Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011

Abstract

The members of the Pediatric Task Force of the Pulmonary Vascular Research Institute (PVRI) were aware of the need to develop a functional classification of pulmonary hypertension in children. The proposed classification follows the same pattern and uses the same criteria as the Dana Point pulmonary hypertension specific classification for adults. Modifications were necessary for children, since age, physical growth and maturation influences the way in which the functional effects of a disease are expressed. It is essential to encapsulate a child's clinical status, to make it possible to review progress with time as he/she grows up, as consistently and as objectively as possible. Particularly in younger children we sought to include objective indicators such as thriving, need for supplemental feeds and the record of school or nursery attendance. This helps monitor the clinical course of events and response to treatment over the years. It also facilitates the development of treatment algorithms for children. We present a consensus paper on a functional classification system for children with pulmonary hypertension, discussed at the Annual Meeting of the PVRI in Panama City, February 2011.

Full text

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Pulmonary Circulation | April-June 2011 | Vol 1 | No 2 280
Guidelines and Consensus
INTRODUCTION
The members of the Pediatric Task Force of the Pulmonary
Vascular Research Institute (PVRI) were very aware of
the need to develop a functional classification system for
children with pulmonary hypertension and discussed the
problem at the Annual Meeting of the PVRI held in Panama
City in February 2011. We now present the consensus
document resulting from our deliberations. We expect
that it will be modified and improved in the years to come.
BACKGROUND
Clinical classifications of functional status are created
Functional classification of pulmonary
hypertension in children: Report from the PVRI
pediatric taskforce, Panama 2011
Astrid E. Lammers1, Ian Adatia2, Maria Jesus del Cerro3, Gabriel Diaz4, Alexandra Heath Freudenthal 5,
Franz Freudenthal5, S. Harikrishnan6, Dunbar Ivy7, Antonio A. Lopes8, J. Usha Raj9, Julio Sandoval9,
Kurt Stenmark10, and Sheila G. Haworth11
1Great Ormond Street Hospital for Children, London, UK, 2Stollery Children’s Hospital, University of Alberta, Edmonton, Canada,
3La Paz University Hospital, Madrid, Spain, 4National University, Bogota, Colombia, 5Kardiozentrum, La Paz, Bolivia, 6Sree Chitra
Tirunal Institute for Medical Sciences and Technology, Trivandrum, India, 7Denver Children’s Hospital, University of Colorado, USA,
8Heart Institute, University of São Paulo, São Paulo, Brazil, 9University of Illinois at Chicago, Chicago, USA, 10University of Colorado,
Denver, USA, 11Emeritus Professor, University College, London, UK
ABSTRACT
The members of the Pediatric Task Force of the Pulmonary Vascular Research Institute (PVRI) were aware of the need to develop a
functional classification of pulmonary hypertension in children. The proposed classification follows the same pattern and uses the same
criteria as the Dana Point pulmonary hypertension specific classification for adults. Modifications were necessary for children, since
age, physical growth and maturation influences the way in which the functional effects of a disease are expressed. It is essential to
encapsulate a child’s clinical status, to make it possible to review progress with time as he/she grows up, as consistently and as objectively
as possible. Particularly in younger children we sought to include objective indicators such as thriving, need for supplemental feeds and
the record of school or nursery attendance. This helps monitor the clinical course of events and response to treatment over the years.
It also facilitates the development of treatment algorithms for children. We present a consensus paper on a functional classification
system for children with pulmonary hypertension, discussed at the Annual Meeting of the PVRI in Panama City, February 2011.
Key Words: pulmonary hypertension, children, functional class
primarily so that physicians can apply a common language
to describe the functional impact of the same underlying
disease on individual patients. Classifications should
facilitate communication between physicians, all those
involved in caring for the patient and the funding agencies
providing financial support. The design of clinical trials
is heavily dependant on an accepted, standardized means
of describing the efficacy or otherwise of the treatment
being evaluated. Studying the natural history of a disease,
both treated and untreated, is dependant on a universally
accepted means of describing functional status. Most
importantly, a good classification should encapsulate
Address correspondence to:
Dr. Astrid E Lammers
Department of Paediatric Cardiology
Great Ormond Street Hospital for Children
Great Ormond Street
London WC1N 3JH, UK
Email: astridlammers@gmx.de
Access this article online
Website: www.pulmonarycirculation.org
DOI: 10.4103/2045-8932.83445
How to cite this article: Lammers AE, Adatia
I, del Cerro MJ, Diaz G, Freudenthal AH,
Freudenthal F, Harikrishnan S, Ivy D, Lopes
AA, Raj JU, Sandoval J, Stenmark K, Haworth
SG. Functional classification of pulmonary
hypertension in children: Report from the
PVRI pediatric taskforce, Panama 2011. Pulm
Circ 2011;1:280-5.
Quick Response Code:

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Pulmonary Circulation | April-June 2011 | Vol 1 | No 2281
the clinical status of a patient and so make it possible to
review the patient’s progress with time and their response
to treatment.
Classifications of functional status are familiar to
cardiologists caring for adult patients. The New York Heart
Association (NYHA), the classification most widely used
by adult cardiologists since 1964 describes the functional
impact of heart failure and places patients with a similar
degree of limitation and similar symptoms into one of
four functional classes, Class IV being the most severely
disabled (Table 1).[1] The Functional Classification of
Pulmonary Hypertension in adults is based on the NYHA
classification (Table 2) and was published in 1998 as a
consensus document of the WHO Symposium held in
Evian in that year.[2]
Maintaining the best possible quality of life is crucial in
any chronic disease. In adults, The Minnesota Living with
Heart Failure (MLHF) questionnaire has been widely used
in both hospital and primary care since it was designed
in 1984, and has good reliability and validity.[3-5] This
questionnaire can be useful in assessing patients with
pulmonary hypertension,[6,7] as is the Short Form Health
Survey 36 (SF-36).[8,9] The MLHF has been considered
a significant predictor of outcome. [10] More recently, a
disease specific questionnaire, the Cambridge Pulmonary
Hypertension Outcome Review Utility Index (CAMPHOR)
has been developed, primarily for cost-utility analysis but
can also be useful in clinical studies.[11,12]
There is no disease specific classification to assess
functional status in children with pulmonary hypertension.
Nor is there a generally accepted functional classification
for children with heart disease. Measures of generic health
status have been developed for children, principally
the Children and Youth Version of the International
Classification of Functioning, Disability and Health
published in 2007.[13,14] This classification assesses
body structure and function, level of activity and social
participation. It was designed primarily to assess children
with neuromotor disabilities. Disease specific functional
classifications have been developed for use in children
with other conditions such as cystic fibrosis, rheumatoid
arthritis and juvenile idiopathic arthritis.[15-18]
Quality of life is particularly difficult to assess in children. A
Short Form Health Survey has been designed for children,
SF-10[19] and can be used in children with pulmonary
hypertension, but it is not disease specific. Nor does it help
assess children less than 5 five years of age.
DESIGNING THE PROPOSED
FUNCTIONAL CLASSIFICATION OF
PULMONARY HYPERTENSION IN
CHILDREN
The proposed Functional Classification of Pulmonary
Hypertension in Children is based on the Evian pulmonary
hypertension specific classification used in adults. It is
not designed to assess quality of life and neither parent
nor child makes a personal, direct contribution to the
assessment. Particularly in children however, assessing
Table 1: NYHA classification of functional status
NYHA Class Symptoms
I Patients with heart disease but without limitation of physical activity
Patients with heart disease resulting in slight limitation of physical activity
Comfortable at rest
Patients with heart disease resulting in marked limitation of physical activity. Comfortable at rest. Less than
ordinary activity causes fatigue, dyspnoea, palpitations, anginal pain
Patients with heart disease resulting in inability to carry out any physical activity without discomfort.
Symptoms of cardiac insufficiency or of the anginal syndrome may be present even at rest. If any physical
activity is undertaken, discomfort increases
II
III
IV
Table 2: WHO classification of pulmonary hypertension in adults
Class Symptoms
IPatients with pulmonary hypertension but without limitation of physical activity. Ordinary physical activity does not
cause undue dyspnoea, fatigue chest pain or near syncope
Patients with pulmonary hypertension resulting in slight limitation of physical activity. Comfortable at rest. Ordinary
physical activity causes undue dyspnoea, fatigue, chest pain or near syncope
Patients with pulmonary hypertension resulting in marked limitation of activity. Comfortable at rest. Less than ordi-
nary activity causes dyspnoea or fatigue, chest pain or near syncope
Patients with pulmonary hypertension resulting in inability to carry out any physical activity without symptoms.
These patients manifest symptoms of right heart failure. Dyspnoea and/or fatigue may be present even at rest.
Discomfort is increased by any physical activity undertaken. Syncope or near syncope can occur
II
III
IV
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Pulmonary Circulation | April-June 2011 | Vol 1 | No 2 282
function and activity does inform the physician about the
quality of life.
Designing a functional classification is particularly
difficult in children because the age, physical growth and
maturation achieved influences the way in which the
functional effects of a disease are expressed. It is essential
therefore to be able to encapsulate a child’s clinical status
as he/she grows up in a consistent manner which makes
it possible to monitor the clinical course of events over
the years. In young children it is particularly challenging
to distinguish whether any apparent deficiency in
expectation can be attributed to the disease or is merely
a normal variant in a developmental milestone. This
is especially the case if pulmonary hypertension is
complicated by syndromic or chromosomal abnormalities
that affect motor and sensory function. Since any parent is
naturally loathe to acknowledge that their child is really
ill, any clinical classification needs to be as objective as
possible.
The proposed Functional Classification of Pulmonary
Hypertension in Children follows the same pattern as the
adult classification (Tables 3-7). There are four classes of
disease severity, Class IV being the most severe. Class III
has been subdivided into a) and b). We have tried to ensure
that the classification will give a comprehensive summary
of the child’s clinical condition, while keeping it practical
and easy to use. We decided to define functional class in
five different age groups (Table 3). Three of the groups
Table 3: Pediatric functional classification for children aged 0–0.5 years
ClassChildren with pulmonary hypertension
IAsymptomatic, growing and developing normally, no limitation of physical activity. Gains head control and
increases body tone from 0 to 3 months, then rolls over and has no head lag. Sitting with support
Slight limitation of physical activity, unduly dyspnoeic and fatigued. Falling behind physical developmental
milestones. Comfortable at rest. Continues to grow along own centiles
Marked limitation of physical activity, unduly fatigued. Regression of learned physical activities. Quiet and needs
frequent naps. Comfortable at rest. Less than ordinary activity causes undue fatigue or syncope and/or
presyncope. Growth compromised. Poor appetite. Requires excessive medical attention
Growth severely compromised. Poor appetite. Supplemental feeding. Less than ordinary activity causes undue
fatigue or syncope. Plus features of Class IIIa
Unable to carry out any physical activity without undue dyspnoea, fatigue or syncope, not interacting with family.
Syncope and/or right heart failure. Plus features of Class III
II
IIIa
IIIb
IV
Lammers et al.: Functional class in children with PH
Table 4: Pediatric functional classification for children aged 0.5–1 years
Class Children with pulmonary hypertension
IAsymptomatic, growing along own centiles, no limitation of physical activity. Mobile, sitting, grasping, starting to
stand, crawling, playing
Slight limitation of physical activity, unduly dyspnoeic and fatigued when playing. Delayed physical development.
Comfortable at rest. Continues to grow along own centiles
Marked limitation of physical activity. Regression of learned physical activities. Stops crawling. Quiet and needs
frequent naps. Hesitant and unadventurous. Comfortable at rest. Less than ordinary activity causes undue fatigue
or syncope and/or presyncope. Growth compromised. Poor appetite. Requires excessive medical attention
Growth severely compromised. Poor appetite. Supplemental feeding. Less than ordinary activity causes undue
fatigue or syncope. Plus features of Class IIIa
Unable to carry out any physical activity without undue dyspnoea, fatigue or syncope, not interacting with family.
Syncope and/or right heart failure. Plus features of Class III
II
IIIa
IIIb
IV
Table 5: Pediatric functional classification for children aged 1–2 years
Class Children with pulmonary hypertension
I Asymptomatic, growing along own centiles, no limitation of physical activity. Standing, starting to walk/walking,
climbing
Slight limitation of physical activity, unduly dyspnoeic and fatigued when playing. Delayed physical development.
Comfortable at rest. Continues to grow along own centiles
Marked limitation of physical activity. Regression of learned physical activities. Reluctant to play. Quiet and needs
frequent naps. Hesitant and unadventurous. Comfortable at rest. Less than ordinary activity causes undue
dyspnoea, fatigue, or syncope and/or presyncope. Growth compromised. Poor appetite
Growth severely compromised. Poor appetite. Supplemental feeding. Less than ordinary activity causes undue
fatigue or syncope. Plus features of Class IIIa
Unable to carry out any physical activity without undue dyspnoea, fatigue or syncope, not interacting with family.
Syncope and/or right heart failure. Plus features of Class III
II
IIIa
IIIb
IV

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Pulmonary Circulation | April-June 2011 | Vol 1 | No 2283
Table 6: Pediatric functional classification for children aged 2–5 years
Class Children with pulmonary hypertension
I Asymptomatic, growing normally attending nursery/school regularly, no limitation of physical activity, playing
sports with his/her classmates
Slight limitation of physical activity, unduly dyspneoic and fatigued when playing with his/her classmates. Comfort-
able at rest. Continues to grow along own centiles. Nursery/school attendance 75% normal. No chest pain
Marked limitation of physical activity
Regression of learned physical activities. Not climbing stairs, reluctant to play with friends. Hesitant and unadven-
turous. Comfortable at rest. Less than ordinary activity (dressing) causes undue dyspnoea, fatigue, syncope and/or
presyncope or chest pain. Nursery/schooling compromised<50% normal attendance
Unable to attend nursery/school, but mobile at home. Wheelchair needed outside home. Growth compromised.
Poor appetite. Supplemental feeding. Less than ordinary activity causes undue fatigue, syncope or chest pain. Plus
features of Class IIIa)
Unable to carry out any physical activity without undue dyspnoea, fatigue, syncope or chest pain, unable to attend
school, wheelchair dependant, not interacting with friends. Syncope and/or right heart failure. Plus features of
Class III
II
IIIa
IIIb
IV
Lammers et al.: Functional class in children with PH
Table 7: Pediatric functional classification for children aged 5–16 years
ClassChildren with pulmonary hypertension
IAsymptomatic, growing along own centiles, attending school regularly, no limitation of physical activity, playing
sports with his/her classmates
Slight limitation of physical activity, unduly dyspnoeic and fatigued when playing when playing with his/her class-
mates. Comfortable at rest. Continues to grow along own centiles. School attendance 75% normal. No chest pain
Marked limitation of physical activity. No attempt at sports. Comfortable at rest. Less than ordinary activity
causes undue dyspnoea, fatigue, syncope or chest pain. Schooling compromised, <50% normal attendance
Unable to attend school, but mobile at home and interacting with friends. Wheelchair needed outside the home.
Growth compromised. Poor appetite. Supplemental feeding. Less than ordinary activity (dressing) causes undue
dyspnoea, fatigue, syncope and/or presyncope or chest pain. Plus features of Class IIIa)
Unable to carry out any physical activity without undue dyspnoea, fatigue, syncope or chest pain, unable to
attend school, wheelchair dependant, not interacting with friends. Syncope and/or right heart failure. Plus
features of Class III
II
IIIa
IIIb
IV
Table 8: What to expect of children in Functional Class I
AgeChildren with pulmonary hypertension, asymptomatic,
growing and developing normally, no limitation of physical activity
0-0.5 yearsGains head control and increases body tone from 0 to 3 months, then rolls over and has no head lag.
Sitting with support
0.5-1 yearMobile, sitting, grasping, starting to stand, crawling, playing
1-2 yearsStanding, starting to walk/walking, climbing
2-5 yearsAttending nursery/school regularly, playing games with his/her peers
6-15 yearsChildren attending school regularly, playing games with his/her peers
deal with children less than two years of age when the
most rapid physical development and maturation occurs.
Between the ages of two and five years it becomes easier
to communicate with the child, while by six years the
child is able to describe what they are able to do and how
they feel. Children become increasingly articulate with
age unless neurodevelopmental problems complicate
the assessment. The adult classification is appropriate
for those aged 16 years and over. Obviously, the patient is
placed in the functional class which best summarises their
clinical status and not all the descriptors of a functional
class need to be fulfilled in order to place the patient in a
particular class.
Bearing in mind that young children cannot tell one
how they feel, we sought to include objective indicators
such as whether or not the child is thriving, the need
for supplemental feeds and the record of school or
nursery attendance. The threshold for supplemental
feeding can vary with age and be influenced by co-
morbidities but the need for supplemental feeding is
usually an indication of disease severity. Parents are
often reluctant to acknowledge the extent to which their
child’s schooling is compromised by illness, realising that
absence from school reflects how sick their child really
is. The days of school missed due to illness provides
a measurable yardstick of illness severity. Therefore
the classification includes guidelines suggesting an
attendance of 75 or 50% or less to encourage them to give a
realistic answer.

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DESCRIPTORS OF FUNCTIONAL
CLASS
Functional Class I
This is defined, at all ages, as children with pulmonary
hypertension who are asymptomatic, growing and
developing normally and have no limitation of physical
activity. Table 8 outlines the most important features
seen in normal children in the five age groups used in
this classification and which we expect to find in children
categorised as functional Class I. The emphasis on motor
development in the first two years of life gradually shifts
to the child’s ability to interact with his/ her peers,
participate in sporting activities and go to nursery and
then school.
Functional Class II
At all ages children with pulmonary hypertension are
categorised as Functional Class II when they have only
a slight limitation of physical activity due to fatigue and
or dyspnoea but are comfortable at rest. Dyspnoea can
interrupt feeding in young children. At this stage of their
disease most children do not experience syncope or
presyncope but some children can do so while still having
a good exercise tolerance. During the first six months of
life they fall behind their developmental milestones but
continue to grow along their own centiles. Infants and
young children are readily fatigued and dyspnoeic when
playing. Beyond two years of age, it is important to assess
attendance at school or nursery, which should be at least
75% that of healthy children.
Functional Class IIIa
Functional Class IIIa is characterised by marked limitation
of physical activity. In addition to failing to achieve their
developmental milestones, children between the ages of
six months and two years may show regression of newly
learnt activities. Inactivity is noticeable, the child being
quiet and taking frequent naps. Less than ordinary activity
such as dressing is tiring and can cause dyspnoea. Children
frequently experience syncope and /or presyncope. Older
children can become withdrawn and less confident,
choosing to spend time with their families rather than
their friends. Growth is compromised and appetite is poor.
Nursery/school attendance is less than 50% of normal.
The parents frequently say that the child has required
excessive medical attention.
Functional Class IIIb
In addition to the features characteristic of Class IIIa,
children in Class IIIb often require supplemental feeding
by nasogastric tube or gastrostomy. Older children can
no longer go to school and although mobile at home they
need a wheelchair when venturing out of doors.
Functional Class IV
Children in Class IV are severely compromised and unable
to carry out any physical activity without fatigue and /or
dyspneoa. They are frequently syncopal, may complain of
chest pain and often become quiet and withdrawn. Signs
of right heart failure are frequently present, particularly
in teenagers.
LIMITATIONS OF THE PROPOSED
FUNCTIONAL CLASSIFICATION FOR
CHILDREN
The growth and development of healthy children varies
considerably and children with pulmonary hypertension
will be subject to the same innate variability, increasing
the difficulty facing the physician trying to assess
the functional impact of disease. In addition, many
children with pulmonary hypertension have multisystem
disorders that may impact heavily on functional capacity
independently of their pulmonary vascular disease.
We have tried to ensure that the features characteristic
of each functional class are objective but the opinion
of the parents can influence judgement, particularly
in a first child of young parents without extended
family support who maybe uncertain about how their
child differs from the normal. Some children are more
stoical than others. Some physicians will recommend
supplemental feeding more readily than others. Co-
morbidity further complicates assessment, primarily in
children with neurodevelopmental handicap who may
be syndromal. Despite all these and other caveats the
Pediatric Task Force of the Pulmonary Vascular Research
Institute believes that the proposed classification will
facilitate the management of children with pulmonary
hypertension.
CONCLUSIONS
The proposed Functional Classification of Pulmonary
Hypertension in Children follows the same pattern
and uses the same criteria as the adult classification,
modified appropriately for children of all ages. The
pediatric classification should therefore be understood
readily by adult physicians during transition from
pediatric to adult medical services, by all those caring
for children with pulmonary hypertension and by those
carrying out clinical research and designing clinical
trials. It should facilitate the development of treatment
algorithms for children, as has the adult classification
in older patients with pulmonary hypertension.[20] This
is a consensus document and we hope and expect that
it will be improved upon with experience.
Lammers et al.: Functional class in children with PH

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REFERENCES
1.?The?Criteria?Committee?of?the?New?York?Heart?Association.?Nomenclature?
and Criteria for Diagnosis of Diseases of the Heart and Great Vessels. 9th
ed. Boston ML, Brown and Co; 1994. p. 253-6.
Rich?S.?Primary?pulmonary?hypertension:?Executive?summary?from?the?
World?Symposium?on?Primary?Pulmonary?Hypertension.?Evian?FWHO,?
1998.
Gorkin?L,?Norvell?NK,?Rosen?RC,?Charles?E,?Shumaker?SA,?McIntyre?KM,?
et al. Assessment of quality of life as observed from the baseline data of
the?Studies?of?Left?Ventricular?Dysfunction?(SOLVD)?trial?quality-of-life?
substudy. Am J Cardiol 1993;71:1069-73.
Heo?S,?Moser?DK,?Riegel?B,?Hall?LA,?Christman?N.?Testing?the?
psychometric properties of the Minnesota Living with Heart Failure
questionnaire. Nurs Res 2005;54:265-72.
Naveiro-Rilo JC, Diez-Juarez DM, Romero Blanco A, Rebollo-Gutierrez
F, Rodriguez-Martinez A, Rodriguez-Garcia MA. Validation of the
Minnesota living with heart failure questionnaire in primary care. Rev
Esp?Cardiol?2010;63:1419-27.
Chua?R,?Keogh?AM,?Byth?K,?O’Loughlin?A.?Comparison?and?validation?of?
three measures of quality of life in patients with pulmonary hypertension.
Intern Med J 2006;36:705-10.
Zlupko?M,?Harhay?MO,?Gallop?R,?Shin?J,?Archer-Chicko?C,?Patel?R,?
et?al.?Evaluation?of?disease-specific?health-related?quality?of?life?in?patients?
with pulmonary arterial hypertension. Respir Med 2008;102:1431-8.
Brazier?JE,?Harper?R,?Jones?NM,?O’Cathain?A,?Thomas?KJ,?Usherwood?
T, et al. Validating the SF-36 health survey questionnaire: new outcome
measure for primary care. BMJ 1992;305:160-4.
Ware?JE?Jr,?Sherbourne?CD.?The?MOS?36-item?short-form?health?survey?(SF?
36).?I.?Conceptual?framework?and?item?selection.?Med?Care?1992;30:473-83.
Cenedese?E,?Speich?R,?Dorschner?L,?Ulrich?S,?Maggiorini?M,?Jenni?R,?
et al. Measurement of quality of life in pulmonary hypertension and its
significance.?Eur?Respir?J?2006;28:808-15.
2.?
3.?
4.?
5.
6.?
7.?
8.?
9.?
10.?
11.?McKenna?SP,?Ratcliffe?J,?Meads?DM,?Brazier?JE.?Development?and?
validation of a preference based measure derived from the Cambridge
Pulmonary?Hypertension?Outcome?Review?(CAMPHOR)?for?use?in?cost?
utility analyses. Health Qual Life Outcomes 2008;6:65.
Meads?DM,?McKenna?SP,?Doughty?N,?Das?C,?Gin-Sing?W,?Langley?J,?
et?al.?The?responsiveness?and?validity?of?the?CAMPHOR?Utility?Index.?
Eur?Respir?J?2008;32:1513-9.
Lollar?DJ,?Simeonsson?RJ.?Diagnosis?to?function:?classification?for?children?
and youths. J Dev Behav Pediatr 2005;26:323-30.
Ogonowski?J,?Kronk?R,?Rice?C,?Feldman?H.?Inter-rater?reliability?in?
assigning ICF codes to children with disabilities. Disabil Rehabil
2004;26:353-61.
Iglesias?MJ,?Cuttica?RJ,?Herrera?Calvo?M,?Micelotta?M,?Pringe?A,?Brusco?
MI. Design and validation of a new scale to assess the functional ability
in?children?with?juvenile?idiopathic?arthritis?(JIA).?Clin?Exp?Rheumatol?
2006;24:713-8.
Hedin?PJ,?McKenna?SP,?Meads?DM.?The?Rheumatoid?Arthritis?Quality?of?
Life?(RAQoL)?for?Sweden:?adaptation?and?validation.?Scand?J?Rheumatol?
2006;35:117-23.
Palmisani?E,?Solari?N,?Pistorio?A,?Ruperto?N,?Malattia?C,?Viola?S,?et?al.?
Agreement between physicians and parents in rating functional ability
of?children?with?juvenile?idiopathic?arthritis.?Pediatr?Rheumatol?Online?
J 2007;5:23.
Cabrera?ME,?Lough?MD,?Doershuk?CF,?Salvator?AE.?An?expanded?scoring?
system?including?an?index?of?nutritional?status?for?patients?with?cystic?
fibrosis.?Pediatr?Pulmonol?1994;18:199-205.
Turner-Bowker?DM,?Kosinski?M,?Zhao?J,?Saris-Baglama?RN.?SF-10?for?
Children™?A?User‘s?Guide.?Lincoln?(RI):?QualityMetric?Inc;?2003.
Galie?N,?Hoeper?MM,?Humbert?M,?Torbicki?A,?Vachiery?JL,?Barbera?JA,?
et al. Guidelines for the diagnosis and treatment of pulmonary
hypertension.?Eur?Respir?J?2009;34:1219-63.
12.?
13.?
14.?
15.?
16.?
17.?
18.?
19.?
20.?
Lammers et al.: Functional class in children with PH
Source of Support: Nil, Conflict of Interest: None declared.
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