Clinical evaluation of recombinant human growth hormone in Noonan syndrome.
(33/225)
The objective of this study was to investigate the effect of administration of recombinant human growth hormone (hGH) in patients with Noonan syndrome. hGH was administered (0.5 IU/kg/week) to 15 patients with Noonan syndrome over a 2 year period. Average patient age prior to therapy was 7.5 +/- 2.5 (mean +/- SD) yr, the height SD score was -2.8 +/- 0.7, and the pretreatment height velocity and bone age were 4.8 +/- 1.0 cm/yr and 5.8 +/- 2.1 yr, respectively. The height velocity in the year prior to treatment, and 0-12 and 12-24 months after commencing treatment was 4.8 +/- 1.0 cm/yr, 7.0 +/- 1.2 cm/yr, and 5.5 +/- 0.6 cm/yr, respectively. The height velocity in the first year of treatment was significantly greater (P = 0.0001, n = 14) than the pretreatment value, but there was no significant difference in the second year. The height SD scores at the commencement of treatment, and after 12 and 24 months of treatment were -2.8 +/- 0.7, -2.4 +/- 0.7, and -2.2 +/- 0.5, respectively. Bone age advanced by 1.1 +/- 0.5 yr in the 12 months after commencing treatment. We conclude that the use of hGH may be beneficial in the treatment of Noonan syndrome, although further research is required. (+info)
A computational TW3 classifier for skeletal maturity assessment. A Computing with Words approach.
(34/225)
This paper proposes a fuzzy methodology to translate the natural language descriptions of the TW3 method for bone age assessment into an automatic classifier. The classifier is built upon a modified version of a fuzzy ID3 decision tree. No large data records are needed to train the classifier, i.e., to find out the classification rules, since the classifier is built upon rules given by the TW3 method. Only small data records are needed to fine-tune the fuzzy sets used to implement the rulebase. (+info)
Computer-assisted bone age assessment: graphical user interface for image processing and comparison.
(35/225)
The current study is part of a project resulting in a computer-assisted analysis of a hand radiograph yielding an assessment of skeletal maturity. The image analysis is based on features selected from six regions of interest. At various stages of skeletal development different image processing problems have to be addressed. At the early stage, feature extraction is based on Lee filtering followed by the random Gibbs fields and mathematical morphology. Once the fusion starts, wavelet decomposition methods are implemented. The user interface displays the closest neighbors to each image under consideration. Results show the sensitivity of different regions to both stages of development and certain feature sensitivity within each region. At the early stage of development, the distal features are more reliable indicators, whereas at the stage of epiphyseal fusion, a larger dynamic range of middle features makes them more sensitive. In the current study, a graphical user interface has been designed and implemented for testing the image processing routines and comparing the results of quantitative image analysis with the visual interpretation of extracted regions of interest. The user interface may also serve as a teaching tool. At the later stage of the project it will be used as a classification tool. (+info)
Slipped capital femoral epiphysis in skeletally immature patients.
(36/225)
Fixation by a single screw is considered the current treatment of choice for a slipped capital femoral epiphysis. This approach promotes premature physeal closure. The use of a modified, standard, single, cannulated screw designed to maintain epiphyseal fixation without causing premature closure of the physis was reviewed in ten patients. The nine boys and one girl aged between 10.6 and 12.6 years with unilateral slipped capital femoral epiphysis (SCFE), were markedly skeletally immature (Tanner stage I, bone age 10 to 12.6 years). Clinical and radiological review at a mean follow-up of 44.3 months (36 to 76) showed no difference in the time to physeal closure between the involved and uninvolved side. Measurement of epiphyseal and physeal development showed continued growth and remodelling in all patients. Use of this device provided epiphyseal stability and maintained the capacity for physeal recovery and growth following treatment for both unstable and stable slipped capital femoral epiphysis. (+info)
Correlation of sex steroids with IGF-1 and IGFBP-3 during different pubertal stages.
(37/225)
Insulin-like growth factor 1 (IGF-1) is the major factor that affects linear bone growth. Also, androgens and estrogens are necessary for increasing longitudinal bone growth during sexual maturation. The aim of this study was to investigate the relationships among IGF-1 axis and sex steroids during pubertal development in healthy adolescents. In this cross-sectional study, IGF-1, IGF binding protein-3 (IGFBP-3) and sex steroid levels (estradiol in girls, testosterone in boys) of 205 healthy adolescents (101 female, 104 male) aged 9-17 years were measured. All subjects were apparently healthy, with no growth retardation and with skeletal ages appropriate for chronological ages, and none were taking medications known to influence calcium homeostasis. Greulich and Pyle's Radiographic Atlas of Skeletal Development of the Hand and Wrist was used for determination of skeletal ages. Tanner's classification was used to determine the pubertal developmental stage. Fasting blood samples were obtained from subjects between 09:00-10:00 h. Serum IGF-1 and IGFBP-3 levels differed significantly between pubertal developmental stages. Serum IGF-1 levels and IGF-1/IGFBP-3 ratios increased with proceeding stages and maximum mean values were found at stages III-IV in girls and at stage IV in boys. Estradiol levels of girls and testosterone levels of boys differed significantly between stages, and in both sexes, serum IGF-1 levels and IGF-1/IGFBP-3 ratios were significantly correlated with sex steroid levels. Increase in growth hormone secretion increases IGF-1 levels. Furthermore, increasing sex steroids with pubertal development increase the IGF-1 levels and IGF-1/IGFBP-3 ratios that affect bone growth. (+info)
Evaluation of fetal growth and estimation of fetal age based on skeletal growth in Hokkaido sika deer (Cervus nippon yesoensis Heude, 1884).
(38/225)
We investigated fetal development and the estimation of fetal age of 127 Hokkaido sika deer fetuses, categorizing them into three groups according to the nutritional condition of populations. The order and time of the appearance of ossification centers were clarified, and fetal age was determined based on bone length and the appearance of ossification centers. Then we observed the differences in fetal growth among three populations, and discussed the effect of poor nutrition on the fetal growth. The results suggest that fetal diaphysial length of the femur was affected very little by nutritional conditions, whereas conception dates were delayed and fetal weight was restricted as the nutritional condition became poorer. Although it is impossible to know the exact accurate fetal age in wild populations, it was possible to create a standard to estimate fetal age more precisely by the method described in this study. Both the bone length and the appearance of ossification centers are reliable indices to estimate fetal age precisely in measurements available from fetuses of unknown age, and can be applied to estimate the fetal age of other populations of sika deer, whereas estimation of fetal age based on weight is prone to great errors. (+info)
Studies of very severe short stature with severe GH deficiency: from the data registered with the foundation for growth science.
(39/225)
The ratio, clinical characteristics, and therapeutic efficacy of hGH treatment in patients with severe short stature (HtSDS below -4SD) with severe GHD (all peak GH values to provocation tests: below 2 mug/L) were studied. From March 1986 to January 1998, 23,110 patients with idiopathic GH deficiency (IGHD) were registered with the Foundation for Growth Science, Japan. These subjects were divided into 5 groups as follows: Group 1 (G1), all subjects; Group 2 (G2), at least one GH peak to provocative test > or = 5 microg/L; Group 3 (G3), 2 microg/L < or = GH peak<5 microg/L; Group 4 (G4), all GH peaks<2 microg/L and HtSDS>-4; Group 5 (G5), all GH peaks<2 microg/L and HtSDS< or = -4. The ratio of G5 was 139 patients (0.6%) out of 23,110 patients with IGHD. In G5, there were no significant differences in birth weight, birth length, gestational age and parental height between G2, G3 and G4. However, asphyxia at delivery was more frequent in G5 and G4 than G2 and G3. Chronological age (CA), bone age (BA) and BA/CA ratio at registration were significantly lower in G5 than G2, G3 and G4. Further, the IGF-I SD score in G5 was significantly lower than those in G2 and G3. After hGH treatment, the final height and final height SDS in G5 remained the lowest, while the DeltaHtSDS value in G5 was the greatest among G2 to G5 groups. In conclusion, the ratio of severe short stature with severe GH deficiency (G5) is only 0.6% of all IGHD cases. Growth failure in G5 seems to occur after birth, and its etiology in G5 seems to be different from that of patients with other forms of IGHD. Early diagnosis and hGH treatment are needed to attain better final height. (+info)
Disentangling the genetic determinants of human aging: biological age as an alternative to the use of survival measures.
(40/225)
The choice of a phenotype is critical for the study of a complex genetically regulated process, such as aging. To date, most of the twin and family studies have focused on broad survival measures, primarily age at death or exceptional longevity. However, on the basis of recent studies of twins and families, biological age has also been shown to have a strong genetic component, with heritability estimates ranging from 27% to 57%. The aim of this review is twofold: first, to summarize growing consensus on reliable methods of biological age assessment, and second, to demonstrate validity of this phenotype for research in the genetics of aging in humans. (+info)