Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recent evidence suggests that a key feature in the pathogenesis of hypertrophic osteoarthropathy (HOA) is an enhanced local platelet vessel wall interaction in the affected extremities. In our investigation we measured plasma levels of von Willebrand Factor Antigen (vWF:Ag; ELISA assay), a marker of platelet and/or endothelial activation, in 5 patients with the primary form of HOA and in 6 patients with the secondary form (due to cyanotic heart disease). Seven subjects matched for sex and age were used as controls. Patients showed statistically significantly higher levels of vWF:Ag in the primary (163.8 +/- 13.9%) and secondary form (152.6 +/- 9.6%, p less than 0.001 for both) when compared to controls (100.4 +/- 6.3%). Our results support the notion that platelet endothelial cell interaction may play a key role in the development of HOA.
 ...
PMID:von Willebrand factor antigen in hypertrophic osteoarthropathy. 161 7

Hypertrophic osteoarthropathy should be considered in any child who presents with a non-inflammatory synovitis of the knees, ankles and wrists and particularly if there is finger clubbing or soft tissue hypertrophy of the fingers. It is rare to get the typical facial appearances until after adolescence. Alteration in epiphyseal growth is associated with the periosteal reaction, as is change in the shape of the patella; whether this leads to premature osteoarthrosis is not yet certain. Secondary disease is common in cyanotic congenital heart disease, but its frequency appears to be decreasing with early treatment of such children. Chest infections are also better controlled so again it is less common, but does still occur, particularly in cystic fibrosis in the older age group who have intractable chest infections. It is seen but rarely in disorders such as inflammatory bowel disease or pulmonary metastases, particularly from bone tumours.
 ...
PMID:Hypertrophic osteoarthropathy in the paediatric age. 162 66

The objectives of this study were: first, to determine the platelet morphology in the peripheral circulation of patients with cyanotic congenital heart diseases and thus its possible link to the development of hypertrophic osteoarthropathy. Second: to test the mathematical model which proposes that in normal individuals megakaryocytes are fragmented in the lungs. We prospectively studied 14 patients with cyanotic heart disease and clubbing of the 20 digits, and compared them with 14 randomly assigned controls. We measured the platelet count, mean platelet volume (MPV) and platelet volume distribution curve (PVDC). Patients had a significantly lower platelet count (x +/- SD, 171,528 +/- 81,810 vs 319,929 +/- 69,460, p less than 0.001) and larger MPV (11.028 +/- 3.09 vs 8.414 +/- 0.79, p less than 0.005). The PVDC of the controls were uniform, in all cases showing a log-normal configuration. The patient's curves were different; they lost the log-normal shape and demonstrated a heterogeneous platelet population. These findings agree with the mathematical model which proposes that in normal individuals platelets are generated in the lungs, and also suggest a role for the macrothrombocytes in the pathogenesis of cardiogenic hypertrophic osteoarthropathy.
 ...
PMID:Macrothrombocytes in the peripheral circulation of patients with cardiogenic hypertrophic osteoarthropathy. 205 70

The association of hypertrophic osteoarthropathy and chronic pulmonary disease is well known. The authors report two cases of hypertrophic osteoarthropathy and tetralogy of Fallot in childhood. Early surgical treatment of the tetralogy of Fallot accounts for the present scarceness of this association. The clinical and radiological features of hypertrophic osteoarthropathy are described, as well as the regressive evolution after the cure of the cyanotic heart disease.
 ...
PMID:[Hypertrophic osteoarthropathy and congenital cyanotic heart defects]. 265 59

We describe a simple method that defines with numbers the drumstick deformity of the digits. We measured 2 circumferences on each of the 10 fingers at the nail bed (NB) and at the distal interphalangeal joint (DIP), the sum of the 10 ratios NB:DIP was termed Digital Index. Intraobserver variation of the Digital Index was small, with a Pearson's correlation coefficient of R = 0.979; interobserver variation was also not significant (R = 0.999). Making use of this Digital Index, we studied 22 patients with digital clubbing associated with cyanotic congenital heart disease and 66 healthy controls. Digital Index clearly separated patients from controls, 10.73 +/- 0.32 vs 9.33 +/- 0.27 (means +/- SD). The Index was independent of age or sex. Patients with hypertrophic osteoarthropathy had significantly higher indices than patients with clubbing alone.
 ...
PMID:Digital clubbing: a numerical assessment of the deformity. 274 91

The relationship of periostitis to disease duration in primary hypertrophic osteoarthropathy and the association of periostitis with cardiopulmonary disorders (secondary type) were studied in order to define distinguishing features between the two. Radiographic skeletal surveys were performed in 24 patients with hypertrophic osteoarthropathy to analyze pattern (single layer, multilayered, irregular) and site of involvement (diaphysis, metaphysis, epiphysis). The six patients with primary hypertrophic osteoarthropathy and the 11 patients with cyanotic congenital heart disease had thicker, more widespread periostitis involving the diaphysis, metaphysis, and epiphysis, in contrast to abnormalities in the seven patients with hypertrophic osteoarthropathy secondary to carcinoma of the lung. Average cortical bone widths as determined by radiogrammetric measurement of the second metacarpals were significantly greater for the patients with primary hypertrophic osteoarthropathy (8.9 +/- 6.0 mm) and cyanotic congenital heart disease (8.5 +/- 6.4 mm) as compared with the patients with bronchogenic carcinoma (6.0 +/- 3.9 mm). Correlation of radiographic patterns with duration of disease confirms that thicker, more extensive alterations are indicative of long-standing disease. The periostitis of hypertrophic osteoarthropathy is therefore not dependent on the primary or secondary nature of the disease but principally on its duration.
 ...
PMID:Periostitis in hypertrophic osteoarthropathy: relationship to disease duration. 349 63

The skeletal manifestations of clubbing of the digits have been occasionally noted and only briefly discussed in the literature. We investigated the radiographic features of digital clubbing in 37 patients with diverse diseases including cyanotic congenital heart disease, lung malignancy-associated hypertrophic osteoarthropathy, and idiopathic cases. We identified two types of bone changes--osteolysis or bone dissolution, and bone formation or hypertrophy. The changes were more evident in the feet than in the hands, and the degree of soft tissue change did not always reflect the underlying osseous abnormalities. The relationship of these changes (ie, osteolysis, hypertrophy) to each other appear to depend in part on the underlying disease as well as the time course or disease duration. Thus, clubbing and hypertrophic osteoarthropathy may not represent distinct entities; our data suggest that they may be stages in an evolving, more generalized process of new bone formation or hypertrophy followed by osteolysis or atrophy affecting many parts of the skeleton.
 ...
PMID:The skeletal manifestations of clubbing: a study in patients with cyanotic congenital heart disease and hypertrophic osteoarthropathy. 390 7

We describe 2 adult patients with cyanotic congenital heart disease whom, as a complication of their cardiopathy, had 2 different rheumatic syndromes: gout and hypertrophic osteoarthropathy. The coexistence of these arthropathies in the same patient, to our knowledge, has not been previously reported. We discuss the possible pathogenic mechanisms that may link these syndromes to cyanotic heart disease.
 ...
PMID:Coexistent gout and hypertrophic osteoarthropathy in patients with cyanotic heart disease. 652 Aug 38

We treated two patients with congenital cyanotic heart disease (CCDH) in whom associated hypertrophic osteoarthropathy (HOA) developed. Both were severely cyanotic and hypoxic for many years before HOA was fully manifested. Clinical and roentgenographic indications of bone and joint lesions disappeared completely shortly after corrective of palliative surgery. There appears to be an etiologic connection between HOA and CCHD.
 ...
PMID:Reversible hypertrophic osteoarthropathy associated with cyanotic congenital heart disease. 710 19

The frequency of hypertrophic osteoarthropathy in cyanotic congenital heart disease has previously been considered to be very low. Only a few isolated reports have described such an association. We studied 32 consecutive patients older than 6 years with various types of cyanotic congenital heart disease and examined each case for hypertrophic osteoarthropathy. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of hypertrophic osteoarthropathy by means of cardiac catheterization. Our results showed that 31% of the patients had hypertrophic osteoarthropathy. When the hemodynamic parameters of the group of patients with hypertrophic osteoarthropathy were compared with those of the remaining patients, we found significant differences in the systemic blood flow (P less than or equal to 0.05), right-to-left shunt (P less than or equal to 0.05), and arterial oxygen unsaturation (P less than or equal to 0.005). Thus, we found a much higher prevalence of hypertrophic osteoarthropathy in patients with cyanotic congenital heart disease than has previously been recognized. The development of hypertrophic osteoarthropathy is related to the degree of bypass of the lung. This is consistent with the concept that hypertrophic osteoarthropathy results from mediators in the systemic venous circulation that escape inactivation in the pulmonary capillary bed.
 ...
PMID:Hypertrophic osteoarthropathy in cyanotic congenital heart disease: its prevalence and relationship to bypass of the lung. 713 92


1 2 3 4 Next >>