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The Turkish Journal of Pediatrics May 2024Hemolytic uremic syndrome (HUS) is a serious cause of acute kidney injury in children. There is a suggestion that coronavirus disease 2019 (COVID-19) may be a trigger...
BACKGROUND
Hemolytic uremic syndrome (HUS) is a serious cause of acute kidney injury in children. There is a suggestion that coronavirus disease 2019 (COVID-19) may be a trigger for HUS. In this study, we present a pediatric case diagnosed with HUS associated with COVID-19, which progressed to end-stage kidney disease.
CASE
A previously healthy 13-year-old girl with fever and vomiting was referred to our hospital. Laboratory investigations revealed direct Coombs-negative hemolytic anemia, thrombocytopenia and renal impairment accompanied by COVID-19 infection. Although anemia and thrombocytopenia showed improvement on the seventh day after admission, the renal impairment persisted. The histopathological findings of a renal biopsy were compatible with both HUS and COVID-19. One month later, the patient had a recurrence of HUS, again testing positive for COVID-19. Kidney function improved with plasma exchange therapy. Eculizumab treatment was recommenced after COVID-19 PCR became negative. Anemia and thrombocytopenia did not recur with eculizumab, while renal impairment persisted. Eculizumab was discontinued after three months when genetic analysis for HUS was negative. Subsequently, the patient was diagnosed with end-stage kidney disease.
CONCLUSIONS
COVID-19 can be associated with HUS relapses, leading to chronic kidney disease. Further studies should investigate the mechanism of HUS associated with COVID-19.
Topics: Humans; COVID-19; Female; Adolescent; Hemolytic-Uremic Syndrome; Kidney Failure, Chronic; Disease Progression; SARS-CoV-2
PubMed: 38814305
DOI: 10.24953/turkjpediatr.2024.4524 -
Research and Practice in Thrombosis and... Mar 2024Assessment of platelet function is key in diagnosing bleeding disorders and evaluating antiplatelet drug efficacy. However, there is a prevailing "one-size-fits-all"...
BACKGROUND
Assessment of platelet function is key in diagnosing bleeding disorders and evaluating antiplatelet drug efficacy. However, there is a prevailing "one-size-fits-all" approach in the interpretation of measures of platelet reactivity, with arbitrary cutoffs often derived from healthy volunteer responses.
OBJECTIVES
Our aim was to compare well-used platelet reactivity assays.
METHODS
Blood and platelet-rich plasma obtained from the Framingham Heart Study ( = 3429) were assayed using a range of agonists in 5 platelet assays: light transmission aggregometry, Optimul aggregometry, Multiplate impedance aggregometry (Roche Diagnostics), Total Thrombus-Formation Analysis System, and flow cytometry. Using linear mixed-effect models, we determined the contribution of preanalytical and technical factors that modulated platelet reactivity traits.
RESULTS
A strong intra-assay correlation of platelet traits was seen in all assays, particularly Multiplate velocity ( = 0.740; ristocetin vs arachidonic acid). In contrast, only moderate interassay correlations were observed ( = 0.375; adenosine diphosphate Optimul E vs light transmission aggregometry large area under the curve). As expected, antiplatelet drugs strongly reduced platelet responses, with aspirin use primarily targeting arachidonic acid-induced aggregation, and explained substantial variance (β = -1.735; = 4.59 × 10; variance proportion = 46.2%) and P2Y antagonists blocking adenosine diphosphate responses (β = -1.612; = 6.75 × 10; variance proportion = 2.1%). Notably, female sex and older age were associated with enhanced platelet reactivity. Fasting status and deviations from standard venipuncture practices did not alter platelet reactivity significantly. Finally, the agonist batch, phlebotomist, and assay technician (more so for assays that require additional sample manipulation) had a moderate to large effect on measured platelet reactivity.
CONCLUSION
Caution must be exercised when extrapolating findings between assays, and the use of standard ranges must be medication-specific and sex-specific at a minimum. Researchers should also consider preanalytical and technical variables when designing experiments and interpreting platelet reactivity measures.
PubMed: 38813256
DOI: 10.1016/j.rpth.2024.102406 -
Revista Paulista de Pediatria : Orgao... 2024To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD).
OBJECTIVE
To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD).
CASE DESCRIPTION
Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response.
COMMENTS
TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.
Topics: Humans; Anemia, Sickle Cell; Thrombotic Microangiopathies; Male; Female; Child; Adolescent
PubMed: 38808867
DOI: 10.1590/1984-0462/2024/42/2023108 -
BMC Health Services Research May 2024Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their...
BACKGROUND
Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their diagnosis, treatment, and outcomes. This study aims to describe the pilot phase of the national pediatric Immune Thrombocytopenia(ITP) registry (NPITP) in Iran, serving as the inaugural interpretive report.
METHODS
This patient-centered software system was implemented as a national program across multiple pediatric centers in Iran. Several focus groups were conducted to establish a minimum data set (MDS) comprising six main classes, 14 sub-classes, and 187 data elements. Following expert consensus on the final data set, a web-based software tool was developed by the dedicated IT team, accessible online and offline via https://disreg.sbmu.ac.ir/q/ITP.html . The registry included children aged between two months and 18 years with a platelet count below 100 × 10/L, based on predefined inclusion criteria.
RESULTS
Within a four-month period, a total of 60 ITP patients were registered, including 41 (68.3%) newly diagnosed cases, 68 (13.6%) persistent cases, and 14 (23.3%) with chronic ITP. The mean age of the registered patients was 55.93 ± 9.72 months. The most frequently observed bleeding symptoms were petechiae (68.3%), purpura (51.6%), and ecchymosis (13.3%). Among the newly diagnosed patients, 20 (33.3%) received intravenous immunoglobulin (IVIG), 17 (28.3%) were treated with prednisolone, and 17 (28.3%) received combined IVIG and steroid therapy. Of all patients, 40 (66.7%) demonstrated a complete response to treatment, while 16 (26.7%) exhibited a partial response. Four patients (6.7%) remained unresponsive to therapy. Treatment-related complications, such as Cushing's syndrome, edema, weight gain, hirsutism, and mood disorders, were reported in 10 patients (16.6%). However, the majority of patients (81.7%) did not experience therapy-related complications.
CONCLUSION
The pilot phase of the NPITP registry successfully implemented a web-based software tool for data collection, aiming to enhance the quality of care, facilitate clinical research, and support health service planning in the future.
Topics: Humans; Registries; Child; Iran; Purpura, Thrombocytopenic, Idiopathic; Child, Preschool; Adolescent; Male; Female; Infant; Pilot Projects
PubMed: 38807135
DOI: 10.1186/s12913-024-11102-z -
Frontiers in Veterinary Science 2024In human medicine, various pathologies, including decompression sickness, thrombocytopenia, and rheumatoid arthritis, have been linked to changes in cellular...
In human medicine, various pathologies, including decompression sickness, thrombocytopenia, and rheumatoid arthritis, have been linked to changes in cellular microparticles (MP) formation, particularly platelet microparticles (PMP). Similar disorders in marine mammals might be attributed to anthropogenic threats or illnesses, potentially impacting blood PMP levels. Thus, detecting platelet phosphatidylserine (PS) exposure and PMP formation could serve as a crucial diagnostic and monitoring approach for these conditions in marine mammals. Our group has developed a methodology to assess real-time PS exposure and PMP formation specifically tailored for marine mammals. This method, pioneered in species such as bottlenose dolphins, beluga whales, walruses, and California sea lions, represents a novel approach with significant implications for both clinical assessment and further research into platelet function in these animals. The adapted methodology for evaluating PS exposure and PMP formation in marine mammals has yielded promising results. By applying this approach, we have observed significant correlations between alterations in PMP levels and specific pathologies or environmental factors. These findings underscore the potential of platelet function assessment as a diagnostic and monitoring tool in marine mammal health. The successful adaptation and application of this methodology in marine mammals highlight its utility for understanding and managing health concerns in these animals.
PubMed: 38799726
DOI: 10.3389/fvets.2024.1393977 -
The Pan African Medical Journal 2024Essential thrombocythemia is the category of myeloproliferative syndromes, generally characterized by a group of clonal stem cell diseases that present a disturbance in...
Essential thrombocythemia is the category of myeloproliferative syndromes, generally characterized by a group of clonal stem cell diseases that present a disturbance in the growth of one or more sets of hematopoietic cells. All long clinical treatment, patients may experience gastrointestinal disorders and other metabolic processes that can lead to weight loss and malnutrition. Cytokine is involved in the control of appetite, digestive, and metabolic processes in the body, it can be assumed that increased stimulation could impair the control of these processes leading to loss of body mass. Effective and systematic nutritional intervention is required to ensure patient compliance with treatment and improved nutritional status.
Topics: Humans; Thrombocythemia, Essential; Weight Loss; Malnutrition; Nutritional Status; Patient Compliance
PubMed: 38799191
DOI: 10.11604/pamj.2024.47.93.32594 -
BioRxiv : the Preprint Server For... May 2024Patients with pathogenic variants in (inherited platelet disorder (IPD)-18) have normal platelet counts but show impaired platelet aggregation due to diminished...
Patients with pathogenic variants in (inherited platelet disorder (IPD)-18) have normal platelet counts but show impaired platelet aggregation due to diminished activation of αIIbβ3 integrin. This defect results in moderate to severe bleeding episodes, especially following surgical procedures, which require patients to be transfused with platelets and/or pro-hemostatic agents. We recently demonstrated that the hemostatic efficacy of transfused platelets is limited by dysfunctional endogenous platelets in a mouse model of IPD-18 ( mice), as dysfunctional platelets were recruited to the forming hemostatic plug but did not participate in clot contraction. Consequently, higher amounts of transfused platelets were required to outcompete these dysfunctional cells and to reverse bleeding. We here studied the usefulness of thromboelastography with platelet mapping (TEG-PM), a method to evaluate platelet-dependent clot contraction, for monitoring of the hemostatic potential in mice transfused with various amounts of wild-type (WT) platelets. whole blood samples did not contract in TEG-PM, consistent with a critical role of this protein in αIIbβ3 activation. Addition of WT platelets improved TEG parameters (K time, α-angle, MA) in a ratio dependent manner, consistent with our recent in vivo studies showing impaired hemostasis at a 5:1, but not at a 2:1 ratio of mutant to WT platelets. Interestingly, K and α values were identified as better predictors of transfusion efficacy than MA, the most platelet-dependent TEG parameter. In conclusion, this proof-of-concept study supports the use of TEG-PM to monitor platelet transfusion ratios and hemostatic potential in IPD-18 and potentially other platelet disorders.
PubMed: 38798516
DOI: 10.1101/2024.05.09.593198 -
Scientific Reports May 2024The treatment of immune thrombocytopenia (ITP) is challenging and treatment outcomes depend on numerous unknown and patient-specific factors. Corticosteroids are the...
The treatment of immune thrombocytopenia (ITP) is challenging and treatment outcomes depend on numerous unknown and patient-specific factors. Corticosteroids are the cornerstone of ITP treatment, but they are associated with many side effects. In this retrospective cohort study, treatment outcomes and treatment adherence in patients with ITP were investigated in 214 ITP patients from November 15, 2022 to March 15, 2023. Multinomial regression analysis models were used to identify predictive factors for treatment outcomes. A p value of less than 0.05 was considered statistically significant. Most study participants were female 161 (75.5%), and the majority 172 (80.4%) of them were taking prednisolone only. In terms of treatment adherence, 178 (83.2%) of the study participants adhered well to their ITP medications. The complete response rate at 3 months was 139 (65.0%). Predictive factors for partial response were increased negative impact of ITP on health-related quality of life (AOR = 1.221, 95% CI 1.096-1.360), being treated at Tikur Abessa Sepcialazed Hospital (AOR = 0.431, 95% CI 0.197-0.941) and the presence of heavy menstrual bleeding (AOR = 2.255, 95% CI 0.925-5.497) compared to patients with complete response. Hepatitis B virus-infected ITP patients (AOR = 0.052, 95% CI 0.004-0.621) were also a predictive factor for no response compared to complete response.
Topics: Humans; Female; Male; Purpura, Thrombocytopenic, Idiopathic; Retrospective Studies; Adult; Ethiopia; Middle Aged; Treatment Outcome; Hospitals, Teaching; Young Adult; Aged; Adolescent; Quality of Life; Medication Adherence; Prednisolone
PubMed: 38789461
DOI: 10.1038/s41598-024-62372-w -
Medicine May 2024Miller-Fisher syndrome (MFS) is a rare subtype of Guillain-Barre syndrome with classic features of ataxia, areflexia, and ophthalmoplegia that can be caused by a...
RATIONALE
Miller-Fisher syndrome (MFS) is a rare subtype of Guillain-Barre syndrome with classic features of ataxia, areflexia, and ophthalmoplegia that can be caused by a preceding infection including COVID-19. We present a current, asymptomatic thrombocytopenic COVID-19 infection as a cause of MFS in a 60-year-old male with a concurrent chronic immune neuropathy.
PATIENT CONCERNS
A 60-year-old male presenting with acute symptoms of MFS including ataxia, areflexia, and ophthalmoplegia on a chronic immune neuropathy for at least 1 year and concurrent asymptomatic COVID-19 positive infection.
DIAGNOSIS
MFS suspected secondary to a current thrombocytopenic COVID-19 infection.
INTERVENTIONS
Five days of intravenous immune globulin with continued monthly intravenous immune globulin as an outpatient, follow-up long-term in a neuromuscular clinic, electromyography as an outpatient, and continued physical therapy.
OUTCOMES
The patient significantly improved after initial treatment.
LESSONS
The full effect of COVID-19 on the various Guillain-Barre syndrome subtypes is unknown, although it clearly can be a cause of the various variants including being caused by a current, asymptomatic infection.
Topics: Humans; Miller Fisher Syndrome; Male; COVID-19; Middle Aged; Immunoglobulins, Intravenous; SARS-CoV-2; Thrombocytopenia
PubMed: 38788032
DOI: 10.1097/MD.0000000000038304 -
PloS One 2024Thrombosis with thrombocytopenia syndrome (TTS) is a very rare prothrombotic disorder that is a safety concern for some COVID-19 vaccines. We aimed to devise a case... (Observational Study)
Observational Study
BACKGROUND
Thrombosis with thrombocytopenia syndrome (TTS) is a very rare prothrombotic disorder that is a safety concern for some COVID-19 vaccines. We aimed to devise a case definition to estimate the incidence of thrombosis with thrombocytopenia as a proxy for TTS in a national insurance claims database.
METHODS
We conducted a retrospective observational study using the National Health Insurance Research Database (NHIRD) in Taiwan over the three-year period prior to the SARS-COV-2 pandemic (2017-2019). Our case definition was all patients with newly diagnosed thrombosis co-occurring with a diagnosis of thrombocytopenia within seven days before or after the thrombosis diagnosis. Cases were identified using International Classification of Disease-10 codes.
FINDINGS
We identified 2010 patients with newly diagnosed thrombosis co-occurring with thrombocytopenia during the study period. The mean age was 64.71 years; female:male ratio 1:1.45. The most frequent thrombotic events were coronary artery disease (18.81%), cerebral infarction (16.87%), and disseminated intravascular coagulation (13.13%). Cerebral venous sinus thrombosis was rare (<0.1%). The average annual incidence rate of co-occurring new diagnoses of thrombosis and thrombocytopenia was 2.84 per 100 000 population. Incidence rates were higher in men than women, except in 20-39 year-olds (higher in females). 20.6% of patients died within the first month after diagnosis.
INTERPRETATION
We observed that the demographic and clinical characteristics of thrombosis with co-occurring thrombocytopenia using our case definition is different from that of TTS. Further research is needed to refine the case definition of TTS in the post-COVID-19 vaccination period.
Topics: Humans; Female; Male; Middle Aged; COVID-19; Thrombocytopenia; Incidence; Thrombosis; Aged; Retrospective Studies; Taiwan; Adult; SARS-CoV-2; Young Adult; Aged, 80 and over; Databases, Factual; Adolescent; Pandemics
PubMed: 38787838
DOI: 10.1371/journal.pone.0301359