1) Transitioning patients with special health care needs from pediatric to adult oral healthcare can be challenging. The goal is to help patients reach their maximum potential through developmentally appropriate healthcare services that continue from adolescence to adulthood. 2) Most respondents said the predominant transition age was between 18-20 years for patients with SHCNs. The major barrier to transition was a lack of availability of general dentists and specialists trained to treat patients with SHCNs. 3) Educational programs for dentists need to include strategies and protocols for effectively transitioning adolescent patients with SHCNs to appropriate adult oral healthcare.

1. Special Care for Special Patients
2018-2 장애아동치과학
소아치과 1년차
Mohammad

2. 새별의 파워포인
트
37. Cerebral Palsy Diagnosis and Management
Moyer, V. A. (2003)

3. 1. Cerebral Palsy Diagnosis and Management
• primary abnormality of movement and posture
secondary to a non-progressive lesion of a
developing brain.
Definition
Epidemiology
• Cerebral palsy has an estimated prevalence rate of
1 to 2.3 per 1000 live births.
• It is well recognized that very low birth weight
infants are at risk for cerebral palsy (birth
weight<1500g)
• Among infants with a birth weight of >2500g, the
occurrence rate is less than 1 in 1000.

4. Cerebral Palsy Subtypes and Features
Airway
Feeding
Hearing
Vision
Speech
• Spasticity is the most commonly occurring
feature of cerebral palsy, accounting for
approximately 75% of the children affected.
• A small percentage of children with CP
demonstrate extrapyramidal features,
including combinations of athetosis, chorea,
dystonia, and ataxia
• Quadriplegic CP, 25% of
the affected children
requiring total care.
• Hemiplegic CP , Children
with hemiplegic learn to
walk by 2 years of age
• Diplegia CP, 50% of
affected children learn to
walk by 3 years of age
• Extrapyramidal CP,
athetoid and dystonic
posturing. Sever Speech
impairments ,characterize
by dysarthria, drooling,
poor initiation of
vocalization.

5. Risk Factors and Diagnosis of Cerebral Palsy
Risk
factors
• CP unclear in more than 50% of cases. The leading predictors
of CP include mental retardation and birth weight <1500 g.
• The death of one twin in utero increases the risk for CP in the
surviving twin by more than 100 times.
• CP in term infants of normal birth weight is typically related
to prenatal factors, such as , prenatal strokes.
• The existence of periventricular leukomalacia is the strongest
independent risk factor for development of cerebral pals
Diagnosis
• Early signs include delay in meeting motor milestones,
toe walking, persistent fisting, seizures, irritability and
poor suck.
• An interdisciplinary approach to making the diagnosis
is recommended and may necessitate input from the
ophthalmologist, audiologist, radiologist, neurologist,
geneticist, and child development teams.

6. Treatment of Cerebral Palsy
• well-child care and health maintenance,
provision of anticipatory guidance,
developmental assessments, and
immunizations, child’s respiratory status.
• Forced Use or Constraint-Induced
Movement Therapy, effective technique
to increase the use of the affected arm in
a child with hemiplegic cerebral palsy.
• Interdisciplinary Care, guide the families
in interpreting the scientific evidence
currently available and to avoid
unsubstantiated interventions.
• Feeding and Nutrition, Early nasogastric
or gastrostomy tube feeding lead to
increased weight and improved immune
competence
.
• Adaptive Equipment, improve
musculoskeletal alignment, prom
ote bone mineralization
• Sleep Disturbances, medications
such as Melatonin and Clonidine
positively affect the sleep-wake
cycle.
• Management of Spastic CP
Intramuscular injections botulinum
toxin and phenol.

7. 새별의 파워포인
트
38. Unmet Dental Needs and
Barriers to Care for Children
with Significant Special Health
Care Needs
Nelson, Linda P., et al (2011)

8. Unmet Dental Needs and Barriers to care for (CSHCN)
• DEFINITION OF (CSHCN): those who have increased risk for a chronic
physical, developmental, behavioral, or emotional condition require health.
Barriers
to dental
care of
(cshcn)
Aspirin
Most reports found Children with craniofacial anomalies had twice
as many unmet needs and children with cystic fibrosis had fewer unmet
needs. Children with cerebral palsy, autism, developmental delay, and
Down syndrome had more aversions to dental treatment.
(1) the primary medical care system
(2) the child’s parents
(3) the child himself
(4) the dentist
(5) payment for dental care

9. Methods and Results
predominant racial group was Caucasian.Most
children with parents who highly educated,
above average incomes. 20% of children in
sample had unmet denta lcare.

10. Methods and Results
Children with craniofacial disorders and cerebral palsy experienced the greatest
number of barriers to care , both environmental and non-environmental, and
children with cystic fibrosis, or hemophilia were impacted the least. Children with
autism and Down syndrome impacted frequently by non-environmental barriers.

11. Conclusion
• Despite having private insurance and parents with higher-than-average
incomes and education levels 20% of the children in our study had
unmet dental needs.
• Children with cystic fibrosis had significantly fewer unmet needs while
children with craniofacial anomalies had significantly more.
• Environmental barriers to care were greater for families who were more
geographically isolated and those who faced linguistic and economic
barriers.

12. 새별의 파워포인
트
39. Dental Management of
Amelogenesis Imperfecta Patients: A
Primer on Genotype- phenotype
Correlations Ng, F. K., & Messer, L. B. (2009)

13. Amelogenesis Imperfecta (AI)
• “A group of conditions, genomic in origin, which affect the structure and
appearance of enamel associated with morphologic or biochemical
changes elsewhere in the body..
• PREVALENCE OF (AI)
ranging from 1:700 in a Swedish community to 1:14,000 in the USA
• CLASSIFICATION OF (AI)
4 phenotypes: (1) hypoplastic; (2) hypomaturation; (3) hypocalcified; and
(4) hypoplastic-hypomaturation
• The purpose of this paper was to provide a narrative review of the
current literature on amelogenesis imperfecta, particularly regarding
recent advances in the identification of candidate genes and the patterns
of inheritance

14. Molecular Etiologies.
• Inheritance patterns and current known gene mutations resulting
in AI– X-linked amelogenesis imperfecta (XLAI) and associated
phenotypes.
• The X-linked forms of AI resulting from mutations in the AMELX
gene located on chromosome Xp22 account for about 5% of all AI
cases.
• 15 AMELX mutations are responsible for the hypoplastic,
hypomaturation,and dysmineralization-hypoplastic phenotypes.
• A major 5-Kb deletion was associated with mottled and opaque-white
enamel.
• Five mutations in the C-terminus coding region all resulted in smooth
hypoplastic phenotypes.
• Mutations in 5 candidate genes are known to cause AI. Mutations in the
AMELX gene coding for the amelogenin protein are responsible for X-linked AI
.Mutations in the ENAM, KLK-4, and MMP-20 gene cause AI with autosomal
pattern of inheritance. Mutations in the distal-less 3 homeobox (DLX3) gene
are associated with hypomaturation-hypoplaastic AI and trichodento-osseous
syndrome.

15. • Autosomal dominant amelogenesis imperfecta (ADAI) and associa
ted phenotypes.
• 7 Mutations in ENAM located on chromosome 4 are associated with
generalized (smooth) hypoplastic and local hypoplastic phenotypes.
• Autosomal recessive amelogenesis imperfecta (ARAI) and associat
ed phenotypes
• In an autosomal recessive inheritance pattern, an individual requires 2
abnormal alleles of the disease gene to manifest the condition.
• Mutations in the MMP-20 (enamelysin) and KLK-4 (kallikrein-4) genes
are associated with hypomaturation ARAI.
 Dental management of affected children and adolescents
 Casein phosphopeptide-amorphous calcium phosphate (CPP-ACP)
and fluoride products
• Topical fluoride and CPP-ACP products applied to dysmineralized ena
mel may aid remineralization, reducing tooth sensitivity to thermal sti
muli and reducing caries risk

16. 새별의 파워포인
트
Dental management
 Bonding with adhesive restorations
bonding is possible in most cases. Typical etch patterns were absent in the
smooth hypoplastic sample, putatively due to abnormalities in prism structure
 Intracoronal restorations and Full coverage restorations
• Intracoronal restorations used to improve esthetics and/or restore teeth with
posteruptive breakdown due to caries or wear.
• Full coverage restorations are required commonly for posterior teeth
particularly in hypocalcification phenotypes
• Full coverage restorations. In the primary and young permanent dentition are
the most effective method to manage tooth sensitivity and poor esthetics,
Conclusions
• Pediatric dentists play an important role in the early identification and manage
ment of amelogenesis imperfecta (AI) patients.
• Maintaining the health of the primary dentition and monitoring the developm
ent of the permanent dentition are both important, aiming to maintain vertical
dimension, reduce sensitivity, and improve esthetics.

17. 새별의 파워포인
트
40. Oral Health Care for
Children With Developmental
Disabilitie
Batshaw, M. L. (1992)

18. Bisphosphonates
• Children with more severe conditions and from low-income families are
particularly at risk with high dental needs and poor access to care.
• children with developmental disabilities are more likely to have unmet
dental needs. The reasons include frequent use of medicine high in sugar.
• Medications to manage seizures may cause gingival overgrowth.
Hyperactivity disorder medications (amphetamine), can result in xerostomia.
 BARRIERS TO ACCESS TO CARE
Oral Health Care for Children With Developmental
Disabilities
Nelson et al7 divided barriers from the parents’ perspective into 2 categories e
nvironmental and nonenvironmental
• environmental barriers included the inability to find a dentist who would
treat their child and to find staff members were not nervous about caring
for a child with special needs. Included are financial barriers .
• Nonenvironmental barriers included the child’s perceived fear of the dentist,
the child’s inability to cooperate for dental exams.

19. • Children Who Do Not Take Food or Fluids Orally
• Depend on gastrostomy tube feedings are at significantly increased risk of
poor oral health, particularly a build-up of tartar and subsequent
gingivitis, The lack of oral experiences and severe motor impairments can
result in hyperactive bite and gag reflexes.
• Children With Functional Limitations in Self-care
• children with intellectual disability and neurodevelopmental disabilities are
compromised because of delays in motor and cognitive abilities. It is not
uncommon for children with intellectual disability to have behavioral
problems in the traditional dental office setting.
ORAL HEALTH CONDITIONS ASSOCIATED WITH DEVE
LOPMENTAL DISABILITIES
• Children With Oral Aversion
Children with autism spectrum disorders often have oral aversions and
characterized by hypersensitivities to textures, smells, tast, colors, thus
signicantly limiting the foods they will eat. Resultant nutritional
deficiencies can affect oral health.

20. INTERPROFESSIONAL PARTNERSHIPS
Oral Health Education in Medical and Residency Training
• A survey of pediatricians, only 36% said they had received previous
training in oral health, with 13% reporting training during medical
school. The majority of survey respondents recognized the importance
of evaluating their patients for dental caries, but only 41% felt that their
ability to identify caries was very good or excellent.
Identification of Children Who Require Referral for Dental Treatment
• Dentistry recommends that all children visit a pediatric dentist when
the first tooth comes in, usually between 6 and 12 months of age (“first
visit by first birthday”). Children with certain craniofacial abnormalities a
nd other at-risk conditions should have their initial pediatric dental visit
during the first 6 months of life.
Safe Use of Procedural Sedation and Analgesia/Anesthesia
• General anesthesia is primarily provided in a hospital setting where the
necessary precautions can be taken to minimize complications. Guide
lines describe the types of precautions that should be taken for mild
and moderate levels of sedation in the dental or pediatric office.

21. SUGGESTIONS FOR PEDIATRICIANS
 Learn how to assess dental and periodontal health in children with special h
ealth care needs.
 Recognize risk factors that contribute to poor oral health with the use of the
Oral Health Risk Assessment Tool.
 Advocate for oral care for children with developmental disabilities.
 Develop collaborations with dental partners to coordinate care for children
with developmental disabilities.

22. 새별의 파워포인
트
41. Facilitating the transition of
patients with special health care
needs from pediatric to adult
oral health care
Nowak, A. J., Casamassimo, (2010)

23. Background
• Transitioning from a pediatric health care system to an adult centered
health care system can be challenging for young adults with SHCNs and
their families.
• The transition, however, frequently does not occur. If it does, it often is
poorly organized and does not meet the goals of a collaborative..
• The goal of transitions with SHCNs is to help them reach their
maximum potential through developmentally appropriate health care
services that continue from adolescence to adulthood.
• Most pediatricians said that transitioning should begin between the
ages 18 and 20 years for patients with and without SHCNs.

24. Methods And Result
Transitioning
of patients
• The predominant transition
age for patients with SHCNs
was between 18 and 20 year
s. For patients without SHCNs
, it was between 15 and 20
years of age.

25. Barriers to and services in support of Transition
• Two thirds or more
of respondents said
that availability of ge
neral dentists and
specialists was the
major barrier to
transitioning
adolescents with
SHCNs
• one-third of respond
ents said that
insura-nce issues.

26. Conclusion
• Most responding dentists helped adolescents with and without SHCNs
make the transition into adult care, but the major barrier was the
availability of general dentists and specialists.
• Educational programs offered in dental school and through specialty and
continuing education should include transitioning strategies, protocols
and experiences, so that adolescent patients with SHCNs can be
transitioned effectively and appropriately to adult oral health care.