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Over a period of two years, joint hypermobility was identified in 95 female and 19 male patients who attended rheumatology and rehabilitation units in Ismailia city. Pauciarticular pains referring to large and medium-sized joints was their most frequent complaint. Clinical diagnosis of carpal and/or tarsal tunnel syndromes was made in 45.6% of patients, and various forms of soft tissue rheumatism were evident in 73% of them. On radiologic evaluation of the involved joints, 60.5% of the examined patients showed significant degenerative lesions. The most prominent finding in the study, however, was the aggregation of varieties of articular and extra-articular abnormalities in the same patient. Extra-articular features included high frequencies of occurrence of varicose veins, piles and uterine prolapse among other abnormalities. Thus, results of the study lend support to the view that joint hypermobility predisposes to several articular and nonarticular lesions raise serious questions about the reputable benignity of the syndrome.
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PMID:Is the benign joint hypermobility syndrome benign? 835 97

We report a 40-year-old Japanese woman with antiphospholipid antibody syndrome (APS) associated with myasthenia gravis (MG). She had a history of miscarriage at the age of 27 followed by pulmonary embolism 3 weeks later. At the age of 40, she developed diplopia, bilateral ptosis and easy fatigability. Serum anti-acetylcholine receptor antibody and tensilon test were positive. She was diagnosed as having MG. The laboratory test revealed mild thrombocytopenia, prolonged activated partial thromboplastin time (aPTT) and positive findings for both beta 2-glycoprotein I-dependent anticardiolipin antibody and lupus anticoagulant. She fulfilled the diagnostic criteria of APS, but did not the criteria proposed by American Rheumatism Association for SLE. An extended total thymectomy was performed after administration of oral prednisolone and low-dose aspirin. This is a patient who had APS associated with MGs: both are known to result from autoimmune abnormality. The clinical and laboratory manifestations of APS were ameliorated after removal of the thymus, suggesting that thymectomy alleviates APS symptoms.
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PMID:[A case of antiphospholipid syndrome associated with myasthenia gravis]. 939 64

Systemic lupus erythematosus is a disease in which inflammatory process provoked by different antibodies affects many organs and systems. The circulatory system is one of them. In patients with systemic lupus erythematosus cardiac disorders are generally known and well proved. It is known that this disease has heritage background. Thus, the offsprings of patients suffering from systemic lupus erythematosus belong to a risk group. Moreover, it is thought that maternal antibodies crossing transplancentally to the fetus cause damages of tissues including the heart. The aim of this study was to evaluate cardiological status of 38 children whose mothers suffered from systemic lupus erythematosus. In all sick mothers the diagnosis fulfilled criteria of the American Rheumatism Association. The mothers have been remaining under medical treatment while the children have been under control and simultaneously prophylaxis of lupus has been undertaken. The study was undertaken in 17 girls and 21 boys aged 3 to 18 years (average: 12 +/- 4.5 years). Physical development of presented children was satisfactory. During cardiological examination all subjects were in good general condition, without any clinical evidence of collagen disease and infection. Obtained results were compared with the ones found in control group of 38 children of healthy mothers, being at the same age. Study methods were: physical examination, arterial blood pressure measurement, standard and 24 hours according to Holter method ECG record, echocardiographic and Doppler examination, and physical performance test according to Bruce's protocol. In children of sick mothers examined laboratory parameters were within the normal limits excluding the presence of antinuclear antibodies (controlled by indirect immunofluorescence test), result of which was positive in 15 studied children (39%). In the group of the children of sick mothers the abnormalities of sinus node function were detected in 12 cases (32%), significantly more often than in the control. There were found abnormalities of atrio-ventricular and intraventricular conduction in 15 subjects (40%). The premature beats of ventricular origin were noticed in 3 cases (8%). These disturbances were significantly different from the control group. In addition, correlation between the presence of antinuclear antibodies and the cardiac abnormalities was taken into consideration. So, significant correlation between antinuclear antibodies and heart rhythm disorders was proved. During echocardiographic examination structural and functional abnormalities were found. They were: ventricular septal defect (muscular part) (1), pericarditis effusion (1), prolapse of the mitral valve anterior leaflet (2), mitral valve regurgitation of the second degree (2) and increased diameter of left atrium (8). One girl from the studied group, suffering from atrio-ventricular block of III* was operated on because of persistent ductus arteriosus still in the newborn's period. At the same time the permanent pace-maker was implanted. After 1 year of age this girl was operated on because of atrial septal defect (ASD II). In studied group of children echocardiographic global indices of left ventricular systolic function were normal. The subclinical impairment of diastolic left ventricle function was found in 8 children with increased left atrium-aorta index. Both the theoretical knowledge and the results of the studies suggest that the offsprings of mothers suffering from SLE need a careful cardiological observation.
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PMID:[Cardiological state of offsprings of the mothers suffering from systemic lupus erythematosus]. 1105 11

Gender-, age- and race-related generalised joint hypermobility (GJH) is elucidated, based on publications that have used validated criteria for GJH. Furthermore, we analysed the connection between GJH and the clinical criteria for benign joint hypermobility syndrome (BJHS), and we looked for literature on the treatment of BJHS. There seems to be evidence in support of an increased prevalence of hypermobility among children, females and certain races when the diagnosis of hypermobility is based on the Carter and Wilkinson criteria (> or =3 positive tests out of 5) and/or Beighton's tests (> or =4 positive tests out of 9). However, there are no unequivocal statements that hypermobility predisposes to the various clinical situations used as major or minor criteria for BJHS, e.g., arthralgia, low back and pelvic pain, joint luxation, soft tissue rheumatism, abnormal cutis or genitourinary prolapse, varicose veins and hernia. There have been no randomised controlled studies of the effect of treatment.
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PMID:[Generalised joint hypermobility and benign joint hypermobility syndrome. II: epidemiology and clinical criteria]. 1630 62

Historically, the use of colonic irrigation or transanal irrigation (TAI) has been viewed as an alternative therapy for the treatment of a wide variety of conditions not relating to bowel dysfunction, including nausea, fatigue, depression, headache, anxiety and rheumatism. However, these days it is viewed as an effective treatment in individuals who may present with bowel dysfunction and related symptoms of constipation and faecal incontinence. Such individuals would include people with neuropathic bowel disorders, conditions that affect sphincter control or bowel motility disorders. injury to the rectum, sphincter or bowel, slow transit times, evacuation difficulties or prolapse due to a weak/damaged pelvic floor, and chronic faecal incontinence. TAI may be performed by the person with bowel dysfunction, or by a carer or health professional. An individual's ability to use a device to undertake the procedure will be influenced by a range of factors, which are explored in this article.
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PMID:Transanal irrigation: is it the magic intervention for bowel management in individuals with bowel dysfunction? 3227 46