Syndromes & Diseases: A Comprehensive Guide for Dental Professionals, Exams of Nursing

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ABPD - Syndromes & Diseases

Cleft Lip & Palate Incidence - correct answer ✔1 in 700 (same as downs, neural tube) CL - is less, 20% CP - 30% CL+CP - 50% Pierre Robin Sequence - correct answer ✔Glossoptosis, micrognathia, cleft palate & 15-25% have heart disease. Mand growth can progress normally. Mandibulofacial Dysostosis (Treacher Collins) - correct answer ✔*1st branchial arch, pouch, groove Down sloping palpebral fissures Depressed cheek bones Deformed pinnae Receding chin Large "fish-like" mouth Hypoplastic Mandible + 30% CP Cleidocranial Dysplasia - correct answer ✔Brachycephalic (short face) w/ frontal boss Depressed nasal bridge Delayed closure of sutures/fontanels Extra teeth, delayed exfoliation/eruption Clavicular defect

Oro-facial-digital syndrome - correct answer ✔Hypoplastic alar cartilage Hypotrichosis (hair) Brachycephalic Cleft tongue and cleft palate Syndactly Hyperplastic frenum w/ clefts Hypo or hyperdontia Hamartomas on tongue Apert Syndrome (Craniosynostosis) - correct answer ✔Syndactyly Turribrachycephaly (point) Ocular hypertelorism (far apart) Cleft Palate (30%) Intellectual disability Crowded teeth, delayed eruption, v-shaped max, class III Crouzon Syndrome (Craniosynostosis) - correct answer ✔Ocluar hypertelorism (far apart) Exophthalmia Normal intelligence CL + CP Crowded teeth, delayed eruption, v-shaped max, max def Pfeiffer Syndrome

(Craniosynostosis) - correct answer ✔Ocular hypertelorism Exophthalmia Normal intelligence Broad thumbs and great toes Carpenter Syndrome (Craniosynostosis) - correct answer ✔Acrocephaly (cone) Soft tissue syndactyly Congenital heart disease Intellectual Disability Hypo-genitalism Obese Acrocephaly AKA... - correct answer ✔Oxycephaly Turricephaly Achondroplasia (Dwarfism) - correct answer ✔80% sporatic, mutations, AD 1-20,000 live births Short limbs, large head Depressed nasal bridge Lordotic lumbar spine Hypopituitarism (Dwarfism) - correct answer ✔Well proportioned body Fine, silky hair

Wrinkled atrophic skin Hypogonadism Eruption/exfoliation delayed Malocclusion (small arch) Mucopolysaccharidosis - correct answer ✔Hunter/Hurler

• all involve some form of dwarfism Chondroectodermal dysplasia (Ellis Van Creveld Syndrome) (Dwarfism) - correct answer ✔Bilateral polydactyly (extra pinky) Cardiac defects (40-50%) Chondrodysplasia (bad cartilage) Hydrotic ED Natal teeth (25%) Hallermann-Streiff Syndrome (Dwarfism) - correct answer ✔Dyscephaly Thin, beaked nose Mand hypoplasia Hypotrichosis Small palpebral fissures Cateracts High palatal vault, hypodontia, natal teeth, over-retained primary, supernumerary

Turner Syndrome (Dwarfism) - correct answer ✔NCIS LA lady Missing X chrom (XO, females only) 45 chromosomes (instead of 46) Normal IQ, sterile Coarc Aorta Webbed neck Enamel Hypoplasia Osteogenesis Imperfecta (Dwarfism) - correct answer ✔Type 1 (most common, mildest), AD Fractured bones, hypermobile joints Hearing loss Blue sclera Dentinogenesis Imperfecta Often on Bisphosphonate tx Hypothyroidism (Dwarfism) - correct answer ✔Dysfunctional thyroid or tumor Dry skin Swollen face/extremities Bradycardia + hypothermia Delayed/failure to erupt Cretinism (Dwarfism) - correct answer ✔Hypothyroidism in infancy

Klinefelter Syndrome - correct answer ✔XXY (males w/ female characteristics) Hypogonadism, Low T, often sterile Lesch Nyhan Syndrome - correct answer ✔X-linked recessive (boys) Too much uric acid Neurologic dysfunction w/ behavior issues Aggressive, self-mutilating CP (spastic) Williams Syndrome - correct answer ✔Excessively Friendly, Elf-like (upturned nose) Wide mouth, small chin Learning Disability Small, widely spaced teeth Small, low wt Heart defect common DiGeorge Syndrome (Velocardiofacial Syndrome) - correct answer ✔Heart defects Poor immune system Cleft Palate (69%) Nasally voice Developmentally Delayed - global Goldenhar Syndrome

(Occluo-auriclo-vertebral syndrome) - correct answer ✔HEMIFACIAL MICROSOMIA Incomplete development of... Ears, eyes, nose, soft palate, lip and mandible Strabismus Heart/kidney/lung problems Prader-Willi - correct answer ✔MR - developmentally delayed Obese Hypogonadism Hypotonia Short stature Cornelia de Lange Syndrome - correct answer ✔Developmentally Delayed Arched eyebrows, unibrow Low set ears Small, widely spaced teeth Agressive, self-injurous Excessive body hair (hypertrichosis) Microcephally Cleft Palate Seizures and heart defects Cri-du-chat - correct answer ✔"Cats cry" - chromosome deletion Developmentally Delayed Microcephally

Hypotonia Hypertelorism (wide set eyes) Down Syndrome - correct answer ✔Incidence same as CL/CP Developmentally Delayed Up slanting palpebral fissures Congenital Heart defects Macroglossia Excessive skin on back of neck High periodontal disease, low caries High palate Small or missing teeth Delayed eruption, both Apert Syndrome - correct answer ✔ 4 diseases that can cause premature exfoliation of primary teeth... - correct answer ✔1. Neutropenia

2. Hypophosphotasia
3. Papillon-Lefevre
4. Histiocytosis X (aka Eosinophilic granuloma) Gardner Syndrome - correct answer ✔Multiple osteomas, multiple polyps of large intestines, supernumerary teeth What four disorders associated with bone often present with an "onion peel" radiopacity? - correct answer ✔1. Histiocytosis (LCH)

2. Ewing Sarcoma
3. Garre's osteomyelitis (proliferative periostitis)
4. Osteosarcoma Syndromes associated with supernumerary teeth... - correct answer ✔1. Apert
5. Cleidocranial dysplasia
6. Gardner
7. Crouzon
8. Down Syndrome Syndromes associated with hypodontia... - correct answer ✔1. Ectodermal dysplasia
9. Crouzon (also hyperdontia)
10. Achondroplasia
11. Ellis van Crevald Syndromes associated with microdontia... - correct answer ✔1. Ectodermal dysplasia
12. Hemifacial microsomia
13. Ellis van Crevald
14. Down Syndrome
15. Crouzon
16. Pituitary dwarfism Syndromes associated with taurodontism... - correct answer ✔1. Klinefelter (30%)

2. Tricho-dento-osseous syndrome
3. Ectodermal Dysplasia
4. AI type IV
5. Down Syndrome
6. Williams syndrome Hypothyroidism in infancy (congenital or primary)... - correct answer ✔Cretinism Most common acquired (autoimmune) hypothyroidism after age of 8... - correct answer ✔Hashimoto Thyroiditis Common blood glucose measurements for a child with Type I Diabetes. - correct answer ✔HbA1C - ~ 7% Fasting blood glucose - 100 - 126mg/dl Cardinal Sx of Type I Diabetes - correct answer ✔polyuria, polydipsia, polyphagia Most likely issue to arise in dental office with a child with Type I Diabetes - correct answer ✔HYPOglycemia (problems arise with a blood glucose < 50mg/dl) - due to stress! Outcome of hypothyroidism in children - correct answer ✔Delayed mentally as well as physically Common oral findings of hypothyroidism - correct answer ✔Delayed eruption/exfoliation

Most common HYPERthyroidism in children (hypermetabolic state due to excess TH) - correct answer ✔Graves Disease Dental related caution of hyperthyroidism - correct answer ✔Epinepherine Common oral findings of hyperthyroidism - correct answer ✔Early eruption/exfoliation Oral findings of hyperparathyroidism - correct answer ✔Ground Glass appearance Loss of lamina dura Jaw lesions (brown tumors) What are the primary causes of hyperparathyroidism? - correct answer ✔Iatrogenic removal during surgery Radiation Di George Syndrome* Dental findings of hyperparathyroidism - correct answer ✔Enamel defects Dentin Dysplasia Delayed eruption Short blunted roots Inherited deficiency of Factor VIII, long PTT (intrensic) w/ normal PT (extrensic), limited to males - correct answer ✔Hemophilia A Inherited deficiency of Factor IX - correct answer ✔Hemophilia B

Most common bleeding disorer - Inherited deficiency of carrier protein for Factor VIII, treated with desmopressin - correct answer ✔vW disease Normal ANC levels - correct answer ✔1,500 - 8,000 (below 2k is concerning) ANC level at which antibiotic coverage is necessary - correct answer ✔<1,000 (some authors say under 2,000) - defer elective care Platelet level at which elective dental care should be postponed? - correct answer ✔<40, Following HCT, how long should elective dental care be postponed? - correct answer ✔9-12months Most common emergency situation encountered in dental office? - correct answer ✔Syncope Treatment of mild allergic or anaphylactic reaction.. - correct answer ✔Diphenhydramine 1-2mg/kg PO Treatment of severe anaphylactic reaction... - correct answer ✔Epinephrine 0.01mg/kg IM q10min Diphenhydramine 1-2mg/kg IM Corticosteriod 1-2mg/kg IM Definciency in C1 esterase inhibitor - correct answer ✔Hereditary Angioedema

Latex allergy is common in this disease - correct answer ✔Spina Bifida w/ or w/o myomeningocele Mild, self-limiting, recurring mucocutaneous reaction characterized by target- lesions. - correct answer ✔Erythema Multiforme