Malignant Wegener's granulomatosis with fibrosing mediastinitis and vena cava superior syndrome.

A 47-year-old man was admitted to hospital for migratory joint pain, fatigue, and cough with bloody sputum and proteinuria with increased serum creatinine level. Diagnosis of Wegener's granulomatosis was established. During follow-up, the vena cava superior syndrome developed. The patient died of respiratory failure after 12 years of follow-up. The autopsy revealed rigid, whitish, 12 mm thick tissue, which embedded and compressed the large vessels upwards from their origin in the heart, thus causing vena cava superior syndrome. This tissue was composed of fibrous material without inflammatory cellulization. We consider this fibrous tissue as a manifestation of fibrosing mediastinitis that may or may not share pathogenesis with Wegener's granulomatosis.

Ambulatory blood pressure, proteinuria and uric acid in children with IgA nephropathy and their correlation with histopathological findings.

BACKGROUND/AIMS: In adults, nighttime hypertension and hyperuricemia are new risk factors for progression of IgA nephropathy (IgAN). In children, nighttime blood pressure (BP) and serum uric acid have never been investigated. The aim of the study was to investigate nighttime BP and uric acid in children with IgAN. METHODS: Data on children with IgAN from two pediatric nephrology centers were retrospectively reviewed (renal biopsy - subclasses according Hass I-V, ambulatory blood pressure monitoring ABPM, serum uric acid, proteinuria). RESULTS: Twenty-eight untreated children with IgAN were included. Hypertension was diagnosed on the basis of ABPM in 54% of children, 50% were nondippers and 25% have isolated nighttime hypertension. The mean ambulatory BP was higher at nighttime than during daytime (systolic nighttime BP 1.11 +/- 0.79 SDS vs. daytime 0.59 +/- 0.79, diastolic nighttime BP 1.16 +/- 0.95 vs. daytime 0.52 +/- 1.10, p < 0.01 for systolic and p = 0.01 for diastolic). Children with severe histological subclasses (III-IV) tended to have higher prevalence of hypertension than children with mild subclasses (I-II), 67% vs. 38%, p = 0.13. Hyperuricemia was diagnosed in 14% of children. A significant correlation was found between proteinuria and histopathological subclasses (r = 0.44, p < 0.05). CONCLUSION: Children with IgAN have often nighttime hypertension. Hypertension and proteinuria are associated with severe histopathological findings. Hyperuricemia is a rare finding in children.

Tubulointerstitial nephritis and uveitis syndrome in a mother and her son.

A mother and her son, both with tubulointerstitial nephritis and uveitis syndrome (TINU) are reported. The nephritis presented itself at 13 years in the mother and at 10 years in her son. Glomerular filtration (GFR) decreased in both, and renal biopsies confirmed the diagnosis. Nephritis preceded the onset of uveitis in both. Clinical course and renal function improved quickly on oral steroids in the boy. The mother's hyperazotemia decreased spontaneously (without steroids), but not to normal range, and remained stable for 35 years of follow-up. Local steroids due to recurrences of uveitis were repeatedly needed in both. We believe this is the first report on familial occurrence of inherited TINU syndrome in two generations.

Mild course of Puumala nephropathy in children in an area with sporadic occurrence Hantavirus infection.

The first three children with Puumala virus nephropathy diagnosis in the Czech Republic are reported on. A boy and two girls were admitted with symptoms of interstitial nephritis. The medical history in all children revealed flu-like symptoms. All patients were mildly pyrexial and had elevated erythrocytes sedimentation rate, C-reactive protein and low hemoglobin levels. Serum creatinine levels were elevated and proteinuria exceeded 700 mg/L in all children. Tubular proteinuria, glycosuria, high urinary N-acetyl-beta-D-glucosaminidase levels and alpha-1-microglobulin levels confirmed the tubular lesion. Renal biopsies revealed a uniform pattern and showed non-purulent interstitial nephritis in all patients. Puumala virus antigen antibodies were detected in the plasma. All patients were treated with steroids and urine abnormalities and renal function returned to normal within 4 weeks. Hantavirus infection should be considered as one of possible causes of interstitial nephritis with decreased GFR in children even in areas with a low incidence of this infection.

The Czech registry of renal biopsies. Occurrence of renal diseases in the years 1994-2000.

BACKGROUND: This report describes data collected by the Czech Registry of Renal Biopsies (CRRB). METHODS: Twenty-eight centres provided data on all biopsies of native kidneys performed in the Czech Republic (population 10.3 million) over the period 1994-2000. Data on serum creatinine concentration (sCr), 24 h proteinuria, haematuria, serum albumin level, arterial hypertension, diabetes mellitus, histological diagnosis and complications after renal biopsy were collected. RESULTS: Altogether 4004 biopsies in 3874 patients were performed (males 57.9%, children < or = 15 years 17.7%, elderly >60 years 14.3%). Microhaematuria was present in 65.9%, macrohaematuria in 9.2%, nephrotic proteinuria (> or = 3.5 g/24 h) in 39.3%, and low-grade proteinuria (<3.5 g/24 h) in 41.4%. Among adults, hypertension was present in 45.2%, mild renal insufficiency in 23% (sCr 111-200 micromol/l) and advanced renal insufficiency in 13.7% (sCr 201-400), while 11.5% of patients had sCr >400 micromol/l. The most frequent renal diseases were primary (59.8%) and secondary (25.4%) glomerulonephritis (GN). Tubulointerstitial nephritis (TIN) was observed in 4.4% and hypertensive nephroangiosclerosis in 3.4%. The samples were non-diagnostic in 4.6%. Among primary GNs, the most frequent diagnoses were: IgA nephropathy (IgAN) 34.5%, minimal change disease (MCD) 12.4%, non-IgA mesangioproliferative GN (MesGN) 11.3%, focal segmental glomerulosclerosis (FSGS) 10.8% and membranous GN (MGN) 9.3%. Among secondary GNs, systemic lupus erythematosus (SLE) represented 23.0%, necrotizing vasculitis (NV) 15.5%, Henoch-Schonlein purpura 5.7%, thin basement membrane glomerulopathy (TBN) 19.3%, Alport syndrome 6.9%, renal amyloidosis 9.9% and myeloma kidney 2.9%. Among children, the most common were IgAN (19.2%), MCD (17.6%) and TBM glomerulopathy (12.3%), while among the elderly the most common were MGN (11.0%), NV (10.7%) and amyloidosis (9.6%). The most common in patients with nephrotic proteinuria were MCD (50.5%) among children, but IgAN (24.6%) in adults aged 16-60 years and MGN (16.8%) among the elderly. IgAN (21.3%) and FSGS (8.3%) were the most common diagnoses among patients with mild renal insufficiency, but TIN (11.6%) and NV (11.3%) were the most common in more advanced renal insufficiency. Since 1999, diabetic patients represented 12.2% of adults, with mean proteinuria 8.9 g/24 h; diabetic glomerulosclerosis was found in 42.4% (with microhaematuria present in 66%) and non-diabetic renal diseases in 47.5% (IgAN in 17.5%, MGN and NAS in 11.1% and NV in 9.5%). The mean annual incidence (per million population) was: primary GN 32.4, secondary GN 13.8, IgAN 11.2, MCD 4.0, MesGN 3.7, FSGS 3.5, SLE 3.2, MGN 3.0, TBM 2.7, TIN 2.4 and NV 2.1. Ultrasound needle guidance was used in 56%, preferably in children (79%). The frequency of serious complications (gross haematuria, symptomatic haematoma, blood transfusion) remained at 3%. CONCLUSION: The CRRB provides important data on the epidemiology of GN based on a whole country population.