Familial articular chondrocalcinosis in Spain
Article Abstract:
Familial articular chondrocalcinosis, first reported in 1957, is characterized by arthritis, inflammation of the joints with calcification or calcium formation in the cartilage tissue of the joint. This joint disorder results from the deposition of calcium pyrophosphate dihydrate (CPPD) crystals in cartilage tissue, but the mechanisms of crystal deposition are not known. Symptoms range from mild, chronic, or diffuse joint pain to the inflammation of several joints resulting from the release of CPPD crystals into the joint cavity. The three forms of chondrocalcinosis are hereditary, sporadic, and a form related to metabolic disorders. Hereditary chondrocalcinosis has been reported worldwide, and two types of the disease have been described. One type is characterized by early onset, involvement of several joints, and a variable outcome. The other type of hereditary chondrocalcinosis is characterized by a later onset and involvement of a smaller number of joints. The differences between the two types of hereditary chondrocalcinosis were evaluated in a Spanish population consisting of 101 first degree relatives of 35 patients with chondrocalcinosis. Eleven persons from nine families had X-ray evidence of chondrocalcinosis, and the prevalence of the familial disease was 26 percent. The disease occurred in two different patterns, commonly affected older persons, and did not occur in young persons or second degree relatives. Clinical symptoms and X-ray findings differed between patients with early and late onset of the disease, but did not differ among patients with either late onset or sporadic types of chondrocalcinosis. These results suggest that the prevalence of familial chondrocalcinosis is underestimated. The genetic transmission of this joint disorder is discussed. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1990
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Hereditary chondrocalcinosis in an Ashkenazi Jewish family
Article Abstract:
Calcium pyrophosphate dihydrate (CPPD) crystal arthropathy, also referred to as chondrocalcinosis, is a joint disease in which CPPD crystals are deposited into the cartilage of the joint. The factors that cause the deposition of crystals have not been determined, although three forms of the disease have been defined. CPPD crystal arthropathy may be inherited, caused by endocrine or metabolic disorders, or can occur sporadically. Several cases of hereditary CPPD crystal arthropathy have been reported which differ in may ways, including the method of genetic transmission. The sporadic form of the disease developed in about 28 percent of residents in a Jewish home for the aged, but the hereditary form was not detected. The hereditary form of chondrocalcinosis is reported for the first time in an Ashkenazi Jewish kindred. Seven of 34 members from five generations were affected with this disorder. Direct evidence of chondrocalcinosis was detected in five of 25 members from three generations. Symptoms of this joint disorder commonly developed in the third decade of life, although X-ray confirmation of the disease was more evident in the fourth decade of life. Joints within the knees, wrists, and elbows were most often affected. The disease progression was chronic and was aggravated by exercise. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1990
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Understanding inorganic pyrophosphate metabolism: toward prevention of calcium pyrophosphate dihydrate crystal deposition
Article Abstract:
Control of the amount of inorganic pyrophosphate (PPi) metabolism through drug therapy may prevent the formation of calcium pyrophosphate dihydrate (CPPD) crystal deposits in joints. CPPD crystal deposits may form in a given joint because of abnormal conditions. Cells within joints may not allow PPi to cross cell boundaries, so CPPD crystals develop. PPi levels in joint fluid may be greater than levels in plasma; PPi levels increase in proportion to the damage found in joints. PPi may be produced in greater amounts in damaged knee joints, and its production may be associated with a particular enzyme functioning outside of cartilage cells. PPi may prevent bone formation within joints, so it may also prevent CPPD crystal deposits.
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1995
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