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Increased insulin like growth factor binding protein acid labile subunit concentration

MedGen UID:
1814347
•Concept ID:
C5676778
•
Finding
Synonym: Increased circulating IGFALS contration
 
HPO: HP:0034184

Definition

An abnormally elevated concentration of insulin like growth factor binding protein acid labile subunit level in the blood circulation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased insulin like growth factor binding protein acid labile subunit concentration

Conditions with this feature

Short stature, Dauber-Argente type
MedGen UID:
1794178
•Concept ID:
C5561968
•
Disease or Syndrome
Short stature of the Dauber-Argente type (SSDA) is characterized by progressive postnatal growth failure, moderate microcephaly, thin long bones, and mildly decreased bone density. Patients have elevated circulating levels of total IGF1 (147440) due to impaired proteolysis of IGFBP3 (146732) and IGFBP5 (146734), resulting in reduced free IGF1 (Dauber et al., 2016).
See: Condition Record
Short stature, Dauber-Argente type

Recent clinical studies

Etiology

Postoperative evaluation of patients with acromegaly: clinical significance and timing of oral glucose tolerance testing and measurement of (free) insulin-like growth factor I, acid-labile subunit, and growth hormone-binding protein levels.
Feelders RA, Bidlingmaier M, Strasburger CJ, Janssen JA, Uitterlinden P, Hofland LJ, Lamberts SW, van der Lely AJ, de Herder WW
J Clin Endocrinol Metab 2005 Dec;90(12):6480-9. Epub 2005 Sep 13 doi: 10.1210/jc.2005-0901. PMID: 16159936
Plasma insulin-like growth factors (IGFs), IGF-Binding proteins (IGFBPs), acid-labile subunit (ALS) and IGFBP-3 proteolysis in individuals with clinical characteristics of Sotos syndrome.
de Boer L, Hoogerbrugge CM, van Doorn J, van Buul-Offers SC, Karperien M, Wit JM
J Pediatr Endocrinol Metab 2004 Apr;17(4):615-27. doi: 10.1515/jpem.2004.17.4.615. PMID: 15198293
The growth hormone/insulin-like growth factor-I axis hormones and bone markers in elite athletes in response to a maximum exercise test.
Ehrnborg C, Lange KH, Dall R, Christiansen JS, Lundberg PA, Baxter RC, Boroujerdi MA, Bengtsson BA, Healey ML, Pentecost C, Longobardi S, Napoli R, Rosén T; GH-2000 Study Group
J Clin Endocrinol Metab 2003 Jan;88(1):394-401. doi: 10.1210/jc.2002-020037. PMID: 12519882
Serum levels of insulin-like growth factor binding proteins in Ecuadorean children with growth hormone insensitivity.
Burren CP, Wanek D, Mohan S, Cohen P, Guevara-Aguirre J, Rosenfeld RG
Acta Paediatr Suppl 1999 Feb;88(428):185-91; discussion 192. doi: 10.1111/j.1651-2227.1999.tb14387.x. PMID: 10102078
Effects of growth hormone on insulin-like growth factors and binding proteins before and after renal transplantation.
van Renen MJ, Harrer S, Jureidini KF, Martin AA
Pediatr Nephrol 1996 Aug;10(4):483-7. doi: 10.1007/s004670050144. PMID: 8865248

Diagnosis

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation.
Aguiar-Oliveira MH, Souza AHO, Oliveira CRP, Campos VC, Oliveira-Neto LA, Salvatori R
Eur J Endocrinol 2017 Aug;177(2):R85-R97. Epub 2017 Apr 20 doi: 10.1530/EJE-16-1047. PMID: 28428227
Postoperative evaluation of patients with acromegaly: clinical significance and timing of oral glucose tolerance testing and measurement of (free) insulin-like growth factor I, acid-labile subunit, and growth hormone-binding protein levels.
Feelders RA, Bidlingmaier M, Strasburger CJ, Janssen JA, Uitterlinden P, Hofland LJ, Lamberts SW, van der Lely AJ, de Herder WW
J Clin Endocrinol Metab 2005 Dec;90(12):6480-9. Epub 2005 Sep 13 doi: 10.1210/jc.2005-0901. PMID: 16159936
The acid-labile subunit of human ternary insulin-like growth factor-binding protein complex in girls with central precocious puberty before and during gonadotropin-releasing hormone analog therapy.
Cisternino M, Draghi M, Lauriola S, Scarcella D, Bernasconi S, Cavallo L, De Luca F, Lomeo A, Tatò L
J Clin Endocrinol Metab 2002 Oct;87(10):4629-33. doi: 10.1210/jc.2002-020308. PMID: 12364447
Diagnostic value of the acid-labile subunit in acromegaly: evaluation in comparison with insulin-like growth factor (IGF) I, and IGF-binding protein-1, -2, and -3.
Arosio M, Garrone S, Bruzzi P, Faglia G, Minuto F, Barreca A
J Clin Endocrinol Metab 2001 Mar;86(3):1091-8. doi: 10.1210/jcem.86.3.7288. PMID: 11238491
The acid-labile subunit of human ternary insulin-like growth factor binding protein complex in serum: hepatosplanchnic release, diurnal variation, circulating concentrations in healthy subjects, and diagnostic use in patients with growth hormone deficiency.
Juul A, Møller S, Mosfeldt-Laursen E, Rasmussen MH, Scheike T, Pedersen SA, Kastrup KW, Yu H, Mistry J, Rasmussen S, Müller J, Henriksen J, Skakkebaek NE
J Clin Endocrinol Metab 1998 Dec;83(12):4408-15. doi: 10.1210/jcem.83.12.5311. PMID: 9851786

Therapy

High protein diets do not attenuate decrements in testosterone and IGF-I during energy deficit.
Henning PC, Margolis LM, McClung JP, Young AJ, Pasiakos SM
Metabolism 2014 May;63(5):628-32. Epub 2014 Feb 17 doi: 10.1016/j.metabol.2014.02.007. PMID: 24641883
Splanchnic exchange of insulin-like growth factor binding protein-1 (IGFBP-1), IGF-I and acid-labile subunit (ALS) during normo- and hyper-insulinaemia in healthy subjects.
Fernqvist-Forbes E, Ekberg K, Lindgren BF, Brismar K
Clin Endocrinol (Oxf) 1999 Sep;51(3):327-32. doi: 10.1046/j.1365-2265.1999.00775.x. PMID: 10469012
Growth hormone stimulates transcription of the gene encoding the acid-labile subunit (ALS) of the circulating insulin-like growth factor-binding protein complex and ALS promoter activity in rat liver.
Ooi GT, Cohen FJ, Tseng LY, Rechler MM, Boisclair YR
Mol Endocrinol 1997 Jun;11(7):997-1007. doi: 10.1210/mend.11.7.9942. PMID: 9178759
Effects of growth hormone on insulin-like growth factors and binding proteins before and after renal transplantation.
van Renen MJ, Harrer S, Jureidini KF, Martin AA
Pediatr Nephrol 1996 Aug;10(4):483-7. doi: 10.1007/s004670050144. PMID: 8865248
Complex formation by human insulin-like growth factor-binding protein-3 and human acid-labile subunit in growth hormone-deficient rats.
Lewitt MS, Saunders H, Phuyal JL, Baxter RC
Endocrinology 1994 Jun;134(6):2404-9. doi: 10.1210/endo.134.6.7514998. PMID: 7514998

Prognosis

Obesity due to melanocortin 4 receptor (MC4R) deficiency is associated with increased linear growth and final height, fasting hyperinsulinemia, and incompletely suppressed growth hormone secretion.
Martinelli CE, Keogh JM, Greenfield JR, Henning E, van der Klaauw AA, Blackwood A, O'Rahilly S, Roelfsema F, Camacho-Hübner C, Pijl H, Farooqi IS
J Clin Endocrinol Metab 2011 Jan;96(1):E181-8. Epub 2010 Nov 3 doi: 10.1210/jc.2010-1369. PMID: 21047921
Postoperative evaluation of patients with acromegaly: clinical significance and timing of oral glucose tolerance testing and measurement of (free) insulin-like growth factor I, acid-labile subunit, and growth hormone-binding protein levels.
Feelders RA, Bidlingmaier M, Strasburger CJ, Janssen JA, Uitterlinden P, Hofland LJ, Lamberts SW, van der Lely AJ, de Herder WW
J Clin Endocrinol Metab 2005 Dec;90(12):6480-9. Epub 2005 Sep 13 doi: 10.1210/jc.2005-0901. PMID: 16159936
Diagnostic value of the acid-labile subunit in acromegaly: evaluation in comparison with insulin-like growth factor (IGF) I, and IGF-binding protein-1, -2, and -3.
Arosio M, Garrone S, Bruzzi P, Faglia G, Minuto F, Barreca A
J Clin Endocrinol Metab 2001 Mar;86(3):1091-8. doi: 10.1210/jcem.86.3.7288. PMID: 11238491
Serum levels of insulin-like growth factor binding proteins in Ecuadorean children with growth hormone insensitivity.
Burren CP, Wanek D, Mohan S, Cohen P, Guevara-Aguirre J, Rosenfeld RG
Acta Paediatr Suppl 1999 Feb;88(428):185-91; discussion 192. doi: 10.1111/j.1651-2227.1999.tb14387.x. PMID: 10102078
Evidence for insulin-like growth factor (IGF)-independent transcriptional regulation of IGF binding protein-3 by growth hormone in SKHEP-1 human hepatocarcinoma cells.
Gucev ZS, Oh Y, Kelley KM, Labarta JI, Vorwerk P, Rosenfeld RG
Endocrinology 1997 Apr;138(4):1464-70. doi: 10.1210/endo.138.4.5060. PMID: 9075703

Clinical prediction guides

MECHANISMS IN ENDOCRINOLOGY: The multiple facets of GHRH/GH/IGF-I axis: lessons from lifetime, untreated, isolated GH deficiency due to a GHRH receptor gene mutation.
Aguiar-Oliveira MH, Souza AHO, Oliveira CRP, Campos VC, Oliveira-Neto LA, Salvatori R
Eur J Endocrinol 2017 Aug;177(2):R85-R97. Epub 2017 Apr 20 doi: 10.1530/EJE-16-1047. PMID: 28428227
Postoperative evaluation of patients with acromegaly: clinical significance and timing of oral glucose tolerance testing and measurement of (free) insulin-like growth factor I, acid-labile subunit, and growth hormone-binding protein levels.
Feelders RA, Bidlingmaier M, Strasburger CJ, Janssen JA, Uitterlinden P, Hofland LJ, Lamberts SW, van der Lely AJ, de Herder WW
J Clin Endocrinol Metab 2005 Dec;90(12):6480-9. Epub 2005 Sep 13 doi: 10.1210/jc.2005-0901. PMID: 16159936
Acid-labile subunit deficiency: phenotypic similarities and differences between human and mouse.
Domené HM, Bengolea SV, Jasper HG, Boisclair YR
J Endocrinol Invest 2005;28(5 Suppl):43-6. PMID: 16114275
Pharmacokinetics of insulin-like growth factor I in hypopituitarism: correlation with binding proteins.
Mauras N, Quarmby V, Bloedow DC
Am J Physiol 1999 Oct;277(4):E579-84. doi: 10.1152/ajpendo.1999.277.4.E579. PMID: 10516115
Functions and regulation of the acid-labile subunit of the 150 K complex.
Barreca A, Ponzani P, Arvigo A, Voci A, Giordano G, Minuto F
Prog Growth Factor Res 1995;6(2-4):231-9. doi: 10.1016/0955-2235(95)00006-2. PMID: 8817666

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