-
[show abstract]
[hide abstract]
ABSTRACT: HPS is defined as arterial hypoxemia because of pulmonary vasodilation as a result of cirrhotic or non-cirrhotic portal hypertension. This report describes a teenager with HPS because of primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome requiring OLT. HPS resolved completely within three months of OLT, but recurred again at 12 months post-OLT following liver dysfunction secondary to a biliary stricture. She underwent a second OLT successfully and remains well two yr and three months post-second OLT. Recurrent HPS after OLT may occur because of graft dysfunction and as this novel case illustrates, retransplantation may lead to a successful outcome.
Pediatric Transplantation 01/2008; 11(8):927-30. · 1.48 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A 16-year-old boy with cystic fibrosis developed 'cepacia syndrome' 9 years after the first isolation of Burkholderia multivorans. It is important to recognise that 'cepacia syndrome' is not restricted to those infected with genomovar type III strains and that rapid, irreversible clinical decline can occur many years after the 1st isolation of Burkholderia cepacia complex (Bcc).
Journal of Cystic Fibrosis 07/2004; 3(2):133-4. · 3.19 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We report on an outbreak of colistin-resistant Pseudomonas aeruginosa (CRPA) that occurred in a United Kingdom pediatric cystic fibrosis (CF) unit and involved six children over a period of 5 years. All CRPA-positive children had received aerosolized colistin therapy before first isolation of resistant organisms (mean duration, 3.1 years). Four of the 6 had also received courses of intravenous colistin in the year before the first isolation of CRPA. No impact of CRPA acquisition on respiratory function, clinical condition, or radiological parameters could be demonstrated. Four of the 6 children carried isolates of CRPA indistinguishable on genotyping. Two of these 4 children were sisters. The other 2 were on the same ward together at time of first isolation, and subsequently shared overlapping admissions with one of the sisters. While there is no conclusive evidence for the route of transmission, the frequency of overlapping in-patient admissions between 3 of these patients is suggestive of patient-to-patient transfer in the nosocomial setting.CF clinicians should be aware that colistin resistance can occur in P. aeruginosa, and some of these strains are capable of spread within CF units.
Pediatric Pulmonology 11/2002; 34(4):257-61. · 2.53 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The measurement of FEV1 in children with cystic fibrosis has been shown to be the most important objective measurement for survival. It has been observed that children receiving intravenous antibiotics usually show a significant improvement in FEV1 with therapy in the short term. We hypothesized that the FEV1 measured pre-antibiotic therapy and followed longitudinally would show a greater rate of decline and may be a better prognostic indicator than the FEV1 post antibiotic therapy. The study cohort consisted of 60 children with cystic fibrosis who attended the St. James' Hospital cystic fibrosis unit between 1993 and 1999. Mixed model regression analysis provided estimates of the average rate of change of the pre-FEV1, post-FEV1 and FEV1 difference in subgroups based on survival, sex and pseudomonas status. There was no significant difference seen in the rate of decline of the FEV1 difference when comparing those who died and those who survived (p = 0.93). This was also the case when males were compared to females (p = 0.09). Both pre-antibiotic FEV1 and post-antibiotic FEV1 measurements showed a significant difference in rate of decline when comparing those who died (FEV1 slope = -6.4, -6.3) to those who survived (FEV1 slope = -1.9, -1.7) [p = 0.001, p = 0.0005] and when males (FEV1 slope = -0.6, -0.03) were compared to females (FEV1 slope = -3.3, -3.5) [p = 0.03, p = 0.002]. Our study demonstrated that there was no additional value in measuring FEV1 pre-antibiotic therapy compared to the FEV1 post antibiotic therapy in improving the sensitivity of FEV1 as a marker of decline. This study confirms that the rate of decline in FEV1 is a strong predictor of mortality and that females in this age group decline faster than their male counterparts.
Irish medical journal 100(7):529-32.
