Connective Tissue Flashcards

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1
Q

[Gen/CT/Marfan]

Most causes of death for Marfan?

A

Aortic root dilatation and rupture

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2
Q

[Gen/CT/Marfan]

Major criteria of Ghent criteria? (5)

A
  1. Ectopic lentis
  2. Aortic dilatation or dissection
  3. FBN1 mutation
  4. Systemic features > 7 scores
  5. Family history

2 of major is diagnostic

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3
Q

[Gen/CT/Marfan]

Orthopedic signs of revised Ghent criteria for Marfan syndrome? (6)

A
  1. Wrist and thumb signs
  2. Pectus carinatum
  3. Hindfoot deformity
  4. Scoliosis or thracolumbar kyphosis
  5. Reduced elbow extension
  6. All 3 of the following: when no scoliosis, reduced upper to lower segment ratio and increased arm span to height ratio (>1.05)
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4
Q

[Gen/CT/Marfan]

Cardiopulmonary signs of revised Ghent criteria for Marfan syndrome? (2)

A
  1. Spontaneous pneumothorax
  2. Mitral valve prolapse
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5
Q

[Gen/CT/Marfan]

Radiographic signs of revised Ghent criteria for Marfan syndrome? (2)

A
  1. Dural ectasia
  2. Protrusio acetabuli
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6
Q

[Gen/CT/Marfan]

Facial features of revised Ghent criteria for Marfan syndrome? (5)

A
  1. Dolichocephaly
  2. Enophthalmos
  3. Down-slanting palpebral fissures
  4. Malar hypoplasia
  5. Retrognathia
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7
Q

[Gen/CT/Marfan]

Skin/eye signs of revised Ghent criteria for Marfan syndrome? (2)

A
  1. Skin striae
  2. Myopia
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8
Q

[Gen/CT/Marfan]

Features for homocystinuria, compared to Marfan (3)

A
  1. Downwardly dislocated lens
  2. Intellectual disability
  3. Stroke/embolic events
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9
Q

[Gen/CT/EDS]

Characteristics of Ehlers-Danlos syndrome? (4)

A
  1. Hyperextensible skin
  2. Hypermobile joints
  3. Easy bruising
  4. Dystrophic scarring
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10
Q

[Gen/CT/EDS]

Abnormal coagulation test for Ehlers-Danlos syndrome

A

Capillary fragility test
(Hess test, torniquet test)

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11
Q

[Gen/CT]

Name of anomaly?
Name of syndrome?

Hyperflexibility, MVP, Increased arm to height

A

Dural ectasia
Marfan syndrome

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12
Q

[Gen/CT]

Name of anomaly?
Name of syndrome?

Hyperflexibility, MVP, Increased arm to height

A

Protrusio actabuli
Marfan syndrome

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13
Q

[Gen/CT]

Name of syndrome?

Begin before 6 months, resolve by 24 months
Leukocytosis, elevated ESR, and ALP

A

Infantile Cortical Hyperostosis
(Caffey disease)

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