[HELLP syndrome and hemolytic uremic syndrome during pregnancy: two disease entities, same causation. Case report and literature review].

Thrombotic microangiopathies (TMA) are a group of diseases that can complicate pregnancy and threaten the lives of both the mother and the fetus. Several conditions can lead to TMA, including thrombotic thrombocytopenic purpura (TTP), HELLP syndrome and hemolytic uremic syndrome (HUS). We describe the case of a 39-year-old woman who presented a HELLP syndrome in the immediate postpartum period. The patient had acute kidney injury (AKI), increased LDH, unmeasurable haptoglobin levels and hypocomplementemia. Her ADAMTS13 value was normal, thus ruling out TTP. Shiga toxin tests were negative, so HUS associated with E. coli was also ruled out. HELLP syndrome and atypical hemolytic-uremic syndrome (aHUS) remained the most probable diagnosis. In the days following childbirth, the patient's transaminase and bilirubin levels normalized while the anemia persisted, as did the AKI, resulting in the institution of dialysis treatment. A diagnosis of aHUS was made and therapy with eculizumab was started. The patient's blood counts progressively improved, urine output was restored, her indices of renal function also concomitantly improved and dialysis was interrupted. A rash appeared after the third administration of eculizumab and the treatment was suspended. The patient is currently being followed up and has not relapsed. At thirteen months after delivery her renal function is normal as are her platelet counts, LDH, haptoglobin levels and proteinuria. Tests for mutations in the genes that regulate complement activity were negative. We believe that childbirth triggered the HELLP syndrome, which in turn brought about and sustained the HUS. In fact, the patient's liver function improved right after delivery, while her kidney injury and hemolysis persisted, and she also had an excellent response to eculizumab. To our knowledge, no other cases of HELLP syndrome associated with haemolytic uremic syndrome during pregnancy have been reported in literature, nor have cases in which treatment with eculizumab was limited to only three administrations.

[A rare case of acute renal failure related to amanita proxima ingestion]. / Nefropatia tubulo-interstiziale acuta da amanita proxima.

OBJECTIVE: The first cases of acute renal tubulopathy related to Amanita proxima poisoning were described in 1994; the mushroom contains a toxin responsible for the allenic-norleucine syndrome, characterised by a kidney damage that occur earlier than in Cortinarius orellanus poisoning and generally improve with complete resolution within ten days. CASE REPORT: A 45 years old woman was admitted to the hospital because of nausea, vomiting, and heartburn started the day before. Symptoms occurred eight hours after eating a single big wild mushroom; three friends who had the same meal were all asymptomatic. Twelve hours after the admission the woman became anuric and blood tests showed an impaired renal function: creatinine 13 mg/dL urea 240 mg/dL, AST 240 U / L, ALT 350 U / l. The patient was transferred to the nephrology unit of the Hospital Di Venere of Bari and the PCC of Milan was consulted. The toxicologist supposed a nephrotoxic syndrome caused by the consumption of Amanita proxima and sent a picture of the mushroom ingested to a mycologist who identified the specie as Amanita proxima Dumé, Bull (typical volva reddish-orange, ivory white hat, scaly stalk) sometimes mistaken for Amanita ovoidea. Haemodialysis was performed for five days followed by supportive care. Urine output gradually increased, serum creatinine decreased and Ast - Alt normalized. CONCLUSIONS: Amanita proxima contains an allenic-norleucine toxin (different from orellanine for the absence of inhibition of alkaline phosphatase), responsible for the reversible kidney damage, characterised by tubulointerstitial nephritis with acute tubular necrosis and renal failure. Occurrence and seriousness of symptoms seem to be variable and dependent on the amount ingested.

[Guiding the patient with end-stage renal disease in the choice of replacement therapy: role of the predialysis outpatient department]. / L'orientamento del paziente uremico nella scelta del trattamento sostitutivo: ruolo dell'ambulatorio di predialisi.

The predialysis outpatient department has three main objectives: ensuring patients' conscious and informed choice of dialysis treatment; clinical followup of patients with stage IV-V kidney disease; supporting patients and their families in their treatment choice. The aim of this work is to evaluate a) the effectiveness of the predialysis outpatient department in informing patients about the problems inherent in their disease; b) the differences in the choice of dialysis treatment between patients who benefited from the predialysis program and those who did not have that opportunity. Ninety-six patients were included in the study: 77 started renal replacement therapy after following a program at the predialysis outpatient department, while the remaining 19 patients started dialysis in an unplanned way. The patients monitored during predialysis were given a questionnaire to evaluate the usefulness of the followed program. The 77 patients who attended the predialysis outpatient department proved to be equally divided between hemodialysis and peritoneal dialysis: 43% vs 57%; the other 19 patients were directed mainly towards hemodialysis: 86% vs 14%, respectively. Analysis of the questionnaires showed that patients expressed a good degree of satisfaction. The results pointed to the usefulness of creating a predialysis outpatient department that should be able to a) optimize the predialytic treatments; b) plan the preparation of vascular or peritoneal access; c) guarantee patients a conscious choice of their replacement therapy.