Portuguese Journal of Nephrology & Hypertension

NDT on the move: an editorial note by the Editor-in-Chief for Portuguese nephrologists

Atypical haemolytic-uraemic syndrome: reflecting over the old and new

Atypical haemolytic uraemic syndrome is a rare disease characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and predominant renal impairment in the absence of Shiga toxin-producing bacteria. For long time it has been difficult to distinguish it from other thrombotic microangiopathies, but in the last decade advances have been made in understanding the pathogenesis of atypical haemolytic uraemic syndrome as a disorder of alternative pathway of the complement system. Knowledge of mutations and polymorphisms in the genes encoding the complement regulatory proteins revealed clinical importance in the management of the patients, altering not only the transplantation perspective but also leading to the search for new drugs, something that will potentially change the poor prognosis of these patients. This article reviews the differential diagnosis of this thrombotic microangiopathy to reflect on current treatment options and discuss new therapies

Orthotopic liver transplantation in familial amyloidotic polyneuropathy is associated with long-term progression of renal disease

Orthotopic liver transplantation has become the treatment of choice for familial amyloidotic polyneuropathy. The aims of this study were to evaluate the renal complications post orthotopic liver transplantation in familial amyloidotic polyneuropathy and their impact. We retrospectively studied 185 recipients who underwent 217 orthotopic liver transplants. Mean age 3 6.8±9.5 years, 59% males, 14.3% with renal dysfunction pre orthotopic liver transplantation. Mean follow-up 3.6±3.7 years. Thirty-two patients died. Univariate and multivariate analysis were performed, and p<0.05 was considered significant. Acute kidney injury occurred in 57 patients and renal replacement therapy was needed in 16/57. In multivariate analysis, acute kidney injury was correlated with development of chronic kidney disease (p<0.001). Relating to development of chronic kidney disease, 23.5% had progress to stage 3, 6% to stage 4 and 5.1% to stage 5 d. According to Spearmen correlation, risk factors for chronic kidney disease development were age (p<0.001), renal dysfunction pre orthotopic liver transplantation (p<0.001) and acute kidney injury post orthotopic liver transplantation (p<0.001). Mortality was correlated with age (p<0.001), retransplantation need (p=0.004), renal dysfunction pre orthotopic liver transplantation (p<0.001), acute kidney injury post orthotopic liver transplantation (p=0.04), and chronic kidney disease stage 5 (p<0.001). Using binary regression, mortality was correlated with chronic kidney disease development (p=0.02). In conclusion, familial amyloidotic polyneuropathy patients are disposed to renal complications that have a negative impact on the survival of these patients

Partial intestinal obstruction: a rare complication of autosomal dominant polycystic kidney disease: Case report and review of the literature

Autosomal dominant polycystic kidney disease is a common disease with an estimated prevalence of 1 in 400-1000 people. It is a multisystem genetic disorder characterised by multiple bilateral kidney cysts, often accompanied by cysts in the liver and other organs. It is the cause of 5-7% of end-stage renal disease on chronic haemodialysis and can lead to various renal and extrarenal complications. We report an unusual complication of this condition that has rarely been described in the available literature - a case of partial intestinal obstruction in a 70-yearold male on chronic haemodialysis due to polycystic kidney disease, with multiple typical associated manifestations. The patient was hospitalised because of cyst haemorrhage and infection. He complained of constipation accompanied by abdominal distension and pain. Ultrasonography and computed tomography showed multiple cysts of large size and partial intestinal obstruction due to cyst compression of the bowel. This was relieved by unilateral right nephrectomy

Gastrointestinal presentation of disseminated histoplasmosiin a transplant recipient

Histoplasmosis is a clinically relevant fungal infection in immunosuppressed patients, in whom it may present as a serious disease. We describe the case of a renal transplant recipient who presented with malaise, dry cough, severe weight loss, abdominal pain and watery diarrhoea. The laboratory workup revealed anaemia and elevated C reactive protein. Chest X-ray showed bilateral reticulonodular infiltrates, and computed tomography scan of the chest and abdomen revealed diffuse thickening of the inter- and intralobular septs, suggestive of lymphangitic infiltration. The abdomen had multiple lymphadenopathies along the mesenteric vessels. Endoscopic evaluation disclosed disseminated ulcers throughout the entire gastrointestinal tract, and biopsies confirmed the diagnosis of histoplasmosis. Despite adequate treatment with liposomal amphotericin B, the patient had a fatal outcome. We describe this case because of the rarity of gastrointestinal presentation in the context of disseminated histoplasmosis, and we also speculate about the transmission through the allograft. It highlights the importance of considering this once geographically limited disease in challenging cases, even in non-endemic areas

Remission of protein-losing enteropathy after surgical repair of a giant umbilical hernia in a dialysis patient

We present a 57-year-old male chronic haemodialysis patient with a giant umbilical hernia. After two months on dialysis, incarceration of the hernia with partial intestinal obstruction occurred, and this led to severe hypoproteinaemia secondary to protein-losing enteropathy, caused by increased lymphatic pressure. These problems were resolved following surgical repair of the hernia. There are few such cases in the literature and none involving a haemodialysis patient