TITLE:
Renal Amyloidosis Following Chronic Osteomyelitis in a Patient with Congenital Insensitivity to Pain and Anhidrosis
AUTHORS:
Sevgi Yavuz, Aydin Ece
KEYWORDS:
Pain Insensitivity, Anhidrosis, Osteomyelitis, Nephrotic Syndrome, Amyloidosis
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.4 No.3,
September
24,
2014
ABSTRACT: Congenital insensitivity
to pain and anhidrosis (CIPA) is a rare form of hereditary sensory and
autonomic neuropathy. It is characterized by impaired perception of pain and
temperature, anhidrosis and intellectual disability. Self mutilating behaviors
lead to accidental injuries. The limb lesions are often infected and frequently
progress to chronic osteomyelitis. In pediatrics, amyloidosis usually occurs
secondary to chronic inflammatory diseases. The coexistence of amyloidosis and
CIPA has not previously been reported in literature. A CIPA case complicated
with nephrotic syndrome and renal amyloidosis following chronic osteomyelitis
is presented here. This report emphasizes the importance of close follow-up of
patients by urine analysis for the risk of developing amyloidosis particularly
in the presence of chronic infections.