ABSTRACT
Cardiac resynchronization therapy (CRT) has been proven its value in adult patients with congestive heart failure of low ejection fraction and wide QRS duration. Contrast to adult patients, CRT has been rarely applied for young patients. We report on a 9-yr-old boy with progressive left ventricular (LV) dilatation and dysfunction following chronic VVI pacemaker therapy for congenital complete atrioventricular block associated with maternal anti-SSA/Ro and SSB/La antibody. His LV dysfunction was improved after epicardially established CRT.
Subject(s)
Child , Humans , Male , Antibodies, Antinuclear/metabolism , Atrioventricular Block/congenital , Cardiac Pacing, Artificial/adverse effects , Cardiac Resynchronization Therapy , Chronic Disease , Electrocardiography , Heart Ventricles , Natriuretic Peptide, Brain/blood , Sjogren's Syndrome/immunology , Ventricular Dysfunction, Left/etiologyABSTRACT
AIM: To study the clinical and immunological profile in patients of systemic sclerosis from North India and compare it with other ethnic groups. METHODS: Patients presenting to us between the years 2001 and 2004 and fulfilling the American Rheumatism Association (ARA) criteria for systemic sclerosis were included. There were 84 females and 16 males with the mean age of 32.5 +/-11.62 years and a mean duration of 6.49 +/- 4.34 years. All patients were admitted to the dermatology ward for detailed history and examination including Rodnan score. Investigations including hemogram, hepatic and renal functions, serum electrolytes, urine for albumin, sugar, microscopy and 24h urinary protein estimation, antinuclear antibody, chest X-ray, barium swallow, pulmonary function test, electrocardiogram and skin biopsy were done. RESULTS: The most common presenting symptoms were skin binding-down (98.5%), Raynaud's phenomenon 92.9%, pigmentary changes 91%, contracture of fingers 64.6%, fingertip ulcer 58.6%, restriction of mouth opening 55.5%, dyspnea 51.1%, joint complaints 36.7% and dysphagia in 35.2%. The mean Rodnan score was 25.81 +/- 10.04 and the mean mouth opening was 24.6 +/- 19.01 mm. The laboratory abnormalities included raised ESR in 87.8%, ANA positive in 89.1%, proteinuria in 6.0%, abnormal chest X-ray in 65.3%, abnormal barium swallow in 70.2% and reduced pulmonary function test in 85.8%. CONCLUSION: The clinical and immunological profile of systemic sclerosis in North India is similar to that of other ethnic groups except that pigmentary changes are commoner and renal involvement is relatively uncommon.
Subject(s)
Adolescent , Adult , Aged , Antibodies, Antinuclear/metabolism , Asian People , Child , Contracture/etiology , Digestive System/physiopathology , Ethnicity , Female , Hand/diagnostic imaging , Humans , India , Male , Middle Aged , Mouth/physiopathology , Osteoporosis/complications , Pigmentation Disorders/etiology , Radiography, Thoracic , Scleroderma, Systemic/complications , Skin/pathology , Tissue DistributionABSTRACT
We concentrated ourselves to evaluate the prognostic significance of the p53 gene mutations, its protein expression and MIB-1 index as a proliferative marker in canine mammary tumors. In the present study, a total of 20 cases were examined, among which there were 5 malignant mixed tumors, 4 mammary gland adenocarcinomas, 1 papillary adenocarcinoma, 8 benign mixed tumors and 2 mammary gland adenomas. Positive immunostaining for p53 with PAb240 antibody was found in 2 benign (20%) and 3 malignant (30%) tumors. However, PAb421 antibody did not give positive result at all. In Western blot analysis, the p53 expression in benign and malignant tumors was detected in 4 and 3 cases, respectively. p53 mutations were found in 6 cases out of the cases with detected p53 protein expression. The MIB-1 index in benign and malignant tumors were 17.6+/-20.8% and 29.0+/-27.2%, respectively and there was no significant difference between tumor types. There was a significant correlation between p53 mutations and p53 overexpression (correlation coefficient = 0.5, p < 0.05). In Kaplan-Meier survival analysis, the p53 index was associated with significantly shortened survival time (p < 0.01). In multivariate analysis, p53 overexpression was only an independent factor for indicator of worse prognosis in canine mammary tumors (p = 0.01). These results demonstrated that p53 gene mutations and protein overexpression using the PAb240 anti-p53 antibody were useful predictors of increased malignant potential and poor prognosis in canine mammary tumors.
Subject(s)
Animals , Dogs , Female , Antibodies, Antinuclear/metabolism , Antibodies, Monoclonal/metabolism , Blotting, Western/veterinary , Dog Diseases/genetics , Genes, p53/genetics , Immunohistochemistry/veterinary , Ki-67 Antigen/metabolism , Mammary Neoplasms, Animal/genetics , Mutation , Predictive Value of Tests , Proportional Hazards Models , Tumor Suppressor Protein p53/biosynthesisABSTRACT
Os autores utilizaram técnicas de imunofluorescência para estudar a topografia de proteínas Ro e La em células linfoblastóides sincronizadas de murinos utilizando soro humano mono-específico anti-Ro/SSA. Durante a fase S di cucki celular, o Ro/SSA foi visto como um salpicado grosso no núcleo e no citoplasma. Na fase M, o Ro/SSA apareceu no corpo celular como um padräo reticulado e ficou menos nítido na fase G1. Por outro lado, o La/SSB foi observado durante todo o ciclo celular. Na fase G1, o La/SSB foi visto no nucléolo; na fase S, no núcleo, como um padräo salpicado fino, e na fase M, o padräo salpicado permaneceu em todo o corpo celular. Essas observaçöes indicam que o Ro/SSA é expresso durante as diversas fases do ciclo celular e sugerem, ainda, a dependência celular desse antígeno