Handgrip Strength and Pulmonary Function in the Pediatric Cystic Fibrosis Population

2021 ◽  
Vol 121 (9) ◽  
pp. A24
Author(s):  
J. Matsunaga ◽  
B. Bice ◽  
M. Jackson ◽  
J. Wells ◽  
M. Timmerman ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Handgrip Strength ◽  
Cystic Fibrosis Population

scholarly journals Liver Transplantation for Cirrhosis in Cystic Fibrosis

2006 ◽  
Vol 20 (7) ◽  
pp. 475-478 ◽  
Author(s):  
T Lamireau ◽  
S Martin ◽  
M Lallier ◽  
JE Marcotte ◽  
F Alvarez
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Portal Hypertension ◽  
Pulmonary Function ◽  
Hepatic Failure ◽  
Hepatic Transplantation ◽  
Significant Deterioration ◽  
Postoperative Course ◽  
The Third ◽  
Cystic Fibrosis Population

BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.AIMS: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.PATIENTS: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.RESULTS: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.CONCLUSION: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.

scholarly journals Health Disparities among adults cared for at an urban cystic fibrosis program

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Emily DiMango ◽  
Kaitlyn Simpson ◽  
Elizabeth Menten ◽  
Claire Keating ◽  
Weijia Fan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
New York ◽  
New York City ◽  
Health Disparities ◽  
Pulmonary Function ◽  
Health Outcomes ◽  
York City ◽  
Median Income ◽  
Cross Sectional ◽  
Caucasian Patients

Abstract Background Evidence is conflicting regarding differential health outcomes in racial and ethnic minorities with cystic fibrosis (CF), a rare genetic disease affecting approximately 28,000 Americans. We performed a cross-sectional analysis of health outcomes in Black/Latinx patients compared with non-Hispanic Caucasian patients cared for in a CF center in New York City. Adult patients enrolled in the CF Foundation Patient Registry at the Columbia University Adult CF Program and seen at least once during 2019 were included. Health metrics were compared between Black/Latinx and non-Hispanic Caucasian patients. Results 262 patients were eligible. 39 patients (15%) identified as Black/Latinx or non-Hispanic Caucasian. Descriptive statistics are reported with mean (standard deviation). Current age was 35.9 (13.3) years for non-Hispanic Caucasian and 32.0 (9.3) years for Black/Latinx patients (p = 0.087). Age of diagnosis did not differ between groups; 9.56 (15.96) years versus 11.59 (15.8) years for non-Hispanic Caucasian versus Black/Latinx respectively (p = 0.464). Pulmonary function, measured as mean forced expiratory volume in one second (FEV1) was 70.6 (22.5) percent predicted in non-Hispanic Caucasian versus 59.50 (27.9) percent predicted in Black/Latinx patients (p = 0.010). Number of visits to the CF clinic were similar between groups. When controlled for age, gender, co-morbidities, median income, and insurance status, there was a continued association between minority status and lower FEV1. Conclusions Minorities with CF have significantly lower pulmonary function, the major marker of survival, than non-Hispanic Caucasians, even when controlled for a variety of demographic and socioeconomic factors that are known to affect health status in CF. Significant health disparities based on race and ethnicity exist at a single CF center in New York City, despite apparent similarities in access to guideline based care at an accredited CF Center. This data confirms the importance of design of culturally appropriate preventative and management strategies to better understand how to direct interventions to this vulnerable population with a rare disease.

Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis

2003 ◽  
Vol 142 (6) ◽  
pp. 624-630 ◽  
Author(s):  
Michael W. Konstan ◽  
Steven M. Butler ◽  
Mary Ellen B. Wohl ◽  
Marcia Stoddard ◽  
Robert Matousek ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Early Life

Diabetes Mellitus in Patients With Cystic Fibrosis: Effect on Survival

PEDIATRICS ◽  
1990 ◽  
Vol 86 (3) ◽  
pp. 374-377
Author(s):  
J. Reisman ◽  
M. Corey ◽  
G. Canny ◽  
H. Levison
Keyword(s):  
Diabetes Mellitus ◽  
Cystic Fibrosis ◽  
Survival Analysis ◽  
Nutritional Status ◽  
Pulmonary Function ◽  
Survival Data ◽  
Diabetes Diagnosis ◽  
Patients With Diabetes ◽  
Insulin Dependent ◽  
Sick Children

Patient data obtained from the cystic fibrosis clinic of the Hospital for Sick Children (Toronto, Canada) over the period 1977 to 1988 were analyzed to compare the diabetic and nondiabetic cystic fibrosis patients. The pulmonary function, nutritional status, and survival data for 713 patients who attended the clinic over the 11-year period are reported. Insulin-dependent diabetes was found to exist in 37 (5.2%) of 713 patients. The patient age at time of diabetes diagnosis ranged from 2 to 34 years, with a mean ± SD of 20.0 ± 7.4 years. Patients who died in both the diabetic and nondiabetic groups had worse pulmonary and nutritional status than the surviving patients, but there were no significant differences between the diabetic and nondiabetic groups in those who died or in those who remained alive. Survival analysis showed a similar prognosis in the diabetic and nondiabetic groups. It is concluded that cystic fibrosis patients with diabetes are, for their age, not different from patients without diabetes with respect to pulmonary function, nutritional status, and survival.

ΔF508 Genotype Does Not Predict Disease Severity in an Ethnically Diverse Cystic Fibrosis Population

PEDIATRICS ◽  
1994 ◽  
Vol 93 (1) ◽  
pp. 114-118
Author(s):  
Lucille A. Lester ◽  
Jerome Kraut ◽  
John Lloyd-Still ◽  
Theodore Karrison ◽  
Carol Mott ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Disease Severity ◽  
Ethnically Diverse ◽  
Clinical Parameter ◽  
Population Based ◽  
Age At Diagnosis ◽  
Chest Roentgenogram ◽  
Sweat Chloride ◽  
Cystic Fibrosis Population ◽  
Cftr Mutations

Objective. As part of a study to determine population-based frequencies of CFTR mutations in an ethnically diverse, midwestern cystic fibrosis (CF) population, clinical histories were studied in 119 CF patients. Methodology. We sought to examine the association between genotype as characterized by the ΔF508 and 11 other commonly occurring mutations and clinical parameters including age at diagnosis, clinical presentation, sweat chloride level, chest roentgenogram score, clinical scores, pulmonary function test results, percent weight for height, and presence of associated CF complications. Results. Age at diagnosis of CF was significantly associated with homozygosity for ΔF508 (mean age at diagnosis ± SE: 1.7 ± 0.3 years for ΔF508/ΔF508 vs 3.9 ± 0.9 years for ΔF508/other and other/other; P = .03). No other age-adjusted clinical parameter was significantly associated with ΔF508 or any other genotype. Conclusion. These data suggest that in this sample of CF patients, ΔF508 genotype is not predictive of disease severity. The lack of association between disease severity and genotype in this ethnically diverse sample may reflect the presence of more severe undetected mutations in our sample, or the effects of modifying genes at other, non-CF loci.

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