The Clinical Features and Outcomes of Renal Amyloidosis in Tunisia.

Systemic amyloidosis is a rare disease characterized by clinical polymorphism. Indeed, the kidney is the most common organ involved and represents a real turning point in the disease. We aimed to determine the clinicopathological prognostic factors of renal amyloidosis (RA). We conducted a retrospective study including 40 cases with biopsy-proven RA collected in our department over a period of 10 years. Biochemical, demographic, and clinicopathological findings at diagnosis, as well as the follow-up data, were evaluated for each patient. The prevalence of amyloidosis was 2.7 per 100 nontransplant renal biopsies. The mean age at presentation was 55.5 ± 15.6 years with a male-to-female ratio of 1.85. The diagnosis of RA was confirmed by a renal biopsy in 85% of cases. Amyloid A (AA) amyloidosis was the most common type of amyloidosis (65%), and chronic infections ranked first in the panel of etiologies (41%). Amyloid light chain amyloidosis was mainly associated with multiple myeloma (57%). The median patient survival was 59 months versus 12 months for kidney survival. Age and extrarenal localization were independent predictors of mortality, whereas renal failure at presentation significantly influenced renal survival. The results of our study emphasize the rarity but also the severity of RA. AA amyloidosis was the most common type identified, which was mainly caused by chronic infections. Prevention remains the best solution until we can achieve therapeutic advances in inflammatory diseases.

Peritoneal dialysis in Tunisia: complications, technique and patient's survival (twenty-seven years of experience in a single center).

INTRODUCTION: peritoneal dialysis (PD) is a renal replacement therapy method that offers various advantages to end-stage renal disease patients. The aim of our study was to analyze patient characteristics, peritonitis and clinical outcome over a 27-year period of PD in our center. METHODS: retrospective study of incident patients on PD from January 1990 to December 2017. A total of 304 patients were enrolled in the study group. All patients over 15 years of age entering the dialysis program were included in the study. Patients dropping out from PD within three months were all excluded. Biochemical and demographic variables, peritonitis episodes and patient and technique survival were analyzed. RESULTS: the PD prevalence in our center was 4.5% during the study period; the mean age was 46.47 ± 18.6 years; diabetic nephropathy was the main cause of chronic kidney disease: 35.5% (n=108). Cardiovascular disease was the main cause of death: 39.6% (n=34). The peritonitis rate was 0.68 episode per patient-year. Ultrafiltration failure was the most important cause of PD withdrawal: 43% (n=60). Occurrence of peritonitis was the only independent predictor of technique failure: adjusted relative risk [aRR] 5.07, 95% CI 2.69-9.58; p<0.001. The overall non-adjusted patient survival was around 100%, 95% and less than 20% at 1, 4 and 25 years respectively basing on the Kaplan-Meier analysis. The group undergoing renal transplantation had the best survival rate. CONCLUSION: peritonitis remains the most common complication as well as the most provider of technique failure and patient´s transfer to hemodialysis. The technique survival was better in case of absence of peritonitis. However, our series showed the superiority of hemodialysis over PD in terms of overall patient survival.

Hemophagocytic Syndrome in a Pregnant Renal Transplant Recipient Associated With Cytomegalovirus Infection.

Hemophagocytic syndrome is a disorder of the mononuclear phagocytic system resulting in uncontrolled hemophagocytosis and cytokine overproduction. We report the first case of hemophagocytic syndrome, which occurred in a pregnantfemale patient 14 years after kidney transplant who displayed an atypical presentation and who had septic shock following cytomegalovirus infection. The patient, a-39-year-old woman at 27 weeks gestation with end-stage renal disease of unknown etiology, was admitted 14 years after living-donor kidney transplant (donor was her father) with high-grade fever, cough, and pancytopenia. Her immunosuppressant regimen included tacrolimus, azathioprine, and prednisone. Initially, she was hospitalized in the intensive care unit for septic shock without an identifiable focus of infection. She received intravenous broad-spectrum antibiotics before being transferred to our department following optimization of her hemodynamic status. Hemophagocytic syndrome was suspected, and bone marrow aspirate was performed, revealing macrophages with hemophagocytic activity. We confirmed the diagnosis of hemophagocytic syndrome given the presence of more than 5 criteria. We extensively investigated the underlying cause of hemophagocytic syndrome, and we diagnosed cytomegalovirus-induced hemophagocytic syndrome in a pregnant patient receiving immunosuppressive therapy after kidney transplantation. She was treated with corticosteroids and intravenous immunoglobulin. At 31 weeks gestation, she underwent a cesarean section; the baby developed newborn respiratory distress syndrome and died despite adequate resuscitation. We administered ganciclovir for 15 days following an increased cytomegalovirus viral load after delivery, leading to complete recovery.To date, optimal therapeutic and diagnostic guidelines for pregnancy-related hemophagocytic syndrome in female kidney transplant recipients are not well defined, and both patient and allograft survival rates remain low.

Ruptured intracranial aneurysm and recessive polycystic kidney Disease: A Rare Association.

Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney disease (ADPKD). However, there are only three reports about ICA in an adult patient with ARPKD. We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.

Brown tumors in dialyzed patients with secondary hyperparathyroidism: report of 16 cases.

Brown tumors (BTs) are relatively uncommon but they are serious complications of renal osteodystrophy. The objective of this study was to analyze the clinical, biological, and radiological characteristics of 16 patients with BTs provoked by secondary hyperparathyroidism (sHPT) and its response to the decrease in parathyroid hormone levels after parathyroidectomy (PTX). The management of that uncommon condition was also reviewed. We conducted a retrospective study including 16 end-stage renal disease patients who underwent subtotal PTX between 1997 and 2007 for severe sHPT with BTs. Our study included 10 men and 6 women, whose average age was 34 years. All patients were on dialysis. Ten of them were on dialysis for more than 5 years. The median duration on dialysis was 84 months. Patients included suffered from swellings associated with functional limitations. BTs had multiple locations in 7 patients. Jaw was the most frequent location (62%). Radiography and tomodensitometry demonstrated a mixed radio lucent and radio-opaque lesions with an expansion of the cortical bone. Bone scan demonstrated an increased uptake of lesions. Chirurgical treatment was indicated in all cases because of severe refractory sHPT with functional limitations and/or disfiguring deformities. In all cases, BTs stopped its progression and even decreased in size. However, it was insufficient in four cases, which required a surgical resection. PTX remains an efficacious approach in resistant cases of sHPT with persistent BTs.