The project: Cystic fibrosis (CF) is the most common, life-threatening autosomal recessive genetic disorder with an incidence of 1 in 2900 white newborns in the US. Although malnutrition is common and has been shown to be an unfavorable prognostic factor in CF, the relationships between malnutrition and clinical outcomes have not been quantified. Such longstanding gap of knowledge can be attributed to lack of prospectively defined, long-term followed patient cohorts, as well as epidemiologists with combined expertise in CF nutrition and biostatistics. The overall objective of this proposal is to use modern statistical and epidemiological methods to quantify the relationships between malnutrition and determinants of long-term clinical outcome, which we define as lung function, progressive respiratory infection and survival. With the unique opportunity to utilize two rich ongoing longitudinal databases collected since 1985, namely, the CF Foundation Patient Registry and the Wisconsin CF Neonatal Screening Study database, we are enthusiastic to test the hypothesis that malnutrition predisposes CF patients to increased risks of pulmonary deterioration, with potentially greater effect on female than male patients.
Specific aims are to develop: 1) baseline risk models, which predict outcomes based on nutritional status at the time of CF diagnosis, 2) short-term risk models, which predict outcomes based on nutritional deterioration in the preceding 2-4 years, and 3) long-term risk models, which utilize nutritional and pulmonary data from the entire patient history to predict survival. Accomplishing this project will generate important information regarding how nutrition affects pulmonary manifestations to impact CF survival. Such information is critical to developing new interventions to improve the life of patients with CF. The candidate: Dr. Lai is a postdoctoral fellow of Pediatrics and Biostatistics at UW-Madison. She has unique experiences combining basic nutrition research, clinical nutrition practice and biostatistics. This project will enable her to gain advanced training to enhance her career toward becoming an independent investigator with expertise in nutritional epidemiology of chronic diseases. The environment: This project will be performed in the Departments of Pediatrics and Biostatistics under the supervision of Dr. Philip Farrell, who is a well-recognized expert in CF research. The candidate will benefit from working with the multidisciplinary CF research team at the Madison CF center as well as from participation in the active and vigorous research community at UW-Madison.

National Institute of Health (NIH)
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Research Scientist Development Award - Research & Training (K01)
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Diabetes, Endocrinology and Metabolic Diseases B Subcommittee (DDK)
Program Officer
Hyde, James F
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University of Wisconsin Madison
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United States
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Lai, HuiChuan J; Shoff, Suzanne M (2008) Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance. Am J Clin Nutr 88:161-6
Shoff, Suzanne M; Ahn, Hong-Yup; Davis, Lisa et al. (2006) Temporal associations among energy intake, plasma linoleic acid, and growth improvement in response to treatment initiation after diagnosis of cystic fibrosis. Pediatrics 117:391-400
Farrell, Philip M; Lai, HuiChuan J; Li, Zhanhai et al. (2005) Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough! J Pediatr 147:S30-6
Lai, Huichuan J; Cheng, Yu; Farrell, Philip M (2005) The survival advantage of patients with cystic fibrosis diagnosed through neonatal screening: evidence from the United States Cystic Fibrosis Foundation registry data. J Pediatr 147:S57-63
Koscik, Rebecca L; Lai, HuiChuan J; Laxova, Anita et al. (2005) Preventing early, prolonged vitamin E deficiency: an opportunity for better cognitive outcomes via early diagnosis through neonatal screening. J Pediatr 147:S51-6
Koscik, Rebecca L; Douglas, Jeffrey A; Zaremba, Kathleen et al. (2005) Quality of life of children with cystic fibrosis. J Pediatr 147:S64-8
Lai, HuiChuan J; Cheng, Yu; Cho, Hyungjun et al. (2004) Association between initial disease presentation, lung disease outcomes, and survival in patients with cystic fibrosis. Am J Epidemiol 159:537-46
Zhang, Zhumin; Lai, HuiChuan J (2004) Comparison of the use of body mass index percentiles and percentage of ideal body weight to screen for malnutrition in children with cystic fibrosis. Am J Clin Nutr 80:982-91
Li, Zhanhai; Lai, HuiChuan J; Kosorok, Michael R et al. (2004) Longitudinal pulmonary status of cystic fibrosis children with meconium ileus. Pediatr Pulmonol 38:277-84
Lai, Hui-Chuan; Kosorok, Michael R; Laxova, Anita et al. (2002) Delayed diagnosis of US females with cystic fibrosis. Am J Epidemiol 156:165-73