21 - 128 - CALCIUM AND OTHER MINERAL DEPOSITION DISORDERS

Question 1

Question 2

Question 3

Question 4

Question 5

Question 6

Question 7

Question 8

Deposition of insoluble calcium salts in cutaneous tissue.

Answer 8

Calcinosis cutis

Question 9

GC, 41/M diagnosed with chronic renal failure complained of firm, extremely painful, reticulated violaceous plaques over the lower extremities. Most likely diagnosis of this case?

a. Tumoral calcinosis
b. Hypervitaminosis D
c. Calciphylaxis
d. Benign nodular calcification

Which is true in relation with your answer to the question above?

a. Life threatening disorder
b. Chronic ingestion of vitamin D
c. Size and number correlate with degree of hyperphosphatemia
d. Deposition of calcific masses around the major joints (hips, shoulders, elbows, knees)

Answer 9

c. Calciphylaxis
a. Life threatening disorder

Question 10

Hypervitaminosis D:

a. 100,000-150,000 u/d
b. 150,000-200,000 u/d
c. 50,000-100,000 u/d
d. 25,000-50,000 u/d

Answer 10

c. 50,000-100,000 u/d

Question 11

Which statement is false in relation with iatrogenic calcification?

a. Complication of IV calcium chloride and calcium gluconate therapy
b. Minor trauma and prolonged contact with calcium salts
c. Skin graft donor sites after application of calcium alginate dressings
d. After liver transplantation
e. All are true

Answer 11

e. All are true

Question 12

G.C, 50/M, recently had his routine serum uric acid test. Which among the following results is considered a risk to develop gout in 5 years?

a. >7mg/dl
b. >8mg/dl
c. >9mg/dl
d. >10mg/dl

Answer 12

a. >7mg/dl

Question 13

Gout: which stage usually manifests erythematous and tender first metatarsophalangeal joint of the lower extremity?

a. Asymptomatic
b. Acute gouty arthritis
c. Intercritical gout
d. Chronic tophaceous gout

Answer 13

B

Question 14

Stage which tophi is seen which is the pathognomonic sign.

a. Asymptomatic
b. Acute gouty arthritis
c. Intercritical gout
d. Chronic tophaceous gout

Answer 14

d. Chronic tophaceous gout

Question 15

Which test/s is/are elevated in acute gouty attacks?

a. Uric acid
b. WBC
c. ESR
d. A and B
e. B and C

Answer 15

e. B and C

Question 16

In patients with chronic tophaceous gout:

a. Uric acid lowering therapy must maintain <6mg/dl or 5mg/dl in patients with tophi or frequent attacks
b. Uric acid lowering therapy must maintain <7mg/dl or 6mg/dl in patients with tophi or frequent attacks
c. Uric acid lowering therapy must maintain <7mg/dl or 5mg/dl in patients with tophi or frequent attacks
d. Uric acid lowering therapy must maintain <6mg/dl or 4mg/dl in patients with tophi or frequent attacks

Answer 16

A

Question 17

stains for calcium

Answer 17

Alizarin red S or von Kossa stains

Question 18

3 regulatory hormones control the ionic calcium concentration in serum

Answer 18

(1) parathyroid hormone (PTH),
(2) calcitonin, and
(3) 1,25-dihydroxyvitamin D3 (1,25(OH)2 D3 ).

Question 19

Calcification is the deposition of insoluble calcium salts; when it occurs in cutaneous tissues, it is known as

Answer 19

calcinosis cutis

Question 20

the formation of true bony tissue by the deposition of calcium and phosphorus in a proteinaceous matrix as hydroxyapatite crystals

Answer 20

Ossification

Question 21

Cutaneous calcification may be divided into 4 major categories

Answer 21

(1) dystrophic,
(2) metastatic,
(3) idiopathic, and
(4) iatrogenic

Question 22

most common type of calcinosis cutis and occurs as a result of local tissue injury

Answer 22

Dystrophic calcification

• Although calcium and phosphate metabolism and serum levels are normal, local tissue abnormalities, such as alterations in collagen, elastin, or subcutaneous fat may trigger calcification.
• The internal organs usually remain unaffected

Question 23

occurs without identifiable underlying tissue abnormalities, abnormal calcium, and/or phosphate metabolism

Answer 23

Idiopathic calcification

Question 23

precipitation of calcium salts in normal tissue secondary to an underlying defect in calcium and/or phosphate metabolism

Answer 23

Metastatic calcification

• The calcification may be widespread and, in addition to the skin, affects predominantly blood vessels, kidneys, lungs, and gastric mucosa.
  All patients presenting with signs of cutaneous calcification should receive a calcium and phosphate metabolic evaluation.

Question 24

# Identify the category of cutaneous calcinosis IV calcium chloride

Answer 24

Iatrogenic

Question 25

# Identify the category of cutaneous calcinosis Chronic renal failure

Answer 25

Metastatic

Question 26

# Identify the category of cutaneous calcinosis Panniculitis

Answer 26

Dystrophic

Question 26

# Identify the category of cutaneous calcinosis Hypervitaminosis D

Answer 26

Metastatic

Question 27

# Identify the category of cutaneous calcinosis Connective tissue diseases

Answer 27

Dystrophic

Question 27

# Identify the category of cutaneous calcinosis IV calcium gluconate therapy

Answer 27

Iatrogenic

Question 28

Which category of calcinosis cutis may present with elevated or normal calcium and phosphate

Answer 28

Metastatic

Question 28

# Identify the category of cutaneous calcinosis Cutaneous neoplasms

Answer 28

Dystrophic

Question 28

# Identify the category of cutaneous calcinosis Tumoral calcinosis

Answer 28

Metastatic

Question 28

# Identify the category of cutaneous calcinosis Milk-alkali syndrome

Answer 28

Metastatic

Question 28

# Identify the category of cutaneous calcinosis infections

Answer 28

Dystrophic

Question 29

# Identify the category of cutaneous calcinosis Inherited disorders

Answer 29

Dystrophic

Question 30

Metastatic calcification most commonly occurs in what condition?

Answer 30

chronic renal failure

Question 30

most common cutaneous neoplasms that manifest calcification and ossification

Answer 30

Pilomatricomas

Question 31

Parasitic infections that may result in calcinosis cutis

Answer 31

onchocerciasis (Onchocerca volvulus) and cysticercosis (Taenia solium)

Question 32

life-threatening disorder characterized by progressive vascular calcification, soft tissue necrosis, and ischemic necrosis of the skin

Answer 32

Calciphylaxis

Question 33

This occurs almost exclusively in patients with a history of chronic renal failure and prolonged secondary hyperparathyroidism.

Answer 33

Calciphylaxis

Question 34

disorder characterized by the deposition of calcific masses around major joints, such as hips, shoulders, elbows, and knees

Answer 34

Tumoral calcinosis (TC)

Question 35

3 well-described ossifying syndromes

Answer 35

1. Fibrodysplasia ossificans progressiva (FOP) 2. Albright hereditary osteodystrophy (AHO), 3. Progressive osseous heteroplasia (POH)

Question 36

autosomal-dominant syndrome characterized by the progressive ossification of deep connective tissues leading to significant morbidity and mortality.

Answer 36

FIBRODYSPLASIA OSSIFICANS PROGRESSIVA (FOP)

Question 37

characteristic feature of fibrodysplasia ossificans progressiva (FOP)

Answer 37

Dysmorphic great toes

Question 38

Gain-of-function point mutations in the **activin (ACVR1/ALK2) gene** have been identified as the cause of this condition

Answer 38

fibrodysplasia ossificans progressiva (FOP)

Question 39

closely related syndromes that are characterized by **intramembranous** **bone formation **in skin.

Answer 39

Albright hereditary osteodystrophy (AHO), and progressive osseous heteroplasia (POH)

Question 40

Heterozygous, inactivating mutations in the gene encoding for the α-subunit of the stimulatory** G protein of adenyl cyclase (GNAS1)**, a negative regulator of bone formation, have been identified in both of these disorders

Answer 40

Albright hereditary osteodystrophy (AHO), and progressive osseous heteroplasia (POH)

Question 41

What do you call the sign characterized by brachydactyly dimpling over the metacarpophalangeal joints? What condition is this seen?

Answer 41

Albright sign Albright hereditary osteodystrophy (AHO)

Question 42

Most patients have a deficient end-organ response to PTH or “pseudohypoparathyroidism” with hypocalcemia, hyperphosphatemia, and elevated levels of PTH. Other patients have “pseudopseudohypoparathyroidism” with normal serum levels of calcium and phosphorus.

Answer 42

Albright hereditary osteodystrophy (AHO)

Question 43

Skin involvement has been described as a papular eruption resembling “rice grains” and having a “gritty” consistency

Answer 43

progressive osseous heteroplasia (POH) ## Footnote * characterized by progressive ossification of skin and deep tissues during infancy or childhood * Ossification usually begins in the dermis and progresses to involve deeper tissues, such as muscle, as well as overlying skin.

Question 44

clinical syndrome caused by a group of heterogeneous diseases characterized by deposition of monosodium urate crystals in synovial fluid and joints with or without hyperuricemia, renal disease, or nephrolithiasis

Answer 44

Gout

Question 45

most commonly affected sites of gout

Answer 45

first metatarsophalangeal joint and the ankle

Question 46

describes the interval that occurs between attacks of gout, an interval of between 6 months and 2 years.

Answer 46

Intercritical gout

Question 47

describes gout where patients rarely have asymptomatic periods

Answer 47

Chronic tophaceous gout

Question 48

pathognomonic sign of gout

Answer 48

tophi

Question 49

goal of urate-lowering drugs is to maintain the serum urate level consistently at less than what level?

Answer 49

less than **6 mg/dL** (5 mg/dL in patients with tophi or frequent attacks)

Question 50

first-line drug for lowering serum urate

Answer 50

Allopurinol ## Footnote It decreases production of uric acid, and is indicated for patients with **nephrolithiasis, renal impairment, those who failed uricosuric agents, **with myeloproliferative disorders on chemotherapy, and patients with hyperuricemia due to enzyme abnormalities.

Question 51

FDA approved for short-course therapy to prevent tumor lysis syndrome

Answer 51

Rasburicase

Question 51

Thus uricase is approved in patients with refractory chronic gout

Answer 51

IV pegloticase

Question 52

* recently Food and Drug Administration (FDA)–approved selective uric acid reabsorption inhibitor * It inhibits URAT1 and OAT4

Answer 52

Lesinurad ## Footnote - URAT1 - urate transporter that is responsible for most renal reabsorption of uric acid - OAT4 - transporter associated with diuretic-induced hyper uricemia