- 1 WHAT IS IT?
- 2 WHAT CAUSES IT?
- 3 WHY IS IT CONCERNING MEDICALLY?
- 4 WHAT IS THE INTIAL PRESENTATION?
- 5 WHAT ARE IMPORTANT ELEMENTS OF THE MEDICAL HISTORY?
- 6 WHAT ARE IMPORTANT FEATURES OF THE PHYSICAL EXAM?
- 7 CLINICAL WORKUP: SERUM STUDIES
- 8 CLINICAL WORKUP: IMAGING
- 9 CLINICAL WORKUP: OTHER
- 10 HOW DO WE NARROW THE DIFFERENTIAL?
- 11 WHAT IS OUR THRESHOLD FOR DIAGNOSING THIS CONDITION?
- 12 PATIENT MANAGMENT: SYMPTOM RELEIF
- 13 PATIENT MANAGEMENT: DISEASE TREATMENT
- 14 PATIENT MANAGEMENT: PROPHYLACTIC MEASURES
- 15 COULD THIS HAVE BEEN PREVENTED?
- 16 ARCHIVE OF STANDARDIZED EXAM QUESTIONS
WHAT IS IT?
Paget disease of bone is a metabolic disorder of the bone that is characterized by focal areas of excessive bone resorption and formation.
WHAT CAUSES IT?
Osteoclast dysfunction and increased bone turnover is responsible for this condition: environmental factors + gene mutations that result in excessive RANK and NF-kappaB activation. This leads to osteoclast differential and activity. There are typically 3 phases of this condition:
- Osteolytic (osteoclast dominant) phase:
- Mixed (osteoclastic-osteoblastic) phase:
- Osteosclerotic (osteoblastic dominant) phase;
WHY IS IT CONCERNING MEDICALLY?
This conditions is characterized by abnormal bone remodeling/deformity which can be responsible for multiple medical complications:
- Hearing loss can be caused by abnormalities involving the bones of the ear
- Headaches can be a result of skull involvement
- Spinal stenosis/radiculopathy can occur if the spine is affected
WHAT IS THE INTIAL PRESENTATION?
Patient Chief Complaints:
- Asymptomatic: most patients do not have any complaints
- Bone pain
- Hearing loss
Detected Medical Problems:
- Elevated alkaline phosphatase
- Increased signal on bone scan
WHAT ARE IMPORTANT ELEMENTS OF THE MEDICAL HISTORY?
WHAT ARE IMPORTANT FEATURES OF THE PHYSICAL EXAM?
CLINICAL WORKUP: SERUM STUDIES
Complete Metabolic Panel (CMP)/Liver Function Tests (LFTs):
- Alkaline phosphatase is often elevated
CLINICAL WORKUP: IMAGING
X-Ray Imaging may be remarkable for:
- Osteolytic lesions
- Mixed lytic/sclerotic lesions
Bone Scan may demonstrate a focal increase in uptake.
CLINICAL WORKUP: OTHER
Bone Turnover Markers will often be elevated:
- Urine hydroxyproline
HOW DO WE NARROW THE DIFFERENTIAL?
Conditions that present similarly and how to exclude them:
WHAT IS OUR THRESHOLD FOR DIAGNOSING THIS CONDITION?
PATIENT MANAGMENT: SYMPTOM RELEIF
PATIENT MANAGEMENT: DISEASE TREATMENT
Bisphosphonates are used to manage this condition:
PATIENT MANAGEMENT: PROPHYLACTIC MEASURES
COULD THIS HAVE BEEN PREVENTED?
ARCHIVE OF STANDARDIZED EXAM QUESTIONS
This archive compiles standardized exam questions that relate to this topic.
Page Updated: 02.21.2017