First experience of enzyme replacement therapy with idursulfase in Spanish patients with Hunter syndrome under 5 years of age: case observations from the Hunter Outcome Survey (HOS).

Hunter syndrome (mucopolysaccharidosis type II [MPS II], OMIM309900) is a rare X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulphatase, resulting in accumulation of glycosaminoglycans (GAGs), multisystem organ failure and early death. Enzyme replacement therapy (ERT) with idursulfase is commercially available since 2007. Early access programs were established since 2005.

[Diagnostic test in emergency departments for bacterial infections in infants younger than 12 months].

Objective: To evaluate clinical and analytic numeric data that may help the emergency departments to identify bacterial infections in infants.

Patients And Methods: A retrospective study of 430 infants with bacterial growth in cultures (culture from blood, 30; urine, 207; stools, 193, and/or cerebrospinal fluid, n 25) was performed. These patients were compared with a control group (n 430), randomly selected from patients aged less than 12 months with negative cultures who were hospitalized with suspected infection.

[Bronchiolitis: revision of 153 cases and comparative study of ribavirin treatment].

Objective: We try in this paper to improve the knowledge of the main epidemiological, clinic, evolutive and therapeutic data in cases of children suffering from bronchiolitis in our medium; we analyze particularly the possible usefulness of ribavirin as a causal treatment.

Material And Methods: Firstly we have accomplished backward studies about the children suffering from bronchiolitis, who has been hospitalized in our Unit from January 1987 to May 1994. Then we have carried out a comparative study of the evolution in both groups of patients with and without RBV treatment.