Path 2 Flashcards
What causes problems in babies in embryo?
Teratogens
Definition of teratogen:
Any agent that can disturb the development of an embryo or fetus. Teratogens may cause a birth defect in
the child. Or a teratogen may halt the pregnancy outright.
Muscular dystrophy:
group of muscle diseases that weaken the musculoskeletal system & hamper locomotion. Characterized by progressive
skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue.
- Muscular dystrophy: muscle weakness, “long face” which is characterized by a lower vertical facial height and open bite/
Muscle dystrophy; after local anesthetic is most likely due to? Lidocaine toxicity, increase duration of action, increase onset, Can’t
be supine
Lidocaine toxicity,
QUESTION: Pt w/ muscular dystrophy condition:
lower face with open bite
What can be seen on a patient with muscle weakness of the face? Cross bite, buccal tilting of molars, long upper face, lower face
with open bite
lower face
with open bite
Considerations for muscular dystrophy: increase in dental disease if OHI is neglected, weakness of muscles of mastication
weakness of muscles of mastication
decrease biting force, open mouth breathing
Addison’s disease
primary adrenal insufficiency
chronic endocrine disorder, adrenal glands do not produce
enough steroid hormones (too little cortisol & sometimes, insufficient aldosterone).
- Symptoms generally come slowly & include abdominal pain, weakness, skin darkening and weight loss.
- Adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness. An adrenal crisis can be triggered
by stress, such as from an injury, surgery, or infection. - Tx: give cortisol
Acute adrenal insufficiency:
hypotension
QUESTION: What Addison disease causes in mouth:
pigmentation of the mucosa
Pheochromocytoma:
neuroendocrine tumor in medulla of adrenal gland à excess catecholamines (ex. epi)
Cerebral palsy (CP):
group of permanent central motor/movement disorders that appear in early childhood, caused by abnormal development
or damage to the parts of the brain that control movement, balance, muscle tone, and posture. Signs and symptoms vary & include: poor
coordination, stiff muscles, weak muscles, and tremors. Other problems w/ sensation, vision, hearing, swallowing, and speaking.
QUESTION: CP patient - which is not true?
a. 95% have cognitive impairment
b. all bruxism
c. increase in periodontitis
c. increase in periodontitis
Cerebral palsy during treatment.
– patient will have spastic oral mucosa
Pt has involuntary uncoordinated movements with larynx problem?
Cerebral palsy
Common finding in a patient with cerebral athetoid palsy:
Anterior Teeth fracture
- cerebral athetoid palsy: damage to basal ganglia, has both hypertonia/hypotonia
Case: Black girl around 7 years old presents with unilateral cross bite; she had a cleft palate that was fixed. Palate in picture looks
like a triangle and laterals are towards the palate.
A) What is the pigmentation?
B) What is the most likely cause of the crossbite?
racial pigmentation
early loss of laterals, due to cleft palate
When does cleft lip and palate develop?
6-9 weeks in utero
QUESTION: Patients with cleft lip and palate, what occlusion is mostly seen?
class III malocclusion
QUESTION: Cleft lip is more common in ; cleft palate more common in .
boys
girls
QUESTION: Pt had cleft lip and palate. Later in life during ortho analysis, what do you see?
Deficient maxilla, normal mand
Most prevalent developmental deformity in Maxilla?
Cleft Palate
What is more commonly seen? o Amelogenesis imperfect o Ectodermal dysplasia o Dentinogenesis imperfect o Cleft lip and palate
o Cleft lip and palate
What is cleft palate class III?
Soft & hard palate plus alveolar process
What mostly gives cleft lip/palate? Genetic, autosomal dominant, autosomal recessive, environmental, multi-factorial
multi-factorial
All of the following are the reasons for closing a cleft lip except?
Support the premax on a unilat cleft
Help speech
Support the ala of the nose.
Support the ala of the nose.
Speech problems associated with cleft lip and palate are usually the result of?
Inability of soft palate to close air flow into the nasal
area
QUESTION: A cleft lip occurs following the failure of permanent union between which of the following?
A. The palatine processes
B. The maxillary processes
C. The palatine process with the frontonasal process
D. The maxillary process with the palatine process
E. The maxillary process with the frontonasal process
E. The maxillary process with the frontonasal process
Age for repair of cleft palate w/ normal canine eruption:
When canine tooth is 3⁄4 formed (8-9years old)
When correcting cleft problem, how do you end/finish?
Suturing lip
Percentage of cleft lip and cleft palate in Caucasians?
1/750
- Asians = 1/500, Asians have it the most common
Incident of cleft palate & lip in US - 1 in ____ vs Incident of cleft palate w/out lip in US 1 in ____ –
palate & lip 1 in 1000
palate without lip 1/2000 (CDC 2012)
QUESTION: What surgery will a pt with cleft palate most likely need?
(mandibular set back)
- Pt get cleft lip & palate surgery. This usually cases future Class III tissues so at later age, they need to come back to move the mandible to
correct Class III (mandibular setback)
How does a kid with fetal alcohol syndrome present with? anencephaly, midface deficiency, cleft lip
midface deficiency
Treacher Collins inheritance
AUTOSOMAL DOMINANT
midface deficienct
weird ears
aka mandibulofacial dysostosis
downslanted eyes
cleft palate, malocclusion, anterior open bite, enamel hypoplasia
Which disorder has the least developmental delay?
Treacher Collins syndrome
Treacher Collins has loss (hypoplasia) of _____. What do patients with cleidocranial dysplasia have?
Treacher Collins: zygomatic bone
Cleidocranial: Loss of clavicle
Describes patient saying they have mandibular hypoplasia, malformed ear, lower eyelids, ear pinna –
Treacher Collins
Treacher Collins syndrome à
know pt’s are not mentally retarded and they have ear abnormalities
Down’s higher risk of
perio dz
NOT CARIES
Down’s which third of face is affected
midfacial hypoplasia
What orthomanifcastion does Turner syndrome and trisomy 21 associated with?
short midface
What is orbital hypertelorism?
Wide-set eyes (seen in Crouzon, Cleidocranial dysostotosis, GOrlin Syndrome, Down’s syndrome)
Hypertelorism definition:
Increased distance between eyes, or other body parts
Which does NOT result in delayed development? Trisomy 21, Trisomy 18, Hurler Syndrome, Cri du Chat
Trisomy 18 (Edward syndrome)
- Edward’s syndrome: small head (microcephaly) accompanied by a prominent back portion of the head (occiput), low-set, malformed ears,
abnormally small jaw (micrognathia), cleft lip/cleft palate, upturned nose, narrow eyelid folds (palpebral fissures), widely spaced eyes
(ocular hypertelorism)
What resembles epiphyseal plate?
Synchondrosis
- Synchondrosis: almost immovable joint between bones bound by layer of cartilage (ex. vertebra,
epiphyseal growth plate)
What age does the mandibular symphysis close?
6-9 months
Symphysis: two flat bones grow together & join
Sphenooccipital closure, what kind of tissue fills it in?
Cartilage
Interstitial growth – occurs by the mitotic division and deposition of more matrix around chondrocytes already established in the
cartilage.
- Ex – CONDYLE, nasal septum, and spheno-occipital snychondrosis
Which of these undergo suture closure latest? • sphenoethmoidal • Sphenoccipital • Intrasphenoid • Intraoccipital
Sphenoccipital
What is synostosis?
ABNORMAL FUSION OF BONES
What is craniosynostosis?
Early closure of suture between bones
Crouzon syndrome
Autosomal dominant, 1st branchial arch syndrome, mutation in fibroblast growth factor
receptor II à fibrous joints between certain bones of the skull (cranial sutures) close prematurely (craniosynostosis).
characteristic x ray of Crouzon
beaten metal
how does crouzon present
Most notable characteristic of Crouzon syndrome is cranial synostosis, but it usually presents as
brachycephaly, which results in the appearance of a short and broad head, exophthalmos or
proptosis (bulging eyes due to shallow eye sockets after early fusion of surrounding bones),
hypertelorism (greater than normal distance between the eyes), hypoplastic maxillary, & mandibular
prognathism
Fuzzy radiograph!
Synostosis – early/late closing of sutures - which syndrome
Crouzon syndrome
Patient w/ deficient mid-face, proptosis, etc?
Crouzon syndrome
Pt has ocular proptosis, maxillary hypoplasia, premature suture closing (synostosis)? treacher-collins Crouzon Pierre robin cleidocranial
Crouzon
Hurler and Hunter syndromes class
Both are lysosomal storage disease (MCUOPOLYSACCHARIDOSIS)
hurler inheritance
mucopolysacch type I, gargoylism
Autosomal recessive
buildup of GAGs
deficienct of alpha-L
heparin sulfate and dermatan sulfate
hunters syndrome inheritance
mucopoluysacch 2 X-linked recessive defect in achoring between epidermis and dermis friction and skin fragility iduronate-2-sulfatase --> GAG buildup
Hurler and Hunter’s syndromes, what do they have in common?
They both have mucopolysaccaridosis & buildup of GAGs
Cleidocranial dysostosis =
hereditary congenital disorder (usually
autosomal dominant), where there is delayed ossification of midline
structures. Bone defects usually involve clavicle (hypoplastic or aplastic clavicle) & skull. Short, big head, shoulders moved in.
Dental – narrow high palate, increased rate of cleft palate, presence of many unerupted permanent, retained primary, & supranumery
teeth w/ distorted crowns/root shape
What is the most significant finding in cleidocranial dysplasia? odontoma, supernumery teeth, sparse hair, multiple impacted teeth,
retained teeth
supernumery teeth
Cleidocrainal dysplasis in mouth–
supernumerary teeth & problems with eruption
QUESTION: Cleidocranial syndrome: x ray with
absence of clavicle
Which will give you very narrow facial structures and delayed eruption of permanent teeth?
- cleidocranial syndrome
- downs syndrome
• cleidocranial syndrome
What allows for compression of skull during birth?
Fontanelles
What is the part of the infant’s head that allows it to change shape?
Fontanelles (enable
the bony plates of the skull to flex
- Fontanelles close anterior 12-18 months, posterior 3-4 months
What is the part of the infant’s head that allows it to change shape?
Fontanelles
Which structures in a baby allow the head to deform in the birth canal?
fontanelles
Papillon–Lefèvre syndrome (PLS) -
palmoplantar keratoderma w/ periodontitis. It’s an
autosomal recessive disorder caused by a deficiency in cathepsin C.
- Severe perio à early/young loss of primary & permanent teeth after eruption of 1st
molar
- Hyperkeratosis of palm & feet sole
15 yr. old w/ edentulous and keratosis on hands and feet. They gave ugly
picture of thick soled feet. à
Papillon Lefevre syndrome
What is Papillon–Lefèvre syndrome?
You get periodontitis, keratosis on hands
and soles, and premature loss of primary teeth.
Hyperkeratosis in hands and feet –
Papillon-Lefevre Syndrome
Pierre Robin Syndrome:
Pierre Robin Syndrome:
Pt has glossoptosis, micrognathia, and cleft palate?
Pierre Robin syndrome
- Glossoptosis = refers to the downward displacement or retraction of the tongue
Sturge-Weber syndrome:
neurological disorder present at birth. Characterized by a port-wine stain on the face and
brain or eye abnormalities due to overabundance of capillaries near skin surface. Sometimes seizures or neurological
symptoms
Sturge-Weber syndrome?
vascular malformation, eye and hemangioma
Portwine stain
Sturge-Weber
Angiomatosis of leptomeninges
Pt has high cholesterol, hypertension and diabetes, metabolic problem, which does he have:
metabolic syndrome
Pt has BMI of 36, is overweight & has high cholesterol. What syndrome?
Metabolic Syndrome
QUESTION: What is the normal % fat intake per day-
30%
- Recommended daily dose of fat: 30% of total calorie and saturated fat is 10% of daily calorie intake
Which is not endocrine gland? Parathyroid, thyroid, adrenal, parotid
parotid
Which do you give a hyperparathyroid child for normal development of teeth?
Vitamin D (Brings in Ca+)
Thyrotoxicosis symptoms –
DIAPHORESIS (sweating), fever, hypertension and TACHYCARDIA
- thyroid storm is a severe version of thyrotoxicosis
Thyrotoxic pt. manifestation?
Tachycardia
QUESTION: LA with epinephrine contraindicated in? Uncontrolled Diabetes, hypothyroidism, hyperthyroidism
hyperthyroidism
Symptoms of hypothyroid attack:
loss of brain function due to severe, longstanding low levels of thyroid hormone in the blood
(hypothyroidism)
- Hypothyroidism primary symptoms are altered mental status & hypothermia. Hypoglycemia, hypotension, hyponatremia, hypercapnia,
hypoxia, bradycardia, hypoventilation may also occur.
Myxedema is due to?
severe hypothyroidism
- Myxedema: swelling of skin, waxy consistency
With which endocrine systemic disease does thick hair become thin hair?
thyroid – hypothyroidism (cretinism in kids and
myxoedema in adults)
PT was gaining weight, has lower voice, fine hair, feels cold –
hypothyroidism
- symptoms of hypothyroidism: weight gain, bradycardia, cool to touch/cold, fatigue
QUESTION: Which thyroid drug doesn’t let iodine bond to hormone?
Radiated Iodide (for hyperthyroidism)
QUESTION: Graves’ Disease
(Hyperthyroidism) -
exophthalmos
Thyroid hormone decrease, which drug do you give?
Levothyroxine (for hypothyroidism)
Hypophosphatasia -
metabolic bone disease, low alkaline phosphatase
Increase in alkaline phosphatase is related to?
Hyperparathyroidism
Decreased alkaline phosphatase is related to:
Hypophosphatasia
What disease causes decrease in alkaline phosphatase? Malnutrition, hypophosphatasia, hypothyroidism, pernicious anemia
hypophosphatasia
Central Giant Cell Granuloma is seen with pts with which condition?
Hyperparathyroidism
Osteoporosis is associated with which of the following diseases?
Hyperparathyroidism
