C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions

We believe that VUS could safely replace VCUG in VUR detection in infants, with no regard to the degree of abnormality of the upper urinary tract seen on US. VCUG should be limited only to cases where bladder and/or urethra pathology are suspected.

Background The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient’s outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically. Results Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4.1%). Genetically confirmed diagnoses were reported in 24% of all pediatric and 12% adult patients, whereas glomerulopathies had been confirmed by kidney biopsy in 80.4% adult versus 38.5% pediatric glomerulopathy cases. Conclusions ERKReg is a rapidly growing source of epidemiological information and patient cohorts for clinical research, and an innovative tool to monitor management quality and patient outcomes.

Summary:Purpose: To investigate the risk of illnesses in a cohort of patients with epilepsy and in matched nonepilepsy controls, by type and complications.Methods: A total of 951 children and adults with idiopathic, cryptogenic, or remote symptomatic epilepsy and 904 matched controls seen in secondary and tertiary centers in eight European countries (England, Estonia, Germany, Italy, the Netherlands, Portugal, Russia, Slovenia) were followed prospectively for 17,484 and 17,206 person-months and asked to report any spontaneous complaint requiring medical attention (illness), its type and complications (hospitalization, absence from work or school, medical action). Risk assessment was done by actuarial methods, relative risks (RR), and 95% confidence intervals (CIs).Results: During the study period 644 patients (68%) and 504 controls (56%) reported an illness (p < 0.0001); 30% were seizure related. The cumulative probability of illness at 12 and 24 months was 49 and 86% in the cases and 39 and 75% in the controls (p < 0.0001). The largest differences regarded disorders affecting the nervous system (NS) (RR, 3.3; 95% CI, 2.3-4.2) and ear, nose, and throat (ENT) (RR, 1.3; 95% CI, 1.0-1.6). In patients with epilepsy, an NS illness was more likely to be followed by hospital admission, work absence, or medical intervention. All risks were significantly reduced after excluding seizure-related events.Conclusions: Patients with epilepsy are at higher risk of NS and ENT illnesses and complications than the general population. However, the risk of illness is significantly reduced when seizure-related events are excluded. Key Words: EpilepsyMorbidity-Illnesses.Patients with epilepsy have been thought to have a higher risk of illness than the general population, but comorbidity in epilepsy has been investigated in few population-based prevalence or analytic studies. Other clinical conditions and disabilities were reported by 47% of adults with epilepsy in Sweden, with psychiatric, cardiovascular, pulmonary, systemic, and renal diseases in decreasing order (1). Lifetime mental and gastric disorders were reported by 7 and 3% of adults with childhoodonset epilepsy in the Czech Republic (2). In Finland, somatic comorbidity was found in 84-95% of patients with childhood-onset epilepsy and 67-74% of controls (3). In a multicenter European cohort study of children and adults with idiopathic, cryptogenic, or remote symptomatic epilepsy, we found one or more illnesses at entry in 30% of cases compared with 17% of age-and sex-matched controls (4).These contrasting results may be explained by differences in the study populations and in the study design and methods. In addition, the morbidity risk attributable Accepted September 14, 2003. Address correspondence and reprint requests to Dr. E. Beghi at Istituto di Ricerche Farmacologiche "Mario Negri," Via Eritrea 62, 20157 Milano, Italy. E-mail: beghi@marionegri.it to the disease cannot be explored in studies on the prevalence of other clinical conditions in epilepsy. For these reasons, i...

Real‐time ultrasound‐guided renal biopsy (RB) with a biopsy gun has become a standard procedure in the treatment of children. The purpose of the study was to establish the complication rate after real‐time ultrasound‐guided RB with a biopsy gun, the adequacy of renal tissue samples for pathohistological tests, the rate of concurrence between clinical and pathohistological diagnoses, and the benefits of the procedure. From January 1994 to October 1999, 88 renal biopsies were performed on 82 children, 81 of whom (35M, 46F, aged 3‐20 y) were included in this retrospective study. The nephrotic syndrome (in infants, older children, those with evidence of nephritis or failing corticosteroid therapy) was the most frequent indication of RB. Other indications were non‐nephrotic proteinuria, nephritic syndrome, glomerular haematuria, renal allograft dysfunction, unexplained acute or chronic renal failure, and kidney disease progression monitoring. No serious complications were noted. The adequacy rate of renal tissue samples ranged from 93.1 to 96.6%, depending on which definition of the adequacy of renal tissue samples was used. Clinical and pathohistological diagnoses matched in 81.4% of the cases. Data obtained by RB were very beneficial to patients in terms of establishing, confirming or altering the diagnosis and, consequently, the treatment. Conclusion: The results confirm that real‐time ultrasound‐guided RB with a biopsy gun is a safe procedure and provides information that is very beneficial to patients.