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ORAL PATH > METABOLIC DISORDERS Part 2 > Flashcards

METABOLIC DISORDERS Part 2 Flashcards

1
Q

a very rare disease described by Huntchinson in
1886

A

Progeria

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2
Q

Characterized by dwarfism and premature aging

A

PROGERIA

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3
Q

CLINICAL FEATURES:
• Infants appear normal at birth but symptoms will appear first few years
• Alopecia (No hair)
• Pigmented areas in the trunk
• Atrophic skin, prominent veins
• High pitch squeaky voice
• Beak like nose
• Hypoplastic mandible
• Exophthalmos (Bulging eyes)
• Muscular atrophy, joint deformities

Intelligence:
• Normal or above normal

A

PROGERIA

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4
Q

ORAL MANIFESTATIONS:
• Fast formation of irregular secondary dentin
• Delayed eruption of teeth

A

PROGERIA

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5
Q

PROGERIA TREATMENT

A

• None
• No patient will live beyond the age of 27 years old

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6
Q

Is also called “protein deficiency” which may occur in simple starvation

A

MARASMUS

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7
Q

is common in prolonged febrile illness, in stress, hyperthyroidism and other conditions with disturbance in digestion and usage of protein

A

MARASMUS

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8
Q

CLINICAL FINDINGS
• Loss of weight
• Loss of subcutaneous fat
• Wasting of muscles
• Pigment changes in skin
• Hair loss
• Hypotension
• Weakness
• Edema
• Anemia

A

MARASMUS

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9
Q

• A combined protein energy deficiency in children
• A form of starvation
• Usually seen in Africa

A

KWASHIORKOR

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10
Q

• Bright red tongue
• Loss of tongue
papillae
• Bilateral angular
cheilosis
• Fissuring of lips
• Xerostomia
• Dirty mouth but
carries free, easily
traumatized, epithelium easily detached from underlying tissues resulting to a raw and bleeding surface

A

KWASHIORKOR

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11
Q

What is lacking in nutrition?
• Milk
• Meat
• Poultry
• Fish eggs

A

KWASHIORKOR

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12
Q

DENTAL SYMPTOMS:

• Decreased jaw growth
• Delayed eruption
• Enamel easily soluble to acids
o If enamel is easily soluble, the tooth is prone to caries
• Increased dental caries
• Gingiva and periodontal membranes are degenerating

A

KWASHIORKOR

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13
Q

Also called:
o Mucopolysaccharidosis I o MPSIH
o Gargoylism

A

HURLER SYNDROME

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14
Q

CLINICAL FEATURES
• Large head
• Prominent forehead
• Broad saddle nose
• Wide nostrils
• Puffy eyelids
• Bushy eyebrows
• Thick lips
• Large tongue
• Open mouth
• Nasal congestion with noisy breathing

A

HURLER SYNDROME

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15
Q

ORAL MANIFESTATIONS
• Shortening and broadening of
mandible
• Diastemas
• Bone destruction
• Microdontia,
abnormal shapes
• Delayed eruption
• Gingival hyperplasia
• Enlarged tongue

A

HURLER SYNDROME

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16
Q

HISTOLOGIC FEATURES IN TISSUES AND ORGANS:
• Liver, spleen, cartilage, bone and heart show Gargoyle cells- these are also called Hurler cells

A

HURLER SYNDROME

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17
Q

These cells are large, with metachromatically staining cytoplasm that is granular or not, with crescent nuclei- the metachromatic granules are called
Reilly Bodies

A

HURLER SYNDROME

18
Q

Reilly Bodies
o Crescent like the moon
o Cytoplasm is granular
o Metachromatically is different colors staining the
cytoplasm

A

HURLER SYNDROME

19
Q

HEREDITARY FRUCTOSE INTOLERANCE
• Nausea and vomiting after taking sugar cane or fruit
• Hereditary from genes
• Patient can’t take sugar from fruits

A

HURLER SYNDROME

20
Q

LIPID PROTEINOSIS

A

HURLER SYNDROME

21
Q

NON-LIPID RETICULOENDOTHELIOSIS

A

Hand-Schuller-Christian Disease

22
Q

Classic Triad ________

o Punched out lesions of the skull
o Exophthalmos
o Diabetes insipidus

A

Hand-Schuller-Christian Disease

23
Q

Oral Manifestations
o Sore mouth
o Gingivitis
o Loose teeth
o Unpleasant
teeth
o Failure of tooth
sockets to heal
o Loss of alveolar
bone

A

Hand-Schuller-Christian Disease

24
Q

The proliferative cell: Only affects bone

A

Eosinophilic Granuloma

25
Q

• The proliferative cell: Affects skeletal and soft tissues but includes skin
• Diffuse involvement of skeletal system
• Rashes on scalp

A

Letterer-Siwe Disease

26
Q

• Clinical Features:

o Occurs in infants before 3 years old
o Skin rash in trunks, scalp, extremities
o Low grade fever, malaise
 Malaise: general weakness of the body
o Splenomegaly (Spleen)
o Hepatomegaly (Liver)
o Lymphadenopathy
 Enlargement of lymph nodes not because of inflammation but may be due to tumor.

A

Letterer-Siwe Disease

27
Q

Oral Manifestations
o Ulcer
o Gingival hyperplasia
o Bone destruction
o Loose teeth

A

Letterer-Siwe Disease

28
Q

Histologic Features

o Very similar to
o Hand-Schuller- Christian disease,
many histiocytes
o With or without
eosinophils

A

Letterer-Siwe Disease

29
Q

Laboratory Features o Anemia
o Leukopenia
o Thrombocytopenia • Treatment and Prognosis
o Extremely poor

A

Letterer-Siwe Disease

30
Q

SUMMARY OF PROGNOSIS:

• Hand-Schuller-Christian Disease: ___
• Eosinophilic Granuloma:_____
• Letterer-Siwe Disease: _____

A

Good, Excellent, Extremely poor

31
Q

Sphingomyelin metabolism disturbance

A

Gaucher’s Disease

32
Q

A disturbance of lipid metabolism wherein keratin is
deposited in the reticuloendothelial system

A

Gaucher’s Disease

33
Q

Cells commonly seen in spleen, lymph nodes, liver,
bone marrow are the lipid filled Gaucher’s cells

A

Gaucher’s Disease

34
Q

cell:

o Round, pale, measuring 20 to 80 microns in
diameter, with small eccentric nucleus and a “wrinkled” or “crumpled silk” cytoplasm

A

Gaucher’s Disease

35
Q

Treatment and Prognosis:
o Very poor prognosis
o Give the patient glucocerebroside

A

Gaucher’s Disease

36
Q

Glucosyl ceramide metabolism disturbance

A

Niemann-pick Disease

37
Q

There is abnormal storage of phospholipids
o Mostly sphingomyelin

A

Niemann-pick Disease

38
Q

cell
o The pathognomonic cell
o They are foamy, lipid laden cells distributed
throughout the reticuloendothelial system.
 They are positive for cholesterol – Weakly positive for alkaline
phosphatase

A

Niemann-pick Disease

39
Q

Corneal clouding
• Hepatosplenomegaly, thus big
abdomen
• Short neck
• Spinal abnormalities
• Claw hand
• Dwarfism
• Mental retardation
• Thick lips
• Open mouth
• Bushy eyebrows
• Wide nostrils

A

hurler syndrome

40
Q

the pathognomonic cell

A

niemann-pick cell

41
Q

they are positive for cholesterol,
weakly positive for alkaline phosphote

A

niemann-pick cell

ORAL PATH (15 decks)

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