ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension. Renal involvement was noted in all cases. Ten patients had extrarenal manifestations. Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases. A genetic study was not available for our patients. During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge. One patient underwent renal transplantation. None of our patients received eculizumab perfusion. The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02). Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01). Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes.
Subject(s)
Atypical Hemolytic Uremic Syndrome , Kidney Diseases , Adult , Atypical Hemolytic Uremic Syndrome/diagnosis , Atypical Hemolytic Uremic Syndrome/immunology , Atypical Hemolytic Uremic Syndrome/mortality , Atypical Hemolytic Uremic Syndrome/therapy , Biomarkers/blood , Complement Activation , Complement System Proteins/analysis , Disease Progression , Female , Humans , Kidney/immunology , Kidney/pathology , Kidney Diseases/diagnosis , Kidney Diseases/immunology , Kidney Diseases/mortality , Kidney Diseases/therapy , Male , Predictive Value of Tests , Prognosis , Risk Factors , Time Factors , Tunisia , Young AdultABSTRACT
THE AIM: of this study is to investigate the prevalence of diabetes-related autoantibodies in a group of children with celiac disease and to compare it with a control group. MATERIAL AND METHODS: We recruited 31 celiac children at diagnosis (on gluten containing diet) and 31 age and sex matched healthy children. Anti-islet cell antibodies (ICA) were detected by indirect immunofluorescence on monkey pancreas. Anti-glutamate decarboxylase (anti-GAD) and anti-tyrosine phosphatase (anti-IA2) antibodies were assessed by a radio-immuno- precipitation method. RESULTS: Three out of 31 celiac patients (9.7%) had one or more diabetes-related autoantibodies. ICA, anti-GAD and anti-IA2 were found in respectively 3.2%; 3.2% and 9.7% of patients. Only one control (3.2%) had anti-GAD. There was no statistically significant difference between the 2 groups. CONCLUSION: The risk to develop diabetes seems to be the same in celiac patients and in healthy subjects thus screening of diabetes-related autoantibodies is not justified in celiac patients.
