Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency of HLA antigens was determined in 133 patients with acquired valvular heart disease and compared with the frequency of HLA antigens in 1,000 normal individuals by the relative incidence ratio method of Woolf (1955). No significant increases were observed. However, when the patients were divided into those with no rheumatic history and those who have had rheumatic fever or chorea, a significant increase in AW 30/31 and A29 was observed in the group with no rheumatic history.
 ...
PMID:HLA antigens and acquired valvular heart disease. 5 62

Analysis of the HLA antigen patterns in patients with acquired valvular heart disease showed that antigens A29 and AW30/31 occurred more often in those patients whose valvular disease was not preceeded by a history of rheumatic fever or chorea. Patients with no rheumatic history but with antigen A29 or AW30/31 had clinical features that distinguished them from others with valvular disease--namely, (a) isolated mitral valve disease and (b) the need for surgery, often at an early age, after a relatively short duration of symptoms.
 ...
PMID:A relation between HLA antigens and clinical features in patients with acquired valvular heart disease. 127 36

Acute rheumatic fever (ARF) is an inflammatory sequela which occurs in 1-3% of children afflicted with group A beta-hemolytic streptococcal pharyngitis (strep throat). The major manifestations are carditis, migratory polyarthritis and chorea. ARF recurs with repeated strep throats and frequently leads to rheumatic heart disease (RHD), usually mitral and aortic regurgitation and mitral stenosis. ARF likely results from an abnormal host immune response with a host-antibody/streptococcal antigen production in pharyngeal tissue and subsequent cross-reaction of host antibodies with host end organs. Treatment includes eradication of the streptococcus, use of high doses of salicylates and adrenal corticosteroids, and prolonged bed rest with gradual ambulation after clinical and laboratory signs of the disease are gone. While the incidence and mortality of ARF and RHD have decreased drastically in the affluent industrialized countries of Europe, North America, and in Japan, the disease is a major health problem in the less affluent, 'developing' countries of Latin America, the Middle East, Africa, India and Southeast Asia. The major risk factors for ARF are believed to be genetic or familial, inadequate medical care and crowded conditions. The last two factors are socioeconomic but may also be influenced by ethnic cultural behavior. Genetic propensity for ARF is supported by recent evidence of a specific DR-HLA marker in the majority of people with ARF or RHD. Finally, while ARF appears to be vanishing in most areas of the country, it is still prevalent in some affluent populations and in some disadvantaged minorities.
 ...
PMID:Acute rheumatic fever in the 1980s. 329 32

Twenty patients with Hungtington's chorea and 30 family members were typed for 30 antigens of the HLA-A, B, and C series. There was an increased frequency of HLA-BW 16, significant at the 1%-level. After correcting for multiple inferences, the association of HLA-BW 16 with Huntington's chorea was no longer statistically significant. There was also no reliable indication of coupling between HLA antigens and Huntington's chorea in the family studies.
 ...
PMID:HLA antigen frequencies in patients with Huntington's chorea and their relatives. 615 1

An analysis of 170 attacks (in 158 children) treated with a diagnosis of rheumatic fever (RF) in 1956-1961 or 1970-1974 showed that the Jones revised criteria of RF were fulfilled in about 1/2 of the attacks. Arthritis occurred in 72%, carditis in 39%, chorea in 18%, and erythema marginatum in 1% of the attacks with confirmed diagnosis. No chorea was observed in 1970-1974. Follow-up of the patients showed favorable prognosis and low frequency of recurrences of RF. HLA analysis showed frequencies of A and B loci were similar, whereas "blank" finding in locus C was rarer in RF patients than in controls.
 ...
PMID:Rheumatic fever and its sequels in children. A follow-up study with HLA analysis. 742 Mar 32

We describe 13 adult patients with reactive arthritis induced by tonsillitis. Arthritis occurred 710 days after tonsillitis and involved the wrists, knees, feet and sternoclavicular joints. Some cases had pain in the Achilles tendon areas. Synovial fluid examined in 4 patients was sterile. All patients except 3 showed unequivocal elevation of serum ASO and/or ASK. Streptococcus was isolated from tonsillar swabs in 7 patients. One had maculopapular erythema and 2 had abdominal pain of unknown origin, but none had cardiac involvement, chorea and subcutaneous nodule. HLA examination revealed that 4 had B39 (p <0.005). Eight cases were treated with antibiotics. Five cases underwent tonsillectomy. All tonsils had cryptic abscess. No exacerbation was seen thereafter. These cases probably represent reactive arthritis induced by tonsillitis and should be distinguished from other rheumatic diseases.
 ...
PMID:Reactive arthritis induced by tonsillitis. 908 84

Sydenham's chorea (SC) may occur in rheumatic fever (RF) patients without arthritis and carditis. In this study we typed HLA antigens and alleles in patients presenting with the distinct major clinical manifestations of RF, i.e., chorea, carditis, or arthritis, in population and family studies. We evaluated 91 patients with RF for HLA-A, HLA-B, and HLA-DR antigens; of these, 33 had pure chorea, 26 pure carditis, 16 pure arthritis, and 16 carditis plus arthritis. We also typed 24 SC patients and their unaffected siblings for HLA-DRB1 and HLA-DQB1 alleles using molecular methods. HLA-B49 and HLA-DR1 antigens were overrepresented in the total group of patients with RF and in all the subgroups studied, excluding the SC subgroup in which the frequency of HLA-DR1 antigen was not increased. The frequencies of the HLA-DRB1 and HLA-DQB1 alleles in patients with pure chorea were not significantly different from those observed in controls. Similarly, the frequencies of HLA class II alleles in SC patients did not differ significantly from those observed in unaffected siblings. These findings show that immunogenetic susceptibility to RF varies according to the major clinical manifestation presented by the patient.
 ...
PMID:HLA class I and class II profiles of patients presenting with Sydenham's chorea. 1075 Nov 15

Dysfunction of the autonomic nervous system is an under-recognised but important aspect of the aetiological and clinical manifestation of primary degenerative dysautonomias such as multiple system atrophy (MSA) and Parkinson's disease (PD). Although the clinical presentation of dysautonomia in these two disorders may overlap, yet pathological and in vivo imaging studies suggest considerable differences. Functional imaging studies suggest that selective cardiac sympathetic denervation may occur early in PD but not in other parkinsonian syndromes. The clinical implication of this apparently disease specific peripheral dysautonomia is unknown and would be the subject of much interest in future years. Dysautonomia in degenerative disorders also affect respiration, genitourinary function and sleep. Sleep related disorders such as rapid eye movement behaviour disorder and urinary voiding dysfunction appear to precede the development of PD related symptoms while patients with sporadic ataxia have been shown to progress to develop MSA. Dysautonomia has also been recognised in other movement disorders, examples being the combination of dystonia and complex regional pain syndrome with elevated HLA-DR13 and late onset Huntington's disease presenting with dominant parkinsonism and minimal chorea. These studies have helped progress in various diagnostic and management parameters in relation to autonomic dysfunction and movement disorders.
 ...
PMID:Autonomic dysfunction in movement disorders. 1147 Sep 68

The HLA system is being paid more and more attention because it is very significant in polymorphous immunological reactions. Several studies have suggested that genetic susceptibility to rheumatic fever (RF) and rheumatic heart disease (RHD) is linked to HLA class II alleles. We hypothesized that HLA class II associations within RHD may be more consistent if analysed amongst patients with a relatively homogeneous clinical outcome. A total of 70 RF patients under the age of 18 years were surveyed and analysed in Latvia. HLA genotyping of DQA1, DQB1 and DRB1 was performed using PCR with amplification with sequence-specific primers. We also used results from a previous study of DQB1 and DRB1 genotyping. In the RF patients, HLA class II DQA1*0401 was found more frequently compared to DQA1*0102. In the RF homogeneous patient groups, DQA1*0402 has the highest odds ratio. This is also the case in the multivalvular lesion (MVL) group, together with DQA1*0501 and DQA1*0301. In the chorea minor patients, DQA1*0201 was often found. Significant HLA DQA1 protective genotypes were not detected, although DQA1 genotypes *0103/*0201 and *0301/*0501 were found significantly and frequently. In the distribution of HLA DRB1/DQA1 genotypes, *07/*0201 and *01/*0501 were frequently detected; these also occurred significantly often in the MVL group. The genotype *07/*0201 was frequently found in Sydenhamn's chorea patients that had also acquired RHD, but DRB1*04/DQA1*0401 was often apparent in RF patients without RHD. In the distribution of HLA DQA1/DQB1 genotypes, both in RF patients and in the homogeneous patient groups, the least frequent were *0102/*0602-8. The genotype DQA1*0501 with the DQB1 risk allele *0301 was often found in the MVL group. The genotype *0301/*0401-2 was frequently found in the RF and Sydenhamn's chorea patient groups. The haplotype *07-*0201-*0302 was frequently found in RF and homogeneous patient groups, including the MVL group. In addition, haplotypes *04-*0401-*0301 and *04-*0301-*0401-2 were frequent amongst patients with Sydenhamn's chorea. The protective alleles DQA1*0102 and DQB1*0602-8 in the haplotype DRB1*15 were less frequently found in RF patients. The results of the present study support our hypothesis and indicate that certain HLA class II haplotypes are associated with risk for or protection against RHD and that these associations are more evident in patients in clinically homogeneous groups.
 ...
PMID:HLA class II DR and DQ genotypes and haplotypes associated with rheumatic fever among a clinically homogeneous patient population of Latvian children. 1755 88