CardiomyopathyEchocardiographic dimensions and function in adults with primary growth hormone resistance (Laron Syndrome)
Section snippets
Methods
The study was approved by the hospital ethical committee, and each patient signed an inform consent before testing.
Cardiac dimensions
The values of LV dimensions are displayed in Table I. Mean estimated LV mass in patients with LS was 85 ± 16 g; normalized to body surface area (BSA), LV mass was 70 ± 17 g/m2 and to height it was 66 ± 13 g/m. It is apparent that LV wall thickness, cardiac dimensions, LV volume, and mass are significantly smaller compared with the control group.
Doppler variables
Doppler measurements are displayed in Table I. LV outflow tract diameter as well as estimated LV stroke volume and cardiac output were significantly
Discussion
This is the first study of cardiac dimensions and functions in adult patients with primary GH resistance (LS), who due to molecular defects of the GH receptor or postreceptor pathways, have no GH signal transmission and no generation of the circulating IGF-I but high levels of GH.7, 8, 9 Because of this syndrome, we were able to evaluate the long-term role of these hormones on cardiac structure and function. In the present study, LV dimensions, Doppler-derived outflow parameters, and estimated
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Cited by (39)
IGF-I deficiency and enhanced insulin sensitivity due to a mutated growth hormone receptor gene in humans
2021, Molecular and Cellular EndocrinologyGrowth hormone receptor-deficient pigs resemble the pathophysiology of human Laron syndrome and reveal altered activation of signaling cascades in the liver
2018, Molecular MetabolismCitation Excerpt :Measurements of organ weights in GHR-KO pigs revealed that growth of liver, kidneys, and heart is particularly dependent on GHR/GH action, as their relative weights were significantly decreased in comparison to control pigs. Reduced relative liver and kidney weights have also been observed in Ghr KO mice [55], and interestingly LS patients have disproportionately reduced cardiac dimensions [59]. The important role of the GH/IGF1 system in the growth of these organs is supported by their disproportionate overgrowth in conditions of GH/IGF1 excess, as in GH-overexpressing transgenic mice [60,61] and in patients with acromegaly [62].
Insulin-like growth factor-1 signaling in cardiac aging
2018, Biochimica et Biophysica Acta - Molecular Basis of DiseaseCitation Excerpt :A recent long-term study of human Laron dwarfs in Ecuador revealed that extreme innate IGF-1 deficiency in humans is connected with a shorter-than-expected lifespan, with the majority of the mortality being a result of CVD and stroke [136]. The available evidence suggested that untreated Laron dwarf patients had reduced cardiac output and dimensions at rest as well as decreased left ventricular contractile reserve following stress; alterations that could be improved by IGF-1 supplementation [137–139]. IGF-1 treatment has also been shown to reduce circulating levels of lipoprotein (a) in patients with Laron syndrome [27,140].
Lessons from 50 years of study of laron syndrome
2015, Endocrine PracticeCitation Excerpt :The head circumference, denoting brain size, is small (35), similar to that reported for IGF-1 gene deletion (30) and IGF-1 receptor mutations (31). The hands (36) and feet (37) are small, and so are the internal organs, causing acro- and organomicria (38). In childhood, the genitalia are small (39,40) and puberty is delayed, mainly in the males (41), but all reach full sexual development with a normal reproduction potential (42).
Importance of circulating IGF-1 for normal cardiac morphology, function and post infarction remodeling
2012, Growth Hormone and IGF ResearchCitation Excerpt :This served as a guide for M-mode tracing of the Left Ventricular Outflow Tract and LV (left ventricle). Pulsed wave Doppler recordings, with minimum sample size and a pulse frequency of 5 MHz, was used to record the peak left ventricular outflow tract velocity in an attempt to estimate stroke volume (SV) and cardiac index (CI) [9]. All tracings were recorded at a sweep of 200 mm/s and were stored in magnetic optical discs for off-line evaluation using an image analysis system (Echo Pac 5.4, VingMed).
IGF-I replacement therapy in children with congenital IGF-I deficiency (Laron syndrome) maintains heart dimension and function
2009, Growth Hormone and IGF Research