Otitis and respiratory distress episodes following a respiratory syncytial virus infection.

OBJECTIVE: To document, over two consecutive respiratory syncytial virus (RSV) seasons, the occurrence of acute otitis media (AOM) and recurrence of respiratory distress in children < 2 years of age hospitalized for respiratory distress. METHODS: Patients were examined during hospitalization and at 6 weeks and 6 months after discharge. RSV testing was performed on all patients, and hospitalized patients were evaluated daily for the occurrence of AOM. RESULTS: In total, 347 children were enrolled; 54.8% were RSV positive, and 45.2% were RSV negative. Children were most frequently diagnosed as having bronchiolitis (71.9%) or asthmatic bronchitis (17.9%); other diagnoses included pneumonia, laryngitis, and rhinitis. During hospitalization, AOM was diagnosed in 16.8% of RSV-positive versus 8.3% of RSV-negative children (P < 0.05). Six weeks after discharge, AOM was reported in 10.4% of RSV-positive as compared with 5.8% of RSV-negative patients. Six months later, AOM was reported in 2.9% of the RSV-positive and 7.6% of the RSV-negative patients. A second episode of acute respiratory distress, which either required (9) or did not require (35) hospitalization, occurred in 18.4% of the total population, with similar proportions of RSV-positive and RSV-negative children (17% versus 18.6%). CONCLUSION: We conclude that RSV appears to be an important contributing factor for the occurrence of AOM in young children hospitalized with respiratory distress. The occurrence of a second episode of acute respiratory distress did not appear to correlate with the previous RSV infection, but longer-term follow-up is required.

Early-onset severe neurological involvement and D409H homozygosity in Gaucher disease: outcome of enzyme replacement therapy.

Gaucher disease, in most cases, is the result of mutations in the beta-glucocerebrosidase gene. More than 150 such mutations have been identified so far. Mutation D409H is the second most frequent in Greek patients, accounting for 15.5% of all identified mutated alleles. D409H homozygosity has, so far, been associated with a unique type III subtype of Gaucher disease that is characterized by the presence of devastating valvular heart disease, oculomotor apraxia, and, sometimes, features normally associated with mucopolysaccharidoses or oligosaccharidoses. Common manifestations of Gaucher disease tend to be less evident or even absent. We report the first Greek patient bearing the D409H/D409H genotype with onset of the disease in the first months of life and a phenotype dominated by severe neurological involvement. Enzyme replacement therapy, while improving the hematological parameters and organomegaly, failed to improve or even arrest the neurological condition.

Splenic granulomatous lesions in immunocompetent pediatric patients with visceral leishmaniasis.

Visceral focal lesions are rarely encountered in patients with leishmaniasis despite the fact that splenomegaly, and to a lesser extent hepatomegaly, predominate among the clinical features of the disease. We present, using high resolution ultrasonography, the first 3 reported cases of focal splenic involvement in immunocompetent children with visceral leishmaniasis. All patients showed a prompt response to the conventional anti-leishmanial treatment administered. This favorable outcome indicates that localized splenic lesions should not be considered as a poor prognostic factor and that alteration of the standard anti-leishmanial regimens is not justified.