Human growth hormone treatment in prepubertal children with achondroplasia

Weber, Giovanna; Prinster, Chiara; Meneghel, Marta; Russo, F; Mora, Stefano; Puzzovio, Maria; Maschio, Maurizia Del; Chiumello, G

doi:10.1002/(sici)1096-8628(19960202)61:4<396::aid-ajmg17>3.0.co;2-n

Cited by 17 publications

(13 citation statements)

References 24 publications

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“…The same finding was reported by Stamoyannou et α/. 11 , no signs of acromegaly were found after GH treatment. In the study of Weber et α/.…”

Section: Effect Of Gh On Glucose Tolerancesupporting

confidence: 89%

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Present Status of the Use of Growth Hormone in Short Children with Bone Diseases (Diseases of the Skeleton)

Kanaka‐Gantenbein

2001

Journal of Pediatric Endocrinology and Metabolism

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Skeletal dysplasias are genetic disorders of bone and cartilage development, mainly characterized by disproportionate short stature. Achondroplasia is the commonest and best described form of skeletal dysplasia, leading to a mean final height of 131+/-5.6 cm for males and 124+/-5.9 cm for females. Growth hormone (GH) has been used in different studies in patients with achondroplasia in order to ameliorate their height, and short term results range from rather positive to moderate. However, disproportionate advancement of bone age has been observed that can compromise the positive effect of such treatment. Furthermore, concern exists about the aggravation of body disproportion necessitating a later leg lengthening procedure in order to achieve proportionate adult stature. In hypochondroplasia, GH treatment seems to give better results when administered at puberty. No data on final height yet exist, however, so that more studies with greater numbers of patients need to be performed before a consensus on GH use in achondroplasia and hypochondroplasia can be reached. Other forms of skeletal dysplasias are quite rare, so that no conclusion on GH use in such patients can be drawn. Finally, in osteogenesis imperfecta, GH administration significantly ameliorates bone density but does not clearly seem to affect final height positively.

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“…The same finding was reported by Stamoyannou et α/. 11 , no signs of acromegaly were found after GH treatment. In the study of Weber et α/.…”

Section: Effect Of Gh On Glucose Tolerancesupporting

confidence: 89%

“…According to Weber et al 11 , oral glucose tolerance tests were normal both before and after 12 months of GH treatment in patients with achondroplasia. The same finding was reported by Stamoyannou et α/.…”

Section: Effect Of Gh On Glucose Tolerancementioning

confidence: 96%

Present Status of the Use of Growth Hormone in Short Children with Bone Diseases (Diseases of the Skeleton)

Kanaka‐Gantenbein

2001

Journal of Pediatric Endocrinology and Metabolism

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“…Growth velocity was increased by a factor of 2.3, being greatest in children with the lowest pretreatment growth velocities, in agreement with our data. Finally, Weber et al [1996] treated six prepubertal children with achon- Fig. These investigators observed an increase in HV, from a mean (± SD) of 4.1 (0.8) to 7.2 (1.4) cm/year.…”

Section: Discussionmentioning

confidence: 93%

“…GH administration was thought to be ineffective for the treatment of children with achondroplasia; how-ever, since development of biosynthetic GH, a number of studies have demonstrated that when GH is given in dosages of 0.5-1.0 IU/kg/week, an increase in height velocity (HV) can be achieved [Horton et al, 1992;Nishi et al 1993;Yamate et al, 1993;Hä genä s 1994;Weber et al, 1996].…”

Section: Introductionmentioning

confidence: 99%

Growth and growth hormone therapy in children with achondroplasia: A two‐year experience

et al. 1997

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The efficacy and safety of recombinant human growth hormone (hGH) administration was studied in children with achondroplasia. Fifteen children with achondroplasia, seven boys (4.8-12.2 years of age) and 12 girls (5.7-2.2 years of age), were treated daily with hGH at a dosage of 1 IU/kg/week. Auxological assessments were performed 6 months before, at initiation of, and at 6, 12, and 24 months following initiation of growth hormone (GH) therapy. Before initiating GH therapy, hypothalamic-pituitary and thyroid functions were evaluated. Levels of serum insulin-like growth factor (IGF)-I and IGF binding protein (BP)-3 (IGFBP-3) were assessed, as was GH response to provocative stimuli. GH responses in two stimulation tests were normal for all but three children. During the first semester of GH treatment, a significant increase in height velocity (HV), from 3.2 to 8.3 cm/year, was observed in all children. However, during the second semester, a relative decrease in growth rate was observed. By the end of the first year, HV had increased from 3.2 to 6.9 cm/year (mean, 3.7 cm/year; range, 1.1-8 cm/year) in 13 children and remained unchanged in two children. HV declined progressively during the next 12 months and, by the end of the second year of treatment, had increased in seven of the nine children who had completed 2 years of therapy (mean increase, 3.1 cm/year); two children did not respond to GH therapy, as shown by the lack of increase in HV. Sitting-height (SH) to standing-height ratio % (SH%) remained unchanged throughout GH therapy, and no significant change in skeletal maturation was observed. In conclusion, hGH treatment resulted in an increased growth rate in some children with achondroplasia; however, this increase waned during the second year of treatment. Children with the lowest pretreatment HVs seemed to benefit most from GH therapy. Nonetheless, the usefulness of GH treatment in achondroplasia will be known only when a study of final height is completed.

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“…Growth hormone plays an important role in regulating linear skeletal growth by promoting chondrocyte proliferation directly or indirectly through insulin-like growth factor-1. The majority of growth hormone trials in children with achondroplasia have been short-term [46][47][48][49][50][51][52]. These studies report an increase in growth velocity (65-75%) during the first year or a gain of 0.2-0.5 SD of height during the first year of treatment.…”

Section: Treatmentmentioning

confidence: 96%

Advances in understanding etiology of achondroplasia and review of management

Carter

Davis

Raggio

2007

Current Opinion in Pediatrics

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Establishing a diagnosis of achondroplasia allows families and clinicians to provide anticipatory care for affected children. Although the primary features of achondroplasia affect the skeleton, a multidisciplinary approach to care for children with achondroplasia helps families and clinicians understand the clinical findings and the natural history of achondroplasia in order to improve the outcome for each patient.

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Human growth hormone treatment in prepubertal children with achondroplasia

Cited by 17 publications

References 24 publications

Present Status of the Use of Growth Hormone in Short Children with Bone Diseases (Diseases of the Skeleton)

Present Status of the Use of Growth Hormone in Short Children with Bone Diseases (Diseases of the Skeleton)

Growth and growth hormone therapy in children with achondroplasia: A two‐year experience

Advances in understanding etiology of achondroplasia and review of management

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