Long-term parenteral nutrition in cystic fibrosis

O. Kirvela, R. C. Stern, J. Askanazi, C. F. Doershuk, Michael Rothkopf, D. P. Katz

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

The effects of parenteral nutrition (PN) with high lipid content were studied in 18 cystic fibrosis patients in this pilot investigation. The patients were randomly assigned to one of two groups. During the first 4-mo period, group 1 received PN and group 2 received routine therapy. During the second 4-mo period, PN was discontinued in group 1 and instituted in group 2. When the effect of PN was considered for both treatment groups, its general effect was to increase body fat content with little or no impact on respiratory function, exercise tolerance, or recurrent infections. However, subsequent analysis and clinical observation suggested that patients receiving PN responded in two seemingly distinct patterns: some demonstrated apparent clinical improvement and benefit, and others did not. A positive response in pulmonary and exercise function was closely correlated to a rise in serum dihomo-γ-linolenic acid (DHLA) concentrations during PN. Pulmonary function improved in patients who normalized their DHLA levels (vital capacity increased from 2.2 ± 0.3 to 2.6 ± 0.3 area%, p < 0.05), whereas those who continued to have undetectable levels of DHLA deteriorated (forced expiratory volume in 1 s decreased from 0.7 ± 0.1 to 0.6 ± 0.1, p < 0.001). PN applied to malnourished patients with cystic fibrosis results in beneficial effects in a subgroup characterized by the presence of DHLA in serum; for the group as a whole the positive effects are minimal.

Original languageEnglish (US)
Pages (from-to)119-126
Number of pages8
JournalNutrition
Volume9
Issue number2
StatePublished - Jan 1 1993
Externally publishedYes

Fingerprint

Parenteral Nutrition
Cystic Fibrosis
alpha-Linolenic Acid
Lung
Exercise Tolerance
Vital Capacity
Forced Expiratory Volume
Serum
Adipose Tissue
Observation
Exercise
Lipids
Therapeutics
Infection

All Science Journal Classification (ASJC) codes

  • Endocrinology, Diabetes and Metabolism
  • Nutrition and Dietetics

Cite this

Kirvela, O., Stern, R. C., Askanazi, J., Doershuk, C. F., Rothkopf, M., & Katz, D. P. (1993). Long-term parenteral nutrition in cystic fibrosis. Nutrition, 9(2), 119-126.
Kirvela, O. ; Stern, R. C. ; Askanazi, J. ; Doershuk, C. F. ; Rothkopf, Michael ; Katz, D. P. / Long-term parenteral nutrition in cystic fibrosis. In: Nutrition. 1993 ; Vol. 9, No. 2. pp. 119-126.
@article{adda465754724a849a23267f39136b03,
title = "Long-term parenteral nutrition in cystic fibrosis",
abstract = "The effects of parenteral nutrition (PN) with high lipid content were studied in 18 cystic fibrosis patients in this pilot investigation. The patients were randomly assigned to one of two groups. During the first 4-mo period, group 1 received PN and group 2 received routine therapy. During the second 4-mo period, PN was discontinued in group 1 and instituted in group 2. When the effect of PN was considered for both treatment groups, its general effect was to increase body fat content with little or no impact on respiratory function, exercise tolerance, or recurrent infections. However, subsequent analysis and clinical observation suggested that patients receiving PN responded in two seemingly distinct patterns: some demonstrated apparent clinical improvement and benefit, and others did not. A positive response in pulmonary and exercise function was closely correlated to a rise in serum dihomo-γ-linolenic acid (DHLA) concentrations during PN. Pulmonary function improved in patients who normalized their DHLA levels (vital capacity increased from 2.2 ± 0.3 to 2.6 ± 0.3 area{\%}, p < 0.05), whereas those who continued to have undetectable levels of DHLA deteriorated (forced expiratory volume in 1 s decreased from 0.7 ± 0.1 to 0.6 ± 0.1, p < 0.001). PN applied to malnourished patients with cystic fibrosis results in beneficial effects in a subgroup characterized by the presence of DHLA in serum; for the group as a whole the positive effects are minimal.",
author = "O. Kirvela and Stern, {R. C.} and J. Askanazi and Doershuk, {C. F.} and Michael Rothkopf and Katz, {D. P.}",
year = "1993",
month = "1",
day = "1",
language = "English (US)",
volume = "9",
pages = "119--126",
journal = "Nutrition",
issn = "0899-9007",
publisher = "Elsevier Inc.",
number = "2",

}

Kirvela, O, Stern, RC, Askanazi, J, Doershuk, CF, Rothkopf, M & Katz, DP 1993, 'Long-term parenteral nutrition in cystic fibrosis', Nutrition, vol. 9, no. 2, pp. 119-126.

Long-term parenteral nutrition in cystic fibrosis. / Kirvela, O.; Stern, R. C.; Askanazi, J.; Doershuk, C. F.; Rothkopf, Michael; Katz, D. P.

In: Nutrition, Vol. 9, No. 2, 01.01.1993, p. 119-126.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Long-term parenteral nutrition in cystic fibrosis

AU - Kirvela, O.

AU - Stern, R. C.

AU - Askanazi, J.

AU - Doershuk, C. F.

AU - Rothkopf, Michael

AU - Katz, D. P.

PY - 1993/1/1

Y1 - 1993/1/1

N2 - The effects of parenteral nutrition (PN) with high lipid content were studied in 18 cystic fibrosis patients in this pilot investigation. The patients were randomly assigned to one of two groups. During the first 4-mo period, group 1 received PN and group 2 received routine therapy. During the second 4-mo period, PN was discontinued in group 1 and instituted in group 2. When the effect of PN was considered for both treatment groups, its general effect was to increase body fat content with little or no impact on respiratory function, exercise tolerance, or recurrent infections. However, subsequent analysis and clinical observation suggested that patients receiving PN responded in two seemingly distinct patterns: some demonstrated apparent clinical improvement and benefit, and others did not. A positive response in pulmonary and exercise function was closely correlated to a rise in serum dihomo-γ-linolenic acid (DHLA) concentrations during PN. Pulmonary function improved in patients who normalized their DHLA levels (vital capacity increased from 2.2 ± 0.3 to 2.6 ± 0.3 area%, p < 0.05), whereas those who continued to have undetectable levels of DHLA deteriorated (forced expiratory volume in 1 s decreased from 0.7 ± 0.1 to 0.6 ± 0.1, p < 0.001). PN applied to malnourished patients with cystic fibrosis results in beneficial effects in a subgroup characterized by the presence of DHLA in serum; for the group as a whole the positive effects are minimal.

AB - The effects of parenteral nutrition (PN) with high lipid content were studied in 18 cystic fibrosis patients in this pilot investigation. The patients were randomly assigned to one of two groups. During the first 4-mo period, group 1 received PN and group 2 received routine therapy. During the second 4-mo period, PN was discontinued in group 1 and instituted in group 2. When the effect of PN was considered for both treatment groups, its general effect was to increase body fat content with little or no impact on respiratory function, exercise tolerance, or recurrent infections. However, subsequent analysis and clinical observation suggested that patients receiving PN responded in two seemingly distinct patterns: some demonstrated apparent clinical improvement and benefit, and others did not. A positive response in pulmonary and exercise function was closely correlated to a rise in serum dihomo-γ-linolenic acid (DHLA) concentrations during PN. Pulmonary function improved in patients who normalized their DHLA levels (vital capacity increased from 2.2 ± 0.3 to 2.6 ± 0.3 area%, p < 0.05), whereas those who continued to have undetectable levels of DHLA deteriorated (forced expiratory volume in 1 s decreased from 0.7 ± 0.1 to 0.6 ± 0.1, p < 0.001). PN applied to malnourished patients with cystic fibrosis results in beneficial effects in a subgroup characterized by the presence of DHLA in serum; for the group as a whole the positive effects are minimal.

UR - http://www.scopus.com/inward/record.url?scp=0027276822&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027276822&partnerID=8YFLogxK

M3 - Article

C2 - 8485323

AN - SCOPUS:0027276822

VL - 9

SP - 119

EP - 126

JO - Nutrition

JF - Nutrition

SN - 0899-9007

IS - 2

ER -

Kirvela O, Stern RC, Askanazi J, Doershuk CF, Rothkopf M, Katz DP. Long-term parenteral nutrition in cystic fibrosis. Nutrition. 1993 Jan 1;9(2):119-126.