Weebly Study Guide Flashcards

Question

Cyclic Neutropenia

Answer 1

Spontaneous episodes of neutropenia recurring every 14-35 days, lasting 3-5 days Cyclic fever, pain, malaise, ulcers, gingivitis Aggressive removal of dental plaque/calculus needed; consider CHX during neutropenia May need antibiotic coverage`

Answer 2

Autosomal recessive Chronic pulmonary disease and pancreatic insufficiency Enamel hypoplasia and discoloration of permanent teeth (from abx) Salivary gland involvement Increased calculus, gingivitis Decreased caries (may be from abx) Increased candidiasis Consult physician for dental tx, antibiotic prescription (to make sure it doesn't interact with antibiotic for CF treatment) Upright sitting position, short appointments, afternoon visits (coughing in morning) Nitrous oxide contraindicated if emphysema

Answer 3

Type I: beta cells don't produce insulin Type II: body is immune to insulin 7% prevalence Fruity odor on breath Diminished salivary flow Peripheral neuropathy Gingivitis/periodontitis Candidiasis Delayed wound healing Make sure HbA1c > 7; may need aggressive antibiotics in uncontrolled Uncontrolled DM = NO ORTHO Be ready for hypoglycemic events

Answer 4

Cardiac anomalies Hypoparathyroidism Intellectual disability/Psych disorders Asymmetric facies, small mouth, velopharyngeal insufficiency, cleft palate, wide eyes, flat nasal bridge Microdontism, enamel aberrations, delayed eruption Difficult airway; increased infection risk *Most common cleft lip syndrome

Answer 5

Varying degrees of cognitive delay Hypotonia, broad/short hands and fingers, cardiac defects 40% Higher tendency towards leukemia, other infectious diseases, Alzheimer's, hypothyroidism Maxillary hypoplasia, flat back of head, small ears, short nose, upward slanting eyes Increased risk atlantoaxial instability (no protective stabilization if so) Increased periodontal disease, low level of caries, mouth breathing, malocclusion, delayed eruption, bruxism, tooth number issues, taurodontism Airway obstruction issues due to macroglossia, large tonsils, atlantoaxial instability, hypotonia

Answer 6

Anorexia, bulimia, and binge eating disorder Anorexia: hirsutism, amenorrhea, brittle nails, hypotension, muscular weakness, osteoporosis Bulimia: normal range of weight, depression, dehydration, electrolyte imbalance, trauma to esophagus, Russell's sign (hardened calluses over knuckles of hand) BED: at least once a week over 3 months All have increased dental caries risk; some dental erosion, perimolysis, enlarged salivary gland with BN Encourage to rinse with alkaline solution after vomiting

Answer 7

Over 150 syndromes with abnormalities of ectodermal structures Most common is hypohidrotic ectodermal dysplasia Sparse hair, thin hair, thin skin with decreased pigment, dry mucosa, small brittle nails, variable function of sweat glands, hypodontia to anodontia of teeth Maxillary hypoplasia, pinched nose, thick lips, possible cleft Teeth often conical/peg shaped, taurodont molars Enamel hypoplasia possible Prosth may be needed as early as 2 years of age with multiple replacements as child grows

Answer 8

Defect in collagen Flexible joints, stretchy skin that bruises easily Hypermobility type, Vascular type, Kyphosis type, Arthrochalasia type, Cardiac Type, or Periodontal Type Slender and asymmetric face, hypertelorism, short nose, low ears, high palate Periodontal disease, TMJ issues, thin mucosa, enamel hypoplasia, pulp stones and malformed roots Avoid fluoroquinolones Be prepared for hemorrhage and test coagulation prior to procedures Ortho relapse common

Answer 9

Simplex, Junctional, Dystrophic, and Kindler Issues with keratin causing blistering in response to mechanical trauma or heat Risk for infection due to open wounds, contracture/mitten deformities, malnutrition and dehydration Bullae, scarring, dry skin and lips with hair loss and blindness occasionally Scarring may lead to limited mouth opening Enamel hypoplasia and hypomineralization Periodontal disease, oral blisters Use lubrication with dental instruments and minimally stretch or touch tissues Avoid blocks with LA Difficult intubation; but most dental treatment needs to be done under GA if invasive

Answer 10

Disruption in neuron activity in the brain Acute symptomatic seizure: occurs with systemic or brain insult Unprovoked seizure; status epilepticus: seizures prolonged or immediately recurrent without return to consciousness Partial: most common type; affects only part of brain, variable presentation Generalized: affects all of brain; absence, atonic, myoclonic, tonic-clonic Increased risk of dental caries due to medication-related xerostomia Medication-induced gingival hypertrophy Trauma risk Verify pt has medications with them, avoid xylocaine and meperidine, avoid seizure triggers (light, stress, etc.)

Answer 11

Chronic, irreversible progressive disease characterized by destruction of 50-75% of nephrons Requires long-term dialysis or kidney transplant Caused by congenital anomalies, glomerulonephritis, DM, HTN Fatigue, malaise, loss of appetite, failure to thrive, HTN, edema, frequent urination Xerostomia, metallic taste, anemia, petechiae/ecchymosis, enamel hypoplasia, rootless teeth, gingivitis, parotitis May require Abx prophylaxis Defer tx if not controlled (ANC < 1000/mm3, PLT < 75k/mm3); if well-controlled no contraindications for dental tx If transplantation is planned, follow normal transplantation guidelines Avoid dental tx day before dialysis Systemic fluoride not recommended due to fluoride retention Avoid meds metabolized by kidneys (NSAIDs, some Abx)

Answer 12

Growth deficits, joint and bone anomalies, intellectual disability, seizures Can have cardiac defects and renal defects Underdeveloped/absent philtrum, thin vermillion border, small head, hypoplastic mandible, hypertelorism, short nose, flat midface, cleft lip/palate Malformed, missing, misaligned teeth Delayed dental development May need SBE, may need to avoid drugs processed by renal system, need good behavior management

Answer 13

Most common cause of inherited intellectual disability Seizures, mitral valve prolapse, flat feet, HTN, hearing issues, autism/ADD High palate, elongated face, macrocephaly, strabismus, large ears Malocclusion, supernumeraries May need sedation for tx due to behavior No N2O if active otitis media

Answer 14

Mutation in chromosome 5 tumor suppressor gene Multiple polyposis of large intestines with high malignant potential Osteomas, sebaceous cysts, fibromas, lipomas, other lesions Increased prevalence of thyroid carcinoma Compound odontomas, hypodontia or hyperdontia, multiple odontomas (esp. in mandible and outer cortex of skull) Increased density of bone slows ortho movements Need early screening for cancers; NSAIDS may reduce number of polyps formed

Answer 15

Various causes (weak sphincter, hernia, obesity, pulmonary disorders, epilepsy, CP, Down syndrome, prematurity) Vomiting, heartburn, poor weight gain, feeding refusal, nocturnal cough/pain, sore throat Dental erosion with enamel loss, dry mouth (medications), impaired taste Increased risk of caries Consider ortho extrusion if needed and full coverage restorations

Answer 16

Hypotrichosis, hypoplastic ribs/clavicles, small nares, congenital cataract, narrow upper airway Brachycephaly with frontal bossing, bird-like face, glossoptosis, microphthalmia Hypodontia, delayed exfoliation/eruption, natal teeth/neonatal teeth, enamel hypoplasia, class II malocclusion Difficult airway due to micrognathia; can have sleep apnea

Answer 17

A: factor VIII; B: factor IX Most common in males (x-linked recessive) Spontaneous bleeding, easy bruising, hematuria Oral bleeding, ulcerations, ecchymosis, poor OH with cavities and gingival disease Caution with IAN or lingual infiltration (hematoma risk) Endo treatment preferred over extraction Use rubber dam to protect soft tissues Consult with hematologist; be ready for hemostasis May need desmopressin (DDAVP), Amicar, factor replacement, etc. Avoid NSAIDs and aspirin

Answer 18

Target CD4 lymphocytes, leading to increased risk for opportunistic infection Early childhood usually from maternal transmission; may see arthralgia, fever, headache, lymphadenopathy, recurrent bacterial infections, candidiasis Gingivitis (can have NUG), fungal infections, viral infections (EBV), bacterial infections, neoplasms (Kaposi's sarcoma) If neutrophil <500 cell/mL; CD4 cell counts <100/mm3 defer treatment and consider antibiotic prophylaxis May process drugs differently due to antiretroviral therapy (OCS risk)

Answer 19

Unknown etiology; an be part of syndrome (Holt-Oram, Rubinstein-Taybi) Underdeveloped left ventricle of heart resulting in inability to support cardiac output needed Pounding heart, weakened pulse, cyanosis, failure to thrive, fatigue Need for SBE Cardiac meds may cause xerostomia, gingival hyperplasia Several heart surgeries required

Answer 20

Increased metabolism, arrhythmias, intolerance to heat, increased appetite, weight loss Goiter = enlarged thyroid; Graves = exophthalmos Increased caries/perio susceptibility, early exfoliation/eruption, osteoporosis, burning mouth syndrome Consult with endocrinologist, ensure thyroid collar with radiographs, caution with hemostasis (avoid NSAIDS), avoid epinephrine in untreated or poorly controlled Thyroid storm: 911, ice, hydrocortisone 100-300mg, IV glucose, CPR

Answer 21

Can be congenital (cretinism) or acquired (iodine deficiency), or autoimmune (Hashimoto's) Slow metabolic rate, weight gain, lethargy, intolerance to cold, slow HR, delayed wound healing If not treated in 6 months, short stature, intellectual disability, low muscle tone, hearing loss, respiratory distress Coarse facial features, hair loss, large head with small jaws Macroglossia, delayed dental eruption, enamel hypoplasia, salivary gland enlargement, malocclusion, impacted 2nd molars

Answer 22

Platelet count of <150mil/L ITP is primary immune, TTP is congenital More in women Petechiae/ecchymosis ITP: increased risk of bleeding from mucous membranes, hematuria TTP: thrombocytopenia, hemolytic anemia Nose bleeds, easy bruising, excessive bleeding after surgery Don't use NSAIDs Perform elective dental treatment only w/ platelet count above 50k; be ready for hemostasis Do not block for LA

Answer 23

Autoimmune disorder Persistent fever for at least 2 weeks and arthritis for at least 6 weeks in child less than 16 Joint swelling, joint pain, persistent loss of function and stiffness; usually affects knees, hands and feet Worse in morning Untreated TMJ may cause growth disturbances Increased risk for dental caries May have difficulty opening or holding toothbrush for OH Steroid use may lead to adrenal cortical insufficiency (consider supplemental steroids)

Answer 24

"Make up" appearance of face Developmental delay, possible seizures, spina bifida, short fifth fingers, heart abnormalities Microcephaly, CLP, long palpebral fissure, strabismus, arched eyebrows, prominent ears, downturning corners of mouth Missing teeth, misaligned or misshaped teeth (microdontia), class III malocclusion Cardiac consult recommended Difficult airway Aggressive caries plan

Answer 25

Vascular - conjunctivitis, rash, adenopathy, strawberry tongue, swelling hands/feet Fever more than 5 days Vasculitis can lead to coronary artery disease Self-limiting - lasts about 12 days without treatment MSIC mimics Kawasaki after COVID infection Treated in hospital with IVIG (reduces cardiovascular complications)

Answer 26

Less testosterone, slow puberty, infertility, gynecomastia Increased endocrine and autoimmune disease risk Reduced muscle tone, weaker bones, low energy, ADHD, learning difficulties Mandibular prognathism, larger teeth, taurodont molars, missing teeth High caries rate Pulp exposures due to taurodontism May be difficult to manage behavior

Answer 27

Neoplastic disease of myeloid cells Classic triad: punched out radiolucency in membranous bones, exophthalmos, diabetes insipidus Radiolucent lesions with ill-defined borders Can have pain and swelling of the face and lymph nodes Gingival ulcerations/lesions are associated with adjacent bone involvement Floating teeth may occur (premature exfoliation) Treated with chemotherapy, bisphosphonates, surgery, radiation, etc. - may need to consult with heme/onc

Answer 28

Self-mutiliation Issue with purine metabolism Almost exclusively in males (X-inked recessive) Kidney stones, renal failure, gout Intellectual disability, CP, dystonia, chorea Microcephaly, distorted facies, atrophic/scarring of lips from self-chewing Enamel hypoplasia May need protective stabilization (relaxed when restrained) May need extraction of teeth for self-injury prevention Poor life expectancy

Answer 29

Most common cancer in children Higher incidence in pt with Down Syndrome (esp. AML) ALL: most common in kids AML: difficult to treat CLL: most common in adults CML: three phases, can have Philadelphia chromosome Gingival bleeding, gingival hypertrophy, ulcers, opportunistic infections, oral pain, enlarged lymph nodes After therapy: mucositis, microdontia, agenesis of teeth, morphology issues, root disturbances, xerostomia, GVH disease Try to complete dental tx prior to cancer therapy ANC > 1000/mm3

Answer 30

Impaired wound healing and frequent soft-tissue infections Three types, different issues with leukocyte adhesion Severe gingivitis and rapidly progressing juvenile periodontitis Rapid bone loss around teeth Complete loss of permanent teeth by late adolescence Oral ulcerations that are slow healing and scarring May. need additional antibiotic therapy for dental tx Rigorous OH including CHX rinses is essential

Answer 31

Cancer predisposition syndrome Early onset of cancers (breast, skin, etc.) Soft tissue sarcomas, bone sarcomas, and brain tumors Most common cancer for kids with LFS to have is soft tissue sarcomas and bone sarcomas Careful with radiation to reduce risk of secondary radiation induced malignancies

Answer 32

Uncommon before 5 Hodgkins more common in adolescents; Non-Hodgkin more common in children Lymphadenopathy, splenomegaly, hepatomegaly, fever, night sweats, weight loss Swelling is nonpainful; bone loss can mimic periodontal disease Can have trouble swallowing Complete dental tx prior to medical tx

Answer 33

Most common site of metastasis is lungs Osteosarcoma: most common bone cancer in children, rapid growth, pain, poorly defined bone destruction lesion with mixed radiographic appearance (sunray); in maxilla usually is in posterior region, in mandible usually is in body and angle of ramus Ewing Sarcoma: several small round blue cell tumors, arises equally in bones of extremities and other parts of body; in mouth, mandible more common Try and do dental treatment prior to cancer treatment The earlier treatment occurs with less metastasis, the better prognosis

Answer 34

Chromosome 15, autosomal dominant Vision issues, cardiovascular abnormalities Tall with long, thin arms and legs, spider-like fingers, funnel chest or pigeon breast, flat feet, hypotonia Asthma, sleep apnea, sudden lung collapse Flexible joints, scoliosis Dolichocephalic face, deep palate, small lower jaw, TMJ issues Gingivitis and periodontitis, flat molars, crowded teeth, abnormal pulp chambers SBE often needed Avoid fluoroquinolones

Answer 35

3 hallmark signs: Endocrine dysfunction, Cafe-au-lait spots, Polyostotic fibrous dysplasia 90% polyostotic lesions occur in bones of skull and jaw -> multilocular radiolucent lesions, asymmetrical Often "ground glass" radiographically High caries risk, can have oral pigmentation, oligodontia, taurodontism Treated with bisphosphonates

Answer 36

Infection with COVID within 2-6 weeks prior to symptom onset Inflammation of organs/tissues Kawasaki-like and toxic-shock-like symptoms Conjunctivitis, rashes, adenopathy, strawberry tongue, swollen hands or feet or red Refer to hospital if MIS-C is suspected Defer elective dental care; may need to get platelet values and use steroid therapy Treatment is supportive care, autoinflammatory care (IVIG) Can result in long-term cardiac compromise, kidney issues, liver injury

Answer 37

Affects CN6 and CN7; other cranial nerves may also be affected Inability to suck at birth Limitations with eye movement and facial expression, difficulty speaking Autism in 30% May have lip paralysis, hypoplastic mandible, hypoplastic tongue, CLP, deformity of tongue/ear/jaw Open bite, crowding, missing teeth, CLP Increased caries risk (less food clearance due to lack of tongue movement) Risk for aspiration

Answer 38

Includes Hurler (I), Hunger (II), and Sanfilippo (III) and others Group of lysosomal storage disorders Multi-organ involvement, short stature, skeletal, joint, airway and cardiac abnormalities Intellectual disabilities Claw-like hands, joint stiffness Coarse facial features, large head with enlarged lips and mouth and tongue Hyperplasia of alveolar ridge and gingival hypertrophy Malocclusions Enamel hypoplasia, taurodontism, impacted teeth; large root chambers and short roots (easy for abscess formation) OCS and protective stabilization contraindicated due to high BMI, limited neck movement, and macroglossia/small airway

Answer 39

Several types; Duchenne most common in boys Progressive muscle weakness Can lead to inability to walk, breathing problems, scoliosis, decreased heart efficiency, swallowing problems Can have malocclusion, delayed eruption, missing teeth May receive treatment in outpatient setting if mild Consult with PCP to determine stability and involvement of other body systems Risk for malignant hyperthermia with GA Contraindicated for OCS (aspiration risk) May require aid with OH

Answer 40

Birth defects of brain, spine, spinal cord due to complications in 1st month of pregnancy Risks: poorly controlled diabetes, obesity, not enough folic acid, anti-seizure meds Spina bifida is most common, most mild form Myelomeningocele is most severe form Others: anencephaly, encephaloceles, hydrancephaly Commonly associated with CLP Increased caries risk due to poor diet, poor OH and chronic syrup-based medications Ensure comfortable positioning to avoid pressure wounds Screen for latex allergy and other food cross-reactivities

Answer 41

Includes NF1, NF2 and Schwannomatosis NF1: birth/infancy onset; pigmentation, neurofibromas on skin/brain/other, cafe-au-lait spots, increased risk for cancers, HTN, seizures, ADHD, pheochromocytoma, nodules in eyes can lead to vision loss NF2: teens/early 20s onset; benign tumors on auditory nerve, cataracts, hearing loss, balance issues Oral findings: neurofibromas, gingival hyperplasia, impacted, SN or missing teeth, macroglossia Increased risk of dental caries Minimal issues delivering care to mildly affected pt SBE may be needed if cardiac issues persist

Answer 42

Short stature, cardiac abnormalities, delayed puberty Most have normal intelligence Wide-set eyes, short neck, webbed neck, low-set ears, low hairline, macrocephaly High palate, malocclusion, micrognathia Be aware of potential bleeding disorders May need SBE for cardiac conditions

Answer 43

Goldenhar is most severe form Defects of 1st and 2nd branchial arches Symptoms commonly unilateral Pre-auricular skin tags, CNS anomalies, hearing issues, colobomas, cardiovascular malformations, autism, renal malformations Hemifacial microsomia in 60% of cases Cleft palate more common than cleft lip Can have small, misshaped, or missing ramus, condyle and TMJ Supernumerary teeth, enamel and dentin malformations, gingival hypertrophy, class II malocclusion Can have difficult airway Intellectual disability can impact OH

Answer 44

Syndactyly/brachydactyly/polydactyly/clinodactyly Polycystic kidney disease (type I), bifid hallux (type II), neurological problems, heart defects Median cleft lip/cleft palate/cleft tongue, micrognathia, high palate, tumors of tongue, wide nose, hypertelorism, alopecia Missing mandibular lateral incisors, supernumerary teeth, enamel dysplasia, malocclusion Beware of drugs metabolized by kidney If otitis media, defer N2O

Answer 45

Defective collagen causing loose joints and hypermobility Commonly mistaken for child abuse Bowing of long bones, scoliosis, multiple fractures without cause Can have hearing loss, respiratory problems 19 types; type 1 most common, type 2 lethal in-utero, type 3/4 associated with DI Blue sclera, triangular face, wormian bones in skull on radiographs, prognathic mandible Occurs with dentinogenesis imperfecta; primary teeth more severely affected Consider full coverage restorations for DI Extractions may be contraindicated if bisphosphonates used Protective stabilization contraindicated RCT contraindicated if bisphosphonate

Answer 46

Dry scaly patches on palms of hands and soles of feet at birth Nail dystrophy Sparse hair Gingivitis and periodontitis Deciduous teeth exfoliate by 4-5; permanent teeth lost by age 17 Radiographs show teeth floating in air Early extractions/bone grafts with prosth treatment No contraindication to N2O, OCS or GA or protective stabilization Systemic antibiotics may slow periodontitis; traditional periodontitis therapy may not make a difference

Answer 47

Gastrointestinal polyposis, mucocutaneous pigmentation, cancer predisposition Small, dark macules present on the lips, inside and around the mouth, near eyes and nostrils that appear during childhood and fade with age Polyps in GI system can cause obstructions, chronic bleeding, abdominal pain and can have malignant transformation Caution using N2O due to potential bowel obstruction

Answer 48

Autoinflammatory disease Onset by 5, resolves by 10 usually (can go into adulthood) Predictable 3-6 day episodes of fever, 1 or more of other 3 symptoms every 21-28 days like clockwork -minor aphthae -pharyngitis -cervical adenitis Palliative care for aphthous ulcers; can consider use of systemic or topical steroids Postpone elective dental care when pt has PFAPA episode

Answer 49

Clinodactyly - broad thumbs and big toes that bend away from other digits Hypertelorism, finger and toe abnormalities, hydrocephalus, underdeveloped midface Often normal intelligence Craniosynostosis, proptosis, small nose, high forehead, OSA Dental crowding, natal teeth sometimes Difficult intubation and airway

Answer 50

Micrognathia, cleft palate, glossoptosis Comorbidities include GERD, Stickler syndrome, trisomies, CHARGE, Moebius, velocardiofacial syndrome, Treacher-Collins among others Middle ear infections common Can also have respiratory or cardiac problems 80% have cleft palate Poor development of mandible Severe class II malocclusion, crowding, natal teeth, hypodontia, erosion from GERD Difficult to intubate Treatment includes distraction osteogenesis, positive airway pressure, tracheostomy, tongue-lip adhesion, obturators, ear tubes, speech therapy

Answer 51

Paternally derived chromosome 15 (Angelman is maternal) Initial failure to thrive reversing to significant weight gain and obesity Hypothalamic hypogonadism leads to infertility, growth hormone insufficiency and intellectual disability with behavioral issues Cardio-respiratory problems due to severe obesity Prominent forehead, strabismus, narrow face with small mandible, downturned mouth with thin upper lip Abundant and thick saliva, increase in gingivitis and candidiasis Enamel defects and erosion from GERD and bruxism High caries risk Crowding Behavioral problems are common

Answer 52

Extremely rare (100-200 people ever reported in literature) Excessive growth of a part or portion of the body that is usually asymmetric and unilateral Neurologic abnormalities including ID, seizures, vision loss, hearing loss Cerebriform connective tissue nevus, linear epidermal nevus, asymmetric overrowth of limbs, skull, lipomas, vascular malformations Dolichocephalic with down-slanting palpebral fissures, macrocephaly Increased risk for salivary gland tumors Impacted teeth, malocclusion, ectopic eruption, asymmetric enamel hypoplasia, missing teeth, asymmetric macroglossia May need antibiotic prophylaxis due to multiple orthopedic procedures (?) IAN/anesthesia landmarks may be different due to overgrowth

Answer 53

Almost exclusively in girls Normal development until 6-18 months of life, followed by regression in speech and developmental milestones Causes problems in brain function 4 criteria: partial/complete loss of purposeful hand skills; partial/complete loss of acquired spoken language, gait abnormality, stereotypic hand movements Comorbidities include prolonged QT, arrhythmias, seizures, ID Associated with bruxism, mouth breathing, digit sucking, high risk for dental trauma, self-injury, difficulty with mastication Beware of diminished response to pain when diagnosing dental conditions More frequent recalls

Answer 54

Short stature, broad thumbs and big toes, intellectual disability, hyperflexible joints, bent pinky finger Increased risk for malignant and benign tumors Microcephaly, convex nasal ridge, low-set ears, small oral opening, bifid uvula, cleft palate Dental crowding, malocclusion, enamel erosion due to GERD Talon cusps Enamel hypoplasia, screwdriver permanent incisors Advanced behavior guidance techniques usually needed

Answer 55

Lysosomal enzyme deficiency Initial signs start at 2-6 years and marked by progressively declined function with mild dementia first, followed by loss of motor function Developmental delay, uncontrollable hyperactivity and aggressive behavior Mild coarse facial features with frontal bossing, broad and thick eyebrows, depressed nasal bridge Decreased mobility of TMJ Higher prevalence of caries, gingival hyperplasia and gingivitis May have excessive pharyngeal tissues, making intubation difficult

Answer 56

Autosomal recessive Defect in hemoglobin (HbS) leading to sickled RBCs Anemia, repeated infections, jaundice, pulmonary hypertension Sickle cell crisis: acute pain/joint pain from occluded vessels leading to organ damage Malocclusion with class II tendency Petechiae, hematomas, mucosal pallor, delayed tooth eruption, spontaneous gingival bleeding, increased periodontitis risk Radiographic expansion of bone marrow and loss of trabeculation Diastemas due to expansion of bone Prophylaxis similar to AHA guidelines Paresthesia risk with >50% N2O If Hg is <7g/dL or <20% Hct, may need transfusion Dental treatment should be conservative and "stress free" Often treated with hydroxyurea but BMT is only cure

Answer 57

Autoimmune disease varying from full-blown severity to chronic low-grade symptoms In children most common presentation is persistent fever, malaise, failure to thrive Anemia, leukopenia, arthritis or arthralgia, nephritis, abdominal complaints can occur Classic malar "butterfly" rash only in 1/3 of patients Can have naso-oral ulcers -ulcers can be aphthous, honeycomb, erythematous palatal ulcers, or lupus cheilitis on lips Sjogren's syndrome, burning mouth and xerostomia potential secondary complications Need blood lab values prior to invasive treatment Oral ulcers may indicate a flare is imminent Consult rheumatologist to determine need for antibiotic prophylaxis due to increased risk for infection

Answer 58

Arise from primitive mesenchymal tissue; 10% in head and neck Rhabdomyosarcoma: most common soft tissue sarcoma, tumor of striated muscle; presentation ranges from small cutaneous nodule on face to extensive fast-growing facial swelling Ewing Sarcoma (soft tissue): mainly occur on trunk and extremities but some in head and neck Can see radiographic displacement of teeth Need to time treatment with regard to cancer treatment modalities (consult with Hem/onc)

Answer 59

Prenatal and postnatal overgrowth Learning disabilities Delay in early developmental milestones Large hands and feet Broad, prominent forehead and jaw with sparse frontotemporal hair, dolichocephaly, hypertelorism, malar flushing High arched palate, dental crowding, deep bite, missing teeth, natal teeth Be aware of potential heart and kidney issues May also have scoliosis, eye issues, HTN

Answer 60

Progressive connective tissue disorder with eyes, ears, skeleton and hypermobile joints Intelligence usually normal Early onset degenerative arthritis May have mitral valve prolapse Pierre-Robin Sequence Flat face, micrognathia, glossoptosis, split uvula, prominent eyes, low set ears Malocclusion, clefts, enamel hypoplasia Difficult airway/intubation

Answer 61

Unilateral excessive blood vessel growth on brain causing calcification and atrophy of cerebral cortex nerves Headaches, seizures, paralysis Port-wine stain Intellectual disabilities in half, ADD, mood disorders, poor social skills Hypothyroidism Can have ipsilateral gingival hemangiomas intraorally that blanch under pressure Early eruption of teeth from increased vascularity Gingival hyperplasia from anticonvulsants Can cause unilateral macroglossia and maxillary bone hypertrophy Hemostasis can be challenging GA with oral intubation, avoid deep sedation due to difficulties in airway maintenance Stress can rupture hemangiomas

Answer 62

Neuroblastoma more than 97% of sympathetic nervous system cancers Neuroblastoma arises in immature nerve cells of SNS Most common cancer of infants <1 year Most common location is adrenal glands Following therapy (similar to other cancers): tooth development/root shunting, enamel hypoplasia, high caries, mucositis, xerostomia, bleeding, changes in taste Consult with hem/onc

Answer 63

VSD, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy Most common cyanotic congenital heart defect "Tet" spells in unrepaired ToF (paroxysmal life-threatening episodes of cyanosis, rapid/deep breathing, syncope) Enamel defects/hypoplasia Stomatitis, cyanotic mucous membranes and tongue Delayed tooth eruption Need consult with cardiology (SBE, anticoagulant therapy, etc.)

Answer 64

Idiopathic etiology 2nd most common cyanotic congenital heart defect 50% also present with VSD Signs of CHF (tachypnea, dyspnea, pounding heart, cyanosis, weak pulse) Cyanotic oral tissues MD consult to determine SBE and cardiac tolerance to invasive procedures

Answer 65

Bilateral 1st and 2nd branchial arch development abnormalities Hypertelorism, down-slanting palpebral fissures, depressed cheekbones Mandibular retrognathia Large mouth Coloboma, sparse eyelashes Cleft in zygoma, palate, lip Dental anomalies - tooth agenesis, enamel deformities, widely spaced teeth Class II malocclusion, micrognathia High airway risk (sedation and N2O contraindicated)

Answer 66

Chronic granulomatous disease of lungs caused by various strains of mycobacteria Chronic cough lasting 3 weeks or longer, coughing up blood, weight loss/failure to thrive, fever for 2 weeks Oral lesions may include ulcers, nodules, tuberculomas, periapical granulomas, osteomyelitis Ulcers most common in mouth - usually painless Defer treatment when active/contagious TB is present

Answer 67

XO Broad chest, low hairline, obesity, webbed neck Thyroid disorders, heart conditions common Higher incidence of diabetes, osteoporosis Most normal intelligence Malocclusion, crowding of teeth due to small jaw and narrow high palate Early eruption of permanent teeth Thinner enamel Shallow roots Need to assess cardiac risk for SBE and assess for obstructed airway

Answer 68

Immune-mediated inflammatory disease involving granulomatous inflammation and ulcers of large intestine (IBS) Persistent diarrhea with loose, urgent bowel movements, blood in stool, abdominal pain, abdominal mass Fever, fatigue, anemia, appetite and weight loss, growth failure Similar to Crohn Disease (limited to the large intestine and colon where Crohn's is not) Increased risk of caries and oral infections Glossitis, cheilitis, cobblestoning appearance of oral mucosa Recurrent aphthous stomatitis Avoid NSAIDs and aspirin (can cause flare ups) Palliative care for oral ulcers

Answer 69

Non-random cluster of congenital malformations (at least three of the following): -Vertebral defects -Anorectal anomalies (anal atresia) -Cardiac defects -Tracheo-esophageal fistula -Renal anomalies -Limb abnormalities Variable dysmorphic facial features but no classical signs Can have bilirubin discolored teeth, gingival overgrowth, calculus MD consult to determine stability prior to dental tx including need for SBE

Answer 70

Risk factors: babies on only cow's milk, low SES, smokers, GI tract disorders, type 1 DM, patients on hemodialysis, pt with malabsorption, and pt with psychiatric disease resulting in poor nutrition Corkscrew hairs, perifollicular hemorrhage and gingival bleeding Initial presentation after 8-12 weeks of deficiency 4 H: Hemorrhage, Hyperkeratosis, Hypochondriasis, Hematologic abnormalities Poor wound healing, bone loss, stomatitis

Answer 71

X linked mutation in phosphate reglating gene that disrupts vitamin D metabolism, resulting in low vitamin D levels Short stature, slow growth, bowing of lower extremities, joint/bone pain, bone deformities and fractures Lab values include hypophosphatemia, normal calcium, normal to high PTH, normal to high alkaline phosphatase, normal or slightly reduced vitamin D Craniosynostosis, dolichocephaly and frontal bossing Thin, hypomineralized enamel and dentin with enlarged pulp chambers and pulp horns -spontaneous or attrition-caused pulp necrosis + abscess -periapical radiolucencies Early loss of teeth Aggressive preventive dentistry is necessary

Answer 72

Cardiac abnormalities Joint problems, soft and loose skin, low muscle tone Short stature, overly friendly and social, DD/learning disability Sensitive hearing, difficulty with visual Phobias common Small upturned nose, broad forehead, wide mouth, full lips, small chin, puffy eyes Small, widely spaced teeth, missing teeth, taurodontism, pulp stones, enamel hypoplasia Caution when administering LA as sudden death has occurred Consider headphones/earplugs due to sensitive hearing Avoid vitamin D and calcium supplements May have malignant hyperthermia with GA SBE may be needed for cardiac anomalies

Answer 73

Atypical electrical activity of heart Shortened PR interval and widened QRS complex Majority are asymptomatic but can have symptomatic arrhythmias with lightheadedness, dizziness, syncope, chest pain, sudden cardiac arrest No evidence of specific dental manifestations but some antiarrhythmics can cause oral manifestations Verapamil, enalapril and diltiazem can cause gingival hyperplasia Need to keep the patient relaxed and use good behavior management techniques to avoid increasing heart rate Not considered SBE requirement, but may want to consult with cardiology Avoid use of some electrical stimulating equipment (piezo-surgery, radiofrequency scalpel, etc.)

Weebly Study Guide Flashcards

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