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Intermittent hyperaldosteronism in a child due to an adrenal adenoma.

UNLABELLED: Aldosterone producing adenoma (APA) is a rare but potentially curable form of paediatric hypertension. We report a case of APA in a 9-year-old boy, suspected due to persistent hypokalaemia. Neither BP nor initial laboratory investigations disclosed the diagnosis and the presence of an APA was suggested by functional tests and radiological findings. Histologically, a cortical tumour was found associated with a marked medullary hyperplasia of both chromaffin and ganglion cells. CONCLUSION: This case reinforces the need for further investigations in patients with misleading clinical and laboratory data.

Adenoma↗

Medical management of primary hyperaldosteronism.

Most forms of primary aldosteronism are surgically correctable. However, when surgery is not appropriate, medical management is just as effective in correcting the pathophysiologic abnormalities due to aldosterone excess. A prerequisite for the rational medical management of primary aldosteronism is an understanding of the mechanisms that sustain hypertension. Primary aldosteronism can be associated with severe and resistant hypertension, and persistent hypervolemia is the primary reason for resistance to therapy. Patients with overriding comorbidities or strong preferences have been medically treated over the intermediate term of 5 to 7 years without evidence of escape or evidence of malignant transformation of adrenal adenomas.

Adenoma↗

Myopathy with hyperaldosteronism. An electron-microscopic study.

In the case reported, an overproduction of aldosterone was accompanied by paretic attacks, a decrease in the serum potassium level, and in muscle tone and the deep tendon reflexes. The decrease of serum potassium level was consistent, but moderate, being to just below the normal lower limit. Loading with carbohydrate produced an attack, forearm ischaemic exercise resulted in less lactic acid production than normally. In the limb-girdle muscles there was also evidence of slight but permanent weakness. The results of histological and ultrastructural examination of a muscle biopsy are reported. It seems probable that the disturbance in the mineralo-corticoid metabolism led periodically to a decrease in the serum potassium, while in some muscle fibres the glycogen content increased and the SR became dilated and partially destroyed. In the attack-free periods tubular aggregates were produced, supposedly through an attempt at regeneration of the SR. During attacks portions of the muscle fibres were destroyed irreversibly and in consequence moderate permanent weakness resulted. Some spheromembranous degradation products which were observed might be regarded as remnants of degenerated tubular aggregates.

Adult↗

[Surgical treatment in primary hyperaldosteronism].

From 1994 to 2000, 32 patients (16 men, 16 women; mean age 53 years) underwent laparoscopic adrenalectomy at Hôpital l'Antiquaille of Lyon. All but one had systemic arterial hypertension and hypokaliemia was noted in all patients. All patients had unilateral adrenalectomy, and only one required conversion to conventional laparotomy. There were no deaths and complications were rare.

Adrenalectomy↗