Clinical features and outcome of patients with amatoxin-containing mushroom poisoning

Objective. We aimed to determine clinical and laboratory findings that were different between those patients who died and those who survived and to look for factors associated with the mortality in amatoxin-containing mushroom poisoning. Methods. The mushroom poisoning patients who were admitted to our clinic between 1996 and 2009 were retrospectively evaluated. The diagnosis was based on a history of mushroom ingestion, clinical picture and the presence of serum alpha-amanitin. Patients were divided into two groups as the survival group and the fatality group. Clinical and laboratory findings were compared between the two groups. Relation between variables and clinical outcome was analyzed. Results. A total of 144 amatoxin poisoning patients were included in this study. Patients who died were more likely to have demonstrated low mean arterial pressure, encephalopathy, mucosal hemorrhage, oliguria-anuria, hypoglycemia, and thrombocytopenia during the hospitalization. Low sodium values and high urea, AST, ALT, total bilirubin, LDH, prothrombin time, international normalized ratio, and activated partial thromboplastin time values were associated with increased likelihood of mortality. Nineteen patients developed acute renal failure. Fourteen patients developed acute hepatic failure. All the 14 patients who died developed acute hepatic failure. The mortality rate was 9.7%. Conclusions. The factors associated with mortality determined in this retrospective study may be helpful for clinical outcome assessment and monitoring of patients with amatoxin-containing mushroom poisoning

Canakinumab treatment in kidney transplant recipients with AA amyloidosis due to familial Mediterranean fever

Background: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent serosal inflammation with fever, which can result in amyloid deposition. Anti-interleukin-1 drugs emerge as a therapeutic option for colchicine-resistant patients. In this study, we aimed to document our experience with canakinumab use in kidney transplant recipients who developed AA amyloidosis due to FMF

A Fabry Disease Patient Who Developed Hypersensitivity Reaction against Agalsidase Beta following COVID-19 Infection

Fabry disease (FD) is a rare, X-linked inherited lysosomal storage disorder, characterized by the accumulation of globotriaosylceramide (Gb3) due to the deficiency or absence of alpha-galactosidase A. Due to the accumulation of Gb3, cardiac, renal, neurological, and skin manifestations can be observed. Enzyme replacement therapy (ERT) with agalsidase alfa or agalsidase beta is the cornerstone in the management of FD. Both enzymes are clinically effective and widely used. In this study, we present a 19-year-old male patient with FD who had received ERT for almost two and half years without any complications. In January 2021, he was diagnosed with COVID-19 infection. Later, he developed an infusion reaction during his first ERT infusion following the resolution of COVID-19 infection. The patient experienced shortness of breath, shivering, and rash. Despite decreased infusion rate and premedication in repetitive infusion, his symptoms were not resolved. Subsequently, he developed an IgE antibody against agalsidase beta, and his skin prick test was positive. Since IgG positivity against agalsidase beta was also detected, agalsidase beta was replaced with agalsidase alfa. The patient did not experience any allergic reaction with agalsidase alfa. Moderate to severe allergic reactions during ERT infusion should be alarming for IgE development. Furthermore, COVID-19 should be considered a trigger for allergic reaction against ERT in patients with FD

Demographic and Clinical Features and Factors Associated with Survival in Patients with Primary Glomerulonephritis: Single Tertiary Center Experience

Aim:Primary glomerulonephritis (GN) is a rare disease that has many different subtypes and is a significant health problem. Patients with primary GN (PGN) often do not achieve a complete cure, typically require immunosuppressive therapy, and can have serious co-morbidities due to the disease, which often progresses to end-stage renal disease (ESRD). The current study aimed to investigate the epidemiological, clinicodemographic characteristics and long-term outcomes of PGN patients.Materials and Methods:The current study retrospectively evaluated the demographic characteristics and complaints as well as the physical examination and laboratory findings of PGN patients who were followed-up and treated in the nephrology department of our university hospital between January 2000 and June 2016.Results:Of the 485 included patients, 265 were male (55%) and 220 were female (45%). The median age at diagnosis was 38.5 years (range; 18-77). The most frequent indication for biopsy was nephrotic syndrome (53.2%). The most common histopathological diagnoses were IgA nephritis (33.2%), focal segmental GN (31.1%), and membranous GN (19.6%), respectively. It was observed that male gender (p=0.01), systemic hypertension (p=0.01) at the time of diagnosis, proteinuria (p=0.001) in the nephrotic range, and histological diagnosis of crescentic GN (p=0.001) contributed negatively to renal survival. The mean follow-up duration after diagnosis was 59.1±48.5 months. The median overall survival was 153 (range; 1-197) months. Survival was significantly lower in patients with ESRD compared to those without ESRD (p=0.003). On clinical follow up, 48 patients died (9.9%), and 94 patients (19.3%) progressed to ESRD.Conclusion:Clearly defining the etiology of PGN as well as determining the factors leading to ESRD may decrease morbidity and mortality

Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report

Tacrolimus is a widely used macrolide immunosuppressant in transplant surgery, with mild and major neurologic side effects. A 21-year-old woman had undergone preemptive transplantation of a kidney from her mother. On the 1st postoperative day, the patient had headache, nausea, vomiting, and agitation. Magnetic resonance imaging (MRI) of the brain showed hyperintensity and a lesion in the right mesial temporal lobe. After we switched from tacrolimus to cyclosporine, the symptoms regressed. Persistence of the lesion, confirmed by repeated MRI, required that the patient be operated on. Pathologic examination showed the gangliocytoma, a rare brain tumor. Our case shows that preexisting brain lesions may cause tacrolimus-induced neurotoxicity in the early postoperative period

Evaluation of visual evoked potentials in chronic renal failure patients with different treatment modalities

Background: Uremia causes central nervous system (CNS) dysfunction. Modern diagnostic methods are useful for the recognition of CNS complications in uremic patients. Data concerning visual evoked potentials (VEPs) of chronic renal failure (CRF) patients is limited

Current Status of Renal Replacement Therapy in Turkey: A Summary of Turkish Society of Nephrology 2014 Annual Registry Report

OBJECTIVE: Turkish Society of Nephrology registry collects data on hemodialysis, peritoneal dialysis and transplantation on annual basis. Registry reports are printed every year as a booklet and this is the 24th year of registry reports. The registry is in close collaboration with international registries