Laron Syndrome (Syndrome, Laron)

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192 relevant articles (1 outcomes, 21 trials/studies) found for this Disease

Description: An autosomal recessive disorder characterized by short stature, defective GROWTH HORMONE RECEPTOR, and failure to generate INSULIN-LIKE GROWTH FACTOR I by GROWTH HORMONE. Laron syndrome is not a form of primary pituitary dwarfism (GROWTH HORMONE DEFICIENCY DWARFISM) but the result of mutation of the human GHR gene on chromosome 5.

Also Known As:

Syndrome, Laron; Growth Hormone Receptor Defect; Laron Dwarfism; Laron Type Dwarfism I; Primary GH Resistance; Primary Growth Hormone Resistance; Severe GH Insensitivity; Dwarfism, Laron; GH Resistance, Primary; Growth Hormone Insensitivity Syndrome

Relationship Network

Disease Context: Research Results

Musculoskeletal Diseases: 83

Bone Diseases: 2376

Developmental Bone Diseases: 63

Dwarfism: 680

Laron Syndrome: 192

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 420

Inborn Genetic Diseases: 548

Dwarfism: 680

Laron Syndrome: 192

Endocrine System Diseases: 390

Dwarfism: 680

Laron Syndrome: 192

Related Diseases

1.	Laron Syndrome (Syndrome, Laron)
2.	Syndrome
3.	Multiple Sclerosis
4.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
5.	Alzheimer Disease (Alzheimer's Disease)
56 Diseases and 59 more articles analyzed in the Research Interface, order at left...

Experts

1.	Laron, Zvi: 13 articles (03/2008 - 12/2002)
2.	Laron, Z: 5 articles (02/2008 - 01/2001)
3.	Lilos, Pearl: 3 articles (12/2007 - 04/2003)
4.	Kornreich, L: 2 articles (02/2008 - 04/2002)
5.	Schwarz, M: 2 articles (02/2008 - 04/2002)
6.	Horev, G: 2 articles (02/2008 - 04/2002)
7.	Kornreich, Liora: 2 articles (10/2007 - 06/2006)
8.	Hershkovitz, Israel: 2 articles (10/2007 - 06/2006)
9.	Weinberger, Dov: 2 articles (07/2006 - 07/2004)
10.	Camacho-Hübner, Cecilia: 2 articles (04/2006 - 02/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Laron Syndrome:

1.	Insulin-Like Growth Factor I (IGF-1)IBA 12/01/1999 - "CONCLUSION: This study reveals that IGF-I treatment at a dose of 80 microg/kg twice daily is effective in patients with growth hormone insensitivity syndrome" 04/01/2006 - "Pharmacokinetic studies of recombinant human insulin-like growth factor I (rhIGF-I)/rhIGF-binding protein-3 complex administered to patients with growth hormone insensitivity syndrome." 01/01/2006 - "Laron syndrome (LS) or primary GH insensitivity is a unique human model to study the effects of congenital IGF-I deficiency" 10/01/2003 - "This study was designed to assess body composition and bone density in children with growth hormone insensitivity syndrome before and after receiving treatment with recombinant IGF-I. DESIGN: A prospective longitudinal study" 04/01/2001 - "Therapy for 6.5-7.5 years with recombinant insulin-like growth factor I in children with growth hormone insensitivity syndrome: a clinical research center study." Get all the results and reference details, order at left...
2.	Growth Hormone (Somatotropin)IBA 02/01/2008 - "Abnormalities of the axial and proximal appendicular skeleton in adults with Laron syndrome (growth hormone insensitivity)." 10/01/2007 - "Comparative skeletal features between Homo floresiensis and patients with primary growth hormone insensitivity (Laron Syndrome)." 11/01/2006 - "Mutations in the STAT5b gene, described in 2 patients, lead to growth hormone insensitivity that resembles Laron syndrome" 01/01/2006 - "Since the first paper that showed the etiology of Laron syndrome [Godowski PJ, et al: Proc Natl Acad Sci USA 1989;86:8083-8087], many mutations in the growth hormone (GH) receptor have been identified" 12/01/2002 - "Growth hormone insensitivity (Laron syndrome)." Get all the results and reference details, order at left...
3.	Somatotropin Receptors (Growth Hormone Receptor)IBA 08/01/2007 - "Laron syndrome is an autosomal recessive disorder caused by defects of growth hormone receptor (GHR) gene" 08/01/2007 - "Clinical features and growth hormone receptor gene mutations of patients with Laron syndrome from a Chinese family." 05/01/2007 - "Growth hormone receptor gene mutations in two Italian patients with Laron Syndrome." 06/01/2006 - "For debate: did the small-bodied hominis from flores (Indonesia) suffer from a molecular defect in the growth hormone receptor gene (Laron syndrome)?" 04/01/2006 - "Growth hormone-insensitivity syndrome (GHIS) is usually caused by mutations in the growth hormone receptor" Get all the results and reference details, order at left...
4.	somatotropin-binding protein (growth hormone-binding protein)IBA 01/01/1998 - "We have previously described two families (H and M) with GH binding protein-positive Laron Syndrome (LS), proposed to have one or more post GHR signaling defects" 12/01/1997 - "A minority of patients with Laron syndrome have normal serum GH binding protein (GHBP), indicating that the defect is elsewhere than in the extracellular domain of the GH receptor" 05/01/1997 - "Girls with Laron syndrome having positive growth hormone binding protein (GHBP) are less retarded in height than those lacking GHBP." 08/01/1995 - "Patients with idiopathic short stature have decreased serum levels of the GH receptor-related GH-binding protein (GHBP), and low GHBP levels are associated with complete GH insensitivity (Laron) syndrome" 06/01/1994 - "In Laron syndrome, growth retardation is caused by GH resistance due to GH receptor (GH-R) defects, which are associated in most cases with absent or low serum concentrations of the GH-R-related GH-binding protein (GHBP)" Get all the results and reference details, order at left...
5.	Insulin (Novolin)FDA Link 09/01/2004 - "GHR(-/-) mice were different from the human Laron syndrome in serum insulin level, insulin responsiveness, and obesity" 01/01/1986 - "These are hypopituitarism, Laron dwarfism, leprechaunism, liver disease, primary hypothyroidism, extrapancreatic tumor hypoglycemia and severe insulin deficiency in experimental animals" 05/01/1987 - "In this study, we found that erythroid progenitor cells and permanently transformed T-cell lines from two patients with Laron dwarfism responded in vitro to added IGF-I in concentrations ranging between 1-10 ng/mL despite no stimulatory response to added GH in concentrations of up to 500 ng/mL. Normal or near-normal responsiveness to insulin was also demonstrated" 02/01/2008 - "OBJECTIVE: To investigate abnormalities in the skeleton (with the exclusion of the skull, cervical spine, hands and feet) in patients with Laron syndrome, who have an inborn growth hormone resistance and congenital insulin-like growth factor-1 (IGF-1) deficiency" 01/01/2004 - "BACKGROUND: Classical Laron syndrome is a recessive disease of primary insulin-like growth factor 1 (IGF-1) deficiency and primary growth hormone insensitivity" Get all the results and reference details, order at left...
6.	Carrier Proteins (Binding Protein)IBA 12/01/1997 - "We have evaluated the effect of long-term IGF-I treatment on serum IGF-binding protein (IGFBP)-3 and the acid-labile subunit (ALS) in three sibling with Laron syndrome caused by a GH post-receptor defect and with normal GHBP" 11/25/1997 - "A mammalian model for Laron syndrome produced by targeted disruption of the mouse growth hormone receptor/binding protein gene (the Laron mouse)." 06/01/1994 - "We have selected GHRD (Laron syndrome) as the prototypic disorder of GHI, in part because such dramatic and rapid progress has been made in this clinical condition over the last 6 yr. These advances represent the fortunate convergence of: 1) the cloning of the GHR gene and the identification of deletions and mutations of this gene in GHRD; 2) the development of assay methods for measurement of the GHBP, IGF peptides, and binding proteins; 3) the discovery of a larger number of affected individuals than had been previously suspected, including the recognition and description of a large genetically homogeneous population of GHRD patients in Ecuador; and 4) the production of recombinant IGF-I for therapeutic trials in GHRD" 03/01/2007 - "In addition, the serum half-life of unbound rhIGF-I is shorter when administered to patients with growth hormone insensitivity syndrome, who have low serum concentrations of its binding proteins IGFBP-3 and acid-labile subunit, than when administered to healthy volunteers or to patients with an IGF-I gene deletion who have normal levels of IGFBP-3" 04/01/2003 - "SomatoKine, a recombinant fusion of insulin-like growth factor-1 and its binding protein, BP-3, is being developed by Insmed (formerly Celtrix Pharmaceuticals) for the potential treatment of growth hormone insensitivity syndrome (GHIS), and as a potential injectable insulin sensitizer for the management of type 1 and type 2 diabetes in patients who are less sensitive to insulin therapy" Get all the results and reference details, order at left...
7.	Fibrinogen (Factor I)IBA 07/01/2006 - "Insulinlike growth factor I affects ocular development: a study of untreated and treated patients with Laron syndrome." 01/01/2008 - "Laron syndrome is an autosomal recessive disorder of GH insensitivity that results in decreased insulinlike growth factor-I (IGF-I) synthesis and growth failure" 07/01/2006 - "OBJECTIVE: To evaluate the ocular dimensions in patients with primary growth hormone receptor insensitivity (Laron syndrome [LS]) and to study the effect of supplemental insulinlike growth factor I (IGF-I) on ocular growth" 04/01/1994 - "A survey to identify children and adolescents with primary growth hormone insensitivity syndrome (GHIS) yielded 38 patients who were positively identified using a scoring system that included five criteria: height, basal growth hormone (GH), GH binding protein, basal insulin-like growth factor I (IGF-I) and the increase of IGF-I after 4 days of GH administration (IGF generation test)" Get all the results and reference details, order at left...
8.	PeptidesIBA 06/01/1994 - "We have selected GHRD (Laron syndrome) as the prototypic disorder of GHI, in part because such dramatic and rapid progress has been made in this clinical condition over the last 6 yr. These advances represent the fortunate convergence of: 1) the cloning of the GHR gene and the identification of deletions and mutations of this gene in GHRD; 2) the development of assay methods for measurement of the GHBP, IGF peptides, and binding proteins; 3) the discovery of a larger number of affected individuals than had been previously suspected, including the recognition and description of a large genetically homogeneous population of GHRD patients in Ecuador; and 4) the production of recombinant IGF-I for therapeutic trials in GHRD" 01/01/2005 - "In classical GH insensitivity syndrome (GHIS), known as Laron syndrome, due to GH receptor (GHR) deficiency, serum IGF-I, IGFBP-3 and ALS are severely reduced with inability to produce these peptides during an IGF-I generation test" Get all the results and reference details, order at left...
9.	Dihydrotestosterone (Androstanolone)IBA 01/01/2004 - "OBJECTIVE: The study describes for the first time, a homozygous mutation in the WSXWS-like motif of the human GH receptor (GHR) in a patient with Laron syndrome and describe laboratory data during treatment with GnRHa to suppress puberty and dihydrotestosterone (DHT)" 01/01/2004 - "The first homozygous mutation (S226I) in the highly-conserved WSXWS-like motif of the GH receptor causing Laron syndrome: supression of GH secretion by GnRH analogue therapy not restored by dihydrotestosterone administration." Get all the results and reference details, order at left...
10.	Prolactin Receptors (Prolactin Receptor)IBA 03/01/1990 - "Since cloned cDNAs for human and rabbit GH receptors and rat PRL receptors show a high degree of sequence homology, we undertook studies of PRL action in cells from patients with Laron dwarfism to determine if the Laron defect was also associated with PRL unresponsiveness" Get all the results and reference details, order at left...
72 Drugs and Important Bio-Agents and 191 more articles analyzed in the Research Interface, order at left...

Therapies and Procedures

Aftercare (After-Treatment)

11/01/1979 - "The mean (+/- SEM) IRSM concentrations in sera from normals and patients with acromegaly, hypopituitarism, GH deficiency before/after treatment, and Laron dwarfism were 1.45 +/- 0.17, 5.49 +/- 0.48, 0.19 +/- 0.07, 0.10 +/- 0.02/0.64 +/- 0.45, and 0.25 +/- 0.11 U/ml, respectively, compared to a pooled normal human serum reference standard which was designated to contain 1 IRSM U/ml. Measurements of total IRSM (bound and free) in serum may not accurately reflect SM bioactivity and will require interpretative caution."
Get all the results and reference details, order at left...

1 Therapies and Procedures and 1 more articles analyzed in the Research Interface, order at left...

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