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1.
Artigo em Inglês | MEDLINE | ID: mdl-39353821

RESUMO

OBJECTIVE: Conduct a systematic review and meta-analysis of the efficacy of therapeutic plasma exchange (TPE) or intravenous cangrelor to prevent thromboembolism in patients with heparin-induced thrombocytopenia (HIT) who undergo cardiopulmonary bypass (CPB) with heparin. DESIGN: Systematic review and meta-analysis. SETTING: N/A. PARTICIPANTS: Adults having cardiac surgery with a history of HIT who received preoperative or intraoperative TPE or intravenous cangrelor as an adjunct to CPB with heparin. INTERVENTIONS: None MEASUREMENTS AND MAIN RESULTS: A systematic review was performed using MEDLINE, PubMed, and Google Scholar. The primary outcome was avoidance of thromboembolism (venous or arterial) during or after CPB. Proportional meta-analysis with a random effects model was used to calculate a weighted-pooled proportion/efficacy for the study's primary outcome. Fifty-seven patients in 17 reports received TPE as an adjunctive treatment to prevent HIT-related thrombosis related to heparinization during CPB and 3 (5.3%) experienced thrombosis. Proportional meta-analysis suggested a weighted-pooled freedom from perioperative thromboembolism rate of 91.0% (95% CI 82.6%-96.9%). Fifteen patients in 6 reports received intravenous cangrelor as an adjunctive treatment to prevent HIT-related thrombosis related to heparinization during CPB and 2 (13.3%) experienced thrombosis. Proportional meta-analysis suggested a weighted-pooled freedom from perioperative thromboembolism rate of 83.0% (95% CI 61.2%- 97.6%). CONCLUSIONS: TPE and cangrelor are feasible strategies to prevent thromboembolism in adults with HIT who require CPB with heparin. Given the relatively small number of cases in the published literature and a high likelihood for publication and detection biases, prudence remains warranted when using these strategies.

2.
Thromb J ; 22(1): 85, 2024 Oct 03.
Artigo em Inglês | MEDLINE | ID: mdl-39363287

RESUMO

Homozygous familial hypercholesterolemia (HoFH), is a rare genetic disorder characterized by dual mutations in the low-density lipoprotein receptor (LDLR) gene, leading to dysfunctional or absent LDLRs, often accompanied by severe premature Atherosclerotic Cardiovascular Disease (ASCVD) and exhibiting refractoriness to aggressive pharmacological interventions. Double filtration plasmapheresis (DFPP), a form of lipoprotein apheresis (LA), has been effectively utilized as an adjunctive treatment modality to reduce serum LDL-C levels in refractory cases of HoFH. Here, we report a case of a 36-year-old female with HoFH who developed xanthomas on her limbs and waist at age 7. Despite maximum-tolerated doses of statins from age 32, combined with ezetimibe and evolocumab, her LDL-C levels remained critically elevated at 12-14 mmol/L. Her genetic testing confirmed a homozygous LDLR mutation. At 35 years old, she experienced exertional chest pain, and percutaneous coronary intervention revealed severe calcific left main stenosis, necessitating stent implantation. Subsequently, she initiated once every 1-2 months DFPP. Pre-DFPP, her LDL-C and total cholesterol (TC) levels were 13.82 ± 3.28 and 15.45 ± 0.78 mmol/L, respectively. Post-DFPP, her LDL-C and TC levels significantly decreased to 2.43 ± 0.33 mmol/L (81.76 ± 4.11% reduction) and 3.59 ± 0.41 mmol/L (76.76 ± 2.75% reduction), respectively. Lipoprotein (a) and triglycerides also decreased by 89.10 ± 1.39% and 42.29 ± 15.68%,respectively. Two years later, there was no progression of coronary artery disease, and her symptoms and xanthomas regressed significantly. Collectively, DFPP effectively reduces LDL-C levels in refractory cases of HoFH and contributes to delaying ASCVD progression, representing an efficacious adjunctive therapeutic modality.

3.
Transfusion ; 2024 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-39385534

RESUMO

OBJECTIVE: This study aimed to clarify the clinical application of centrifugal-membrane hybrid plasmapheresis (CMHP) in the treatment of hyperlipidemia. METHODS: A retrospective study was conducted on 48 patients who were diagnosed with hyperlipidemia and had received CMHP treatment. Serum total cholesterol (TC), triglycerides (TG), high-density lipoprotein cholesterol (HDL-C), and low-density lipoprotein cholesterol (LDL-C) were monitored, and adverse reactions to the treatment were observed. RESULTS: Forty-eight patients with hyperlipidemia received CMHP over 59 sessions. The average age of the 48 patients with hyperlipidemia, including 32 males (66.67%) and 16 females (33.33%), was 44.23 ± 12.02 years. Twenty-nine outpatients (60.42%) and 19 inpatients (39.58%) were included. Hypertriglyceridemia was diagnosed in 16 cases (33.33%), mixed hyperlipidemia in 31 cases (64.58%), and hypercholesterolemia in one case (2.08%). The pretreatment blood lipid concentrations were significantly different after the 59 CMHP treatments (p < .001). The concentrations of TC, TG, HDL-C, and LDL-C decreased significantly after the treatment, and the median ratios of reduction were 67.06% (range: 58.97%-71.87%), 63.33% (range: 55.20%-74.86%), 45.87% (range: 35.86%-52.95%), and 66.09% (range: 44.37%-73.94%), respectively. Three adverse reactions (5.08%) were recorded. No differences were detected in therapeutic parameters, effects, or adverse reactions between the two blood cell separators, there was no difference in Lipoprotein apheresis efficacy. CONCLUSION: This preliminary study demonstrated the clinical application of CMHP in patients in the treatment of hyperlipidemia. However, further studies are needed applying CMHP with hyperlipidemia.

4.
Cureus ; 16(9): e68690, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39371869

RESUMO

Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. This case report details the clinical presentation, diagnosis, and management of a 49-year-old female who developed aHUS following elective hip arthroplasty. The patient, with a history of cardiovascular events and no prior renal disease, presented with elevated LDH levels, thrombocytopenia, and acute renal failure on the first postoperative day. A diagnostic workup confirmed aHUS, and the patient was successfully treated with therapeutic plasma exchange (TPE) and hemodialysis. The case underscores the importance of early recognition and aggressive management of aHUS, especially in the perioperative setting, and highlights the need for a multidisciplinary approach to optimize patient outcomes. Through this case, we aim to raise awareness about the potential for surgical stress to trigger aHUS and emphasize the critical role of TPE and supportive care in the treatment of this rare condition.

6.
J Artif Organs ; 2024 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-39390213

RESUMO

A thyroid storm is the most extreme and life-threatening presentation of thyrotoxicosis. Thyroidectomy can be used for definitive treatment. It should be performed after euthyroidism is accomplished. The use of therapeutic plasma exchange (TPE) is a last resort option in cases where standard pharmacological therapy proves to be ineffective. Due to its rare prevalence, there are limited data evaluating the usefulness and efficacy of TPE as a bridging therapy to thyroidectomy. The absence of relevant literature prompted us to conduct a scoping review. The following bibliographic databases were searched for articles dated 30 November 2023: Medline, EMBASE, Web of Science and Google Scholar. The search identified 1047 records, of which 42 articles were accepted with a total of 234 patients. The dominant indications for TPE were side effects due to conventional treatment. The mean fT4 level decreased 51.9% of baseline after TPE, while the mean fT3 level decreased 66.6% of baseline. The main side effects observed with FFP were allergic reactions, while the use of an albumin solution was associated with perioperative bleeding. Based on the limited data available in the literature, we recognize plasmapheresis as an effective treatment option for reducing thyroid hormone levels prior to thyroidectomy in patients with thyrotoxicosis. Available data suggest that it might be reasonable to limit the number of sessions in favor of an earlier surgical intervention. To reduce the risk of bleeding, FFP may be a better option as a replacement fluid, especially in the session prior to thyroidectomy.

7.
Cureus ; 16(8): e68090, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39347200

RESUMO

Myasthenia gravis is a rare disease that can lead to a serious condition known as myasthenic crisis (MC). The diagnosis of MC is clinical and relies on the presence of typical symptoms that can be absent, emphasizing the importance of attracting the attention of emergency physicians to this rare cause of respiratory failure. We present the case of a 66-year-old woman presenting to the emergency department with a history of recent muscle fatigue and dehydration who developed acute respiratory failure requiring mechanical ventilation.

8.
Pediatr Nephrol ; 2024 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-39320552

RESUMO

BACKGROUND: Anti-GBM disease is a rare vasculitis mediated by pathogenic antibodies against collagen IV. Anti-GBM disease presents with rapid progressive glomerulonephritis and leads to kidney failure if untreated. KDIGO recommends plasma exchanges (PEX) for antibody elimination and steroids plus cyclophosphamide (CTX) to suppress antibody production. CTX is associated with severe side effects including gonadal toxicity. Rituximab (RTX) and mycophenolate mofetil (MMF) might be a less toxic but equally efficient alternative to CTX. Studies in pediatric anti-GBM disease patients receiving RTX and MMF instead of CTX are lacking. METHODS: A retrospective survey in 8 tertiary German centers was performed. The clinical data of patients diagnosed between 2014 and 2022 were collected and analyzed. RESULTS: Five adolescent patients treated with PEX and RTX and/or MMF due to anti-GBM disease were analyzed. All patients had anti-GBM antibodies, hematuria, glomerular proteinuria, and pulmonary hemorrhage. eGFR was 124 ml/min/1.73 m2 (range 47-162), and all patients were non-dialysis-dependent but with relevant histological kidney affection (mean crescents on kidney biopsy 77%). Antibody clearance was achieved after 13 PEX cycles (range 6-31). Four out of 5 patients received methylprednisolone pulses. All patients received oral prednisolone and MMF, and four patients received a median of 4 RTX doses (range 2-4). After a mean follow-up of 27 months, 4/5 patients had conserved or improved kidney function, while one patient (20%) developed kidney failure. CONCLUSIONS: In this small series of pediatric non-dialysis-dependent anti-GBM disease patients, first-line treatment with RTX and MMF showed a favorable kidney outcome in 4/5 cases and had an acceptable side effect profile.

9.
Ren Fail ; 46(2): 2400539, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-39258391

RESUMO

BACKGROUND: Anti-glomerular basement membrane (anti-GBM) disease presents with rapidly progressive glomerulonephritis and alveolar hemorrhage, requiring urgent management. In this study, we analyzed the relationship between plasmapheresis strategy, immunosuppressive therapy and the prognosis of anti-GBM disease patients. METHOD: We screened newly diagnosed anti-GBM disease patients at West China Hospital of Sichuan University from 2010 to 2021. The primary outcome was a composite endpoint of in-hospital death or dialysis dependency upon discharge. RESULTS: This study enrolled 107 anti-GBM disease patients. The use of plasmapheresis was independently associated with a reduced risk of primary outcome (OR: 0.179, 95% Cl: 0.051-0.630, p = 0.007), better 2-year (HR: 0.146; 95% CI: 0.038-0.553; p = 0.005) and 8-year patient survival (HR: 0.309; 95% CI: 0.112-0.850; p = 0.023). Restricted cubic spline regression suggested that patients with 5-10 sessions of plasmapheresis had already achieved maximum risk reduction in the primary outcome. Patients who started plasmapheresis at lower serum creatinine (42.9% vs. 96.2%, p < 0.001) or lower anti-GBM antibody levels (44.4% vs. 93.3%, p = 0.030) had lower risk of primary outcome than those at higher levels. Use of high-dose methylprednisolone (p = 0.505), pulsed cyclophosphamide (p = 0.343) or ANCA positivity (p = 0.115) were not related to primary outcome in anti-GBM disease. CONCLUSION: Plasmapheresis was protective for both in-hospital outcome and long-term survival in anti-GBM disease. Patients who initiated plasmapheresis early had a better prognosis and might only need 5-10 plasmapheresis sessions to achieve maximal risk reduction. Use of high-dose methylprednisolone or cyclophosphamide pulses was not related to improved short- or long-term outcomes in anti-GBM disease.


Assuntos
Doença Antimembrana Basal Glomerular , Imunossupressores , Plasmaferese , Humanos , Plasmaferese/métodos , Masculino , Doença Antimembrana Basal Glomerular/terapia , Feminino , Pessoa de Meia-Idade , Adulto , Imunossupressores/uso terapêutico , Prognóstico , China/epidemiologia , Estudos Retrospectivos , Idoso , Diálise Renal , Estudos de Coortes
10.
J Pak Med Assoc ; 74(9): 1707-1710, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39279084

RESUMO

Paediatric acute liver failure (PALF) is a rare yet severe condition that is associated with high mortality. Apart from liver transplant, no specific therapy exists, particularly in developing countries. Evidence suggests that removal of damage-associated molecular patterns, cytokines, toxins, and other metabolites that accumulate due to impaired liver function can enhance natural recovery. Plasmapheresis can be used to remove these products; however, there is limited evidence to support this approach. This case series discusses three critically ill patients with acute liver failure who underwent plasmapheresis. The patients included a seven-year-old boy (Case 1), a 17-year-old boy (Case 2), and a 16-monthold boy (Case 3). Two patients showed significant improvement in bilirubin level, coagulation profile, inotropes requirement, and Glasgow coma scale score. Unfortunately, one patient with PALF, complicated with multi-organ dysfunction, died due to refractory shock on the fourth day of hospitalisation. Our findings illustrate that early use of therapeutic plasmapheresis in PALF can lead to improvement in clinical outcome. It may serve as a bridging therapy for liver transplant and for the spontaneous regeneration of the patient's liver.


Assuntos
Falência Hepática Aguda , Plasmaferese , Humanos , Plasmaferese/métodos , Masculino , Falência Hepática Aguda/terapia , Adolescente , Criança , Lactente , Evolução Fatal , Resultado do Tratamento
11.
Transfus Med Rev ; : 150851, 2024 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-39244430

RESUMO

Most plasma used for manufacturing plasma-derived medicinal products (PDMPs) such as albumin, immunoglobulin (Ig), and clotting factors is obtained from source plasma collected via plasmapheresis, the majority of which is contributed by the United States (US). While the demand for PDMPs continues to rise, it remains unclear whether high-frequency plasmapheresis, such as the twice-weekly plasma donation allowed in the US, may have any (long-term) adverse health effects on the donor. To investigate the frequency at which plasma can be donated without harm to the donor, the current systematic review explores the impact of plasma donation frequency on cardiovascular health, protein depletion, and adverse events in healthy plasma donors. We asked the following research question: What is the impact of plasmapheresis frequency (Intervention) on the safety or health (Outcome) of healthy donors (Population)? Six databases (PubMed, Embase, Web of Science, CINAHL, the Cochrane Library, and Transfusion Evidence Library), 2 clinical trial registries (ICTRP and clinicaltrials.gov), and the PROSPERO database were searched. Four observational and 2 experimental studies were included. The results showed that very high-frequency donation (twice per week) may result in a clinically relevant decrease in ferritin and bring IgG levels below the lower threshold of 6 g/l. However, the evidence is of low to very low certainty, and solid conclusions are hindered by the healthy donor effect and methodological limitations of the included studies. To determine a safe threshold donation frequency that minimizes any possible harmful effect on the donor, more high-quality prospective cohort studies and experimental studies are needed. We should expedite such studies to support recommendations, as conclusive evidence confirming or refuting the safety of maximum allowed donation frequencies is lacking. Donor protection is essential, given that healthy donors receive no direct medical benefit from donating plasma.

12.
Ther Apher Dial ; 2024 Sep 08.
Artigo em Inglês | MEDLINE | ID: mdl-39245561

RESUMO

Double filtration plasmapheresis (DFPP) is a semi-selective blood purification modality derived from the plasma exchange modality. DFPP can be applied to a variety of refractory disorders including metabolic disorders, organ transplants, rheumatic disorders, neurological disorders, and dermatologic disorders. Familial hypercholesterolemia and lipoprotein (a) hyperlipoproteinemia are major chronic metabolic disorders. Lipoprotein apheresis (LA) is applied for those patients to remove low-density lipoprotein cholesterol (LDL-C) and lipoprotein (a) (Lp(a)). DFPP is used as one of the modalities in LA. In addition to removing LDL-C and Lp(a), DFPP has pleiotropic effects such as removal of lipid metabolism-related substances, C-reactive protein lowering effect, removal of adhesion molecules, removal of inflammatory cytokines, and anti-oxidative effect. This article summarizes the pleiotropic effects of DFPP based on recent clinical articles.

13.
Artigo em Inglês | MEDLINE | ID: mdl-39251499

RESUMO

OBJECTIVE: Hypertriglyceridemia-induced acute pancreatitis (HTG-AP) hospitalizations are increasing in the USA; however, the impact of race and ethnicity on key outcomes in Hispanic and non-Hispanic white HTG-AP hospitalizations has not been studied. METHODS: We queried the National Inpatient Sample (NIS) between 2016 and 2020 identifying all patients with discharge diagnosis AP. HTG-AP hospitalizations were identified for Hispanic and non-Hispanic white patients. Primary outcomes included yearly rate of HTG-AP and in-hospital mortality from HTG-AP. Secondary outcomes were length of stay (LOS) and inflation-adjusted hospital costs. RESULTS: HTG-AP hospitalizations accounted for 5.9% of all AP hospitalizations; 17,440 and 48,235 hospitalizations included a Hispanic and non-Hispanic white patient, respectively. The yearly rate of HTG-AP hospitalizations per 100,000 adult population was statistically higher for Hispanics compared to non-Hispanic whites. The HTG-AP hospitalization rate increased for both Hispanics and non-Hispanic whites (both ptrend < 0.001); however, the trends were not statistically different. The number of observed in-hospital deaths for Hispanics was too low to report, precluding subsequent analysis. Hispanics were younger, more likely to be female, more commonly Medicaid recipients, and from zip codes with lower income quartiles. Despite clinically similar rates of plasmapheresis use and LOS, adjusted hospital costs were 18.9% higher for Hispanics compared to non-Hispanic whites (95% CI, 15.4 to 22.6% higher, p < 0.001). CONCLUSIONS: HTG-AP incidence is increasing in the USA in Hispanic and non-Hispanic whites. Despite clinically similar outcomes, HTG-AP hospitalizations in Hispanic patients were associated with $26,805,280 in excess costs compared to non-Hispanic white hospitalizations.

14.
Arch Dermatol Res ; 316(9): 644, 2024 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-39325061

RESUMO

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe, adverse drug reaction that is notoriously complex in both its presentation and treatment. Although early diagnosis and cessation of the causative agent are universally accepted as the initial interventions for DRESS, the subsequent management lacks a standardized approach. Historically, systemic steroids have been used as first-line treatment, but there is debate about the optimal dosing and route of administration, and evidence persists on the long-term complications associated with steroid use. Novel treatment approaches with targeted therapy, cyclosporine, intravenous immunoglobulin, and plasmapheresis have been gaining interest as alternative mono- and adjuvant therapies, but their use has yet to be supported by clinical trials. This narrative review provides a summary of the current knowledge of DRESS, with a focus on clinical management. The various mono- and adjuvant therapy options are discussed, with literature-supported suggestions for their optimal use in clinical practice. The risks for relapses, viral reactivation, and long-term complications are also considered. The PubMed and Medline databases were searched for articles on DRESS, published between January 1, 2008, and May 1, 2023. 334 articles met the inclusion criteria. Based on the literature, a DRESS management tool with step-by-step guidance is provided. Further suggestions for management are woven throughout this review, giving clinicians a toolbelt of resources with which to approach diagnosis, treatment, and follow-up.


Assuntos
Síndrome de Hipersensibilidade a Medicamentos , Humanos , Síndrome de Hipersensibilidade a Medicamentos/diagnóstico , Síndrome de Hipersensibilidade a Medicamentos/etiologia , Síndrome de Hipersensibilidade a Medicamentos/terapia , Guias de Prática Clínica como Assunto , Plasmaferese/métodos , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas Intravenosas/efeitos adversos , Ciclosporina/uso terapêutico , Ciclosporina/efeitos adversos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico
15.
Pak J Med Sci ; 40(8): 1669-1674, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39281214

RESUMO

Background and objectives: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired treatable autoimmune disorder. Due to limited availability and affordability of IV immunoglobulins and therapeutic plasma exchange in Pakistan, oral immunosuppressive drugs (ISDs) are used despite limited role in literature. The study aimed to determine the response to ISDs in CIDP patients by assessing the frequency of remission, reduction of disability using a neuropathy related disability score called Inflammatory Neuropathy Cause and Treatment score (or INCAT score), as well as reduction in steroid maintenance dose. Methods: The retrospective observational study of six months duration (May to October, 2020) was carried out in Aga Khan University Hospital, Karachi, Pakistan. Medical record of all the patients with idiopathic CIDP taking oral ISDs in last five years was selected which included bio-data, clinical signs and symptoms, medication details, and INCAT scores. Descriptive statistics were described i.e. frequency, percentages, mean/standard deviation using Microsoft Excel v.2021. Results: Out of thirteen patients, Azathioprine was used in nine, Mycophenolate mofetil in two and Cyclosporine in two, with remission (INCAT score improvement ≥ 1) achieved in eight, one and zero patients respectively. Duration of ISDs ranged from three to twenty-four months (average 15.8 months). Patients with monoclonal paraproteinemia and prior exposure to ISDs had a poor response to the introduction of subsequent ISDs. Conclusion: The study describes preliminary experience of the potential role of relatively cheaper and more convenient oral ISDs (especially Azathioprine) as an alternative or sparing agent to first line agents for CIDP and sets the stage for larger scale studies and randomized controlled trials.

16.
Cureus ; 16(7): e65762, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39211686

RESUMO

Acute transverse myelitis (TM) is a rare, acquired neuro-immune spinal cord disorder that can be idiopathic or related to a secondary disease. Clinical signs and symptoms include motor weakness, sensory alterations, and bowel or bladder dysfunction. Often TM occurs in the younger population or middle-aged adults. This patient's presentation is unique in the fact that he does not fall into either of these age categories. In this case, a 72-year-old male with a past medical history of hypertension and type 2 diabetes mellitus presented to the emergency department due to a five-day history of worsening weakness of the upper extremities bilaterally. In addition, the patient reported a new onset of abdominal wall numbness. The patient reported being at a theme park a few days prior, denying any injuries and only complaining of neck discomfort during the car ride home. Labs and imaging were quickly ordered for diagnostic purposes. The patient was diagnosed with TM using magnetic resonance imaging (MRI), lumbar puncture, and clinical signs. The etiology was later discovered to be due to a new diagnosis of Sjögren's autoimmune disease. The patient was treated with high-dose intravenous steroids for five days while being monitored for any neurologic changes. The plan was to continue steroids by mouth once discharged from the hospital. Due to poor adherence to discharge instructions, the patient was readmitted after presenting to the emergency department with worsening symptoms. Physicians need to recognize and diagnose TM quickly, as some etiologies are treatable and can prevent further damage to the spinal cord.

17.
Cureus ; 16(7): e65561, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39192912

RESUMO

The symptoms of Miller-Fisher syndrome (MFS) are a triad of areflexia, ataxia, and ophthalmoplegia. The condition is a rare variant of Guillain-Barré syndrome (GBS), an acute immune-mediated nerve disorder. Both conditions involve abnormal autoimmune responses that may often be triggered by infections such as Campylobacter jejuni, human immunodeficiency virus, Epstein-Barr virus, and Zika virus, among others. As a result, the immune system mistakenly attacks the body's own nerve tissues. MFS is characterised by ophthalmoparesis, which can progress to complete external ophthalmoplegia and may include ptosis, facial nerve paralysis, sensory impairments, and muscle weakness. Diagnosis is supported by lumbar puncture, revealing albumin-cytologic dissociation, although initial tests may not always be indicative. A diagnostic marker for MFS is the presence of anti-GQ1b antibodies, which target the GQ1b ganglioside in nerves and affect oculomotor function in particular. Electrodiagnostic studies often show absent or reduced sensory responses without reduced conduction velocity. Treatment options include intravenous immunoglobulin therapy and plasmapheresis, which are both equally effective. This case study demonstrated significant clinical improvement in a patient undergoing plasmapheresis due to financial constraints, highlighting the efficacy of this treatment approach. A 50-year-old female presented with limb paraesthesia, progressive ptosis, imbalance, and transient diplopia following a recent fever. Examination revealed stable vitals, decreased deep tendon reflexes, reduced vibratory sensation, cerebellar ataxia, and cranial nerve abnormalities. Cerebrospinal fluid analysis showed elevated protein, suggesting MFS. Normal magnetic resonance imaging and nerve conduction studies indicated GBS, with positive anti-GQ1b antibodies. After five plasma exchange cycles, the patient improved substantially and was discharged with no residual symptoms after one month.

18.
J Thorac Dis ; 16(7): 4584-4596, 2024 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-39144329

RESUMO

Background: Cardiovascular surgeries often require deep hypothermic circulatory arrest and cardiopulmonary bypass (CPB), which can disrupt blood clotting and lead to excessive bleeding. Traditional treatments involve transfusing blood and blood products, which can have adverse effects and place significant strain on the global blood supply. Research suggests that autologous platelet-rich plasmapheresis (aPRP) may reduce the need for transfusions by preserving blood components. However, the impact of aPRP on postoperative blood loss and clinical outcomes in cardiovascular surgery remains controversial. This study aimed to examine the effects of aPRP on postoperative blood loss and recovery in patients undergoing heart valve surgery. Methods: A total of 183 patients were divided into either aPRP or control groups. The aPRP group received aPRP before CPB, whereas the control group did not. The primary endpoint was postoperative bleeding between the groups. The secondary endpoints were postoperative bleeding risk factors and clinical outcome assessment. Logistic regression analysis with covariate adjustment was used to calculate these risk factors. Results: A total of 76 patients (41.5%) in the aPRP group and 107 patients (58.5%) in the control group were included in the analysis. No significant difference was found in the occurrence of postoperative bleeding [odds ratio (OR) =0.53, 95% confidence interval (CI): 0.28-1.00, P=0.05], and the aPRP group had fewer complications than the controls (OR =0.28, 95% CI: 0.10-0.68, P=0.009). However, after adjusting for the New York Heart Association (NYHA) classification, diabetes, arrhythmology, mean activated clotting time (ACTmean), CPB, bleeding, thoracotomy, and body mass index (BMI), there was a significant difference in postoperative bleeding (adjusted OR =0.47, 95% CI: 0.22-0.98, P=0.04) and complications (adjusted OR =0.23, 95% CI: 0.07-0.64, P=0.008) between the two groups. Conclusions: Preoperative aPRP can improve postoperative outcomes and reduce complications in patients undergoing heart valve surgery.

19.
Adv Exp Med Biol ; 1448: 601-609, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39117842

RESUMO

Cytokine storm syndromes (CSS) include different entities such as macrophage activation syndrome, primary and secondary hemophagocytic lymphohistiocytosis (HLH), and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. An effective management strategy is critical in CSS. While biologics have become an essential part of CSS treatment, hematopoietic stem cell transplantation (HSCT) has changed the fate of primary HLH patients. This chapter will focus on the available alternative immunomodulatory therapies in CSS, which include corticosteroids, cyclosporine A, intravenous immunoglobulin, interleukin 18 binding protein, therapeutic plasmapheresis, HSCT, and mesenchymal stromal cell-based therapies.


Assuntos
COVID-19 , Síndrome da Liberação de Citocina , Humanos , Síndrome da Liberação de Citocina/imunologia , Síndrome da Liberação de Citocina/terapia , COVID-19/imunologia , COVID-19/terapia , COVID-19/complicações , Transplante de Células-Tronco Hematopoéticas , SARS-CoV-2/imunologia , Linfo-Histiocitose Hemofagocítica/terapia , Linfo-Histiocitose Hemofagocítica/imunologia , Plasmaferese/métodos , Agentes de Imunomodulação/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Transplante de Células-Tronco Mesenquimais/métodos , Ciclosporina/uso terapêutico , Síndrome de Resposta Inflamatória Sistêmica/imunologia , Síndrome de Resposta Inflamatória Sistêmica/terapia
20.
J Pharm Pract ; : 8971900241273234, 2024 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-39140484

RESUMO

Carbamazepine is utilized for various indications. Due to its pharmacokinetic profile and drug properties, toxicity can be delayed and persistent despite supportive care. We report a severe case of intentional carbamazepine toxicity in a carbamazepine naive individual mimicking brain death that was not diagnosed until three days after consumption of carbamazepine when the patient was comatose. Symptoms of overdose persisted for several days despite attempted treatment with activated charcoal and whole bowel irrigation, hemodialysis, and plasmapheresis. Symptoms only began to improve with bowel evacuation as a result of administration of neostigmine intravenously plus hemodialysis and plasmapheresis additionally. Despite previous literature that reported success with hemodialysis and/or plasmapheresis we did not find either to be overly effective in our case possibly due to lack of ability to perform multidose activated charcoal and whole bowel irrigation. To our knowledge this is one of the few cases of carbamazepine overdose utilizing both hemodialysis and plasmapheresis but without activated charcoal and the only case report in which neostigmine was administered as an attempt to remove drug via the gastrointestinal tract with success.

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