الفهرس 
AcknowledgementOnly 14 pages are availabe for public view
 |  | from | 146 |  |  |
| | | from | 146 | | |
Abstract
Connective tissue diseases (CTDs) are defined as a group of acquired diseases resulting from persistent immune mediated inflammation. In most CTDs there is immune dysregulation resulting in generation of autoreactive T cells or auto-antibodies. These antibodies can attack any organ of the body, resulting in a wide array of signs and symptoms. CTDs, though rare in childhood, are an important cause of morbidity and mortality. Most of them involve multiple organ systems and are associated with presence of auto-antibodies. Systemic lupus eryethematosus (SLE) is the most common CTDs, the others being Juvenile idiopathic arthritis, Juvenile dermatomyositis, systemic sclerosis, mixed connective disease and Sjogren syndrome and others.
Diagnosis of CTDs should suspect when a child has a multi system disease with no apparent cause. Other clinical features that suggest CTDs include prolonged fever, oral ulcers, Raynaud’s phenomenon, skin rash (malar rash, Heliotrope rash, nodules), photosensitivity, alopecia, pleuropericarditis, glomerulonephritis, arthritis, unexplained abdominal pain, muscle weakness, sicca symptoms (dry eyes and dry mouth). Final diagnosis of specific type of CTDs depends on the clinical presentation and the presence of disease specific auto-antibodies.
Patients with CTDs have a higher risk of cardiac involvement, which can result in atrio-ventricular dysfunction. Little is known about the acute and chronic influence of CTDs on heart function in children and adolescents. There are many cardiovascular manifestations of CTDs include pericarditis, myocarditis, Libman Sacks endocarditis, cardiomegaly, congestive heart failure. Left atrio-ventricular diastolic dysfunction has been reported in systemic lupus erythematosus (SLE). However there are little informations about cardiac functions and cardiac enzymes markers in CTDs especially in developing country.
There for the aim of this work to study cardiac functions as ejection fraction (%), fraction shorting (%), left ventricular function assessment (left ventricular end diastolic and systolic time interval), pericardium and endocarium valve assessments in different types CTDs in children by echocardiography and ECG. Furthermore, the role of myoglobin and other cardiac markers as troponnin I, creatine kinase (CK-MB) and lactate dehydrogenase (LDH) in early detection of myocardial damage in children with CTDs were studied. Moreover, correlate of echocardiography, ECG and cardiac enzymes markers finding with various clinical, laboratory, disease activity parameters and medications used in treatments in CTDs patients.
All included children and adolescence were subjected to detailed history taking, clinical examination, laboratory investigations, auto-antibodies assessments as rheumatoid factors, antinuclear antibodies (ANA), anti-double-stranded DNA antibodies (Anti ds DNA), anti-smooth muscle antibody (anti-SM antibody. Two–dimensional (2D) echocardiography, ECG and chest x-ray were performed to all patients. Cardiac markers as myoglobin, troponnin I, creatine kinase (CK-MB) and lactate dehydrogenase (LDH) were done to all children included in the study.
This study carried out at Sohag University Hospitals, during the period from 1/1/2015 to 31/12/2015 at the Department Of Pediatrics, at Sohag University, in collaboration with the Rheumatology Department at Sohag University. A total of children 44 children and adolescence met the inclusion criteria (22 males and 22 females) with the mean age 11.68±3.04 years and range from 7-16 years, Mean ± SD duration of diseases/year were 3.05±2.61years. In this study, 44 cases were diagnosis to had CTDs, 20 (45.45%) cases with idiopathic rheumatoid arthritis (IRA), 14 (31.82%) cases with SLE and 10 (22.73%) cases with dermatomyositis.
As regard cardiac functions of studied population, the mean ± SD of ejection fraction was 64.32±5.93, the mean ± SD of shorting fraction was 34.59±3.71, the mean ± SD of left atrial dimension (LA) was 21.18±2.25, mean ± SD of Left Ventricular End Systolic dimensions (LVESD) was 34.68±5.29, the Mean ± SD of Left ventricular End Diastolic dimensions (LVEDD) was 38.68±12.31, the mean ± SD of Left ventricular posterior wall dimensions 13.27±1.62, the Mean ± SD of septal wall thickness(IVS) was 12.95±1.47, in this study, there were 4 (9.09%) cases had mild effusion and only one cases had thick pericardium, mitral regurge in 8 (18.18%) patients, tricuspid regurge in 8 (18.18%) patients,
As regard cardiac marker of studied population, the mean ± SD of troponin was 0.07±0.07 (ng/ml), mean ± SD of CK-MB was 148.09±167.66 (IU/L), the mean ± SD of LDH was 260.09±244.16 (IU/L), and the mean ± SD of myoglobin was 61±27.38 (ng/mL).
The change in cardiac function and cardiac enzymes were not diseases specific. Nevertheless, in patients with CTDs with age more than 10 years, there were statically significant differences between the presence of mitral regurge and tricuspid regurge, there were 6 cases in each group compared to 2 cases in CTDs patient with age less than 10 (P Value= 0.01 and 0.01 respectively). Moreover, with increase the durations of CTDs, there were statically significant impairment in echo finding in Ejection fraction and Shorting fraction, mitral regurg, tricusp regurg (P Value= 0.02, 0.02, 0.001, and 0.001 respectively). Furthermore, with increase the durations of CTDs more than 6 years, there were statically significant increase in cardiac markers as Mean ± SD of serum troponin (ng/ml), Mean ± SD of serum CK-MB (IU/L), Mean ± SD of serum LDH and Mean ± SD of serum myoglobin (ng/ml) (P Value= 0.002, 0.0006, 0.0003, and 0.0002 respectively).
Moreover, there were no statically differences between CTDs patients with auto-antibodies and the presences of cardiac functions and cardiac markers impartment’s Furthermore, the most frequents used medication in the treatments of CTDs was Steroids therapy in 36 (81.82%) patients, flowed by Leflunomide (Arthfree®) 16 (36.36%) patients, then Methotrexate in 10 (22.73%) patients, plus some vitamins and minerals in most of the patients. There was no statically significant difference between the effect of without or with steroid treatment in CTDs patients and the presences cardiac function impairments of studied population.
Conclusion:
CTDs, though rare in childhood, are an important cause of morbidity and mortality. Most of them involve multiple organ systems and are associated with presence of auto-antibodies. Diagnosis of CTDs should suspect when a child has a multi system disease with no apparent cause. A total of children 44 children and adolescence met the inclusion criteria (22 males and 22 females) with the mean age 11.68±3.04 years and range from 7-16 years, Mean ± SD duration of diseases/year were 3.05±2.61years. In this study, 44 cases were diagnosis to had CTDs, 20 (45.45%) cases with idiopathic rheumatoid arthritis (IRA), 14 (31.82%) cases with SLE and 10 (22.73%) cases with dermatomyositis. The change in cardiac function and cardiac enzymes were not diseases specific or related to the presence of auto-antibodies or use of mediations. But with increase the duration of CTDs and age of patients there were statically significant increase in cardiac function and cardiac enzymes. Furthermore the presences of mitral regurege associated in mild impairments in cardiac functions by Echo, ECG and cardiac enzymes.