**Cystic Fibrosis Foundation**

**Proposed Changes**

**June 13, 2005**

**CHILD LISTINGS**

**103.00 Respiratory System**

A. *Introduction.* The listings in this section describe impairments
resulting from respiratory disorders based on symptoms, physical signs,
laboratory test abnormalities, and response to a regimen of treatment
prescribed by a treating source. Respiratory disorders, along with any
associated impairment(s) must be established by medical evidence.
Evidence must be provided in sufficient detail to permit an independent
reviewer to evaluate the severity of the impairment. Reasonable efforts
should be made to ensure evaluation by a program physician specializing
in childhood respiratory impairments or a qualified pediatrician.

Many children, especially those who have listing-level impairments, will
have received the benefit of medically prescribed treatment. Whenever
there is such evidence, the longitudinal clinical record must include a
description of the treatment prescribed by the treating source and
response, in addition to information about the nature and severity of
the impairment. It is important to document any prescribed treatment and
response because this medical management may have improved the child\'s
functional status. The longitudinal record should provide information
regarding functional recovery, if any.

Some children will not have received ongoing treatment or have an
ongoing relationship with the medical community, despite the existence
of a severe impairment(s). A child who does not receive treatment may or
may not be able to show an impairment that meets the criteria of these
listings. Even if a child does not show that his or her impairment meets
the criteria of these listings, the child may have an impairment(s) that
medically or functionally equals the listings.

Unless the claim can be decided favorably on the basis of the current
evidence, a longitudinal record is still important because it will
provide information about such things as the ongoing medical severity of
the impairment, the level of the child\'s functioning, and the
frequency, severity, and duration of symptoms. Also, the asthma listing
specifically includes a requirement for continuing signs and symptoms
despite a regimen of prescribed treatment.

Evaluation should include consideration of adverse effects of
respiratory impairment in all relevant body systems, and especially on
the child\'s growth and development or mental functioning, as described
under the growth impairment (100.00), neurological (111.00), and mental
disorders (112.00) listings.

It must be remembered that these listings are only examples of common
respiratory disorders that are severe enough to find a child disabled.
When a child has a medically determinable impairment that is not listed,
an impairment that does not meet the requirements of a listing, or a
combination of impairments no one of which meets the requirements of a
listing, we will make a determination whether the child\'s impairment(s)
medically or functionally equals the listings. (See §§ 404.1526,
416.926, and 416.926a.)

[Add information about advances in medicine that allow people with
respiratory impairments to obtain lung transplants, and the need
to]{.mark} [continue to review]{.mark} [their functional status
indefinitely. This should be done several years after the procedure, and
take into account the multi-system impact of the initial diagnosis, and
complications related to the transplant itself and its
treatments.]{.mark}

B. *Documentation of Pulmonary Function Testing.* The results of
spirometry that are used for adjudication, under the 103.02 A and B,
103.03, and 103.04 of these listings should be expressed in liters (L),
body temperature and pressure saturated with water vapor (BTPS). The
reported one-second forced expiratory volume (FEV~1~) and forced vital
capacity (FVC) should represent the largest of at least three
satisfactory forced expiratory maneuvers. Two of the satisfactory
spirograms should be reproducible for both pre-bronchodilator tests and,
if indicated, postbronchodilator tests. A value is considered
reproducible if it does not differ from the largest value by more than 5
percent or 0. 1 L, whichever is greater. The highest values of the
FEV~1~, and FVC, whether from the same or different tracings, should be
used to assess the severity of the respiratory impairment.

Peak flow should be achieved early in expiration, and the spirogram
should have a smooth contour with gradually decreasing flow throughout
expiration. The zero time for measurement of the FEV~1~ and FVC, if not
distinct, should be derived by linear back-extrapolation of peak flow to
zero volume. A spirogram is satisfactory for measurement of the FEV~1~
if the expiratory volume at the back-extrapolated zero time is less than
5 percent of the FVC or 0.1 L, whichever is greater. The spirogram is
satisfactory for measurement of the FVC if maximal expiratory effort
continues for at least 6 seconds, or if there is a plateau in the
volume-time curve with no detectable change in expired volume (VE)
during the last 2 seconds of maximal expiratory effort.

Spirometry should be repeated after administration of an aerosolized
bronchodilator under supervision of the testing personnel if the
prebronchodilator FEV~1~ value is less than the appropriate reference
value in table I or III, as appropriate. If a bronchodilator is not
administered, the reason should be clearly stated in the report.
Pulmonary function studies should not be performed unless the clinical
status is stable (e.g., the child is not having an asthmatic attack or
suffering from an acute respiratory infection or other acute illness.).
Wheezing is common in asthma, chronic bronchitis, or chronic obstructive
pulmonary disease and does not preclude testing.

Pulmonary function studies performed to assess airflow obstruction
without testing after bronchodilators cannot be used to assess levels of
impairment in the range that prevents a child from performing age
appropriate activities unless the use of bronchodilators is
contraindicated. Post-bronchodilator testing, should be performed 10
minutes after bronchodilator administration. The dose and name of the
bronchodilator administered should be specified. The values in 103.02
and 103.04 must only be used as criteria for the level of ventilatory
impairment that exists during the child\'s most stable state of health
(i.e., any period in time except during or shortly after an
exacerbation).

If the spirogram was generated by any means other than direct pen
linkage to a mechanical displacement-type spirometer, the testing device
must have had a recorded calibration performed previously on the day of
the spirometric measurement.

The appropriately labeled spirometric tracing, showing the child\' s
name, date of testing, distance per second on the abscissa and the
distance per liter (L) on the ordinate, must be incorporated into the
file. The manufacturer and model number of the device used to measure
and record the spirogram should be stated. The testing device must
accurately measure both time and volume, the latter to within 1 percent
of a 3 L calibrating volume. If the spirogram was generated by any means
other than by direct pen linkage to a mechanical displacement-type
spirometer, the spirometric tracing must show a recorded calibration of
volume units using a mechanical volume input such as a 3 L syringe.

If the spirometer directly measures flow, and volume is derived by
electronic integration, the linearity of the device must be documented
by recording volume calibrations at three different flow rates of
approximately 30 L/min (3 L/6 sec), 60 L/min (3 L/3 sec), and 180 L/min
(3 L/sec). The volume calibrations should agree to within 1 percent of a
3 L calibrating volume. The proximity of the flow sensor to the child
should be noted, and it should be stated whether or not a BTPS
correction factor was used for the calibration recordings and for the
child\'s actual spirograms.

The spirogram must be recorded at a speed of at least 20 mm/sec, and the
recording device must provide a volume excursion of at least 10 mm/L. If
reproductions of the original spirometric tracings are submitted, they
must be legible and have a time scale of at least 20 mm/sec and a volume
scale of at least 10 mm/L to permit independent measurements.
Calculation of FEV~1~ from a flow-volume tracing is not acceptable;
i.e., the spirogram and calibrations must be presented in a volume-time
format at a speed of at least 20 mm/sec and a volume excursion of at
least 10 mm/L to permit independent evaluation.

A statement should be made in the pulmonary function test report of the
child\'s ability to understand directions, as well as his or her effort
and cooperation in performing the pulmonary function tests.

Purchase of a pulmonary function test is appropriate only when the child
is capable of performing reproducible forced expiratory maneuvers. This
capability usually occurs around age 6. Purchase of a pulmonary function
test may be appropriate when there is a question of whether an
impairment meets or is equivalent in severity to a listing, and the
claim cannot otherwise be favorably decided.

The pulmonary function tables in 103.02 and 103.04 are based on
measurement of standing height without shoes. If a child has marked
spinal deformities (e.g. kyphoscoliosis), the measured span between the
fingertips with the upper extremities abducted 90 degrees should be
substituted for height when this measurement is greater than the
standing height without shoes.

C. Documentation of chronic impairment of gas exchange.

1**.** Arterial blood gas studies (ABGS). An ABGS performed at rest
(while breathing room air, awake and sitting or standing) should be
analyzed in a laboratory certified by a State or Federal agency. If the
laboratory is not certified, it must submit evidence of participation in
a national proficiency testing program as well as acceptable quality
control at the time of testing. The report should include the altitude
of the facility and the barometric pressure on the date of analysis.

Purchase of a resting ABGS may be appropriate when there is a question
of whether an impairment meets or is equivalent in severity to a
listing, and the claim cannot otherwise be favorably decided. Before
purchasing resting ABGS a program physician, preferably one experienced
in the care of children with pulmonary disease, must review the clinical
and laboratory data short of this procedure, including spirometry, to
determine whether obtaining the test would present a significant risk to
the child.

2\. *Oximetry.* Pulse oximetry may be substituted for arterial blood
gases in children under 12 years of age. The oximetry unit should employ
the basic technology of spectrophotometric plethysmography as described
in Taylor, M.B., and Whitwain, J.G., \"Current Status of Pulse
Oximetry,\" \"Anesthesia,\" Vol. 41. No. 9, pp. 943-949, 1986. The unit
should provide a visual display of the pulse signal and the
corresponding oxygen saturation. A hard copy of the readings (heart rate
and saturation) should be provided. Readings should be obtained for a
minimum of 5 minutes. The written report should describe patient
activity during the recording; i.e., sleep rate, feeding, or exercise.
Correlation between the actual heart rate determined by a trained
observer and that displayed by the oximeter should be provided. A
statement should be made in the report of the child\'s effort and
cooperation during the test.

Purchase of oximetry may be appropriate when there is a question of
whether an impairment meets or is equivalent in severity to a listing,
and the claim cannot otherwise be favorably decided.

D. *Cystic fibrosis* is a disorder that [can]{.mark} [affect the]{.mark}
respiratory, digestive, [endocrine,]{.mark} [reproductive,]{.mark}
[sinus and hepatobiliary systems]{.mark} and may impact on a child\'s
growth and development. It is responsible for a wide and variable
spectrum of clinical manifestations and complications.
**[~~abnormalities in the cystic fibrosis gene or in the function of its
protein product.~~]{.mark}**

[New]{.mark} [wording for bolded section:\
Diagnosis of CF should be based on the presence of one or more
characteristic phenotypic features, a history of CF in a sibling or a
positive newborn screening test, plus laboratory evidence of a CFTR
abnormality as document by elevated sweat chloride concentrations; or
identification of two CF mutations; or]{.mark} *[in vivo]{.mark}*
[demonstration of characteristic abnormalities in ion transport across
the nasal epithelium. Phenotypic features include:]{.mark}

[]{.mark}

-   [Chronic sino-pulmonary disease manifested by:]{.mark}

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-   [Persistent colonization/infection with typical CF
    pathogens.]{.mark}

-   [Chronic cough and sputum production.]{.mark}

-   [Persistent chest radiograph abnormalities (e.g., bronchiectasis,
    atelectasis, infiltrates, hyperinflation).]{.mark}

-   [Airway obstruction manifested by wheezing and air trapping.]{.mark}

-   [Nasal polyps; radiograph or CT abnormalities of the paranasal
    sinuses.]{.mark}

-   [Digital clubbing.]{.mark}

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-   [Gastro-intestinal and nutritional abnormalities, including:]{.mark}

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-   [Intestinal: meconium ileus; distal intestinal obstruction syndrome
    (DIOS); rectal prolapse.]{.mark}

-   [Pancreatic: pancreatic insufficiency; recurrent
    pancreatitis.]{.mark}

-   [Hepatic: chronic hepatic disease manifested by clinical or
    histologic evidence of focal biliary cirrhosis or multi-lobular
    cirrhosis.]{.mark}

-   [Nutritional: failure to thrive (protein-calorie malnutrition);
    hypoproteinemia and edema; complications secondary to fat-soluble
    vitamin deficiency.]{.mark}

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-   [Salt loss syndromes: acute salt depletion; chronic metabolic
    alkalosis.]{.mark}

-   [Male urogenital abnormalities resulting in obstructive
    azoospermia.]{.mark}

[]{.mark}

[Source: Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a
consensus statement. Cystic Fibrosis Foundation Consensus Panel.]{.mark}
[[J Pediatr.]{.underline}]{.mark} [1998 Apr;132(4):589-95.]{.mark}

[]{.mark}

The quantitative pilocarpine iontophoresis procedure for collection of
sweat [and subsequent analysis]{.mark} must be utilized. Two methods are
acceptable: [the test must be]{.mark} [carried out in accordance with
the guidelines of the]{.mark} [National Committee for Clinical
Laboratory Standards (NCCLS), now the Clinical and Laboratory Standards
Institute (CLSI). Source: Sweat testing: sample collection and
quantitative analysis- approved guideline \[Document C34-A2\] Wayne (PA)
2000. (Address; 940 W. Valley Road, Suite 1400, Wayne Pa 19087]{.mark}
[[www.clsi.org](http://www.clsi.org/)).]{.mark} [or the "Wescor
Macroduct System" must be used.]{.mark} []{.mark}

[]{.mark}

**[~~Another~~]{.mark}** **[~~test is~~]{.mark}** [**~~of the cystic
fibrosis gene.~~**]{.mark}

[]{.mark}

The pulmonary manifestations of this disorder should be evaluated under
103.04. The nonpulmonary aspects of cystic fibrosis should be evaluated
under the listings for digestive system (105.00[),]{.mark} growth
impairments (100.00[)]{.mark} [and endocrine system (109.00).]{.mark}
Because cystic fibrosis may involve the respiratory[,]{.mark}
[]{.mark}digestive[,]{.mark} [endocrine]{.mark} [and
hepatobiliary]{.mark} body systems, as well as impact on a child\'s
growth and development, the combined effects of this involvement must be
considered in case adjudication.

Medically acceptable imaging includes, but is not limited to, x-ray
imaging, computerized axial tomography (CAT scan) or magnetic resonance
imaging (MRI), with or without contrast material, myelography, and
radionuclear bone scans. \"Appropriate\" means that the technique used
is the proper one to support the evaluation and diagnosis of the
impairment.

[Add information about the need to document]{.mark} [the exacerbations,
including the time period required for]{.mark} [daily]{.mark}
[self-treatment]{.mark} [of the disease.]{.mark} [To achieve this,
please add information]{.mark} [from]{.mark} [adult listings]{.mark}:

> ["C. Episodic respiratory disease. When a respiratory impairment is
> episodic in nature, as can occur with exacerbations of asthma, cystic
> fibrosis, bronchiectasis, or chronic asthmatic bronchitis, the
> frequency and intensity of episodes that occur despite prescribed
> treatment are often the major criteria for determining the level of
> impairment.]{.mark}
>
> []{.mark}
>
> [Documentation for these exacerbations should include available
> hospital, emergency facility and/or physician records indicating the
> dates of treatment; clinical and laboratory findings on presentation,
> such as the results of spirometry and arterial blood gas studies
> (ABGS); the treatment administered; the time period required
> for]{.mark} [**daily self-treatment**; and the clinical
> response.]{.mark}
>
> []{.mark}
>
> [Attacks of asthma, episodes of bronchitis or pneumonia or hemoptysis
> (more than blood-streaked sputum), or respiratory failure as referred
> to in]{.mark} *[paragraph B of 3.03, 3.04, and 3.07,]{.mark}* [are
> defined as prolonged symptomatic episodes lasting one or more days and
> requiring intensive treatment, such as intravenous bronchodilator or
> antibiotic administration or prolonged inhalational bronchodilator
> therapy in a hospital, emergency room or equivalent setting.]{.mark}
>
> []{.mark}
>
> [Hospital admissions are defined as inpatient hospitalizations for
> longer than 24 hours. The medical evidence must also include
> information documenting adherence to a prescribed regimen of treatment
> as well as a description of physical signs. For asthma, the medical
> evidence should include spirometric results obtained between attacks
> that document the presence of baseline airflow obstruction."]{.mark}

E. *Bronchopulmonary dysplasia (BPD).* Bronchopulmonary dysplasia is a
form of chronic obstructive pulmonary disease that arises as a
consequence of acute lung injury in the newborn period and treatment of
hyaline membrane disease, meconium aspiration, neonatal pneumonia and
apnea of prematurity. The diagnosis is established by the requirement
for continuous or nocturnal supplemental oxygen for more than 30 days,
in association with characteristic changes on medically acceptable
imaging and clinical signs of respiratory dysfunction, including
retractions, rales, wheezing, and tachypnea.

**103.01 Category of Impairments, Respiratory System**

**103.02 *Chronic Pulmonary insufficiency****.* With:

A. Chronic obstructive pulmonary disease due to any cause with the
FEV~1~ equal to or less than the value specified in Table I
corresponding to the child\'s height without shoes. (In cases of marked
spinal deformity, see 103.00B.);

Table I

  ---------------------------- ----------------------- -------------------
  **Height without Shoes       **Height without Shoes  **FEV~1~ equal to
  (centimeters)**              (inches)**              or less than
                                                       (L,BTPS)**

  119 or less. . . . .         46 or less . . . .      0.65

  120-129. . . . . . .         47-50 . . . . . . .     0.75

  130-139 . . . . . . .        51-54 . . . . . . .     0.95

  140-149 . . . . . . .        55-58 . . . . . . .     1.15

  150-159 . . . . . . .        59-62 . . . . . . .     1.35

  160-164 . . . . . . .        63-64 . . . . . . .     1.45

  165-169 . . . . . . .        65-66 . . . . . . .     1.55

  170 or more . . . .          67 or more . . .        1.65
  ---------------------------- ----------------------- -------------------

*Or*

B. Chronic restrictive ventilatory disease, due to any cause, with the
FVC equal to or less then the value specified in Table II corresponding
to the child\'s height without shoes. (In cases of marked spinal
deformity, see 103.00B.);

**Table II**

  --------------------------- ----------------------- -------------------
  **Height without Shoes      **Height without Shoes  **FVC equal to or
  (centimeters)**             (inches)**              less than
                                                      (L,BTPS)**

  119 or less . . . .         46 or less . . . .      0.65

  120-129 . . . . . .         47-50 . . . . . . .     0.85

  130-139 . . . . . .         51-54 . . . . . . .     1.05

  140-149 . . . . . .         55-58 . . . . . . .     1.25

  150-159 . . . . . .         59-62 . . . . . . .     1.45

  160-164 . . . . . .         63-64 . . . . . . .     1.65

  165-169 . . . . . .         65-66 . . . . . . .     1.75

  170 or more . . .           67 or more . . .        2.05
  --------------------------- ----------------------- -------------------

*Or*

C. Frequent need for:

1\. Mechanical ventilation; or

2\. Nocturnal supplemental oxygen as required by persistent or recurrent
episodes of hypoxemia;

*Or*

D. The presence of a tracheostomy in a child under 3 years of age;

*Or*

E. Bronchopulmonary dysplasia characterized by two of the following;

1\. Prolonged expirations; or

2\. Intermittent wheezing or increased respiratory effort as evidenced
by retractions, flaring and tachypnea; or

3\. Hyperinflation and scarring on a chest radiograph or other
appropriate imaging techniques; or

4\. Bronchodilator or diuretic dependency; or

5\. A frequent requirement for nocturnal supplemental oxygen; or

6\. Weight disturbance with:

a\. An involuntary weight loss (or failure to gain weight at an
appropriate rate for age) resulting in a fall of 15 percentiles from
established growth curve (on standard growth charts) which persists for
2 months or longer; or

b\. An involuntary weight loss (or failure to gain weight at an
appropriate rate for age) resulting in a fall to below the third
percentile from established growth curve (on standard growth charts)
which persists for 2 months or longer;

*Or*

F. Two required hospital admissions (each longer than 24 hours) within a
6-month period for recurrent lower respiratory tract infections or acute
respiratory distress associated with:

1\. Chronic wheezing or chronic respiratory distress; or

2\. Weight disturbance with;

a\. An involuntary weight loss (or failure to gain weight at an
appropriate rate for age) resulting in a fall of 15 percentiles from
established growth curve (on standard growth charts) which persists for
2 months or longer; or

b\. An involuntary weight loss (or failure to gain weight at an
appropriate rate for age) resulting in a fall to below the third
percentile from established growth curve (on standard growth charts)
which persists for 2 months or longer;

*Or*

G. Chronic hypoventilation (Pa CO~2~ greater than 45 mm Hg) or chronic
cor pulmonale as described under the appropriate criteria in 104.02;

*Or*

H. Growth impairment as described under the criteria in 100.00.

**103.03 *Asthma***. With:

A. FEV~1~ equal to or less than the value specified in Table I\
of 103.02A:

*Or*

B. Attacks (as defined in 3.00C), in spite of prescribed treatment and
requiring physician intervention, occurring at least once every 2 months
or at least six times a year. Each inpatient hospitalization for longer
than 24 hours for control of asthma counts as two attacks, and an
evaluation period of at least 12 consecutive months must be used to
determine the frequency of attacks.

*Or*

C. Persistent low-grade wheezing between acute attacks or absence of
extended symptom-free periods requiring daytime and nocturnal use of
sympathomimetic bronchodilators with one of the following:

1\. Persistent prolonged expiration with radiographic or other
appropriate imaging techniques evidence of pulmonary hyperinflation or
peribronchial disease; or

2\. Short courses of corticosteroids that average more than 5 days per
month for at least 3 months during a 12-month period;

*Or*

D. Growth impairment as described under the criteria in 100.00.

**103.04 *Cystic fibrosis****.* With:

A. An FEV~1~ equal to or less than the appropriate value specified in
Table III corresponding to the child's height without shoes. (In cases
of marked spinal deformity, see. 103.00B.);

*Or*

B. For children in whom pulmonary function testing cannot be performed,
the presence of two of the following:

1\. History of dyspnea on exertion or accumulation of secretions as
manifested by repetitive

coughing or cyanosis; or

2\. Persistent bilateral rales and rhonchi or substantial reduction of
breath sounds related to mucous plugging of the trachea or bronchi; or

3\. Appropriate medically acceptable imaging evidence of extensive
disease, such as thickening of the proximal bronchial airways or
persistence of bilateral peribronchial infiltrates;

*Or*

C. Persistent pulmonary infection accompanied by superimposed,
recurrent, symptomatic episodes of increased bacterial infection
occurring at least once every 6 months and requiring intravenous or
nebulization antimicrobial treatment;

*Or*

D. Episodes of bronchitis or pneumonia or hemoptysis (more than
bloodstreaked sputum) or respiratory failure (documented according to
[3.00C),]{.mark} requiring physician intervention, occurring at least
once every 2 months or at least six times a year, [or pneumothorax
occurring]{.mark} [once in a 2-3 year period.]{.mark} Each inpatient
hospitalization for longer than 24 hours for treatment counts as two
episodes, and an evaluation period of at least 12 consecutive months
must be used to determine the frequency of episodes;

*Or*

E. Growth impairment as described under the criteria in 100.00.

**Table III**

(Applicable only for evaluation under 103.04A - cystic fibrosis)

  ----------------------------- ----------------------- ------------------
  **Height without Shoes        **Height without Shoes  **FEV~1~ equal to
  (centimeters)**               (inches)**              or less than
                                                        (L,BTPS)**

  119 or less . . . . .         46 or less . . . .      0.75

  120-129 . . . . . . .         47-50 . . . . . . .     0.85

  130-139 . . . . . . .         51-54 . . . . . . .     1.05

  140-149 . . . . . . .         55-58 . . . . . . .     1.35

  150-159 . . . . . . .         59-62 . . . . . . .     1.55

  160-164 . . . . . . .         63-64 . . . . . . .     1.85

  165-169 . . . . . . .         65-66 . . . . . . .     2.05

  170 or more . . .             67 or more . . .        2.25
  ----------------------------- ----------------------- ------------------

**103.05 *Lung Transplant*.** Consider under a disability for
[36]{.mark} [months]{.mark} [or indefinitely]{.mark} following the date
of surgery; thereafter, evaluate the residual impairment(s).

[For individuals]{.mark} [with cystic fibrosis]{.mark} [who received
lung transplant,]{.mark} [evaluate]{.mark} [under listings for
respiratory (including sinus), growth]{.mark} [impairment,
digestive,]{.mark} [endocrine]{.mark} [and]{.mark}
[hepatobiliary]{.mark} [systems]{.mark} [to assess]{.mark}
[multi-system]{.mark} [impact]{.mark} [of disease.]{.mark} [Also
consider impact of transplant]{.mark} [on health,]{.mark} [such as
episodes of rejection, side effects of transplant medications,
fractures, liver]{.mark} [dysfunction, bronchiolitis obliterans, as well
as the development of other complications such as diabetes]{.mark}
[mellitus]{.mark} [and cancer.]{.mark}

**OTHER** **100.02 *Growth impairment****,* considered to be related to
an additional specific medically determinable impairment, and one of the
following:

A. Fall of greater than 15 percentiles in height which is sustained; or

B. Fall to, or persistence of, height [at or]{.mark} below the
[tenth]{.mark} percentile.

[Add language]{.mark} [on]{.mark} [Body Mass Index,]{.mark} [and state
that an individual who experiences a fall to, or persistence of Body
Mass Index (BMI*)*]{.mark} [at or below the]{.mark} [tenth]{.mark}
[percentile should be considered impaired.]{.mark}

[To support use of]{.mark} [10^th^]{.mark} [percentile, see]{.mark}
[Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for
pediatric patients with cystic fibrosis.]{.mark} *[[J Pediatr
Gastroenterol Nutr.]{.underline}]{.mark}* [2002
Sep;35(3):246-59.]{.mark}