Sliding Aortoplasty for Severe Supravalvular Aortic Stenosis After the Lecompte Procedure.

This report describes the case of a 3-year-old boy with supravalvular aortic stenosis after an arterial switch operation in whom the stenosis was successfully repaired using an ascending sliding arch aortoplasty without using a patch. Because patches were avoided, growth of the surgical site is expected. Ascending sliding arch aortoplasty and longitudinal expansion of the pulmonary bifurcation are useful for relieving stenosis and preventing supravalvular aortic stenosis recurrence after an arterial switch operation.

Clinical assessment of upper airway and its complications in Hunter syndrome.

BACKGROUND: Hunter syndrome (HS) is an X-linked, recessive, lysosomal storage disease caused by a deficiency of the lysosomal enzyme, iduronate sulfatase (IDS). It is characterized by multisystem accumulations of glycosaminoglycans and upper airway obstruction is one of the major causes of death. While the current disease severity classifications for HS are mainly based on the degree of neurocognitive impairment, its association with the level of upper airway obstruction has not been assessed. METHODS: A retrospective chart review of HS patients who were followed at the Jikei University School of Medicine was performed. Association between the degree of airway obstruction and the currently used disease severity scores was evaluated. RESULTS: We identified eight HS patients and they were enrolled in the study. The Modified Mallampati classification (MMC) score, used to predict difficulties for oropharyngeal procedures, was significantly correlated with the HS severity. It was also correlated with the Apnea-Hypopnea Index (AHI). No significant correlation between IDS enzymatic activity and the severity of HS disease was identified. CONCLUSIONS: Variable clinical expressivities exist in HS, but the risk of respiratory complications is likely to be associated with disease severity, assessed by the previously recognized neurocognitive function-based severity scoring systems. MMC can be a simple supplementary tool to evaluate disease severity as well as predict difficulties for oropharyngeal procedures and respiratory function complications in HS, such as sleep apnea.

Incidence and risk factors for mortality in paediatric severe sepsis: results from the national paediatric intensive care registry in Japan.

PURPOSE: To assess the incidence, background, outcome and risk factors for death of severe sepsis in Japanese paediatric intensive care units (PICUs). METHODS: A data analysis of a prospective, multicentre, 3-year case registry from nine medical-surgical Japanese PICUs. Children with severe sepsis, aged 0-15 years, who were consecutively admitted to the participating PICUs from 1 January 2007 to 31 December 2009 were enrolled. The incidence, background, causative pathogens or infective foci, outcome and risk factors for death caused by severe sepsis were analysed. RESULTS: One hundred forty-one cases were registered. After the exclusion of 14 patients because of incomplete data or inappropriate entry, 127 patients were eligible for the analysis. There were 60 boys and 67 girls, aged 23 [5-68] (median [IQR]) months and weighed 10 [5.5-16.5] kg. The incidence was 1.4 % of total PICU admissions. Sepsis was community-acquired in 35 %, PICU-acquired in 37 % and acquired in hospital general wards in 28 %. Methicillin-resistant Staphylococcus aureus was the most frequent pathogen. The crude 28-day mortality was 18.9 %, comparable to the mean PIM-2 predicted mortality (17.7 %). The mortality rate in patients with shock was significantly increased to 28 % compared to those without shock (5 %). The presences of existing haematological disorders (OR 8.97, 95 % CI, 1.56-51.60) and shock (OR 5.35, 1.04-27.44) were significant factors associated with mortality by multivariate analysis. CONCLUSIONS: The mortality from severe sepsis/septic shock in Japanese PICUs was ~19 %. Haematological disorders and presence of shock were associated with death.