Longitudinal analysis of growth over the first 3 years of life in Turner’s syndrome

Even, Lea; Cohen, Ayala; Marbach, Naama; Brand, Manuela; Kauli, Rivka; Sippell, Wolfgang G.; Hochberg, Zèev

doi:10.1067/mpd.2000.109110

Cited by 46 publications

(32 citation statements)

References 16 publications

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“…This study specifically focused on the initiation of r-hGH treatment in early childhood, during the years when growth rate is most affected by the disease (4,5). In this study, 80% of the 61 girls receiving 4 years of treatment with r-hGH were able to attain a normal height by a mean age of 6.6 years.…”

Section: Discussionmentioning

confidence: 99%

Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome

Linglart

Cabrol²,

Berlier³

et al. 2011

European Journal of Endocrinology

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Objective: Adult height deficit seen in Turner syndrome (TS) originates, in part, from growth retardation in utero and throughout the first 3 years of life. Earlier diagnosis enables earlier therapeutic intervention, such as with recombinant human GH (r-hGH), which may help to prevent growth retardation. In this open-label, multicentre phase III study, we investigated efficacy and safety in r-hGH treatment in young girls with TS. Subjects and methods: Girls (nZ61) aged !4 years with TS receiving 0.035-0.05 mg/kg per day r-hGH for 4 years were compared with an historical control group (nZ51) comprising untreated, age-and height-matched girls with TS. The main outcome measure was change in height SDS (H-SDS). Other measures included changes in height velocity SDS, IGF1 levels and glucose metabolism. Results: After 4 years, a gain in mean H-SDS of 1.0 SDS (from K2.33G0.73 to K1.35G0.86 SDS) was observed with r-hGH treatment, in contrast to the decrease in mean H-SDS of 0.3 SDS in the control group (from K2.09G0.81 to K2.44G0.73 SDS; P!0.0001). r-hGH treatment was the main predictor of H-SDS gain and accounted for 52% of variability (multivariate analysis). r-hGH was well tolerated. As expected, IGF1 levels rose with treatment. A case of transient glucose intolerance resolved after dietary adaptation. Conclusion: Early treatment with r-hGH helps to prevent natural evolution towards short stature in most girls with TS. IGF1 levels and glucose metabolism should be monitored routinely during r-hGH therapy.

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Section: Discussionmentioning

confidence: 99%

Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome

Linglart

Cabrol²,

Berlier³

et al. 2011

European Journal of Endocrinology

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“…1,2,4,5 Because of delayed epiphyseal closure, small gains in height may occur even after 20 years of age. Average height is rarely achieved, except in girls with mosaicism (the presence in an individual of 2 or more chromosomally different cell lines, both originating from the same zygote) or those whose parents are of above average height.…”

Section: Phenotypementioning

confidence: 99%

Health Supervision for Children With Turner Syndrome

Frías¹,

Davenport²

2003

Pediatrics

108

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ABSTRACT. This report is designed to assist the pediatrician in caring for the child in whom the diagnosis of Turner syndrome has been confirmed by karyotyping. The report is meant to serve as a supplement to the American Academy of Pediatrics' "Recommendations for Preventive Pediatric Care" and emphasizes the importance of continuity of care and the need to avoid its fragmentation by ensuring a medical home for every girl with Turner syndrome. The pediatrician's first contact with a child with Turner syndrome may occur during infancy or childhood. This report also discusses interactions with expectant parents who have been given the prenatal diagnosis of Turner syndrome and have been referred for advice.

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“…It is widely accepted that growth in girls with TS has four cardinal features: (1) moderate intrauterine growth retardation, (2) normal growth velocity between birth and 3 years of age, (3) decreasing height velocity between ages 3 and 12 years, and (4) failure to experience a pubertal growth spurt [2, 6, 10, 11, 12]. A preliminary report from one center in North Carolina [13]and recent data from Northern Europe and Israel, however, suggest that the growth failure that begins in intrauterine life continues postnatally, and is particularly profound in the first 2–3 years of life [9, 14, 15, 16]. Furthermore, the widely used growth charts for TS girls 2–18 years of age indicate that the majority of these girls are below the 5th percentile for height by 2 years of age [7].…”

Section: Introductionmentioning

confidence: 99%

Growth Failure in Early Life: An Important Manifestation of Turner Syndrome

et al. 2002

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The goals of this study were to test the hypothesis that girls with Turner syndrome (TS) experience growth failure early in life and to establish model-based normative growth charts for 0- to 8-year-old American girls with TS. Full-term girls with TS who had 5 or more measurements of height obtained during their first 10 years of life prior to initiation of growth hormone, estrogen and/or androgen therapy were eligible for this study. A nonlinear mixed-effects model comprising the first two components of the infancy-childhood-puberty (ICP) model of growth was fitted to the longitudinal height measurements and compared with those of healthy American girls. Height measurements (n = 1,146) from 112 girls with TS (45,X: 57.1%; 45,X/46,XX: 12.5%; 46,X, iso(X): 4.5%, and other: 25.9%) were analyzed. Mean height SDS fell from –0.68 at birth to –1.60 at 1 year, –1.80 at 2 years and –1.95 at 3 years. When compared to controls (676 girls, 4,537 measurements), girls with TS grew more slowly due to three principal factors: a slow growth rate of the infancy component, a slow growth rate at the onset of the childhood component, and delayed onset of the childhood component. Traditional concepts of growth failure in TS should be revised. Physicians should consider the diagnosis of TS in any girl with unexplained failure to thrive or short stature, even in the first 3 years of life.

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Longitudinal analysis of growth over the first 3 years of life in Turner’s syndrome

Cited by 46 publications

References 16 publications

Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome

Growth hormone treatment before the age of 4 years prevents short stature in young girls with Turner syndrome

Health Supervision for Children With Turner Syndrome

Growth Failure in Early Life: An Important Manifestation of Turner Syndrome

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