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1.
J Neurosci Methods ; 367: 109444, 2022 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-34921842

RESUMO

BACKGROUND: In preclinical research involving murine models of neurological diseases, Motor Evoked Potentials (MEPs) can detect pathological alterations in nerve conduction throughout the cortico-spinal tract. In mice, MEPs elicited by electrical stimulation of the motor cortex can be performed with epicranial or subdermal electrodes such as implanted screws or removable needles, which are associated with invasive surgery and variability in placement of the stimulating electrodes, respectively. METHODS: We compared MEPs induced by epicranial or subcutaneous stimulation with a non-invasive surface cup electrode over five recording sessions, in healthy C57BL/6 mice. Additionally, using surface stimulation, we examined the recordings obtained with intramuscular needles or surface electrodes to understand if MEP reproducibility could be improved. RESULTS: Resting motor threshold (RMT), MEP latency and amplitude were comparable among the different stimulation methods. Epicranial, subcutaneous and surface stimulation techniques presented good repeatability over time, with surface stimulation showing a significantly reduced inter-session variability. Compared with intramuscular needles, MEPs recorded with surface electrode showed reduced peak-to-peak amplitude at all timepoints. RMT and MEP latency were comparable with both recording methods. On the other hand, amplitudes recorded with the surface electrode presented a significantly lower inter-session variance, resulting in improved repeatability. CONCLUSION: Overall, there is evidence for highly reproducible results using different stimulating methods, with indication for reduced inter-session variability for surface stimulation. Moreover, MEP recording with surface electrode provided a decrease in amplitude variability over time, indicating improved measurement stability when considering amplitude as functional outcome in longitudinal studies.

2.
Blood Cancer J ; 11(12): 199, 2021 12 11.
Artigo em Inglês | MEDLINE | ID: mdl-34897288

RESUMO

Arterial (AT) and venous (VT) thrombotic events are the most common complications in patients with polycythemia vera (PV) and are the leading causes of morbidity and mortality. In this regard, the impact of JAK2V617F variant allele frequency (VAF) is still debated. The purpose of the current study was to analyze the impact of JAK2V617F VAF in the context of other established risk factors for thrombosis in a total of 865 2016 WHO-defined PV patients utilizing two independent cohorts: University of Florence (n = 576) as a training cohort and Policlinico Gemelli, Catholic University, Rome (n = 289) as a validation cohort. In the training cohort VT free-survival was significantly shorter in the presence of a JAK2V617F VAF > 50% (HR 4; p < 0.0001), whereas no difference was found for AT (HR 0.9; p = 0.8). Multivariable analysis identified JAK2V617F VAF > 50% (HR 3.8, p = 0.001) and previous VT (HR 2.2; p = 0.04) as independent risk factors for future VT whereas diabetes (HR 2.4; p = 0.02), hyperlipidemia (HR 2.3; p = 0.01) and previous AT (HR 2; p = 0.04) were independent risk factors for future AT. Similarly, JAK2V617F VAF > 50% (HR 2.4; p = 0.01) and previous VT (HR 2.8; p = 0.005) were confirmed as independent predictors of future VT in the validation cohort. Impact of JAK2V617F VAF > 50% on VT was particularly significant in conventional low-risk patients, both in Florence (HR 10.6, p = 0.005) and Rome cohort (HR 4; p = 0.02). In conclusion, we identified JAK2V617F VAF > 50% as an independent strong predictor of VT, supporting that AT and VT are different entities which might require distinct management.

3.
Medicina (Kaunas) ; 57(12)2021 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-34946271

RESUMO

The occurrence of chronic myeloid leukemia (CML), or other myeloproliferative diseases, after the development of idiopathic thrombocytopenic purpura (ITP) is very rare in the current medical literature. Considering the advances in ITP management, and the wide use of new drugs for ITP and CML, we report an unusual case with this association. Our case report focused on a 64-year-old man with long-standing ITP treated with eltrombopag, who developed hyperleukocytosis during follow-up; after specific laboratory exams, it was diagnosed as CML and he began treatment with imatinib. The treatment with eltrombopag was balanced with imatinib to stabilize his platelet count. Data on bcr-abl and JAK2 transcripts were collected and revealed an optimal response with the achievement of negativization of both molecular signatures. We could demonstrate that treatment with imatinib and eltrombopag was well tolerated and allowed complete molecular remission of CML to be achieved, as well as of ITP.


Assuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva , Púrpura Trombocitopênica Idiopática , Benzoatos , Humanos , Hidrazinas/uso terapêutico , Mesilato de Imatinib/uso terapêutico , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Pirazóis
4.
Nature ; 599(7885): 411-415, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34789903

RESUMO

Propulsion is a critical subsystem of many spacecraft1-4. For efficient propellant usage, electric propulsion systems based on the electrostatic acceleration of ions formed during electron impact ionization of a gas are particularly attractive5,6. At present, xenon is used almost exclusively as an ionizable propellant for space propulsion2-5. However, xenon is rare, it must be stored under high pressure and commercial production is expensive7-9. Here we demonstrate a propulsion system that uses iodine propellant and we present in-orbit results of this new technology. Diatomic iodine is stored as a solid and sublimated at low temperatures. A plasma is then produced with a radio-frequency inductive antenna, and we show that the ionization efficiency is enhanced compared with xenon. Both atomic and molecular iodine ions are accelerated by high-voltage grids to generate thrust, and a highly collimated beam can be produced with substantial iodine dissociation. The propulsion system has been successfully operated in space onboard a small satellite with manoeuvres confirmed using satellite tracking data. We anticipate that these results will accelerate the adoption of alternative propellants within the space industry and demonstrate the potential of iodine for a wide range of space missions. For example, iodine enables substantial system miniaturization and simplification, which provides small satellites and satellite constellations with new capabilities for deployment, collision avoidance, end-of-life disposal and space exploration10-14.

5.
Clin Pharmacol Ther ; 2021 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-34743317

RESUMO

Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by enhanced platelet production and thrombotic complications. The inhibition of platelet cyclooxygenase (COX) activity by the standard once-daily aspirin is mostly incomplete due to accelerated thrombopoiesis. The phase II Aspirin Regimens in EsSential thrombocythemia (ARES) trial has recently compared the efficacy of once- vs. twice- or three-times daily low-dose aspirin in inhibiting platelet thromboxane (TX) A2 production, as reflected by serum (s) TXB2 measurements. The present substudy characterized the determinants of the highly variable response to the standard aspirin 100 mg once-daily regimen in fully compliant patients with ET and the effects of the experimental dosing regimens on response variability. By multivariable analysis, the platelet count (directly) and cytoreductive treatment (inversely) were significantly associated with sTXB2 values in 218 patients with ET. However, the platelet count positively correlated with sTXB2 in patients not being treated with cytoreductive drugs (ρ = 0.51, P < 0.01, n = 84), but not in patients on cytoreduction. Patients in the lowest sTXB2 quartile were older, more often on cytoreductive drugs, had lower platelet count and Janus-Associated Kinase2 (JAK2)-V617F allele frequency as compared with patients in the upper sTXB2 quartiles. After 2 weeks of a twice- or 3-times daily aspirin regimen, the association between the platelet count and sTXB2 became similar in cytoreduced and non-cytoreduced patients. In conclusion, the platelet count appears the strongest determinant of TXA2 inhibition by once-daily low-dose aspirin in ET, with different patterns depending of cytoreductive treatment. More frequent aspirin dosing restores adequate platelet inhibition and reduces interindividual variability, independently of cytoreduction.

6.
Ther Adv Hematol ; 12: 20406207211048361, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34646432

RESUMO

Introduction: In patients with primary immune thrombocytopenia (ITP), a short course of steroids is routinely given as first-line therapy. However, the response is often transient and additional therapy is usually needed. Thrombopoietin receptor agonists (TPO-RAs) are frequently used as second-line therapy, although there is little clinical guidance on the timing of their administration and on tapering/discontinuation of the drug. To provide clinical recommendations, we used the Delphi technique to obtain consensus for statements regarding administration and on tapering/discontinuation of second-line TPO-RAs among a group of Italian clinicians with expertise in management of ITP. Methods: The Delphi process was used to obtain agreement on five statements regarding initiation and on tapering/discontinuation of second-line TPO-RAs. Agreement was considered when 75% of participants approved the statement. Eleven experts participated in the voting. Results: Full consensus was reached for three of the five statements. The experts held that an early switch from corticosteroids to a TPO-RA has the dual advantage of sparing patients from corticosteroid abuse and improve long-term clinical outcomes. All felt that dose reduction of TPO-RAs can be considered in patients with a stable response and platelet count >100 × 109/L that is maintained for at least 6 months in the absence of concomitant treatments, although there was less agreement in patients with a platelet count >50 × 109/L. Near consensus was reached regarding the statement that early treatment with a TPO-RA is associated with an increase in clinically significant partial or complete response. The experts also agreed that optimization of tapering and discontinuation of TPO-RA therapy in selected patients can improve the quality of life. Conclusion: The present consensus can help to provide guidance on use of TPO-RAs in daily practice in patients with ITP. Plain language summary: Second-line administration of thrombopoietin receptor agonists in immune thrombocytopenia There is little guidance on the timing of administration and tapering/discontinuation of thrombopoietin receptor agonists (TPO-RAs) in patients with primary immune thrombocytopenia (ITP).The Delphi technique was used to obtain consensus for five statements.The present consensus among Italian clinicians aims to provide guidance on second-line use of TPO-RAs for patients with ITP in daily practice.

7.
Eur J Haematol ; 2021 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-34716957

RESUMO

The lack of a randomized trial comparing carfilzomib (K) versus elotuzumab (Elo) associated with lenalidomide and dexamethasone (Rd) prompted us to assess the relative usefulness of one triplet over the other. Five independent retrospective cohorts of 883 relapsed/refractory multiple myeloma (RRMM) patients, including 300 EloRd and 583 KRd cases, outside clinical trials, entered this non-randomized comparison. KRd cohort accounted for a higher incidence of younger patients, cases with ≥3 lines of therapy, already exposed to lenalidomide, International Staging System (ISS) stage III, and abnormal lactic dehydrogenase (LDH) level compared with EloRd cohort. Moreover, cytogenetic risk categories, detected in roughly one-third of cases, were equally distributed between the two therapy arms. The probability of CR+VGPR response was significantly higher in KRd (n = 314, 53.9%) than in EloRd patients (n = 111, 37.0%). Likewise, the cumulative incidence function of CR+VGPR, taking into account the competitive risk of death, was significantly higher in KRd arm patients than those in the EloRd arm (p = .003). Moreover, KRd treatment significantly reduced the progression or death risk by 46% in an adjusted multivariate analysis (HR: 0.54, 95% CI 0.42-0.69, p < .0001). Finally, in an adjusted illness-progression/death model, the effect of KRd versus EloRd was of higher magnitude among those who achieved CR+VGPR (-39% hazard ratio reduction, p = .02) than among those who achieved < VGPR (-29% hazard ratio reduction, p = .007). With limitations characteristic to any retrospective analysis, this current clinical practice study's overall results demonstrated potential benefits of KRd therapy compared with EloRd. This observation may help the daily clinical practice.

8.
Clin Cosmet Investig Dermatol ; 14: 1465-1471, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34675583

RESUMO

Objective: To describe the development and validation of the 5-grade photographic IBSA inner upper arm scale. Methods: From 2 real-life pictures, a scale made up of 5 morphed images showing increasing severity of inner upper arm laxity was created. For validation, a set of 50 images (half of which real and the other morphed) was developed and sent to 5 trained physicians in two rounds 30 days apart. Raters' task was to make a selection of each image according to the given scale. Inter-rater and intra-rater reliability were evaluated in both rounds. Results: As to intra-rater reliability, single-rater kappa scores between 0.74 and 1.00 and a global kappa score of 0.846 were observed, while inter-rater agreement was calculated with intra-class correlation coefficient reporting scores higher than 0.91, which indicate excellent reliability. Conclusion: IBSA inner upper arm laxity scale proved to be a validated and reliable tool.

9.
Am J Med Genet A ; 2021 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-34713961

RESUMO

PUS3 encodes the pseudouridylate synthase 3, an enzyme catalyzing the formation of tRNA pseudouridine, which plays a critical role in tRNA structure, function, and stability. Biallelic pathogenic variants of PUS3 have been previously associated with severe intellectual disability, microcephaly, epilepsy, and short stature. We identified a novel homozygous PUS3 frameshift variant in a child with facial dysmorphisms, growth failure, microcephaly, retinal dystrophy, cerebellar hypoplasia, congenital heart defect, and right kidney hypoplasia. This patient further expands the phenotypic spectrum of PUS3-related disorders to include a more severe syndromic presentation.

10.
Skin Res Technol ; 2021 Sep 23.
Artigo em Inglês | MEDLINE | ID: mdl-34555218

RESUMO

BACKGROUND: Both mesenchymal stromal cells (MSCs) and acellular dermal matrices (ADMs) represent fascinating therapeutic tools in the wound healing scenario. Strategies aimed at combining these two treatment modalities are currently under investigation. Moreover, scarcity of quantitative, nondestructive techniques for quality assessment of engineered tissues poses great limitations in regenerative medicine and collagen autofluorescence-based imaging techniques are acquiring great importance in this setting. OBJECTIVE: Our goals were to assess the in vitro interactions between ADSCs and ADMs and to analyze extracellular-matrix production. METHODS: Adipose-derived MSCs (ADSC) were plated on 8-mm punch biopsies of a commercially available ADM (Integra®). Conventional histology with hematoxylin-eosin staining, environmental scanning electron microscopy, and confocal-laser scanning microscopy were used to obtain imaging of ADSC-seeded ADMs. Collagen production by ADSCs was quantified by mean fluorescence intensity (MFI), expressed in terms of positive pixels/field, obtained through ImageJ software processing of three-dimensional projections from confocal scanning images. Control conditions included: fibroblast-seeded ADM, ADSC- and fibroblast-induced scaffolds, and Integra® alone. RESULTS: ADSCs were efficiently seeded on Integra® and were perfectly incorporated in the pores of the scaffold. Collagen production was revealed to be significantly higher when ADSCs were seeded on ADM rather than in all other control conditions. Collagen autofluorescence was efficiently used as a surrogate marker of ECM production. CONCLUSIONS: Combined therapies based on MSCs and collagenic ADMs are promising therapeutic options for chronic wounds. Not only ADSCs can be efficiently seeded on ADMs, but ADMs also seem to potentiate their regenerative properties, as highlightable from fluorescence confocal imaging.

11.
Am J Hematol ; 96(11): 1472-1480, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34424575

RESUMO

The current retrospective study involving a total of 1607 patients was designed to identify clinical and molecular variables that were predictive of inferior myelofibrosis-free survival (MFS) in WHO-defined essential thrombocythemia (ET), utilizing three independent patient cohorts: University of Florence, Italy (n = 718); Mayo Clinic, USA (n = 479) and Policlinico Gemelli, Catholic University, Rome, Italy (n = 410). The Florence patient cohort was first examined to identify independent risk factors for MFS, which included age > 60 years (HR 2.5, 95% CI 1.3-4.9), male sex (2.1, 1.2-3.9), palpable splenomegaly (2.1, 1.2-3.9), CALR 1/1-like or MPL mutation (3.4, 1.9-6.1) and JAK2V617F variant allele frequency > 35% (4.2, 1.6-10.8). Subsequently, an operational molecular risk category was developed and validated in the other two cohorts from Mayo Clinic and Rome: "high molecular risk" category included patients with JAK2V617F VAF >35%, CALR type 1/1-like or MPL mutations; all other driver mutation profiles were assigned to "low molecular risk" category. The former, compared to the latter molecular risk category, displayed significantly higher risk of fibrotic transformation: Florence cohort with respective fibrotic transformation risk rates of 8% vs. 1.2% at 10 years and 33% vs. 8% at 20 years (p < 0.001; HR 6.1; 95% CI 3.2-11.7); Mayo Cohort, 16% vs. 7% at 10 years and 44% vs. 25% at 20 years (p < 0.001; HR 2.5; 95% CI 1.6-4.1); and Rome cohort 7.8% vs. 4.6% at 10 years and 31.2% vs. 7.1% at 20 years (p = 0.007, HR 2.7; 95% CI 1.3-5.8). The present study provides practically useful risk signals for fibrotic transformation in ET and facilitates identification of patients who require close monitoring and appropriate counseling.


Assuntos
Trombocitemia Essencial/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Fibrose , Humanos , Janus Quinase 2/genética , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico , Receptores de Trombopoetina/genética , Estudos Retrospectivos , Trombocitemia Essencial/diagnóstico , Trombocitemia Essencial/genética , Adulto Jovem
14.
Ital J Dermatol Venerol ; 156(2): 244-247, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33960754

RESUMO

Reconstruction of large defects of the upper cheek defects still remains a challenge for the surgeon, who can apply different techniques. We present a new method involving the use of a dermal regeneration template to achieve an improved, faster healing of pedicled buccal fat flap in a 75-year-old woman affected by melanoma of the upper-middle cheek. The tumor involved soft tissue, zygomatic arch and periocular fact. The choice of the surgical technique consisted first in the creation of a buccal fat pad to restore the important lack of tissue over the underlying bones, and then in the position of a dermal acellular matrix (Integra® Dermal Regeneration Template; Integra LifeSciences Holdings Corporation, Plainsboro, NJ, USA). Three weeks later, once the neodermal formation was finished, a split thickness graft was placed. This is a not yet described association that represents a good surgical option for the restoration of large cheek defects that allows good functional and cosmetic result in older patient when minimal surgical invasion and operative duration are necessary because of a patient's general condition. The postoperative course with this surgical technique was regular and a good functional result was achieved. This technique provides an adequate functional coverage, a restoration of soft tissue lacking and an acceptable cosmetic result without ectropion.


Assuntos
Procedimentos Cirúrgicos Reconstrutivos , Tecido Adiposo/transplante , Idoso , Bochecha/cirurgia , Feminino , Humanos , Retalhos Cirúrgicos , Cicatrização
15.
Eur J Cardiothorac Surg ; 60(5): 1074-1081, 2021 11 02.
Artigo em Inglês | MEDLINE | ID: mdl-33963841

RESUMO

OBJECTIVES: The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS: We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS: A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4-20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively). CONCLUSIONS: A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle.

16.
Clin Lymphoma Myeloma Leuk ; 21(8): 536-544, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33985932

RESUMO

This retrospective study is focused on the possible clinical implications of texture analysis-derived PET parameters in patients with smoldering multiple myeloma. Several texture features are significantly associated with progression to symptomatic multiple myeloma and with a shorter time to progression. The results of this study may lead to early identification of patients who could benefit from specific therapies. BACKGROUND: The aim of the study was to determine whether positron emission tomography parameters derived from texture analysis of axial and peripheral skeleton predict progression to symptomatic multiple myeloma (MM) in patients undergoing 18F-​fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) without evidence of focal sites of 18F-FDG uptake. PATIENTS AND METHODS: Patients with smoldering MM who underwent 18F-FDG PET/CT from May 2014 to June 2018 were retrospectively reviewed. Volumes of interest (VOIs) were placed on T5-T7 and L2-L4, iliac crests, and femoral diaphyses. Dedicated software (LIFEx) allowed us to obtain PET-derived first-, second-, and higher order texture features. Possible associations between PET parameters and progression to symptomatic MM were determined. Kaplan-Meier curves allowed to assess time to progression (TTP) based on the PET parameters. RESULTS: Forty-five patients were included: 26 patients (58%) did not meet the criteria for symptomatic MM, but 19 patients (42%) progressed to symptomatic MM. Several texture features extracted from VOIs placed on iliac crests and femoral diaphyses were significantly associated with progression to symptomatic MM and with a shorter TTP (P < .05); conversely, the above-mentioned parameters extracted from VOIs placed on T5-T7 and L2-L4 did not significantly differ among the patients with regard to their progression to symptomatic MM and length of TTP, except for the gray-level zone length matrix-short-zone low-gray-level emphasis and gray-level zone length matrix-low gray-level zone emphasis. Particularly, second- and higher order texture features showed a significant association with the above-mentioned outcomes. CONCLUSION: Texture features derived from PET may be an expression of subtle disease distribution in the axial and peripheral bone marrow.

17.
Front Immunol ; 12: 637512, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34017327

RESUMO

The aim of this review is to update the current status of allogeneic hemopoietic stem cell transplants (HSCT) for patients with myelofibrosis (MF). We have first summarized the issue of an indication for allogeneic HSCT, discussing several prognostic scoring systems, developed to predict the outcome of MF, and therefore to identify patients who will benefit of an allogeneic HSCT. Patients with low risk MF are usually not selected for a transplant, whereas patients with intermediate or high risk MF are eligible. A separate issue, is how to predict the outcome of HSCT: we will outline a clinical molecular myelofibrosis transplant scoring system (MTSS), which predicts overall survival, ranging from 90% for low risk patients, to 20% for very high risk patients. We will also discuss transfusion burden and spleen size, as predictors of transplant outcome. The choice of a transplant platform including the conditioning regimen, the stem cell source and GvHD prophylaxis, are crucial for a successful program in MF, and will be outlined. Complications such as poor graft function, graft failure, GvHD and relapse of the disease, will also be reviewed. Finally we discuss monitoring the disease after HSCT with donor chimerism, driver mutations and hematologic data. We have made an effort to make this review as comprehensive and up to date as possible, and we hope it will provide some useful data for the clinicians.


Assuntos
Rejeição de Enxerto/prevenção & controle , Reação Enxerto-Hospedeiro/imunologia , Transplante de Células-Tronco Hematopoéticas , Mielofibrose Primária/terapia , Animais , Quimerismo , Humanos , Tolerância Imunológica , Condicionamento Pré-Transplante , Transplante Homólogo
18.
Ann Ital Chir ; 102021 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-33843723

RESUMO

We report an uncommon case of idiopathic acute chylous peritonitis mimicking an acute appendicitis in a 30-year-old female patient with a 2-day history of abdominal pain, nausea and vomiting. Chylous ascites is a rare form of ascites characterized by the presence of a milky fluid rich in triglycerides. It occurs as a result of a damage to the lymphatic system due to trauma or other benign and malignant pathologies. Although the most common clinical presentation is progressive painless abdominal distension, less frequently it can cause acute abdomen symptoms. The management is based on identifying and treating the underlying pathology. Aspiration of the fluid and drainage are the only therapy required if a clinically diagnosis cannot be made. Surgical laparoscopic exploration is necessary to make a diagnosis and to treat effectively acute abdomen cases.In the absence of a significant determining pathology, we talk about idiopathic chylous peritonitis. KEY WORDS: Chylous ascites, peritonitis, laparoscopy.


Assuntos
Abdome Agudo/etiologia , Apendicite/diagnóstico , Ascite Quilosa/diagnóstico , Doença Aguda , Adulto , Apendicite/cirurgia , Apêndice/patologia , Apêndice/cirurgia , Ascite Quilosa/etiologia , Ascite Quilosa/cirurgia , Diagnóstico Diferencial , Drenagem , Feminino , Humanos , Irrigação Terapêutica
19.
Ann Med Surg (Lond) ; 64: 102244, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33898024

RESUMO

Background: POPF derives from the pancreatic stump, which follows pancreatic resection and the pancreatoenteric anastomosis following pancreaticoduodenectomy. Since 1978 sealants have been used in pancreatic surgery to prevent pancreatic fistula after resection of the pancreatic head and tail or for the management of trauma and the treatment of low-output pancreatic fistula. Different types of fibrin sealants have been evaluated for their potential to reduce the occurrence of POPF. Methods: A systematic search of the electronic literature was performed using PubMed, Cochrane Library, and Scopus databases to obtain access to all publications, especially clinical trials, randomised controlled trials, and systematic reviews concerning fibrin sealants pancreatic surgery. Searching for "fibrin sealants pancreas," we found a total of 73 results on Pubmed, 61 on Scopus, and 14 on Cochrane Library (148 total results). Results: Eighteen studies were found on literature, following the criteria already described, concerning the use of fibrin sealants in pancreatic surgery. All articles described were published in the period between 1989 and 2019.Most of these were single centre studies. A total of 1032 patients were enrolled in this review. In the studies, sealants were used to reinforce pancreatic anastomoses and for the occlusion of the main pancreatic duct. Conclusion: CR-POPF is a fearful complication of pancreatic surgery; among the possible solutions to reduce the risk of onset, sealants were used on the pancreatic stump; today the sealants should be considered such as an option to reduce the CR-POPF, but the routine use in clinical practice has to be validated.

20.
Blood ; 138(7): 571-583, 2021 08 19.
Artigo em Inglês | MEDLINE | ID: mdl-33889952

RESUMO

The efficacy and safety of thrombopoietin receptor agonists (TRAs) in older patients with primary immune thrombocytopenia (ITP) are unknown. We investigated TRA response and switch, thrombotic/hemorrhagic risk, and sustained responses off-treatment (SROTs) in 384 patients with ITP aged ≥60 years. After 3 months, 82.5% and 74.3% of eltrombopag- and romiplostim-treated patients, respectively, achieved a response; 66.7% maintained the response (median follow-up, 2.7 years). Eighty-five (22.2%) patients switched to the alternative TRA; although no cross-toxicity was observed, 83.3% of resistant patients had a response after the switch. Thirty-four major thromboses (3 fatal) and 14 major hemorrhages (none fatal) occurred in 18 and 10 patients, respectively, while on TRAs and were associated with thrombosis history (subdistribution hazard ratio, 2.04, P = .05) and platelet count <20 × 109/L (subdistribution hazard ratio, 1.69; P = .04), respectively, at TRA start. A recurrent event occurred in 15.6% of patients surviving thrombosis, in all cases but 1 during persisting TRA treatment (incidence rate, 7.7 per 100 patient-years). All recurrences occurred in the absence of adequate antithrombotic secondary prophylaxis. Sixty-two (16.5%) responding patients discontinued TRAs; 53 (13.8%) patients maintained SROTs, which were associated with TRA discontinuation in complete response (P < .001). Very old age (≥75 years; 41.1%) was associated with the more frequent start of TRAs in the persistent/acute phase but not with response or thrombotic/hemorrhagic risk. TRAs are effective in older patients with ITP, with no fatal hemorrhages and with SROTs in a significant portion of patients. Caution is warranted in patients with a history of thrombosis, and a careful risk/benefit balance should be considered.

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