pyrophosphate and Ochronosis

Topics: Animals; Arthropathy, Neurogenic; Calcium Pyrophosphate; Cartilage, Articular; Cholesterol; Chondrocalcinosis; Crystallization; Diphosphates; Disease Models, Animal; Durapatite; Hand; Hemochromatosis; Humans; Hydroxyapatites; Knee Joint; Menisci, Tibial; Ochronosis; Osteoarthritis; Postoperative Complications; Radiography; Shoulder Joint; Uric Acid

The post-mortem examination of an unsuspected case of alkaptonuria revealed extensive ochronosis. Histological examination of undecalcified sections of tracheal, costal, femoral and patellar cartilage revealed, in addition to ochronotic pigment, extensive calcium pyrophosphate dihydrate (CPPD) deposition. Similar deposits were present in intervertebral discs and were related to ossification of the discs resulting in partial or complete ankylosis. The calcific deposits were present around chondrocytes in the articular cartilage and this may be an important factor in the initiation of the osteoarthrotic process which characterises ochronotic arthropathy as it affects large diarthrodial joints.

Topics: Aged; Calcium Pyrophosphate; Cartilage; Cartilage, Articular; Diphosphates; Femur Head; Hip Joint; Humans; Intervertebral Disc; Joint Diseases; Male; Ochronosis; Trachea

After description of etiopathogenetic, anatomopathological and clinical aspects of the disease, the authors relate about a case of ochronotic arthropathy, lining on its typical radiological patterns. Scintigraphic study with 99mTc-pertechnetate and with 99mTc-pyrophosphate, excluding any phlogistic component, agree with dysmetabolic and degenerative nature of this arthropathy.

Topics: Arthritis; Diagnosis, Differential; Diphosphates; Humans; Joint Diseases; Ochronosis; Radiography; Radionuclide Imaging; Technetium