[A case of encapsulating of peritoneal sclerosis with fast and ominous evolution]. / Un caso di sclerosi peritoneale incapsulante a rapida infausta evoluzione.

Encapsulating peritoneal sclerosis (EPS) is a rare and life-threatening complication of long-term peritoneal dialysis and until now, there is no established medical treatment for it. Many factors have been incriminated in its pathogenesis but they do not explain all risk conditions. EPS is significantly associated with PD duration, particularly more than 5 years, however the relationship between long-term PD and EPS increased risk is currently unknown. The "two-hit hypothesis" analyzes the relationship between the histological changes Peritoneal Dialysis-induced and intercurrent acute inflammatory episodes, but it does not explain the transition from Sclerosis Simple to EPS. We report our experience and we investigate the predisposing factors. The abdominal cocooning is a pathognomonic finding but it requires further investigations. Probably unidentified factors make some patients more susceptible to developing encapsulating peritoneal sclerosis.

Acute kidney injury in a patient with metabolic syndrome.

INTRODUCTION: The metabolic syndrome (MS) encompasses many metabolic abnormalities and the insulin resistance is considered as one of the most significant denominators. The chronic kidney disease (CKD) is an emerging health problem but only few patients would reach the end stage renal disease. There exists an increasing strong association between MS and CKD, but up until now the link between MS and CKD is unclear and there are few studies regarding the renal histology in MS. METHODS: We describe an acute tubulointerstitial nephritis case, due to both infective and pharmacological aetiology, overlapping relevant histological changes (focal segmental glomerulosclerosis [FSG], hyaline arteriosclerosis) in a patient with MS and previously normal renal function. Despite the severe vascular finding (elevated renal arterial resistive index), the patient recovered a normal renal function. RESULTS: We reviewed the kidney pathological studies in MS and analyzed the principal renal histological images of glomerulomegaly, segmental glomerulosclerosis, and obesity-related glomerulopathy. CONCLUSION: Despite the strong association, the renal involvement in MS has not been proven. A greater knowledge of the combination of histological renal changes in MS can help to understand the pathophysiological mechanism(s) of MS.

A case of acute EPS with local cocoon formation in a patient on peritoneal dialysis.

Encapsulating peritoneal sclerosis is a rare and life-threatening complication of long-term peritoneal dialysis and until now there is no established medical treatment. Many factors have been incriminated in its pathogenesis but they do not explain all risk conditions. We report our experience and we investigate the predisposing factors. Probably unidentified factors make some patients more susceptible to developing encapsulating peritoneal sclerosis.

[Renal involvement in Erdheim-Chester disease]. / Il coinvolgimento renale nella Malattia di Erdheim-Chester.

The Erdheim-Chester disease is a rare form of Langherans cells. Since 1987 it is distinguished from other istiocytosis previously identified. The diagnosis of the disease relies on defined radiological (bone imaging) and pathological (histiocytic infiltration) criteria. Bone disease is crucial but systemic manifestations are reported more frequently at onset. Renal involvement is always asymptomatic at onset of disease or in the follow-up. In this review we analyze the reports of the literature; we highlight 3 pathological mechanisms of renal involvement: renal and retroperitoneal infiltration, urinary tract obstruction, renal arteries stenosis. No treatment to date has demonstrated an improvement in survival of patients with EC. Renal involvement is therefore symptomatic (ureteral stenting, percutaneous nephrostomy) or is adopted a wait-and-see attitude.