History:
A 13-year-old boy presents with obsessive self mutilation.
1. Which of the following condition are not possible causes of abnormalities seen in figure 1 and 2?
A. Epidermolysis bullosa
B. Trauma
C. Leprosy
D. Congenital insensitivity to pain
E. Fibrous dysplasia
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2. In relation to congenital insensitivity to pain with anhydrosis, which statement is false?
A. This disease is transmitted by autosomal recessive inheritance.
B. It is characterized by recessive inheritance, generalized loss of pain sensation, mental retardation, episodes of recurrent fever, anhidrosis and alacrima.
C. Charcot joints develop in the first year of life.
D. Most affected children have mental retardation, microcephaly and hypoactivity.
E. CT scan is the study of choice to evaluate bony abnormalities in this disorder.
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References:
1 Schulman H, Tsodihow V, Einhorn M. Congenital Insensitivity to Pain with Anhidrosis(CIPA): The Spectrum of Radiological findings.Pediatr Radiol 2001;31:701-705.
2 Tomioka T, Awaya T, Nihei K. Post-herpetic Neuralgia in a Patient with Congenital Insensitivity to Pain and Anhidrosis. J Anesth 2002; 16:84-86.
3 Szoke G, Renyi-Vamos A, Bider MA .Osteoarticular Manifestations of Congenital Insensitivity to Pain with Anhydrosis. International Orthopaedics; 1996: 107-110.