Updated: 6/17/2021

Ochronosis

Average 4.1 of 17 Ratings

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Flashcards

N/A

Questions

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Topic

summary
- Ochronosis is a metabolic arthropathy caused by alkaptonuria with excess homogentistic acid deposited in the joints. Patients present with early degenerative joint disease, spondylitis, and black urine.
- Diagnosis is made with detection of homogentisic acid in urine.
- Treatment is similar to that of osteoarthritis with observation, NSAIDs, tramadol and corticosteroids for mild to moderate symptoms. Arthroplasty is indicated for severe symptoms.

Etiology
- Pathophysiology
  - excess homogentistic acid is deposited in the joints
  - acid polymerizes in joint and leads to early joint arthritis
- Genetics
  - rare inborn defect in homogentisic acid oxidase enzyme system
- Associated conditions
  - orthopaedic manifestations
    
    ochronotic spondylitis
    
    commonly occurs in fourth decade
    
    progressive degenerative changes in spine
    
    calcification
    
    narrowing of disc spaces

Presentation
- Symptoms
  - may complain of black urine
    
    caused by polymerization of homogentistic acid

Imaging
- Radiographs
  - spine
    
    findings
    
    irregular calcification
    
    narrowing of intervertebral discs

Treatment
- Nonoperative
  - no current medical treatment available

Flashcards (2)

Cards

1 of 2

Action	Numeric Key	Letter Key	Function Key
Show Bullets		S	Enter (frontside only)
20%	1	N
40%	2	H
60%	3	F	Enter (backside only)
80%	4	E
100%	5	M
Toss	0	T

Questions (1)

(OBQ11.153) A 55-year-old male presents with a 6 month history of low back pain. Radiographs of the spine are shown in Figures A and B and a lateral knee radiograph is shown in Figure C. A urine specimen is exhibited in Figure D. What is the most likely diagnosis in this patient?

QID: 3576

FIGURES: