DOI: 10.1111/j.1365-263X.2011.01153.

Salivary flow rate and oral findings in PraderWilli syndrome:

a case-control study
RONNAUG SAEVES1, HILDE NORDGARDEN1, KARI STORHAUG1, LEIV SANDVIK2
& IVAR ESPELID2
1

TAKO-centre, Lovisenberg Diakonale Hospital, Oslo, Norway, and 2Faculty of Dentistry, University of Oslo, Oslo, Norway

International Journal of Paediatric Dentistry 2012; 22:

2736
Background. PraderWilli syndrome (PWS) is a
rare complex multisystemic genetic disorder.
Aim. The objective of this study was to provide a
systematic assessment of whole saliva secretion
and oral manifestations associated with PWS.
Design. Fifty individuals (540 years) with PWS
and an age- and sex-matched control group were
included. Whole saliva was collected. All participants underwent an anamnestic interview. Radiological and dental clinical examinations were
carried out to identify hypodontia, dental caries,
enamel defects and gingival inflammation.
Results. Mean whole salivary flow rate was
0.12 0.11 mL min in the study group compared

Introduction

PraderWilli syndrome (PWS) is a complex

genetic disorder resulting from failed expression of paternally inherited genes on chromosome
15q1113.
The
majority
of
individuals with PWS (70%) have a paternally derived deletion of 15q1113, whilst
maternal disomy 15 (UPD) occurs in 25%
and the remaining 25% have imprinting
defects1,2. Clinical diagnostic criteria have
been developed3 but as clinically overlapping
disorders exist, the diagnosis must be confirmed by genetic testing4. The syndrome is
characterized by severe neonatal hypotonia,
endocrine disturbances, hyperphagia and
obesity, short stature, mild to moderate mental retardation, facial dysmorphology and oral
abnormalities. The syndrome is recognized
as the most common syndromal cause of
Correspondence to:
Ronnaug Saeves, TAKO-centre, Lovisenberg Diakonale
Hospital, Lovisenberggt 17, 0440 Oslo, Norway.
E-mail: ronnaug.saeves@tako.no

with 0.32 0.20 mL min in the control group

(P < 0.001). Hypodontia was significantly more
common in PWS (P < 0.001), and dental caries in
the age group >19 years was significantly lower in
PWS (P = 0.04) compared with the controls. There
was no significant difference in the prevalence of
dental caries in the primary dentition or in the
frequency of enamel defects in the permanent
dentition between the two groups. Median Gingival Index was significantly higher in the Prader
Willi group compared with the controls
(P = 0.02).
Conclusions. Low salivary flow is a consistent
finding in PWS. Nevertheless, despite dry
mouth and dietary challenges, dental caries is
not increased in Norwegian individuals with
PWS.

obesity in children. The population prevalence is estimated to be up to 1 52,0005 and

the gender ratio close to 1 15,6. Hypotonia
may lead to poor feeding in infancy. From 3
to 4 years of age, hyperphagia results in
obesity, if untreated. Obesity can be controlled by diet restrictions. Growth hormone
treatment improves growth, physical phenotype and body composition4,7.
Stephenson8 reported sticky saliva to be a
diagnostic indicator of PWS in neonates, and
thick, viscous saliva has been a consistent
finding in PWS911. Unstimulated salivary
flow rates in people with PWS have been
found to be approximately 20% of that in
controls9,10 and salivary ions and proteins are
present in increased amounts10. Oral findings,
including caries1215, enamel defects1518 and
poor oral hygiene14,15, are described in case
reports. However, a survey of 15 patients
with PWS identified more favourable oral
health than previous studies11. To our knowledge, no studies on oral findings in a PWS
population compared with a control group
are currently available.

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27

28

R. Saeves et al.

This study is part of a larger survey with

the aim to further characterize the intraoral
and dental features in individuals with PWS.
Specific aims in this study were to examine
whole salivary flow rates, dental caries,
enamel defects and gingival inflammation in
individuals with PWS and to compare the
findings with a control group without PWS.
Further aims were to examine oral health
and dietary habits in individuals with PWS
and to relate these as well as medical history
and treatment to their oral health status.
Material and methods

The study was carried out at the TAKO-centre, a national resource centre for oral health
in rare medical conditions (frequency of
<1 : 10,000), Lovisenberg Diakonale Hospital
(LDH) and at the Faculty of Dentistry, University of Oslo. The study protocol was
approved by the Regional Committee for
Medical Research Ethics. Establishment of a
biobank was approved, and informed consent
was obtained from all participants. If they
were under 18 years of age or were adults
with a guardian, informed consent was also
obtained from the parents or guardian. This
study used an observational, matched casecontrol design.
Study participants

Number of individuals

Participants were recruited through the Norwegian PWS association, although several of
the participants were already known to the
TAKO-centre. The association members (n =

12
10

95, >5 years) received written information,

designed for both children and adults,
describing the study. Fifty-four participants
with PWS aged 5 years and older from all
over the country responded. Two individuals
who initially agreed to participate later changed their minds owing to the long travelling
distance to the clinic. Two individuals were
excluded following a new genetic test, negative multiplex ligation-dependent probe amplification. The first letters were sent in
November 2006 and consultations took place
between January 2007 and April 2009. The
final study group comprised 50 individuals
(24F, 26M), mean age = 20.1 9.6 (range
5.640.9), Fig. 1. Confirmation of the genetic
diagnosis of PWS was obtained from three
different medical genetic centres. Five children (613 years) in the study group were
not ethnic Norwegians, as their parents came
from Africa, Asia and Eastern Europe.
The control group was age- and sex
matched to the PWS group, mean age =
20.5 10.2 (range 5.842.5). Twenty-four
individuals (518 years) were recruited from
the recall list at The Department of Pediatric
Dentistry, Faculty of Dentistry, University of
Oslo, and 26 adults were recruited among the
staff at LDH. The study was described on the
hospitals intranet and healthy volunteers in
appropriate gender and age groups were
invited to participate. All individuals with
PWS and 26 of the controls were examined at
the TAKO-centre, whereas the remaining 24
controls (518 years) were examined at the
Department of Pediatric Dentistry, University
of Oslo. All study participants received a small

Female
Male

8
6
4
2
0

510 year

1118 year

1929 year

3040 year

Ages groups
Fig. 1. Age and gender distribution of individuals with PraderWilli syndrome (n = 50).
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Oral health in PraderWilli syndrome

gift in appreciation of their willingness to participate.

Questionnaires
All participants with PWS underwent a thorough, structured anamnestic interview either
during the consultation or by telephone (if
parents did not attend the consultation). The
interview focused on general health, age at
diagnosis, sleep patterns, medications (e.g.,
use of growth hormone) and nutrition. Information about habits such as tooth grinding,
tooth-brushing routines and use of fluoride
was also obtained. Control individuals completed a questionnaire that had been modified
to exclude irrelevant anamnestic information.
Dental records (from 3 years of age), including radiographs, were recovered from the
Norwegian public dental health service for all
participants with PWS and for controls under
19 years of age. Adult controls lacking one or
more teeth were asked specifically whether
they had had dental extractions.
Clinical assessments
Study participants and controls were examined once (all before 1 p.m.) by the same
examiner, an experienced paediatric dentist
(RS). Body mass index (BMI) was calculated
on the basis of measured height and weight.
To define BMI categories (kg m2) for adults,
the sample was divided into three groups
[normal weight (2024.9), overweight (25
29.9) and obese (30)]. BMI criteria for the
age group 518 years of age (n = 24) were
age- and gender adjusted by comparing BMI
with the age- and gender-specific cut-off
values provided by the International Obesity
Task Force19.
All participants (and or their carers)
received written information about how to
avoid stimulation of saliva (not to eat, drink,
brush teeth or use chewing gum) during the
hour immediately prior to the examination.
The participants rested in a quiet room for a
few minutes before salivary tests were performed. Unstimulated whole saliva (UWS)
was collected for 5 min. Participants were
asked to swallow immediately before the

29

collecting period began. Samples were

obtained by requesting the participants to tilt
the head forward and expectorate all saliva
into a cup every minute without swallowing.
The collected saliva was weighed, and the salivary volumes were determined by weight
(1 g = 1 mL).
Clinical dental examination was carried out
by RS. Intraoral photographs and bitewings
were taken of all participants. Either conventional Kodak Insight dental films (size 0
IP-01, size 1 IP-12 and size 2 IP-21) or the
digital radiographic storage phosphor system,
Digora, was used. For practical reasons,
conventional radiographs were taken of
control persons at the Department of Pediatric
Dentistry. Some study participants tolerated
conventional but not digital bitewings. Panoramic X-ray images (Instrumentarium OC100,
Instrumentarium Dental, Tuusula, Finland)
were taken of all participants.
All participants were examined with regard
to number of teeth present, developmental
enamel defects, decayed (d), missing owing to
caries (m) and filled (f) surfaces in the primary (dmfs) and permanent (DMFS) dentitions. Also gingival inflammation (GI) was
recorded. Collected dental records were used
to classify absent teeth as either missing
owing to hypodontia (congenital absence of
one or more teeth) or missing owing to
extraction caused by infection or orthodontic
treatment. All permanent teeth were assessed
for developmental enamel defects on surface
level by one examiner (RS). The teeth were
air-dried 35 s prior to examination, and the
enamel defects were classified according to
the modified developmental defects of
enamel index20. Caries assessment was made
by RS using compressed air to dry the teeth,
a dental mirror, probe and optimal light.
A detailed caries diagnostic system, using five
severity grades (d1d5) based on written
description and photographs, was applied21.
The two incipient grades of caries d1 and d2
were noted to be enamel lesions and d3, d4
and d5 to be dentin lesions. Four examiners
(including RS) were calibrated with specially
designated software for examiner calibration
(DIL ver 1.21; University of Bergen, Bergen,
Norway). The calibration was based on judge-

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30

R. Saeves et al.

ment of 51 surfaces on radiographs of

extracted teeth. The inter- and intraobserver
agreement expressed weighted kappa22 were
0.68 and 0.79, respectively. Before evaluating
the radiographs of the study participants, the
bitewing films were scanned, and the digital
radiographs were exported from Digora in
two different contrasts. Then, all images were
imported to a PowerPoint presentation displaying one radiograph at a time. The blinded
examination of radiographs was carried out
by each observer separately under subdued
light and the same conditions. Caries registration was made using a dental diagram, and
each surface was coded as either no caries
(0), primary caries (15), secondary caries
(S1S5) or missing. Gingival condition was
scored according to the criteria of Gingival
Index (GI03)23. Before the examination, the
gingiva was dried with either a blast of air or
cotton rolls. The observations were dictated
by RS to a dental nurse who recorded the
scores in a diagram. Participants who presented with dental treatment needs were
treated at the TAKO-centre or referred to a
public dental health clinic.
Statistical analyses
The analyses and statistical comparisons were
carried out only when both PWS and control
had provided material for analysis. When
comparing UWS between the groups, a twosided t-test was used. A chi-square test was
used to compare frequencies between the two
groups. The Spearman correlation coefficient
was used to analyse the association between
two continuous variables. Interexaminer
agreement was measured using weighted
Cohens kappa. A two-sided MannWhitney
test was used to compare median dmfs DMFS
and median number of surfaces affected with
enamel defects in the two groups. When the
aim was to study the relationship between
several variables simultaneously on UWS or
DMFS, linear regression analyses were performed. The following variables were candidates for analysis; age, obesity, Gingival index
and use of fluoride. Among these, only variables significantly correlated with UWS and
DMFS were included in regression analyses.

Table 1. Characteristics of the study population n = 50.

Age 518 Age 1940
years, n
years, n
Total, n (%)
Gender
Female
Male
Genetic mechanisms
Del 15
UPD 15
Meth+
Age at diagnosis (years)
<1
15
>5
Supplementary diseases
Diabetes
Epilepsy
Heart disease
Birthweight (g)
<2500
2500
BMI
Normal weight
Overweight
Obese
Medication
Growth hormone (GH)*
Psycopharmaca
Antihistamins
Others

13
11

11
15

24 (48)
26 (52)

16
7
1

17
6
3

33 (66)
13 (26)
4 (8)

14
5
5

6
7
13

20 (40)
12 (24)
18 (36)

0
2
0

3
1
1

3 (6)
3 (6)
1 (2)

6
18

7
19

13 (26)
37 (74)

10
8
6

5
9
12

15 (30)
17 (34)
18 (36)

21
0
1
5

22
11
6
20

43
11
7
25

(86)
(22)
(14)
(50)

Del15, deletion of the paternal chromosome 15q1113; UPD15,

maternal uniparental disomy for chromosome 15; meth+, positive
methylation test. Body mass index (BMI) criteria were age- and
gender adjusted for individuals 518 years according to
International Obesity Task Force (IONT)19.
*Duration of GH treatment ranged between 6 and 192 months.
Mean duration was 69 months for individuals younger than
18 years and 74 months for individuals over 18 years.

Medications having dry mouth as a known side effect.

A significance level of 5% was used throughout. The statistical analysis was carried out
using the statistical software program. (SPSS
v. 18.0; SPSS Inc., Chicago, IL, USA).
Results

Medical information about the study population is presented in Table 1. Sixteen individuals (15 > 18 years) had a systemic disease
and or were treated with one or more medications having dry mouth as a known side
effect (Norwegian physicians desk reference,
Felleskatalogen, 2009 edition). Anamnestic
information revealed that adenotonsillectomy
had been performed in 18 individuals. Former
polysomnography in 27 individuals had led to

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Oral health in PraderWilli syndrome

Mean salivary flow rate ml/min

continuous positive airway pressure (CPAP)

treatment for five individuals. Thirteen participants who had not been previously examined for sleep disorder were referred to the
earnosethroat department at LDH. CPAP
treatment was subsequently recommended
for seven patients and adenotonsillectomy for
three. All controls were healthy, with normal
weight, and did not take any regular medication.
Based on the examiners clinical impression,
the saliva was generally more viscous in individuals in the PWS group compared with that
of the control group and also more viscous
than in individuals with reduced salivary
secretion because of other medical reasons.
Difficulties in compliance led to unsuccessful
saliva sampling in two patients (F 13, F 28
years). In four individuals, the UWS rate was
below detection limit, despite good cooperation during the sampling process. A clinical
inspection confirmed a totally dry mouth in
these individuals, and the observations were
entered as zero in the analyses. Mean
whole salivary flow rate in the PWS group
was 0.12 0.11 mL min and in the control
group 0.32 0.20 mL min (P < 0.001) (Fig. 2).
Hyposalivation (0.10 mL min)24 was found
in 26 individuals (54%) in the PWS group
compared with three individuals (6%) in the
control group. Thirty-two per cent of the
PWS group used medications that could affect
salivary flow. When excluding individuals
taking medication with a side effect of dry

0.6
0.5

Unstimulated whole saliva

PWS
Control

0.4
0.3
0.2
0.1
0

PWS

Control

Fig. 2. Quantification of whole salivary flow rate in Prader

Willi syndrome (n = 48) and Control (n = 48). Data are
expressed as mean and standard deviation (SD). The
difference between the groups was significant (P < 0.001).

31

mouth (supplementary diseases included), the

mean age of the PWS group was significantly
altered compared with that of the control
group (P = 0.01). After adjusting for age,
however, the mean UWS remained significantly different in the two groups
(P < 0.001). The study group included a high
number of obese individuals, whereas there
were none in the control group. Excluding
obese individuals did not significantly alter
the age and gender distribution between the
groups. Mean difference in saliva flow rate
between the PWS and control groups still
remained significant (P < 0.001).
Statistical analyses showed a significant
association between UWS and gender in the
control group (P = 0.047) and no association
between UWS and age. In the PWS group,
however, correlation was found between
UWS and age (r = 0.56; P < 0.001). No significant associations were found for obesity, gender or genotype in the study group.
Furthermore, mean salivary flow rate was significantly higher among individuals taking
medication compared with individuals without medication (P = 0.006). When multivariate linear regression analysis was performed
with age and medication as independent variables, only age retained a significant relation
with UWS rate (P = 0.02).
Hypodontia of a total of 24 teeth (20 premolars, one molar, one maxillary lateral incisor and two canines) was demonstrated in 12
individuals (24%) with PWS (one tooth in
six, two teeth in two, three teeth in two and
four teeth in two individuals). The corresponding number in the control group was
two missing teeth (two maxillary lateral incisors) in one individual. In the age group 518
years (PWS n = 24, control n = 24), hypodontia of a total of 11 teeth was observed in six
individuals (25%) with PWS but there was
no evidence of hypodontia in the equivalent
control group. The difference was significant
(P < 0.001) within both the young age group
(518 years) and across the study group as a
whole.
Statistical analyses of developmental enamel
defects (demarcated opacity, diffuse opacity
and hypoplasia) were performed on permanent teeth in the 618 years age group [PWS

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32

R. Saeves et al.

(n = 23) and in the control (n = 23)]. Eighteen individuals in the PWS group and 17 in
the control group had one or more surfaces
affected with enamel defects. The median
number of surfaces affected did not differ
between the study group (4.0, range 1.09.0)
and the control group (4.0, range 07.0).
Caries assessment of bitewing radiographs
on present teeth (from occlusal surface of the
second molar to distal surface of the canine
tooth) performed by four examiners is shown
in Table 2. The interexaminer agreement was
measured using weighted Cohens kappa
(0.51). Median scores of caries experience are
presented in Table 3. A total of 14 individuals
(5.513.2 years) in the PWS group and 14
individuals (5.811.9 years) in the control
group had primary teeth (220 teeth). When
enamel caries was included, six individuals
(43%) were caries-free in the PWS group and
five individuals (36%) in the control group.
When only dentine caries was included,
seven individuals (50%) in the PWS group
and eight individuals (57%) in the control
group were caries-free. There was no significant difference in the prevalence of dental

Table 2. Caries experience ds15, DS15 (mean and range) by

four examiners.

Examiners

PraderWilli
syndrome

Control

I
II
III
RS

1.3
1.0
3.9
2.0

1.7
1.8
6.5
3.7

(015)
(016)
(020)
(018)

(017)
(017)
(024)
(019)

Table 3. Median (interquartile range) values of caries

lesions.

Caries

PraderWilli
syndrome

dmfs15
dmfs35
DMFS15
DMFS15
DMFS35
DMFS35

1.0
0.5
6.0
12.5
1.0
6.0

(02.0)
(01.3)
(2.013.5)
(6.823.0)
(07.5)
(1.016.8)

Control
1.0
0
12.0
17.0
4.0
10.5

*Statistical significance (P < 0.05).

n = 14, n = 49, n = 26 > 19 years.

P-value

(04.0)
(02.0)
(2.518.5)
(12.731.0)
(011.0)
(6.020.8)

0.40
0.92
0.15
0.04*
0.35
0.20

Table 4. Association between DMFS and selected variables

in the PraderWilli syndrome group. Results from linear
regression analysis.
Variables

95% CI

P-value

Age
Obesity
Unstimulated whole saliva
Gingival inflammation
Fluoride

0.5
3.1
11.1
17.3
)1.6

0.00.9
)2.89.0
)19.141.3
7.926.7
)7.24.0

0.038*
0.30
0.46
0.001*
0.56

B, Unstandardized coefficient.
*Statistical significance (P < 0.05).

caries in the primary dentition between PWS

and control group.
Forty-nine individuals in each group had
permanent teeth (including thirteen with
mixed dentition). Five individuals in the PWS
group had dental crowns owing to extreme
tooth wear. Three of them had all their teeth
covered with dental crowns. One had four of
27 teeth crowned and another had crowns on
six of 25 teeth. Median DMFS15 in adults
(>19 years) was significantly lower in PWS
compared with that in the control group
(P = 0.04). Relative dmfs DMFS did not differ
significantly between the PWS and control
groups.
In the PWS group age, UWS, GI, obesity
and use of fluoride showed a significant association with caries experience (DMFS15). The
correlation between DMFS15 and age was
(r = 0.73; P < 0.001), between DMFS15 and
UWS (r = 0.31; P = 0.03), between DMFS15
and GI (r = 0.66; P < 0.001). Obesity was significantly associated with DMFS15 (P = 0.04)
and those who reported regular use of fluoride had less dental caries (P = 0.01). When
multivariate linear regression analysis was
performed with age, UWS, GI, obesity and
fluoride as independent variables, only age
and GI retained significant relations to dental
caries experience (Table 4). Median GI in the
PWS group was 0.29 (0.060.65) and 0.17
(0.040.25) in the control group (P = 0.02).
Daily tooth brushing was reported by 46 50
(92%) in the PWS group and 50 50 (100%)
in the control group. Twenty-eight individuals (56%) in each group reported regular use
of fluoride (tablets or rinsing fluid). Significantly, fewer individuals with PWS than

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Oral health in PraderWilli syndrome

controls reported regular use of dental floss or

toothpicks: 20% and 62%, respectively
(P < 0.001).
Discussion

This is the first study to systematically and

comprehensively examine oral findings in a
large group of individuals with a confirmed
genetic diagnosis of PWS (tooth wear and
malocclusion will be presented in other
reports) and to compare findings with those
of a control group. A total of 130 individuals
(70F, 60M) in Norway (065 years) have a
known diagnosis of PWS (clinically or genetically confirmed), and the participants in this
study represent about 50% of known individuals with PWS over 5 years of age in Norway.
Some of those who chose not to participate
explained that they were participating in
other studies at that time or that the travel
distance to the clinic was too great. The participants came from all over the country. The
age groups 518 years (24 individuals) and
>19 years (26 individuals) were equally represented, as were both sexes. The distribution
of different genetic subtypes found in this
study is similar to those found in other studies1,2,25, and there was no reason to expect
demographic bias based on age-range, gender
or genetic background.
Children with parents coming from Africa,
Asia and Eastern Europe (n = 5) were all
born in Norway where the public dental
health system offers free dental care to all
children.
The adults in the control group represented
different occupations (student, nurse, medical
doctor, physiotherapist and hospital orderly).
All study participants received written information about the examination. It is well
known that changes in routines or expectations are likely to trigger temper outbursts in
individuals with PWS26. The study group,
therefore, received information specifically
designed for them, including photographs
showing the clinic, the examiner and what
was going to happen. In a few cases, additional preparation was needed and the
patients received a second appointment. This
use of additional time and effort was, in our

33

opinion, crucial in facilitating the collection

of high-quality data.
Our study showed that individuals with
PWS have a low UWS secretion rate as compared with healthy, unmedicated age- and
sex-matched controls. This supports current
literature911. The mean whole salivary flow
rate was significantly higher in the age group
>19 years compared with the age group
518 years in the PWS group. As demonstrated in our controls, most studies have
found no relationship between salivary flow
rate and age27. Other investigators have, however, reported reduction with age28. Two individuals with PWS were unable to cooperate
sufficiently for the collection and calculation
of UWS rate (primarily owing to developmental delay). The possibility that other participants may have had difficulties following the
instructions cannot be excluded. A reduced
salivary flow correlated, however, well with
the clinical impression of dry mucous membranes in PWS. The secretion of fluid and proteins in the salivary glands are two distinct
processes. As the saliva present in individuals
with PWS appears extremely thick and sticky,
it seems likely that these mechanisms are
affected differently by the condition. It is possible that the protein secretion in salivary
glands may be functioning properly or even
be overexpressed, whereas fluid secretion is
reduced. Further research is necessary to
explore the salivary secretory mechanisms in
PWS.
Despite the reduced salivary secretion, the
caries experience (dmfs DMFS) was equal or
even significantly lower (age group >19
years) in individuals with PWS compared
with that in controls. More surfaces in the
PWS group were covered with dental crowns
(because of tooth wear), which protect
against caries lesions. This may partly explain
some of the difference in dental caries
between the two groups. The low caries experience in the PWS-group may also be
explained by the quality of information given
to families regarding diet restrictions (regular
meals and low sugar diet) and the importance
of good oral hygiene for individuals with
PWS. Most Norwegian PWS families have
been offered regular information courses by a

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R. Saeves et al.

multidisciplinary team including dentists,

since the 1980s, which may contribute to
explain the low caries experience. The
increased caries risk factors for families with
an immigrant background29 have probably
been reduced in Norway through advice and
follow-up in the dental health care system.
There may be some bias in the registrations,
as all study participants and controls were
examined once (duration of consultation
34 h) by a single examiner. More than one
examination (using two or more examiners)
was not possible because of the significant
travel distance to the clinic for many of the
participants. To reduce the limitations of the
single examiner, the bitewings were blinded
and four calibrated examiners assessed dental
caries. The results indicate that examiner RS
was not over- or under-registering. Bitewing
radiographs were obtained from all study participants (PWS and controls) but the quality
was not always optimal in the PWS group
owing to cooperation difficulties. This may
contribute to the relatively low Cohens
kappa value (0.51).
Hypodontia was found in 24% of the individuals in the PWS group. This is a significantly higher frequency than in the control
group (2%). The frequency is also high compared with earlier reports in a Scandinavian
population (4.510%)30,31. Hypodontia in
PWS has not been reported in the literature
previously. Clinical suspicion of hypodontia
was verified with panoramic X-ray and information from dental records from the public
dental health service for all participants in the
study group and the controls under 19 years
of age. Small study groups and missing data
from previous dental records and radiographs
from adult controls could be a confounding
factor.
In the present study, the frequency of disturbances in enamel mineralization in permanent teeth was not significantly different in
the PWS group compared with that in the
control group. Individuals older than 19 years
were excluded from the analysis owing to
dental restorations and crowns and consequent problems registering disturbances in
enamel. Our findings are in line with those
in Belgian individuals with PWS and do not

support earlier reports (mainly case reports)

of high frequencies of enamel defects12,1518.
Severe malnutrition in infancy and early
childhood may be associated with enamel
hypoplasia or hypomineralization in permanent teeth32,33. Today, individuals with PWS
are usually diagnosed in early childhood, and
indeed, about 60% of the individuals in the
518 years age group were diagnosed before
the age of 1. Consequently, optimal feeding
was initiated during early infancy, and this
may explain the low frequency of enamel
defects. The prevalence of enamel defects in
the primary dentition is significantly influenced by birth weight34. In the present study,
13 individuals (26%) in the study population
had low birth weight (<2500 g), and high
prevalence of enamel defects would therefore be expected. Owing to tooth wear in the
primary dentition, registration of disturbances
in enamel mineralization in primary teeth
was not possible to perform in the present
study.
Significantly higher GI index in the PWS
group may indicate poor oral hygiene and
high levels of plaque in the study group. Gingival inflammation and GI index, however,
may also be affected by medications and
mouth breathing. Employing both a GI and a
plaque index would therefore have provided
a more comprehensive basis for evaluating
oral hygiene. Cooperation in the PraderWilli
group was a limiting factor, and we used the
GI index to give a view over time, rather
than a snapshot. Despite these limitations, the
findings of the present study suggest that oral
hygiene may be poor amongst individuals
with PWS. This supports earlier case
reports19,21,22. Most adults with PWS manage
their oral hygiene independently. The results
may be poor; yet, they do not permit parents
or guardians to help them. Poor oral hygiene
and low salivary flow rate are known risk factors for caries. Nevertheless, our study supports previous findings of low caries
experience in individuals with PWS18. Individuals with PWS in Norway eat a regular,
restricted and low sugar diet and are regularly
followed up in the dental health care system.
This may contribute to the low caries experience in the PWS group.

 2011 The Authors

International Journal of Paediatric Dentistry  2011 BSPD, IAPD and Blackwell Publishing Ltd

Oral health in PraderWilli syndrome

What this paper adds

d This paper describes oral heath in 50 individuals with
PWS with a novel finding of hypodontia.
d Whole salivary flow rate in individuals with PWS is
low, independent of supplementary diseases or medication with an adverse effect of dry mouth.
d In spite of low salivary flow, individuals with PWS do
not have increased caries prevalence.
Why this paper is important for paediatric
dentists
d Predictability is important for individuals with PWS,
and it is important that paediatric dentists know about
the syndrome.
d Owing to low salivary flow rate and higher risk of gingival inflammation, prevention with regular professional cleaning as part of a comprehensive oral
preventive programme is particularly important for
individuals with PWS.

8
9

10
11

12

13

14

Acknowledgements

The authors thank the Norwegian PWS association, all those who participated in the
study and their families and all our co-operators at the Faculty of Dentistry, University of
Oslo. This study was supported by a grant
from the Norwegian Foundation of Health
and Rehabilitation, through the Norwegian
PWS association.

15

16
17

18

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