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Medicine May 2024Amniotic fluid embolism (AFE) is a fatal obstetric condition that often rapidly leads to severe respiratory and circulatory failure. It is complicated by obstetric... (Review)
Review
RATIONALE
Amniotic fluid embolism (AFE) is a fatal obstetric condition that often rapidly leads to severe respiratory and circulatory failure. It is complicated by obstetric disseminated intravascular coagulation (DIC) with bleeding tendency; therefore, the introduction of venoarterial extracorporeal membrane oxygenation (VA-ECMO) is challenging. We report the case of a patient with AFE requiring massive blood transfusion, rescued using VA-ECMO without initial anticoagulation.
PATIENTS CONCERNS
A 39-year-old pregnant patient was admitted with a complaint of abdominal pain. An emergency cesarean section was performed because a sudden decrease in fetal heart rate was detected in addition to DIC with hyperfibrinolysis. Intra- and post-operatively, the patient had a bleeding tendency and required massive blood transfusions. After surgery, the patient developed lethal respiratory and circulatory failure, and VA-ECMO was introduced.
DIAGNOSIS
Based on the course of the illness and imaging findings, the patient was diagnosed with AFE.
INTERVENTIONS
By controlling the bleeding tendency with a massive transfusion and tranexamic acid administration, using an antithrombotic ECMO circuit, and delaying the initiation of anticoagulation and anti-DIC medication until the bleeding tendency settled, the patient was managed safely on ECMO without complications.
OUTCOMES
By day 5, both respiration and circulation were stable, and the patient was weaned off VA-ECMO. Mechanical ventilation was discontinued on day 6. Finally, she was discharged home without sequelae.
LESSONS
VA-ECMO may be effective to save the lives of patients who have AFE with lethal circulatory and respiratory failure. For safe management without bleeding complications, it is important to start VA-ECMO without initial anticoagulants and to administer anticoagulants and anti-DIC drugs after the bleeding tendency has resolved.
Topics: Humans; Female; Embolism, Amniotic Fluid; Extracorporeal Membrane Oxygenation; Adult; Pregnancy; Cesarean Section; Blood Transfusion; Tranexamic Acid; Disseminated Intravascular Coagulation; Anticoagulants
PubMed: 38758915
DOI: 10.1097/MD.0000000000038176 -
Medicine May 2024Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by widespread blood vessel clotting and bleeding. It can affect individuals of any age but is more... (Review)
Review
RATIONALE
Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by widespread blood vessel clotting and bleeding. It can affect individuals of any age but is more commonly observed in females, particularly during pregnancy. Pregnancy combined with TTP is a critical and rapidly progressing condition that is often misdiagnosed as an obstetric disorder like severe preeclampsia or HELLP syndrome. To deepen the understanding of TTP during pregnancy with the help of a clinical case.
PATIENT CONCERNS
A 20-year-old patient, is pregnancy 1 birth 0, 32 weeks dated by her last menstrual period, presented chest tightness, and shortness of breath after physical activity for 3 days.
DIAGNOSES
TTP.
INTERVENTIONS
At present, there are no preventive measures. Timely diagnosis and treatment are useful. Plasma exchange and treat to the patient hinder autoantibodies, such as gamma globulin, methylprednisolone, rituximab, and cyclosporine were effective.
OUTCOMES
The patient exhibited stable vital signs, normal examination results, and experienced no complications. We continued to monitor her progress after she was discharged.
LESSONS SUBSECTIONS
The acute onset of TTP is often associated with pregnancy, as it is a triggering factor. Timely identification, accurate diagnosis, and a comprehensive treatment approach involving plasma exchange, immunosuppressants, and the termination of pregnancy can lead to remission and a favorable outlook for the majority of patients.
Topics: Humans; Female; Pregnancy; Purpura, Thrombotic Thrombocytopenic; Plasma Exchange; Young Adult; Pregnancy Complications, Hematologic
PubMed: 38758904
DOI: 10.1097/MD.0000000000038112 -
Scientific Reports May 2024Immune thrombocytopenia (ITP) is an autoimmune disease caused by T-cell dysfunction. Recently, several studies have shown that a disturbed Th17/Treg balance contributes...
Immune thrombocytopenia (ITP) is an autoimmune disease caused by T-cell dysfunction. Recently, several studies have shown that a disturbed Th17/Treg balance contributes to the development of ITP. MicroRNAs (miRNAs) are small noncoding RNA moleculesthat posttranscriptionally regulate gene expression. Emerging evidences have demonstrated that miRNAs play an important role in regulating the Th17/Treg balance. In the present study, we found that miR-641 was upregulated in ITP patients. In primary T cells, overexpression of miR-641 could cause downregulation of its target genes STIM1 and SATB1, thus inducing a Th17 (upregulated)/Treg (downregulated) imbalance. Inhibition of miR-641 by a miR-641 sponge in primary T cells of ITP patients or by antagomiR-641 in an ITP murine model could cause upregulation of STIM1 and SATB1, thus restoring Th17/Treg homeostasis. These results suggested that the miR-641-STIM/SATB1 axis plays an important role in regulating the Th17/Treg balance in ITP.
Topics: MicroRNAs; Matrix Attachment Region Binding Proteins; Th17 Cells; T-Lymphocytes, Regulatory; Stromal Interaction Molecule 1; Humans; Animals; Mice; Purpura, Thrombocytopenic, Idiopathic; Female; Male; Neoplasm Proteins; Adult; Middle Aged; Gene Expression Regulation; Disease Models, Animal
PubMed: 38755179
DOI: 10.1038/s41598-024-61660-9 -
Frontiers in Medicine 2024As one of the most common gynecological disorders, PD significantly impacts the quality of life for women. TSD, a well-known traditional Chinese medical prescription,...
BACKGROUND
As one of the most common gynecological disorders, PD significantly impacts the quality of life for women. TSD, a well-known traditional Chinese medical prescription, has gained popularity for its use in treating gynecological cold coagulation and blood stasis syndromes such as PD. However, the lack of comprehensive data hinders our understanding of its molecular mechanism.
PURPOSE
The objective of the present study is to investigate the therapeutic effects of TSD on PD and elucidate its plausible mechanism.
METHODS
HPLC was employed to confirm the presence of the principal metabolites of TSD. The rat model of PD was induced by OT exposure following IWM and EB pretreatment, and subsequently treated with TSD via gastric gavage. The effects and potential mechanisms of TSD on PD rats were explored, encompassing general behavior, morphological alterations in the uterus and ovaries, biochemical indicators in the uterus and serum, and levels of proteins related to the PI3K/AKT signaling pathway.
RESULTS
Gallic acid, hydroxysafflower yellow A, albiflorin, paeoniflorin, and ferulic acid were determined to be the primary active metabolites of TSD. The pharmacological studies yielded results indicating the successful establishment of the PD model in rats. Additionally, TSD demonstrated its ability to protect PD rats by ameliorating general behavior, mitigating pathological damage to uterine and ovarian tissues, and modulating the expression levels of correlated factors (PGE2, PGF2, Ca2+, TXB2, IL-6, TNF-, NO, and COX-2) as well as p-PI3K/PI3K and p-AKT/AKT proteins.
CONCLUSION
TSD exhibited protective effects against PD in rats through its interaction with multiple targets including P13K/AKT signaling pathway, indicating that TSD holds therapeutic potential for PD treatment and providing evidence supporting the rational utilization of TSD.
PubMed: 38751973
DOI: 10.3389/fmed.2024.1343179 -
Head & Face Medicine May 2024Chronic kidney disease (CKD) directly affects oral health. Yet data about halitosis in young CKD patients and the impact of dental prophylaxis is limited. Therefore, as... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Chronic kidney disease (CKD) directly affects oral health. Yet data about halitosis in young CKD patients and the impact of dental prophylaxis is limited. Therefore, as part of this randomized clinical trial, halitosis in young CKD patients undergoing intensive or standard oral preventive procedures was to be explored.
METHODS
Three volatile sulfur compounds (hydrogen sulfide, methyl mercaptan and dimethyl sulfide) were measured in 30 young patients with CKD (mean age 14.2 years; 16 males, 14 females). Breath samples were taken after 3 and 6 months and analyzed with selective gas chromatography (OralChroma). Tongue coating (Winkel Index) and clinical indices to determine local inflammation or oral hygiene (Papillary Bleeding Index and Quigley-Hein Index) were assessed. Within an extended anamnesis, patients and their mothers and nurses were questioned about the perceived halitosis. Corresponding quotes were noted verbatim. Patients were randomized to either intensive need-related oral health care measures (oral preventative program, OPP) or a one-stage standard prevention (treatment as usual, TAU).
RESULTS
While there were no differences in volatile sulfur compound levels between TAU and OPP at the three time points of measurements (p > 0.05), there was a tendency towards a reduction in dimethyl sulfide and hydrogen sulfide of affected patients within the OPP group over time. Looking at potential differences between both groups with regard to tongue coating, significant differences were observed between baseline and 3 months after study start in the OPP group, and between baseline and 6 months after study start in the TAU group (p < 0.05). The burden of halitosis was frequently reported by patients' mothers and nurses.
CONCLUSIONS
Young CKD patients regularly suffered from halitosis and dimethyl sulfide was its main source. Preventive measures mainly resulted in a reduction of tongue coating.
TRIAL REGISTRATION
The German Clinical Trial Register (# DRKS00010580).
Topics: Humans; Halitosis; Female; Male; Adolescent; Renal Insufficiency, Chronic; Breath Tests; Child; Oral Hygiene; Sulfur Compounds; Chromatography, Gas; Hydrogen Sulfide
PubMed: 38750491
DOI: 10.1186/s13005-024-00428-y -
The American Journal of Managed Care May 2024To describe people with hemophilia B (PWHB) in the US who experience bleeds despite factor replacement therapy and to quantify the associated burden from the third-party... (Observational Study)
Observational Study
OBJECTIVES
To describe people with hemophilia B (PWHB) in the US who experience bleeds despite factor replacement therapy and to quantify the associated burden from the third-party payer perspective.
STUDY DESIGN
Observational study of adult male PWHB treated with factor IX replacement therapy identified from the PharMetrics Plus claims data from 2010 to 2019.
METHODS
Patients with medically recorded bleeds (MRBs) were identified using diagnostic codes. Rates and rate ratios of inpatient admissions, emergency department (ED) visits, and outpatient visits among PWHB with and without MRBs were estimated. The presence of comorbidities was identified using diagnostic codes, and the analysis was stratified by age group.
RESULTS
There were 345 PWHB with MRBs and 252 without MRBs. More than half of PWHB with MRBs (56.8%) had 1 or more comorbidity vs 39.3% of PWHB without MRBs. The prevalence of anxiety and depression was high in PWHB, regardless of bleed status and age group, whereas the prevalence of other comorbidities increased with age group. The rate of all-cause inpatient admissions for PWHB with MRBs was 14.8 per 100 person-years (95% CI, 12.8-17.1), 2.5 times higher than for PWHB without MRBs. The rate of all-cause ED visits for PWHB with MRBs was 67.6 per 100 person-years (95% CI, 63.2-72.3), 2.7 times higher than for those without MRBs.
CONCLUSIONS
This study reports significant resource use and clinical burden among PWHB who seek medical care. PWHB with MRBs had considerable all-cause resource use compared with PWHB without MRBs. The prevalence of mental illness was consistently high across all age groups.
Topics: Humans; Male; Hemophilia B; Adult; Retrospective Studies; Middle Aged; Hemorrhage; Comorbidity; United States; Young Adult; Factor IX; Aged; Adolescent
PubMed: 38748916
DOI: 10.37765/ajmc.2024.89543 -
Sao Paulo Medical Journal = Revista... 2024Until recently, the treatment of people with hemophilia A and inhibitors (PwHAi) was based on the use of bypassing agents (BPA). However, the advent of emicizumab as... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Until recently, the treatment of people with hemophilia A and inhibitors (PwHAi) was based on the use of bypassing agents (BPA). However, the advent of emicizumab as prophylaxis has demonstrated promising results.
OBJECTIVES
We aimed to compare the bleeding endpoints between PwHAi on BPA and those on emicizumab prophylaxis.
DESIGN AND SETTING
Systematic review of interventions and meta-analysis conducted at the Universidade Federal de Goiás, Goiânia, Goiás, Brazil.
METHODS
The CENTRAL, MEDLINE, Scopus, and LILACS databases were searched on February 21, 2023. Two authors conducted the literature search, publication selection, and data extraction. The selected publications evaluated the bleeding endpoints between PwHAi on emicizumab prophylaxis and those on BPA prophylaxis. The risk of bias was evaluated according to the Joanna Briggs Institute criteria. A meta-analysis was performed to determine the annualized bleeding rate (ABR) for treated bleeds.
RESULTS
Five publications (56 PwHAi) were selected from the 543 retrieved records. Overall, bleeding endpoints were lower during emicizumab prophylaxis than during BPA prophylaxis. All the publications had at least one risk of bias. The only common parameter for the meta-analysis was the ABR for treated bleeds. During emicizumab prophylaxis, the ABR for treated bleeds was lower than during BPA prophylaxis (standard mean difference: -1.58; 95% confidence interval -2.50, -0.66, P = 0.0008; I2 = 68.4%, P = 0.0031).
CONCLUSION
Emicizumab was superior to BPA in bleeding prophylaxis in PwHAi. However, both the small population size and potential risk of bias should be considered when evaluating these results.
SYSTEMATIC REVIEW REGISTRATION
CRD42021278726, https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=278726.
Topics: Humans; Hemophilia A; Antibodies, Bispecific; Antibodies, Monoclonal, Humanized; Hemorrhage
PubMed: 38747872
DOI: 10.1590/1516-3180.2023.0102.R1.20022024 -
Anesthesiology Jun 2024
Topics: Extracorporeal Membrane Oxygenation; Humans; Antithrombin III Deficiency
PubMed: 38742999
DOI: 10.1097/ALN.0000000000004948 -
Platelets Dec 2024Immune thrombocytopenia (ITP) is a common autoimmune hematological disorder. Despite this, diagnosis is still challenging due to clinical heterogeneity and the lack of a...
Immune thrombocytopenia (ITP) is a common autoimmune hematological disorder. Despite this, diagnosis is still challenging due to clinical heterogeneity and the lack of a specific diagnostic test. New findings in the pathology and the availability of new drugs have led to the development of different guidelines worldwide. In the present study, the Delphi methodology has been used to get a consensus on the management of adult patients with ITP in Spain and to help in decision-making. The Delphi questionnaire has been designed by a scientific ad hoc committee and has been divided into 13 topics, with a total of 127 items, covering the maximum possible scenarios for the management of ITP. As a result of the study, a total consensus of 81% has been reached. It is concluded that this Delphi consensus provides practical recommendations on topics related to diagnosis and management of ITP patients to help doctors to improve outcomes. Some aspects remain unclear, without consensus among the experts. Thus, more advances are needed to optimize ITP management.
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Spain; Delphi Technique; Consensus; Surveys and Questionnaires
PubMed: 38742687
DOI: 10.1080/09537104.2024.2336104 -
Orphanet Journal of Rare Diseases May 2024Adeno-associated virus (AAV)-based gene therapy for haemophilia has advanced substantially in the last 13 years; recently, three products have received approvals from... (Review)
Review
BACKGROUND
Adeno-associated virus (AAV)-based gene therapy for haemophilia has advanced substantially in the last 13 years; recently, three products have received approvals from regulatory authorities. Although the impact on quality of life seems promising, some limitations remain, such as the presence of pre-existing anti-AAV neutralising antibodies and the occurrence of hepatotoxicity. This review follows the CSL Behring-sponsored symposium at the 27th Congress of the European Hematology Association (EHA) 2022 that examined the haemophilia gene therapy process from a 360-degree multidisciplinary perspective. Here, the faculty (haematologist, nurse and haemophilia patient) summarised their own viewpoints from the symposium, with the aim of highlighting the key considerations required to engage with gene therapy effectively, for both patients and providers, as well as the importance of multidisciplinary collaboration, including with industry.
RESULTS
When considering these new therapies, patients face a complex decision-making process, which includes whether gene therapy is right for them at their current stage of life. The authors agreed that collaboration and tailored education across the multidisciplinary team (including patients and their carers/families), starting early in the process and continuing throughout the long-term follow-up period, is key for the success of gene therapy. Additionally, patient expectations, which may surround eligibility, follow-up requirements and treatment outcomes, should be continually explored. During these ongoing discussions, transparent communication of the unknown factors, such as anticipated clotting factor levels, long-term factor expression and safety, and psychological changes, is critical. To ensure efficiency and comprehensiveness, clearly‑defined protocols should outline the whole process, which should include the recording and management of long-term effects.
CONCLUSION
In order to engage effectively, both patients and providers should be familiar with these key considerations prior to their involvement with the haemophilia gene therapy process. The future after the approval of haemophilia gene therapies remains to be seen and real-world evidence is eagerly awaited.
Topics: Humans; Genetic Therapy; Hemophilia A; Dependovirus; Physicians; Nurses; Quality of Life
PubMed: 38741157
DOI: 10.1186/s13023-024-03181-2