Growth hormone deficiency in Down's syndrome children

Castells, Salvador; Torrado, C.; Bastian, William; Wisniewski, Krystyna E.

doi:10.1111/j.1365-2788.1992.tb00469.x

Cited by 21 publications

(9 citation statements)

References 46 publications

(28 reference statements)

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“…Growth retardation therefore becomes pronounced during the period when GH starts to stimulate growth. There is no obvious deficiency of GH ir\ serum (Milunsky et al 1968;Ruvalcaba et al 1972;Anneren et al 1986), eveti though suboptimal endogenous GH production due to a hypothalamic dysfunction has been suggested in DS (Castells et al 1988). There is a selective deficiency of IGF-1 in sertim in patients with DS older than 2 years of age (Sara etal.…”

Section: Introductionmentioning

confidence: 99%

Normalized growth velocity in children with Down's syndrome during growth hormone therapy

Annerén

Gustafsson

Sara

et al. 1993

J intellect Disabil Res

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Between 6 months and 3 years of age, growth velocity in children with Down's syndrome (DS) is markedly reduced in comparison to that of healthy children. However, after 3 years of age, it is almost normal. Thus, growth retardation becomes pronounced during the period when growth hormone (GH) starts to regulate growth. The present authors report the long‐term effects of GH‐therapy in 16 children with DS, who are being treated for 3 years from the age of 6–9 months. The treatment, Genotropin®, 0–1 U kg−1 BW day−1, was started at a mean age of7.4 (6.9) months. The results after 12 (n= 16), 24 (n= 12) and 30 (n = 8) months are presented. The mean height standard deviation score, SDS (range; Swedish standard), before therapy was ‐ 1.8 (−0.5 to −3.1) and the mean head circumference was −1.2 (−0.4 to −3.5). After 12, 24 and 30 months, the mean height SDS were −1.1 (−0.8 to −1.9), −0.9 (0 to −1.5) and −0.9 (0.1 to ‐ 1.5) and the mean head circumference SDS were −1.1 (0 to −2.5), ‐ M (0 to −2.2) and −1.2 (−0.5 to −2.0), respectively. During hGH‐treatment, the children with DS thus gained height during the first year, and then followed the growth rate of healthy Swedish children. When compared to growth charts for children with DS the mean height of these children started at the fiftieth centile and reached the ninety‐fifth centile after 24 months of treatment. Head circumference only slightly increased during the therapy, and not to the same extent as height. This indicates that small head circumference in DS is not only an effect of growth retardation, but also due to microcephaly.

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Section: Introductionmentioning

confidence: 99%

Normalized growth velocity in children with Down's syndrome during growth hormone therapy

Annerén

Gustafsson

Sara

et al. 1993

J intellect Disabil Res

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“…Children with GH deficiency show reduced SSR, while patients with acromegaly have a higher SSR than healthy controls [ 21 , 23 ]. It is known that DS is associated with growth hormone deficiency [ 24 ]. In our albeit small population, children with DS and a height below the 3rd percentile showed no reduced SSR rate compared with the other children (data not shown).…”

Section: Discussionmentioning

confidence: 99%

Is sweat testing for cystic fibrosis feasible in patients with down syndrome?

et al. 2018

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BackgroundRecurrent airway infections are common in patients with Down’s syndrome (DS). Hence, ruling out Cystic Fibrosis (CF) in these patients is often required. In the past, the value of sweat testing – the gold standard to diagnose CF – has been questioned in DS as false positive results have been reported. However, these reports are based on measurements of sweat osmolality or sodium concentrations, not chloride concentrations. This study analyses sweat secretion rate and chloride concentration in sweat samples of patients with DS in comparison to healthy controls.MethodsWe assessed sweat samples in 16 patients with DS and 16 healthy controls regarding sweat secretion rate (SSR) and sweat chloride concentration.ResultsAll measured chloride concentrations were within the normal range. The chloride concentrations were slightly, but not significantly lower in patients with DS (15,54 mmol/l (±4,47)) compared to healthy controls (18,31 mmol/l (±10,12)). While no gender gap in chloride concentration could be found, chloride concentration increased with age in both groups.Insufficient sweat was collected in 2 females with DS (12.5% of the study group) but not in an individual of the control group. A significant lower sweat secretion rate was found in the DS group (27,6 μl/30 min (± 12,18)) compared to the control group (42,7 μl/30 min (± 21,22)). In a sub-analysis, female patients produced significantly less sweat (20,8 ± 10,6 μl/30 min) than male patients with DS (36,4 ± 7,8 μl/30 min), which accounts for the difference between patients and controls. Furthermore, while the sweating secretion rate increased with age in the control group, it did not do so in the DS group. Once again this was due to female patients with DS, who did not show a significant increase of sweat secretion rate with age.ConclusionsSweat chloride concentrations were within the normal range in patients with DS and therefore seem to be a reliable tool for testing for CF in these patients. Interestingly, we found a reduced sweat secretion rate in the DS group. Whether the last one has a functional and clinical counterpart, possibly due to a disturbed thermoregulation in DS patients, requires further investigation.

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“…Some authors reported reduction of IGF-I levels in DS patients, without any age-related differences, to an extent similar to that in GHD patients [165,[170][171][172], while others showed a progressive decrease during advacing age, although within the age-related normal range [173]. Nutritional, lifestyle and/or social differences may explain these controversial findings.…”

Section: Gh/igf-i Axis In Down's Syndrome: a Clin-ical Model Of Anticmentioning

confidence: 95%

“…Low GH response to L-DOPA and clonidine, two indirect GH-releasing agents, have been shown in prepubertal DS patients, while normal arginine-as well as GHRH-induced GH rise was reported in DS children [171][172][173][174][175][176]. Normal GH response in DS children was also observed after maximal provocative stimuli, such GHRH combined with pyridostigmine or arginine, as well as after hexarelin, a synthetic GH secretagogue mimicking ghrelin action [173,176,177].…”

Section: Gh/igf-i Axis In Down's Syndrome: a Clin-ical Model Of Anticmentioning

confidence: 99%

Human Ageing and the Growth Hormone/Insulin-Like Growth Factor-I (GH/IGF-I) Axis - The Impact of Growth Factors on Dementia

Giordano¹

2012

TOEJ

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There is clear evidence that human ageing is associated with reduced activity of the growth hormone (GH)/insulin-growth factor-I (IGF-I) axis, the so called "somatopause". Although this condition is likely to contribute to age-related changes in body composition, structure functions and metabolism, we are now in face of the paradox of lifelong GH/IGF-I deficiency or resistance resulting in prolonged life expectancy. This evidence questions whether GH deficiency is or is not a beneficial adaptation to ageing. On the other hand, neuroendocrine studies provided evidence that brain ageing is associated to peculiar age-related alterations in the control of GH/IGF-I axis, mostly including GHRH deficiency and SS hyperactivity, reflecting the age-related cholinergic impairment. This hormonal pattern is present in normal and demented elderly subjects, and a neuroendocrine distinction among these conditions is impossible. This review will focus on the age-related decline in the activity of GH/IGF-I axis as a function of either normal or pathological brain ageing. Particularly, the influence of GH/IGF-I axis on cognitive functions and related disorders will be discussed.

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Growth hormone deficiency in Down's syndrome children

Cited by 21 publications

References 46 publications

Normalized growth velocity in children with Down's syndrome during growth hormone therapy

Normalized growth velocity in children with Down's syndrome during growth hormone therapy

Is sweat testing for cystic fibrosis feasible in patients with down syndrome?

Human Ageing and the Growth Hormone/Insulin-Like Growth Factor-I (GH/IGF-I) Axis - The Impact of Growth Factors on Dementia

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