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Caspian Journal of Internal Medicine 2024Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement,...
BACKGROUND
Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement, antiphospholipid antibodies (aPL) have been proposed in recent years.
METHODS
In this prospective cohort of 48 pediatric patients who were admitted with IgA vasculitis from September 2015 to June 2017, two serum samples were taken 12 weeks apart to detect Anti-Phospholipid antibodies. All patients were followed-up for renal involvement for six months.
RESULTS
Renal involvement occurred in 14 out of 48 patients with IgA vasculitis (29.16%). APLs were positive in nine out of 14 patients with IgA vasculitis and renal involvement (64.28%), in contrast to only six out of 34 patients with IgA vasculitis without renal involvement (17.64%). The presence of aPL antibodies was statistically associated with renal involvement (P=0.002). Although, the relationship between both sex (P=0.025) and age (P=0.046) with aPL positivity was statistically significant, performing a modified logistic regression test, the odds ratio was significant between the groups with and without renal involvement only in term of age and aPL positivity).
CONCLUSION
The presence of aPL antibodies was statistically associated with renal involvement. We found a significant relationship between the age and aPL positivity. Hence, we need multicenter, more extensive cohort studies to reach a better and more accurate conclusion on the relationship between serum aPLs and renal involvement in IgA vasculitis patients.
PubMed: 38807720
DOI: 10.22088/cjim.15.2.287 -
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 -
Cureus May 2024Thrombopoietin receptor agonist (TPO-RA) is effective for aplastic anemia (AA) and idiopathic thrombocytopenic purpura (ITP). However, the risk of thrombosis during ITP...
Thrombopoietin receptor agonist (TPO-RA) is effective for aplastic anemia (AA) and idiopathic thrombocytopenic purpura (ITP). However, the risk of thrombosis during ITP treatment with TPO-RA is higher than without TPO-RA. It is unclear whether TPO-RA increases the risk of thrombosis in patients with AA. We report a case of a 66-year-old female with severe AA having paroxysmal nocturnal hemoglobinuria (PNH) clones in the peripheral blood who developed ischemic colitis after three days of starting eltrombopag. Contrast-enhanced computed tomography showed ischemic colitis and contrast enhancement defect in the left atrial appendage, which indicated a thrombus in the heart. Stopping eltrombopag and providing supportive care improved her symptoms, and her blood cell counts gradually increased. Thrombosis should be considered when TPO-RA is administered during the immunosuppressive treatment of AA.
PubMed: 38800784
DOI: 10.7759/cureus.61135 -
Scientific Reports May 2024To investigate neonatal injuries, morbidities and risk factors related to vaginal deliveries. This retrospective, descriptive study identified 3500 patients who...
To investigate neonatal injuries, morbidities and risk factors related to vaginal deliveries. This retrospective, descriptive study identified 3500 patients who underwent vaginal delivery between 2020 and 2022. Demographic data, neonatal injuries, complications arising from vaginal delivery and pertinent risk factors were documented. Neonatal injuries and morbidities were prevalent in cases of assisted vacuum delivery, gestational diabetes mellitus class A2 (GDMA2) and pre-eclampsia with severe features. Caput succedaneum and petechiae were observed in 291/3500 cases (8.31%) and 108/3500 cases (3.09%), respectively. Caput succedaneum was associated with multiparity (adjusted odds ratio [AOR] 0.36, 95% confidence interval [CI] 0.22-0.57, P < 0.001) and assisted vacuum delivery (AOR 5.18, 95% CI 2.60-10.3, P < 0.001). Cephalohaematoma was linked to GDMA2 (AOR 11.3, 95% CI 2.96-43.2, P < 0.001) and assisted vacuum delivery (AOR 16.5, 95% CI 6.71-40.5, P < 0.001). Scalp lacerations correlated with assisted vacuum and forceps deliveries (AOR 6.94, 95% CI 1.85-26.1, P < 0.004; and AOR 10.5, 95% CI 1.08-102.2, P < 0.042, respectively). Neonatal morbidities were associated with preterm delivery (AOR 3.49, 95% CI 1.39-8.72, P = 0.008), night-time delivery (AOR 1.32, 95% CI 1.07-1.63, P = 0.009) and low birth weight (AOR 7.52, 95% CI 3.79-14.9, P < 0.001). Neonatal injuries and morbidities were common in assisted vacuum delivery, maternal GDMA2, pre-eclampsia with severe features, preterm delivery and low birth weight. Cephalohaematoma and scalp lacerations were prevalent in assisted vaginal deliveries. Most morbidities occurred at night.Clinical trial registration: Thai Clinical Trials Registry 20220126004.
Topics: Humans; Female; Pregnancy; Risk Factors; Infant, Newborn; Adult; Retrospective Studies; Vacuum Extraction, Obstetrical; Delivery, Obstetric; Birth Injuries; Infant, Newborn, Diseases; Diabetes, Gestational; Pre-Eclampsia
PubMed: 38796580
DOI: 10.1038/s41598-024-62703-x -
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 -
Metabolomics : Official Journal of the... May 2024IgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker.
INTRODUCTION
IgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker.
OBJECTIVE
We aimed to explore the baseline serum metabolome of adult patients with IgA vasculitis to identify potential diagnostic biomarkers.
METHODS
We performed a study comparing the serum metabolome of patients with IgA vasculitis to that of patients with inflammatory condition, namely spondyloarthritis. Serum analyses were performed by high-performance liquid chromatography-mass spectrometry.
RESULTS
Fifty-five patients with IgA vasculitis and 77 controls with spondyloarthritis (age- and sex-matched) were included in this study. The median age of IgA vasculitis patients was 53 years. Two-thirds of patients were female (n = 32). At the time of vasculitis diagnosis, 100% of patients had skin involvement and 69% presented with glomerulonephritis (n = 38). Joint and digestive involvement were observed in 56% (n = 31) and 42% (n = 23) of patients. Four discriminative metabolites between the two groups were identified: 1-methyladenosine, L-glutamic acid, serotonin, and thymidine. The multivariate model built from the serum metabolomes of patients with IgA vasculitis and spondyloarthritis revealed an accuracy > 90%. As this model was significant according to the permutation test (p < 0.01), independent validation showed an excellent predictive value of the test set: sensitivity 98%; specificity 98%, positive predictive value 97% and negative predictive value 98%.
CONCLUSION
To our knowledge, this study is the first to use the metabolomic approach for diagnostic purposes in adult IgA vasculitis, highlighting a specific diagnostic metabolome signature.
Topics: Humans; Female; Male; Middle Aged; Metabolome; Adult; Biomarkers; Immunoglobulin A; Chromatography, High Pressure Liquid; Vasculitis; Metabolomics; Aged; Mass Spectrometry; IgA Vasculitis
PubMed: 38787468
DOI: 10.1007/s11306-024-02107-0 -
Frontiers in Medicine 2024Pulmonary alveolar microlithiasis (PAM) is a rare disease whose clinical and imaging manifestations are non-specific, characterized by the deposition of microliths,...
BACKGROUND
Pulmonary alveolar microlithiasis (PAM) is a rare disease whose clinical and imaging manifestations are non-specific, characterized by the deposition of microliths, which primarily consist of calcium and phosphorus, within the alveoli. In the cases of PAM, patients combined with calcification of other organs such as gastric mucosal calcification are less common.
CASE PRESENTATION
A 59-year-old woman was admitted to our hospital due to cough producing white, foamy sputum, accompanied by dyspnea and fever for 20 days. The CT scan showed diffuse ground-glass opacities and calcification of the gastric mucosa. Lung tissue biopsy revealed the presence of calcification and granulomatous foreign bodies in the interstitium and alveolar cavity. In the later stages, she developed painful skin petechiae. For this patient, the diagnosis of PAM, gastric mucosal calcification, and purpura fulminans was made. However, the genetic test results hinted that the patient and her son had a heterozygous mutation in the gene, but her daughter's genetic test results were normal. Although the patient received anti-infection treatment, steroids, and oxygen therapy, her condition did not improve.
CONCLUSION
We reported a rare case of PAM combined with calcification of other organs and purpura fulminans. Treatment of steroids did not show any benefit. The causative mechanism and effective treatment of this disease remain unclear. More treatments need to be explored.
PubMed: 38784230
DOI: 10.3389/fmed.2024.1357260 -
JAMA Network Open May 2024SARS-CoV-2 viral load (VL) in the nasopharynx is difficult to quantify and standardize across settings, but it may inform transmission potential and disease severity.
IMPORTANCE
SARS-CoV-2 viral load (VL) in the nasopharynx is difficult to quantify and standardize across settings, but it may inform transmission potential and disease severity.
OBJECTIVE
To characterize VL at COVID-19 diagnosis among previously uninfected and unvaccinated individuals by evaluating the association of demographic and clinical characteristics, viral variant, and trial with VL, as well as the ability of VL to predict severe disease.
DESIGN, SETTING, AND PARTICIPANTS
This secondary cross-protocol analysis used individual-level data from placebo recipients from 4 harmonized, phase 3 COVID-19 vaccine efficacy trials sponsored by Moderna, AstraZeneca, Janssen, and Novavax. Participants were SARS-CoV-2 negative at baseline and acquired COVID-19 during the blinded phase of the trials. The setting included the US, Brazil, South Africa, Colombia, Argentina, Peru, Chile, and Mexico; start dates were July 27, 2020, to December 27, 2020; data cutoff dates were March 26, 2021, to July 30, 2021. Statistical analysis was performed from November 2022 to June 2023.
MAIN OUTCOMES AND MEASURES
Linear regression was used to assess the association of demographic and clinical characteristics, viral variant, and trial with polymerase chain reaction-measured log10 VL in nasal and/or nasopharyngeal swabs taken at the time of COVID-19 diagnosis.
RESULTS
Among 1667 participants studied (886 [53.1%] male; 995 [59.7%] enrolled in the US; mean [SD] age, 46.7 [14.7] years; 204 [12.2%] aged 65 years or older; 196 [11.8%] American Indian or Alaska Native, 150 [9%] Black or African American, 1112 [66.7%] White; 762 [45.7%] Hispanic or Latino), median (IQR) log10 VL at diagnosis was 6.18 (4.66-7.12) log10 copies/mL. Participant characteristics and viral variant explained only 5.9% of the variability in VL. The independent factor with the highest observed differences was trial: Janssen participants had 0.54 log10 copies/mL lower mean VL vs Moderna participants (95% CI, 0.20 to 0.87 log10 copies/mL lower). In the Janssen study, which captured the largest number of COVID-19 events and variants and used the most intensive post-COVID surveillance, neither VL at diagnosis nor averaged over days 1 to 28 post diagnosis was associated with COVID-19 severity.
CONCLUSIONS AND RELEVANCE
In this study of placebo recipients from 4 randomized phase 3 trials, high variability was observed in SARS-CoV-2 VL at the time of COVID-19 diagnosis, and only a fraction was explained by individual participant characteristics or viral variant. These results suggest challenges for future studies of interventions seeking to influence VL and elevates the importance of standardized methods for specimen collection and viral load quantitation.
Topics: Humans; COVID-19; Nasopharynx; Viral Load; Male; SARS-CoV-2; Female; Adult; Middle Aged; COVID-19 Vaccines; Randomized Controlled Trials as Topic; United States; Aged
PubMed: 38780941
DOI: 10.1001/jamanetworkopen.2024.12835 -
Epilepsia Open May 2024
PubMed: 38780493
DOI: 10.1002/epi4.12962 -
Cureus Apr 2024Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura (HSP), is a disease that causes inflammation and bleeding in small blood vessels in the skin,...
Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura (HSP), is a disease that causes inflammation and bleeding in small blood vessels in the skin, joints, intestines, and kidneys. Although various infections and chemicals are known to be triggers, the underlying cause of IgAV remains unknown. Here, we describe a case of an 86-year-old male patient with IgAV that developed after anti-tuberculosis treatment for tuberculous pleurisy. There have been several case reports implicating and other acid-fast bacterium in the development of IgAV, but only a few case reports implicating anti-tuberculous drugs. This case highlights the importance of recognizing that IgAV can be caused by anti-tuberculous drugs.
PubMed: 38779276
DOI: 10.7759/cureus.58707