All of the following are features of pyle disease except-
**Core Concept**
Pyle disease, also known as osteopathia striata with cranial sclerosis, is a rare genetic disorder characterized by abnormal bone formation and sclerosis. It is a congenital condition that affects the bones, particularly the skull, and is often associated with other systemic abnormalities.
**Why the Correct Answer is Right**
Pyle disease is characterized by the presence of osteopathia striata, which is a condition where the bones develop striations or bands. This is due to the abnormal deposition of bone tissue, leading to an increase in bone density and sclerosis. The condition is often associated with other systemic abnormalities, including intellectual disability, seizures, and vision and hearing impairments.
**Why Each Wrong Option is Incorrect**
**Option A:** Not a feature of Pyle disease. Pyle disease is not typically associated with anemia, which is more commonly seen in other conditions such as thalassemia or sickle cell disease.
**Option B:** Not a feature of Pyle disease. Pyle disease is not typically characterized by a high white blood cell count, which is more commonly seen in conditions such as leukemia or infection.
**Option C:** Not a feature of Pyle disease. Pyle disease is not typically associated with joint pain or arthritis, which is more commonly seen in conditions such as rheumatoid arthritis or osteoarthritis.
**Clinical Pearl / High-Yield Fact**
Pyle disease is a rare genetic disorder that requires a high index of suspicion for diagnosis. It is essential to consider Pyle disease in the differential diagnosis of children with abnormal bone formation or sclerosis, particularly if they have other systemic abnormalities.
**Correct Answer: D.**