keyword
https://read.qxmd.com/read/38432967/nivolumab-induced-thrombotic-thrombocytopenic-purpura-in-patients-with-gastric-tube-cancer
#21
JOURNAL ARTICLE
Yuya Yoshida, Sakura Toriyabe, Hiroo Imai, Keiju Sasaki, Yuki Kasahara, Kota Ouchi, Ken Saijo, Koichi Onodera, Chikashi Ishioka
Recently, immune checkpoint inhibitors (ICIs) have been used to treat several cancer types. ICIs have been reported to cause a wide variety of immune-related adverse events, including endocrine, neurologic, gastrointestinal, and cutaneous disorders. Thrombotic thrombocytopenic purpura (TTP) is an autoimmune hematologic disorder characterized by the presence of autoantibodies against a disintegrin and metalloprotease with thrombospondin-1, member 13. Several previous cases of TTP were thought to have been caused by ICI treatment...
March 4, 2024: Internal Medicine
https://read.qxmd.com/read/38432067/autoimmune-cytopenia-and-kabuki-syndrome-in-paediatrics-insights-in-11-patients
#22
JOURNAL ARTICLE
Chloé Bianchi, Henri Margot, Helder Fernandes, Marlène Pasquet, Laurence Priqueler, Frédérique Roy-Peaud, Frédéric Bauduer, Sophie Bayart, Nathalie Garnier, Olivier Fain, Julien Van Gils, Sandrine Baron Joly, Fanny Rialland, Catherine Paillard, Marianna Deparis, Anne Lambilliotte, Thierry Leblanc, Mony Fahd, Guy Leverger, Sébastien Héritier, David Geneviève, Frédéric Rieux-Laucat, Capucine Picard, Caroline Neyraud, Nathalie Aladjidi
Kabuki syndrome (KS) is now listed in the Human Inborn Errors of Immunity (IEI) Classification. It is a rare disease caused by KMT2D and KDM6A variants, dominated by intellectual disability and characteristic facial features. Recurrently, pathogenic variants are identified in those genes in patients examined for autoimmune cytopenia (AIC), but interpretation remains challenging. This study aims to describe the genetic diagnosis and the clinical management of patients with paediatric-onset AIC and KS. Among 11 patients with AIC and KS, all had chronic immune thrombocytopenic purpura, and seven had Evans syndrome...
March 3, 2024: British Journal of Haematology
https://read.qxmd.com/read/38426374/treatment-of-erythematous-acne-scars-using-595-nm-pulsed-dye-laser-combined-with-1565-nm-resurfx-nonablative-fractional-laser
#23
JOURNAL ARTICLE
Chenxi Zhou, Meijuan Yao, Weiliang Chen, Liyuan Zhang, Lidan Zhang, Jiaojiao Chen, Kai Li, Xiailei Qin
BACKGROUND: Acne vulgaris is a common inflammatory disease associated with various sequelae after skin lesion remission. Acne erythema has been considered simple erythema or a vascular lesion; however, because the understanding of this disease has improved, acne erythema is currently considered an early scar with erythematous components. AIMS: This study evaluated the efficacy of using both a 595-nm pulsed dye laser (PDL) and 1565-nm nonablative fractional laser (NAFL) for the treatment of erythematous scars caused by acne...
March 1, 2024: Journal of Cosmetic Dermatology
https://read.qxmd.com/read/38407854/how-complicated-can-be-a-case-with-immune-thrombocytopenic-purpura-postsplenectomy-arterial-thrombosis
#24
JOURNAL ARTICLE
Ahmet Deniz Kaya, Oguzhan Tekin, Yasin Colak, Istemi Serin
Arterial thrombus associated with the surgery can be seen in postsplenectomy cases, but there is no clear data in patients diagnosed with immune thrombocytopenic purpura (ITP). A 52-year-old female patient was admitted to the emergency department due to ecchymotic skin changes. Her initial platelet count was 6000/mm3; after two courses of high dose-dexamethasone, intravenous immunoglobulin and rituximab, splenectomy was planned for the patient whose platelet count was again <40 000/mm3. She presented to the emergency department with complaints of pain and pallor in the right arm in the second week of follow-up...
February 22, 2024: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/38406548/does-caplacizumab-for-the-management-of-thrombotic-thrombocytopenic-purpura-increase-the-risk-of-relapse-exacerbation-and-bleeding-an-updated-systematic-review-and-meta-analysis-based-on-revised-criteria-by-the-international-working-group-for-thrombotic-thrombocytopenic
#25
REVIEW
Niraj Neupane, Sangharsha Thapa, Amir Mahmoud, Abhinav Bhattarai, Anil Kc, Shreeja Shikhrakar, Sayuri Gurusinghe, Peter Kuiodes
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening condition marked by abnormal blood clotting and organ damage. Caplacizumab is a potential treatment for the TTP management. This systematic review and meta-analysis aimed to assess Caplacizumab's effectiveness and safety in the TTP management. A comprehensive database search identified nine studies, including randomized controlled trials and observational studies. Primary outcomes included TTP exacerbation, relapse, and major bleeding...
February 2024: EJHaem
https://read.qxmd.com/read/38406537/autoimmune-thrombotic-thrombocytopenic-purpura-associated-with-disseminated-sarcoidosis-a-case-report
#26
Maxime Billotte, Peter Eszto, Benoît Thomas, Pierre Groizard, Pierre Perez, Julien Campagne
Sarcoidosis is an inflammatory disease known to be associated with multiple autoimmune disorders. There is a restricted number of descriptions of the association between sarcoidosis and autoimmune thrombotic thrombocytopenic purpura (aTTP). We present the case of a 63-year-old woman admitted to the hospital to investigate a possible sarcoidosis who had hemolytic anemia and thrombocytopenia, with low ADAMTS13 activity and anti-ADAMTS13 antibodies, leading to a diagnosis of aTTP. Sarcoidosis was later confirmed and the two conditions evolved separately after 6 months, questioning the link between them...
February 2024: EJHaem
https://read.qxmd.com/read/38405595/case-report-in-situ-pulmonary-artery-thrombosis-in-a-12-year-old-girl-classified-as-systemic-lupus-erythematosus
#27
Yong Feng, Ning Chen, Bing Dai, Yunxiao Shang
In situ pulmonary artery thrombosis (ISPAT) is a relatively rare but potentially life-threatening complication of systemic lupus erythematosus (SLE) in children. We report the case of a 12-year-old girl who presented with fever, chest pain, and dyspnea. Immune thrombocytopenia was identified due to purpura and menorrhagia 3 months before presentation with a lowest platelet count of 12 × 109 /L. The sudden onset of fever, chest pain, and dyspnea were misdiagnosed as hyperinflammatory responses caused by pneumonia; these symptoms ameliorated with glucocorticoid and antibiotic treatment...
2024: Frontiers in Pediatrics
https://read.qxmd.com/read/38383372/radiation-induced-cutaneous-vasculopathy-of-the-breast-a-rare-case-report
#28
JOURNAL ARTICLE
Hilde Van Parijs, Yves Sinove, Marilyn Carprieaux, Mark De Ridder
BACKGROUND: Radiation therapy is often indicated as part of the treatment for breast cancer and is therefore used frequently worldwide. Vasculopathy is a general term used to describe any condition that affects blood vessels. We present a case report of a patient who presented with vasculopathy as a rare late side effect of radiation therapy to the breast. CASE PRESENTATION: This 66-year-old woman was initially treated with breast-conserving surgery for early-stage receptor-positive left breast carcinoma...
February 21, 2024: World Journal of Surgical Oncology
https://read.qxmd.com/read/38379266/occlusive-cutaneous-vasculopathies-as-cause-of-chronic-ulcers
#29
JOURNAL ARTICLE
Moritz Ronicke, Carola Berking, Cornelia Erfurt-Berge
The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer...
February 20, 2024: Journal der Deutschen Dermatologischen Gesellschaft: JDDG
https://read.qxmd.com/read/38373491/-indirects-causes-of-maternal-deaths-except-stroke-cardiovascular-diseases-and-infections-in-france-2016-2018
#30
JOURNAL ARTICLE
Véronique Le Guern, Mathias Rossignol, Jacques Lepercq
Maternal deaths from indirect obstetric causes result from a pre-existing condition or a condition that occurred during pregnancy without obstetric causes but was aggravated by the physiological effects of pregnancy. Twenty-nine deaths with an indirect cause related to a pre-existing condition, excluding circulatory diseases or infections, were analysed by the expert committee. Pre-pregnancy pathology was documented in 16 women (epilepsy, n=7; amyloid angiopathy, n=1; Dandy Walker syndrome, n=1; autoimmune diseases, n=3; diffuse infiltrative pneumonitis, n=1; thrombotic thrombocytopenic purpura, n=1; ovarian cancer in fragile X, n=1; major sickle cell disease, n=1)...
February 17, 2024: Gynecologie, Obstetrique, Fertilite & Senologie
https://read.qxmd.com/read/38360215/mechanism-underlying-severe-deficiency-of-plasma-adamts-13-activity-in-immune-thrombotic-thrombocytopenic-purpura
#31
JOURNAL ARTICLE
X Long Zheng
BACKGROUND: Immune-mediated thrombotic thrombocytopenic purpura is caused by autoantibodies against ADAMTS-13, a plasma enzyme that cleaves von Willebrand factor. However, the mechanism resulting in severe deficiency of plasma ADAMTS-13 activity remains controversial. OBJECTIVES: To determine the mechanism of autoantibody-mediated severe deficiency of plasma ADAMTS13 activity in immune-mediated thrombotic thrombocytopenic purpura. METHODS: Fluorescence resonance energy transfer-VWF73 was used to determine plasma ADAMTS-13 activity...
February 14, 2024: Journal of Thrombosis and Haemostasis: JTH
https://read.qxmd.com/read/38358894/bleeding-disorders-in-saudi-arabia-causes-and-prevalence-a-review
#32
JOURNAL ARTICLE
Tareg M Belali
As bleeding disorders are a worldwide health concern, Saudi Arabia is experiencing a notable prevalence of such disorders. Studying the frequency and cause of hemostatic disorders is the key to successful clinical interventions and instigating effective public policies that limit the spread of such disorders. The current review aims to highlight the major findings of the body of literature that has investigated the causes, prevalence, and major challenges associated with bleeding disorders in the country. The current review summarizes the major findings of different studies that have been conducted in Saudi Arabia regarding different bleeding disorders...
February 15, 2024: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/38317027/susceptibility-to-mycobacterial-infection-in-vexas-syndrome
#33
JOURNAL ARTICLE
Stanislas Riescher, Raphael Lecomte, Gwenvael Danic, Julie Graveleau, Yannick Le Bris, Muriel Hello, Aurélie Guillouzouic, Vianney Guardiolle, Alice Garnier, Olivier Grossi, Benjamin Gaborit, Antoine Néel
OBJECTIVES: VEXAS is a recently described acquired auto-inflammatory and hematologic syndrome caused by somatic mutations in UBA1. To date, VEXAS is not a recognized cause of acquired immunodeficiency. PATIENTS AND METHODS: Two of our 10 VEXAS patients developed a disseminated Mycobacterium avium infection. To shed light on this observation, we retrospectively studied all patients with disseminated non-tuberculous mycobacterial infections (NTMi) seen at our institution over 13 years...
February 5, 2024: Rheumatology
https://read.qxmd.com/read/38298753/stasis-dermatitis-pathophysiology-current-treatment-paradigms-and-the-use-of-the-flavonoid-diosmin
#34
REVIEW
Nardin Awad, John D Hetzel, Vishnu Bhupalam, Mark S Nestor
OBJECTIVE: We sought to examine the role of flavonoids, particularly diosmin, as a therapeutic agent for stasis dermatitis (SD) through discussion of pathophysiology, current treatment paradigms, potential mechanisms of action, and a systematic review of evidence on clinical efficacy. METHODS: In addition to articles on pathophysiology and standard treatment, a search of PubMed was conducted using the following query: ("Diosmin" OR "MPFF" OR "Micronized Purified Flavonoid Fraction" OR "Flavonoid") AND ("Stasis Dermatitis" OR "Venous Ulcer" OR "Lipodermatosclerosis")...
January 2024: Journal of Clinical and Aesthetic Dermatology
https://read.qxmd.com/read/38287922/chronic-isolated-fallopian-tube-torsion-in-a-sexually-inactive-adolescent-female-diagnosed-peroperatively
#35
Yuhya Hirahara, Koichi Nagai, Kazunori Mukaida
INTRODUCTION: Isolated fallopian tube torsion (IFTT) has rarely been confirmed in sexually inactive adolescents, and preoperative diagnosis of IFTT is difficult because of the absence of specific symptoms. Therefore, pediatric patients with IFTT tend to be misdiagnosed before the surgery. CASE: A 15-year-old female patient with no history of abdominal surgery or sexual intercourse presented with acute left lower abdominal pain and purpura. MRI revealed hydrosalpinx in the left adnexal region...
2024: Case Reports in Surgery
https://read.qxmd.com/read/38282948/refractory-acquired-amegakaryocytic-thrombocytopenia-with-rapid-progression-to-aplastic-anaemia-in-sle
#36
Bana Hadid, Arif Kodza, Sumatha Channapatna Suresh, Aleksander Feoktistov
Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare cause of thrombocytopenia seen in systemic lupus erythematosus (SLE) that is frequently misdiagnosed as immune thrombocytopenic purpura (ITP). Often patients do not respond to standard ITP treatment. Prompt bone marrow biopsy and further workup should ensue as it is a diagnosis of exclusion. While no standard guidelines exist, the mainstay of treatment is immunosuppressive therapy. Some cases are refractory and should have a follow-up biopsy, typically showing worsening disease...
December 2023: Mediterranean journal of rheumatology
https://read.qxmd.com/read/38281080/a-review-of-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura-past-present-and-future
#37
REVIEW
Dinah V Parums
The clinical association of purpura, arthralgia, and arthritis was first described in 1837 in a publication by Johann Lukas Schönlein, a German physician. In 1874, Eduard Henoch, a student of Schönlein, reported cases of children with purpura, abdominal pain, bloody diarrhea, and joint pain. IgA vasculitis, or Henoch-Schönlein purpura, is a systemic hypersensitivity vasculitis caused by the deposition of immune complexes in small blood vessels, including the renal glomeruli and mesangium. In the skin, the presentation is with non-thrombocytopenic purpura or urticaria...
January 28, 2024: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://read.qxmd.com/read/38236411/-immunoglobulin%C3%A2-a-vasculitis
#38
REVIEW
Sabine Adler
The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulin A (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children...
February 2024: Inn Med (Heidelb)
https://read.qxmd.com/read/38200557/progress-in-the-clinical-effects-and-adverse-reactions-of-ticagrelor
#39
REVIEW
Peng Wei, Xiaoqing Wang, Qiang Fu, Bangming Cao
BACKGROUND: Ticagrelor is a novel receptor antagonist that selectively binds to the P2Y12 receptor, thereby inhibiting adenosine diphosphate (ADP)-mediated platelet aggregation. Compared to clopidogrel, ticagrelor has the advantages of a fast onset, potent effects, and a reversible platelet inhibition function, which make this drug clinically suitable for treating acute coronary syndrome (ACS), especially acute ST-segment elevation myocardial infarction (STEMI). OBJECTIVE: This review was performed to determine the basic characteristics, clinical effects, and adverse reactions of ticagrelor...
January 10, 2024: Thrombosis Journal
https://read.qxmd.com/read/38183639/severe-cutaneous-adverse-drug-reactions-incidence-clinical-characteristics-treatment-and-outcome-in-pediatric-patients
#40
JOURNAL ARTICLE
Patawee Na Bangxang, Wanee Wisuthsarewong, Rattanavalai Nitiyarom
BACKGROUND: Severe cutaneous adverse drug reactions (SCARs) can cause significant morbidity and mortality. Clinical data regarding such conditions is still limited in the pediatric population. OBJECTIVE: To investigate the incidence, clinical characteristics, treatment, and outcome of SCARs in Thai pediatric patients. METHODS: This retrospective study enrolled 52 patients aged less than 18 years who were diagnosed with acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS/TEN overlap during January 2005 to August 2021 at Siriraj Hospital...
January 6, 2024: Asian Pacific Journal of Allergy and Immunology
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