Special Health Care Needs Flashcards
(181 cards)
1
Q
Definition of special health care needs
A
Special health care needs include any physical, developmental, mental, sensory, behavioral, cognitive, or emotional impairment or limiting condition that requires medical management, health care intervention, and/or use of specialized services or programs.
The condition may be developmental or acquired and may cause limitations in performing daily self-maintenance activities or substantial limitations in a major life activity.
2
Q
6 “critical steps” to ensuring the successful transition from a pediatric-centered to an adult-centered dental home for individuals with SHCN
A
- All patients with SHCN have a health care provider that take responsibility of the transition
- Identify the core competencies required to provide care for SHCN patients
- To develop a portable, accessible, medical summary to facilitate the transition
- To develop an up-to-date detailed transition plan
- To ensure that the same standards for primary and preventive care are applied
- To ensure that affordable, comprehensive, continuous health insurance is available to patients with SHCN
3
Q
There is agreement in the literature that specific transition planning should begin between what ages?
A
14 and 16 years
4
Q
Barriers to care for SHCN
A
- Dentistry has been found to be the most common category of unmet health care for children with SHCN.
- Only 10% of surveyed general dentist reported that they treat patients with SHCN often, while 70% reported that they rarely or never treat patients with SHCN.
- 95% of pediatric dentist reported to a survey from the AAPD that they routinely provided care to patients with SHCN.
- There are approximately 10.2 million children with SHCN under 19 years of age (representing 14% of all U.S. children).
- When patients reach adulthood, their oral needs may go beyond the scope of pediatric dentist’s expertise.
- Oral health care for adults with SHCN is often difficult to access because lack of trained providers.
- A 2015 survey of senior dental students noted that the provision of oral health care to patients with SHCN was among the top 4 topics in which they were least prepared.
5
Q
Preventive strategies
A
- Education of parents
- Dietary counseling
- Sealants and topical fluoride
- Interim therapeutic restorations
- Toothbrushes with modifications
6
Q
Pathophysiology of Lesch-Nyhan syndrome
A
- Inherited as an x-linked trait that affects how the body builds and breaks down purines.
- Individuals affects with this syndrome are missing or are severely lacking an enzyme called hypoxanthine guanine phosphoribosyltransferase (HGP) –> without this enzyme high levels of uric acid build up in the body
- LNS is produced by the mutation in the HPRT1 gene that codes for the enzyme HGP
7
Q
Clinical manifestation/evaluation of Lesch-Nyhan
A
- Intellectual disability (mild to severe)
- Speech articulation problems
- Extensor spasm of the trunk
- Self-mutilative behavior (begins with the eruption of teeth)
- Dramatic and extremely rapid loss of tissue –> hallmark
8
Q
Dental findings for Lesch-Nyhan
A
- Self mutilating behavior usually involving the lips and fingers
- Tophi on pinna (due to high uric acid)
9
Q
Epidemiology of Hunter and Hurler Syndrome
A
- Hurler syndrome, also known as mucopolysaccharidosis type I (MPS I), is a genetic disorder that results in the buildup of glycosaminoglycans due to a deficiency of alpha-L iduronidase (this enzyme is responsible for the degradation of mucopolysaccharides in lysosomes).
- MPS II –> Hunter syndrome
- The overall frequency is 1 per 100,000 births, the MPS as a whole have a frequency of 1 in every 25,000 births.
10
Q
Clinical evaluation/manifestations for Hurler Syndrome (MPS I)
A
- Abnormal bones in the spine
- Claw hand **
- Cloudy corneas
- Deafness
- Halted growth
- Heart valve problems
- Joint disease, including stiffness
- Mental cognitive delay that gets worse over time
- Thick, coarse facial features with low nasal bridge
11
Q
Dental findings for Hunter/Hurler
A
- Progressive coarsening of facial features
- Papular skin lesions
- Macrocephaly
- Short necks
- Widely spaced teeth and enlarged tongue
12
Q
Clinical evaluation/manifestation for Sturge-Weber syndrome
A
- Manifested at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face. **
- The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nerve, just under the surface of the face.
- There is also malformation of blood vessels in the pia mater overlying the brain on the same side of the head as the birthmark.
- This causes calcification f tissue and loss of nerve cells in the cerebral cortex. - Neurological symptoms include seizures that begin in infancy and may worsen with age. There may be muscle weakness on the same side. **
- Some children will have developmental delays and mental retardation; about 50% will have glaucoma.
13
Q
Dental findings for Sturge-Weber Syndrome
A
- Oral manifestations occur in about 40% of patients.
- Bluish-red lesion that blanches on pressure.
- Mostly on the buccal mucosa and lips, less on maxillary gingiva and palate.
- Floor of the mouth, tongue and mandibular gingiva are rarely involved.
- The lesions resemble a vascular hyperplasia or large tumor-like mass.
- Early eruption of teeth reported from increased vascularity.
- Dilantin hyperplasia is common (not any more!)
- Oral hygiene problems in cognitive delay patients.
14
Q
Special considerations for Sturge-Weber Syndrome
A
- Major problem is hemorrhage from the angiomas **
- Hospitalization with complete workup is recommended
- For GA, oral intubation may be safer
- Special hygiene aids for patients with intellectual disability
15
Q
Pathophysiology of neurofibromatosis
A
- The neurofibromatoses are genetic disorders that cause tumors to grow in the nervous system.
- The tumors begin in the supporting cells that make up the nerves and the myelin sheath.
- Although many affected persons inherit the disorder, between 30 and 50 percent of new cases arise spontaneously through mutation (change) in an individual’s genes. Once this change has taken place, the mutant gene can be passed on to succeeding generations.
16
Q
Classification of neurofibromatosis
A
- The disorders is classified as neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis, a type that was once considered to be a variation of NF2. ***
- NF1 is the more common type of the neurofibromatoses. ***
- NF2 is less common and is characterized by slow-growing tumors on the eighth cranial nerves. The tumors cause pressure damage to neighboring nerves.
- The distinctive feature of schwannomatosis is the development of multiple schwannomas (except on the vestibular branch of the 8th cranial nerve). The dominant symptom is pain.
17
Q
Dental findings for neurofibromatosis
A
- Enlarged fungiform papilla
- Oral neurofibromas
- Hyperplasia of soft and oral tissues associated with bony hypoplasia
- Malpositioned teeth
- Intrabony lesions
- Wide inferior alveolar canal
18
Q
Clinical evaluation/findings of Rubenstein-Taybi
A
- Broadening of the thumbs and big toes
- Constipation
- Excess hair on body (hirsutism)
- Heart defects possibly requiring surgery ***
- Intellectual disability
- Seizures
- Short stature that is noticeable after birth
- Slow development of cognitive skills
- Slow development of motor skills accompanied by low muscle tone
19
Q
Dental findings for Rubenstein-Taybi
A
- Small mouth
- Thin upper lip
- Retrognathia
- High arched narrow palate
- Malpositioned crowded teeth
- Talon cusp **
- High caries rate
20
Q
Clinical evaluation/findings for Treacher Collins Syndrome
A
(OMENS)
- Orbit - abnormal orbital size and position (O0-O3)
- Mandible - small mandible and short ramus; ramus abnormally short and abnormally shaped; complete absence of the ramus, glenoid fossa and TMJ (M0-M3) ***
- Ear - malposition of the lobule with absent auricle (E0-E3)
- Facial nerve - All branches of the facial nerve are affected (N0-N3)
- Soft tissue - Severe tissue or muscle deficiency (S0-S3)
21
Q
Dental findings for Treacher Collins Syndrome
A
- Hypoplasia of the facial bones (underdevelopment of the mandible and zygomatic arch)
- Small mandible —> malocclusion ***
- Cleft palate
- Dental anomalies (60% of patients): tooth agenesis, enamel malformation, ectopic eruption of the first molars
- High arch palate
- Lip incompetency (small mandible)
22
Q
Clinical finding/evaluation for Pierre Robin Sequence
A
- Airway obstruction —> if not appropriate treatment: hypoxia, cor pulmonale, failure to thrive, and cerebral impairment
- The most important medical problems are difficulties in breathing and feeding ***
23
Q
Dental findings for Pierre Robin Sequence
A
- Micrognathia and retrognathia
- Cleft palate
- Glossoptosis (often associated with airway obstruction): in many cases the tongue is not larger than normal, but because of the small mandible, the tongue is large for the airway ***
24
Q
Clinical evaluation/findings for Rett Syndrome
A
• An infant with Rett syndrome usually has normal development for the first 6-18 months
• Symptoms can range from mild to severe.
• Symptoms may include:
- Floppy arms and legs — frequently the first sign **
- Apraxia: a disorder of the brain and nervous system in which a person is unable to perform tasks or movements when asked even though:
• The request or command is understood
• They are willing to perform the task
• The muscles needed to perform the task work properly
• The task may have already been learned
- Breathing problems — tend to worsen with stress
- Excessive saliva and drooling **
- Intellectual disabilities and learning difficulties **
• Assessing cognitive skills in those with Rett syndrome, however, is difficult because of the speech and hand motion abnormalities
- Scoliosis
- Shaky, unsteady or stiff gait, toe walking; about 50% are not ambulatory
- Seizures (up to 80%)
- Loss of purposeful hand movements; for example- the grasp used to pick up small objects is replaced by repetitive hand motions like hand wringing or constant placement of hands in mouth
- Loss of social engagement ***
- Ongoing, sever constipation and gastro-esophageal reflux (GERD)
- Poor circulation
- Severe language development problems
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Most frequent oral habits associated with Rett Syndrome
* Digit/hand sucking and/or biting
* Bruxism
* Mouth breathing
* Drooling
* Tongue thrusting
26
Additional dental findings for Rett Syndrome
* Gingivitis is the most common alteration of soft tissues.
* Dental attrition is frequently present in these children.
* Palatal shelving could be seen, probably related to the digit/hand sucking and/or biting habits.
* A high prevalence of anterior open bite.
* No associations with anomalies of tooth number, size, form, structure, or eruption.
27
What is Beckwith-Wiedemann Syndrome?
Congenital growth disorder that causes large body size, large organs, and other symptoms
28
Signs of Beckwith-Wiedemann Syndrome
```
• A ridge on the forehead caused by premature closure of the bones (metopic ridge) ***
• Enlarged fontanelle (soft spot)
• Enlarged kidneys, liver, and spleen
• Large size (90th percentile)
• Low blood sugar (hypoglycemia)
• Macroglossia ***
• Midline abdominal wall defects
- Omphalocele
- Umbilical hernia
- Diastasis recti
• External ear (pinna) abnormalities and low-set ears
```
29
Beckwith-Wiedemann Syndrome and Cancer
* While most children with BWS do not develop cancer, these individuals do have a significantly increased risk of certain childhood cancers, particularly Wilms’ tumor (nephroblastoma) and hepatoblastoma
* Children with BWS are most at risk during early childhood and should receive cancer screening during this time
30
Dental findings for Beckwith-Wiedemann Syndrome
• Macroglossia is very common (>90%) and a prominent feature of BWS
• Infants with BWS and macroglossia typically cannot fully close their mouth in front of their large tongue, causing it to protrude out
• Macroglossia in BWS becomes less noticeable with age and often requires no treatment; bit it does cause problems for some children
• In severe cases, macroglossia should be evaluated by a craniofacial team for surgical intervention
• The best time to perform the surgery for a large tongue is not known.
- Some surgeons recommend performing the surgery between 3 and 6 months of age.
31
What is Crouzon Syndrome (Craniofacial Dysostosis)?
* Crouzon syndrome is characterized by the premature fusion of certain skull bones (craniosynostosis) ***
* This early fusion prevents the skull from growing normally and affects the shape of the head and face
32
Characteristics of Crouzon Syndrome
• Brachycephaly, which results in the appearance of a short and broad head.
• Exophthalmos (bulging eyes due to shallow eye sockets after early fusion of surrounding bones)
• Hypertelorism (greater than normal distance between the eyes)
• External strabismus
• Psittichorhina (beak-like nose)
• Hypoplasia maxilla (insufficient growth of the mid face)
• Crouzon syndrome is also associated with PDA (patent ductus arteriosus) and aortic coarctation
• Relative mandibular prognathism (chin appears to protrude despite normal growth of mandible)
• Cleft lip and palate
• Low-set ears
- Ear canal malformations are extremely common, generally resulting in some hearing loss
• People with Crouzon syndrome are usually of normal intelligence ***
33
Dental findings for Crouzon Syndrome
* Possible cleft lip and palate
* Narrow/high-arched palate
* Posterior bilateral crossbite
* Hypodontia
* Crowding of teeth
* Due to maxillary hypoplasia, Crouzon patients generally have a considerable negative OJ and subsequently cannot chew using their incisors
34
What is Ellis-Van Creveld Syndrome (Chondroectodermal dysplasia)?
Ellis-van Creveld syndrome is a rare genetic disorder that affects bone growth.
35
Anomalies associated with Ellis-van Creveld syndrome
```
• Post-axial polydactyly (extra fingers)
• Congenital heart defects
- Most commonly an atrial septal defect producing a common atrium, occurring in 60% of affected individuals
• Short-limbed dwarfism
• Short ribs
• Cleft lip and palate
• Various dental abnormalities
• Pre-natal tooth eruption
• Fingernail dysplasia
• Malformation of the wrist bones
- Fusion of the hamate and capitate bones
```
36
Dental findings for Ellis-van Creveld Syndrome (Chondroectodermal dysplasia)
* Cleft lip and palate
* Natal teeth
* Presence of multiple frenulum and abnormal attachments ***
* Abnormally shaped teeth
* Microdontia
* Congenitally missing teeth
37
Prominent characteristics of Fragile X (Martin Bell Syndrome)
* Elongated face (vertical maxillary excess) ***
* High-arched palate (related to above)
* Large or protruding ears
* Flat feet
* Larger testes (macroorchidism)
* Low muscle tone and flexible joints
38
Behavior problems associated with Fragile X Syndrome
```
• Delay in crawling, walking, or twisting
• Hand clapping or hand biting
• Hyperactive or impulsive behavior
• Intellectual disability
• Seizure disorder
• Speech and language delay
• Atypical social development
- Shyness
- Limited eye contact
- Memory problems
- Difficulty with facial recognition
```
39
Dental findings for Fragile X (Martin Bell Syndrome)
* Study compared the oral findings in fragile X syndrome individuals to those of normal age-matched patients
* Low caries rate (decayed, missing and filled = 12.3) and minimal intraoral soft tissue disease
* Rate of malocclusion, as determined by the first permanent molar classification, was not significantly different from that of matched subjects
* Fragile X subjects had a significantly higher occurrence of malocclusion as compared to matched subjects using cross-bite and open-bite as criteria
* The fragile X subjects also demonstrated significantly more severe occlusal wear of their teeth than the matched sample
40
What is Marfan syndrome?
A connective tissue disorder that affects the following:
• Skeletal system:
- Tall with long, thin arms and legs
- Arachnodactyly- “Spider-like” fingers
- Wingspan is much greater than their height
- A chest that sinks in or sticks out—funnel chest (pectus excavatum) or pigeon breast (pectus carinatum)
- Hypermobile joints
* Spine that curves to one side (scoliosis)
* Thin, narrow face
* Cardiovascular system:
- Mitral valve prolapse
- The aorta may stretch or become weak (aortic dilation or aortic aneurysm)
41
Dental findings in Marfan Syndrome
* High, arched palates ***
* Narrow jaws ***
* Crowding ***
* Small lower jaw (micrognathia) ***
There is limited research regarding specific management of the orthodontic problems commonly seen in people with Marfan Syndrome
42
Prader-Willi Syndrome symptoms
• PWS is present from birth
• Newborns are small for gestational age
• Have problems sucking and swallowing and often do not gain weight well
• May seem floppy and feel like a “rag doll” when held
• Often have a weak cry
• Obesity:
- Affected children have an intense craving for food and will do almost anything to get it
- This results in uncontrollable weight gain and morbid obesity
- Morbid obesity may lead to lung failure, which includes low blood oxygen levels, right sided heart failure and even death
• Facial features:
- “Almond-shaped” eyes
- Small, downturned mouth
• Irregular areas of skin that look like bands, stripes, or lines
• Limb (skeletal) abnormalities:
- Very small hands and feet compared to the body
43
Dental findings for Prader-Willi Syndrome
* Soft tooth enamel
* Thick, sticky saliva
* Poor oral hygiene
* Bruxism
44
Clinical findings/evaluation for tuberous sclerosis (a neurocutaneous syndrome)
* Hypomelanotic macules
* Facial angiofibromas
* Subungal fibromas
45
Dental findings for tuberous sclerosis (a neurocutaneous syndrome)
* Enamel hypoplasia/pitted enamel — seen in 90%
* Gingival fibromas
* Gingival hyperplasia secondary to seizure meds
46
What is Sotos Syndrome?
Also known as “Cerebral Gigantism”
47
Features of Sotos Syndrome
1) Characteristic facial features:
• Long, narrow face
• Unusually large head (height and/or head circumference >=2 SD above the mean)
• High forehead
• Flushed cheeks
• Small, pointed chin
• Down-slanting palpebral fissures
• This facial appearance is most notable in early childhood. Affected infants and children tend to grow quickly; they are significantly taller than their peers. Adult height is usually in the normal range, however.
• The cardinal features for this condition are characteristic facial features, learning disability, and overgrowth. ***
2) Intellectual delay and behavioral issues (ADHD, compulsive behaviors, phobias, obsessions). Learning disabilities can range from mild to very severe (requiring lifelong care).
3) Problems with speech and language
4) Hypotonia [may delay development of motor skills such as sitting and crawlng]
5) May have scholiosis, seizures, heart or kidney defects, hearing loss, and problems with vision
6) Neonatal jaundice
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Dental findings for Sotos Syndrome
* Premature eruption of teeth ***
* Second premolar hypodontia
* Enamel defects and excessive tooth wear
* Mandibular prognathism
* High, vaulted palate
* Note: in many cases, fingernails were brittle and thin and patients had sparse frontoparietal hair in early childhood.
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Clinical evaluation/findings for hemihyperplasia (hemihypertrophy)
* Cases with hemihypertrophy not fulfilling criteria of complicated hemihypertrophies are grouped under isolated hemihypertrophy or hemihyperplasia (so this excludes Beckwith-Weidemann syndrome, etc.)
* Usually non-progressive and the body disproportion does not change. Bone age may or may not be increased on the hypertrophied side.
* Some cases have other features like nevi, capillary haemangiomas, and hypertrichosis.
* Intellectual disability may be present.
* No specific laboratory abnormalities.
* Viscera (kidney) on the hypertrophied side may be enlarged)
* Risk for neoplasm is about 5% (most common is Wilms tumor — nephroblastoma)
50
Dental findings for hemihyperplasia (hemihypertrophy)
* Dental issues seen in hemifacial hyperplasia
* Hemimandibular hypertrophy: excessive unilateral growth of the mandible occurs in individuals who seem metabolically normal. Unknown etiology. Usually between ages 15-20 yo. Formerly called condylar hyperplasia. **
51
Pathophysiology of Oculo-auriculo-vertebral syndrome (Goldenhar; hemifacial microsomia)
* Now believed that Goldenhar and HFM part of spectrum of issues, now referred to as the Oculo-auriculo-vertebral Spectrum.
* Seems to occur around day 30-45 of gestation; may be caused by an early bleed in utero — hemorrhage in the area of the 1st and 2nd branchial arches
* Most cases are sporadic and not familial
52
Clinical evaluation/findings for oculo-auriculo-vertebral spectrum
* Can be as mild as microtia of one ear or be very severe and include renal, cardiac, and skeletal issues. If bilateral, can resemble Treacher Collins.
* Facial asymmetry.
* Hypoplasia of the facial musculature.
* Frontal bossing.
* Ear - microtia, pinna displaced anteriorly and inferiority, preauricular tags and/or pits, middle ear anomaly with variable deafness.
* Eye - palpebral fissure is somewhat lowered on the affected side; epibulbar dermoids (growth on sclera)
* May have normal intelligence (esp with Goldenhar), but one study found that infants with OAV were at increased risk for neurodevelopmental problems.
* Spinal: hemivertebrae or hypoplasia of vertebrae, usually cervical.
53
Dental findings for oculo-auriculo-vertebral syndrome
* Oral- diminished to absent parotid secretion, anomalies in function or structure of the tongue ***
* Hypoplasia of malar, maxillary, and/or mandibular region
* Cleft palate with or without cleft lip often seen
54
Dental findings for fetal alcohol syndrome
* Smooth philtrum, thin upper lip
* Mild micrognathia, hypoplasia mandible
* Flat mid-face
* Minor ear abnormalities
* Cleft palate often
55
Clinical findings/evaluation for Cornelia De Lange Syndrome
* Low birth weight
* Intellectual disability and growth delay
* Hirutism
* Microcephaly
* Intellectual delay (range from mild to severe) and learning disabilities
* GERD (85% of patients)
* Behavioral issues (self-injury, compulsive repetition, autistic-like behaviors)***
* Prominent facial features: thin eyebrows that often meet at the midline (synophrys), long eyelashes, short upturned nose, thin downturned lips, low-set ears, coloboma of eyes, short neck, high forehead.
* High palate or cleft palate.
* Limb differences: small hands and feet, in curved fifth fingers (clinodactyly), partial joining of the second and third toes, proximally placed thumbs, and upper limb abnormalities, including missing fingers, hands or forearms.
56
Dental findings for Cornelia De Lange Syndrome
* Micrognathia
* Delayed eruption
* Prognathic maxilla with protruded maxillary anterior teeth
* Class II div I
* Cleft palate
57
Clinical evaluation/findings for Apert Syndrome
* Craniosynostosis
* Syndactyly (of hard and soft tissues)
* Mid-face hypoplasia
* Cervical spine fusion
58
Dental findings for Apert Syndrome
* Tooth agenesis (esp of canines)
* Cleft palate/bifid uvula; often see “Byzantine cleft”, which is not a true cleft, but a deep crevice.
* Enamel hypocalcification/hypoplasia
* Supernumerary teeth
* Ectopic eruption of maxillary 1st molars
* Maxillary/mid-face hypoplasia
* Class III malocclusion
* Lateral palatal swellings
* Delayed eruption
* Shovel-shaped incisors
59
Clinical evaluation/findings for Turner Syndrome
* Swollen hands and feet
* Wide and webbed neck
* Absent or incomplete development at puberty, including sparse pubic hair and small breasts***
* Broad, flat chest shaped like a shield
* Drooping eyelids
* Dry eyes
* Infertility
* Absent menstruation
* Short height
60
Dental findings for Turner Syndrome
* Micrognathia
* Premature eruption of permanent molars
* High arched palate
* Malocclusion
61
Clinical evaluation/findings for Noonan syndrome
* Delayed puberty
* Down-slanting or wide-set eyes
* Hearing loss
* Low-set or abnormally shaped ears
* Mild intellectual disability (only in about 25% of cases)
* Sagging eyelids (ptosis)
* Small penis
* Short stature
* Undescended testicles
* Unusal chest shape (usually a sunken chest called pectus excavatum)
* Webbed and short-appearing neck
* Pulmonary stenosis
* Other possible heart defects are hypertrophic cardiomyopathy, atrial septum defect, ventricular septum defect, septal hypertrophy or a combination of all of these defects.**
62
Dental findings for Noonan syndrome
* 1/3 of children with Noonan syndrome have malpositioned teeth.
* Delayed eruption and atypical eruption sequence.
63
Dental findings for Lowe Syndrome (Oculo-cerebro-renal syndrome)
* Periodontal disease with severe bone loss (due to defective inositol phosphate metabolism)***
* Impaction of permanent teeth
* Taurodontism***
* Underdevelopment of the maxilla and mandible
64
Clinical evaluation/findings for Klinefelters
* Only in males***
* Many men who have Klinefelter syndrome do not have obvious symptoms.
* Weak muscles
* Slow motor development
* Sparse body hair
* Enlarged breasts
* Wide hips
* Small testicles
* Penis may not reach adult size
* Voice may not be as deep
* Infertility, but can have normal sex life
* Some have language and learning problems
65
Dental findings in Klinefelters
* Maxillary and mandibular prognathism
* Permanent tooth crowns larger than usual
* Taurodontism***
* No recognizable features apparent at birth
66
Pathophysiology of Down Syndrome
A chromosomal condition characterized by the presence of an extra copy of genetic material on chromosome 21 (trisomy 21); 47 chromosomes instead of the usual 46
67
Common characteristics and physical signs of Down Syndrome
* Intellectual disability
* Stunted growth
* Atypical fingertips
* Flexible ligaments
* Decreased muscle tone at birth
* Brachycephaly
* Shortened extremities
* Oval palate
* Small teeth
* Macroglossia
* Low set and rounded ears
* Flattened nose
* Short neck
* Congenital heart disease***
* Separated joints between the bones of the skull (sutures)
* Single crease in the palm of the hand
* Upward slanting eyes
* Wide, short hands with short fingers
* White spots on the colored part of the eye (Brushfield spots)
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Dental findings for Down Syndrome
* Microgenia (small chin)
* Macroglossia
* Microdontia (small teeth)
* Delayed eruption of teeth
* Small conical roots
* Hypodontia or partial anodontia
* Oval palate
* Hypoplasia of mid-facial region
69
Dental findings for Cri-du-chat (French term “Cat-cry” or “Call of the cat”)
* Micrognathia
* High palate
* Variable malocclusion (more commonly anterior open-bite)
* Cleft lip and palate
* Most patients also present with perioral muscle relaxation with labial incompetence and short philtrum
* These patients suffer dental erosions provoked by gastro-esophageal reflux and attritions because of intense day-and-night bruxism
70
Clinical evaluation/findings for achondroplasia
* Abnormal hand appearance with persistent space between the long and ring fingers
* Bowed legs
* Decreased muscle tone
* Disproportionately large head-to-body size difference
* Prominent forehead (frontal bossing)
* Shortened arms and legs (especially the upper arm and thigh)
* Short stature (significantly below the average height for a person of the same age and sex)
* Spinal stenosis
* Spine curvatures called hypnosis and lordosis
71
Dental findings for achondroplasia
* Depressed nasal bridge
* Maxillary hypoplasia
* Disturbed genesis of the teeth
* Disturbed development (partially formed)
* Disturbed eruption
* Shape abnormalities
72
What is attention deficit hyperactivity disorder (ADHD)?
* A neurobehavioral disease that is defined by persistent and maladaptive symptoms of hyperactivity/impulsivity and inattention.
* Also associated with several comorbid conditions and disorders such as mood disorders, disruptive behavior, and learning disabilities.
* Although diagnosed as a categorical disorder, ADHD may actually represent the extreme end of a normal continuum for traits of attention, inhibition, and the regulation of motor activity.
* Current advances in cognitive neuroscience and behavioral and molecular genetics have provide evidence that ADHD is a complex neurobiological disorder.
73
Prevalence of attention deficit hyperactivity disorder (ADHD)
* Estimated to be 3% to 5% of school-age children. However, more recent studies place the prevalence closer to 7% to 8%.
* Prevalence varies with risk factors: age, male gender, chronic health problems, family dysfunction, low socio-economical status, and urban living.
74
Rate of occurrence of ADHD in the US vs the world
The disorder is found in all the countries in the world with similar rates than those found in the United States.
75
Region of the brain and neurotransmitter implicated with attention deficit hyperactivity disorder (ADHD)
Pre-frontal cortex and dopamine
76
Treatment for ADHD
* For most patients with ADHD, stimulants remain the first choice for medication management.
* Research has continued to suggest that osmotic-release oral systems (OROS) methylphenidate decreased ADHD symptoms through the day and is associated with a great adherence (one day dose).
* Dexmethylphenidate extended release (XR) and transdermal methylphenidate also offer this benefit.
* Comorbid anxiety was not found to affect stimulant efficacy in a recent study.
* Some children may not respond to stimulant medications, or may not be able to tolerate the stimulant medications due to side effects (for example loss of appetite and trouble with sleep onset).
* Recent evidence has shown that treatment with stimulants in childhood modestly reduced expected height and weight.
* Modafinil and reboxetine have both shown some promise in the treatment of ADHD.
* Non-stimulants approved by the FDA are atomoxetine (SNIR), guanfacine, and clonidine.
77
Names of drugs for ADHD
```
• Methylphenidate class:
- Immediate release: Ritalin
- Intermediate acting: Ritalin SR
- Extended release: Concerta
• Dexmethylphenidate class:
- Focalin and Focalin SR
• Amphetamines:
- Adderral and Adderral XR
```
Mechanism of action: Blocks uptake of dopamine and norephrinephrine
78
Etiology of intellectual disability/developmental delay
* Organic causes
* Genetic disorders
* Environment
79
Symptoms of intellectual disability/developmental delay
* ID is not a disease entity in itself but rather a symptom of CNS disorder
* Many systems involved
* Physical or psychological abnormalities
80
Classification of intellectual disability/developmental delay
```
The Stanford-Binet IQ test — not longer legally used as determiner of intelligence provided rough equivalents for the categories of intellectual disability which are still in use:
• Borderline 68-73
• Mild 52-67
• Moderate 36-51
• Severe 21-35
• Profound <21
```
81
Dental considerations for intellectual disability/developmental delay
* Wide variations of behavior and level of understanding between mild and profound intellectual disability.
* Degree of management difficulty proportional to the level of cognitive functioning.
* Informed consent.
* Medical history.
* Good communication: slow - simple and repetitive
* Pay attention to what the patient is trying to tell you
* Oral and IV sedation
82
What is cerebral palsy?
* CP is a non-progressive disorder resulting from malfunction of the motor centers and pathways of the brain.
* CP is characterized by paralysis, weakness, in coordination or other aberrations of motor function.
* Most commonly occurs during prenatal or perinatal period.
* 1.5-3.6 cases/1000 children.
* No cure, but many patients enjoy near normal lives if their neurological problems are properly managed.
83
Classification of cerebral palsy
* Spastic-tightness: + common —> lack of control of muscle movements ***
* Dyskinetic: slow, involuntary movements, hypotonia
* Ataxic: uncoordinated voluntary movements
84
Medications for cerebral palsy
* Antiparkinsonian drugs: levodopa
* Anticonvulsants
* Antidepressants, botox, antidopaminergic drugs
85
Clinical manifestations of cerebral palsy
* ID 60%
* Seizures 30%
* Sensory deficits 35%
* Speech disorders
* Joint contracture
* Microcephaly
* GI problems
* Spasticity
86
Dental considerations for cerebral palsy
* Environment
* Do not force limbs into unnatural positions
* Considering treating in wheel chair
* Stabilize patient’s head during treatment
* Consider support for limbs
* Use mouth prop
* Keep patient’s back slightly elevated to minimize swallowing difficulties
* User rubber dam and minimize time in the chair
87
Common dental/oral findings in Cerebral Palsy
* Periodontal disease
* Dental caries/poor oral hygiene
* Malocclusions —> anterior open bite, class II
* Bruxism
* Increased dental erosions
* Hyperactive bite reflex
* Increase gag reflex
* Increased drooling
88
Incidence rate of congenital heart disease
Incidence rate of approximately 8 to 10 cases/1,000 live births
89
Risk factors associated with congenital heart disease
* Maternal rubella
* Diabetes
* Alcoholism
* Irradiation
* Drugs such as thalidomide, phenytoin, warfarin
90
What is congenital heart disease?
* Most lesions occur individually; several form major components of syndromes such as Down (trisomy 21) and Turner (XO chromosome).
* Turbulent blood flow is caused by structural abnormalities of the heart anatomy and presents clinically as an audible murmur.
* CHD can be classified into cyanosis (shunt or stenotic) and acyanotic lesions depending on the clinical presentation.
91
No cyanotic lesions (congenital heart disease)
No cyanotic lesions are characterized by a connection between the systemic and pulmonary circulations or stenosis of either circulation (left to right shunts).
The most common:
1) Atrial septal defect (ASD) ***
2) Ventricular septal defect (VSD)
3) Patent ductus arteriosus (PDA): caused by failure of closure of the ductus connecting the pulmonary artery with the aorta (normally closes soon after birth)
4) Coarctation of the aorta
5) Aortic stenosis
6) Pulmonary stenosis
92
Cyanotic lesions (congenital heart disease)
Cyanotic lesions: all cyanotic conditions exhibit RIGHT to LEFT shunting of desaturated blood. Infants with mild cyanosis may be pink at rest but become more blue during crying or physical exertion. Children with cyanotic defects are at significant risk for desaturation during general anesthesia.
The most common cyanotic lesions are:
1) Tetrology of Fallot (VSD, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy)
2) Transposition of the great vessels
3) Tricuspid atresia ***
4) ** No corrected acyanotic congenital heart defects
93
Tricuspid atresia
* Hypoplastic right ventricle
* Restrictive ventricular septal defect (VSD)
* Sub-pulmonary narrowing
* Atrial septal defect
94
Implications of congenital heart defect for the practice of pediatric dentistry
Infective endocarditis prophylaxis
95
Infective endocarditis mortality rates
```
• 100% fatal if not treated
• With antibiotic treatment, fatality rate:
- NVE (native valve)
• Streptococcus < 10%
• Staphylococcus 25-40%
• Gram negatives 75-83%
• Fungi 50-60%
- Late PVE (prosthetic valve) 30-53%
```
96
What is infective endocarditis?
* Bacteremia resulting from daily activities is much more likely to cause IE than bacteremia associated with a dental procedure
* Only an extremely small number of cases, if any, of IE might be prevented by antibiotic prophylaxis, even if prophylaxis is 100% effective
97
Summary of the changes from the 1997 guidelines from AHA
* A significant reduction in the numbers and types of Adrian conditions for which antibiotic prophylaxis is recommended (only those with the most serious adverse outcomes of IE)
* A significant increase in the types of dental procedures recommended for prophylaxis in at-risk individuals (almost all dental procedures)
* A minor modification in the timing of antibiotic administration (30-60 minutes prior)
98
Cardiac conditions associated with the highest risk of adverse outcome from endocarditis for which prophylaxis with dental procedures is recommended
• Prosthetic cardiac valve
• Previous infective endocarditis
• Cardiac transplantation recipients who develop cardiac valvulopathy
• Congenital heart disease (CHD) [except for the conditions listed below, AB prophylaxis is no longer recommended for any other form of CHD]:
- Unrepaired cyanotic CHD including those with palliative shunts and conduits
- Completely repaired CHD with prosthetic material or device either by surgery or catheter intervention during rest 6 months after procedure (to allow time for endothelial covering of the material)
- Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device which inhibits endothelialization
99
Conditions for which prophylaxis is no longer recommended (1997 moderate risk conditions)
* Mitral valve prolapse with regurgitation
* Rheumatic heart disease and other types of acquired valvular heart disease (e.g. systemic lupus erythematosus)
* Ventricular septal defect
* Atrial septal defect
* Hypertrophic cardiomyopathy
100
Dental procedures for which endocarditis prophylaxis is recommended
• All dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa.
• Except the following:
- Routine anesthetic injections through non-infected tissue
- Taking dental radiographs
- Placement of removable prosthodontics or orthodontic appliances
- Adjustment of orthodontic appliances
- Shedding of deciduous teeth and bleeding from trauma to the lips or oral mucosa
101
Regimens for a dental procedure
• Situation — oral
- Agent: Amoxicillin
- Adults: 2 gm
- Children: 50mg/kg
• Situation — unable to take oral medication
- Agent: Ampicillin or cephazolin (adults 2 mg IM or IV, 50 mg/kg IM or IV); cephtriaxone (adults 1 gm IM or IV, children 50 mg/kg IM or IV)
• Situation — allergic to penicillin or ampicillin oral
- Agent: Cephalexin (adults 2 gm, children 50 mg/kg); Clindamycin (adults 600 mg, children 20 mg/kg); Azithromycin (adults 500 mg, children 15 mg/kg); Clarithromycin (adults 500 mg; children 15 mg/kg)
• Situation — allergic to penicillins or ampicillin and unable to take oral medication
- Cephazolin or cephtriaxone (adults 1 gm IM or IV, children 50 mg/kg IM or IV); clindamycin phosphate (adults 600 mg IM or IV, children 20 mg/kg IM or IV)
102
Example of infectious lung diseases
* Tuberculosis
| * Pneumonia
103
Example of neoplasia lung diseases
* Lung cancer
| * Mesotelioma
104
Example of restrictive lung diseases
* Asbestos
* Silicosis
* Asthma
105
Example of obstructive lung diseases
* COPD
| * Asthma
106
Example of inflammatory lung diseases
* Asthma
| * Bronchiolitis
107
Example of congenital lung diseases
• Hypoplasia
108
Definition of asthma
* A chronic inflammatory disease characterized by reversible airway obstruction.
* The disorder is characterized by bronchial edema caused by hypersensitivity which results in airway narrowing
109
Diagnosing asthma: patient checklist
* Troublesome cough, particularly at night
* Awakened by coughing
* Coughing or wheezing after physical activity
* Breathing problems during particular seasons
* Coughing, wheezing, or chest tightness after exposure to allergens
* Colds that last more than 10 days
* Relief when medication is used
110
Diagnosing asthma: physical examination
* Sounds of wheezing during normal breathing
* Hyperexpansion of the thorax
* Increased nasal secretions or nasal polyps
* Atopic dermatitis, eczema, or other allergic skin conditions
111
Category of drugs used by asthmatics
* Anti-inflammatory agents (1st agents)
| * Bronchodilators - B2 agonists (2nd agents)
112
Anti-inflammatory agents for asthmatics (1st agents)
* Corticosteroid inhalants (fluticasone —> Advair)
* Leukotriene receptor inhibitors: Zafirlukast (Accolate), Montekulast (Singulair, Zyflo)
* Mast cell stabilizers (Cromolyn)
113
Bronchodilators - B2 Agonists used by asthmatics
* Albuterol (Proventil, Ventolin)
* Metaproterenol (Alupent, Metaprel)
* Terbutaline (Bricanyl)
* Isoetharine (Bronkometer, Bronkosol), Isoproterernol (Isuprel, Medihaler-ISO)
* Bitolterol (Tornalate), Pirbuterol (Maxair)
* Salmeterol (Serevent) only long acting
114
Oral manifestations of asthma
• Altered naso-respiratory function (mouth breathing) results in increased upper anterior and total anterior facial height, higher palatal vaults, greater overjets, higher prevalence of crossbite
• Increased prevalence of caries with moderate to severe asthma ***
- B2 agonist decrease salivary flow by 20-35%, associated with increased # of lactobacilli
• Inhaled corticosteroids and candidiasis
115
Oral/dental considerations for asthma
* Optimal asthma control is desirable before dental treatment.
* Aspirin as well as other non-steroidal anti-inflammatory medications should be avoided in aspirin-sensitivity persons.
* Approximately between 10% to 28% of children with asthma may be intolerant to aspirin.
* Opiates may be used with caution (respiratory depression).
* Macro life antibiotics (erythromycin) may elevate theophylline levels. **
116
Is there a relationship between asthma and dental caries?
Five hypothesized links give credence to the asthma-caries association:
• Epidemiological evidence (children with asthma have higher caries experience) —> conclusion: lack of positive association between asthma and caries
• Pathological changes in the amount and composition of the saliva in children —> Inhalers have a relatively low pH (5.5) and contain carcinogenic sweeteners as carriers. Patients often misused them by placing the medication in the mouth rather than in the airway —> potential carcinogenic insult
• Salivary gland hypofunction secondary with medications used to treat asthma
- Ryberg et al found that children with asthma who were exposed to daily inhalation of B2-selective adrenergic bronchodilator (Albuterol for example) exhibited a decreased salivary flow rate and diminished protein output when compared with controls.
- Bjerkeborn et al found that caries prevalence in patients with severe asthma (more than 10 attacks per year) who had received long-term medication was not significantly different from that of patients with moderate asthma who were not receiving long-term medication therapy.
• Considering the large number of contributing factors that may be operating in the asthma-caries association, it is hardly that his relationship has been difficult to clinicians to interpret.
• The specific intensity of medications regimens may change, and these regiments may be modified in response to an evolving clinical scenario (oral environment).
• On the basis of the literature, it would appear that there is not strong evidence suggesting that a causal link exist between caries and asthma.
117
Three “P’s” for Diabetes Mellitis
• A constellation of abnormalities caused by lack of insulin and characterized by:
- Polyuria
- Polydipsia
- Polyphagia
• Weight loss or weight gain, hyperglycemia, glycosuria, ketosis, acidosis, and coma
118
Diabetes mellitus vs diabetes insipidus
Diabetes insipidus is a pituitary disorder due to a deficiency of vasopressin (ADH) that is characterized by the excretion of excessive quantities of urine. It is unrelated to diabetes mellitus.
119
Diabetes mellitus classification
Previously used terms of “insulin dependent” or “juvenile” diabetes, and “non-insulin dependent” or “adult diabetes” have been replaced by:
- Type I diabetes (absolute insulin deficiency,
- Type II diabetes (relative, progressive insulin deficiency; non-autoimmune etiology)
- Type III diabetes: caused by other identifiable etiology; genetic defect for example
- Type IV: gestational (occurrence only during pregnancy); at increased
- Impaired glucose homeostasis (prediabetes); moderate elevation of blood glucose; have high risk of developing diabetes
120
Where is insulin produced?
* One of the two principle hormones produced and secreted by the pancreas (the other is glucagon)
* Insulin is produced by the beta cells and glucagon is produced by the alpha cells in the islets of Langerhans
121
Function of insulin ***
Insulin promotes the entry of glucose into most cells of the body and thus controls the rate of carbohydrate metabolism; glucose can then be used immediately for energy or it will be stored in the form of glycogen or fat.
122
How is insulin is produced?***
Insulin undergoes extensive post translational modification along the production pathway. Production and secretion are largely independent; prepared insulin is stored awaiting secretion. Both C-peptide and mature insulin are biologically active.
123
Effects of insulin on glucose uptake and metabolism**
Insulin binds to its receptor, which starts may protein activation cascades. These include translocation of Glut-4 transporter to the plasma membrane and influx of glucose, glycogen synthesis, glycolysis, and triglyceride.
124
Symptoms of type I diabetes
```
Cardinal symptoms:
• Polydipsia (increased thirst)
• Polyuria (increased urination)
• Polyphagia (increased hunger)
• Weight loss
• Loss of strength
```
```
Other symptoms:
• Skin infections
• Irritability
• Headache
• Drowsiness
• Malaise
• Dry mouth
```
125
Laboratory diagnosis for diabetes mellitus
• Random blood glucose (by itself is not reliable for diagnosis but can provide information on real time blood glucose or monitoring purposes)
• Normal fasting blood glucose is < 100 mg/dl
• Diagnostic criteria for DM:
- Fasting blood glucose >= 126 mg/dl, or,
- Symptoms of DM (polyuria, polydipsia, et. loss), plus casual blood glucose that is >= 200 mg/dl, or,
- Two hour post-prandial blood glucose >= 200 mg/dl
- Glycosylated hemoglobin (HbA1c > 7%; measures blood glucose past 2-3 months)
• Urinalysis - not reliable
126
HbA1c test scores (ranges)
• Action suggested:
- 9.0 to 14.0 --> mean blood glucose 215-380 mg/dL or 11.9-21.1 mmol/L
• Good:
- 7.0 to 8.0 --> mean blood glucose 150-180 mg/dL or 8.2-10.0 mmol/L
• Excellent:
- 4.0, 5.0, 6.0 --> mean blood glucose 50, 80, 115 mg/dL or 2.6, 4.7, 6.3 mmol/L
127
Complications of diabetes mellitus (more common and severe with type I)
```
1) Macrovascular (large vessel) disease
(accelerated atherosclerosis)
• Heart: CHD, congestive heart failure
• Cerebrovascular: stroke
• Peripheral: gangrene
```
2) Microvascular (small vessel) disease
(thickened capillary basement membrane)
• Nephropathy: kidney failure
• Retinopathy: blindness
```
3) Neuropathy (>50% of all diabetes)
• Impotence
• Bladder dysfunction
• Paresthesias
• Neuropathic pains (diabetic neuropathy, including burning mouth)
```
4) Neuromuscular dysfunction
• Muscle weakness
• Muscle cramps
5) Decreased resistance to infection
128
Metabolic complications of diabetes mellitus
• Hyperglycemia (deficient insulin, diabetic ketoacidosis); chronic, slowly progressive
• Hypoglycemia (excess insulin, excess exercise, stress, poor diet); acute, rapidly progressive
- Most likely problem to be encountered in the pediatric dental office
129
Hypoglycemia
* It is unlikely that hypoglycemic symptoms will occur if blood glucose levels are > than 45 mg/dl
* Central nervous system (CNS)/Adrenergic effects -- headache, mental confusion, somnolence, sweating, tachycardia, tremors, nervousness (40 mg/dl or less)
* Disorientation -- 30 mg/dl or less
* Seizures/coma -- 25 mg/dl or less
130
Medical management of diabetes mellitus
```
• Diet (both type 1 and 2)
• Exercise (both type 1 and 2)
• Medications
- Oral hypoglycemics (type 2 only)
- Insulin (type 1 and 2)
• Injectable
• Pancreatic transplant
- Pancreas
- Pancreas and kidney
- Beta cells
```
131
Dental management considerations for diabetes mellitus
* Screening/identification
* Prevention of hypoglycemia
* Planning dental treatment and surgery
* Infection management
* Antibiotic prophylaxis
* Oral manifestations
132
Prevention of hypoglycemia (insulin reaction)
* Make sure patient has normal meals along with insulin
* AM appointments best -- avoids peak insulin action ***
* Watch for hypoglycemic symptoms: mood change, hungry, anxiety, tremor, headache, lightheadedness, sweating, nausea, tachycardia
* Tell patient to advise you at first onset of symptoms
* Check with glucometer if patient becomes symptomatic
* Treatment: oral carbohydrates (CHO) (sugar, OJ, cola, candy, cake icing); do not give oral CHO if unconscious!
133
Dental treatment guidelines for diabetes mellitus
• A well controlled, stable diabetic taking insulin, requires little or no modification for routine dental care, including surgery. ***
- Make sure patient has normal meals and continues normal insulin administration
• For poorly controlled, uncontrolled or symptomatic diabetes, defer elective treatment and consult with physician to determine stability and control of their disease.
• In case of a hypoglycemic episode: stop dental treatment, high CH beverage (orange juice or soft drink) or IM glucagon, seek medical attention for unconscious patient.
• Uncontrolled diabetes not a good candidate for orthodontic treatment
• Other issues: Difficult to intubate (about 1/3 of patients with long standing DM type I), modified treatment based on systemic complications: renal, cardiac, ocular, and neurologic.
134
Diabetes mellitus following oral surgery
* If the patient is unable to eat a normal diet as a result of the surgery, encourage alternate dietary intake such as a liquid supplement (e.g. Ensure)
* Insulin may need to be decreased if food intake is decreased
* Presence of infection may temporarily increase the insulin requirement
* Postoperative antibiotics are not necessary if diabetes is well controlled; may be indicated for poorly controlled diabetic, especially if oral/dental infection present
135
Oral manifestations of diabetes mellitus
```
• None are pathognomonic
• Commonly associated conditions:
- Xerostomia
- Enlargement of parotid glands
- Burning mouth/tongue
- Altered taste
- Candidiasis
- Mucormycosis
- Periodontal disease
- Increased caries risk
```
136
What is chronic kidney disease?
* CKD is defined as a renal injury (proteinuria) and/or glomerular filtration rate <60 mL/min
* The prevalence of CKD in the pediatric population is approximately 8 per million.
* The prognosis for the infant, child, or adolescent with CKD has improved dramatically since the 1970s because improvement in medical management (aggressive nutritional support, recombinant erythropoietin, recombinant growth hormone), dialysis, and kidney transplantation.
137
Laboratory findings for chronic kidney disease
* Laboratory findings can include elevations in blood urea nitrogen and serum creatinine.
* Hyperkalemia, hyponatremia, acidosis, hypocalcemia, hyperphosphatemia, and election of the uric acid
* CBC: normocytic, normochromic anemia
* Elevated cholesterol
* Inulin clearance is the gold standard, however --> not easy to measure
* Endogenous creatinine clearance --> most widely used marked
138
Treatment for chronic kidney disease
1) Treatment is aimed at replacing absent or decreased renal functions:
- Acidosis: metabolic acidosis develops in almost all children with CKD as a result of decreased net acid excretion by the failing kidneys --> bicarbonate is always in the list of medications.
- Nutrition: progressive restriction of several dietary components as the renal function declines
- Growth: short stature is a significant long-term sequela of childhood CKD. Children with CKD have an apparent growth hormone resistant state --> treatment with recombinant growth hormone.
2) Management requires a multidisciplinary team and close monitoring of the patient clinical and laboratory status:
- Fluid and electrolyte management: most children maintain normal sodium and water balance from appropriate diet. Children with CHH may require fluid and sodium restriction (usually when the CKD is close to end-stage renal disease)
- Potassium balance is usually maintained until renal function deteriorates to the level that dialysis is required.
139
Pathophysiology of renal osteodystrophy
Complex:
When the GFR declines to approximately 50% of the normal, the decrease in functional kidney mass leads to a decrease in renal 1a-hydroxylase activity --> decrease production of activated vitamin D --> decreases in intestinal calcium intake --> hypocalcemia --> excessive parathyroid hormone --> increase bone resorption
140
What is renal osteodystrophy?
- The term RO is used to indicate a spectrum of bone disorders seen in patients with CKD.
- The MOST common condition seen in children with RO is high turn-over bone disease caused by secondary hyperparathyroidism --> consequence: osteitis fibrous cystica ***
141
Clinical manifestations of renal osteodystrophy
* Muscle weakness
* Bone pain
* Fractures with minor trauma
142
Treatment for renal osteodystrophy
The cornerstone --> administration of vitamin D
143
Dental findings for chronic kidney disease and renal osteodystrophy
* Bone loss
* Lack of interior cortex
* Delayed permanent tooth eruption
* Fragile bones
144
Anemia and chronic kidney disease
The anemia in patients with CKD is caused by inadequate production of erythropoietin --> treatment with recombinant human factor.
145
Hypertension and chronic kidney disease
Angiotensin-converting enzyme (ACE) inhibitors are usually the medication of choice
146
Drug doses and chronic kidney disease
Adjustment in drug dose: because many medications are excreted by the kidneys, their dosing might need to be adjusted in patients with CKD to maximize the effectiveness and minimize the risk of toxicity
147
What is end stage renal disease (ESRD)?
* Progressive deterioration and destruction of nephrons (glomerulus, tubules and vasculature)
* Different segments affected first, but eventually the entire nephron is affected
* Normal kidney function is maintained until 50-75% of the nephrons are destroyed; function is compensated by hypertrophy of remaining nephrons
* ESRD represents the state in which a patient's renal dysfunction has progressed to the point at which hemostasis and survival can no longer be sustained with native kidney function and medical management.
148
Ultimate goal of ESRD
* ESRD --> renal replacement therapy
* The ultimate goal --> Kidney transplantation
* In the USA, 75% of children with ESRD require a period of dialysis before transplantation can be performed.
* Dialysis --> stage 4
* The selection of the dialysis modality must be individualized
149
ESRD and dialysis
In the USA:
• 2/3 children with ESRD --> peritoneal dialysis
• 1/3 children with hemodialysis
** Age is an important factor. From birth to 5 years old --> peritoneal dialysis
150
Peritoneal dialysis
• Continuous or cycles (majority of children)
• Daily
• Usually at night
• Advantages:
- Low cost
- Ease of performance
- Reduced risk of infectious disease transmission
- Anticoagulation not needed
• Disadvantages:
- Need for frequent sessions
- Risk of peritonitis (once per patient every 18 months)
- Hernia
- Much lower effectiveness than hemodialysis
151
ESRD and dental management concerns
* Bleeding tendencies
* Impaired drug excretion
* Hypertension
* Shunt infections
* Hepatitis B or C
* Anemia
* Renal osteodystrophy
152
Dental management - conservative care for ESRD
* Medical consultation to determine stability and control
* Avoid dental treatment in patients whose disease is poorly controlled
* Screen for bleeding disorder prior to surgery -- bleeding time, prothrombin, platelet count, hemoglobin, hematocrit
* Anemia generally well tolerated with hematocrit > 25
* Monitor blood pressure closely
* Use good surgical technique
* Avoid nephrotoxic drugs
* Consider reduction of dosage or increase time intervals between doses of active drugs excreted by the kidney
* Manage orofacial infections aggressively
* Consider hospitalization for patients with severe infection or needing major dental procedures
153
Dental management for hemodialysis
* Same as those recommended for patients receiving conservative care
* Be aware of presence of arteriovenous shunt; avoid blood pressure cuff and IV medications in arm with shunt
* Avoid dental treatment on day of dialysis due to heparin (half life 1.5 hours), best to treat day after dialysis to avoid excessive bleeding
* Assess status of liver function because of increased risk of hepatitis B, C, viruses and HIV infection
154
Hemodialysis Shunt
* Shunt/graft is at risk for endarteritis
* Can lead to endocarditis (occurs in 2% to 9% of patients)
* Almost all infections (endarteritis and endocarditis) are caused by Staph aureus; oral flora have not been implicated in these infections
* AHA recommendation states that these vascular access devices are not recommended for prophylaxis for dental procedures
* Hemodialysis patients with other risk factors for endocarditis such as previous BE or prosthetic heart valves should receive antibiotic prophylaxis
155
Oral changes in renal failure
* Pallor of mucosa (anemia)
* Red-orange discoloration of mucosa (carotene-like pigments)
* Xerostomia, parotid infections and candidiasis
* Ammonia-like breath odor (high urea content)
* Metallic taste
* Uremic stomatitis (red, burning mucosa covered with gray exudates that may ulcerate) seen with high BUN levels
* Petechiae and ecchymoses
* Osseous changes -- lytic bone lesions among others
* Hemodialysis can lessen the severity or reverse many of these changes
156
Oral manifestations in renal failure
* Palatal enlargement due to secondary hyperparathyroidism
* Brown tumor of hyperparathyroidism showing scattered multinucleate giant cells
* Periapical radiograph showing the "ground glass" appearance of the trabeculae and loss of lamina dura in a patient with secondary hyperparathyroidism
* Multiple lucency's associated with secondary hyperparathyroidism
* Histology of the lesions revealing numerous giant cells among fibroblasts
157
Management of kidney transplant patient
These patients will be on immunosuppressive drugs for the remainder of their lives (e.g. corticosteroids, azathioprine, cyclosporine, tacrolimus), thus susceptibility to infection is the primary concern.
• Consultation with physician/transplant coordinator
• Frequent recall and prophylaxis
• Daily antibacterial mouth rinses (chlorhexidine)
• Any indicated dental care
• Avoid NSAIDs (increases bleeding with corticosteroids and potentiation of nephrotoxicity of cyclosporine and tacrolimus)
• Consider antibiotic prophylaxis for invasive procedures (controversial)
• Screen for head and neck cancers
• Consider need for supplemental corticosteroids (if patient is on or has recently discontinued taking corticosteroids)
158
Most frequent documented source of sepsis in the immunosuppressed cancer patient
The most frequent documented source of sepsis in the immunosuppressed cancer patient is the mouth; therefore, early and definitive dental intervention, including comprehensive oral hygiene measures, reduces the risk for oral and associate systemic infections.
159
Guideline on dental management of pediatric patients receiving chemotherapy, Hematopoietic cell transplantation (HCT), and/or radiation
• All patients with cancer should have an oral examination before initiation of the oncology therapy, and treatment of pre-existing conditions is essential to minimize complications in this population.
• Goals for dental and oral care before the initiation of cancer therapy:
- To identify and stabilize or eliminate existing and potential sources of infection and local irritants in the oral cavity.
- To educate the patient and parents about the importance of optimal oral care in order to minimize the problems before/during and after treatment.
160
Initial evaluation for pediatric patients receiving chemotherapy, HCT, and/or radiation
```
• Medical history review
- The AHA recommends that antibiotic prophylaxis for non-valvular devices (catheters) is indicated ONLY at the time of placement
• Dental history; oral-dental assessment
• Preventive strategies:
- Oral hygiene
- Ultrasonic brushes and dental floss**
- Chlorhexidine rinses**
- Diet
- Fluoride
- Trisumus preparation
```
161
Hematologic considerations for pediatric patients receiving chemotherapy, HCT, and/or radiation
1) Absolute neutrophil count (ANC)
• >1,000/mm^3: no need for AB prophylaxis. However, some authors suggest that antibiotic coverage may be prescribed when the ANC is in between 1,000 and 2,000/mm^3. If infection is present or unclear, more aggressive antibiotic therapy may be indicated.
• <1,000/mm^3: defer elective dental care until the ANC rises. In dental emergency cases, discuss antibiotic prophylaxis with the medical team before proceeding with treatment.
2) Platelet count
• 75,000/mm^3: no additional support needed but the pediatric dentist should be prepared to treat prolonged bleeding by using suture, hemostatic agents, pressure packs, etc.
• 40,000-75,000/mm^3: Platelet transfusions may be considered pre- and 24 hours post-operatively. Localized procedures to manage prolonged bleeding may include sutures, hemostatic agents, pressure packs or gelatin foams.
• <40,000/mm^3: defer care, dental emergency --> platelet transfusion
162
dental and oral care before the INITIATION of cancer therapy for pediatric patients receiving chemotherapy, HCT, and/or radiation
1) In general, most oncology/hematology protocols (**HCT) are divided into two phases. The patient blood counts normally start falling 5 to 7 days after the beginning of each cycle, staying low for approximately 14-21 days, before rising again to normal levels. Ideally: all dental treatment before cancer therapy starts.
2) Prioritizing procedures: infections, extractions, periodontal care, and sources of tissue irritation.
3) Pulp therapy in primary teeth: although there have been no studies to date that address the safety of performing pulp therapy in primary teeth, many pediatric dentist prefer to provide a more definitive treatment in the form of extractions.
4) Endodontic treatment in permanent teeth: symptomatic non-vital permanent teeth should receive RCT at least 1 week before initiation of cancer therapy:
• If RCT is not possible --> extraction is indicated --> AB therapy (PNC x 1 week)
5) Orthodontic appliances and space maintainers: poorly fitting appliances can abrade oral mucosa and increase the risk of microbial invasion into deeper tissues.
• Appliances should be removed if the patient has poor oral hygiene and/or the treatment protocol or HCT conditioning regimen carries a risk for the development of moderate to severe mucositis.**
• Simple appliances that are not irritating to the soft tissues may be left in place in patients who present good OH.
6) Periodontal treatment: partially erupted molar can become a source of infection because of pericoronitis. The overlying gingival tissue should be excised if the dentist believe it is a potential for infection.
7) Extractions: there are NOT clear recommendations for the use of prophylactic AB for extractions. If there is documented infection associated with the tooth, AB should be administered for about 1 week.
• Minimize the risk of osteonecrosis associated with radiation therapy.
• Loose primary teeth should be allowed to exfoliate naturally.
• If extractions are necessary --> 7-10 days before cancer therapy starts.***
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Dental and oral care DURING immunosuppression for pediatric patients receiving chemotherapy, HCT, and/or radiation
* Preventive strategies: Thrombocytopenia should not be the sole determinant of oral hygiene as patients are able to brush without bleeding at widely different levels of platelet count.
* If patients as skilled at flossing without traumatizing the tissues, it is reasonable to continue flossing throughout treatment.
* Toothpicks and water irrigation devices should not be used when the patient is pancytopenia to avoid tissue trauma.
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Elective dental care during immunosuppression
During immunosuppression, elective dental care must not be provided.
• If a dental emergency arises, the treatment plan should be discussed with the patient's physician who will make recommendations for supportive medical therapies.
• The patient should be seen every 6 months (or shorter intervals if there is a risk for xerostomia).
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Management of oral conditions associated with cancer therapy (dental and oral care DURING immunosuppression)
Mucositis:
• Conflictive results in the use of chlorhexidine
• Patient-controlled analgesia has been helpful in relieving pain associated with mucositis
• No significant evidence of the benefit of the use of "Magic Mouth Wash"
• Topical anesthetics: side effects associated with the use of topical lidocaine
Oral mucosal infections:
• Clinical appearance is different from classical presentation
• Prophylaxis nystatin is NOT effective for the prevention and treatment of fungal infections
Dental sensitivity:
• Patients who are using plant alkaloid chemotherapeutic agents (vincristine and vinblastine) may present with deep, constant pain affecting the mandibular molars --> pain is usually transitory
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Dental and oral care AFTER the cancer therapy is complete (exclusive of hematopoietic cell transplantation)
* Patients should floss daily
* The patient should be seen at least every 6 months (or shorter intervals if issues such as chronic oral GVHD, xerostomia or trismus)
* Patients who experienced GVHD --> shorter FU intervals because the risk of cancer in the oral mucosa
* Orthodontic treatment: may start or resume after completion of all therapy and after at least 2 year disease-free survival when the risk of relapse is decrease and the patient is NOT longer using immunosuppressive drugs
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Specific oral complications can be correlated with phases of hematopoietic cell transplantation (HCT)
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Phase I (pre-transplatation):
• In HCT the patient receives all the chemotherapy and/or radiation in JUST a few days before the transplant
• In HCT will be prolonged immunosuppression following the transplant
• Elective dentistry MUST be postponed until immunological recovery --> 9-12 months after HCT
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Phases:
• Phase I (pre-transplatation)
• Phase II (conditioning/neutropenic phase)
• Phase III (initial engraftment to hematopoietic reconstitution)
• Phase IV (immune reconstitution/late post transplantation)
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What does aspirin do?
Inhibits TXA2 formation
| functions to stimulate activation of new platelets as well as increases platelet aggregation
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The amount of bleeding with thrombocytopenia depends on the severity
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Platelets/Spontaneous bleeding/post-traumatic bleeding:
• >50,000/no/rare
• >30,0000-50,000/rare/occasional
• 10,000-30,000/occasional/always
• <10,000/frequent/always
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What is hemophilia A (classic hemophilia)?
* Inherited bleeding disorder
* Hereditary deficiency of factor VIII
* Virtually limited to males who transmit the trait only to their daughters***
* Females are asymptomatic but transmit the abnormal gene to half their daughters (carriers) and half of their sons (hemophilia A)
* 1:10,000 males
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Symptoms of hemophilia A or classic hemophilia
• Severity of the symptoms can vary and severe forms become apparent early on.
• Prolonged bleeding is the hallmark.
• Mild cases may go unnoticed until later in life when is excessive surgery in response to trauma or surgery.
• Bleeding into joints is common.
• Clinical manifestations --> vary in proportion to coagulant factor activity:
- Severe: <1% factor VIII activity
- Moderate: 1-5% factor VIII activity
- Mild: 5-25% factor VIII activity
• Signs and symptoms:
- Delayed bleeding from small wounds
- Hemarthroses, hematomas, and hematuria
- Joint destruction and muscle atrophy
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Incidence of hemophilia A
* The incidence of hemophilia A is 1:10,000 live male births.
* About 17,000 Americans have hemophilia.
* Women may have it, but it's very rare.
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Laboratory findings for Hemophilia A
Long partial thromboplastin time (PTT) with normal prothrombin time (PT)
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Treatment for Hemophilia A or B
• Key --> replacement for the clotting factor VIII or IX (hemophilia B)
• Dose for factor replacement therapy --> severity of the disease, the site
- Each unit of factor VIII per kg body wt raises the plasma level by 2% --> injection of 1,700 U factor VIII will provide for an incremental rise of 50%
- Declines according to the pharmacokinetics associated with factor VIII (1/2 8-12 hours)
- Common practice --> double the initial dose
- Continuous infusion protocols --> excellent --> decreases 30-75% use of concentrate
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Factor Replacement Therapy
Spontaneous bleeding:
• Desire plasma level: 30%
• Initial dose: 15 UI/kg body wt
• Duration: q8-12h/1-2 days
Oropharyngeal or dental:
• Desire plasma level: 25-30%
• Initial dose: 20 UI/kg
• Duration: q8-12h/1-2 days
Major surgery:
• Desire plasma level: 50%
• Initial dose: 50 UI/kg
• Duration: q8-12h/10-14d
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Hemophilia B
* Hereditary deficiency of factor IX***
* Symptoms mimic classic hemophilia
* Incidence 1:50,000
* Two types have been described: failure of synthesis of factor IX and synthesis of defective IX
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Factor XI deficiency
* Mild, factor deficiency in both sexes due to a quantitative deficiency of factor XI
* The inheritance is autosomal and is most common in jewish patients
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Von Willebrand's Disease
* Autosomal dominant disease due to a plasma deficiency of vWF (1% of the population)
* Most common inherited bleeding disorder ***
* Inherited disease in the platelet adhesion
* The affected gene has been localized to chromosome 12***
* Several variants depending on the genetic expression
* Signs and symptoms --> mucocutaneous bleeding, epistaxis, menorrhagia, easy bruising
* vWf serves as the carrier protein for factor VIII which increases its half-life
* Most common subtype: I (quantitative decrease in vWf)
* Diagnosis --> long closure time
* Treatment --> Desmopressin or vWf replacement
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Basic platelet reaction
* Adhesion
* Aggregation
* Release reaction (secretion)
* Clot formation and retraction
**Affected if deficiency of vWf exists**
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Treatment of Von Willebrand's Disease
* Depends on the clinical condition of the patient and the type involved
* Desmopressin acetate (DDAVP) is administered prophylactically prior to minor surgical procedures or with post traumatic bleeding in mild vWD
* Factor VII(? -- factor VIII) concentrate that retain high molecular weight vWF (not effective to all types of vW disease)
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Bernard-Soulier Disease (BS disease)
* BSD also represents a disorder of platelet adhesion; however, the platelets are defective and unable to interact with vWF.
* The basic defect is the absence of glycoprotein Ib (GP-Ib) from the membrane of the platelets
* Only available treatment --> transfusion with normal platelets
