How Do You Treat Adrenal Crisis?
Discussion
Septo-optic dysplasia (SOD) is a disorder of midline prosencephalic development early in gestation. It causes agenesis of the septum pellucidum and/or thinning or absence of the corpus callosum and pituitary hormone deficiencies. It has an incidence of 1:10,000 live births. Most cases appear to be due to a combination of genetic and environmental factors but there are rare familial cases which are most often autosomal recessive.
The phenotype is variable and diagnosis can occur at birth with more severe problems or later with milder ones. Patients must have at least 2 of 3 problems for diagnosis including uni- or bi-lateral optic nerve hypoplasia, midline brain defects (e.g. septum pellucidum absence or agenesis of the corpus callosum) and hypopituitarism (which is a hypothalamic hypopituitarism not a primary dysfunction of the gland itself). Patients have poor visual function, developmental delay, sleep problems, seizures and various endocrine deficiencies. The most common endocrinopathy is growth hormone deficiency, then thyroid stimulating hormone deficiency, then adrenocorticotropic hormone (ACTH) deficiency. The endocrinopathies do not have to be present at birth but can develop later. Patients with SOD are at risk for adrenal insufficiency (AI) as they obviously do not have the normal capacity to increase ACTH during stress.
The hypothamic-pituitary-adrenal axis is important for maintaining homeostasis in the metabolic and immune systems. Normally the hypoth...
Source: PediatricEducation.org - Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
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