Nutritional assessment a consensus report1’2 Bonnie

W Ramsey,

ABSTRACT vened

Philip

This

by the Cystic

among

nutrition

garding

optimal

fibrosis.

The

is a summary and

factors

care

provides

malnutrition

givers with

genetic

and

The second section of nutrition in these

conrecystic

a rationale

of this

for

disorder.

a negative

provides patients.

the

a consensus

ofpatients

report

and

of a meeting fibrosis

management causing

are outlined. assessment

cystic

of the

Pencharz,

to develop

management

section

The

Paul

Foundation

nutritional nutritional

balance routine

report

specialists first

multiple

M Farrell,

Fibrosis

emphasizing

and management

energy

guidelines for Five categories

ofnutritional status are defined based on ideal weight for height, age, and gender. These categories are used to formulate a graded response for nutritional intervention. Recommendations are provided and

for routine

pancreatic

report

dietary

enzyme

is to educate

assessments 108-16.

and

clinicians

early

vitamin The

as to the

supplements,

primary

aim

importance

intervention.

Am

of this

of frequent

J Clin

Nutr

l992;55:

Nutrition,

insufficiency,

cystic

consensus

fibrosis,

malnutrition,

pan-

report

umented

(1, 2). There

the nutritional which are still ported

status in long-term survival cystic fibrosis (CF) is well doc-

are multiple

interrelated

status of patients not fully understood.

the concept

that

genetic

as associated

with

factors

presence or absence ofpancreatic with P1 and steatorrhea (based as well

factors

this Recent

disorder, studies

have

have

on the

(P1) (2). fat-balance

a worse

affect

many of have sup-

an influence

insufficiency on a 3-d stool

undernutrition

that

Patients study)

prognosis

in

terms of growth, pulmonary function, and long-term survival than do pancreatic-sufficient patients (4). Although most patients with the most common genetic defect, the SF508 mutation, have ferences zygous severe

steatorrhea,

the present

data

indicate

that

there

between P1 patients who are homozygous for this defect. It appears that P1 is a marker genetic

defect.

Furthermore,

males have a worse prognosis than Maldigestion and/or malabsorption

among

patients

males. of fat (and

are no difor heterofor a more with

acids are frequently erbate maldigestion who have undergone

P1, fe-

fat-soluble

tamins) is only one of many variables affecting energy and nutrition in patients with CF. Losses of bile salts

108

struction

(because

of meconium

capabilities.

The

tional

is dependent

losses

patient’s

reduce

appetite

from ratory

coughing infections,

and

vi-

balance and bile

associated with steatorrhea and can exacand/or malabsorption. In addition, patients intestinal resection secondary to bowel ohAm

J Clin Nuir

l992;55:108-l6.

Downloaded from https://academic.oup.com/ajcn/article-abstract/55/1/108/4715278 by guest on 25 February 2018

ileus)

ability

have

reduced

to compensate

on their

quality offood consumed. excellent eating habits,

appetite

absorptive

for these

and

nutri-

the quantity

consumption,

and/or and

including

recurrent

gastroesophageal reflux, psychosocial stresses.

vomiting

chronic

respi-

is a clear association between malnutrition and lung function (5). Chronic pulmonary infections

ticularly

with

with

Pseudomonas

anorexia

but also

aeruginosa)

are associated

with

increased

metabolic

more

common

in adolescent

factors

adult patients Diabetes can

are diabetes mellitus and cholestatic increase caloric losses as a result

Nutritional

focal

deficiency

during of lung

nutritional of the

From Address

ranges

from

dietary

the Cystic reprint

must

Fibrosis requests

mildly

components,

Foundation, to BW Ramsey,

energy

and

young

the sesecretion.

depleted

fat

and protein malat times of rapid

and with increased such a broad impact

be vigilant

regarding

only

and

liver disease. of glucosuria.

exacerbate bile acid

symptoms of energy are most likely to occur

patients

different

I

in CF

clinicians

instructing

2

and

cirrhosis may ofinadequate

pulmonary exacerbations disease. Because CF has

status,

properly

biliary because

dete(par-

not

rate

requirements. Two other

disease with ofmalabsorption

and

Although some children with CF have there are several factors that commonly

There riorating

growth verity

The importance of nutritional well-being of patients with

Committee3

stores to frank signs nutrition. Deficiencies

Background

and

Consensus

Liver verity

KEY WORDS creatic

supplements,

replacement.

in cystic fibrosis:

in monitoring

the appropriate including

Bethesda, Cystic

seon and

ingestion total

calories,

MD. Fibrosis

Foundation,

693 1 Arlington Road, Bethesda, MD 20814. 3 Contributing members of the Consensus Committee were Robert Beall, Cystic Fibrosis Foundation, Bethesda, MD; Scott Davis, Tulane University, New Orleans; Peter Durie, Hospital for Sick Children, Toronto; Van S Hubbard, NIDDK/NIH, Bethesda, MD; Angela Ibrahim, Cystic Fibrosis Foundation, Bethesda, MD; J Nevin Isenberg, University ofTexas Medical Center, Austen; John Lloyd-Still, Children’s Memorial Hospital, Chicago; Elisabeth Luder, Mt Sinai School of Medicine, New York; Russell J Merritt, Carnation Company, Los Angeles; Suzanne Michel, Hahnemann University, Philadelphia; Elaine Mischler, University of Wisconsin, Madison; Donna Mueller, Drexel University, Philadelphia; John Patrick, University of Ottawa; Ron Sokol, University of Colorado, Denver, Virginia Stallings, Children’s Hospital of Philadelphia; Lori J Stark, Rhode Island Hospital, Providence; Robert Stone, Children’s Hospital & Medical Center, Akron, OH; and William Zipf, Children’s Hospital, Columbus, OH. ReceivedJuly

Accepted Printed

1, 1991.

for publication in USA.

July 3, 1991.

© 1992 American

Society

for Clinical

Nutrition

CYSTIC TABLE

FIBROSIS

CONSENSUS

109

REPORT

1

Nutritional-status

assessment Minimum frequency

Index Anthropometry Weight Height (children Head circumference

2 y old); length

(children

Every Every Every

2 y old)


2 mo. Nutritional assessment must include measurements (length), weight, head circumference (for those aged

age)standard in weight weight-

>

ofideal

5 y




given as Aquasol PA) or Liqui-E

first year

(chewed

up,

a-tocopheryl

or two.

squeezed

acetate

vitamin

E (>

1000

sociated

with

vitamin

Nutritional

regimen

research

out,

preparaAquasol

A,

PA) are available. follow

mo, 50 IU/d; 10 y, 200-400

these

guidelines:

1-4 y, 100 IU/d; 4IU/d. Vitamin E is

E (Rover Pharmaceuticals, (Twin Labs, Ronkonkoma,

After

the

first year

or swallowed)

may be used. Note IU/d) may exacerbate

or two,

Fort NY)

capsules

or dthat excessive doses of the coagulopathy asof a-tocopherol

K deficiency.

is needed

for vitamin

(eg,

ad-

adequate

a multivitamin

A preparations

E supplementation

currently Washington,

not maintaining

with

vitamin

Pharmaceuticals,

ages 0-6 mo, 10 y, 100-200

More

For patients

A concentrations

a

D and

5000 IU vitamin A in a dose of 1 tablet/d. Older children, olescents, and adults need a standard adult multiple-vitamin

the

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to these

lactation

of nutritional care, follow the guidelines

be taken

should

lactation

be taken

and

women

during

should

providing optimal fluwith CF are constantly counseling

adjust

and Nutrition Board, National Academy attention should be given to adequate

particularly

Multivitamins

for the

and MSW). CF patients

for the

intake,

important;

must

parents.

(eg, RN,

are

Food

(21).

preparation, pu-

bertal delay are common and come at a time of extreme social pressures and psychosocial stresses. Females are at greatest risk for nutritional failure because of their lower lean body mass.

Frequent

ofthe

of Sciences

standard

because high

by some

Committee

(Mead

developmental

requirements

development,

adults

experience

pregnancy

to the usual principles that pregnant women

multivitamin

nutrient

endocrine

increasing

students

to help

Widespread

that

gree of malabsorption

during

period when long-term feeding habits are developing. Schoolage (age 6-12 y). Children in this age group are exposed to significant degrees of peer pressure and are challenged to selfmanage their disease. Compliance with prescribed medications

understanding

lactation.

successfully

patient

team

this

such as pancreatic a major problem

pragmatic

in this population. Fat-soluble vitamins are absorbed fectively when taken in the morning with fat-containing

are important.

guidance

and

complished

adequate research

in this age group express individual

and

has demonstrated

CF and

Dietary intake and degree of physical activity to day. For these reasons, close monitoring of

habits,

Dietary

to the

AAP, Elk Grove Village, by the CF Center to Women,

formulas

of need. Toddlers to preschool (age have developed self-feeding food vary

might

according

ofPediatrics

to be introduced

predigested

Pregnancy

be able

should

American

infant

dose

be practical

changes.

to

interval.

foods

ofthe

it is prudent

pancreatic-enzyme-supplement

that

Complementary guidelines

CF centers,

for 1 2 mo and up to 24 mo. If an infant at a normal rate, a change to whole cow milk between 12 and 1 8 mo of age; however,

of the

adjustment

be needed

different

must

dition mended

of nutrients

oflife.

AL

years

especially

safe dose

El

to define

K in patients

the optimal with

CF.

supplementation Until

further

infor-

CYSTIC mation

is available,

it is prudent

to prescribe

FIBROSIS

vitamin

CONSENSUS

K supple-

baseline

ments

as follows: ages 0- 12 mo-2.5 mg/wk, or 2.5 mg twice weekly ifon antibiotics; ages > 1 y-5.0 mg twice weekly when on antibiotics or if cholestatic liver disease is present.

caloric

ments

guidance

It should

1-2

wk with

families

rapid

and

patients

growth)

or decreased

Special patients

attention during

proper

guidance

nutritional

The intake

and

booster and

food

needs

concept

intake

and

without For

can minimize

considers

the

increases

dramatically example,

is boosted

oral

person’s

usual

food

the total

nutrient

and

increasing

margarine

the amount

or butter

may

of

be added

to potatoes, vegetables, rice, and cereal. Instead oftomato soup made with water [1 .74 kJ/L(50 kcal/l20 mL)], it can be prepared with half-and-half(milk and cream) and garnished with grated cheese socially

and croutons acceptable,

and crackers, sandwiches,

[6.97 kJ/L(200 nutrient-dense

cold cuts, whole milk, peanut or pizza may be added as snack

soda or fruit juice. The third level ofnutritional

proprietary plements

and

Recipes

have

can

preferences centers recommend powders,

arc

may

and

homemade

or

provide

and

changes

oral

nourishment

in health

status,

must

life span intake

in children and

parent-child

mealtime

with

CF

may

taste

fatigue, the

assess

and

the

Families

and

discomfort. a CF

child

which

the

vision.

mealtimes

family

Parents

be encouraged sits together,

should

and these

child alike. Expansion gradually. Similarly,

establish

should

to have without clear

apply

regular

distractions rules

to their

and

mealtimes such

consequences

healthy

offood choice is important to increase energy consumption,

child and

consumption

child

self-

be instructed

alternatives

are

to

acceptable

strategies

in the

during

issues

physician

and

should

ad-

be placed

image

success

rather

than

of behavioral

pro-

not

successfully.

even

optimal

be able

to sustain needs.

failed

fail and

have

unable

to

It is important

adequate

or that

growth

been

feeding

Families

is

intervention behaviors

have

strategies

caloric

have

eating

the parents with

dimay

(eg, grandparents),

behavioral

problematic

when

that

high

mealtimes

intensive

when

above

they

nutritional the

visits. Emphasis enhancing body

factor

and/or

will very

adolescence, between

for more

to families

that

Supportive

intervention

When

a CF patient’s

90% of ideal,

is necessary.

some

nutrition

and

because

patients

we blame

to

patterns,

them

must when

never feeding

is impaired.

energy

and

identification

in

as telefor

and CF

best done the child’s

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density

initial

guidance. haviors

weight-for-height

or his weight

Treatment

and

should

of any

Emphasis

should

and adding

on increasing

the frequency

of enzyme

level

be placed

therapy,

stressors.

of nutritional

in the previous

section,

decline

If these

is

Anticipatory

on optimizing

oral supplements.

85%

reevaluation

psychological

for this

outlined

falls between

declines,

optimization

remediable

management

as that

focus

of meals,

ratio

velocity

feeding

measures

be-

are not

successful within 3 mo or if the patient’s weight-height ratio declines to < 85% ofideal, more aggressive nutritional management should be considered, as outlined below. The onset ofnutritional failure necessitates an immediate nutritional

and

medical

ofnutntional

under

strategies.

should

clarify

into

clinic and/or

patient

the

The

behaviors,

of time

CF

Referral

implement

the same

eating

amount

food

To promote should

such

refusals, program,

and formal contracting. In these cases, goals should be set that will gradually

be considered

include

factors

abdominal

in general,

of both parents in the program, including as well as significant-other adults who

identified

by many

These

whether the child consistently eats or whether the child dawdles, and how the parent handles the situation. Long mealtimes (> 20-30 mm), slow eating, and reports by parents that they must constantly nag the child to eat are indicative of problem areas that can be addressed through behavioralmanagement

for the

been

and to

throughout

be influenced

child

of eating

should

taught

factors.

interactions,

sequencing

marketed

of approaches

and

both

the

by consistent

problematic structured

limits.

parents

directly

is a key

necessary.

supplemen-

issues,

progresses

addressed

via discussion caloric-intake

children

with CF.

environmental

CF care providers spends at meals,

of nutrient

be managed

their

leave

New

and

a combination

be offered

of the person

psychosocial

the temporal

source

child

Inclusion households

care

feel

and development

members

for adequate

in which

is eating.

to children

time

foods,

choices

Consistency

some CF beverages,

an adjunctive

as a reward

reasonable

increase at successive on increasing strength on weight gain.

oftheir

manufactured

charts

be best

olescent reasonable

individual

convenience.

should

to

of calories,

and keeping

family

be applicable

of appropriate

As the

ingredients.

their

may

within

food

may

available

of

to eat

every meals

choices.

numerous

being

ofgrowth

as sticker

offer

readily

because

refusal

other

unless dinner is eaten. For need to implement a more

to satisfy

medical conditions. However, (and patients select) commercial

continuously

Because

Oral

from

formulated

puddings

and

products

entire

is adding

when

child

nagging

that

should support

be prepared

been

taste

tation.

and jelly instead of

oral supplements to the usual diet. Homemade supsuch as milk shakes are often more palatable, less cx-

pensive,

and

butter items

from

incre-

by complimenting

their

as no dessert parents may

grams. vorced

kcal/120 mL)J. In addition, foods such as nuts, cheese

be discouraged

and

increase

eating

when

small

be added

foods

the gradual

consequences

and eating

daily food and vi-

for

attention

beyond

A child’s

selection

level

table

and

should

to encourage

paying

should

kcal/d)

with

be taught

and

at the

such

below.

second

child meal.

Iwo areas of antici(22) and 2) behavioral

The then

(eg,

of females years. With

nutrition support is adequate pancreatic-enzyme-replacement

habits

consumed.

should child

be established

recommendations

In conjunction

Parents

infections).

and patients

are outlined

supplementation.

preferences

caloric

families

modification,

tamin-mineral The

caloric

(eg, pulmonary

during these periods. 1) oral supplementation

first level oforal with adequate

intake.

appetite

and training,

guidance,

assessment

of increased

and prepare

should be directed to the families the preadolescent and adolescent

decline

patory

for periods

to educate

should

( 100-200

J/d

specific

be targeted. their

be the goal of CF care givers

intake

of 4 1 8-836

parents Anticipatory

113

REPORT

failure

Evaluation

Enteral

When

the

by enterostomy

with

the

use

status

ofCF

is needed a controlled techniques,

patient.

The

assessment

nutritional

techniques

modification)

patients,

within

potential

(eg,

fails to ima 3-mo

with enteral feedings. clinical trial ofnutritional

the following

definition are outlined

failure.

of noninvasive

and behavioral

nutritional

further intervention there has not been

of the

an appropriate

of patients

feeds.

oral supplementation prove

evaluation and

period,

Although support benefits

have

114

RAMSEY

been

observed

status

in studies

of body

characteristics,

of pulmonary

during

thus

2)

increased

4 ) increased body image,

sense of well-being, weight, 5) improved sexual

performed

composition,

7) less

infections,

the terminal

phase

strength,

loss

more results ofclinical investigation on the use ofsupplemental tube feedings, guidelines Before should

are

ofan

enterostomy

the patient

of tube

acceptance family

feedings

and are

supplements. tem

status

published

have been the following

and

tube,

family

and

long-term

necessary

the

should

is needed

for glucose

must

be a routine

Parenteral

assessment

be educated be assessed.

possible

need

a successful

term

about Com-

support

du-

have

all been

used

ceptable

for short-term

patients

followed

trostomy

successfully. nutritional

in centers

tubes

can

choice

enteral

as well as under general anesthesia tubes carry the risk ofgastroesophageal iting. ofthe

Most patients feeding rate,

because

with GER appropriate

by a surgeon.

are ac-

motivated

Gas-

reflux

patients

(GER)

and vom-

some cases use of prokinetic agents (eg, domperidone). nostomy tubes potentially increase the problem ofnutrient absorption

but

centers.

have

Patients

quire

a pulmonary

terostomy

been

with

used

significant

tune-up

very

successfully

lung

disease

before

surgical

will

in

Jejumal-

choice

of a nutritional

supplement

there

is no compelling

for tube

to support

primwith Furany

par-

or growth that

issue,

it is generally

Initiation of enteral tube mately 1 wk of hospitalization

for advancing advancing

nutrient

is reached,

mum The

should

50-75%

rate tolerated

volume

by each

recommended

ment

of one-fourth intake.

This

to nasogastric tubes.

slowly

The and

and then

strength

tubes

as

principle

for

increasing

the

the volume.

formula

be increased

for the slowly

It is first

24

density

to a maxi-

patient.

nutritional

be individualized

that

and

supplementation

flexible,

as indicated. of additional

to one-third is a good

with

Generally, energy

the previously starting

point

regimen

adjustments

made

tube feedings with an incre-

consumed but

some

require

approach surgery.

long-term

risks.

are par-

parenteral

supplementation

Furthermore,

it has

the

gut

should

always

be used

and

growth

for

first

hormone.

and

is not

recommended.

complications promoting

growth

of growth agent

hormone

in CF might

hormone are

have

not

known.

negative

ofglucose intolerance and Until additional information

potential as an anThe

effects

on

diabetes melis available

from

carefully controlled trials, the use of growth hormone in is not recommended. Treatment ofthe CFpatient with diabetes meiitus. The patient with both CF and sufficient glucose intolerance to warrant directed therapy needs special attention. The CF care provider CF

consult the Report Diabetes Mellitus

of the Consensus (26).

Conference

on CF-

13

begin

requires approxievaluation, and

over 48 h. After full caloric may

to achieve nutritional goals involve nocturnal infusions caloric

first,

to full strength hourly

applies

starting

formula

with

usually education,

jejunostomy

involves

ofthe

to start

h and advance

This

and

feedings

density

advisable

feedings for

the feedings.

well as to gastrostomy

recommended

enzymes as the tube feedings do not require enzymes.

that

for energy

needs to be followed. appetite stimulants,

the increased incidence litus in CF is ofconcern.

References

on this

There

might

The

been

take pancreatic Elemental diets

patients

be recognized

that

support agents,

and/or

should Related

completed

syndrome

support.

during the past decade that the enteral feeding is generally satisfactory. The well-es-

advantages

ticular option although the supplements should be nutritionally balanced with respect to micronutrients. There is no ideal method for enzyme delivery at night. Until further research has

the patients at bedtime.

short-gut

refuse the enterostomy for heart-lung-transplant

is not appropriate

principle

advantages possibility

en-

who

CF

as short-

and for the had intestinal

significant abolic

ofan

in which

for short-

such

to attempt to shown to have

re-

(formula

evidence

problems,

The use of appetite stimulants and/or androgens enhance growth in children with CF has not been

CF

feeding) should be based on gastrointestinal and nutrition ciples. There are advantages and disadvantages associated each option (eg, elemental formulas, high-fat mixtures). thermore,

specific

of its high cost and increased

nutritional Anabolic

in some

placement

At-

supplements

indicated

severe gastroenteritis, of patients who have

it should

been well-established approach of tube

generally

tube.

The

with

eg, patients

support

tablished

Gastrostomy

can be controlled by adjustment positioning during sleep, and

etc.

imbalance

nutrition

per se are only

who are candidates

Nevertheless,

tubes

in sedated

situations

nutrition,

Nasogastric

to this approach.

sys-

pumps,

electrolyte

Parenteral

nutrition

pancreatitis, management

special

nutrition

percutaneously

support.

and

other

in highly

caloric

care.

and

and jejunostomy

committed

be placed

fluid

the patient The

daily home-care

infusion

surgery or similar procedures. Patients with may require long-term parenteral nutrition

tubes,

support

and

part of follow-up

in CF patients

gut syndrome, postoperative

involved. tubes

more

planned

The

intervention.

gastrostomy

require

contact,

intolerance

nutrition

and patients

tubes,

some a carefully

follow-up

parenteral

of an enterostomy tube and technique for its placement should be based on local factors and the expertise of the CF center Nasogastric

and

insulin.

for

ofboth

less

with

itoring approach

should as to the anticipated

commitment

for

a family

with

In all instances

tention needs to be given to the patient’s position during nocturnal tube feedings. It may be necessary to raise the head of the bed. Ifvomiting occurs, medical assessment is needed. Mon-

exacerbations

nutritional

be repleted

and total

the technique, and glucose tolerance plete information should be provided ration

can

suggested.

placement be done,

1) improved

of CF.

Until

and

during

8) improved

AL

3) increased

sense of control over body 6) development of secondary

weight

and

far(23-25):

El

oral patients

Downloaded from https://academic.oup.com/ajcn/article-abstract/55/1/108/4715278 by guest on 25 February 2018

1. Kraemer R, Rudeberg A, Hadom B, et al. Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 1978;67:33-7. 2. Corey M, McLaughlin FJ, Williams M. A comparison of survival, growth and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988;41:588-63. 3. Kerem B, Corey M, Kerem B, et al. Relationship between genotype and phenotype in cystic fibrosis-analysis ofthe most common mutatiom (SF508). N Engl J Med 1990;323:15l7-22. 4. Gaskin IU, Gurwitz D, Dune PR, Corey ML, Levison H, Forstner

G. Improved

respiratory

prognosis

in patients

with cystic fibrosis

with normal fat absorption. J Pediatr l982;lOO:857-62. 5. Dune PR, Pencharz PB. A rational approach to the nutritional care of patients with cystic fibrosis. J R Soc Med 1989;82: I 1-20. 6. Parsons HG, Dumas A, Beaudry P, Pencharz PB. Energy needs and growth in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 1983;2:44-9.

7. Daniels L, Davidson GP, Martin Al. Comparison of macromutrient intake of healthy controls and children with cystic fibrosis on low fat or non-restricted fat diets. J Pediatr Gastroenterol Nuts l987;7:38l-6.

CYSTIC

FIBROSIS

8. Chase PM, Long MA, Lavin MH. Cystic fibrosis and malnutrition. J Pediatr l979;95:337-47. 9. Congden PJ, Bruce G, Rothburn MM, et al. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child 198 1;8 1:708-14. 10. Underwood BA, Denning CR. Blood and liver concentrations of vitamin A and E in children with cystic fibrosis of the pancreas. Pediatr Res

1972:6:26-31.

I I. Farrell PM, Bieri JG, Fratantoni iF, et al. The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis. J Clim Invest l977;60:233-4l. 12. Hubbard VS, Farrell PM, di Sent’ Agnese PA. 25-hydroxycholecalciferol levels in patients with cystic fibrosis. J Pediatr 1979;94:84-6. 13. Gordon CC, Chumlea WC, Roche AF. Stature, recumbant length and weight. In: Lohman TG, Roche AF, Martorell R, eds. Anthropometric standardization reference manual. Champaign, IL: Human Kinetics

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14. Callaway CW, Chumlea WC, Buchard C, et al. Circumferences. In: Lohman TG, Roche AF, Martorell R, eds. Anthropometric standardization reference manual. Champaign, IL: Human Kinetics Books, 1988:51-2. 15. Harrison GG, Buskirk ER, Carter JEL, et al. Skinfold thicknesses and measurement technique. In: Lohman TG, Roche AF, Martorell R, eds. Anthropometric standardization reference manual. Champaign, IL: Human Kinetics Books, 1988:56-7. 16. Waterlow JC, Rutishauser IHE. Malnutrition in man. In: Cravioto J, Hambraeus L, Vahlquist B, eds. Early malnutrition and mental development. Sweden: Almquist and Wiksell, 1974. 17. Frisancho AR. New norms of upper limb fat and muscle areas for assessment of nutritional status. Am J Clin Nutr 198 l;34:2540-5. 18. Mischler EH, Chesney PJ, Chesney RW, Mazess RB. Demineralization in cystic fibrosis detected by direct photon absorptometry. Am J Dis Child 1979;l33:632-5. 19. Levy L, Dune P. Pencharz PB, Corey M. Prognostic factors associated with patient survival during nutritional rehabilitation in malnourished children and adolescents with cystic fibrosis. J Pediatr Gastroenterol Nutr 1986;5:97-102. 20. Luder E, Kattan M, Tamzer-Torres G, et al. Current recommendations for breast-feeding in cystic fibrosis centers. Am J Dis Child l990;144:1

153-6.

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24.

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APPENDIX

CONSENSUS Step the

2: Determine

same

y-old

centile

girl

with

Experience

for height Step and

determine

use the

of weight

third

as a percentage

of ideal

patients

the

patient’s height

height centile

(length) (if less

on the than

the

the

that

age,

Is weight

and

25th

gender,

centile,

growth third

chart centile

centile).

Downloaded from https://academic.oup.com/ajcn/article-abstract/55/1/108/4715278 by guest on 25 February 2018

on

eg, for a 6-

the

ideal

weight

of the weight chart for a 6-y-old only go up to 18 or 19 y of age.

calculation

is satisfactory

(actual

when

ofideal

weight-for-height

the values

at the oldest

18 or 19 y ofage) are used. weight as a percent of ideal

weight/ideal

weight-for-height)

end

weight-for-

X I 00.

Reference I . Moore BJ, Dune PR, Forstner ofnutritional status in children.

APPENDIX Determination For

of energy

patients

with

CF

(RDAs)

(1) for age and

patients growth.

do

not

fails

based

on

are

growing

than

to grow RDAs,

the

the

(2; see

Step plying

2: Calculate the BMR

Table

below:

activity

receiving

caloric

should

be used

Sample predicted

volume ofthat lung

to bed

in I s (FEV10)

with

predicted disease,

(BMR

range

80%

ofthat

[BMR

BMR

X (1.5

=

BMR

X 1.7

up to 0.5 with very severe

(in kcal) Females

predicted

lung disease, ie, X (AC + 0.2)1; for pa-

ie, FEV10

=

BMR

X 1.3);

moderate