The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is characterized by congenital or early-onset camptodactyly, childhood-onset noninflammatory arthropathy associated with synovial hyperplasia. Some patients have pro-gressive coxa vara deformity and/or noninflammatory pericardial effusion. CACP is inherited as an autosomal recessive mode and the disease gene is assigned to a 1.9-cM interval on human chromosome 1q25-31. We describe a 10-yr-old boy who has typical features of CACP without familial association.
Adolescent ; Fingers/*abnormalities ; Hip Joint/*abnormalities ; Humans ; Joint Diseases/*congenital/*diagnosis ; Male ; Pericarditis/*congenital/*diagnosis ; Syndrome ; Toes/*abnormalities

Constrictive pericarditis is caused by fibrosis of the pericardium leading to decrease in ventricular compliance. The diagnosis is often delayed due to nonspecific signs and symptoms. The authors experienced eight cases of constrictive pericarditis detected on chest CT scan while being treated for considerable length of time under the clinical impressions of intrathoracic tumor, tuberculous pleural effusion, liver cirrhosis, etc. Constrictive hemodynamics of these patients were confirmed by echocardiogram and cardiac catheterization. Among them five cases were due to tuberculosis. In four cases with pathologically proven tuberculous granuloma, the pericardium was markedly thickened and intensely enhanced. Associated pericardial effusion (n=3), and mediastinal lymphadenitis (n=3) were present, but pericardial calcifications were not seen. On the other hand, the fibrosis group (n=3) displayed mild pericardial thickening. All the three patients showed pericardial calcifications, mild or absent enhancement of pericardium, but no mediastinal lymphadenitis. The cardiovascular changes such as inferior or superior vena caval distension, left ventricular deformity, interventricular septum angulation, and biatrial enlargements were more severe than those in patients with active granuloma. In patients with constrictive pericarditis with nonspecific signs and symptoms, CT scan is very helpful in making the diagnosis and can give informations about the evolution of the disease.
Cardiac Catheterization ; Cardiac Catheters ; Compliance ; Congenital Abnormalities ; Diagnosis* ; Fibrosis ; Granuloma ; Hand ; Hemodynamics ; Humans ; Liver Cirrhosis ; Lymphadenitis ; Pericardial Effusion ; Pericarditis, Constrictive* ; Pericardium ; Pleural Effusion ; Tomography, X-Ray Computed ; Tuberculosis

Constrictive pericarditis is caused by fibrosis of the pericardium leading to decrease in ventricular compliance. The diagnosis is often delayed due to nonspecific signs and symptoms. The authors experienced eight cases of constrictive pericarditis detected on chest CT scan while being treated for considerable length of time under the clinical impressions of intrathoracic tumor, tuberculous pleural effusion, liver cirrhosis, etc. Constrictive hemodynamics of these patients were confirmed by echocardiogram and cardiac catheterization. Among them five cases were due to tuberculosis. In four cases with pathologically proven tuberculous granuloma, the pericardium was markedly thickened and intensely enhanced. Associated pericardial effusion (n=3), and mediastinal lymphadenitis (n=3) were present, but pericardial calcifications were not seen. On the other hand, the fibrosis group (n=3) displayed mild pericardial thickening. All the three patients showed pericardial calcifications, mild or absent enhancement of pericardium, but no mediastinal lymphadenitis. The cardiovascular changes such as inferior or superior vena caval distension, left ventricular deformity, interventricular septum angulation, and biatrial enlargements were more severe than those in patients with active granuloma. In patients with constrictive pericarditis with nonspecific signs and symptoms, CT scan is very helpful in making the diagnosis and can give informations about the evolution of the disease.
Cardiac Catheterization ; Cardiac Catheters ; Compliance ; Congenital Abnormalities ; Diagnosis* ; Fibrosis ; Granuloma ; Hand ; Hemodynamics ; Humans ; Liver Cirrhosis ; Lymphadenitis ; Pericardial Effusion ; Pericarditis, Constrictive* ; Pericardium ; Pleural Effusion ; Tomography, X-Ray Computed ; Tuberculosis