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September 2009 KUWAIT MEDICAL JOURNAL 245 hemophilia in the pediatric population. Our patient showed dramatic response with no significant side effects. It appears, therefore, that the drug may be a useful addition to the treatment regime in childhood acquired hemophilia. ACKNOWLEDGEMENTS The authors acknowledge the contribution of all doctors, general pediatricians, and hematologists who were involved in this patient’s care, especially Dr. Lori Luchtman-Jones of the Department of Hematology/Oncology and Clinical Laboratories, Washington University Medical School, St. Louis MO, USA. REFERENCES 1 Huang YW, Saidi P, Philipp C. Acquired factor VIII inhibitors in non-haemophilic patients: clinical experience of 15 cases. Haemophilia 2004; 10:713-721. 2 Moraca RJ, Ragni MV. Acquired anti-FVIII inhibitors in children. Haemophilia 2002; 8:28-32. 3 Zakarija A, Green D. Acquired hemophilia: diagnosis and management. Curr Hematol Rep 2002; 1:27-33. 4 de la Fuente B, Panek S, Hoyer LW. The effect of 1- deamino 8 D- arginine vasopressin (DDAVP) in a nonhaemophilic patient with an acquired type II factor VIII inhibitor. Br J Haematol 1985; 59:127-131. 5 Kessler CM, Ludlam CA. The treatment of acquired factor VIII inhibitors: worldwide experience with porcine factor VIII concentrate. International Acquired Hemophilia Study Group. Semin Hematol 1993; 30: S22-S27. 6 Hay CR, Lozier JN, Lee CA, et al. Safety profile ofporcine factor VIII and its use as hospital and home-therapy for patients with haemophilia - A and inhibitors: the results of an international survey. Thromb Haemost 1996; 75:25-29. 7 Negrier C, Goudemand J, Sultan Y, Bertrand M, Rothschild C, Lauroua P. Multicenter retrospective study on the utilization of FEIBA in France in patients with factor VIII and factor IX inhibitors. French FEIBA Study Group. Factor Eight Bypassing Activity. Thromb Haemost 1997; 77:1113-1119. 8 Santagostino E, Morfini M, Rocino A, Baudo F, Scaraggi FA, Gringeri A. Relationship between factor VII activity and clinical efficacy of recombinant factor VIIa given by continuous infusion to patients with factor VIII inhibitors. Thromb Haemost 2001; 86:954- 958. 9 Narukawa N, Abe T, Shouno M, et al. A case of factor VIII inhibitor-positive acquired hemophilia treated by plasmapheresis. Ther Apher 1999; 3:323-325. 10 Jansen M, Schmaldienst S, Banyai S, et al. Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig–Therasorb). Br J Haematol 2001; 112:91-97. 11 Green D, Kwaan HC. An acquired factor VIII inhibitor responsive to high- dose gamma globulin. Thromb Haemost 1987; 58:1005-1007. 12 Schwartz RS, Gabriel DA, Aledort LM, Green D, Kessler CM. A prospective study of treatment of acquired (autoimmune) factor VIII inhibitors with high-dose intravenous gammaglobulin. Blood 1995; 86:797-804. 13 Spero JA, Lewis JH, Hasiba U. Corticosteroid therapy for acquired F VIII: C inhibitors. Br J Haematol 1981; 48:635-642. 14 Green D, Rademaker AW, Briet E. A prospective, randomized trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies. Thromb Haemost 1993; 70:753-757. 15 Silverman GJ, Weisman S. Rituximab therapy and autoimmune disorders: prospects for anti- B cell therapy. Arthritis Rheum 2003; 48:1484-1492. 16 Binstadt BA, Caldas AM, Turvey SE, et al. Rituximab therapy for multisystem autoimmune diseases in pediatric patients. J Pediatr 2003; 143:598-604. 17 Wiestner A, Cho HJ, Asch AS, et al. Rituximab in the treatment of acquired factor VIII inhibitors. Blood.2002; 100:3426-3428. 18 Stasi R, Brunetti M, Stipa E ,Amadori S. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood 2004; 103; 4424-4428.
246 KUWAIT MEDICAL JOURNAL September 2009 Case Report Short QT Syndrome: A Case Report and Review of Literature Jamal Hilal, Manal Al Suraikh, Rashid J Al Hamdan Department of Medicine, Al Jahra Hospital, Kuwait Kuwait Medical Journal 2009; 41 (3): 246-247 ABSTRACT Short QT syndrome (SQTS) was described for the first time in 2000. This report describes a case of SQTS in a 29- year-old male patient with resuscitated cardiac arrest in whom the diagnosis was missed in the first instance due to a lack of suspicion. Characteristic electrocardiographic findings provided the diagnosis. The patient was referred for the definitive implantable cardioverter defibrillator (ICD). KEY WORDS: atrial fibrillation, short QT syndrome, sudden cardiac death, ventricular fibrillation, ventricular tachycardia INTRODUCTION Short QT syndrome (SQTS) is a recently described familial disorder. The real incidence and prevalence of this disorder is not known. Patients with this syndrome have a high risk for cardiac arrhythmias [1,2] . Mutations in genes encoding for cardiac potassium channels have been identified to cause SQTS [3] . The diagnosis depends on a combination of one or more symptoms of syncope, cardiac arrest, palpitation, family history of sudden cardiac arrest, and the characteristic electrocardiographic (ECG) findings. The mainstay of treatment is the implantable cardioverter defibrillator (ICD) [4] . CASE REPORT A 29-year-old male presented to the emergency department (ED) with sudden collapse and loss of consciousness of 20 minutes duration. Basic life support was initiated by a bystander. In the emergency room patient had decreased level of consciousness, blood pressure (BP) 100/70, pulse 110/min regular and a respiratory rate of 16/min. Examination of other systems was normal. The patient was intubated for airway protection and ventilated and then transferred to the intensive care unit (ICU). In the ICU the patient developed many attacks of ventricular tachycardia (VT) in the form of polymorphic ventricular tachycardia (Fig. 1) which was treated successfully with direct current (DC) shock and intravenous (IV) amiodarone. Further history obtained from the patient confirmed the absence of any symptoms before the collapse, as well as the absence of any past medical or drug history. Family history revealed that his sister died suddenly at the age of 35 years. Investigation showed a normal complete blood count, erythrocyte sedimentation rate (ESR), renal, liver and thyroid function tests. Glucose and electrolytes including sodium, potassium, calcium, magnesium, phosphorous and arterial blood gases (ABG) were normal. Toxicology screen was negative. Electrocardiogram (Fig. 2) showed normal sinus rhythm with a short QT interval; absolute QT = 280 ms, corrected QT = 285 ms and peaked, symmetrical T wave in the precordial leads. Echocardiography was normal. The patient was treated with loading dose of intravenous amiodarone followed by a maintenance dose of 200 mg twice daily and did not show any recurrence of arrhythmias, but the QT interval was persistently short. He was referred to a tertiary hospital for the insertion of an automatic ICD. DISCUSSION SQTS was first described as a new congenital clinical syndrome by Gussak et al in 2000 [1] . It has been described to have an autosomal dominant inheritance due to repolarization abnormalities involving plateau phase of the action potential as a malfunction of potassium channels [2] . Three different mutations in genes encoding for cardiac potassium channels (KCNH2, KCNQ1 and KCNJ2) have been identified [3] . All mutations lead to abnormal function of the affected current IK(r), IK(s), IK(1). The possible substrate for the development of VTs may be a significant transmural dispersion of the repolarization due to a heterogeneous abbreviation of the action potential duration [4] . This lead to a high risk for an arrhythmia such as ventricular tachyarrhythmia. Furthermore, Address correspondence to: Dr. Jamal Hilal, P O Box 265, Al Jahra, Kuwait. E-mail: drhilal1@hotmail.com
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