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1.

Short stature due to primary acid-labile subunit deficiency

Acid-labile subunit deficiency is characterized by severely reduced serum insulin-like growth factor I (IGF1; 147440) and IGF-binding protein-3 (IGFBP3; 146732) concentrations that are incongruent with an associated mild growth retardation (height, -2 to -3 SD before and during puberty). Pubertal delay in boys and insulin insensitivity are common findings (summary by Domene et al., 2011). [from OMIM]

MedGen UID:
859716
Concept ID:
C3900122
Laboratory or Test Result
2.

Reduced insulin like growth factor binding protein acid labile subunit concentration

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

MedGen UID:
1390100
Concept ID:
C4321359
Finding

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