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Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is an inherited disorder characterized by vascular dysplasiasleading to hemorrhages. If affects approximately 1 in10,000 Caucasian people. The most common presentation is chronic and recurrent epistaxis whereas bleeding from other sites can lead to life-threatening complications.
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Background: Diabetes is the most common non-communicable disease known today. This study was conducted to assess the clinical profile and complications in patients with type 2 diabetes mellitus.Methods: A prospective study was carried out between April 2017 to April 2018 in patients attending a tertiary care hospital in Chennai. Patients with type 2 diabetes of age 25-80 years, minimum of 5 years duration were enrolled.Results: Out of 66 patients, 23(34.8%) were males whereas 43 (65.2%) were females. Mean age in this study population was 55.36 years with a standard deviation of 11.362. In present study, the mean fasting blood sugar level was 196.12±77.180, mean postprandial blood sugar level was 303.26±115.385 and the mean HbA1C levels was 10.95±2.369. 77.3% were on oral hypoglycaemic agents, 13.6% on insulin and 9.1% on combined therapy with oral hypoglycemic agents and insulin. The complications associated with diabetes found in present study were microvascular complications which include peripheral neuropathy 41(62.1%), retinopathy 31(46.96%), nephropathy 28 (42.42%). Peripheral neuropathy was found to be most commonly associated. Macrovascular complications include cardiovascular disease in 12.12% (n=8) and cerebrovascular disease in 4.54% (n=3). Autonomic neuropathy was found in 69.69% (n=46).Conclusions: The complications of DM are commonly seen in patients with poor glycaemic control. Among microvascular complications, peripheral neuropathy was most commonly seen. Autonomic neuropathy usually goes unnoticed and asymptomatic in most of the individuals. Increasing levels of HbA1c was found to be significantly correlated with neuropathy.
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Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.
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Background: Autoimmune haemolytic anaemia (AIHA) is relatively uncommon condition with grave consequences, if not diagnosed and treated early. The literature on the clinical outcome and response to treatment is relatively scarce. Aim was to study the clinic-pathological profile and the treatment outcomes in patients with AIHA.Methods: Around 25 patients with AIHA attending a tertiary care hospital over a period of one year were included in the study. The patients were divided based on severity of anaemia and etiology. All the patients data were analysed for the demographic data, clinico -pathological findings and the response to treatment. All the patients data were analysed using SPSS software (version 22).Results: Out of 25 patients, 76% were females and 24% were males. Based on severity of anaemia, 60%, 28% and 8% had severe, moderate and mild anaemia. Around 48% of the patients had thrombocytopenia along with anaemia. 8 (32%) and 17 (68%) patients have primary and secondary AIHAs respectively. In our study the commonest cause for the secondary AIHA was Systemic Lupus Erythematosus (SLE) followed by haematological malignancy, primary Sjogrens, Anti-phospholipid antibody (APLA) syndrome, carcinoma colon and Wilsons disease. Hepatosplenomegaly and lymphadenopathy were present in 36% and 4% respectively. Out of 22 (88%) patients on corticosteroid therapy, 15 (60%) patients responded to corticosteroids alone and 6(24%) patients required corticosteroid plus immunosuppressive therapy.Conclusions: AIHA has to be ruled out in all anaemia patients with indirect hyperbilirubinemia and abnormal peripheral smear. Secondary AIHA is more common than primary. Corticosteroids and immunosuppressive agents are the mainstay of treatment of AIHA.