ABSTRACT
Background: In 1972, Dr Sharp and colleagues described a new connective tissue disease, characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis/ dermatomyositis (PM/DM) and by the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1 snRNP). This condition was termed mixed connective tissue disease (MCTD) and proposed as a distinct disease. Later, after observing the clinical evolution of MCTD patients, Sharp himself agreed that the original concept of MCTD had to be modified and that Internal organs were at risk for serious complications; patients were not always steroid responsive; prognosis was not always benign. Materials and methods: Patients in the age group of 15-50 years diagnosed to have connective tissue diseases were included. 8 patients in the age group of 15-50 admitted in Medicine department were taken and they were evaluated for the clinical profile of sharp syndrome by thorough clinical examination, routine laboratory tests and special investigations depending on the clinical profile. Results: 8 patients with connective tissue disease attending the medicine OPD were studied. Of these 8 patients were female patients. The median age of onset was 36 years, 8 patients met criteria by sharp and Alarcon-Segovia. The clinical features of patients at presentation are Raynaud抯 phenomena, Puffy fingers, esophagus dysmotility, skin rash, interstitial lung disease, arthritis, pulmonary hypertension, myositis, anemia. Conclusion: SHARP syndrome is a rare condition, as evidenced by the small series of cases reported to date. Diagnosis is based on clinical and paraclinical criteria. The evolution can be interspersed with various complications that can affect the short, medium and long-term prognosis
ABSTRACT
Background: The most common, non –renal, chronic disorder in patients with ESRD is gastro intestinal disorders, accompanying ESRD including those receiving renal replacement therapy. Among upper gastrointestinal lesions caused by chronic kidney disease- gastritis, esophagitis, gastric ulcer are the most prevalent lesions. Here an attempt is being made to study the upper gastro intestinal changes in chronic kidney disease and evaluate their relationship with the stage of CKD or GFR. Materials and methods: A cross sectional study on 50 patients of, who were diagnosed to have chronic kidney disease and being presented to OPD and admission in Malla Reddy Institute of Medical Sciences, Suraram over a period of one year. All patients with chronic kidney disease who underwent upper gastrointestinal endoscopy were included in the study. Results: 50 patients were included in the study with mean age and males 28 and 22 females. Among 50 patients 42 patients manifested with upper gastrointestinal lesion among them erosive gastritis 13(26%) was most common upper gastrointestinal lesion followed by gastro esophageal reflux disease with or without duodenitis 8 (16%), duodenal ulcer, gastric ulcer 4(8%) each, pangastritis 3(6%), GERD with gastritis, erosive duodenitis, erosive esophagitis, pale gastric mucosa 2(4%) each, angiodysplasia and hiatus hernia 1(2%) each. Conclusion: Majority of the patients with chronic kidney disease have upper gastrointestinal involvement on endoscopic evaluation. Erosive gastritis is the most common lesion Esophageal and duodenal involvement is less common than the gastric lesions. Upper gastrointestinal findings are frequently observed in chronic kidney disease patients on dialysis. Early diagnosis and managementof these upper gastrointestinal lesions in CKD can reduce mortality and morbidity and prevent fatal complication like massive upper gastrointestinal bleed.