Over the year 2011 we followed-up, at our Institution, a 14-year-old girl with multi-cystic kidney disease and chronic solute imbalance coupled with hypokalemia and hypomagnesaemia. The girl, over the first months after referral (i.e., by late February 2011), developed secondary hyperaldosteronism and mild hypertension, both likely related to her underlying disease and well documented by mildly high plasma aldosteron levels [upright (8,30 am): 35 ng/dL, supine (8,45 am): 21 ng/dL], (high) aldosteron/renin ratio [(ARR; > 66.3 ng/dL (after 30 minutes seated)] and blood pressure monitoring [BP: systolic 159, diastolic 99 mmHg]. By middle March 2011, she complained of throbbing headache and visual disturbances. A magnetic resonance imaging (MRI) study of the brain and full ophthalmologic examination, obtained at that time, revealed increased intracranial pressure with partial empty sella and optic disc swelling (grade II papilledema): the girl was diagnosed with idiopathic intracranial hypertension (IIH; or pseudotumor cerebri). There are only few cases reported in the adult medical literature on the relationships between primary hyperaldosteronism (PAL) and IIH [1, 2]. The pathogenic mechanism coupling aldosteron increase in plasma with increased cerebrospinal fluid (CSF) pressure, leading in turn to the development of pseudotumor cerebri (i.e., IIH), was tentatively elucidated in a study, which inferred an aldosterone-mediated disturbance in CSF formation and electrolyte composition . This study recalls also the past empirical evidence, clinically reported by Conn in the 50’, on the occurrence of headache and visual disturbances in the medical records of his patients with adrenal adenomas. Additional cases have been recorded in the paediatric medical literature, reporting children affected by hereditary tubular disorders and secondary hyperaldosteronism (SAL) who in turn developed IIH . A recent retrospective study  investigated the relationship between hyperaldosteronism and hypocalcaemia in paediatric patients with pseudotumor cerebri who had high plasma aldosteron levels (a well-known cause of hypocalcaemia). Of note, all these children manifested clinical symptoms/signs related to hypocalcaemia before the development of symptoms/signs of IIH . The present child, who had SAL and IIH, was initially put under acetazolamide, which is regarded as the gold standard therapy for IIH, developing progressive renal dysfunction, increasing electrolyte imbalance and fast progression of the natural history of her mild chronic kidney failure (as also reported in a single patient in a previous study ). She was therefore switched to spironolactone, an aldosterone receptor antagonist, which is known to mildly (if at all) affect renal function  and to calcium and magnesium replacement therapy with good IIH control and no side effects. Based on present findings and on literature review [3-5] we propose that symptoms/signs of IIH, in paediatric patients with SAL, could be controlled by spironolactone coupled with the correction of hypokalemia and hypomagnesaemia by electrolyte replacement therapy and as add-on therapy with the use also of calcium and magnesium-conserving diuretics [3,4]. When renal dysfunction in the context of IIH is severe, an alternative choice could be serial lumbar punctures so as to avoid the later development of chronic kidney failure.
|Titolo:||New insights on the relationship between pseudotumor cerebri and secondary hyperaldosteronism in children|
|Data di pubblicazione:||2012|
|Appare nelle tipologie:||1.1 Articolo in rivista|