Journal ArticleClin Adv Hematol Oncol · March 2024
The treatment landscape for BCR/ABL-negative myeloproliferative neoplasms (MPNs), driven by JAK2, CALR, and MPL mutations, has evolved significantly over the last decade. Recent regulatory approvals in polycythemia vera (PV) include the JAK inhibitor ruxol ...
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Journal ArticleBone Marrow Transplant · February 2024
Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF) and is recommended for patients with higher risk disease. However, there is a risk of early mortality, and optimal timing is unknown. JAK inhibitor (JAK ...
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Journal ArticleBlood · May 12, 2022
The goal of therapy for patients with essential thrombocythemia (ET) and polycythemia vera (PV) is to reduce thrombotic events by normalizing blood counts. Hydroxyurea (HU) and interferon-α (IFN-α) are the most frequently used cytoreductive options for pat ...
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Journal ArticleClin Lymphoma Myeloma Leuk · May 2022
INTRODUCTION: Treatment options in patients with myelofibrosis (MF) presenting with thrombocytopenia are limited. Final results of the phase 2 study (NCT01348490) of ruxolitinib in patients with MF and low baseline platelet counts (50 - 100 × 109/L) are re ...
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Journal ArticleAdv Radiat Oncol · 2021
BACKGROUND: Osteolytic lesions are present in 75% of patients with multiple myeloma (MM) and frequently require palliation with radiation therapy (RT). Prior case series of patients with MM with bone pain undergoing palliative RT suggests doses ≥12 Gy (equ ...
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Journal ArticleCase Rep Transplant · 2021
Immune thrombocytopenia is a consumptive coagulopathy that can be either idiopathic or associated with infectious or autoimmune etiologies. Here, we present a case of immune thrombocytopenia in the setting of acute liver failure due to coexisting diagnoses ...
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ConferenceBlood · November 5, 2020
IntroductionAllogeneic hematopoietic cell transplantation (HCT) remains the only potentially curative therapy for myelofibrosis (MF). However, despite improvements in donor availability, most patients receive non-HC ...
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Journal ArticleBlood Adv · September 22, 2020
Momelotinib (MMB) is a JAK1/2 and ACVR1 inhibitor with demonstrated clinical activity in all 3 hallmarks of myelofibrosis (MF): anemia, constitutional symptoms, and splenomegaly. In this phase 2 open-label translational biology study (NCT02515630) of 41 tr ...
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Journal ArticleBlood Adv · May 12, 2020
Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF). In this large multicenter retrospective study, overall survival (OS) in MF patients treated with allogeneic HCT (551 patients) and without HCT (non-HCT ...
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ConferenceBlood · November 13, 2019
BackgroundInterferons are recognized as active agents in the treatment of patients with high risk essential thrombocythemia (ET) or polycythemia vera (PV), both in the upfront setting as well as beyond. Several tria ...
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Journal ArticleBlood · October 31, 2019
Prior studies have reported high response rates with recombinant interferon-α (rIFN-α) therapy in patients with essential thrombocythemia (ET) and polycythemia vera (PV). To further define the role of rIFN-α, we investigated the outcomes of pegylated-rIFN- ...
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Journal ArticleEur J Haematol · October 2019
OBJECTIVE: Clozapine is the favoured antipsychotic for treatment-refractory schizophrenia, but has a 1%-2% incidence of agranulocytosis. Patients who require chemotherapy therefore pose a unique management dilemma for haematologists, oncologists and psychi ...
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Journal ArticleGenes Dev · October 1, 2019
Short telomere syndromes manifest as familial idiopathic pulmonary fibrosis; they are the most common premature aging disorders. We used genome-wide linkage to identify heterozygous loss of function of ZCCHC8, a zinc-knuckle containing protein, as a cause ...
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ConferenceBlood · November 29, 2018
AbstractIntroductionHU is the treatment of choice for patients (pts) with high risk ET/PV, however, PEG has been proposed as an alternative option due to its proposed potenti ...
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Journal ArticleClin Adv Hematol Oncol · March 2024
The treatment landscape for BCR/ABL-negative myeloproliferative neoplasms (MPNs), driven by JAK2, CALR, and MPL mutations, has evolved significantly over the last decade. Recent regulatory approvals in polycythemia vera (PV) include the JAK inhibitor ruxol ...
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Journal ArticleBone Marrow Transplant · February 2024
Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF) and is recommended for patients with higher risk disease. However, there is a risk of early mortality, and optimal timing is unknown. JAK inhibitor (JAK ...
Full textLink to itemCite
Journal ArticleBlood · May 12, 2022
The goal of therapy for patients with essential thrombocythemia (ET) and polycythemia vera (PV) is to reduce thrombotic events by normalizing blood counts. Hydroxyurea (HU) and interferon-α (IFN-α) are the most frequently used cytoreductive options for pat ...
Full textLink to itemCite
Journal ArticleClin Lymphoma Myeloma Leuk · May 2022
INTRODUCTION: Treatment options in patients with myelofibrosis (MF) presenting with thrombocytopenia are limited. Final results of the phase 2 study (NCT01348490) of ruxolitinib in patients with MF and low baseline platelet counts (50 - 100 × 109/L) are re ...
Full textLink to itemCite
Journal ArticleAdv Radiat Oncol · 2021
BACKGROUND: Osteolytic lesions are present in 75% of patients with multiple myeloma (MM) and frequently require palliation with radiation therapy (RT). Prior case series of patients with MM with bone pain undergoing palliative RT suggests doses ≥12 Gy (equ ...
Full textLink to itemCite
Journal ArticleCase Rep Transplant · 2021
Immune thrombocytopenia is a consumptive coagulopathy that can be either idiopathic or associated with infectious or autoimmune etiologies. Here, we present a case of immune thrombocytopenia in the setting of acute liver failure due to coexisting diagnoses ...
Full textLink to itemCite
ConferenceBlood · November 5, 2020
IntroductionAllogeneic hematopoietic cell transplantation (HCT) remains the only potentially curative therapy for myelofibrosis (MF). However, despite improvements in donor availability, most patients receive non-HC ...
Full textCite
Journal ArticleBlood Adv · September 22, 2020
Momelotinib (MMB) is a JAK1/2 and ACVR1 inhibitor with demonstrated clinical activity in all 3 hallmarks of myelofibrosis (MF): anemia, constitutional symptoms, and splenomegaly. In this phase 2 open-label translational biology study (NCT02515630) of 41 tr ...
Full textLink to itemCite
Journal ArticleBlood Adv · May 12, 2020
Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF). In this large multicenter retrospective study, overall survival (OS) in MF patients treated with allogeneic HCT (551 patients) and without HCT (non-HCT ...
Full textOpen AccessLink to itemCite
ConferenceBlood · November 13, 2019
BackgroundInterferons are recognized as active agents in the treatment of patients with high risk essential thrombocythemia (ET) or polycythemia vera (PV), both in the upfront setting as well as beyond. Several tria ...
Full textCite
Journal ArticleBlood · October 31, 2019
Prior studies have reported high response rates with recombinant interferon-α (rIFN-α) therapy in patients with essential thrombocythemia (ET) and polycythemia vera (PV). To further define the role of rIFN-α, we investigated the outcomes of pegylated-rIFN- ...
Full textLink to itemCite
Journal ArticleEur J Haematol · October 2019
OBJECTIVE: Clozapine is the favoured antipsychotic for treatment-refractory schizophrenia, but has a 1%-2% incidence of agranulocytosis. Patients who require chemotherapy therefore pose a unique management dilemma for haematologists, oncologists and psychi ...
Full textLink to itemCite
Journal ArticleGenes Dev · October 1, 2019
Short telomere syndromes manifest as familial idiopathic pulmonary fibrosis; they are the most common premature aging disorders. We used genome-wide linkage to identify heterozygous loss of function of ZCCHC8, a zinc-knuckle containing protein, as a cause ...
Full textLink to itemCite
ConferenceBlood · November 29, 2018
AbstractIntroductionHU is the treatment of choice for patients (pts) with high risk ET/PV, however, PEG has been proposed as an alternative option due to its proposed potenti ...
Full textCite
Journal ArticleJ Hematol Oncol · February 22, 2017
BACKGROUND: The randomized, double-blind, placebo-controlled, phase 3 COMFORT-I trial evaluated the JAK1/JAK2 inhibitor ruxolitinib in patients with intermediate-2/high-risk myelofibrosis. The primary and planned 3-year analyses of COMFORT-I data demonstra ...
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Journal ArticleOpen Forum Infect Dis · September 2016
We report the case of a 60-year-old man with septic shock due to Capnocytophaga canimorsus that was diagnosed in 24 hours by a novel whole-genome next-generation sequencing assay. This technology shows great promise in identifying fastidious pathogens, and ...
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Journal ArticleBiol Blood Marrow Transplant · March 2016
The impact of Janus kinase (JAK) 1/2 inhibitor therapy before allogeneic hematopoietic cell transplantation (HCT) has not been studied in a large cohort in myelofibrosis (MF). In this retrospective multicenter study, we analyzed outcomes of patients who un ...
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Journal ArticleEur J Intern Med · June 2015
Erythrocytosis is frequently encountered as an incidental abnormality on laboratory testing that reveals persistent elevation of the hematocrit level (>52% in men and >48% in women). In many cases, erythrocytosis is the manifestation of an underlying cardi ...
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Journal ArticleHaematologica · April 2015
In the phase III COMFORT-I study, the Janus kinase 1 (JAK1)/JAK2 inhibitor ruxolitinib provided significant improvements in splenomegaly, key symptoms, and quality-of-life measures and was associated with an overall survival benefit relative to placebo in ...
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Journal ArticleJ Natl Compr Canc Netw · April 2015
The classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), which include essential thrombocythemia, polycythemia vera, and myelofibrosis (MF), are in a new era of molecular diagnosis, ushered in by the identification of the JAK2(V61 ...
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Journal ArticleEur Respir J · July 2014
Lung transplantation is the only intervention that prolongs survival in idiopathic pulmonary fibrosis (IPF). Telomerase mutations are the most common identifiable genetic cause of IPF, and at times, the telomere defect manifests in extrapulmonary disease s ...
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Journal ArticleHaematologica · February 2014
Prior to Janus kinase inhibitors, available therapies for myelofibrosis were generally supportive and did not improve survival. This analysis compares efficacy outcomes of patients with myelofibrosis in the control arms (placebo [n=154] and best available ...
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Journal ArticleHaematologica · December 2013
COMFORT-I is a randomized, double-blind, placebo-controlled trial of the Janus kinase 1/Janus kinase 2 inhibitor ruxolitinib in 309 patients with intermediate-2 or high-risk myelofibrosis. This analysis of COMFORT-I describes the long-term efficacy and saf ...
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Journal ArticleBr J Haematol · May 2013
Myelofibrosis (MF) patients can present with a wide spectrum of disease characteristics. We analysed the consistency of ruxolitinib efficacy across patient subgroups in the COntrolled MyeloFibrosis Study With ORal JAK Inhibitor Treatment (COMFORT-I,) a dou ...
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Journal ArticleAm J Clin Pathol · December 2012
We describe 41 cases of myeloid neoplasms (MNs) secondary to plasma cell myeloma (PCM). The types of MN included myelodysplastic syndrome (MDS) in 34 (82.9%), acute myeloid leukemia (AML) in 4 (9.8%), and myeloproliferative neoplasm (MPN) or MDS/MPN in 3 ( ...
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Journal ArticleOncogene · March 29, 2012
Erythropoietin (Epo) is widely used clinically to treat anemia associated with various clinical conditions including cancer. Data from several clinical trials suggest significant adverse effect of Epo treatment on cancer patient survival. However, controve ...
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Journal ArticleN Engl J Med · March 1, 2012
BACKGROUND: Ruxolitinib, a selective inhibitor of Janus kinase (JAK) 1 and 2, has clinically significant activity in myelofibrosis. METHODS: In this double-blind trial, we randomly assigned patients with intermediate-2 or high-risk myelofibrosis to twice-d ...
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Journal ArticleBMC Nephrol · December 12, 2011
BACKGROUND: High-dose erythropoiesis-stimulating agents (ESA) for anemia of chronic kidney disease (CKD) have been associated with adverse clinical outcomes and do not always improve erythropoiesis. We hypothesized that high-dose ESA requirement would be a ...
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Journal ArticleExp Cell Res · May 15, 2011
Recombinant human erythropoietin (rhEPO), the prototype erythropoiesis-stimulating agent developed in the 1980s, was among the first recombinant human proteins to be marketed for clinical use in the oncology setting. Anemia is a frequent concern in patient ...
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Journal ArticleJ Clin Oncol · November 20, 2010
PURPOSE: To update American Society of Clinical Oncology/American Society of Hematology recommendations for use of erythropoiesis-stimulating agents (ESAs) in patients with cancer. METHODS: An Update Committee reviewed data published between January 2007 a ...
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Journal ArticleBlood · November 18, 2010
PURPOSE: To update American Society of Hematology/American Society of Clinical Oncology recommendations for use of erythropoiesis-stimulating agents (ESAs) in patients with cancer. METHODS: An Update Committee reviewed data published between January 2007 a ...
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Journal ArticleExp Hematol · December 2009
OBJECTIVE: Polycythemia vera (PV) is characterized by erythrocytosis associated with the presence of the activating JAK2(V617F) mutation in a variable proportion of hematopoietic cells. JAK2(V617F) is detected in other myeloproliferative neoplasms, does no ...
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Journal ArticleBiochem Biophys Res Commun · February 13, 2009
The role of erythropoietin receptor (EpoR) expression in tumor cells and the potential of EpoR-mediated signaling to contribute to cellular proliferation and invasiveness require further characterization. To determine whether EpoR expression and activation ...
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Journal ArticleMol Cell Biol · December 2008
Erythroid Krüppel-like factor (EKLF) is a Krüppel-like transcription factor identified as a transcriptional activator and chromatin modifier in erythroid cells. EKLF-deficient (Eklf(-/-)) mice die at day 14.5 of gestation from severe anemia. In this study, ...
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Journal ArticleClin Cancer Res · August 1, 2008
Erythropoiesis-stimulating agents (ESA) used for the treatment of chemotherapy-induced anemia in cancer patients have been associated with adverse outcomes of enhanced tumor progression and impaired survival in a series of recent clinical trials. As clinic ...
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Journal ArticleJ Thromb Thrombolysis · August 2008
BACKGROUND AND OBJECTIVE: Internet-based disease management programs have the potential to improve patient care. The objective of this study was to determine whether an interactive, internet-based system enabling supervised, patient self-management of oral ...
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Journal ArticleBr J Haematol · April 2008
In the haematopoietic system, the principal function of erythropoietin (Epo) is the regulation of red blood cell production, mediated by its specific cell surface receptor (EpoR). Following the cloning of the Epo gene (EPO) and characterization of the sele ...
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Journal ArticlePLoS One · June 20, 2007
BACKGROUND: The induction of tumor angiogenesis, a pathologic process critical for tumor progression, is mediated by multiple regulatory factors released by tumor and host cells. We investigated the role of the hematopoietic cytokine erythropoietin as an a ...
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Journal ArticleBiochem Biophys Res Commun · March 9, 2007
The cardiotoxic adverse effects of anthracycline antibiotics limit their therapeutic utility as essential components of chemotherapy regimens for hematologic and solid malignancies. Here we show that the hematopoietic cytokine erythropoietin attenuates dox ...
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Journal ArticleMol Cancer Ther · February 2006
Evidence for erythropoietin signaling has been shown in several nonhematopoietic tissues, including many tumor types. Clinically, recombinant erythropoietin treatment of malignancy-related anemia has yet to be definitively associated with any modulation of ...
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Journal ArticleClin Cancer Res · January 15, 2006
Erythropoietin (Epo) has long been known to be the principal hematopoietic growth factor that regulates cellular proliferation and differentiation along the erythroid lineage. Recent studies have shown that Epo is a pleiotropic cytokine that is proangiogen ...
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Journal ArticleFASEB J · August 2005
Langendorff-perfused rat hearts treated with EPO exhibited significantly improved postischemic recovery of left ventricular developed pressure (LVDP) and reduced infarct size compared with control hearts. Perfusion with the mitogen/extracellular signal-reg ...
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Journal ArticleBr J Haematol · July 2005
The regeneration of circulating red blood cells in response to anaemia associated with blood loss or haemolysis involves an increased rate of erythropoiesis and expansion of proerythroblasts, the bone marrow precursor cells that terminally differentiate in ...
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Journal ArticleBiochim Biophys Acta · April 15, 2005
Erythropoietin receptor (EPOR) gene mutations leading to truncations of the cytoplasmic, carboxy-terminal region of EPOR have been described in some patients with primary familial and congenital polycythemia (PFCP), a disorder characterized by isolated ery ...
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Journal ArticleExp Hematol · March 2005
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OBJECTIVE: In a previous study, we showed that activation of a transfected human erythropoietin receptor (EPOR) in the murine myeloid cell line 32D resulted in the development of morphologic features of granulocytic differentiation and expression of the ne ...
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Journal ArticleMod Pathol · March 2005
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Erythropoietin is a hematopoietic cytokine that regulates the production of red blood cells. Erythropoietin is normally produced in the adult kidney in a hypoxia-inducible manner. The recombinant form of human erythropoietin is in clinical use for the prev ...
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Journal ArticleClin Cancer Res · January 1, 2005
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PURPOSE: Erythropoietin, an oxygen-regulated glycoprotein hormone, is a hematopoietic cytokine that stimulates erythropoiesis by binding to its cellular receptor [erythropoietin receptor (EPOR)]. The recombinant form of human erythropoietin is used to prev ...
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Journal ArticleFASEB J · June 2004
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Erythropoietin (EPO), the principal hematopoietic cytokine that regulates mammalian erythropoiesis, exhibits diverse cellular effects in non-hematopoietic tissues. The physiologic functions of EPO are mediated by its specific cell-surface receptor EPOR. In ...
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Journal ArticleAm J Pathol · September 2003
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In this study, we investigated the role of the hematopoietic cytokine erythropoietin (EPO) during wound healing, the physiological response to tissue injury. We used an in vivo wound-healing assay (fibrin Z-chambers) consisting of fibrin-filled chambers im ...
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Journal ArticleBiochem Biophys Res Commun · August 8, 2003
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Erythropoietin (EPO) regulates mammalian erythropoiesis by binding to its transmembrane receptor EPOR. Recent studies demonstrated functional EPOR expression in human cancer cells. Recombinant human EPO was reported to stimulate the proliferation of monola ...
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Journal ArticleLab Invest · July 2002
Erythropoietin (EPO) is the principal hematopoietic cytokine that regulates mammalian erythropoiesis by binding to its transmembrane receptor EpoR. Recent experimental evidence suggests that the biologic effects of EPO are not limited to the regulation of ...
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Journal ArticleBlood · June 15, 2002
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A drug that specifically inhibits erythropoiesis would be clinically useful. The erythropoietin (Epo) mutant Epo (R103A) could potentially be used for this purpose. Epo (R103A) has a single amino acid substitution of alanine for arginine at position 103. B ...
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Journal ArticleBlood · April 15, 2002
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Primary familial erythrocytosis (familial polycythemia) is a rare myeloproliferative disorder with an autosomal dominant mode of inheritance. We studied a new kindred with autosomal dominantly inherited familial erythrocytosis. The molecular basis for the ...
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Journal ArticleBull Exp Biol Med · August 2001
We studied functional activity of the system responsible for generation of reactive oxygen species by blood neutrophils and involved in pathophysiological mechanisms of bronchopulmonary diseases. Insufficiency of this system can be classified as relative, ...
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Journal ArticleEur J Haematol · August 2001
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OBJECTIVES: The precise role of hematopoietic cytokine/cytokine receptor interactions in lineage-restricted hematopoietic differentiation giving rise to mature blood cells of diverse function is incompletely defined. To study lineage-specific effects of cy ...
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Journal ArticleBlood · December 1, 2000
Hematopoietic cytokines and intracellular signals emanating from their cell surface receptors support the proliferation, viability and differentiation of bone marrow cells. The precise role of cytokine/cytokine receptor interactions in lineage-restricted h ...
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Journal ArticleTrans Am Clin Climatol Assoc · 2000
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We studied a kindred with dominantly inherited familial erythrocytosis associated with heterozygosity for a deletion of seven nucleotides in exon 8 of the EpoR gene resulting in an EpoR peptide that is truncated by 59 amino acids in its C-terminal intracyt ...
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Journal ArticleSemin Hematol · October 1999
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This article provides an overview of the techniques currently available for the molecular diagnosis of hemoglobinopathies and other inherited erythrocyte disorders. Advances in both clinical practice and molecular biology have permitted rapid genetic diagn ...
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Journal ArticleExp Hematol · January 1999
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The erythropoietin receptor (EpoR) has been previously shown to contain a cytoplasmic C-terminal negative regulatory domain, experimental deletion or mutation of which leads to increased sensitivity of expressing cells to the effects erythropoietin (Epo). ...
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Journal ArticleBlood · August 15, 1998
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The role of hematopoietic growth factors in lineage commitment and differentiation is unclear. We present evidence that heterologous expression of an erythroid specific receptor allows granulocytic differentiation of a myeloid cell line. We have previously ...
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Journal ArticleBlood · June 15, 1997
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Familial erythrocytosis (familial polycythemia) inherited as an autosomal dominant trait has recently been reported to be associated with mutations in the gene encoding the erythropoietin receptor (EpoR) in a small number of families. We studied a new kind ...
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Journal ArticleMol Cell Biol · April 1997
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Persistent expression of the gamma-globin genes in adults with deletion types of hereditary persistence of fetal hemoglobin (HPFH) is thought to be mediated by enhancer-like effects of DNA sequences at the 3' breakpoints of the deletions. A transgenic mous ...
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Journal ArticleSemin Perinatol · December 1995
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Hematologic disorders are implicated in approximately 10% to 27% of cases of nonimmune hydrops fetalis. In almost all of these disorders, anemia leading to heart failure, edema, ascites, and anasarca is the final common denominator. The etiology of the ane ...
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Journal ArticleBlood · February 1, 1992
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Previous analysis of the hemoglobin phenotype of the K562 human erythroleukemia cell line showed regulated expression of the epsilon-, zeta-, gamma-, alpha-, and delta-globin genes. Expression of the beta-globin genes has not been previously detected in th ...
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Journal ArticleUrology · February 1992
We examined the relationship between cigarette smoking and erectile physiology in 314 men with erectile dysfunction. All of the men studied were currently cigarette smokers. Evaluations included interviews, physical examinations, and polysomnographic asses ...
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Journal ArticleJ Biol Chem · November 5, 1991
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p18 is a phosphoprotein that is present in great abundance in acute leukemia blasts and in less abundance in proliferating lymphocytes. This protein undergoes major changes in its state of phosphorylation upon induction of differentiation of leukemic cells ...
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Journal ArticleUrology · September 1990
Sleep studies were performed on 1,025 patients complaining of erectile dysfunction. In addition to standard measures of sleep stage and nocturnal penile tumescence, respiratory activity was evaluated. The number of episodes of sleep apnea per hour (Apnea I ...
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