Growth alterations in rare forms of primary adrenal insufficiency: a neglected issue in paediatric endocrinology

Area of Science:

• Pediatric Endocrinology
• Genetics
• Metabolic Disorders

Background:

• Primary adrenal insufficiency (PAI) is a condition of direct adrenal failure.
• In children, congenital adrenal hyperplasia (CAH) is the most common cause of PAI.
• Rarer forms of PAI include autoimmune polyglandular syndrome, adrenoleukodystrophy, adrenal hypoplasia congenita, familial glucocorticoid deficiency, and Allgrove's Syndrome.

Purpose of the Study:

• To review current evidence on growth alterations in children with rare forms of PAI.
• To highlight the impact of comorbidities on growth in these patients.
• To address the lack of specific studies on growth rate in rare pediatric PAI.

Main Methods:

• Literature review of existing studies on pediatric PAI and growth.
• Focus on rare etiologies of PAI.
• Analysis of comorbidities affecting growth.

Main Results:

• Growth alterations are a significant concern in children with PAI.
• Both under- and over-treatment with glucocorticoids can impair growth.
• Data on growth in rare PAI forms is limited.

Conclusions:

• Growth abnormalities in rare pediatric PAI require further investigation.
• Understanding comorbidities is crucial for managing growth in these children.
• Specific studies on growth rates in rare PAI forms are needed.