Focus on adrenal and related causes of hypertension in childhood and adolescence: Rare or rarely recognized?

Abstract

High blood pressure (BP) is not restricted to adults; children and adolescents may also be affected, albeit less frequently. Aside from unfavorable environmental factors, such as obesity and sedentary life leading to early-onset essential hypertension (HT), several secondary causes must be investigated in the occasional hypertensive child/adolescent. Endocrine causes are relevant and multiple, related to the pituitary, thyroid, parathyroid, gonads, insulin, and others, but generally are associated with adrenal disease. This common scenario has several vital components, such as aldosterone, deoxycorticosterone (DOC), cortisol, or catecholamines, but there are also monogenic disorders involving the kidney tubule that cause inappropriate salt retention and HT that simulate adrenal disease. Finally, a blood vessel disease was recently described that may also participate in this vast spectrum of pediatric hypertensive disease. This review will shed some light on the diagnosis and management of conditions, focusing on the most prevalent adrenal (or adrenal-like) disturbances causing HT.

Keywords: Hypertension; adrenal; childhood.

Figure 1. Diagram of causes of pediatric hypertension, classified as low-renin (LRH) and vasoconstrictive hypertension.

Figure 2. Biosynthesis of adrenocortical steroids in the two hypertensive forms of congenital adrenal hyperplasia: deficiencies of 17-α-hydroxylase (Panel A) and 11-β-hydroxylase (Panel B).

Figure 3. Algorithm for genetic testing in patients with a diagnosis of PPGL (pheochromocytoma/paraganglioma syndrome).

Figure 4. Simplified illustration of the renal tubules, denoting areas (membrane receptors, channels and ions transport) where specific mutations affect sodium transport, promoting inappropriate sodium retention and subsequent hypertension. The percentage of reabsorbed sodium in the different nephron portions is also represented. See Item 5 (The kidney tubule as a key player) for a better comprehension.