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Related Experiment Video

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A Novel Method: Super-selective Adrenal Venous Sampling

A Novel Method: Super-selective Adrenal Venous Sampling

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Adrenal function in Smith-Lemli-Opitz syndrome.

Simona E Bianconi¹, Sandra K Conley, Meg F Keil

¹Program in Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health & Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.

American Journal of Medical Genetics. Part A

|October 13, 2011

Summary

This summary is machine-generated.

Smith-Lemli-Opitz syndrome (SLOS) patients show higher baseline ACTH levels, suggesting compensated adrenal insufficiency. However, they maintain adequate glucocorticoid responses, indicating stress steroid coverage may not be necessary for mild to moderate cases.

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Area of Science:

Endocrinology
Genetics
Metabolic Disorders

Background:

Smith-Lemli-Opitz syndrome (SLOS) is a genetic disorder caused by DHCR7 gene mutations, affecting cholesterol synthesis and leading to 7-dehydrocholesterol accumulation.
SLOS presents a wide clinical spectrum, from severe malformations to milder intellectual disability.
Adrenal insufficiency has been noted in some children with SLOS.

Purpose of the Study:

To investigate adrenal function in SLOS patients using ovine corticotropin (oCRH) and adrenocorticotropin (ACTH) stimulation tests.
To determine if SLOS patients require stress steroid coverage.

Main Methods:

Ovine corticotropin (oCRH) testing was performed on 35 SLOS patients and 16 controls.
Prior ACTH stimulation tests were reviewed for 19 SLOS patients, and 10 additional SLOS patients' tests were reviewed.
Baseline and peak ACTH and cortisol levels, as well as the area under the curve (AUC), were analyzed.

Main Results:

SLOS patients exhibited significantly higher baseline ACTH levels compared to controls (P = 0.034).
No significant differences were found in peak ACTH, baseline/peak cortisol, or AUC for ACTH and cortisol between groups.
ACTH stimulation tests were normal in 28 out of 29 SLOS patients.

Conclusions:

Elevated baseline ACTH in SLOS patients may indicate compensated adrenocortical insufficiency.
SLOS patients demonstrate adequate glucocorticoid response to stimulation.
Stress steroid coverage may not be necessary for mild to moderate cases of SLOS.