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Article ; Online: Is pharmacological thromboprophylaxis necessary in persons with haemophilia undergoing major orthopaedic surgery?

Linari, Silvia / Pieri, Lisa / Carulli, Christian / Demartis, Francesco / Fjerza, Rajmonda / Prisco, Domenico / Castaman, Giancarlo

Haemophilia : the official journal of the World Federation of Hemophilia

2024

Language	English
Publishing date	2024-04-04
Publishing country	England
Document type	Letter
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.15012
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Management of thrombocytopenia in cancer.

Castaman, Giancarlo / Pieri, Lisa

Thrombosis research

2018 Volume 164 Suppl 1, Page(s) S89–S93

Abstract: Thrombocytopenia is a common finding in cancer patients and can have different and/or multifactorial pathogenesis. While in solid tumors it occurs often as a consequence of chemotherapy treatment, it is frequently observed at diagnosis in patients with ... ...

Abstract	Thrombocytopenia is a common finding in cancer patients and can have different and/or multifactorial pathogenesis. While in solid tumors it occurs often as a consequence of chemotherapy treatment, it is frequently observed at diagnosis in patients with hematological malignancies being aggravated to a potentially life-threatening level during chemotherapy. Other associated conditions (infections, drugs, consumption coagulopathy etc.) can add to influence the degree of thrombocytopenia and the inherent risk of bleeding and they should be recognized and corrected to improve platelet count. Platelet transfusion remains the cornerstone of treatment, but its use should always be weighted taking into consideration the actual risk of bleeding to avoid inappropriate use and wasting of resources. While in hematological malignancies a threshold level of 10,000 platelets/μL is widely accepted as the minimal level prompting prophylactic platelet transfusion, this limit is less frequently observed in patients with solid tumors and platelet transfusions are usually administered for a few days, possibly at a higher platelet level. Alternative treatments for the latter patients including thrombopoietin-mimetic agents are increasingly used and found to be promising.
MeSH term(s)	Humans ; Neoplasms/complications ; Thrombocytopenia/etiology ; Thrombocytopenia/therapy
Language	English
Publishing date	2018-01-13
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	121852-9
ISSN	1879-2472 ; 0049-3848
ISSN (online)	1879-2472
ISSN	0049-3848
DOI	10.1016/j.thromres.2018.02.001
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Zs.A 863: Show issues

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Article ; Online: An Assessment of Faculty Perceptions of Wellness Needs and Strategies to Optimize Engagement With Wellness Resources in Academic Medicine.

Pieri, Matteo F / Becker-Haimes, Emily M / Weiss, Michal / Grabill, Megan / Neergaard, Rebecca / Bellini, Lisa / Wolk, Courtney Benjamin

Journal of occupational and environmental medicine

2023 Volume 65, Issue 7, Page(s) e478–e484

Abstract: Objective: Faculty at academic medical institutions are at increased risk for burnout. This study aimed to assess faculty perceptions of wellness needs and identify strategies to optimize engagement with individualized wellness resources.: Methods: ... ...

Abstract	Objective: Faculty at academic medical institutions are at increased risk for burnout. This study aimed to assess faculty perceptions of wellness needs and identify strategies to optimize engagement with individualized wellness resources. Methods: Semistructured interviews were conducted with 37 faculty members in one US academic medical center. Results: Participants identified significant barriers to achieving emotional health and wellness goals. Areas where participants identified needing the most support included interpersonal relationships, accountability for wellness goals, career support, financial resources, and mentorship. Most participants were unaware of all wellness resources available at their institution. Participants recommended regular marketing and emphasizing confidentiality of employer-sponsored programs. They also provided feedback on specific dissemination and marketing methods. Conclusions: This research underscores the need for wellness resources for faculty and the importance of intentional dissemination of these resources to optimize uptake.
MeSH term(s)	Humans ; Faculty, Medical/psychology ; Interpersonal Relations ; Burnout, Professional/psychology ; Academic Medical Centers ; Burnout, Psychological
Language	English
Publishing date	2023-04-11
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1223932-x
ISSN	1536-5948 ; 1076-2752
ISSN (online)	1536-5948
ISSN	1076-2752
DOI	10.1097/JOM.0000000000002863
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Article ; Online: Improving prognostic tools in systemic mastocytosis: Insights from mutations.

Pieri, Lisa / Vannucchi, Alessandro M

American journal of hematology

2016 Volume 91, Issue 9, Page(s) 867–868

Language	English
Publishing date	2016
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.24472
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Zs.A 1251: Show issues

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Article ; Online: Combined use of 177Lu-DOTATATE and metronomic capecitabine (Lu-X) in FDG-positive gastro-entero-pancreatic neuroendocrine tumors.

Nicolini, Silvia / Bodei, Lisa / Bongiovanni, Alberto / Sansovini, Maddalena / Grassi, Ilaria / Ibrahim, Toni / Monti, Manuela / Caroli, Paola / Sarnelli, Anna / Diano, Danila / Di Iorio, Valentina / Grana, Chiara Maria / Cittanti, Corrado / Pieri, Federica / Severi, Stefano / Paganelli, Giovanni

European journal of nuclear medicine and molecular imaging

2021 Volume 48, Issue 10, Page(s) 3260–3267

Abstract: Purpose: FDG-positive neuroendocrine tumors (NETs) have a poorer prognosis and exhibit shorter response duration to peptide receptor radionuclide therapy (PRRT). The aim of this prospective phase II study was to evaluate the efficacy and toxicity of ... ...

Abstract	Purpose: FDG-positive neuroendocrine tumors (NETs) have a poorer prognosis and exhibit shorter response duration to peptide receptor radionuclide therapy (PRRT). The aim of this prospective phase II study was to evaluate the efficacy and toxicity of PRRT with Patients and methods: Patients with advanced somatostatin receptor- and FDG-positive G1-G3 GEP-NETs (Ki67 < 55%) were treated with a cumulative activity of 27.5 GBq of Results: From August 2015 to December 2016, 37 subjects were consecutively enrolled. A total of 25 (68%) were affected by pancreatic neuroendocrine tumors (P-NETs), and 12 (32%) had gastrointestinal neuroendocrine tumors (GI-NETs). By grading (WHO 2010 classification), 12 patients (32%) had G1 (Ki67 ≤ 2%), 22 (59%) had G2 (3% < Ki67 ≤ 20%), and 3 patients (9%) had G3 (Ki67 > 20%) NETs. Grade 3 (G3) or 4 (G4) hematological toxicity occurred in 16.2% of patients. Other G3-G4 adverse events were diarrhea in 5.4% of cases and asthenia in 5.4%. No renal toxicity was observed for the duration of follow-up. In 37 patients, 33 were evaluable for response. Objective responses included partial response (PR) in 10 patients (30%) and stable disease (SD) in 18 patients (55%), with a DCR of 85%. The median follow-up was 38 months (range 4.6-51.1 months). The median PFS was 31.4 months (17.6-45.4), and mOS was not reached. Conclusions: This study demonstrated that the combination of PRRT with
MeSH term(s)	Capecitabine/adverse effects ; Fluorodeoxyglucose F18 ; Humans ; Neuroendocrine Tumors/drug therapy ; Neuroendocrine Tumors/radiotherapy ; Octreotide/adverse effects ; Octreotide/analogs & derivatives ; Organometallic Compounds ; Pancreatic Neoplasms/drug therapy ; Pancreatic Neoplasms/radiotherapy ; Positron Emission Tomography Computed Tomography ; Prospective Studies
Chemical Substances	Organometallic Compounds ; Fluorodeoxyglucose F18 (0Z5B2CJX4D) ; Capecitabine (6804DJ8Z9U) ; lutetium Lu 177 dotatate (AE221IM3BB) ; Octreotide (RWM8CCW8GP)
Language	English
Publishing date	2021-02-18
Publishing country	Germany
Document type	Clinical Trial, Phase II ; Journal Article
ZDB-ID	8236-3
ISSN	1619-7089 ; 0340-6997 ; 1619-7070
ISSN (online)	1619-7089
ISSN	0340-6997 ; 1619-7070
DOI	10.1007/s00259-021-05236-z
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Zs.A 1227: Show issues

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Article ; Online: Infective Uvulitis in a Child.

De Pieri, Carlo / Valentini, Elena / Pusiol, Anna / Passone, Eva / Gamalero, Lisa / Cogo, Paola Elisa

Pediatric emergency care

2020 Volume 38, Issue 2, Page(s) e501–e502

Abstract: Abstract: Infective uvulitis is a rare condition in children. In this report, we describe the case of a 4-year old-patient who presented a group A Streptococcus pharyngitis with uvulitis. No signs of epiglottitis were detected at nasal fibroscopy. She ... ...

Abstract	Abstract: Infective uvulitis is a rare condition in children. In this report, we describe the case of a 4-year old-patient who presented a group A Streptococcus pharyngitis with uvulitis. No signs of epiglottitis were detected at nasal fibroscopy. She recovered rapidly with intravenous antibiotic therapy and 2 days of corticosteroid. Uvulitis is usually caused by group A Streptococcus or Haemophilus influentiae, but also other bacteria can be detected. Uvulitis can be isolated, or it can occur with epiglottitis and become an emergency.
MeSH term(s)	Child ; Child, Preschool ; Epiglottitis/diagnosis ; Epiglottitis/drug therapy ; Female ; Haemophilus Infections/diagnosis ; Haemophilus Infections/drug therapy ; Humans ; Pharyngitis ; Stomatitis ; Streptococcus pyogenes ; Uvula
Language	English
Publishing date	2020-10-21
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	632588-9
ISSN	1535-1815 ; 0749-5161
ISSN (online)	1535-1815
ISSN	0749-5161
DOI	10.1097/PEC.0000000000002258
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Zs.A 2083: Show issues

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Article: Safe and Successful Surgical Outcome in Persons with Hemophilia A with and without Inhibitors Treated with Emicizumab: A Large, Single Center, Real-World Experience.

Castaman, Giancarlo / Linari, Silvia / Pieri, Lisa / Carulli, Christian / Prosperi, Paolo / Tonelli, Paolo / Demartis, Francesco / Fjerza, Rajmonda / Attanasio, Monica / Coppo, Mirella / Salvianti, Francesca

Journal of clinical medicine

2023 Volume 12, Issue 6

Abstract: Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with severe hemophilia A ...

Abstract	Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with severe hemophilia A with and without inhibitors. This approach provides constant protection, with significant reduction in bleeding rate and improved quality of life. However, protection provided by emicizumab is not absolute, and clotting factor concentrates (FVIII, rFVIIa, aPCC) may be necessary for post-traumatic bleeding or surgery, with a potential thrombotic risk or difficulty in preventing bleeding. Real world evidence is still scanty, especially for managing major surgery. In this study, 75 surgeries were managed in 28 patients (27 major procedures in 15 patients and 48 minor procedures in 20 patients. In 17 patients without inhibitors, 30 minor surgeries were carried out by using FVIII in 5, with only a bleeding event, which was successfully treated with FVIII concentrate. Six major surgeries were uneventfully performed with FVIII concentrate. Eleven PWHA and high-titer inhibitors underwent 39 surgical procedures (18 minor and 21 major surgeries). Minor surgeries were mostly performed without prophylaxis with rFVIIa, with only a single bleeding complication. All 21 major surgeries were covered with a homogeneous protocol using rFVIIa. In four instances, bleeding complications occurred, treated with rFVIIa. Of them, a single patient only failed to respond and died because of an uncontrollable bleeding from a large ruptured retroperitoneal pseudotumor. Surgery in patients with emicizumab can be safely carried out with the use of appropriate replacement therapy protocols.
Language	English
Publishing date	2023-03-16
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662592-1
ISSN	2077-0383
ISSN	2077-0383
DOI	10.3390/jcm12062317
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Article ; Online: Low bone mass and hypovitaminosis D in haemophilia: A single-centre study in patients with severe and moderate haemophilia A and B.

Linari, Silvia / Melchiorre, Daniela / Pieri, Lisa / Tofani, Lorenzo / Fanelli, Alessandra / Brogi, Marco / Castaman, Giancarlo

Haemophilia : the official journal of the World Federation of Hemophilia

2020 Volume 26, Issue 5, Page(s) 898–906

Abstract: Introduction: Haemophilia (H) is frequently associated with a multifactorial reduction in bone mineral density (BDM), but little is known about possible differences between HA and HB according to their severity.: Aim: To evaluate the association ... ...

Abstract	Introduction: Haemophilia (H) is frequently associated with a multifactorial reduction in bone mineral density (BDM), but little is known about possible differences between HA and HB according to their severity. Aim: To evaluate the association between low bone mineral density (BMD), 25-hydroxyvitamin D [25(OH)D] concentrations and bone turnover markers in patients with HA and HB younger or older than 50 years. Methods: In 78 patients <50 years and 33 patients >50 years with severe (S) or moderate (M) HA and HB, BMD was measured by dual-energy X-ray absorptiometry at femoral neck (FN) and lumbar spine and then correlated to annual bleeding rate (ABR), World Federation of Haemophilia orthopaedic joint scale (WFH score), 25(OH)D concentrations, parathyroid hormone (PTH), amino-terminal telopeptide of type 1 collagen (NTx), urinary pyridinolines, osteocalcin and bone-specific alkaline phosphatase. Results: Overall, a high prevalence of hypovitaminosis D was diagnosed. In patients <50 years, low FN-BMD was significantly more frequent in HA than in HB, while PTH, pyridinolines, ABR and WFH score were associated with H type and severity. In patients >50 years, similarly low FN-BMD was observed in HA and HB, while ABR and WFH score were associated with H type and severity, being milder in HB. Conclusions: Low bone mass is a frequent comorbidity in haemophilic patients of all ages, apart from those with MHB. Clinical and laboratory assessments confirm a higher bone impairment and faster bone resorption in HA compared with HB. Looking at H type and severity, MHB seems to have a normal bone metabolism and a less severe disease.
MeSH term(s)	Bone Density/physiology ; Female ; Hemophilia A/blood ; Hemophilia A/complications ; Hemophilia B/blood ; Hemophilia B/complications ; Humans ; Male ; Middle Aged ; Vitamin D Deficiency/etiology
Language	English
Publishing date	2020-08-25
Publishing country	England
Document type	Journal Article
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.14127
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Safe and Successful Surgical Outcome in Persons with Hemophilia A with and without Inhibitors Treated with Emicizumab

Giancarlo Castaman / Silvia Linari / Lisa Pieri / Christian Carulli / Paolo Prosperi / Paolo Tonelli / Francesco Demartis / Rajmonda Fjerza / Monica Attanasio / Mirella Coppo / Francesca Salvianti

Journal of Clinical Medicine, Vol 12, Iss 2317, p

A Large, Single Center, Real-World Experience

2023 Volume 2317

Abstract	Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with severe hemophilia A with and without inhibitors. This approach provides constant protection, with significant reduction in bleeding rate and improved quality of life. However, protection provided by emicizumab is not absolute, and clotting factor concentrates (FVIII, rFVIIa, aPCC) may be necessary for post-traumatic bleeding or surgery, with a potential thrombotic risk or difficulty in preventing bleeding. Real world evidence is still scanty, especially for managing major surgery. In this study, 75 surgeries were managed in 28 patients (27 major procedures in 15 patients and 48 minor procedures in 20 patients. In 17 patients without inhibitors, 30 minor surgeries were carried out by using FVIII in 5, with only a bleeding event, which was successfully treated with FVIII concentrate. Six major surgeries were uneventfully performed with FVIII concentrate. Eleven PWHA and high-titer inhibitors underwent 39 surgical procedures (18 minor and 21 major surgeries). Minor surgeries were mostly performed without prophylaxis with rFVIIa, with only a single bleeding complication. All 21 major surgeries were covered with a homogeneous protocol using rFVIIa. In four instances, bleeding complications occurred, treated with rFVIIa. Of them, a single patient only failed to respond and died because of an uncontrollable bleeding from a large ruptured retroperitoneal pseudotumor. Surgery in patients with emicizumab can be safely carried out with the use of appropriate replacement therapy protocols.
Keywords	hemophilia A ; FVIII inhibitors ; FVIII concentrates ; recombinant FVIIa ; aPCC ; prophylaxis ; Medicine ; R
Subject code	616
Language	English
Publishing date	2023-03-01T00:00:00Z
Publisher	MDPI AG
Document type	Article ; Online
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Article ; Online: Inhibitors in Nonsevere Hemophilia A: What Is Known and Searching for the Unknown.

Abdi, Amal / Linari, Silvia / Pieri, Lisa / Voorberg, Jan / Fijnvandraat, Karin / Castaman, Giancarlo

Seminars in thrombosis and hemostasis

2018 Volume 44, Issue 6, Page(s) 568–577

Abstract: Nonsevere hemophilia A (NSHA) is an inherited X-linked bleeding disorder, caused by mutations of ... ...

Abstract	Nonsevere hemophilia A (NSHA) is an inherited X-linked bleeding disorder, caused by mutations of the
MeSH term(s)	Factor VIII/pharmacology ; Factor VIII/therapeutic use ; Hemophilia A/pathology ; Hemophilia A/therapy ; Humans
Chemical Substances	F8 protein, human (839MOZ74GK) ; Factor VIII (9001-27-8)
Language	English
Publishing date	2018-02-13
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0037-1621717
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