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Quoted phrase not found in phrase index: "Abnormal insulin like growth factor binding protein acid labile subunit level"
Page 1
Human acid-labile subunit deficiency: clinical, endocrine and metabolic consequences.
Domené HM, Hwa V, Argente J, Wit JM, Camacho-Hübner C, Jasper HG, Pozo J, van Duyvenvoorde HA, Yakar S, Fofanova-Gambetti OV, Rosenfeld RG; International ALS Collaborative Group. Domené HM, et al. Horm Res. 2009;72(3):129-41. doi: 10.1159/000232486. Epub 2009 Sep 1. Horm Res. 2009. PMID: 19729943 Free article. Review.
Fourteen different mutations of the human IGFALS gene have been identified in 17 patients, suggesting that ALS deficiency may be prevalent in a subset of patients with extraordinarily low serum levels of IGF-I and IGFBP-3 that remain abnormally low upon growth hormo …
Fourteen different mutations of the human IGFALS gene have been identified in 17 patients, suggesting that ALS deficiency may be prevalent i …
IGF-I-IGFBP-3-acid-labile subunit (ALS) complex in children and adolescents with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH).
Völkl TM, Rauh M, Schöfl C, Dörr HG. Völkl TM, et al. Growth Horm IGF Res. 2011 Aug;21(4):191-8. doi: 10.1016/j.ghir.2011.05.001. Epub 2011 Jun 1. Growth Horm IGF Res. 2011. PMID: 21636299 Clinical Trial.
It has been shown that changes in IGF-I and IGFBP levels in children with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) are correlated with different states of metabolic control. ...All patients had genetically proven CAH and receiv …
It has been shown that changes in IGF-I and IGFBP levels in children with classical congenital adrenal hyperplasia due to 21-h …
Noonan syndrome: relationships between genotype, growth, and growth factors.
Limal JM, Parfait B, Cabrol S, Bonnet D, Leheup B, Lyonnet S, Vidaud M, Le Bouc Y. Limal JM, et al. J Clin Endocrinol Metab. 2006 Jan;91(1):300-6. doi: 10.1210/jc.2005-0983. Epub 2005 Nov 1. J Clin Endocrinol Metab. 2006. PMID: 16263833
Birth length was reduced [mean -1.2 sd score (SDS); six m+ and two m- were < -2 SDS] but not birth weight. ...M- patients. ALS levels (n = 10) were also very low. By contrast, the mean basal IGFBP-3 value (n = 19) was normal. ...

Birth length was reduced [mean -1.2 sd score (SDS); six m+ and two m- were < -2 SDS] but not birth weight. ...M- patients. ALS

IGFALS gene dosage effects on serum IGF-I and glucose metabolism, body composition, bone growth in length and width, and the pharmacokinetics of recombinant human IGF-I administration.
Högler W, Martin DD, Crabtree N, Nightingale P, Tomlinson J, Metherell L, Rosenfeld R, Hwa V, Rose S, Walker J, Shaw N, Barrett T, Frystyk J. Högler W, et al. J Clin Endocrinol Metab. 2014 Apr;99(4):E703-12. doi: 10.1210/jc.2013-3718. Epub 2014 Jan 13. J Clin Endocrinol Metab. 2014. PMID: 24423360 Clinical Trial.

RESULTS: IGF-I and IGFBP-3 increased above baseline (P < .05) for 2.5 hours, returning to baseline 7 hours after rhIGF-I injection. Mean (SD) IGF-I Z-score increased by 2.49 (0.90), whereas IGFBP-3 Z-score increased by 0.57 (0.10) only. ...Bone analysis showed th

RESULTS: IGF-I and IGFBP-3 increased above baseline (P < .05) for 2.5 hours, returning to baseline 7 hours after rhIGF-I injection. Mean

IGF-1 Levels, Complex Formation, and IGF Bioactivity in Growth Hormone-Treated Children With Prader-Willi Syndrome.
Bakker NE, van Doorn J, Renes JS, Donker GH, Hokken-Koelega AC. Bakker NE, et al. J Clin Endocrinol Metab. 2015 Aug;100(8):3041-9. doi: 10.1210/jc.2015-1410. Epub 2015 May 29. J Clin Endocrinol Metab. 2015. PMID: 26050733
RESULTS: Serum IGF-1, IGFBP-3, and ALS levels and IGF-1 to IGFBP-3 ratio were significantly higher in GH-treated PWS children than in healthy controls. ...IGF bioactivity during GH showed a wide variation, and there was a disrupted correlation with immunoreactive IGF-1 …
RESULTS: Serum IGF-1, IGFBP-3, and ALS levels and IGF-1 to IGFBP-3 ratio were significantly higher in GH-treated PWS children than in …
Insulin-like growth factors (IGF-I, free IGF-I and IGF-II) and insulin-like growth factor binding proteins (IGFBP-2, IGFBP-3, IGFBP-6, and ALS) in blood circulation.
Yu H, Mistry J, Nicar MJ, Khosravi MJ, Diamandis A, van Doorn J, Juul A. Yu H, et al. J Clin Lab Anal. 1999;13(4):166-72. doi: 10.1002/(SICI)1098-2825(1999)13:4<166::AID-JCLA5>3.0.CO;2-X. J Clin Lab Anal. 1999. PMID: 10414596 Free PMC article.
There was little difference in IGF-II levels between genders. IGFBP-2 levels declined with age from birth to puberty. ...Freeze-thaw treatment up to five cycles had little impact on plasma levels of IGFs and IGFBP-3. Our observations suggest that le
There was little difference in IGF-II levels between genders. IGFBP-2 levels declined with age from birth to puberty. ...Freez …
Total absence of functional acid labile subunit, resulting in severe insulin-like growth factor deficiency and moderate growth failure.
Hwa V, Haeusler G, Pratt KL, Little BM, Frisch H, Koller D, Rosenfeld RG. Hwa V, et al. J Clin Endocrinol Metab. 2006 May;91(5):1826-31. doi: 10.1210/jc.2005-2842. Epub 2006 Feb 28. J Clin Endocrinol Metab. 2006. PMID: 16507628
OBJECTIVE: We investigated a case of IGFD in which serum IGF-I and IGFBP-3 were abnormally low, yet growth failure was modest (-2.1 sd score at 15.5 yr of age). RESULTS: The young male subject, from a consanguineous pedigree, had a postnatal growth profile consisten …
OBJECTIVE: We investigated a case of IGFD in which serum IGF-I and IGFBP-3 were abnormally low, yet growth failure was modest (-2.1 s …
Idiopathic short stature: will genetics influence the choice between GH and IGF-I therapy?
Savage MO, Camacho-Hübner C, David A, Metherell LA, Hwa V, Rosenfeld RG, Clark AJ. Savage MO, et al. Eur J Endocrinol. 2007 Aug;157 Suppl 1:S33-7. doi: 10.1530/EJE-07-0292. Eur J Endocrinol. 2007. PMID: 17785695 Review.
CONCLUSIONS: Analysis for single-gene defects does not give sensitive predictions of phenotype and cannot predict responses to GH or IGF-I therapy. Endocrine abnormalities have closer correlations with phenotype and may thus be a better guide to therapeutic r …
CONCLUSIONS: Analysis for single-gene defects does not give sensitive predictions of phenotype and cannot predict responses to …
Whole-exome sequencing gives additional benefits compared to candidate gene sequencing in the molecular diagnosis of children with growth hormone or IGF-1 insensitivity.
Shapiro L, Chatterjee S, Ramadan DG, Davies KM, Savage MO, Metherell LA, Storr HL. Shapiro L, et al. Eur J Endocrinol. 2017 Dec;177(6):485-501. doi: 10.1530/EJE-17-0453. Epub 2017 Sep 4. Eur J Endocrinol. 2017. PMID: 28870985
IGF-1 insensitivity results in pre- and post-natal growth failure with normal/high IGF-1 levels. The prevalence of genetic defects is unknown. OBJECTIVE: To identify the underlying genetic diagnoses in a paediatric cohort with GH or IGF-1 insensitivity using candidate gene …
IGF-1 insensitivity results in pre- and post-natal growth failure with normal/high IGF-1 levels. The prevalence of genetic defects is …
The correlation of the IGF-I, IGFBP-3, and ALS generation test to height velocity after 6 months of recombinant growth hormone therapy in girls with Turner syndrome.
Collett-Solberg PF, Pessoa de Queiroz AN, Cardoso ME, Jusan RC, Vaisman M, Guimarães MM. Collett-Solberg PF, et al. Growth Horm IGF Res. 2006 Aug;16(4):240-6. doi: 10.1016/j.ghir.2006.06.002. Growth Horm IGF Res. 2006. PMID: 16908209 Clinical Trial.
Levels of IGF-I and IGFBP-3 were measured before and 5, 30 and 90 days after starting hGH (0.05 mg/kg/day), ALS levels were measured only prior and after 5 days. ...Most of the data was too scattered to be used individually for each patient. CONCLUSIONS: Even though
Levels of IGF-I and IGFBP-3 were measured before and 5, 30 and 90 days after starting hGH (0.05 mg/kg/day), ALS levels were me