DOCSLIB.ORG

Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male Judy Wang, MD Thomas Jefferson University

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male Judy Wang, MD Thomas Jefferson University The Medicine Forum Volume 13 Article 17 2012 Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male Judy Wang, MD Thomas Jefferson University Niluk Peiris, MD Thomas Jefferson University Follow this and additional works at: https://jdc.jefferson.edu/tmf Part of the Medicine and Health Sciences Commons Let us know how access to this document benefits ouy Recommended Citation Wang, MD, Judy and Peiris, MD, Niluk (2012) "Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male," The Medicine Forum: Vol. 13 , Article 17. DOI: https://doi.org/10.29046/TMF.013.1.018 Available at: https://jdc.jefferson.edu/tmf/vol13/iss1/17 This Article is brought to you for free and open access by the Jefferson Digital Commons. The effeJ rson Digital Commons is a service of Thomas Jefferson University's Center for Teaching and Learning (CTL). The ommonC s is a showcase for Jefferson books and journals, peer-reviewed scholarly publications, unique historical collections from the University archives, and teaching tools. The effeJ rson Digital Commons allows researchers and interested readers anywhere in the world to learn about and keep up to date with Jefferson scholarship. This article has been accepted for inclusion in The eM dicine Forum by an authorized administrator of the Jefferson Digital Commons. For more information, please contact: [email protected]. Wang, MD and Peiris, MD: Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male The Medicine Forum Thrombotic Thrombocytopenic Purpura Revisited: Upshaw Schulman Syndrome in a 20-Year-Old Male Judy Wang, MD and Niluk Peiris, MD Case Report nisolone, folate, and blood products until an apheresis catheter could be placed for plasma exchange therapy. Once the catheter A 20-year-old male with no significant past medical history was placed, the patient underwent five sessions of plasma presented to an outside hospital with a two-week history of exchange therapy without complications. Human immuno- progressive fatigue, malaise and decreased appetite. Prior to deficiency virus (HIV) antibody, hepatitis B and C serologies these events, he had been in his usual state of health with normal were negative. His steroids were tapered, and blood counts and exercise tolerance and energy level. Upon development of his LDH were monitored daily. Upon discharge, hemoglobin had symptoms, the patient felt as though he had developed an upper increased to 8.2 mg/dL, RDW 20.3%, platelet count 259 B/L, respiratory infection (URI). The URI symptoms eventually and LDH 263 IU/L. Haptoglobin had increased to 46 mg/dL and resolved; however complaints of fatigue and malaise still serum creatinine had improved to 0.8 mg/dL. A Disintegrin-like remained. He then developed progressive dyspnea on exertion And Metalloprotease with ThromboSpondin type 1 member and tea-colored urine. The patient reported a singular febrile 13 (ADAMTS13) activity was determined to be severely low episode to 38.9˚C associated with nonbilious vomiting two days at <5%, and ADAMTS13 inhibitor activity was <0.4 units. prior to admission. Upon further questioning, he admitted to Following discharge, the patient returned to the Emergency a twenty-pound weight loss over the last month. The patient’s Department two weeks later for fever of 38.4˚C and malaise, mother also noticed unprovoked bruising on his forearms however blood counts showed hemoglobin 9.8 g/dL, RDW bilaterally. He denied night sweats, gingival bleeding, epistaxis, 15.9%, platelet count 145 B/L, and serum creatinine 1.1 mg/dL, hematuria, or melena. so he was deemed stable for close follow-up as an outpatient. The patient is of Afro-Jamaican descent; his family originating from Trinidad. He reported no family history of hematologic disorders, and only acknowledged a maternal grandmother Introduction with breast cancer. The patient denied chronic medications, Congenital thrombotic thrombocytopenic purpura (CTTP), including herbal and over-the-counter medications. He denied also known as Upshaw-Schulman Syndrome (USS), is a very food or drug allergies, as well as alcohol, tobacco or illicit drug rare, but potentially life-threatening disease. It is characterized use. He reported being sexual active with one partner in the past by the symptoms also seen in acquired or idiopathic TTP, 6 months and denied any sexually transmitted infections. including thrombocytopenia, MAHA, and subsequent microvascular thrombosis. However this constellation of Hospital Course symptoms is seen in conjunction with significant and persistent deficiency in ADAMTS13 with concurrent absence of an On admission to the outside hospital, laboratory results ADAMTS13 inhibitor. ADAMTS13 is a protease responsible for revealed a profound anemia (hemoglobin 6.3 g/dL) with an modifying the larger precursor of von Willebrand factor (vWF), elevated red cell distribution width (RDW) (22.4%), thrombo- a necessary component of the clotting cascade. Continued cytopenia (platelet count 44 B/L) and acute kidney injury disruption of this process leads to microvascular embolization (serum creatinine 1.9 mg/dL). Subsequent testing reported and may ultimately cause potential end organ failure, including the presence of 4+ schistocytes on peripheral blood smear heart, brain, and kidneys. concerning for microangiopathic hemolytic anemia (MAHA). Given his triad of anemia, thrombocytopenia and presence of schistocytes, there was a concern for thrombotic thrombocy- Historical Background topenic purpura (TTP). He was transferred to our institution Cases concerning for CTTP were first reported in 1953, when for further management. Prior to transfer, the patient was Dacie and colleagues described a 6-year-old female who suffered medically supported with blood products and underwent a bone repeated episodes of thrombocytopenia, hemolytic anemia, marrow biopsy that was determined to be non-malignant. presence of schistocytes, and significant jaundice since birth. The Upon arrival to our institution, further laboratory results young patient underwent splenectomy without improvement of revealed a significantly elevated lactate dehydrogenase (LDH) symptoms, and eventually died of renal failure at age 7. Upon (956 IU/L) with essentially undetectable haptoglobin (<10 mg/ review of the patient’s family history, she had two other siblings, dL). Urinalysis was positive for 2+ protein and 1+ blood. Direct who were jaundiced as newborns and both died of hemorrhage Coombs test was negative. Repeat peripheral blood smear was by the age of 4. However the fourth child and both parents positive for the presence of 3+ schistocytes, confirming concern were asymptomatic, so Dacie suspected that the patient and her for TTP. The patient was transferred to the medical intensive family had some yet to be determined inherited blood disorder.1 care unit and immediately started on intravenous methylpred- In 1960, Schulman reported an 8-year-old female who suffered 43 Published by Jefferson Digital Commons, 2012 1 The Medicine Forum, Vol. 13 [2012], Art. 17 from repeated bleeding episodes secondary to chronic thrombo- ultra-large vWF (UL-vWF), of which ADAMTS13 immediately cytopenia and MAHA. Symptoms dramatically improved with cleaves into vWF for appropriate function in the clotting fresh frozen plasma (FFP), suggesting that the patient had a cascade. In the absence of ADAMTS13, UL-vWF released from congenital deficiency in some “platelet-stimulating factor.”2 vascular endothelial cells is not cleaved appropriately, which Upshaw and colleagues in 1978 would later report a 29-year-old induces platelet hyperagglutination under high shear stress.9 female who had repeated episodes of thrombocytopenia and Uncleaved UL-vWF then binds to circulating platelets forming MAHA since childhood, that was treated with FFP.3 microthrombi, which both embolize into the microvasculature of end organs and cause fragmentation of adjacent circulating The syndrome for CTTP was coined by Rennard and Abe, erythrocytes, thereby producing MAHA. who recognized the similarities between the Upshaw and Schulman’s cases, and they postulated that these patients developed their sequelae as a result of increased fibronectin Epidemiology during the symptomatic phase of their disease.4 However, multiple subsequent studies would later show there was no The incidence and prevalence of USS is extremely rare. It association with fibronectin and disease activity.5,6,7 The assay is estimated that there have only been 100 cases worldwide to assess ADAMTS13 activity was created in 1997, but it was to date, with an incidence rate of no more than 1/1,000,000 9,10 not until 2001 that the link between ADAMTS13 mutation and person/year. USS comprises only 10% of all TTP patients with 11 subsequent deficiency and CTTP or USS was confirmed by Levy known ADAMTS13 deficiency, Time of initial presentation and colleagues by means of linkage analysis performed on four varies considerably; in one study of 43 USS patients in Japan, 12,13 pedigrees with CTTP.8 age of onset ranged from early childhood to 79 years. With regard to racial predilection, USS cases have been reported in all continents, although there is an increased reported Pathophysiology incidence among patients of Japanese, Central European, and Jamaican descent.9 The most commonly reported mutation USS is inherited through an autosomal recessive pattern, in ADAMTS13, c.4143dupA, was found through haplotype though based upon clinical
Load more

Recommended publications
  • 081999 Disseminated Intravascular Coagulation
    The New England Journal of Medicine Current Concepts Systemic activation+ of coagulation DISSEMINATED INTRAVASCULAR COAGULATION Intravascular+ Depletion of platelets+ deposition of fibrin and coagulation factors MARCEL LEVI, M.D., AND HUGO TEN CATE, M.D. Thrombosis of small+ Bleeding and midsize vessels+ ISSEMINATED intravascular coagulation is and organ failure characterized by the widespread activation Dof coagulation, which results in the intravas- Figure 1. The Mechanism of Disseminated Intravascular Coag- cular formation of fibrin and ultimately thrombotic ulation. occlusion of small and midsize vessels.1-3 Intravascu- Systemic activation of coagulation leads to widespread intra- lar coagulation can also compromise the blood sup- vascular deposition of fibrin and depletion of platelets and co- agulation factors. As a result, thrombosis of small and midsize ply to organs and, in conjunction with hemodynam- vessels may occur, contributing to organ failure, and there may ic and metabolic derangements, may contribute to be severe bleeding. the failure of multiple organs. At the same time, the use and subsequent depletion of platelets and coag- ulation proteins resulting from the ongoing coagu- lation may induce severe bleeding (Fig. 1). Bleeding may be the presenting symptom in a patient with disseminated intravascular coagulation, a factor that can complicate decisions about treatment. TABLE 1. COMMON CLINICAL CONDITIONS ASSOCIATED WITH DISSEMINATED ASSOCIATED CLINICAL CONDITIONS INTRAVASCULAR COAGULATION. AND INCIDENCE Sepsis Infectious Disease Trauma Serious tissue injury Disseminated intravascular coagulation is an ac- Head injury Fat embolism quired disorder that occurs in a wide variety of clin- Cancer ical conditions, the most important of which are listed Myeloproliferative diseases in Table 1.
    [Show full text]
  • What Everyone Should Know to Stop Bleeding After an Injury
    What Everyone Should Know to Stop Bleeding After an Injury THE HARTFORD CONSENSUS The Joint Committee to Increase Survival from Active Shooter and Intentional Mass Casualty Events was convened by the American College of Surgeons in response to the growing number and severity of these events. The committee met in Hartford Connecticut and has produced a number of documents with rec- ommendations. The documents represent the consensus opinion of a multi-dis- ciplinary committee involving medical groups, the military, the National Security Council, Homeland Security, the FBI, law enforcement, fire rescue, and EMS. These recommendations have become known as the Hartford Consensus. The overarching principle of the Hartford Consensus is that no one should die from uncontrolled bleeding. The Hartford Consensus recommends that all citizens learn to stop bleeding. Further information about the Hartford Consensus and bleeding control can be found on the website: Bleedingcontrol.org 2 SAVE A LIFE: What Everyone Should Know to Stop Bleeding After an Injury Authors: Peter T. Pons, MD, FACEP Lenworth Jacobs, MD, MPH, FACS Acknowledgements: The authors acknowledge the contributions of Michael Cohen and James “Brooks” Hart, CMI to the design of this manual. Some images adapted from Adam Wehrle, EMT-P and NAEMT. © 2017 American College of Surgeons CONTENTS SECTION 1 3 ■ Introduction ■ Primary Principles of Trauma Care Response ■ The ABCs of Bleeding SECTION 2 5 ■ Ensure Your Own Safety SECTION 3 6 ■ A – Alert – call 9-1-1 SECTION 4 7 ■ B – Bleeding – find the bleeding injury SECTION 5 9 ■ C – Compress – apply pressure to stop the bleeding by: ■ Covering the wound with a clean cloth and applying pressure by pushing directly on it with both hands, OR ■Using a tourniquet, OR ■ Packing (stuff) the wound with gauze or a clean cloth and then applying pressure with both hands SECTION 6 13 ■ Summary 2 SECTION 1: INTRODUCTION Welcome to the Stop the Bleed: Bleeding Control for the Injured information booklet.
    [Show full text]
  • Using Tourniquets to Stop Bleeding
    JAMA PATIENT PAGE | Trauma Using Tourniquets to Stop Bleeding After the April 15, 2013, Boston Marathon bombings, 27 patients with life-threatening bleeding were saved by placement of tourniquets by people at the scene. What Is a Tourniquet? Applying a tourniquet with a windlass device A tourniquet is a device that is placed around a bleeding arm or leg. Apply direct pressure 1 Place a 2-3” strip of material Tourniquets work by squeezing large blood vessels. The squeezing to the wound for about 2” from the edge helps stop blood loss. at least 15 minutes. of the wound over a long bone between the wound and the heart. Use a tourniquet only How Do I Put a Tourniquet On? when bleeding cannot be stopped and Tourniquets can be made out of any available material. For ex- is life threatening. ample, you can use a bandage, strip of cloth, or even a t-shirt. The material should be at least 2 to 3 inches wide. The material should also overlap itself. Using thin straps or material less than 2 inches wide can rip or cut the skin. Tourniquets often use a windlass device to increase tighten- 2 Insert a stick or other strong, straight ing. Inflated tourniquets (for example, those made from blood pres- item into the knot to act as a windlass. sure cuffs) can work well. But they must be carefully watched for small leaks. The injured blood vessel is not always right below the skin wound. Place the tourniquet between the injured vessel and the heart, about 2 inches from the closest wound edge.
    [Show full text]
  • ER Guide to Bleeding Disorders
    Bleeding disorders ER guide to bleeding disorders 1 Table of contents 4 General Guidelines 4–5 national Hemophilia Foundation guidelines 5–10 Treatment options 10 HemopHilia a Name:__________________________________________________________________________________________________ 10–11 national Hemophilia Foundation guidelines Address:________________________________________________________________________________________________ 12 dosage chart Phone:__________________________________________________________________________________________________ 14–15 Treatment products 16 HemopHilia B In case of emergency, contact: ______________________________________________________________________________ 16 national Hemophilia Foundation guidelines Relation to patient:________________________________________________________________________________________ 17 dosage chart 18 Treatment products 19 HemopHilia a or B with inHiBiTors Diagnosis: Hemophilia A: Mild Moderate Severe 20 national Hemophilia Foundation guidelines Inhibitors Inhibitors Bethesda units (if known) ____________________________________ 21 Treatment products Hemophilia B: Mild Moderate Severe 22–23 Von willeBrand disease Inhibitors Inhibitors Bethesda units (if known) ____________________________________ 23–24 national Hemophilia Foundation guidelines von Willebrand disease: Type 1 Type 2 Type 3 Platelet type 25 Treatment products 27 Bibliography Preferred product:_________________________________________________________________________________________ Dose for life-threatening
    [Show full text]
  • Thrombocytopenia.Pdf
    THROMBOCYTOPENIA DIFFERENTIAL DIAGNOSIS FALSELY LOW PLATELET COUNT In vitro platelet clumping caused by EDTA-dependent agglutinins Giant platelets COMMON CAUSES OF THROMBOCYTOPENIA Pregnancy (gestational thrombocytopenia, preeclampsia) Drug-induced thrombocytopenia (i.e., heparin, quinidine, quinine, and sulfonamides) Viral infections (ie. HIV, rubella, infectious mononucleosis) Hypersplenism due to chronic liver disease Dilutional (massive transfusion) OTHER CAUSES OF THROMBOCYTOPENIA Myelodysplasia Congenital thrombocytopenia Thrombotic thrombocytopenic purpura (TTP) -hemolytic-uremic syndrome (HUS) Chronic disseminated intravascular coagulation (DIC) Autoimmune diseases, such as systemic lupus erythematosus-associated lymphoproliferative disorders (CLL and NHL) Sepsis Idiopathic thrombocytopenic purpura (ITP)* DIFFERENTIAL FOR THROMBOCYTOPENIA BASED ON CLINICAL SETTING CLINICAL SETTING DIFFERENTIAL DIAGNOSES Cardiac surgery Cardiopulmonary bypass, HIT, dilutional thrombocytopenia, PTP Interventional cardiac Abciximab or other IIb/IIIa blockers, HIT procedure Sepsis syndrome DIC, ehrlichiosis, sepsis, hemophagocytosis syndrome, drug-induced, misdiagnosed TTP, mechanical ventilation, pulmonary artery catheters Pulmonary failure DIC, hantavirus pulmonary syndrome, mechanical ventilation, pulmonary artery catheters Mental status TTP, ehrlichiosis changes/seizures Renal failure TTP, Dengue, HIT, DIC, HUS Continuous hemofiltration HIT, consumption by filter and tubing Cardiac failure HIT, drug-induced, pulmonary artery catheter Post-surgery
    [Show full text]
  • Ten Patient Stories Illustrating the Extraordinarily Diverse Clinical Features of Patients with Thrombotic Thrombocytopenic Purpura and Severe ADAMTS13 Deficiency
    Journal of Clinical Apheresis 27:302–311 (2012) Ten Patient Stories Illustrating the Extraordinarily Diverse Clinical Features of Patients With Thrombotic Thrombocytopenic Purpura and Severe ADAMTS13 Deficiency James N. George,* Qiaofang Chen, Cassie C. Deford, and Zayd Al-Nouri Department of Biostatistics and Epidemiology, College of Public Health, Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma Patients with thrombotic thrombocytopenic purpura (TTP) and severe ADAMTS13 deficiency are often consid- ered to have typical clinical features. However, our experience is that there is extraordinary diversity of the pre- senting features and the clinical courses of these patients. This diversity is illustrated by descriptions of 10 patients. The patients illustrate that ADAMTS13 activity may be normal initially but severely deficient in subse- quent episodes. Patients with established diagnoses of systemic infection as the cause of their clinical features may have undetectable ADAMTS13 activity. Patients may have a prolonged prodrome of mild symptoms with only microangiopathic hemolytic anemia and thrombocytopenia or they may have the sudden onset of critical ill- ness with multiple organ involvement. Patients may die rapidly or recover rapidly; they may require minimal treatment or extensive and prolonged treatment. Patients may have acute and severe neurologic abnormalities before microangiopathic hemolytic anemia and thrombocytopenia occur. Patients may have concurrent TTP and systemic lupus erythematosus. Patients may have hereditary ADAMTS13 deficiency as the etiology of their TTP rather than acquired autoimmune ADAMTS13 deficiency. These patients’ stories illustrate the clinical spectrum of TTP with ADAMTS13 deficiency and emphasize the difficulties of clinical diagnosis. J. Clin.
    [Show full text]
  • Modern Management of Traumatic Hemothorax
    rauma & f T T o re l a t a m n r e u n o t J Mahoozi, et al., J Trauma Treat 2016, 5:3 Journal of Trauma & Treatment DOI: 10.4172/2167-1222.1000326 ISSN: 2167-1222 Review Article Open Access Modern Management of Traumatic Hemothorax Hamid Reza Mahoozi, Jan Volmerig and Erich Hecker* Thoraxzentrum Ruhrgebiet, Department of Thoracic Surgery, Evangelisches Krankenhaus, Herne, Germany *Corresponding author: Erich Hecker, Thoraxzentrum Ruhrgebiet, Department of Thoracic Surgery, Evangelisches Krankenhaus, Herne, Germany, Tel: 0232349892212; Fax: 0232349892229; E-mail: [email protected] Rec date: Jun 28, 2016; Acc date: Aug 17, 2016; Pub date: Aug 19, 2016 Copyright: © 2016 Mahoozi HR. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Hemothorax is defined as a bleeding into pleural cavity. Hemothorax is a frequent manifestation of blunt chest trauma. Some authors suggested a hematocrit value more than 50% for differentiation of a hemothorax from a sanguineous pleural effusion. Hemothorax is also often associated with penetrating chest injury or chest wall blunt chest wall trauma with skeletal injury. Much less common, it may be related to pleural diseases, induced iatrogenic or develop spontaneously. In the vast majority of blunt and penetrating trauma cases, hemothoraces can be managed by relatively simple means in the course of care. Keywords: Traumatic hemothorax; Internal chest wall; Cardiac Hemodynamic response injury; Clinical manifestation; Blunt chest-wall injuries; Blunt As above mentioned the hemodynamic response is a multifactorial intrathoracic injuries; Penetrating thoracic trauma response and depends on severity of hemothorax according to its classification.
    [Show full text]
  • Immune Thrombocytopenic Purpura (ITP) — Adult Conditions for Which Ivig Has an Established Therapeutic Role
    Immune thrombocytopenic purpura (ITP) — adult Conditions for which IVIg has an established therapeutic role. Specific Conditions Newly Diagnosed Immune thrombocytopenic purpura (ITP) Persistent Immune thrombocytopenic purpura (ITP) Chronic Immune thrombocytopenic purpura (ITP) Evans syndrome ‐ with significant Immune thrombocytopenic purpura (ITP) ‐ adult Indication for IVIg Use Newly diagnosed ITP — initial Ig therapy ITP in pregnancy — initial Ig therapy ITP with life‐threatening haemorrhage or the potential for life‐threatening haemorrhage Newly diagnosed or persistent ITP — subsequent therapy (diagnosis <12 months) Refractory persistent or chronic ITP — splenectomy failed or contraindicated and second‐line agent unsuccessful Subsequent or ongoing treatment for ITP responders during pregnancy and the postpartum period ITP and inadequate platelet count for planned surgery HIV‐associated ITP Level of Evidence Evidence of probable benefit – more research needed (Category 2a) Description and Diagnostic Immune thrombocytopenic purpura (ITP) is a reduction in platelet count Criteria (thrombocytopenia) resulting from shortened platelet survival due to anti‐platelet antibodies, reduced platelet production due to immune induced reduced megakaryopoeisis and/or immune mediated direct platelet lysis. When counts are very low (less than 30x109/L), bleeding into the skin (purpura) and mucous membranes can occur. Bone marrow platelet production (megakaryopoiesis) is morphologically normal. In some cases, there is additional impairment of platelet function related to antibody binding to glycoproteins on the platelet surface. It is a common finding in patients with human immunodeficiency virus (HIV) disease, and while it may be found at any stage of the infection, its prevalence increases as HIV disease advances. Around 80 percent of adults with ITP have the chronic form of disease.
    [Show full text]
  • Treatment of Hemothorax in the Era Of€The€Minimaly Invasive Surgery
    Mil. Med. Sci. Lett. (Voj. Zdrav. Listy) 2019, 88(4), 180-187 ISSN 0372-7025 (Print) ISSN 2571-113X (Online) DOI: 10.31482/mmsl.2019.011 Since 1925 REVIEW ARTICLE TREATMENT OF HEMOTHORAX IN THE ERA OF THE MINIMALY INVASIVE SURGERY Radek Pohnán 1,2 , Šárka Blažková 2, Vladislav Hytych 2, Petr Svoboda 1, Michal Makeľ 1, Iva Holmquist 3,4 and Miroslav Ryska 1 1 Department of Surgery, Central Military Hospital – Faculty Military Hospital, 2nd Faculty of Medicine, Charles University, Prague, Czech Republic 2 Department of Thoracic Surgery, Thomayer's Hospital, 140 59 Prague, Czech Republic 3 Emory University Hospital Midtown, Maternity Center, Atlanta, Georgia, USA 4 Department of Epidemiology, Faculty of Health Sciences, University of Defence, Hradec Kralove, Czech Republic Received 19th July 2018. Accepted 13th May 2019. On-line 6th December 2019. Summary Hemothorax is a frequent clinical situation often associated with chest injury or with iatrogenic lesions. Spontaneous hemothorax is uncommon and among its cause may include coagulation disorders, pleural, pulmonary or vascular pathology. Diagnostics is based on radiography or ultrasound and thoracentesis which may be also therapeutic solution. The majority of hemothoraxes can be managed non-operatively but hemodynamic instability, the volume of evacuated blood and persisting blood loss or persisting hemothorax require surgery. A surgical approach may vary from open thoracotomy to rapidly developing minimally invasive methods - video-assisted thoracoscopic surgery (VATS) and videothoracoscopy (VTS). Key words: hemothorax; surgery; VATS; thoracotomy Introduction Hemothorax is a pathological collection of the blood within the pleural cavity. Hemothorax most frequently origin in a thoracic injury but the exact incidence is not known.
    [Show full text]
  • Gastrointestinal Bleeding Gary A
    Article gastroenterology Gastrointestinal Bleeding Gary A. Neidich, MD* Educational Gaps Sarah R. Cole, MD* 1. Pediatricians should be familiar with diseases that may present with gastrointestinal bleeding in patients at varying ages. Author Disclosure 2. Pediatricians should be aware of newer technologies for the identification and therapy Drs Neidich and Cole of gastrointestinal bleeding sources. have disclosed no 3. Pediatricians should be familiar with polyps that have and do not have an increased financial relationships risk of malignant transformation. relevant to this article. 4. Pediatricians should be familiar with medications used in the treatment of children This commentary does with gastrointestinal bleeding. not contain a discussion of an Objectives After completing this article, readers should be able to: unapproved/ investigative use of 1. Formulate a diagnostic and management plan for children with gastrointestinal a commercial product/ bleeding. device. 2. Describe newer techniques and their limitations for the identification of bleeding, including small intestinal capsule endoscopy and small intestinal enteroscopy. 3. Differentiate common and less common causes of gastrointestinal bleeding in children of varying ages. 4. Identify types of polyps that may present in childhood and which of these have malignant potential. Introduction An 11-year-old boy is seen in the emergency department after fainting at home. He has a 2-day history of headache and dizziness. Epigastric pain has been present during the past 2 days. His pulse is 150 beats per minute, and his blood pressure is 90/50 mm Hg. An in- travenous bolus of normal saline is administered; his hemoglobin level is 8.1 g/dl (81 g/L).
    [Show full text]
  • Spontaneous Hemopericardium Leading to Cardiac Tamponade in a Patient with Essential Thrombocythemia
    SAGE-Hindawi Access to Research Cardiology Research and Practice Volume 2011, Article ID 247814, 3 pages doi:10.4061/2011/247814 Case Report Spontaneous Hemopericardium Leading to Cardiac Tamponade in a Patient with Essential Thrombocythemia Anand Deshmukh,1, 2 Shanmuga P. Subbiah,3 Sakshi Malhotra,4 Pooja Deshmukh,4 Suman Pasupuleti,1 and Syed Mohiuddin1, 4 1 Department of Cardiovascular Medicine, Creighton University Medical Center, Omaha, NE 68131, USA 2 Creighton Cardiac Center, 3006 Webster Street, Omaha, NE 68131, USA 3 Department of Hematology and Oncology, Creighton University Medical Center, Omaha, NE 68131, USA 4 Department of Internal Medicine, Creighton University Medical Center, Omaha, NE 68131, USA Correspondence should be addressed to Anand Deshmukh, [email protected] Received 30 October 2010; Accepted 29 December 2010 Academic Editor: Syed Wamique Yusuf Copyright © 2011 Anand Deshmukh et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Acute cardiac tamponade requires urgent diagnosis and treatment. Spontaneous hemopericardium leading to cardiac tamponade as an initial manifestation of essential thrombocythemia (ET) has never been reported in the literature. We report a case of a 72-year-old Caucasian female who presented with spontaneous hemopericardium and tamponade requiring emergent pericardiocentesis. The patient was subsequently diagnosed to have ET. ET is characterized by elevated platelet counts that can lead to thrombosis but paradoxically it can also lead to a bleeding diathesis. Physicians should be aware of this complication so that timely life-saving measures can be taken if this complication arises.
    [Show full text]
  • The Voice of the Patient: Hemophilia A, Hemophilia B, Von Willebrand Disease and Other Heritable Bleeding Disorders
    The Voice of the Patient A series of reports from the U.S. Food and Drug Administration’s (FDA’s) Patient-Focused Drug Development Initiative Hemophilia A, Hemophilia B, von Willebrand Disease and Other Heritable Bleeding Disorders Public Meeting: September 22, 2014 Report Date: May 2016 Center for Biologics Evaluation and Research (CBER) U.S. Food and Drug Administration (FDA) 1 Table of Contents Introduction ..............................................................................................................3 Overview of bleeding disorders ................................................................................................ 3 Meeting overview ..................................................................................................................... 4 Report overview and key themes .............................................................................................. 5 Topic 1: Disease Symptoms and Daily Impacts That Matter Most to Patients 6 Perspectives on symptoms ........................................................................................................ 7 Perspectives on the overall impact of bleeding disorders on daily life ..................................... 9 Topic 2: Patient Perspectives on Current Approaches to Treatments ............11 Perspectives on current treatment for conditions or symptoms .............................................. 12 Perspectives on an ideal treatment .......................................................................................... 16
    [Show full text]