Tubulointerstitial nephritis and uveitis syndrome with concomitant Hashimoto's thyroiditis.

The authors report the association of tubulointerstitial nephritis and uveitis (TINU) with Hashimoto's thyroiditis in a 16-year-old boy. Oral prednisolone was administered and the renal function normalized and has remained stable for 2 years. However, two recurrences of uveitis occurred in the 2-year follow-up period.

Tubulointerstitial nephritis and uveitis syndrome with concomitant Hashimoto's thyroiditis.

The authors report the association of tubulointerstitial nephritis and uveitis (TINU) with Hashimoto's thyroiditis in a 16-year-old boy. Oral prednisolone was administered and the renal function normalized and has remained stable for 2 years. However, two recurrences of uveitis occurred in the 2-year follow-up period.

Clinical impact of altered immunoglobulin levels in Henoch-Schönlein purpura.

BACKGROUND: The aim of the present study was the identification of immunological features, present at the time of diagnosis, that would predict the severity of Henoch-Schönlein purpura and its outcome. METHODS: A cohort study was carried out in a tertiary pediatric hospital of 69 children with Henoch-Schönlein purpura, in whom serum complement components C3, C4 and IgA, IgM, IgG were repeatedly determined. RESULTS: During the acute phase of the disease in 54/69 patients (78.3%) immunological imbalances were observed. In 24/54 cases (44.4%) certain complications involving the kidneys and the gastrointestinal tract were noted as opposed to in 3/15 children (20%) without immunologic abnormalities. In 50/69 children (72.5%), elevated serum IgA was detected and 16 of them (32%) developed renal involvement while only 1/19 children (5.3%) with normal IgA concentration had renal involvement. Considering separately the group of 9/69 children (13%) with increased IgM and those with normal IgM levels (53/69; 76.8%), irrespective of IgA and IgG concentration, we found a comparable percentage of children who had both renal and intestinal involvement without, however, developing severe complications, which were exclusively seen in patients with increased IgA (5/7 children) and reduced IgM levels. Serum C3 fraction was elevated in 26 children (37.7%) and in 73% of cases it was associated with increased serum IgA values. CONCLUSION: Renal involvement was seen in 32% of children with increased IgA values. Most importantly, elevated IgA concentration along with reduced IgM levels was associated with higher prevalence of severe complications.

Vesicoureteric reflux deterioration in monozygotic twins.

Primary vesicoureteric reflux (VUR) is the most common inherited structural urinary tract disorder, conforming closely to autosomal dominant transmission. A pair of monozygotic siblings is described exhibiting a remarkably parallel clinical course. VUR grade II was diagnosed in girl A during urinary tract infection at the age of 2 yr; screening of her asymptomatic sister (girl B) revealed the same. Renal cortical scintigraphy unveiled unilateral hypo-dysplasia in both the twins. Despite trimethoprime-sulfamethoxazole prophylaxis, infection recurred in girl A after 7 months, while girl B had a first episode 2 months later that prompted regimen switch to nitrofurantoin. Follow-up at the age of 4 depicted bilateral reflux deterioration; an urodynamics study that followed revealed functional bladder instability in both girls and the oral antispasmodic oxybutynin was initiated with good results. Evaluation for reflux should be prompt in infants with urinary infection. Patient siblings display a higher relative reflux risk, being highest in identical twins. Heredity issues, the impact of age and dysfunctional voiding in the clinical course, and the contribution of nuclear medicine in VUR management are discussed.

Provocation of neurocardiogenic syncope during head-up tilt testing in children: comparison between isoproterenol and nitroglycerin.

OBJECTIVE: Although nitroglycerin- and isoproterenol-augmented tilt tests are of equal value in the diagnosis of neurocardiogenic syncope in adults, no data exist in children. We compared the sensitivity and specificity of the 2 tests in a pediatric population. PATIENTS AND METHODS: We studied 85 patients (33 boys; mean age: 11.6 +/- 2.9 years). Of them, 56 had a diagnostic history of neurocardiogenic syncope, whereas 29 served as controls. After a negative passive phase, they were randomly assigned to either intravenous isoproterenol or sublingual nitroglycerin, and tilt was continued for 20 minutes. RESULTS: Sensitivity was 0.78 for the isoproterenol test and 0.79 for the nitroglycerin test, but specificity was significantly higher for isoproterenol test compared with nitroglycerin test. In patients with a positive test, the duration of the recovery period was significantly longer after nitroglycerin (8.4 +/- 2.7 minutes) than after isoproterenol (5.1 +/- 1.6 minutes). CONCLUSIONS: Nitroglycerin- and isoproterenol-augmented tilt tests are associated with equal sensitivity in the diagnosis of neurocardiogenic syncope in children and adolescents. However, nitroglycerin results in more false-positive tests and produces more prolonged vasovagal symptoms. Our data do not support the routine use of nitroglycerin in the evaluation of syncope in this age group.