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417 results
  • Ockham's razor defeated: about two atypical cases of hemolytic uremic syndrome. [Journal Article]
    BMC Nephrol. 2020 Jul 11; 21(1):269.Schwarz C, Brehon A, … Rafat C
  • CONCLUSIONS: Both cases defy Ockham's razor in the sense that multiple causes could be traced to a single outcome; furthermore, they invite us to reflect on the physiopathology of HUS as they question the classical distinction between STEC-HUS and atypical HUS. We propose a two-hit mechanism model leading to HUS. Indeed, in case 1, HUS unfolded as a result of the synergistic interaction between an infectious trigger and a genetic predisposition. In case 2 however, it is the simultaneous occurrence of two infectious triggers that led to HUS. In dissent from Ockham's razor, an exceptional disease such as HUS may stem from the sequential occurrence or co-occurrence of several rare conditions.
  • Dermatological Toxicities of Bruton's Tyrosine Kinase Inhibitors. [Review]
    Am J Clin Dermatol. 2020 Jul 01 [Online ahead of print]Sibaud V, Beylot-Barry M, … Ysebaert L
  • The development of Bruton's tyrosine kinase (BTK) inhibitors represents a major breakthrough in the treatment of chronic lymphocytic leukemia and other B cell malignancies. The first-generation inhibitor ibrutinib works by covalent irreversible binding to BTK, a non-receptor tyrosine kinase of the TEC (transient erythroblastopenia of childhood) family that plays a critical role in the B-cell rece…
  • Transient aplastic crisis triggered by parvovirus B19 in a family with hereditary spherocytosis. [Case Reports]
    IDCases. 2020; 21:e00802.Cilla N, Domitien L, … Foulongne V
  • Acute parvovirus B19 infection may lead to erythroblastopenia crisis in patients with underlying red blood cells disorders. We report herein an uncommon concomitant transient aplastic crisis in a mother and her daughter, both affected by hereditary spherocytosis. The diagnosis was confirmed by the detection of a very high parvovirus B19 DNA load in both the mother's and daughter's sera, associate…
  • Rpl5-Inducible Mouse Model for Studying Diamond-Blackfan Anemia. [Journal Article]
    Discoveries (Craiova). 2019 Sep 30; 7(3):e96.Kazerounian S, Yuan D, … Gazda HT
  • Diamond-Blackfan anemia (DBA) is a rare congenital bone marrow disorder with mutations in ribosomal protein genes. Several animal models have been developed to study the pathological mechanism of DBA. Previously, we reported that the complete knock-out of both Rpl5 and Rps24 alleles were lethal, while heterozygous Rpl5+/- and Rps24+/- mice showed normal phenotype. To establish a more efficient mo…
  • Transient Erythroblastopenia of Childhood: A Review for the Pediatric Emergency Medicine Physician. [Case Reports]
    Pediatr Emerg Care. 2019 Mar; 35(3):237-240.Burns RA, Woodward GA
  • Transient erythroblastopenia of childhood is a form of pure red cell aplasia that is self-limited and occurs in children 4 years old and younger. It is characterized by an absence or a significantly reduced quantity of erythroblasts in the bone marrow without underlying congenital red blood cell abnormalities. Transient erythroblastopenia of childhood should be considered in previously healthy ch…
  • Regulation of globin-heme balance in Diamond-Blackfan anemia by HSP70/GATA1. [Journal Article]
    Blood. 2019 03 21; 133(12):1358-1370.Rio S, Gastou M, … Da Costa L
  • Diamond-Blackfan anemia (DBA) is a congenital erythroblastopenia that is characterized by a blockade in erythroid differentiation related to impaired ribosome biogenesis. DBA phenotype and genotype are highly heterogeneous. We have previously identified 2 in vitro erythroid cell growth phenotypes for primary CD34+ cells from DBA patients and following short hairpin RNA knockdown of RPS19, RPL5, a…
  • Pure red cell aplasia caused by azathioprine: case report and review of the literature. [Journal Article]
    Cardiovasc Hematol Disord Drug Targets. 2018 Aug 28 [Online ahead of print]Kounatidis D, Vallianou N, … Kokkinakis E
  • Pure red cell aplasia (PRCA) is a clinical entity comprising severe normochromic normocytic anemia, reticulocytopenia, erythroblastopenia in the bone marrow, with normal leukocyte and platelets count. PRCA can be classified into congenital and acquired, with the latter characterized as idiopathic or secondary to various infections, hematological malignancies, collagen vascular diseases, thymoma, …
  • Molecular approaches to diagnose Diamond-Blackfan anemia: The EuroDBA experience. [Review]
    Eur J Med Genet. 2018 Nov; 61(11):664-673.Da Costa L, O'Donohue MF, … MacInnes AW
  • Diamond-Blackfan anemia (DBA) is a rare congenital erythroblastopenia and inherited bone marrow failure syndrome that affects approximately seven individuals in every million live births. In addition to anemia, about 50% of all DBA patients suffer from various physical malformations of the face, hands, heart, or urogenital region. The disorder is almost exclusively driven by haploinsufficient mut…
  • [Thymoma and autoimmune diseases]. [Review]
    Rev Med Interne. 2018 Jan; 39(1):17-26.Jamilloux Y, Frih H, … Sève P
  • The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cy…
  • [Pregnancy induced erythroblastopenia: about a case and review of the literature]. [Case Reports]
    Pan Afr Med J. 2016; 24:149.Jihad D, Jaouad K, … Mohamed D
  • Pregnancy induced erythroblastopenia is an exceptional pathologic entity. Only isolated cases have been described in English literature. The aim of this study is to analyze the characteristics of this extremely rare disease through the description of a new case of pregnancy induced erythroblastopenia and through the study of the 17 cases reported before.
  • Glucose-6-phosphate dehydrogenase deficiency: the added value of cytology. [Case Reports]
    Ann Biol Clin (Paris). 2016 Jun 01; 74(3):299-305.Roelens M, Dossier C, … Da Costa L
  • We report the case of a 2 year-old boy hospitalized into the emergency room for influenza pneumonia infection. The evolution was marked by a respiratory distress syndrome, a severe hemolytic anemia, associated with thrombocytopenia and kidney failure. First, a diagnosis of hemolytic uremic syndrome (HUS) has been judiciously suggested due to the classical triad: kidney failure, hemolytic anemia a…
  • Clinicopathological associations of acquired erythroblastopenia. [Journal Article]
    Int J Clin Exp Med. 2015; 8(12):22515-9.Gunes G, Malkan UY, … Buyukasik Y
  • CONCLUSIONS: As a result, erythroblastopenia in the bone marrow may be an early sign of MDS. In those AE cases possibility of being MDS must be kept in mind as it can be mistaken for PRCA.To conclude, in adults MDS without excess blast is one of the most common causes of erythroblastopenia in clinical practice and in case of erythroblastopenia the presence of MDS should be investigated.
  • Update of the human parvovirus B19 biology. [Review]
    Transfus Clin Biol. 2016 Feb; 23(1):5-12.Servant-Delmas A, Morinet F
  • Since its discovery, the human parvovirus B19 (B19V) has been associated with many clinical situations in addition to the prototype clinical manifestations, i.e. erythema infectiosum and erythroblastopenia crisis. The clinical significance of the viral B19V DNA persistence in sera after acute infection remains largely unknown. Such data may constitute a new clinical entity and is discussed in thi…
  • Transient erythroblastopenia of childhood is an underdiagnosed and self-limiting disease. [Journal Article]
    Acta Paediatr. 2014 Jul; 103(7):e288-94.van den Akker M, Dror Y, Odame I
  • CONCLUSIONS: Transient erythroblastopenia of childhood is now diagnosed less frequently in our institution than in the last two decades. Although the aetiology remains largely unknown, it is possible that changes in causative environmental factors contribute to making TEC a rare disease. Clinicians need to be aware of TEC in order to prevent unnecessary diagnostic and therapeutic measurements.
  • Pure red cell aplasia associated with autoimmune hepatitis successfully treated with cyclosporine A. [Case Reports]
    Clin J Gastroenterol. 2014 Feb; 7(1):74-8.Sato A, Sano F, … Okuse C
  • A 47-year-old female with a 17-year history of autoimmune hepatitis had been treated with prednisolone, azathioprine, and ursodeoxycholic acid. Although her alanine aminotransferase level occasionally showed mild abnormality, the prednisolone dose could not be increased because she had developed cataract during the course of her illness. In May 2012, she developed severe normochromic normocytic a…
  • [The pure red cell aplasia in children (observation 2010-2012 years)]. [Case Reports]
    Georgian Med News. 2013 MarMtvarelidze Z, Kvezereli-Kopadze A, Kvezereli-Kopadze M
  • This study was designed to investigate the children with congenital (Diamond-Blackfan Anaemia - DBA) and acquired pure red cell aplasia (PRCA). 4 children, aged 1 month to 3 years with PRCA were enrolled in a trial. Investigations include: detailed history and physical examination, complete blood count with red blood cell indices, reticulocyte count, bone marrow examination, iron metabolism, vira…
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