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Terapia enzimatica sostitutiva nella malattia di Fabry: cosa abbiamo imparato dallo switch?
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Renal Denervation for Resistant Hypertension: Time to Improve Patient Selection. The Lesson From ADPKD
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Treatment with Agalsidase Alfa during Pregnancy in a Heterozygous Female with Fabry Disease
Full text linkSWITCH TO AGALSIDASE ALFA AFTER THE SHORTAGE OF AGALSIDASE BETA IN FABRY DISEASE: A SYSTEMATIC REVIEW WITH METANALYSIS OF THE LITERATURE
No full textTolvaptan vs. somatostatin in the treatment of ADPKD: A review of the literature
Full text link: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic disorder with an estimated prevalence between 1 : 1,000 and 1 : 2,500. Until a few decades ago, ADPKD was considered an untreatable disease, relentlessly progressing towards end-stage renal disease because of the lack of specific interventions. In the last decade, some aberrant molecular pathways involved in ADPKD development have been identified, and controlled clinical trials have been conducted to investigate the potential role of active drugs on these pathophysiological mechanisms such somatostatin and tolvaptan. Somatostatin analogues have been shown to be effective not only in ADPKD, but also in polycystic liver disease (PLD) with beneficial effect on cardiac function and a better cost/benefit profile; the only somatostatin analogue currently available for clinical use is octreotide long-acting release (octreotide-LAR), and it is approved only in Italy. On the contrary, tolvaptan is authorized worldwide and has received more attention in the last years, even if its clinical use is widely limited by aquaresis tolerability. The aim of this review is to investigate the advantages and drawbacks of somatostatin analogues and tolvaptan in the treatment of ADPKD
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