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: J Pediatr 2002 Dec;141(6):804-10 Related Articles, Links

The impact of early cystic fibrosis diagnosis on pulmonary function in

children.

Wang SS, O'leary LA, Fitzsimmons SC, Khoury MJ.

Office of Genetics and Disease Prevention.

OBJECTIVE: To investigate the impact of early diagnosis on pulmonary

function in a large cohort of children with cystic fibrosis (CF).Study

design: CF cases identified from the CF Foundation National Patient

Registry and diagnosed between 1982 and 1990 were categorized as: early

asymptomatic diagnosis (EAD; n = 157), early symptomatic diagnosis (ESD;

n = 227), later asymptomatic diagnosis (LAD; n = 161), and later

symptomatic diagnosis (LSD; n = 3080). Early CF diagnosis was diagnosis

before 6 weeks of age; later diagnosis was diagnosis at 6 weeks to 36

months of age, inclusive. Asymptomatic diagnosis included diagnosis by

either family history, genotype, prenatally, or neonatally. Pulmonary

function was measured as percentage of predicted forced expiratory volume

in one second (FEV(1)). RESULTS: There were no overall differences in

pulmonary function among the 4 diagnostic groups. However, EAD cases born

more recently (1987 or later) had a higher mean FEV(1) throughout the

study, compared with the remaining diagnostic groups. For this later

birth cohort, regression analysis for those diagnosed later and/or

symptomatically, demonstrated a 2-fold increase in risk (P =.06) for

having moderate-to-severe pulmonary function (FEV(1) <70%) at ages 6 to

10 years, compared with EAD cases. CONCLUSIONS: Children diagnosed with

CF early, asymptomatically and more recently may have better pulmonary

function throughout early childhood, probably as a result of improved CF

treatments in recent years.

PMID: 12461497 [PubMed - in process]

Am J Respir Crit Care Med 2002 Dec 12; [epub ahead of print] Related

Articles, Links

Significant microbiologic effect of inhaled tobramycin in young children

with cystic fibrosis.

Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A,

Hamblett N, Accurso F, Dovey M, Hiatt P, Konstan MW, Moss R,

Retsch-Bogart G, Wagener J, Ramsey B.

Pediatrics, Children's Hospital and Regional Medical Center/University of

Washington, Seattle, WA, USA.

We conducted a double-blind, placebo-controlled, multicenter, randomized

trial to test the hypothesis that 300 milligrams of tobramycin solution

for inhalation administered twice daily for 28 days would be safe and

result in a profound decrease in Pseudomonas aeruginosa density from the

lower airway of young children with cystic fibrosis. Ninety-eight

subjects were to be randomized, however the trial was stopped early due

to evidence of a significant microbiologic treatment effect. Twenty-one

children under age 6 were randomized (8 active; 13 placebo) and underwent

bronchoalveolar lavage at baseline and Day 28. There was a significant

difference between treatment groups in the reduction in P. aeruginosa

density; no P. aeruginosa was detected at Day 28 in 8 of 8 active group

patients compared to 1 of 13 placebo group patients. We observed no

differences between treatment groups for clinical indices, markers of

inflammation, or incidence of adverse events. No abnormalities in serum

creatinine or audiometry and no episodes of significant bronchospasm were

observed in association with active treatment. We conclude that 28 days

of tobramycin solution for inhalation 300 milligrams twice daily is safe

and effective for significant reduction of lower airway P. aeruginosa

density in young children with cystic fibrosis.

PMID: 12480612 [PubMed - as supplied by publisher]

Hepatology

Volume 36, Issue 6, December 2002, Pages 1374-1382

DOI: 10.1053/jhep.2002.37136

PII: S0270-9139(02)00317-8

Copyright © 2002 The American Association for the Study of Liver

Diseases. Published by Elsevier Science (USA). Original Articles

Liver disease in cystic fibrosis: A prospective study on incidence, risk

factors, and outcome

Carla Colombo 1, Pier Battezzati 2, Crosignani 2, Alberto

Morabito 3, Costantini 1, Rita Padoan 1 and maria Giunta 1

From the 1CF Center, Department of Pediatrics, University of Milan;

2Cattedra di Medicina Interna II, and 3Istituto di Biometria e Statistica

Medica, School of Medicine Ospedale San Paolo, University of Milan,

Milan, Italy.

Received 20 February 2002.  Available online 5 December 2002.

Abstract

Incidence of liver disease (LD) associated with cystic fibrosis (CF) and

its clinical characterization still is unsettled. We have assessed

prospectively the incidence and risk factors of this complication, and

its impact on the clinical course of CF. Between 1980 and 1990, we

enrolled 177 CF patients without LD in a systematic clinical, laboratory,

ultrasonography screening program of at least a 10-year duration. During

a 14-year median follow-up (2,432 patient-years), 48 patients developed

LD, with cirrhosis already present in 5. Incidence rate (number of cases

per 100 patient-years) was 1.8% (95% confidence interval: 1.3-2.4), with

sharp decline after the age of 10 years and higher risk in patients with

a history of meconium ileus (incidence rate ratio, 5.5; 2.7-11), male sex

(2.5; 1.3-4.9), or severe mutations (2.4; 1.2-4.8) at multivariate

analysis. Incidence of cirrhosis was 4.5% (2.3-7.8) during a median

period of 5 years from diagnosis of liver disease. Among the 17 cirrhotic

patients, 13 developed portal hypertension, 4 developed esophageal

varices, 1 developed liver decompensation requiring liver

transplantation. Development of LD did not condition different mortality

(death rate ratio, 0.4; 0.1-1.5) or higher incidence of other clinically

relevant outcomes. In conclusion, LD is a relatively frequent and early

complication of CF, whose detection should be focused at the first life

decade in patients with history of meconium ileus, male sex, or severe

genotype. Although LD does not condition a different clinical course of

CF, in some patients it may progress rapidly and require liver

transplantation. (H 2002;36:1374-1382.)

Becki

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