Prospective evaluation of a clinical practice guideline for diagnosis of appendicitis in children.

Objectives: The objective was to assess the performance of a clinical practice guideline for evaluation of possible appendicitis in children. The guideline incorporated risk stratification, staged imaging, and early surgical involvement in high-risk cases.

Methods: The authors prospectively evaluated the clinical guideline in one pediatric emergency department (ED) in a general teaching hospital.

Hereditary multiple exostoses with spine involvement in a 4-year-old boy.

Hereditary multiple exostosis (HME) is an autosomal dominant disorder characterized by multiple osteochondromas. We describe a case of acute cervical spinal cord compression arising from an exostosis at the lamina of C7 and T1 in a 4-year-old Mexican-American boy with HME. His affected sibling also displayed spinal cord compression because of a bony exostosis.

A follow-up study of MPS I patients treated with laronidase enzyme replacement therapy for 6 years.

Recombinant human alpha-L-iduronidase (Aldurazyme, laronidase) was approved as an enzyme replacement therapy for patients with the lysosomal storage disorder, mucopolysaccharidosis I (MPS I). In order to assess the long-term safety and efficacy of laronidase therapy, 5 of 10 patients in the original laronidase Phase 1/2 clinical trial were re-evaluated after 6 years of treatment. Lysosomal storage was further improved at 6 years (urinary glycosaminoglycans (GAG) excretion decreased 76%; mean liver size at 1.

Intramedullary abscess of the spinal cord in children: a case report and review of the literature.

A case report of an intramedullary spinal cord abscess in a 13-month-old boy and a review of relevant existing pediatric literature is presented. Thirty-eight cases of pediatric intramedullary spinal cord abscess are analyzed for presenting signs and symptoms, microbiology of isolated organisms, surgical intervention, antibiotic administration and outcome. The most significant variable on outcome is timely surgical intervention, followed by appropriate antibiotic administration.