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1.
Tunis Med ; 102(6): 343-347, 2024 Jun 05.
Artigo em Francês | MEDLINE | ID: mdl-38864197

RESUMO

INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma exchange (PE) must be started immediately and until a response is obtained. AIM: Evaluate PE in terms of responses and complications in the treatment of TTP. METHODS: This was a monocentric, descriptive, retrospective study including patients in whom TTP was diagnosed and treated with plasmapheresis in the clinical hematology department at Aziza Othmana Hospital, between January 2010 and December 2020. RESULTS: Our study included 26 patients. PE was initiated within a median of 1 day. The rhythm of exchanges was daily in 22 patients. Twenty PE-related complications were noted, hypocalcemia being the most frequent (30%). CR was achieved in 15 patients after PE alone. Nine patients were refractory, and six received 2nd-line treatment, with CR achieved in five patients. Relapse was noted in six patients (40%). They were treated by PE and only one patient received rituximab. Four patients had a response. The overall response rate was 69% and overall mortality was 30%. OS at 2 years was 68,3% and RFS was 84,4%. Factors associated with the achievement of CR were the fall in LDH at D5 of treatment (p=0,027,OR=0,59 ;IC 95%[0,32-1,08]) and the daily rhythm of PE (p=0,005, OR=0,35; IC 95%[0,14-0,91]). CONCLUSION: Our results were comparable to those of the literature, but the rate of refractory disease was higher. Rituximab may enhance our results.


Assuntos
Troca Plasmática , Púrpura Trombocitopênica Trombótica , Humanos , Púrpura Trombocitopênica Trombótica/terapia , Púrpura Trombocitopênica Trombótica/diagnóstico , Estudos Retrospectivos , Troca Plasmática/métodos , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Adulto Jovem , Resultado do Tratamento , Idoso , Recidiva , Plasmaferese/métodos , Adolescente , Rituximab/uso terapêutico , Rituximab/administração & dosagem
2.
J Dermatolog Treat ; 35(1): 2350229, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38880493

RESUMO

BACKGROUND/PURPOSE: Amidst the emergence of new therapeutic options, traditional therapeutic plasmapheresis (TPE) used in diseases involving a toxic substance in the plasma, remains a viable alternative for cases of recalcitrant solar urticaria (SU). We emphasize the importance of documenting successful experience with repeated plasmapheresis to increase awareness amongst physicians and dermatologists regarding this effective treatment option. MATERIAL AND METHOD: We reported a case of recalcitrant SU that had not responded to a combination of H1-antihistamines, immunosuppressants, omalizumab and intravenous immunoglobulin. We introduced serial TPE, which involved two consecutive days of procedures for each course was introduced. We detailed the regimen and highlighted the clinical and objective benefits observed with multiple treatments. Additionally, we compared this to other plasmapheresis regimens and their treatment responses previously reported for solar urticaria. RESULTS: Our patient underwent serial TPE, totaling 42 procedures over five years. Following the last TPE session, phototesting showed a sustained prolongation of minimal urticating doses (MUDS), which exceeded the maximum tested doses across nearly all ultraviolet (UV) and visible light ranges, with the exception of the two short ultraviolet B (UVB) wavelengths. MUDs increased to 25 from 6 mj/cm2 at 307.5± 5nm, and to 500 from 15 mj/cm2 at 320 ± 10nm, before the initial TPE. In our review, we included five articles covering eight SU patients who received TPE. Of these, the five patients with positive intradermal tests responded particularly well immediately after treatment. However, the condition relapsed within two weeks in one patient and within two months in another. In contrast, the other three patients with negative intradermal tests, showed no significant benefits from the treatment. No serious side effects from TPE were reported amongst the patients. CONCLUSIONS: This review underscores the efficacy of serial plasmapheresis procedures in treating refractory cases of SU, high3lighting the robust results observed.


Assuntos
Plasmaferese , Urticária , Humanos , Urticária/terapia , Resultado do Tratamento , Feminino , Luz Solar/efeitos adversos , Masculino , Transtornos de Fotossensibilidade/terapia , Adulto , Pessoa de Meia-Idade , Urticária Solar
3.
J Clin Apher ; 39(3): e22133, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38881050

RESUMO

Bullous pemphigoid is the most common autoimmune blistering disease induced by autoantibodies against basement membrane anchoring proteins (anti-BP-180 and anti-BP-230). The disease generally appears after the age of 70 and is associated with a 23.5% 1-year mortality, especially in diabetics, or in the presence of ischemic heart disease and high anti-BP-180. Treatment starts with topical steroids but some patients may require oral steroids and systemic immunosuppression. We, hereby, discuss a diabetic patient on chronic hemodialysis, with severely relapsed bullous pemphigoid under biotherapy with omalizumab, who was successfully treated with five sessions of double filtration plasmapheresis, thus avoiding the need for systemic steroids.


Assuntos
Penfigoide Bolhoso , Plasmaferese , Diálise Renal , Humanos , Penfigoide Bolhoso/terapia , Plasmaferese/métodos , Masculino , Idoso , Feminino
4.
Artigo em Russo | MEDLINE | ID: mdl-38884442

RESUMO

Presented clinical observation of anti-NMDA-receptor encephalitis, which was first described in 2007, is rare and to date has not been sufficiently studied. The disease often manifests with psychopathological symptoms and catatonia, so patients are transferred into a mental healthcare institution and often require intensive care and resuscitation, due to the development of life-threatening respiratory and hemodynamic disorders. Diagnosis is based on detection of autoantibodies to the NR1- and NR2 subunits of the glutamate NMDA receptor in blood serum and cerebrospinal fluid. Pathogenesis-based therapy includes the administration of glucocorticoids and intravenous immunoglobulins, plasmapheresis, as well as the introduction of monoclonal antibodies in also used, and in severe cases, cytostatics are prescribed. The widespread comorbidity of anti-NMDA receptor encephalitis with ovarian neoplasms in women (up to 60%) requires appropriate diagnosis and early removal of ovarian neoplasms when they are detected. With timely diagnosis and adequate treatment strategies, the outcome of this rare disorder is usually positive.


Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato , Autoanticorpos , Neoplasias Ovarianas , Receptores de N-Metil-D-Aspartato , Humanos , Feminino , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Receptores de N-Metil-D-Aspartato/imunologia , Autoanticorpos/sangue , Autoanticorpos/líquido cefalorraquidiano , Adulto , Imunoglobulinas Intravenosas/uso terapêutico , Transtornos Mentais/etiologia , Transtornos Mentais/diagnóstico , Plasmaferese , Catatonia/etiologia , Catatonia/diagnóstico
5.
Front Immunol ; 15: 1368487, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38846936

RESUMO

Background: Contactin-1 (CNTN1) antibody-positive nodopathy is rare and exhibits distinct clinical symptoms such as tremors and ataxia. However, the mechanisms of these symptoms and the characteristics of the cerebral spinal fluid (CSF) remain unknown. Case presentation: Here, we report a case of recurrent CNTN1 antibody-positive nodopathy. Initially, a 45-year-old woman experiencing numbness in the upper limbs and weakness in the lower limbs was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Eleven years later, her symptoms worsened, and she began to experience tremors and ataxia. Tests for serum CNTN1, GT1a, and GQ1b antibodies returned positive. Subsequently, she was diagnosed with CNTN1 antibody-positive nodopathy and underwent plasmapheresis therapy, although the treatment's efficacy was limited. To gain a deeper understanding of the disease, we conducted a comprehensive literature review, identifying 52 cases of CNTN1 antibody-positive nodopathy to date, with a tremor prevalence of 26.9%. Additionally, we found that the average CSF protein level in CNTN1 antibody-positive nodopathy was 2.57 g/L, with 87% of patients exhibiting a CSF protein level above 1.5 g/L. Conclusion: We present a rare case of recurrent CNTN1 antibody-positive nodopathy. Our findings indicate a high prevalence of tremor (26.9%) and elevated CSF protein levels among patients with CNTN1 antibody-positive nodopathy.


Assuntos
Autoanticorpos , Contactina 1 , Humanos , Feminino , Pessoa de Meia-Idade , Autoanticorpos/sangue , Autoanticorpos/imunologia , Contactina 1/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Recidiva , Tremor/imunologia , Tremor/etiologia , Plasmaferese
6.
Ther Drug Monit ; 46(3): 281-284, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38723114

RESUMO

ABSTRACT: This is a case description of a patient with bipolar disorder undergoing lithium therapy who received plasmapheresis for neuromyelitis optica spectrum disorder. Plasmapheresis resulted in lower and subtherapeutic serum lithium levels. Using therapeutic drug monitoring, a dose escalation of 80% was necessary to maintain therapeutic serum lithium levels. This underscores the importance of individualized therapy through therapeutic drug monitoring.


Assuntos
Transtorno Bipolar , Monitoramento de Medicamentos , Neuromielite Óptica , Plasmaferese , Humanos , Plasmaferese/métodos , Transtorno Bipolar/terapia , Transtorno Bipolar/sangue , Neuromielite Óptica/terapia , Neuromielite Óptica/sangue , Monitoramento de Medicamentos/métodos , Feminino , Lítio/sangue , Lítio/uso terapêutico , Unidades de Terapia Intensiva , Antimaníacos/uso terapêutico , Antimaníacos/sangue , Adulto , Pessoa de Meia-Idade
7.
Ren Fail ; 46(1): 2356708, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38803220

RESUMO

As no unified treatment protocol or evidence yet exists for plasmapheresis without plasma, this study explored the outcomes of using 4% human albumin (ALB) solution as a replacement solution in patients undergoing plasma exchange for multiple myeloma (MM) patients with acute kidney injury (AKI). This study was prospectively registered (ChiCTR2000030640 and NCT05251896). Bortezomib-based chemotherapy plus therapeutic plasmapheresis (TPP) with 4% human ALB solution was assessed for three years in patients with MM aged >18 years, with AKI according to the Kidney Disease Improving Global Outcomes criteria, and without previous renal impairment from other causes. The primary endpoints were changes in renal function over 18 weeks and survival outcomes at 36 months. The secondary endpoints were the incidence of adverse reactions and symptom improvement. Among the 119 patients included in the analysis, 108 experienced renal reactions. The M protein (absolute changes: median -12.12%, interquartile ranges (IQRs) -18.62 to -5.626) and creatine (median -46.91 µmol/L, IQR -64.70 to -29.12) levels decreased, whereas the estimated glomerular filtration rate (eGFR) increased (median 20.66 mL/(min·1.73 m2), IQR 16.03-25.29). Regarding patient survival, 68.1% and 35.3% of patients survived for >12 and >36 months, respectively. The three symptoms with the greatest relief were urine foam, poor appetite, and blurred vision. All 11 patients (7.6%) who experienced mild adverse reactions achieved remission. In conclusion, in MM patients with AKI, plasma-free plasmapheresis with 4% human ALB solution and bortezomib-based chemotherapy effectively alleviated light chain damage to kidney function while improving patient quality of life.


Assuntos
Injúria Renal Aguda , Bortezomib , Taxa de Filtração Glomerular , Mieloma Múltiplo , Plasmaferese , Humanos , Mieloma Múltiplo/complicações , Mieloma Múltiplo/terapia , Injúria Renal Aguda/terapia , Injúria Renal Aguda/etiologia , Plasmaferese/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Prospectivos , Idoso , Bortezomib/administração & dosagem , Bortezomib/uso terapêutico , Estudo de Prova de Conceito , Albumina Sérica Humana/análise , Albumina Sérica Humana/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Resultado do Tratamento , Adulto , Terapia Combinada , Proteínas do Mieloma
8.
Medicine (Baltimore) ; 103(19): e38103, 2024 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-38728448

RESUMO

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by reduced activity of the von Willebrand factor-cleaving protease (ADAMTS13), which can be life-threatening. The patient reported in this case study also had concurrent Sjögren syndrome and renal impairment, presenting multiple symptoms and posing a great challenge in treatment. PATIENT CONCERNS: A 25-year-old woman in the postpartum period visited the hospital due to indifference in consciousness for more than 1 day following cesarean section 8 days prior. DIAGNOSIS: Notable decreases were observed in platelets, hemoglobin, creatinine, and ADAMTS13 levels. After a consultative examination by an ophthalmologist, she was diagnosed with retinal hemorrhage in the right eye and dry eye syndrome in both eyes. INTERVENTIONS: Having been diagnosed with TTP with Sjögren syndrome and renal impairment, she received repeated treatments with plasmapheresis combined with rituximab. OUTCOMES: Following treatment and during the follow-up period, the patient's platelet counts and bleeding symptoms significantly improved. LESSONS: TTP has a high mortality rate, and when combined with Sjögren syndrome and renal impairment, it poses an even greater challenge in treatment. However, after administering standard plasmapheresis combined with rituximab treatment, the treatment outcome is favorable.


Assuntos
Plasmaferese , Púrpura Trombocitopênica Trombótica , Rituximab , Síndrome de Sjogren , Humanos , Feminino , Síndrome de Sjogren/complicações , Síndrome de Sjogren/terapia , Plasmaferese/métodos , Adulto , Púrpura Trombocitopênica Trombótica/terapia , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Rituximab/uso terapêutico , Rituximab/administração & dosagem , Terapia Combinada , Insuficiência Renal/terapia , Insuficiência Renal/etiologia , Fatores Imunológicos/uso terapêutico , Fatores Imunológicos/administração & dosagem
9.
Pregnancy Hypertens ; 36: 101128, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38728925

RESUMO

OBJECTIVES: Preeclampsia (PE) is a major cause of maternal and fetal mortality, and preterm birth. Previous studies indicate that lipid-apheresis may prolong pregnancy, namely heparin-mediated extracorporeal LDL-precipitation (HELP)- and dextran sulfate cellulose (DSC)-apheresis. We now report on double membrane plasmapheresis (DFPP) in early-onset preeclampsia (eoPE). STUDY DESIGN: Open pilot study assessing the prolongation of pregnancy in PE by lipoprotein-apheresis (DRKS00004527). Two women with eoPE were treated by DFPP and compared to a historical cohort of 6 patients with eoPE treated by HELP-apheresis (NCT01967355). MAIN OUTCOME MEASURES: Clinical outcome of mothers and babies and prolongation of pregnancies (time of admission to birth). RESULTS: Patient 1 (33y; 22 + 5/7GW) received 4 DFPP. Delivery day 19; birthweight 270 g; weight at discharge 2134 g on day 132. Patient 2 (35y; 21 + 4/7GW) received 2 DFPP. Delivery day 19; birthweight 465 g; weight at discharge 2540 g on day 104. DFPP was well tolerated by both patients. CONCLUSIONS: DFPP proved to be save and pregnancies remained stable as long as 19 days. Although babies were born very preterm both babies could finally be dismissed from hospital. No relevant clinical differences between DFPP and HELP-apheresis could be observed. Therefore, DFPP may extend the range of available apheresis techniques to prolong pregnancies in early-onset preeclampsia. However, further studies are necessary to gain more information. REGISTER: (DRKS00004527).


Assuntos
Remoção de Componentes Sanguíneos , Heparina , Plasmaferese , Pré-Eclâmpsia , Humanos , Feminino , Gravidez , Pré-Eclâmpsia/terapia , Plasmaferese/métodos , Adulto , Heparina/administração & dosagem , Remoção de Componentes Sanguíneos/métodos , Projetos Piloto , Lipoproteínas LDL/sangue , Resultado do Tratamento , Recém-Nascido
10.
J Clin Neurosci ; 125: 76-82, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38759351

RESUMO

BACKGROUND: Intravenous immunoglobulin (IVIg) and plasmapheresis (PLEX) are recommended in moderate to severe Guillain-Barré Syndrome (GBS), but there is paucity of studies evaluating its effect on nerve conduction studies (NCS). We report the effect of IVIg and PLEX on the NCS parameters and clinical outcomes compared to natural course (NC) of GBS patients. METHOD: Moderate to severe GBS patients were included based on clinical, cerebrospinal fluid, and NCS finding. Six motor and sensory nerves were evaluated at admission, one month and 3 months, and NCS subtyping was done. Axonal and demyelination burden in motor nerves and early reversible conduction block (ERCB) were noted. Patients receiving IVIg, PLEX or on NC were noted. Outcome was defined at 3 months into complete, partial and poor using a 0-6 GBS Disability Scale (GBSDS). RESULT: Seventy-two patients were included, whose median age was 36 years and 22(30.6 %) were females. 44 patients received IVIg, 9 PLEX and 19 were in NC, and they had comparable peak disability. AIDP was the dominant subtype at admission (58.3 %), which remained so at 3 months (50 %). The shift of subtypes was the highest from the equivocal group followed by AMAN and the least from AIDP. IVIg and PLEX group had more reduction in axonal burden and had ERCB compared to NC. 33(44 %) patients had complete recovery, and 40(55.5 %) patients had concordance in clinical and neurophysiological outcome. CONCLUSION: Transition of GBS subtype may occur at follow-up from all the subtypes, the highest from the equivocal and the lowest from the AIDP group. IVIg/PLEX treatment may help in reducing conduction block and axonal burden.


Assuntos
Síndrome de Guillain-Barré , Imunoglobulinas Intravenosas , Condução Nervosa , Plasmaferese , Humanos , Síndrome de Guillain-Barré/terapia , Síndrome de Guillain-Barré/fisiopatologia , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas Intravenosas/administração & dosagem , Feminino , Masculino , Adulto , Plasmaferese/métodos , Condução Nervosa/fisiologia , Condução Nervosa/efeitos dos fármacos , Pessoa de Meia-Idade , Adulto Jovem , Resultado do Tratamento , Fatores Imunológicos/uso terapêutico , Fatores Imunológicos/administração & dosagem , Adolescente
11.
J Clin Apher ; 39(3): e22112, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38634442

RESUMO

INTRODUCTION: Autoimmune encephalitis (AE) comprises a heterogeneous group of autoantibody-mediated disorders targeting the brain parenchyma. Therapeutic plasma exchange (TPE), one of several first-line therapies for AE, is often initiated when AE is suspected, albeit prior to an established diagnosis. We sought to characterize the role of TPE in the treatment of suspected AE. METHODS: A single-center, retrospective analysis was performed of adults (≥18 years) who underwent at least one TPE procedure for "suspected AE." The following parameters were extracted and evaluated descriptively: clinicopathologic characteristics, treatment course, TPE-related adverse events, outcomes (e.g., modified Rankin scale [mRS]), and diagnosis once investigation was complete. RESULTS: A total of 37 patients (median age 56 years, range 28-77 years, 62.2% male) were evaluated. Autoimmune antibody testing was positive in serum for 43.2% (n = 16) and cerebrospinal fluid for 29.7% (n = 11). Patients underwent a median of five TPE procedures (range 3-16), with 97.3% (n = 36) via a central line and 21.6% (n = 8) requiring at least one unit of plasma as replacement fluid. Fifteen patients (40.5%) experienced at least one TPE-related adverse event. Compared with mRS at admission, the mRS at discharge was improved in 21.6% (n = 8), unchanged in 59.5% (n = 22), or worse in 18.9% (n = 7). Final diagnosis of AE was determined to be definite in 48.6% (n = 18), probable in 8.1% (n = 3) and possible in 27.0% (n = 10). Six (16.2%) patients were ultimately determined to have an alternate etiology. CONCLUSION: Empiric TPE for suspected AE is generally well-tolerated. However, its efficacy remains uncertain in the absence of controlled trials, particularly in the setting of seronegative disease.


Assuntos
Doenças Autoimunes do Sistema Nervoso , Encefalite , Doença de Hashimoto , Troca Plasmática , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Feminino , Troca Plasmática/métodos , Estudos Retrospectivos , Plasmaferese , Autoanticorpos
12.
Eur J Anaesthesiol ; 41(7): 530-534, 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38586903

RESUMO

Since 2019 when a cluster of cases with acute respiratory distress syndrome (ARDS) associated with e-cigarettes in the United States was reported, there have been increasing numbers of reports. Electronic-cigarette or Vaping Use-associated Lung Injury (EVALI) represents a recent entity of respiratory clinical syndromes, primarily in young adults. We report a previously healthy 16-year-old boy who developed severe ARDS following a brief nonspecific prodromal phase after excessive consumption of e-cigarettes. Despite maximum intensive care therapy, including several weeks of venovenous extracorporeal membrane oxygenation, plasmapheresis and repeated administration of immunoglobulins seemed the only way to achieve therapeutic success. Although many case reports have been published, to our knowledge, there are none to date on the therapeutic use of plasmaphoresis in severe EVALI. This case highlights the clinical features of EVALI and the diagnostic dilemma that can arise with EVALI occurring against the background of an expired SARS-CoV-2 infection, with a paediatric inflammatory syndrome (PIMS) as differential diagnosis. EVALI is a diagnosis of exclusion, and the medical history of vaping and e-cigarette use can provide valuable clues. Ethical approval for this case report (protocol number 23-145 RS) was provided by the Ethical Committee of the Department of Medicine, Philipps-Universität Marburg, Germany on 13 th of June 2023. Written informed consent to publish this case and the associated images was obtained from the patient and his mother.


Assuntos
Plasmaferese , Vaping , Humanos , Masculino , Adolescente , Plasmaferese/métodos , Vaping/efeitos adversos , Síndrome do Desconforto Respiratório/terapia , Síndrome do Desconforto Respiratório/etiologia , Síndrome do Desconforto Respiratório/diagnóstico , COVID-19/terapia , COVID-19/diagnóstico , Oxigenação por Membrana Extracorpórea , Sistemas Eletrônicos de Liberação de Nicotina , Resultado do Tratamento
13.
J Pediatr Gastroenterol Nutr ; 78(6): 1364-1373, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38623928

RESUMO

OBJECTIVES: Paediatric acute liver failure (PALF) is a life-threatening disease. Management aims to support hepatic regeneration or to bridge to liver transplantation. High-volume plasmapheresis (HVP) removes protein-bound substances, alleviates inflammation, and improves survival in adult acute liver failure. However, experience with HVP in PALF is limited. Aim of this study is to report on feasibility, safety, efficacy and outcomes of HVP in PALF. METHODS: Retrospective observational study in children with PALF. HVP was performed upon identification of negative prognostic indicators, in toxic aetiology or multiorgan failure (MOF). Exchanged volume with fresh-frozen plasma corresponded to 1.5-2.0 times the patient's estimated plasma volume. One daily cycle was performed until the patient met criteria for discontinuation, that is, liver regeneration, liver transplantation, or death. RESULTS: Twenty-two children with PALF (body weight 2.5-106 kg) received 1-7 HVP cycles. No bleeding or procedure-related mortality occurred. Alkalosis, hypothermia and reduction in platelets were observed. Haemolysis led to HVP termination in one infant. Seven children (32%) survived with their native livers, 13 patients (59%) underwent liver transplantation. Two infants died due to MOF. Overall survival was 86%. International normalization ratio (INR), alanine aminotransaminases (ALT), bilirubin and inotropic support were reduced significantly (p < 0.05) after the first HVP-cycle (median): INR 2.85 versus 1.5; ALT 1280 versus 434 U/L; bilirubin 12.7 versus 6.7 mg/dL; norepinephrine dosage 0.083 versus 0.009 µg/kg/min. Median soluble-interleukin-2-receptor dropped significantly following HVP (n = 7): 2407 versus 950 U/mL (p < 0.02). CONCLUSIONS: HVP in PALF is feasible, safe, improves markers of liver failure and inflammation and is associated with lowering inotropic support. Prospective and controlled studies are required to confirm efficacy of HVP in PALF.


Assuntos
Falência Hepática Aguda , Transplante de Fígado , Plasmaferese , Humanos , Plasmaferese/métodos , Estudos Retrospectivos , Falência Hepática Aguda/terapia , Falência Hepática Aguda/mortalidade , Masculino , Criança , Feminino , Pré-Escolar , Lactente , Adolescente , Resultado do Tratamento , Estudos de Viabilidade
14.
Curr Opin Pulm Med ; 30(4): 391-397, 2024 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-38656281

RESUMO

PURPOSE OF REVIEW: To review what is currently known about the pathogenesis, diagnosis, treatment, and prevention of acute rejection (AR) in lung transplantation. RECENT FINDINGS: Epigenomic and transcriptomic methods are gaining traction as tools for earlier detection of AR, which still remains primarily a histopathologic diagnosis. SUMMARY: Acute rejection is a common cause of early posttransplant lung graft dysfunction and increases the risk of chronic rejection. Detection and diagnosis of AR is primarily based on histopathology, but noninvasive molecular methods are undergoing investigation. Two subtypes of AR exist: acute cellular rejection (ACR) and antibody-mediated rejection (AMR). Both can have varied clinical presentation, ranging from asymptomatic to fulminant ARDS, and can present simultaneously. Diagnosis of ACR requires transbronchial biopsy; AMR requires the additional measuring of circulating donor-specific antibody (DSA) levels. First-line treatment in ACR is increased immunosuppression (pulse-dose or tapered dose glucocorticoids); refractory cases may need antibody-based lymphodepletion therapy. First line treatment in AMR focuses on circulating DSA removal with B and plasma cell depletion; plasmapheresis, intravenous human immunoglobulin (IVIG), bortezomib, and rituximab are often employed.


Assuntos
Rejeição de Enxerto , Transplante de Pulmão , Humanos , Transplante de Pulmão/efeitos adversos , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/diagnóstico , Imunossupressores/uso terapêutico , Doença Aguda , Plasmaferese , Biópsia
15.
J Med Case Rep ; 18(1): 193, 2024 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-38553729

RESUMO

BACKGROUND: Plasmapheresis represent an alternative therapeutic option for hyperthyroidism with thyroid storm or refractory cases. It provides a rapid decrease in plasma thyroid hormones and anti-thyroid antibodies. The aim of this paper was to report our single center's experience in managing particular situations of hyperthyroidism using apheresis. CASES PRESENTATION: The following case series describes three young African patients (two females, one male) aged 29, 37, and 25 years old, respectively, with Graves' disease who presented with drug ineffectiveness, drug-induced agranulocytosis, and thyroid storm with multi-organ failure. The three patients underwent plasmapheresis sessions leading to effective decline of thyroid hormone levels and offering a window for processing total thyroidectomy. DISCUSSION/CONCLUSION: The standard management of thyrotoxicosis and thyroid storm was usually codified by the concomitant use of antithyroid medication, iodine, beta-blockers, and corticosteroids. This medical preparation can be effective in most cases. However, drug toxicity or ineffectiveness can limit the use of such therapeutics. Our paper supports the efficiency and safety of therapeutic plasma exchange in the preoperative management of thyrotoxicosis.


Assuntos
Doença de Graves , Crise Tireóidea , Tireotoxicose , Feminino , Humanos , Masculino , Antitireóideos/uso terapêutico , Doença de Graves/complicações , Plasmaferese , Crise Tireóidea/complicações , Hormônios Tireóideos , Tireotoxicose/terapia , Adulto
16.
Trials ; 25(1): 175, 2024 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-38468338

RESUMO

BACKGROUND: The demand for plasma products is growing, necessitating an increase in plasma collection by plasmapheresis. While the 20th edition of the European Guidelines permits plasma donors in Europe to donate with 96-h donation intervals, the potential short- and long-term consequences of high-frequency plasma donations on donor health remain unknown. This study aims to measure the effect of plasma donation frequency on plasma protein composition, including total serum protein (TSP) and immunoglobulin G (IgG), in Norwegian male blood donors. METHODS: This randomized controlled trial (RCT) included 120 male blood donors who were randomized into two intervention groups and one control group: high-frequency plasma donors (HFPDs) who donated 650 mL of plasma 3 times every 2 weeks, whereas regular-frequency plasma donors (RFPDs) who donated 650 mL of plasma 1 time every 2 weeks. The control group consisted of whole blood donors. The primary outcomes are the concentrations of TSP and IgG. DISCUSSION: The findings from this study may have implications for recommendations related to donor health and plasma donation frequencies and may contribute to supporting the strategic independence of plasma products in Norway and Europe without compromising donor health. TRIAL REGISTRATION: ClinicalTrials.gov: NCT05179200 . Registered December 20th, 2021.


Assuntos
Doadores de Sangue , Plasmaferese , Masculino , Humanos , Plasmaferese/métodos , Imunoglobulina G , Tempo , Europa (Continente) , Ensaios Clínicos Controlados Aleatórios como Assunto
18.
BMJ Case Rep ; 17(3)2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38442982

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening form of antiphospholipid syndrome characterised by diffuse arterial and venous thrombosis, in the presence of positive antiphospholipid antibodies. The multiple sites of thrombosis in small, medium and large vessels progress to multiorgan failure, accounting for the high mortality rate associated with CAPS. Unregulated complement activation is increasingly recognised as critical to the pathogenesis of CAPS. Early diagnosis is essential to initiate prompt life-saving treatment with the triple therapy of anticoagulation, immunosuppression and either plasmapheresis or intravenous immunoglobulin. Among other immunosuppressive agents, eculizumab, a complement inhibitor has demonstrated efficacy in treatment-resistant cases.We report an instructive case of a woman presenting with both clinical and laboratory findings consistent with primary CAPS, resistant to initial treatment and responsive to eculizumab, with emphasis on genetic testing and implications for future therapy.


Assuntos
Síndrome Antifosfolipídica , Feminino , Humanos , Síndrome Antifosfolipídica/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Antifosfolipídeos , Plasmaferese
19.
Mult Scler Relat Disord ; 85: 105521, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38457882

RESUMO

PURPOSE: To compare the efficacy of treatment of optic neuritis (ON) with corticosteroids (CTC) alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). DESIGN: After an episode of ON, although visual recovery is usually good, some patients may have significant visual sequelae. While the efficacy of first-line CTC is now indisputable, there is no consensus on the nature of second-line treatment. To date, no systematic review has compared the efficacy of treatment of ON with CTC alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). A meta-analysis is needed to compare the efficacy of PLP and IVIG in steroid-resistant ON. METHODS: This systematic review included all studies comparing at least two of the three treatments for steroid-resistant ON (CTC alone, CTC+PLP, and CTC+IVIG). From all articles published on PubMed between January 2000 and June 2022, two independent ophthalmologists selected studies of interest using the PRISMA method. Methodology, patient characteristics, and outcomes were identified. A network metaanalysis was then performed to compare the efficacy of the three treatments. RESULTS: Six comparative studies were included, representing 209 patients. The percentage of significant visual recovery after CTC alone, CTC+PLP, and CTC+IVIG in the acute treatment of steroid-resistant ON was 30 %, 45 %, and 77 %, respectively. Comparison of CTC+IVIG vs CTC alone, CTC+PLP vs CTC only, and CTC+PLP vs CTC+IVIG yielded odds ratios of 12.81, 2.47, and 0.19 respectively. CONCLUSION: Treatment of steroid-resistant ON with CTC+PLP or CTC+IVIG is more effective than treatment with CTC alone. Although no study has directly compared the two treatments, IVIG may be more effective than PLP.


Assuntos
Corticosteroides , Imunoglobulinas Intravenosas , Metanálise em Rede , Neurite Óptica , Plasmaferese , Neurite Óptica/tratamento farmacológico , Neurite Óptica/terapia , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Corticosteroides/uso terapêutico , Corticosteroides/administração & dosagem , Plasmaferese/métodos , Terapia Combinada , Fatores Imunológicos/administração & dosagem , Doenças Desmielinizantes/tratamento farmacológico , Doenças Desmielinizantes/terapia
20.
G Ital Nefrol ; 41(1)2024 Feb 28.
Artigo em Italiano | MEDLINE | ID: mdl-38426683

RESUMO

Therapeutic apheresis is an important hematological and nephrological method for conditions with altered plasma composition. It is also indicated for the removal of protein-bound molecules, such as bilirubin. Several techniques can remove these compounds, such as the extracorporeal circulation molecular adsorption system (MARS), plasma exchange (PEX), and plasma adsorption and perfusion (PAP). Here we report our experience in the comparison between MARS, PEX and PAP, since current guidelines do not specify which method is the most appropriate and under which circumstances it should be used. The choice of technique cannot be based on the desired plasma bilirubin concentration, since these three techniques show similar results with a similar final outcome (exitus). In fact, PAP, PEX and MARS significantly reduce bilirubin levels, but the degree of reduction is not different among the three. Furthermore, the three techniques do not differ in the rate of cholinesterase change, while less reduction of liver transaminases was found by using PAP. MARS should be preferred in the case of renal involvement (hepatorenal syndrome with hyperbilirubinemia). PAP has the advantage of being simple and inexpensive. PEX remains an option when emergency PAP is not available, but the risk of using blood products (plasma and albumin) must be considered.


Assuntos
Remoção de Componentes Sanguíneos , Nefrologia , Humanos , Hiperbilirrubinemia/terapia , Plasmaferese/métodos , Bilirrubina , Diálise Renal/métodos
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