Diabetic ketoacidosis with Ebstein's anomaly in an adult

Ebstein's anomaly is a rare form of congenital malformation of the heart, characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. It may not be detected until late in adolescence or adulthood. The clinical manifestations of Ebstein's anomaly vary greatly. We are reporting a case of 35-year male who presented with generalized fatigue, palpitation and effort intolerance. Laboratory investigations confirmed the diagnosis of diabetes ketosis. Transthoracic echocardiography showed severe Ebstein's anomaly with severe tricuspid regurgitation, no residual atrial septal defect, but with severe right ventricular dysfunction. Though only few studies showed the high prevalence of abnormal glucose metabolism in young adult patients with complex congenital heart disease, but Epstein's anomaly with diabetes ketosis was nowhere mentioned

Short term efficacy and safety of low dose tolvaptan in patients with acute decompensated heart failure with hyponatremia: a prospective observational pilot study from a single center in South India

In acute decompensated heart failure [ADHF], diuretic use, the mainstay therapy for congestion, is associated with electrolyte abnormalities and worsening renal function. Vasopressin mediates fluid retention in heart failure. In contrast to diuretics, the vasopressin antagonist tolvaptan may increase net volume loss in heart failure without adversely affecting electrolytes and renal function. Hyponatremia [serum sodium concentration, <135 mEq/L] is a predictor of death among patients with heart failure. We prospectively observed the short term efficacy and safety of low dose [15 mg] tolvaptan in admitted patients with hyponatremia and ADHF in Indian population. A total of 40 patients with ADHF along with hyponatremia [<125 mEq/L] on standard therapy were treated with 15 mg of tolvaptan at a single oral dose for 7 days. Serum sodium concentrations increased significantly after treatment with tolvaptan from baseline [P < 0.02]. There was a significant improvement in symptoms and New York Heart Association [NYHA] class after starting tolvaptan [P

Recurrent myocardial infarction in a case of congenital afibrinogenemia

Afibrinogenemia is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:1,000,000. Usual presentation of this disorder is spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures. Paradoxically, few patients with afibrinogenemia may also suffer from severe thromboembolic complications. The management of these patients is particularly challenging because they are not only at risk of thrombosis but also of bleeding. We are presenting a case of 33-year-old male patient of congenital afibrinogenemia who had two episodes myocardial infarction in a span of two years. The patient was managed conservatively with antiplatelet therapy and thrombolytic therapy was not given due to high risk for bleeding

Lichen scrofulosorum presenting as pyrexia of unknown origin

Cutaneous tuberculosis forms a subset of extra pulmonary tuberculosis. It accounts for about 1.5% of all the cases of extrapulmonary tuberculosis. Lichen Scrofulosorum is a rare form of cutaneous tuberculosis. It is usually seen in children or young adults and an important marker of occult tuberculosis. Usually, the eruption appears in children and adolescents with strong immune sensitivity to Mycobacterium tuberculosis and consists of tiny follicular papules. Underlying systemic involvement is more common in children, compared with adults. We report a case of 3 years old boy who was investigated for pyrexia of unknown origin and finally diagnosed as Lichen Scrofulosorum associated with tuberculous lymphadenitis of mediastinal lymph nodes. A skin biopsy showed extensive tuberculoid granulomas surrounding hair follicles which were consistent with the diagnosis of Lichen Scrofulosorum. The child was treated with antitubercular drugs and it showed improvement within 2 weeks of starting treatment with resolution of skin lesions

Urorectal septum malformation sequence in a newborn with VACTERL association

Urorectal septum malformation sequence [URSMS] is an extremely rare anomaly, consists of multiple system anomalies including ambiguous genitalia, absence of a perineal opening, an imperforate anus, and urological, colonic and lumbosacral defects. We describe a newborn with characteristic URSMS who also had features of congenital varus deformity of leg, polydactyly, tracheo-oesophageal fistula, cardiac defect, anal atresia and hydronephrosis in antenatal ultrasound characteristic of VACTERL association

Demographic and clinical profile of children under two years of age with recurrent wheezing

Sixty-seven children less than two years of age with recurrent wheezing were evaluated clinically and demographically by proper history and clinical examinations. The mean age of the study subjects at enrolment and at onset of wheezing was 10.8 +/- 5.24 months and 7.1 +/- 3.857 months, respectively. The male-female ratio was 9:2. The majority of these cases [82%] had onset of wheezing at less than 1 year of age. One-third of the cases were diagnosed as asthma and viral infection associated wheeze. Increasing age in the first 2 years of life was significantly associated with decreasing trend of cases with history suggestive of reflux. The children with wheeze due to causes other than gastroesophageal reflux were more likely to be not exclusively breast fed. The present study did not find any significant difference in the prevalence of various socioeconomic, environmental factors and clinical factors among the various types of the early wheezers