Severe pulmonary arterial hypertension in congenital sideroblastic anemia from PUS1 mutation - a case report.

Background: Myopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate.

"Assessing the hemodynamic impact of various inotropes combination in patients with cardiogenic shock with Non-ST elevation myocardial infarction -the ANAPHOR study".

Background: Various inotropic agents/vasopressors combinations are used in patients of cardiogenic shock. We performed this study to observe hemodynamic effects of various inotrope/vasopressor combinations in patients with NSTEMI cardiogenic shock (CS) at tertiary cardiac centre METHODS AND MATERIALS: Of 3832 NSTEMI, we studied 59 consecutive such patients with CS who hadn't undergone revascularization in the first 24 h in a prospective, open label, observational study. Group 1 comprised of background Dopamine with Noradrenaline titration(N = 38), Group 2 had background Dobutamine and Noradrenaline titration(N = 15) and Group 3 comprised of triple combination of Dopamine, Noradrenaline & Adrenaline(N = 6).

How does clinical profile and outcome differ in patients with Chronic Kidney Disease undergoing percutaneous coronary revascularization according to the severity of CKD? - CHANNEL Study.

Background: Chronic kidney disease (CKD) is an independent risk factor for the development of coronary artery disease. We evaluated outcomes amongst patients of CKD undergoing percutaneous coronary intervention (PCI) as assessed on severity of CKD based on estimated glomerular filtration rate (eGFR) at the time of PCI.

Method And Materials: We analyzed 100 consecutive CKD patients who underwent PCI and were followed up for 1 year; an observational, prospective, open-label study.

Diagnostic accuracy of a novel 'winking coronary angiographic sign' in patients presenting with ventricular septal rupture complicating acute myocardial infarction.

Ventricular septal rupture (VSR) is an uncommon but potentially lethal complication of acute myocardial infarction (MI). Its prompt recognition is essential to permit timely institution of corrective measures. The present study was undertaken to assess the diagnostic accuracy of a novel and unique angiographic sign, the 'winking coronary sign (WCS)', for recognizing post-MI VSR.

Androgenic alopecia, premature graying, and hair thinning as independent predictors of coronary artery disease in young Asian males.

Unlabelled: We herewith aimed to explore the association of premature graying, androgenic alopecia (AGA), and hair thinning with coronary artery disease (CAD) in young (≤40 years) male individuals from Western India.

Patients And Methods: In this prospective, case-control study, 1380 male individuals from a super speciality cardiac care center were enrolled, of which 468 were established cases of CAD and 912 were age-matched healthy male individuals not having history of any major illness including CAD. Details of demographics, cardiovascular risk factors, and cutaneous markers were collected for both the groups.

Facial Diplegia with Paresthesia: An Uncommon Variant of Guillain-Barre Syndrome.

Facial nerve palsy (FNP) is a common medical problem and can be unilateral or bilateral. Unilateral facial palsy has an incidence of 25 per 100,000 population and most of them are idiopathic. However, facial diplegia or bilateral facial nerve palsy (B-FNP) is rare with an incidence of just 1 per 5,000,000 population and only 20 percent cases are idiopathic.

Benign Recurrent Intrahepatic Cholestasis in a Young Adult.

Benign Recurrent Intrahepatic Cholestasis (BRIC) is a rare genetic disorder characterized by recurrent episodes of cholestatic jaundice. The initial episode of jaundice generally occurs before second decade of life and can persist for several weeks to months before resolving spontaneously. It is a benign disease and even after repeated episodes of jaundice, fibrosis of liver cell does not occur.

Leukemoid Reaction in Chikungunya Fever.

Chikungunya is a viral illness caused by an arbovirus which is transmitted by Aedes mosquito. Fever and polyarthralgia are hallmark of this viral illness. Viral infections are generally associated with leucopenia and bacterial infections with leukocytosis.

Intravenous Immunoglobulin Responsive Persistent Thrombocytopenia after Dengue Haemorrhagic Fever.

Dengue outbreak is common in Indian subcontinent and causes significant morbidity and mortality. Year 2015 has witnessed yet another Dengue epidemic in northern India and the number of cases this year is maximum in a decade. Dengue infection is a viral disease and there are 4 different serotypes DENV1, DENV2, DENV3 and DENV4.

Metolazone Associated Stevens Johnson Syndrome-Toxic Epidermal Necrolysis Overlap.

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe mucocutaneous disease with high mortality rate. It is characterised by severe necrosis and detachment of the epidermis. Drugs are the most common triggering agent for SJS/TEN.