Hypothalamic versus pituitary dysfunction in Down's syndrome as cause of growth retardation

Castells, Salvador; Beaulieu, Isabelle; Torrado, C.; Wisniewski, Krystyna E.; Zarny, S.; Gelato, Marie C.

doi:10.1111/j.1365-2788.1996.tb00661.x

Cited by 12 publications

(3 citation statements)

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“…In addition, these patients also have reduced pubertal growth spurt, contributing to the low final height [Cronk et al, 1988;Anner en et al, 1990]. It was demonstrated that children with DS do not have growth hormone (GH) deficiency [Anner en et al, 1986], but hypothalamic dysfunction was reported by Castells et al [1996]. A study performed by Anner en et al [1999] revealed that GH was given to children with DS increased growth velocity.…”

Section: Introductionmentioning

confidence: 99%

Growth charts of Turkish children with Down syndrome

Tüysüz

Göknar

Öztürk

2012

American J of Med Genetics Pt A

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We present growth curves of 1,726 Turkish children with Down syndrome (DS) between 0 and 18 years of age and investigate the factors that affect growth including congenital heart disease (CHD) and hypothyroidism. Longitudinal measurements of height, weight, and head circumference (HC) were assessed and accompanying major malformations were recorded. Growth curves were monitored using Cole's LMS method. The mean birth length was reduced by À0.5 standard deviation (SD) for Turkish standards in both boys and girls. Pubertal growth spurt of the girls with DS started 1 year earlier, their puberty duration was short and pubertal annual growth rate was inadequate, and as a result the final height was À3.06 SD for Turkish standards. Although the age at onset of pubertal growth spurt and puberty duration of the boys were similar to normal population, their pubertal annual growth rate was inadequate; thus the final height was À2.56 SD for Turkish standards. The final weight values were similar to normal population. The mean HC values of DS children were corresponded to À0.9 SD for Turkish standards at birth; however after 6 months values were below À2 SD. The final HC values were À1.02 SD for boys and À2.21 SD for girls for Turkish standards. We observed that weight was decreased in DS children with severe CHD during first 4 years of life. However, there is no statistically significant difference in values of height and head circumference between patients with or without severe CHD group. In addition, hypothyroidism had no effect on growth in DS patients. Ó 2012 Wiley Periodicals, Inc.Key words: Down syndrome; growth; charts; height; weight; head circumference INTRODUCTION Down syndrome (DS) is the most common chromosomal disorder and leading cause of intellectual disability and growth retardation. Average height is around the À2 standard deviation (SD) for most ages compared to normal population. The growth pattern is impaired from birth to adolescence and particularly during the interval of 6 months to 3 years and puberty [Cronk et al., 1988]. In addition, these patients also have reduced pubertal growth spurt, contributing to the low final height [Cronk et al., 1988;Anner en et al., 1990]. It was demonstrated that children with DS do not have growth hormone (GH) deficiency [Anner en et al., 1986], but hypothalamic dysfunction was reported by Castells et al. [1996]. A study performed by Anner en et al. [1999] revealed that GH was given to children with DS increased growth velocity. However, Myrelid et al. [2010] reported that GH treatment had no effect on final height.Since children with DS have different growth patterns than other normal children, it is critically important to utilize syndrome specific growth charts. First growth charts for DS have been developed by Roche [1965], followed by Rarick and Seefeldt [1974]. Cronk et al. [1988] was studied 780 children with DS between 1 month and 18 years of age and established growth charts with these data in USA and also showed that moderate to severe CHD affected growt...

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Section: Introductionmentioning

confidence: 99%

Growth charts of Turkish children with Down syndrome

Tüysüz

Göknar

Öztürk

2012

American J of Med Genetics Pt A

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“…Este patro ´n de crecimiento diferente al de la poblacio ´n general hace que, en el seguimiento de estos pacientes, se recomiende la utilizacio ´n de tablas de referencia especı ´ficas para talla/longitud, peso y PC, como las realizadas en nuestro paı ´s por la Fundacio ´n Catalana del Sı ´ndrome de Down 8 . No obstante, los mecanismos fisiopatolo ´gicos que median el hipocrecimiento de estos pacientes son desconocidos, habie ´ndose sugerido una posible alteracio ´n en la neurorregulacio ´n de la secrecio ´n de hormona de crecimiento 9 .…”

Section: Discusio ´Nunclassified

Alteraciones endocrinológicas en 1.105 niños y adolescentes con síndrome de Down

et al. 2011

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“…Much interest has been paid to growth hormone (GH) and insulin-like growth factor I (IGF-I), as the pronounced growth retardation in DS coincides with the time when GH become essential for growth. Suboptimal production of GH [31] and a selective deficiency of IGF-I [24,32] have been demonstrated in children with DS, but there is no clear evidence of a general GH deficiency [33].…”

Section: Statural Growth In Down Syndromementioning

confidence: 99%

Current Knowledge on Growth Hormone and Insulin-Like Growth Factors and their Role in the Central Nervous System: Growth Hormone in Down Syndrome

Myrelid¹

2012

TOEJ

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Down syndrome (DS) is a chromosomal disorder associated with short stature and psychomotor delay. Growth hormone (GH) and insulin-like growth factor I (IGF-I) are of interest in DS as the pronounced growth retardation coincides in time when GH becomes essential for growth. Simultaneously mean intelligence quotient (IQ) decreases markedly.There is no evidence of a general GH deficiency in children with DS, but suboptimal endogenous production of GH and selective deficiency of IGF-I have been demonstrated. GH and IGF-I are important brain growth promoting factors. IGF receptors are present in brain cells from foetuses with DS, but there is no information as to whether disturbances of intracerebral GH binding or IGF-I production may contribute to the brain dysfunction in DS.GH therapy normalized growth velocity and improved fine motor performance in young children with DS, but the limited therapy was followed by a marked "catch-down" growth and lacked effect on final height. In adolescents with DS treatment with GH during early childhood show some positive late effects, where subjects previously treated had greater head circumference and improved performance regarding cognitive function and motor skills.Few studies have addressed the effect of GH in Down syndrome. Nevertheless, GH has been designated beneficial effects on cognition, energy, mood and behaviour through extensive studies in several other conditions and it is not unlikely that GH may have similar effects in DS. It should be emphasised that even small changes in psychomotor attainment may be of substantial importance in a developmentally delayed population.

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Hypothalamic versus pituitary dysfunction in Down's syndrome as cause of growth retardation

Cited by 12 publications

References 39 publications

Growth charts of Turkish children with Down syndrome

Growth charts of Turkish children with Down syndrome

Alteraciones endocrinológicas en 1.105 niños y adolescentes con síndrome de Down

Current Knowledge on Growth Hormone and Insulin-Like Growth Factors and their Role in the Central Nervous System: Growth Hormone in Down Syndrome

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