Update: epidemiology of Cystic Fibrosis

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Current Opinion in Pulmonary Medicine 2002; 8(6):552-553

Update: epidemiology of cystic fibrosis

S. Krimsky, MD; H. Worth , MD

The natural history of cystic fibrosis (CF) is an evolving phenomenon. As

such, it is continually being redefined as epidemiologic data is

collected and processed and as advances in treatment for patients with CF

improve the quality and quantity of life. The past year was no exception

and has produced exciting new insights regarding outcomes in CF. We

review the recent literature and focus our discussion on two specific

areas with regard to their impact on the natural history of the disease:

(1) low socioeconomic status and (2) wasting and cystic fibrosis related

diabetes.

Abbreviations

CF cystic fibrosisCFFPR Cystic Fibrosis Foundation Patient RegistryCFRD

cystic fibrosis related diabetesCFRDM cystic fibrosis related diabetes

mellitusEERCF European Epidemiologic Registry of Cystic FibrosisRR

relative riskSES socioeconomic status

Department of Medicine Division of Pulmonary and Critical Care Medicine,

Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA.

Correspondence to H. Worth , MD, Department of Medicine, Division

of Pulmonary and Critical Care Medicine, Dartmouth Hitchcock Medical

Center, One Medical Center Drive, Lebanon, New Hampshire 03756, USA;

e-mail: H.Worth.@...

Current Opinion in Pulmonary Medicine 2002; 8(6):552-553

Copyright © 2002 Lippincott & Wilkins

All rights reserved

/* */ Current Opinion in Pulmonary Medicine 2002; 8(6):542-546

Proper usage of pancreatic enzymes

ne Schibli, MD *; R. Durie, MD *†; D. Tullis, MD ‡

With the recognition of the close link between nutritional status and

pulmonary function in cystic fibrosis (CF), treatment and prevention of

malnutrition have become a major focus in the modern therapeutic approach

for patients with CF. Thereby, pancreatic enzyme replacement therapy

plays a central role. This article reviews key publications on important

aspects of pancreatic enzyme replacement therapy contained in the

literature over the last 12 months. New insights into the pathogenesis of

exocrine pancreatic disease, efficacy and dosing of pancreatic enzyme

preparations, occurrence of fibrosing colonopathy, enzyme replacement in

the context of enteral nutrition, and assessment of pancreatic function

are addressed.

Abbreviations

CF cystic fibrosisCFTR cystic fibrosis transmembrane conductance

regulatorPI pancreatic insufficiencyPPI proton-pump inhibitorPS

pancreatic sufficiencyVIP vasoactive intestinal peptide

*The Hospital for Sick Children, Division of Gastroenterology and

Nutrition, Toronto; †Professor of Pediatrics, University of Toronto, and

Head of CF Research Group, Research Institute, the Hospital for Sick

Children, Toronto; ‡Medical Director Adult Cystic Fibrosis Program,

Division of Respirology, St. 's Hospital, Toronto, and Program

Director Respiratory Medicine, University of Toronto, Toronto, Canada.

Correspondence to ne Schibli, The Hospital for Sick Children,

Division of Pediatric Gastroenterology and Nutrition, 555 University

Avenue Toronto, ON M5G 1X8, Canada; e-mail: susanne.schibli@...

Current Opinion in Pulmonary Medicine 2002; 8(6):542-546

Copyright © 2002 Lippincott & Wilkins

All rights reserved

The Pseudomonas aeruginosa genome How do we use it to develop strategies

for the treatment of patients with cystic fibrosis and Pseudomonas

infections?

A. L. Erwin, PhD; D. R. VanDevanter, PhD

In the 2 years since the complete sequence of Pseudomonas aeruginosa

strain PAO1 was published, at least 200 papers have been published

describing research that made use of the PAO1 genome sequence. Some of

this research included genome-wide studies of gene expression or the

effect of mutation on bacterial functions such as biofilm formation; this

type of global analysis would not have been possible without the

availability of the sequence. As a result of these and other, more

traditional, research studies, there is a wealth of new knowledge about

the physiology of this pathogen. This raises the possibility of new

strategies for the treatment of patients with P. aeruginosa infection,

either by novel antibiotics or by drugs targeting bacterial functions

essential for survival and virulence in the human host.

Abbreviations

CF cystic fibrosisLPS lipopolysaccharidePA P. aeruginosaQSC

quorum-sensing controlledRSCV rough small-colony variantTLR4 toll-like

receptor 4

Chiron Corporation, Seattle, Washington, USA.

Correspondence to Alice L. Erwin, PhD, Chiron Corporation, 201 Elliott

Ave W. Suite 150, Seattle, WA 98119, USA; e-mail: alice_erwin@...

Current Opinion in Pulmonary Medicine 2002; 8(6):547-551

Copyright © 2002 Lippincott & Wilkins

All rights reserved

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