[Acute HCV-induced hepatitis in a patient affected by atypical hemolytic uremic syndrome (aHUS) treated with Eculizumab - case report].

Atypical hemolytic uremic syndrome (aHUS) is a rare and heterogenous disease caused by a disregulation of the alternative pathway of the complement cascade. Specifically, microvascular damage is produced that can lead to acute kidney disease, hemolytic anemia and thrombocytopenia. It accounts for 10% of all hemolytic uremic syndromes and can result in death or in end stage renal disease since the first episode. We can differentiate two forms of aHUS: a sporadic form (80%), affecting adult people, and a familial form (20%) that usually became manifest during infancy. In the acute phase of the disease, frequent and severe anemia requires multiple blood transfusions, exposing patients to the risk of catching an infective disease. HCV hepatitis is the most prevalent chronic hepatitis worldwide, with approximately 170 million chronically infected individuals - many of which are unaware of their condition. The evolution of the HCV infection is variable: almost 20% of patients spontaneously clear the infection over time (Anti HCV positive, HCV RNA negative patients); 80% of patients cannot control the virus and develop chronic infection (Anti HCV positive; HCV RNA positive patients) that can evolve into liver cirrhosis and hepatocellular carcinoma. The aim of this paper is to describe a clinical case of acute HCV hepatitis in a patient with aHUS treated with Eculizumab.

Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy.

Severe ADAMTS13 deficiency (activity < 10%) is pathognomonic of thrombotic thrombocytopenic purpura. ADAMTS13 testing is time-consuming and unavailable in many hospitals. Recently, a seven-variables score named PLASMIC score, has been developed to stratify acute patients, based on their risk of having a severe ADAMTS13 deficiency. We present the application of this score in a cohort of patients referred to our Center. From 2012 to 2017, 42 patients with suspected thrombotic microangiopathies from 6 Centers were referred to Hemostasis and Thrombosis Center of "Casa Sollievo della Sofferenza" Hospital/Research Institute for ADAMTS13 testing. For all patients, relevant medical and laboratory information were collected. To obtain the statistical measure of the discriminatory power of PLASMIC scoring system, the Area Under the Curve Receiver Operating Characteristic (AUC ROC) was calculated. We were able to calculate the PLASMIC score in 27 out of 42 patients; we found a good discrimination performance of the score with a resulting AUC value of 0.86 (95% CI 0.71-1.0; p = 0.015). All patients but one with a high risk PLASMIC score (6-7) showed a severe deficiency. Among patients belonging to the intermediate risk (PLASMIC score 5) group, 2 showed normal ADAMTS13 activity and 2 levels below 10%. In none of the patients in the low risk group (PLASMIC score 0-4), a severe ADAMTS13 deficiency was found. Present results confirm and extend previous data regarding the predictive value of the PLASMIC score. Indeed, it shows a good diagnostic performance and can be useful for decision makers to properly and promptly define the better therapeutic approach.

[Iodide mumps following low-osmolarity contrast medium use in haemodialysis patients]. / Iodide mumps dopo somministrazione di mezzo di contrasto a bassa osmolarità in pazienti sottoposti ad emodialisi.

Sialadenitis (Iodide Mumps) is a rare complication following the administration of iodinated contrast media. It is characterized by the painless bilateral enlargement of the submandibular salivary glands. The pathogenesis of this adverse reaction remains unclear. It may be due to an idiosyncratic reaction or related to toxic accumulation of iodide in the ductal systems of the salivary glands. Iodide mumps may occur after administration of any iodinated contrast agent: 98% of injected iodide is eliminated by the kidneys and only 2% is excreted from other organs such as salivary, sweat and lacrimal glands. Normally, the injected dose of contrast medium does not provide a sufficiently high iodide concentration to cause iodide mumps, but impaired renal excretion of contrast medium results in liberation of iodide and enables the salivary glands to concentrate inorganic iodide. This condition is rare and self-limiting. The current management is supportive therapy using analgesics and dialysis. We report two cases of iodide mumps in end-stage renal disease (ESRD) patients treated with haemodialysis after administration of iodinated contrast media. The cases were characterized by rapid onset and resolution of the symptoms in the absence of specific treatment.