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Gut Pathogens Jun 2024Intussusception, a common cause of abdominal pain in children, often lacks clear underlying causes and is mostly idiopathic. Recurrence, though rare, raises clinical...
BACKGROUND
Intussusception, a common cause of abdominal pain in children, often lacks clear underlying causes and is mostly idiopathic. Recurrence, though rare, raises clinical concerns, with rates escalating after each episode. Factors like pathological lead points and Henoch-Schönlein purpura (HSP) are associated with recurrent cases. On the other hand, the prevalence of Helicobacter pylori (H. pylori), often asymptomatic, in children has been declining. Although its infection is reported to be linked with HSP, its role in recurrent intussusception remains unexplored. Further research is needed to understand the interplay among H. pylori (culprit pathogen), HSP (trigger), and intractable intussusception so as to develop effective management strategies.
CASE PRESENTATION
A two-year-old girl experienced four atypical episodes of intussusception at distinct locations, which later coincided with HSP. Despite treatment with steroids, recurrent intussusception persisted, suggesting that HSP itself was not a major cause for intractable presentations. Subsequent identification of H. pylori infection and treatment with triple therapy resulted in complete resolution of her recalcitrant intussusception.
CONCLUSION
This instructive case underscored a sequence wherein H. pylori infection triggered HSP, subsequently resulting in recurrent intussusception. While H. pylori infection is not common in young children, the coexistence of intractable intussusception and steroid-resistant recurrent HSP necessitates consideration of H. pylori infection as a potential underlying pathogen.
PubMed: 38824586
DOI: 10.1186/s13099-024-00621-z -
Reumatologia Clinica May 2024Scurvy is a nutritional disease caused by ascorbic acid (vitamin C) deficiency. Althought currently it is a rare disease, we should considerer it in the differential...
Scurvy is a nutritional disease caused by ascorbic acid (vitamin C) deficiency. Althought currently it is a rare disease, we should considerer it in the differential diagnosis of purpura and arthritis in patients with restrictive diets. We present the case of a 49-year-old man with a history of a nutritional disorder presented to our hospital with generalized purpura and hemarthros. Following the anamnesis and laboratory findings, rheumatological, infectious and hematological etiologies were excluded. Finally, the diagnosis of scurvy was made upon demostration poor levels of vitamin C and a spectacular response to nutritional supplements. We compare this case with 19 similar cases reported in the medical literature.
Topics: Scurvy; Humans; Male; Middle Aged; Diagnosis, Differential; Vasculitis; Ascorbic Acid
PubMed: 38821739
DOI: 10.1016/j.reumae.2024.05.003 -
Cureus Apr 2024Necrotizing fasciitis (NF) is a rapidly progressive bacterial infection with high mortality. Invasive group A (GAS) infection is the leading cause of NF. Our...
Necrotizing fasciitis (NF) is a rapidly progressive bacterial infection with high mortality. Invasive group A (GAS) infection is the leading cause of NF. Our understanding regarding clinicopathological features and pathogenesis of invasive GAS infection is expanding as the incidence of NF in healthy individuals increases. However, clinicopathological features of NF in the presence of autoimmune diseases have been poorly defined. We experienced NF in a patient treated with infliximab and prednisolone for ulcerative colitis and rheumatoid arthritis. Herein, we present time kinetics findings of clinical symptoms and laboratory data of GAS-associated NF in the presence of immunosuppressant-treated immune disorders.
PubMed: 38817521
DOI: 10.7759/cureus.59346 -
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 -
Genetics Jun 2024Since the days of Ramón y Cajal, the vast diversity of neuronal and particularly dendrite morphology has been used to catalog neurons into different classes. Dendrite... (Review)
Review
Since the days of Ramón y Cajal, the vast diversity of neuronal and particularly dendrite morphology has been used to catalog neurons into different classes. Dendrite morphology varies greatly and reflects the different functions performed by different types of neurons. Significant progress has been made in our understanding of how dendrites form and the molecular factors and forces that shape these often elaborately sculpted structures. Here, we review work in the nematode Caenorhabditis elegans that has shed light on the developmental mechanisms that mediate dendrite morphogenesis with a focus on studies investigating ciliated sensory neurons and the highly elaborated dendritic trees of somatosensory neurons. These studies, which combine time-lapse imaging, genetics, and biochemistry, reveal an intricate network of factors that function both intrinsically in dendrites and extrinsically from surrounding tissues. Therefore, dendrite morphogenesis is the result of multiple tissue interactions, which ultimately determine the shape of dendritic arbors.
Topics: Animals; Caenorhabditis elegans; Dendrites; Morphogenesis; Caenorhabditis elegans Proteins; Sensory Receptor Cells
PubMed: 38785371
DOI: 10.1093/genetics/iyae056