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.. DD ORAL PATHOLOGY .. > Metabolic & Genetic Jaw Diseases > Flashcards

Metabolic & Genetic Jaw Diseases Flashcards

1
Q

Cleft palate usually occurs starting in the _____ week of embryonic life whereas cleft lip usually issues starting in the ______ week.

A

8th week, 6th week

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2
Q

A child with achondroplasia has a:

A

Relatively normal torso and short arms and legs

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3
Q

Type I dentinogenesis imperfecta is associated with which genetic disease?

A

Osteogenesis imperfecta

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4
Q

Clinical characteristic of OI: extreme fragility and porosity of the bones with proneness to fracture. Other features?:

A

Pale blue sclera
Deafness due to otosclerosis
Abnormal teeth
Loose joints
Low muscle tone
Triangular face
Tendency toward spinal curvature

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5
Q

Most common and mildest form of OI; blue sclera throughout life

A

Type I OI

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6
Q

Most severe form resulting in multiple fractures just from birth process; not compatible with life

A

Type II OI

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7
Q

Most severe form beyond the perinatal period

A

Type III OI

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8
Q

Mild to moderately severe bone fragility

A

Type IV OI

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9
Q

Hyphosphatasia is a genetic metabolic disorder of bone mineralization caused by a deficiency in:

A

Alkaline phosphatase

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10
Q

An enzyme that is essential to the production of bone tissue.

A

Alkaline phosphatase

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11
Q

Teeth display enlarged pulp chambers and pulp canals, deficient root development as well as alveolar bone loss.

A

Hypophosphatasia

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12
Q

Pituitary adenoma in 9 year old will most likely lead to:

A

Gigantism

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13
Q

Tumor prior to adolescence (nonfusion of epiphyses)

A

Gigantism

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14
Q

Tumor after adolescence (fusion of epiphyses)

A

Acromegaly

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15
Q

X-linked recessive condition characterized by abnormal development of the skin and associated structures (hair, nails, teeth and sweat glands). Involves all structures that are derived from the ectoderm.

A

Ectodermal dysplasia

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16
Q

Clinical findings include:

Hypotrichosis
Anhidrosis ( no sweat glands leading to heat intolerance)
Depressed bridge of nose
Lack of sweat glands
Child appears much older than what she is

A

Ectodermal dysplasia

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17
Q

Characterized by: hypoplasia or aplasia of the clavicles, cranial bossing, ocular hypertelorism, retained primary teeth, malaligned teeth, presence of multiple supernumerary teeth and unerupted teeth

A

Cleidocranial dysplasia

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18
Q

An inherited disorder that presents the following in the neonates: severe micrognathia, mandibular hypoplasia, severe glossoptosis, and high arched or cleft palate; also characterized by respiratory problems.

A

Pierre Robin Syndrome

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19
Q

Marked by hyperpigmentation of the lips and sometimes other parts of the face, hands and feet followed by the development of benign polyps called hamartomas throughout the intestines (small intestine); risk of malignancy is 18x higher than normal.

A

Peutz-Jeghers Syndrome

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20
Q

Other name for osteopetrosis

A

Albers-Schonberg disease or Marble bone disease

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21
Q

An absence of physiologic bone resorption owing to reduced osteoclastic activity. Manifests itself in skeletal disturbances including bone cavity occlusion, decreased hematopoietic activity and growth retardation. Bone pain and fractures are the most common.

A

Osteopetrosis

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22
Q

Signs and symptoms of Paget’s disease:

A

Hypercementosis
Enlarged cranium
Headaches
Hearing loss

23
Q

Also called as osteitis deformans

A

Paget’s disease of bone

24
Q

Name this disease:

Maxilla is affected more than mandible
Pt. Complains Max. Denture Not fitting
Hypercementosis of tooth roots
Increasing size of hats
“Cotton wool” appearance of rx.
Increased serum alkaline phosphatase
Tx:?

A

Paget’s disease of bone

Calcitonin and bisphosphonates

25
Q

This condition exhibits all of the ff. Characteristics:

Histologically close to central giant cell granuloma
Clinically presents as non-tender swelllings
Tx. is frequent monitoring
Most cases occur in mandible
Affects males than females

A

Cherubism

26
Q

Pathognomonic for cherubism

A

Perivascular collagen cuffing

27
Q

Radiograohic sign for cherubism

A

Multiple, well-defined multilocular radiolucencies

28
Q

A deficiency of parathyroid hormone can be treated with:

A

Vitamin D

29
Q

Most important consequence of hypoparathyroidism; ionized calcium level drops to less than 2.5-3mg/100 mL.

A

Hypocalcemia

30
Q

Clinical manifestation of hypocalcemia?

A

Tetany

31
Q

Distinct characteristic of hypoparathyroidism?

A

Chvostek sign (twitching of facial muscles)

32
Q

Dental manifestation of hypoparathyroidism?

A

Delayed eruption
Pitting enamel hypoplasia
Blunted root apices

Tx: vitamin D

33
Q

Graves’ disease is the most acute and severe form of hyperthyroidism caused by the autoimmune disease targeting which receptor?

A

TSH receptor

34
Q

One or more nodules or lumps in the thyroid may become overactive, a condition known as:

A

Nodular goiter or Plummer disease

35
Q

Its primary role is to stimulate cellular metabolism, growth and differentiation of all tissues.

A

Thyroxin

36
Q

Most common form of hyperthyroidism occurs most frequently in women under 50

A

Grave’s disease

Manifests:
Goiter
Exophthalmos
Thickened skin over shin area

37
Q

Toxic nodular goiter; effects both genders usually over 50

A

Plummer disease

Manifests:
Exophthalmos
May present with only cardiac disease

38
Q

Three main types of thyroiditis:

A

Hashimoto’s
Subacute granulomatous
Silent lymphocytic

39
Q

Severe hypothyroidism is called ______ in children, whereas it is called ______ in adults.

A

Cretinism, myxedema

40
Q

Dental findings in child with hypothyroidism:

A

Underdeveloped mandible with an overdeveloped maxilla
Enlarged tongue (leads to malocclusion)
Delayed eruption of teeth
Deciduous teeth being retained longer

41
Q

The clinical feature of the primary form of which disease is classically described as “stones, bones, groans, and moans?”

A

Hyperparathyroidism

42
Q

Patients with hyperparathyroidism caused by renal failure (renal osteodystrophy) may develop remarkable jaw enlargement with a ________ rx appearance.

A

Ground-glass

43
Q

Histological feature of hyperparathyroidism?

A

Multinucleated giant cells
Hemosidirin
Extravasated rbc

44
Q

Patients with fibrosis of the brown tumors may experience the most severe manifestation in the skeleton termed:

A

Osteitis fibrosca cystica (Von Recklinghausen’s disease of bone)

45
Q

Difficulty with mastication and swallowing
Higher incidence of periodontal diseases and caries
Attrition of the teeth

Are manifestations of what condition?

A

Cerebral palsy

46
Q

Oral manifestations of Down syndrome:

A

Mandibular prognathism
Increased incidence of periodontal disease
Delayed eruption of teeth
Higher incidence of congenitally missing teeth
Malooclusion
Enamel dysplasia

47
Q

What disease is associated with a decrease in caries ?

A

Cystic fibrosis

48
Q

A congenital metabolic disorder that causes the exocrine glands to produce abnormal secretions, resulting in several symptoms.. the most important of which affect the digestive tract and lungs.

A

Cystic fibrosis

49
Q

Symptoms of Cystic Fibrosis?

A

Poor growth despite good appetite
Malabsorption and foul, bulky stools (steatorrhea)
Chronic bronchitis with cough
Recurrent pneumonia
Clubbing of fingers and toes
Barrel-chested appearance

50
Q

Where in the oral cavity is malignant melanoma most commonly found?

A

Palate and maxillary gingiva

51
Q

Most malignant skin cancer?

A

Melanoma

52
Q

A malignant, epithelial cel tumor that characteristically begins as papule and enlarges peripherally, developing a central that erodes, crusts, and bleeds.

A

Basal cell carcinoma

53
Q

BCC primary cause:

A

Sun or x-ray exposure

54
Q

Risk factors of BCC:

A

Childhood sun exposure
Blistering sunburns
Fair skin
Blue eyes
Blonde
Red hair

.. DD ORAL PATHOLOGY .. (6 decks)

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