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115 Cards in this Set

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Prader-Willi Syndrome

Down syndrome

Angel man syndrome

Neurofibromatosis

Type 1 usually diagnosed in children


Type 2 usually diagnosed in adulthood

High RBC


High WBC


High platelets

Polycythemia Vera

JAK2 protein mutation usually involved

Factor VIII deficiency

Hemophilia A


Sex-linked, Recessive


Most common sex linked bleeding

Factor IX deficiency

Hemophilia B


Sex linked, Recessive

Factor VII deficiency

Alexander's disease


Autosomal, Recessive


F7 Gene


Chromosome 13


Can be acquired

Von Willibrands

VWF deficiency


Autosomal Recessive


Most common hereditary bleeding


Causes decrease in factor VIII

Cleft palate repair timing

Rule of 10's


First sx - cleft lip around 10 weeks


Second sx - soft palate around 10 months


Third sx - bone graft around 10 years

Conditions with Taurodontism

Kleinfelter


Tricho-dento-osseous


Mohr


Hypohidrotic ectodermal dysplasia


Amelogenesis imperfecta IV


Down


Williams


Smith-Magenis

Prominent lip, wide mouth, Taurodontism, enamel hypoplasia, microdontia, partial anodontia

Conditions associated with hyperdontia

Apert


Cleidocranial dysplasia


Gardner syndrome


Crouzon syndrome


Down syndrome


Sturge-weber


Orofaciodigital syndrome I


Cleft lip/palate

Conditions associated with hypodontia and oligodontia

Ectodermal dysplasia


Crouzon syndrome


Achondroplasia


Chondroectodermal dysplasia


Williams syndrome


Non-syndromic cleft lip/palate


Incontinentia pigmenti


Orofaciodigital syndrome I


Riegee syndrome

Conditions associated with microdontia

Ectodermal dysplasia


Chondroectodermal dysplasia


Hemifacial microsomia


Down syndrome


Crouzon syndrome


Pituitary dwarfism

Conditions associated with macrodontia

Hemifacial hyperplasia


Crouzon syndrome


Otodental syndrome


XXY syndrome


Pituitary gigantism


Pineal hyperplasia

Anomalies of initiation and proliferation

Anodontia


Hypodontia


Oligodontia

Anomalies of Morphodifferentiation

Dens in dente


Dens evaginatus


Taurodontism


Dilaceration

Anomalies of histodifferentiation

Amelogenesis imperfecta


Dentingenesis imperfecta


Osteogenesis imperfecta


Ehlers-Danlos


Goldblatt syndrome


Schimke immune-osseous dysplasia


Anomalies of apposition

Enamel hypoplasia


Enamel pearls


Dentin dysplasia


Regional odontodysplasia


Rickets


Hypoparathyroidism


Pseudohypoparathyroidism


Ehlers-Danlos


Albright hereditary


Hypophosphatasia


Epidermolysis bullosa


Cleidocranial dysplasia

Anomalies of mineralization

Enamel hypomineralization


MIH


Amelogenesis imperfecta III


Fluorosis


Sclerotic dentin


Anomalies of maturation

Amelogenesis imperfecta II


Amelogenesis imperfecta IV

Intrinsic staining

Anemia - gray


Bile duct defects - green


Trauma - red, gray, black


Bilirubin - blue-green


Porphyria - purplish-brown


Tetracycline - yellowish-gray


Idiopathic bone sclerosis

Asymptomatic


Non-odontogenic


Radiopaque, well defined, localized, non corticated, Apex of vital teeth, no teeth displaced.


90% in mandible by molars

Simple bone cyst

Bone cavity, not true cyst


Mandible posterior region


No effect on surrounding teeth


Associated with cemento-osseous dysplasia


(question: simple bone cyst, nothing around lesion)

Fibrous dysplasia

Radiopaque, ill-defined


Orange peel


Ground glass



Replacement of normal bone by fibrous tissue containing abnormal appearing bone.



70% are monostotic


Displacement of teeth

Focal cemento-osseous dysplasia

3 stages: lucent, mixed, opaque


Hispanic


Female



Replacement of normal bone with cementum-like material



Vital teeth

Radicular cyst

Originated from epithelial rests of malassez


Most common cyst in jaws


Non-vital tooth


Periapical cyst

Buccal bifurcation cyst

Inflammatory odontogenic cyst


Associated with previous pulp therapy (pulpotomy)


In children only


Radiolucent, well defined

Dentigerous cyst

Radiolucent, CEJ to CEJ


Around crown of unerupted tooth, usually supernumerary teeth or 3rd molars


From accumulation in layers of reduced enamel epithelium


Second most common cyst in jaws

Odontogenic keratocyst (OKC)

Odontogenic from dental lamina



Epithelium has Innate growth potential



White cheese inside



Superior to IA canal

Naso-palatine canal cyst

Non-odontogenic cyst


Anterior maxilla


Heart shape at naso-palatine canal

Adenomatoid odontogenic tumor

Benign


Calcifications around canine area


Radiolucent


Snowflakes

Ameloblastic fibroma

Similar to OKC but Small calcifications inside lesion


Compound odonontoma

Radiopaque surrounded by radiolucent surrounded by radiopaque line



Tooth like structures

Complex odontoma

Golf ball


Benign cementoblastoma

Radiopaque attached to apex of vital but painful tooth


Ewing's sarcoma

Malignant tumor of long bones


Swelling, pain, loose teeth,


Floating teeth


Radiolucent, ill-defined


UNEVEN destruction of bone


Finger like projections

Acute lymphocytic leukemia (ALL)

Tumor of hematopoietic stem cells


Periapical lesions


No expansion of bone but displaced unerupted teeth


Premature tooth loss

Kids with developmental disabilities are how likely to desaturate with sedation?

3X more likely

DiGeorge syndrome

Chromosome 22Q11


Thymic hypoplasia


Deficient T-cell function

Chediak-Higashi syndrome

Oculocutaneous albinism


Early childhood periodontitis


Defective gene inhibits killing phagocytosed bacteria

Percentage of children in US with caries by age

2-5 years: 23% or 1 in 4


6-8 years: 56%


Anomalies of proliferation and Morphodifferentiation

Microdontia


Macrodontia


Gemination


Fusion


Concrescence

Moderate caries risk factors for age >6

SHCN


Recent immigrant


Defective restorations


Intraoral appliance

Kwashiorkor

Protein malnutrition characterized by edema and enlarged liver with fatty infiltrates.


Abrupt weaning


Low antioxidants


Altered intestinal bacteria



Sufficient calories, insufficient protein (Different from marasmus)

Nutritional causes of anemia

Deficiencies in:



Iron


Folate


B12


Riboflavin


Copper


If you have pea sized amount of 1000ppm fluoride toothpaste, how much fluoride in mg?

0.25mg

Define overweight in childhood

BMI at or above 85th percentile

Define obesity in childhood

BMI at or above 95th percentile

ECC defined as

1 or more dmft surface in any primary tooth before age 6

S-ECC defined as

Sign of smooth surface caries under age 3


1 or more dmft in primary max anterior teeth ages 3-5


Dmft greater than 4 at age 3


Dmft greater than 5 at age 4


Dmft greater than 6 at age 5

Pierre Robin sequence

Micrognathia


Macroglossia


Glossoptosis


Cleft lip and palate


Intellectual impairment

Treacher Collins syndrome


(Mandibulofacial disostosis)

-Auricular and ocular defects


-Zygomatic and mandibular hypoplasia


-Microstomia


-Palatopharyngeal incompetence


-Cleft palate


-Normal intelligence

Crouzon syndrome

Craniofacial synostosis


Proptosis


Maxillary hypoplasia


Beaked nose


High arched palate and malocclusion


Cleft lip and pallet


Cervical vertebral fusions

Apert syndrome


(Acrocephalosyndactyly)

-Craniofacial synostosis



-Maxillary hypoplasia


-Prognathism


-Cleft palate


-Tracheobronchial cartilaginous anomalies


- cervical vertebral fusions


- syndactyly

Goldenhar syndrome


(Eye-ear-spine)


(Hemifacial microsomia)

Articular and ocular defects


Zygomatic/mandibular hypoplasia


Occipitalization of Atlas


Cleft lip and palate


Velopharyngeal insufficiency


Cervical vertebral fusion

Down syndrome

Narrow nasopharynx


Large tonsils and adenoids


Macroglossia


Underdeveloped nose bridge


Small subglottic area


Microcephaly


Cervical spine abnormalities


Atlantoaxial subluxation


Broad short neck

Klippel Feil syndrome

Cleft palate


Restricted neck movement


Congenital fusion of a variable number of cervical vertebrae

Beckwith-Wiedeman syndrome

Macroglossia


Neoplasms involving head and neck

Cherubism


(Familial fiberous dysplasia)

Tumorous lesions in the mandible and maxilla with intra-oral masses



Limited jaw closure



Tongue displacement

Cretinism


(Congenital hypothyroidism)

Compression of trachea


Macroglossia


Laryngeal nerve palsy


Paradoxical vocal cord motion

Cri du chat syndrome

Deletion 5p syndrome


Microcephaly


Micronathia


Laryngomalacia


Stridor


Cleft lip and pallet


Hemivertebrae

Von Recklinghausen disease


(Neurofibromatosis)

Macroglossia


Cafe au lait


More...

Hurler syndrome

Gargoyle facies


Enlarged tongue


Small mouth


Stiff joints


Kyphoscoliosis

Hunter syndrome

Similar to hurler syndrome but less severe

Pompe's disease

Glycogen storage disease


Macroglossia


Muscle deposits


Respiratory muscle weakness

Prader Willi syndrome

Increase grelin


Severe obesity


Hypotonia


Chromosome 15

De Lange syndrome

Severe growth deficiency


Recurrent respiratory infection


Depressed nasal bridge


High arched palate


Micronesia


Cleft palate

Saethre-chotzen syndrome

Craniosynostosis


Maxillary hypoplasia


Cleft palate

HPV greatest protection

Age 9 - 12

Normal crown color and shape with short constricted roots



Rootless teeth

Dentin dysplasia type I

Thin roots with normal length


Amber colored teeth


Bulbous crown


Cervical constriction


Early pulp obliteration


Thistle-tube shaped chamber


Dentin dysplasia type 2

Bulbous crowns


Thin roots


Cervical constriction


Early canal obliteration


Occurs with osteogenesis imperfecta


Amber colored tooth

Dentinogenesis imperfecta type 1

Opalescent Dentin


Same characteristics as dentinogenesis imperfecta type 1


Does not occur with osteogenesis imperfecta

Dentinogenesis imperfecta type 2

Bell-shaped crowns


Shell-like appearance of teeth


Often multiple pulp exposures


Normal thickness of enamel


Very thin Dentin

Dentinogenesis imperfecta type 3

Lesch-Nyhan syndrome

X-linked trait that affects how body breaks down purines



Missing enzyme: hypoxanthine guanine phosphoribosyltransferase



Causes buildup of uric acid



Self-mutilation behavior

Hunter/hurler syndrome

Results in buildup of glycosaminoglycans



Claw hands

Sturge-Weber syndrome

Manifested at birth by seizures



Port-wine stain



Early eruption of teeth



Major problem is hemorrhage of angiomas

Neurofibromatosis

Wide inferior alveolar canal


Rubenstein-Taybi

Heart defects possibly requiring Sx



Talon cusp

Treacher Collins

Small mandible and short ramus


Normal intelligence


Facial nerve affected


Cleft palate

Pierre Robin sequence

Small jaw effects tongue size and position which blocks airway


Cleft palate


Glossoptosis (relative)

Rett syndrome

Normal development for first 6-18 months


Floppy arms and legs - first sign


Apraxia - unable to perform task


Lots of social engagement


Seizures

Beckwith-Wiedmann syndrome

Craniosynostosis ridge on forehead


Macroglossia


Large organs

Crouzon syndrome

Craniosynostosis


Patent ductus arteriosus


Usually normal intelligence


Posterior bilateral crossbite


Hypodontia

Ellis-van creveld syndrome


(Chondroectodermal dysplasia)

Post-axial polydactyly


Congenital heart defects


- atrial septal defect most common


Short limb dwarfism


Cleft lip and palate


Multiple frenula


Microdontia

Fragile X syndrome

Elongated face


Mostly males


Large ears


Malocclusion common

Tuberous sclerosis

Hypomelanotic macules


Facial angiofibromas


Sublingual fibromas


Enamel hypoplasia


Gingival hyperplasia from seizure meds

Sotos syndrome

Cerebral gigantism


Long narrow face


Down-slanting palpebral fissures


Learning disability


Overgrowth


Hypotonia


Premature/early eruption


Hemi-mandibular hypertrophy

Oculo-auriculo-vertebral syndrome

Goldenhar syndrome


Hemifacial microsomia


Branchial Arch 1 and 2 affected


Day 30 - 45 of gestation


In utero hemorrhage

Fetal alcohol syndrome

Smooth filtrum, thin upper lip


Flat mid-face


Minor ear abnormalities


Cleft palate common


Hypoplastic mandible


Intellectual disabilities

Cornelia de Lange syndrome

Behavioral issues: self-injury, compulsive repetition, autistic-like


GERD


Thin eyebrows meet at Midline


Cleft palate


Delayed eruption

Apert syndrome

Craniosynostosis


Syndactyly


Mid-face hypoplasia


Cervical spine fusion


Supernumerary teeth


Ectopic eruption of Max 1st molars


Shovel shaped incisors


Intellectual disability

Turner syndrome

Incomplete puberty


Swollen hands and feet


Early eruption of permanent molars


Micrognathia

Noonan syndrome

Heart defects: hypertrophic cardiomyopathy, atrial septal defect, ventricular septal defect, septal hypertrophy


Delayed and atypical eruption sequence

Lowe syndrome

Oculo-cerebro-renal syndrome


Perio disease


Taurodontism

Kleinfelter syndrome

Only males affected


Learning problems


Taurodontism

Down syndrome

Microdontia


Macroglossia


Congenital heart disease


Delayed eruption


Hypodontia


Atlanto-axial instability

Cri-du-chat


(Call of the cat)

Micronathia


Cleft lip and palate


GERD erosion

Achondroplasia

Large head-to-body size difference


Depressed nasal bridge


Maxillary hypoplasia


Tooth shape abnormalities

ADHD

3 to 5% of school-age children


Prefrontal cortex


dopamine system


Stimulants first choice of med:


Methylphenidate, amphetamines

Cerebral palsy

Malfunction of motor centers in the brain


No cure


Spastic tightness


Intellectual disability - 60%


Fetal alcohol syndrome?

Congenital heart disease

Risk factors:


-Maternal rubella


-Diabetes


-Alcoholism


-Irradiation


-Drugs: thalidomine, phenytoin, warfarin


Down syndrome, Turner syndrome

Tetralogy of Fallot

Ventricular septal defect


Pulmonary stenosis


Overriding aorta


Right ventricular hypertrophy

Infective endocarditis prophylaxis

Prosthetic cardiac valve


Previous infective endocarditis


Cardiac transplant with valvulopathy


Unrepaired cyanotic CHD


Repaired CHD within 6 months

Asthma

Corticosteroids - 1st choice


Beta 2 agonist (Albuterol) 2nd choice


Inflammatory, obstructive, restrictive


Increase prevalence of caries (meds)


Decrease salary flow - increase lactobacilli


No NSAIDs


No more macrolides (erythromycin)

Diabetes

A1C > 7%


Insulin


Heart: CHD


Cerebrovascular: Stroke


Peripheral: Gangrene


Neuropathy


Decreased resistance to infection

Chronic kidney disease

Renal Osteodystrophy


Lack of mineralization of bone


Bleeding tendencies


Impaired drug excretion


Hypertension


Shunt infection


Hepatitis B or C


Anemia

Stickler syndrome

Genetic disorder of connective tissue


Joint hypermobility


Mutation alpha-1 chain of type 2 collagen


Hearing problems


Flat midface