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Giacomo Garibotto,Pasquale Esposito,Daniela Picciotto,Daniela Verzola 대한신장학회 2019 Kidney Research and Clinical Practice Vol.38 No.4
Both myostatin (MSTN) and activin A, two peptide members of the transforming growth factor (TGF)-β super family, have been suggested to play major roles in complications of chronic kidney disease (CKD), including vascular and bone disease. Both MSTN and activin A share many similarities in terms of structure, signaling pathway, and functions with TGF-β, and have been initially studied as players in muscle cachexia in CKD and several other chronic diseases [1]. However, the last few years have witnessed a paradigm shift with respect to our understanding of the effects of MSTN/activin signaling in organs distant from muscle. There is ever-increasing evidence that the MSTN/activin pathway impacts the heart, arterial vessels, insulin sensitivity and vascular remodeling [2]. In addition, recent observations strongly suggest that activin A signaling plays a major role in the progression of kidney disease and CKD/mineral bone disorder (MBD) [2]. These “off target” actions of MSTN and activin A might contribute substantially to the pathophysiology of wasting, inflammation, vascular damage, and possibly progressive renal dysfunction in CKD
( Giovanni Di Nardo ),( Federica Viscogliosi ),( Francesco Esposito ),( Vincenzo Stanghellini ),( Maria Pia Villa ),( Pasquale Parisi ),( Alessia Morlando ),( Girolamo Cal ),( Roberto De Giorgio ) 대한소화기기능성질환·운동학회(구 대한소화관운동학회) 2019 Journal of Neurogastroenterology and Motility (JNM Vol.25 No.4
Pediatric chronic intestinal pseudo-obstruction is a rare disorder characterized by a severe impairment of gastrointestinal motility leading to intestinal obstruction symptoms in the absence of mechanical causes. The diagnosis is usually clinical and diagnostic work is usually aimed to rule out mechanical obstruction and to identify any underlying diseases. Treatment is challenging and requires a multidisciplinary effort. In this manuscript we describe the youngest child successfully treated with the orally administrable, longacting, reversible anti-cholinesterase drug, pyridostigmine. Like other drugs belonging to cholinesterase inhibitors, pyridostigmine enhances gut motility by increasing acetylcholine availability in the enteric nervous system and neuro-muscular junctions. Based on the direct evidence from the reported case, we reviewed the current literature on the use of pyridostigmine in severe pediatric dysmotility focusing on intestinal pseudo-obstruction. The overall data emerged from the few published studies suggest that pyridostigmine is an effective and usually well tolerated therapeutic options for patients with intestinal pseudo-obstruction. More specifically, the main results obtained by pyridostigmine included marked reduction of abdominal distension, reduced need of parenteral nutrition, and improvement of oral feeding. The present case and review on pyridostigmine pave the way for eagerly awaited future randomized controlled studies testing the efficacy of cholinesterase inhibitors in pediatric severe gut dysmotility. (J Neurogastroenterol Motil 2019;25:508-514)