidence of both increased intracranial pressure during the perinatal period and early and severe symptoms of brainstem dysfunction. In 18 boys, sufficient data for complete analysis of both growth spurt and Tanner stages were obtained. Analysis showed that in 14 boys out of 18 the growth spurt was the first sign of puberty. Conclusion We found an increased incidence of E/PP in the boys with MMC. Early puberty was significantly associated with high intracranial pressure during perinatal period. In addition, we found that pubertal development seemed to be accelerated in many boys with an early growth spurt. [ABSTRACT FROM AUTHOR]

Background/aims: Gonadotropin-releasing hormone analogues (GnRHa) are the accepted treatment of idiopathic central precocious puberty. As it has been found that growth velocity may be decreased with GnRHa treatment, clinical trials with GnRHa combined with growth hormone (GH) have been carried out. In a recent study 46 adopted girls with early or precocious puberty were randomly assigned to treatment with either GnRHa or GnRHa combined with GH, and followed to final height (FH). It was found that FH was significantly higher in the combined treatment group, 158.9 compared with 155.8 cm in the GnRHa treated group. In order to select the patients who could benefit from added GH, predictions of FH at the start of treatment according to the methods of bone age determination of Greulich-Pyle (GP) and Tanner-Whitehouse 2 (TW2) were compared. It was found that the GP method was the most useful method for patient selection. Recently, a revision of the Tanner-Whitehouse method, named Tanner-Whitehouse 3 (TW3), has been developed. The present study examined the usefulness of the TW3 method in selecting suitable patients for combined treatment., Method: The TW3 method bone age determinations of the 46 girls were compared to the GP and TW2 method determinations, using the differences between actual FH and predicted adult height (PAH). Beside accuracy of prediction of FH, the criteria of efficiency of selection and replicability were applied in the comparison., Results: We found that the GP method, also when compared to the TW3 method, gave the most accurate prediction of the FH on only GnRHa treatment. This gives the best ground for selection of patients who can benefit from combined treatment. The GP method was also the most efficient in selecting patients, i.e., it could select the least number of patients that needed the combined treatment. The only drawback of the GP method was that it requires an experienced pediatric radiologist. Automated methods are being developed and may soon facilitate the use of the GP method for those less experienced. The FH after combined treatment could be predicted with an equation including PAH GP as well as PAH TW3 as variables., Conclusion: The GP method remains the most useful method for selection of those patients who will benefit most from the addition of GH to GnRHa in the treatment of idiopathic central precocious or early puberty. FH prediction after combined treatment requires PAH GP as well as PAH TW3.

In treatment of idiopathic central precocious puberty, GnRH analogues (GnRHa) have been accepted as the treatment of choice. Since growth velocity may be impaired with GnRHa treatment growth hormone (GH) treatment has been added in clinical trials. Recently, a study followed adopted girls with early or precocious puberty on GnRHa or combined GnRHa and GH treatment to final height. It was found that final height was significantly higher in the combined treatment group, although the difference was small. It was seen that patients that were extremely short at arrival and short at start of treatment seemed to be candidates for combined treatment. We have now analysed the data in order to define criteria for the sub-group in need of combined GnRHa-GH treatment in order to achieve normal final height, i.e. above -2 SDS. Bone ages of 46 patients at start of treatment, randomized to either GnRHa treatment or GnRHa treatment combined with GH, were examined blindly by the same radiologist and the PAH calculated. The methods according to Greulich-Pyle / Bayley-Pinneau (GP/BP) and Tanner-Whitehouse (TW2) were used. Predictions versus final height data were analysed. The accuracy of FH prediction was greatest for GnRHa treated group using the GP/BP method. The GP/BP method gave useful cut off limits for when combined treatment was necessary to possibly achieve normal height. If pre-treatment GP/PAH was > 157cm, the patients attained normal height with GnRHa treatment only. Ten out of 13 (77%) such girls could be correctly identified. Using TW2 with a cut off of 164 cm, 9 out of 13 could be selected. Using a multi regression equation of best fit the number of correctly selected cases for GnRHa treatment only, could not be further increased in this group. We conclude that bone age determination and adult height prediction with the Greulich-Pyle/Bayley-Pinneau method, provides useful criteria for selecting the subgroup of adopted girls with early puberty where combined treatment with GnRHa and GH is not necessary to reach normal final height.

Girls adopted from developing countries often have early or precocious puberty, requiring treatment with gonadotropin-releasing hormone (GnRH) analogues. During such treatment decreased growth velocity is frequent. The aim of this investigation was to study whether the addition of growth hormone (GH) to GnRH analogue treatment improves height velocity and final height in girls with early or precocious puberty. Forty-six girls with early or precocious puberty adopted from developing countries were randomized for treatment with GnRH analogue or a combination of GH and GnRH analogue. After 2 y of treatment the mean growth in the GH/GnRH analogue group was significantly higher, 14.6 cm, compared to 10.9 cm in the control group. The increase in bone age did not differ, while the difference in predicted adult height increased by 2.7 cm in favour of the combination group. Although data on final height are not yet available, combined GH/GnRH analogue treatment for 2 y resulted in a higher growth velocity and predicted final height compared to GnRH analogue treatment alone.

Thirty-four children were observed prospectively during a period of 5 years from the onset of diabetes regarding height, weight and subcutaneous fat in relation to treatment and diabetes control. Height, weight and body mass index (BMI) did not differ from those in a control group. The girls with diabetes increased their triceps and subscapular fat significantly despite a normal BMI. This increase in relation to control girls was most pronounced during late adolescence. Triceps skinfold and BMI after 5 years were both negatively correlated to insulin dose per kilogram body weight in the diabetic girls. We conclude that girls with diabetes accumulate subcutaneous fat in certain areas rapidly during adolescence in a completely different way from healthy girls.

Thirty-four children were followed up prospectively for 5 years from the onset of diabetes regarding serum magnesium, zinc and some proteins. Serum magnesium decreased to significantly lower values (0.76 +/- 0.05 mmol l-1) than those in matched controls after 2 and 5 years, with the lowest mean values in diabetic girls. Serum zinc concentration was higher in the diabetic group than in the control children, and again the diabetic girls differed most from the controls. Serum prealbumin was significantly lower in the diabetic patients after 2 and 5 years than in the controls. Serum albumin was also slightly reduced in the diabetic patients, while orosomucoid was normal. These data indicate chronic magnesium deficiency and insufficient liver synthesis of certain serum proteins in diabetic children.

A secular trend towards higher final height and earlier pubertal maturation is seen in countries with favourable socio-economic development and higher social classes in countries are also associated with taller height and earlier maturation. Environmental factors, such as nutrition and infections, appear to be the main causes for differences in growth and maturation between ethnic and social groups. Differences in final height are mainly due to prepubertal growth. Studies on immigrant children and children adopted into privileged conditions from developing countries confirm the influence of early growth on subsequent growth. Catch-up growth in adopted children could only partially compensate for early stunting, and in several cases was cut short by early pubertal development. A minority developed very early puberty and eventually very short final height. The optimal rate of catch-up growth, the trigger mechanism for early puberty, and the effect of various types of nutritional intervention need to be studied. The studies reported here indicate the critical importance of optimal intra-uterine, infant and childhood growth as a basis for satisfactory growth during adolescence.