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https://www.readbyqxmd.com/read/28533057/late-effects-screening-guidelines-after-hematopoietic-cell-transplantation-hct-for-inherited-bone-marrow-failure-syndromes-ibmfs-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international-conference-on-late-effects-after
#1
REVIEW
Andrew C Dietz, Sharon A Savage, Adrianna Vlachos, Parinda A Mehta, Dorine Bresters, Jakub Tolar, Carmem Bonfim, Jean Hugues Dalle, Josu de la Fuente, Roderick Skinner, Farid Boulad, Christine N Duncan, K Scott Baker, Michael A Pulsipher, Jeffrey M Lipton, John E Wagner, Blanche P Alter
Patients with inherited bone marrow failure syndromes (IBMFS) such as Fanconi anemia (FA), dyskeratosis congenita (DC), and Diamond Blackfan anemia (DBA) can have hematologic manifestations cured through hematopoietic cell transplantation (HCT). Subsequent late effects seen in these patients arise from a combination of the underlying disease, the pre-HCT therapy, and the HCT process. During the international consensus conference sponsored by the Pediatric Blood and Marrow Transplant Consortium entitled "Late Effects Screening and Recommendations Following Allogeneic Hematopoietic Cell Transplant for Immune Deficiency and Nonmalignant Hematologic Disease" held in Minneapolis, Minnesota in May of 2016, a half-day session was focused specifically on the unmet needs for these patients with IBMFS...
May 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28530458/-early-symptoms-of-childhood-malignant-diseases
#2
Zsuzsanna Erzsébet Papp, Izabella Kelemen, Adrienne Horváth
INTRODUCTION: Childhood malignant diseases are rare in pediatric pathology. Early symptoms are not specific, fatigue, pallor, compression signs and bone marrow failure are often mentioned. AIM: To summarize the most frequent early symptoms of childhood malignancies in order to help the physicians in the early recognition. METHOD: In our retrospective study, we processed a period of 5 years between 2012-2016, with an accent on the onset manifestations of malignancies...
May 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28527586/gastrointestinal-bowel-obstruction-in-acute-ischemic-stroke-incidence-risk-factors-and-outcomes-in-a-u-s-nationwide-analysis-of-3-998-667-hospitalizations
#3
Kavelin Rumalla, Ashwath S Kumar, Manoj K Mittal
OBJECTIVE: The prognosis from acute ischemic stroke (AIS) is worsened by poststroke medical complications. The incidence, risk factors, and outcomes of gastrointestinal bowel obstruction (GIBO) in AIS are not known. METHODS: We queried the Nationwide Inpatient Sample (2002-2011) to identify all patients with a primary diagnosis of AIS and subsets with and without a secondary diagnosis of GIBO without hernia. Multivariable analysis was utilized to identify risk factors for GIBO in AIS patients and the association between GIBO, in-hospital complications, and outcomes...
May 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28523896/hemophagocytic-lymphohistiocytosis-in-a-newborn-presenting-as-blueberry-muffin-baby
#4
Krista N Larson, Sergio R Gaitan, Benjamin J Stahr, Dean S Morrell
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by uncontrolled activation and proliferation of proinflammatory cytokines. Initial presentation commonly includes fever, hepatosplenomegaly, and pancytopenia; 6 to 65% of cases also have a concurrent cutaneous eruption. We present the case of a 6-day-old premature infant boy with congenital severe thrombocytopenia, anemia, and hepatosplenomegaly who presented with several cutaneous violaceous papules and nodules and was found to have HLH...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28523047/alpha-thalassemia-deletions-found-in-suspected-cases-of-beta-thalassemia-major-in-pakistani-population
#5
Saba Shahid, Muhammad Nadeem, Danish Zahid, Jawad Hassan, Saqib Ansari, Tahir Shamsi
BACKGROUND & OBJECTIVE: Alpha (α) thalassemia is a hereditary disorder and is caused by deletions or mutations in globin genes. It is present in two clinically significant forms: hemoglobin Bart hydrops fetalis (Hb Bart) syndrome and hemoglobin H (HbH) disease. It is highly prevalent in South-East Asia or Mediterranean countries. The most common deletion reported in alpha thalassemia in Pakistani population was -α(3.7) with a frequency of 8.3%, and the rare forms were -α(4.2) (0.2%) and ααα(anti3...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28516093/iatrogenic-supravalvular-aortic-stenosis
#6
Paolo Bosco, Antonella Ferrara, Samer A M Nashef
We describe a case of hemolytic anemia and proximal anastomotic site stenosis following emergency repair of a Type A aortic dissection. This rare complication led to a reoperation to correct the iatrogenic aortic stenosis and cure the consequent hemolysis. A "sandwich" technique (with two Teflon strips on the outside and inside of the aortic wall) was used in the initial repair to reinforce the suture line and prevent bleeding from the aortic anastomoses. At the time of reoperation, the inner Teflon strip at the proximal aortic anastomosis was found to have inverted into the aortic lumen, as suggested by the preoperative magnetic resonance imaging...
October 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/28512588/severe-aplastic-anemia-following-parvovirus-b19-associated-acute-hepatitis
#7
Masanori Furukawa, Kosuke Kaji, Hiroyuki Masuda, Kuniaki Ozaki, Shohei Asada, Aritoshi Koizumi, Takuya Kubo, Norihisa Nishimura, Yasuhiko Sawada, Kosuke Takeda, Tsuyoshi Mashitani, Masayuki Kubo, Itsuto Amano, Tomoyuki Ootani, Chiho Ohbayashi, Koji Murata, Tatsuichi Ann, Akira Mitoro, Hitoshi Yoshiji
Human parvovirus (HPV) B19 is linked to a variety of clinical manifestations, such as erythema infectiosum, nonimmune hydrops fetalis, and transient aplastic anemia. Although a few cases have shown HPVB19 infection as a possible causative agent for hepatitis-associated aplastic anemia (HAAA) in immunocompetent patients, most reported cases of HAAA following transient hepatitis did not have delayed remission. Here we report a rare case of severe aplastic anemia following acute hepatitis with prolonged jaundice due to HPVB19 infection in a previously healthy young male...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28512422/acute-onset-ocular-hypotony-after-coronary-artery-bypass-surgery
#8
Sarah Farukhi Ahmed, Audrey Xi Tai, Mason Schmutz, John Combs, Sameh Mosaed
IMPORTANCE: The purpose of this case report is to evaluate risk factors associated with post-coronary artery bypass graft (CABG) ocular hypotony compared to post-CABG ischemic optic neuropathy. OBSERVATIONS: The patient described here is a single case at the University of California, Irvine Medical Center, from July 2016. This case demonstrates the rare incidence of acute post-CABG ocular hypotony and vision loss in a patient with prior history of optic atrophy...
January 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/28512409/immunoglobulin-d-multiple-myeloma-presenting-as-spontaneous-fracture
#9
Samer Al Hadidi, Khalil Katato, Ghassan Bachuwa
Immunoglobulin D multiple myeloma is a rare type of multiple myeloma that usually presents as bone pain, fatigue, or weight loss. We report a case of immunoglobulin D multiple myeloma in a 53-year-old Caucasian male patient with previous medical history of anaplastic oligodendroglioma status post-surgical resection who was evaluated for back pain while mowing the lawn. His physical examination showed tenderness over the lower thoracic vertebrae with no sensory or motor impairment. Initial lab investigations showed normocytic anemia and hypercalcemia with low parathyroid hormone...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28508982/renal-hemorrhage-caused-by-acquired-inhibitors-to-coagulation-factors-viii-and-v-in-a-hemodialysis-patient
#10
Naoya Niwa, Tadashi Yoshida, Ryuichi Mizuno, Mototsugu Oya, Matsuhiko Hayashi
Acquired coagulation factor deficiency is a rare bleeding disorder caused by the inhibitors to coagulation factors. We report a case of an elderly hemodialysis patient who presented with the intermittent hematuria and anemia, associated with the prolonged activated partial thromboplastin time and prothrombin time. Laboratory examination revealed undetectable factor VIII activity, decreased factor V activity, and the presence of inhibitors to these coagulation factors. The patient was diagnosed to have inhibitors to coagulation factors VIII and V simultaneously...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#11
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508588/at-the-cross-section-of-thrombotic-microangiopathy-and-atypical-hemolytic-uremic-syndrome-a-narrative-review-of-differential-diagnostics-and-a-problematization-of-nomenclature
#12
REVIEW
Alexander Åkesson, Eva Zetterberg, Jenny Klintman
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigger (e.g. infection) is needed for the excessive response to develop...
May 16, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28507571/transcatheter-paravalvular-leak-closure-and-hemolysis-a-prospective-registry
#13
Grzegorz Smolka, Piotr Pysz, Andrzej Ochała, Michał Kozłowski, Wojciech Zasada, Zofia Parma, Michał Tendera, Wojciech Wojakowski
INTRODUCTION: Paravalvular leak (PVL) related to a surgical prosthetic valve may be associated with clinically significant hemolysis. The influence of transcatheter PVL closure (TPVLC) on hemolysis remains uncertain. MATERIAL AND METHODS: The prospective registry included patients undergoing TPVLC due to PVL-related heart failure and/or hemolysis. Procedural data, laboratory markers of hemolysis and heart failure status were recorded at baseline, discharge and at 1- and 6-month follow-up...
April 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28502540/trends-and-outcomes-in-endovascular-and-open-surgical-treatment-of-visceral-aneurysms
#14
Jason A Chin, Adele Heib, Cassius I Ochoa Chaar, Jonathan A Cardella, Kristine C Orion, Timur P Sarac
OBJECTIVE: Visceral artery aneurysms (VAAs) are rare but often repaired because of dire consequences of rupture. This is a population-based evaluation of chronologic trends in management, risk factors, and outcomes of endovascular and open therapy. METHODS: The 2003 to 2013 Agency for Healthcare Research and Quality (AHRQ) National Inpatient Sample (NIS) database was reviewed. Cases with primary diagnosis of VAA and undergoing endovascular or open repair were identified...
May 11, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#15
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28500741/mouth-examination-performance-by-children-s-parents-and-by-adolescents-in-fanconi-anemia
#16
Allana Pivovar, Camila Pinheiro Furquim, Carmem Bonfim, Cassius Carvalho Torres-Pereira
BACKGROUND: Fanconi anemia (FA) is a rare genetic syndrome characterized by increased risk of developing malignant neoplasms, particularly oral squamous cell carcinoma. This study aims to ascertain the extent to which adolescents and guardians/parents of children with FA are aware of their oral cancer risks and assess their ability to perform mouth examination (ME). PROCEDURE: A cross-sectional study was conducted among patients with FA (between 6 and 16 years) and their parents...
May 13, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28495927/an-infant-with-pearson-syndrome-a-rare-cause-of-congenital-sideroblastic-anemia-and-bone-marrow-failure
#17
Corey P Falcon, Thomas H Howard
No abstract text is available yet for this article.
May 11, 2017: Blood
https://www.readbyqxmd.com/read/28493097/gastric-adenosquamous-carcinoma-producing-granulocyte-colony-stimulating-factor-a-case-of-a-rare-malignancy
#18
Kazuki Moro, Masayuki Nagahashi, Tetsuya Naito, Yu Nagai, Tomohiro Katada, Masahiro Minagawa, Jun Hasegawa, Tatsuo Tani, Naohiro Shimakage, Hiroyuki Usuda, Emmanuel Gabriel, Tsutomu Kawaguchi, Kazuaki Takabe, Toshifumi Wakai
BACKGROUND: A gastric adenosquamous carcinoma (ASC) that produces granulocyte-colony stimulating factor (G-CSF) is an uncommon malignancy with a poor prognosis. Due to the rarity of this lesion, a standard treatment for the disease has not been established. CASE PRESENTATION: We describe a case of a 66-year-old male with a G-CSF-producing gastric ASC who presented with severe anemia and leukocytosis. A radical resection was performed, followed by a course of adjuvant chemotherapy...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28492007/colchicine-may-assist-in-reducing-granulation-tissue-in-junctional-epidermolysis-bullosa
#19
Minhee Kim, Swaranjali Jain, Adam G Harris, Dedee F Murrell
Epidermolysis bullosa (EB) is a rare, inherited blistering genodermatosis. Patients with junctional EB (JEB) due to LAMB3 mutations have widespread blisters and erosions of skin, mucosae, and nails, creating significant physical, emotional, and psychosocial burdens. Here we report the use of colchicine for ameliorating hypergranulating wounds in a 41-year-old female with JEB generalized intermediate. Her skin wounds and granulation tissue gradually exacerbated under silicone dressings such that she became profoundly anemic...
June 2016: International Journal of Women's Dermatology
https://www.readbyqxmd.com/read/28488841/catastrophic-antiphospholipid-syndrome-an-update
#20
Gerard Espinosa, Ignasi Rodriguez-Pinto, Ricard Cervera
Catastrophic antiphospholipid syndrome (CAPS) is a rare variant that accounts for 1% of patients with APS. Despite its low frequency, the mortality-related is very high ranging from 50% of patients in the first series to 37% in the most recent data. The current knowledge of this potential devastating entity comes from the International Registry of patients with CAPS, named CAPS Registry. Small vessel thrombosis, laboratory features of microangiopathic haemolytic anemia, and development of multisystem involvement in a very short period of time are the main characteristics of this syndrome...
May 8, 2017: Panminerva Medica
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