Fifty seven years of follow-up of the Israeli cohort of Laron Syndrome patients—From discovery to treatment

Laron, Zvi; Kauli, Rivka

doi:10.1016/j.ghir.2015.08.004

Cited by 49 publications

(66 citation statements)

References 26 publications

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“…Patients with GH insensitivity, particularly Laron syndrome caused by to mutations in the GHR, produce little to no IGF-I or IGFBP-3, resulting in decreased longitudinal bone growth; short body length at birth is followed by short stature during and after growth (Laron and Kauli, 2015). Mouse models of GH insensitivity indicate significant reductions in cortical bone parameters as well as measures of endosteal and periosteal bone formation (Wu et al, 2013).…”

Section: Gh/igf Effects On Bonementioning

confidence: 99%

“…Mouse models of GH insensitivity indicate significant reductions in cortical bone parameters as well as measures of endosteal and periosteal bone formation (Wu et al, 2013). In GH-insensitive patients, IGF-I administration can stimulate some longitudinal bone growth, although it cannot fully compensate for the absent GH effects (Collett-Solberg et al, 2008; Laron and Kauli, 2015; Laron and Klinger, 1994; Pfäffle, 2015; Walker et al, 1992; Wu et al, 2013). Furthermore, GH and IGF synergize to produce greater effects than either would independently (Fielder et al, 1996).…”

Section: Gh/igf Effects On Bonementioning

confidence: 99%

“…However, further studies are needed to determine the safety and magnitude of increase in final height resulting from long-term GH therapy in all patient groups (Pfäffle, 2015). Furthermore, children with GH insensitivity, most commonly caused by resistance to GH due to mutations in the GHR (called Laron syndrome, described in section 3.1), have been successfully treated with IGF-I (Laron and Kauli, 2015). The inability of IGF-I to fully compensate for the lack of GH action in these patients may be attributable to the lack of IGFBP-3 carrying, stabilizing, and enhancing IGF-I in circulation as well as the lack of direct GH stimulation in bone (Pfäffle, 2015).…”

Section: Gh/igf Therapymentioning

confidence: 99%

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Skeletal effects of growth hormone and insulin-like growth factor-I therapy

Lindsey

Mohan

2016

Molecular and Cellular Endocrinology

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The growth hormone/insulin-like growth factor (GH/IGF) axis is critically important for the regulation of bone formation, and deficiencies in this system have been shown to contribute to the development of osteoporosis and other diseases of low bone mass. The GH/IGF axis is regulated by a complex set of hormonal and local factors which can act to regulate this system at the level of the ligands, receptors, IGF binding proteins (IGFBPs), or IGFBP proteases. A combination of in vitro studies, transgenic animal models, and clinical human investigations has provided ample evidence of the importance of the endocrine and local actions of both GH and IGF-I, the two major components of the GH/IGF axis, in skeletal growth and maintenance. GH- and IGF-based therapies provide a useful avenue of approach for the prevention and treatment of diseases such as osteoporosis.

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Section: Gh/igf Effects On Bonementioning

confidence: 99%

Section: Gh/igf Effects On Bonementioning

confidence: 99%

Section: Gh/igf Therapymentioning

confidence: 99%

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Skeletal effects of growth hormone and insulin-like growth factor-I therapy

Lindsey

Mohan

2016

Molecular and Cellular Endocrinology

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“…This was further confirmed when Laron cloned the GH receptor (GHR) from two of his patients, revealing a partial gene deletion [2]. Since the rise of the polymerase chain reaction technique, numerous mutations in the GHR gene have been identified [1].…”

Section: Introductionmentioning

confidence: 82%

Primary growth hormone insensitivity and psychomotor delay

Castilla‐Cortázar

Ita

Aguirre

et al. 2018

Clinical Case Reports

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“…The authors suggested two possible mechanisms for the patients' short stature: either an abnormal structure of GH or resistance to a GH of normal structure. It is remarkable that 49 years later, Zvi Laron just reported “Fifty Seven Years of Follow Up of the Israeli Cohort of Laron Syndrome Patients - From Discovery to Treatment” [8]. The following are some highlights of his work.…”

Section: The Pastmentioning

confidence: 99%

Past, Present, and Future in the Relationship between Growth Retardation and the IGF System: Excerpts from the Cesar Bergada Lecture Given during the SLEP 2015 Annual Meeting

Jásper¹

2016

Horm Res Paediatr

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This mini review presents a personal view about the past, the present and the future of the relationship between growth retardation and the IGF system. Looking back, it is pertinent to include a brief look at the evolution of the somatomedin hypothesis, the use of IGF-I determinations in the clinic, and a review of the literature beginning in the late 1980s with the description of mutations in the Growth Hormone Receptor (GHR) gene.The present possibly started in the mid-1990s with the description of mutations in the IGF-I gene, followed in 2003 by reports of mutations in the genes coding for the IGF-I receptor and in the signal transducer and activator of transcription 5b (STAT5b).Finally, in 2004, mutations in the IGFALS gene were described. A diffuse limit between the present and the future might have been reached (the author's arbitrary decision) with the clinical applications of whole exome sequencing, which rapidly showed mutations in genes coding for STAT3, PAPP-A2 (pregnancy-associated plasma protein A2), and IGF-II.

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Fifty seven years of follow-up of the Israeli cohort of Laron Syndrome patients—From discovery to treatment

Cited by 49 publications

References 26 publications

Skeletal effects of growth hormone and insulin-like growth factor-I therapy

Skeletal effects of growth hormone and insulin-like growth factor-I therapy

Primary growth hormone insensitivity and psychomotor delay

Past, Present, and Future in the Relationship between Growth Retardation and the IGF System: Excerpts from the Cesar Bergada Lecture Given during the SLEP 2015 Annual Meeting

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