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Down syndrome
Trisomy 21
Underdeveloped Midfacial region
Class 3 small maxilla
Open bite, mouth breather, large tongue, cracked tongue and lips
Missing primary and permanent teeth or delayed eruption
low chance of decay, high chance of periodontal disease
Atrial septal defect
Apert syndrome
M/C craniosynostotic syndrome + syndactyly
Autosomal dominant
Nursing bottle syndrome, (BBTD) baby bottle tooth decay , ECC
Most affected teeth maxi ant. > maxi post. > mandi post. > mandi ant.
Streptococcus mutans
Hyperdontia
Hypodontia
Excess in tooth number
Deficiency in tooth number
Van der woude syndrome
Clefs + lip pits
Melkersson-rosenthal syndrome
Fissure tongue + facial paralysis + granulomatous cheilitis
Sturge - weber syndrome
Angiomas of leptomeninges (arachnoid & pia mater) + skin along the distribution of the trigeminal nerve
Reye’s syndrome
Cause by Aspirin
Rare
Confution, brain swelling & liver damage
Osler-weber-Rendu syndrome
Hereditary Hemorrhagic Telangiectasia
TIE
Telangiectasia
Iron deficiency
Epistaxis
Tx: Bevacizumab
Albergs-schonberg disease or marble bone disease
Osteopetrosis
Dense bone
Brittle & prone to fracture
Autosomal dominant or recessive
Tx: prevent caries and extraction Sx for severe deformity
Peutz - jeghers syndrome (PJS)
Pigmented (melanotic macules) lips/mouth and Jejunal(intestinal) polyp
Colon cancer risk
Sjogren syndrome
Sicca syndrome
Keratoconjuctivits sicca/xeropthalmia (dry eyes)
And xerostomia (dry mouth)
Ramsay Hunt syndrome
HZV reactivation in geniculate ganglion impact on CN 7 & 8 leading to facial paralysis, vertigo & deafness
Tx: Acyclovir
Behcet’s syndrome
Multi system vasculitis
Ulcer in oral , genitals and eye inflammation
Knee inflammation
Swelling and tender ant and post neck
Plummer vinson syndrome
Mucosal Atrophy, dysphasia, iron deficiency anemia
Risk factor of OSCC
The Plumber has a MAD IDeA
Steven-Johnson Syndrome
Toxic epidermal necrolysis
Life threatening blisters and necrotic skin with febrile prodrome oral & ocular involvement
Due to medication
D/D: erythema multiform
Fibroma
Aka hyperplastic scar or traumatic or irritation fibroma
Chronic trauma
M/C connective tissue (CT) tumor
Fibromatosis
Benign,,, aggressive,, high recurrence,, difficult to eliminate
Neoplasm of fibroblasts
Lofgren's syndrome
LANA
Lofgren syndrome, Arthritis, erythema Nodosum, bilateral hilarious lymphAdenopathy
Tx: corticosteroids
Heerford's syndrome
HAPPy
Heerford's, Anterior uveitis, Parotid enlargement, facial nerve Palsy
Tx: corticosteroids
McCune-Albright syndrome (MAS)
Polyostic fibrous dysplasia (Multiple bone)
Cutaneous cafe au lait Spots
Endocrine Abnormalities (precocious puberty)
Goldenhar syndrome
Affect eye, ear, and spine Also some facial structure
Usually unilateral
Digeorge Syndrome
CATCH-22
Cheomosome 22 deletion
Defective neural crest cells , pharyngeal pouches 3&4 (impairment in developing Thymus and Parathyroid glands)
Cardiac abnormalities
Abnormal facies
Cleft palate
Hypocalcemia