Long-term results of GH therapy in GH-deficient children treated before 1 year of age.
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OBJECTIVES: To evaluate the long-term effects of GH therapy in early diagnosed GH-deficient patients treated before 1 year of age. STUDY DESIGN: We studied all 59 patients (33 males) recorded by Association France-Hypophyse and treated with GH (0.50+/-0.15 IU/kg (S.D.) per week) before 1 year of age. Clinical presentation and growth parameters under GH treatment were analyzed. RESULTS: Neonatal manifestations of hypopituitarism were frequent: hypoglycemia (n=50), jaundice (n=25) and micropenis (n=17/33). Although birth length was moderately reduced (-0.9+/-1.4), growth retardation at diagnosis (5.8+/-3.8 months) was severe (-3.5+/-1.9 standard deviation scores (SDS)). Fifty patients (85%) had thyrotropin and/or corticotropin deficiency. After a mean duration of GH therapy of 8.0+/-3.6 years, change in height SDS was +3.11+/-2.06 S.D., exceeding 4 SDS in 19 patients. Only 9 patients (15%) did not reach a height of -2 S.D. for chronological age and 20 patients (34%) exceeded their target height. Pretreatment height SDS was independently associated with total catch-up growth. CONCLUSION: Conventional doses of GH allow normalization of height in patients with early GH deficiency and treatment. (+info)
Growth hormone treatment in young children with Down's syndrome: effects on growth and psychomotor development.
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BACKGROUND: Learning disability and short stature are cardinal signs of Down's syndrome. Insulin-like growth factor I (IGF-I), regulated by growth hormone (GH) from about 6 months of age, may be involved in brain development. AIMS: To study long term effects of GH on linear growth and psychomotor development in young children with Down's syndrome. Study design-Fifteen children with Down's syndrome were treated with GH for three years from the age of 6 to 9 months (mean, 7.4). Linear growth, psychomotor development, skeletal maturation, serum concentrations of IGF-I and its binding proteins (BPs), and cerebrospinal fluid (CSF) concentrations of IGF-II were studied. RESULTS: The mean height of the study group increased from -1.8 to -0.8 SDS (Swedish standard) during treatment, whereas that of a Down's syndrome control group fell from -1.7 to -2.2 SDS. Growth velocity declined after treatment stopped. Head growth did not accelerate during treatment. No significant difference in mental or gross motor development was found. The low concentrations of serum IGF-I and IGFBP-3 became normal during GH treatment. CONCLUSIONS: GH treatment results in normal growth velocity in Down's syndrome but does not affect head circumference or mental or gross motor development. Growth velocity declines after treatment stops. (+info)
Growth in Sotos syndrome.
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Although there are several reports on infant and childhood growth in patients with Sotos syndrome, there is little information on the final height achieved and puberty. Growth data on 40 patients (20 female and 20 male) aged 2-31 years were collected. These showed that patients with Sotos syndrome are excessively tall at birth, during infancy, and during childhood. Disproportionately long limbs constitute much of the increase in stature. However, the combination of advanced bone age and early onset of menarche led to a mean (SD) final height of 172.9 (5.7) cm in women. This is within the normal range for the population. Most of the men also attained a final height (mean, 184.3 cm; SD, 6.0) within the normal range, although exceptions were more likely in men than in women. Therefore, these results show that most patients with Sotos syndrome do not require intervention to limit their adult height. (+info)
Estrogen supplementation for bone dematuration in young epileptic man treated with anticonvulsant therapy; a case report.
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We encountered a young man treated with anticonvulsant therapy who had greatly reduced bone mineral density. An 18-year-old man was admitted to our hospital for shoulder pain and further evaluation of decreased bone mineral density. He had been treated with anticonvulsants, including phenytoin, phenobarbital, valproic acid and zonisamide for seizures. Although testosterone was found within the normal range for adult men, the serum estrogen concentration was below the detection limit (< 10 pg/ml) and his wrist epiphyses were not yet closed. After 10 months of treatment with the conjugated estrogen, both his height and weight showed improvement, while his bone mineral density and bone age were increased. These findings suggested that estrogen therapy had a significant effect on his skeletal growth and bone maturation in man. This is the first report showing the beneficial effect of estrogen supplementation in an epileptic man receiving treatment with anticonvulsants. (+info)
Bone age assessment: a large scale comparison of the Greulich and Pyle, and Tanner and Whitehouse (TW2) methods.
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PURPOSE: Comparison of bone age assessed using either the "atlas matching" method of Greulich and Pyle or the "point scoring system" of Tanner and Whitehouse (TW2). MATERIALS AND METHODS: 362 consecutive "bone age" radiographs of the left hand and distal radius performed in a large provincial teaching hospital. Data were analysed using the "method comparison" statistical technique. Ten per cent of the radiographs were re-analysed to assess intra-observer variation. RESULTS: The 95% confidence interval for the difference between the two methods was 2.28 to -1.52 years. Intra-observer variation was greater for the Greulich and Pyle method than for the TW2 method (95% confidence limit, -2.46 to 2.18 v -1.41 to 1.43). CONCLUSION: The two methods of bone age assessment as used in clinical practice do not give equivalent estimates of bone age and we suggest that one method only (preferably the TW2) should be used when performing serial measurements on an individual patient. (+info)
Skeletal maturity in Pakistani children.
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Skeletal maturity in 750 normal Pakistani children (400 males, 350 females) aged 1-18 y was determined by the Greulich-Pyle atlas system. Male children during first year and female children during first 2 y of life matured in conformity with Greulich-Pyle standards. After that period mean bone ages were lower than the American standards up to 15 y in males and 13 y in females (at or around puberty), which may be due to malnutrition, ill health or other environmental factors. After puberty bone ages were higher than the American standards indicating earlier maturity in Pakistani than Western children. Hence for the proper evaluation of skeletal age in a given region, a longitudinal study on individuals in that region to establish normal standards is necessary. (+info)
The potential of digital dental radiography in recording the adductor sesamoid and the MP3 stages.
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The current study was undertaken to evaluate the reliability of using a recent advance in clinical radiographic technique, digital dental radiography, in recording two growth indicators: the adductor sesamoid and MP3 stages. With an exposure time five times less than that used in the conventional approach, this method shows greatest flexibility in providing a high quality digitized radiographic images of the two growth indicators under investigation. Refereed Paper (+info)
Determination of age at death using combined morphology and histology of the femur.
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Bone is characterised by age-related morphological and histological changes. We have previously established an automated method of recording bone morphometry and histology from entire transverse sections of cortical bone. Our aim was to determine whether data acquired using this automated system were useful in the prediction of age. Ninety-six specimens of human femoral middiaphysis were studied from subjects aged 21-92 y. Equations predicting specimen age were constructed using macroscopic data (total subperiosteal area (TSPA), periosteal perimeter (PP), endosteal perimeter (EP), cortical bone area (CA) and moments of area) and microscopic data (the number, size and diversity of pores and intracortical porosity) together with sex, height and weight. Both TSPA and PP were independent predictors of age but the number of pores was not a significant predictor of age in any equation. The age predicted by these equations was inaccurate by more than 8 y in over half the subjects. We conclude that we could not predict age at a clinically acceptable level using data from our automated system. This most likely reflects an insensitivity to regional age-related changes in bone histology because we recorded data from each entire cortex. Automated bone measurement according to cortical region might be more useful in the prediction of age. The inclusion of TSPA together with PP as independent predictors of age raises the possibility that a future measure of periosteal shape at the femoral diaphysis could also be helpful in the prediction of age. The accuracy reached with the relatively simple methods described here is sufficient to encourage the development of image-analysis systems for the automatic detection of more complex features. (+info)