Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?

It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS).We collected annual lung function data between the ages of 8-15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2-15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic.

Mycoplasma pneumonia and pulmonary embolism in a child due to acquired prothrombotic factors.

We report on a child with Mycoplasma pneumonia, who developed an unexplained new oxygen requirement. He was found to have an ileo-femoral thrombosis and an acute pulmonary embolus, with positive anti-phospholipid antibodies and acquired activated protein C resistance. These are both acquired risk factors for venous thrombosis.

Factors effecting impact of Aspergillus fumigatus sensitization in cystic fibrosis.

The clinical impact of Aspergillus fumigatus (Af) sensitization in cystic fibrosis (CF) is controversial. We examined the effect of Af sensitization (Afs) on pulmonary function and growth using a retrospective cohort analysis over two 5-year study periods: 1996-2000 (19 Afs cases and 19 controls) and 2001-2005 (24 Afs cases and 23 controls). Sensitization was defined as Af specific radioallergosorbent test (RAST) >or= 17.

Diagnostic omission errors in acute paediatric practice: impact of a reminder system on decision-making.

Background: Diagnostic error is a significant problem in specialities characterised by diagnostic uncertainty such as primary care, emergency medicine and paediatrics. Despite wide-spread availability, computerised aids have not been shown to significantly improve diagnostic decision-making in a real world environment, mainly due to the need for prolonged system consultation. In this study performed in the clinical environment, we used a Web-based diagnostic reminder system that provided rapid advice with free text data entry to examine its impact on clinicians' decisions in an acute paediatric setting during assessments characterised by diagnostic uncertainty.

Voriconazole therapy in children with cystic fibrosis.

Background: There is increasing evidence for the efficacy of the antifungal voriconazole, particularly in immunosuppression. We describe our experience of using voriconazole in children with CF.

Methods: We performed a retrospective case note review of children with CF treated with voriconazole in a single centre over an 18 month period.

Is there still a gender gap in cystic fibrosis?

Objectives: Previous studies have shown that female patients with cystic fibrosis (CF) have a significantly poorer prognosis than male patients. Such studies investigating gender-related differences have generally combined data from several centers. The aim of this study was to determine whether with modern aggressive treatment of CF this is still true when care is standardized within a single center.