Search Results (131)

Background/Objectives: Henoch–Schönlein purpura (HSP), or IgA vasculitis, is the most common small-vessel vasculitis in children, yet Indian cohort data remain limited. We aimed to describe the clinical profile, renal involvement, treatment patterns, relapse, and outcomes of pediatric HSP at a tertiary centre in South India. Methods: We conducted a retrospective review of children <18 years diagnosed with HSP (January 2013–October 2018) using EULAR/PRINTO/PRES criteria. Demographics, clinical features, laboratory parameters, treatments, and outcomes were abstracted from records and analyzed in SPSS (descriptive statistics; Chi-square/Fisher’s exact and t/non-parametric tests as appropriate). Subgroup comparisons included renal vs. non-renal disease and age <6 vs. ≥6 years. An exploratory analysis examined predictors of nephritis. Results: Of 43 children identified, 2 were excluded (misclassified as systemic lupus erythematosus); 41 were analyzed. Mean age was 8.5 years (range 3–17), male: female 1.4:1. A preceding febrile illness or upper respiratory tract infection was noted in 41.4% and 17%, respectively. Palpable purpura was universal; joint involvement 73.1%, abdominal pain 61.0%, vomiting 41.5%. Renal involvement 17% occurred only in children ≥6 years; exploratory testing supported a strong age-linked signal for nephritis. Laboratory abnormalities included anemia (48.7%), thrombocytosis (19.5%), and elevated ESR (51.2%). Skin biopsy (n = 29) showed IgA and complement deposition; renal biopsy (n = 2) showed ISKDC grades II–III. Treatments included NSAIDs 71.6%, corticosteroids 31.7%, and dapsone 24.4% (used for severe systemic/persistent cutaneous disease). Rash relapse 7.3% clustered with joint plus abdominal symptoms and was not observed among children with nephritis. At a mean 18.9-month follow-up, one child required long-term antihypertensives; no child progressed to end-stage renal disease. Conclusions: Pediatric HSP in this South-Indian cohort followed a largely self-limited course with favourable renal outcomes. Age ≥6 years flagged higher renal risk, supporting age-targeted urine and blood-pressure surveillance, while relapse appeared to follow a non-renal trajectory (joint/abdominal clustering). Steroid and dapsone use reflected clinical severity rather than relapse risk. Findings align with Indian series and suggest lower renal morbidity than some East-Asian reports, adding region-specific evidence to guide monitoring and counselling. Full article

Background: Emergency laparotomy (EL) carries high morbidity and mortality relative to elective abdominal surgery. While Enhanced Recovery After Surgery (ERAS) principles improve outcomes in elective care, their translation to emergencies is inconsistent. The emergency department (ED) provides a window for rapid risk stratification and pre-optimization, provided that interventions do not delay definitive surgery. Methods: We conducted a PRISMA-ScR–conformant scoping review to map ED-initiated, ERAS-aligned strategies for EL. PubMed, Scopus, and Cochrane were searched in February 2025. Eligible sources comprised ERAS guidelines, systematic reviews, cohort studies, consensus statements, and programmatic reports. Evidence was charted across five a priori domains: (i) ERAS standards, (ii) comparative effectiveness, (iii) ED-feasible pre-optimization, (iv) risk stratification (Emergency Surgery Score [ESS], frailty, sarcopenia), and (v) oncological emergencies. Results: Thirty-four sources met inclusion. ERAS guidelines codify rapid assessment, multimodal intraoperative care, and early postoperative rehabilitation under a strict no-delay rule. Meta-analysis and cohort data suggest ERAS-aligned pathways reduce complications and length of stay, though heterogeneity persists. ED-feasible measures include multimodal analgesia, goal-directed fluids, early safe nutrition, respiratory preparation, and anemia/micronutrient optimization (IV iron, vitamin B12, folate, vitamin D). Sarcopenia, frailty, and ESS consistently predicted adverse outcomes, supporting targeted bundle activation. Evidence from oncological emergencies indicates feasibility under no-delay governance. Conclusions: A minimal, ED-initiated, ERAS-aligned bundle is feasible, guideline-concordant, and may shorten hospitalization and reduce complications in EL. We propose a practical framework that links rapid risk stratification, opportunistic pre-optimization, and explicit continuity into intra- and postoperative care; future studies should test fidelity, costs, and outcome impact in pragmatic emergency pathways. Full article

Background/Objectives: The occurrence of non-communicable diseases (NCDs) globally is rising rapidly, largely due to modifiable risk factors such as unhealthy diets. Studies have shown that poor dietary habits are prevalent among university students and may persist in later life, increasing the risk of chronic health conditions. The objective of this study was to evaluate the diet of two different groups of university students, in the United Arab Emirates (UAE) and United Kingdom (UK), with the aim of identifying areas for intervention to improve overall health and wellbeing. Methods: Detailed 7-day diet diaries were collected from undergraduate university participants in the UAE and UK. Diet diaries were quantitatively assessed using Nutritics software generating reports on mean intakes for energy, macro- and micronutrients. Independent sample t-tests were utilized to compare nutrient intake between cohorts in the two different regions. Results: A total of 158 students participated in this study. Results showed significant differences in intake levels in most macronutrients and micronutrients (p ≤ 0.05). Upon comparison, UK participants consumed diets higher in sugar (+9.4 g/day), saturated fat (+4.2 g/day), cholesterol (+90 mg/day), and sodium (+307 mg/day) compared to their UAE counterparts, placing them at risk of cardiovascular diseases (CVDs). Cholesterol intake was oversufficient in both UAE and UK males by 40% and 57%, respectively. In UAE females, there were notable deficiencies in protein intake, omega 3, vitamin D, iron, iodine, and folic acid (p ≤ 0.05), placing them at risk of CVDs, anemia, diabetes, and cancer. Interestingly, both UAE males and females were 100% deficient in dietary vitamin D intake. Conclusions: Nutritional imbalances should be addressed through campus-based nutrition education programs. This study also highlights the importance of dietary guidelines targeted at specific populations accounting for cultural differences. Full article

Furunculosis caused by Aeromonas salmonicida is one of the most common diseases in aquaculture, leading to significant economic losses. This study comprehensively investigated the dynamics of pathophysiological and histopathological disorders in rainbow trout (Oncorhynchus mykiss) infected with the moderately virulent strain A. salmonicida SL0n. Whole-genome analysis showed that strain SL0n belongs to the A. salmonicida species complex, possessing a single circular chromosome. The genome encodes a wide range of virulence factors, including adhesion systems (type IV pili, fimbriae), toxins (aerolysin, hemolysins), and a type II secretion system (T2SS), but notably lacks plasmids and a type III secretion system (T3SS). This genomic profile likely dictates a pathogenic mechanism reliant on secreted exotoxins (via T2SS), explaining the observed systemic cytotoxic damage. In an acute experiment, the 4-day LD₅₀ was determined to be 1.63 × 10⁶ CFU/fish. In a prolonged experiment, fish were injected with a sublethal dose (1.22 × 10⁶ CFU/fish—75% of LD50). The disease progressed through three consecutive stages. The early stage (1–2 DPI) was characterized by maximal bacterial load and activation of nonspecific immunity. The acute stage (4 DPI) manifested as severe septicemia and anemia, associated with systemic organ damage, which correlated with peak AST and ALT enzyme activity. The recovery stage (6 DPI) was marked by partial regression of inflammation, key biochemical and histological parameters indicated persistent liver and kidney dysfunction, signifying an incomplete recovery. These results demonstrate the pathogenesis of acute furunculosis and reveal that the genomic profile of the SL0n strain causes a sequential, systemic infection characterized by severe organ dysfunction. Full article

Background: Non-islet cell tumor hypoglycemia is increasingly reported with gastrointestinal stromal tumors (GIST), but population-level estimates of its clinical impact are limited. We evaluated associations between hypoglycemia and inpatient outcomes among GIST hospitalizations. Methods: We conducted a retrospective cross-sectional study of the National Inpatient Sample (NIS) 2018–2020. Adult GIST discharges were identified by ICD-10-CM codes and stratified by hypoglycemia. Primary outcomes were in-hospital mortality and resource utilization—length of stay (LOS) and total hospital charge. Secondary outcomes included malnutrition, sepsis, ascites, peritonitis, bowel perforation, intestinal obstruction, gastrointestinal bleeding, and iron deficiency anemia. Analyses used survey-weighted logistic regression for binary outcomes and generalized linear models for continuous outcomes. A propensity score-matched sensitivity analysis balanced sepsis and malnutrition. Results: Among 61,725 GIST hospitalizations, 0.72% had hypoglycemia. Mortality was 12.6% with hypoglycemia vs. 3.1% without; adjusted odds of death were higher (aOR 4.16, 95% CI 2.06–8.37; p < 0.001). Hypoglycemia was also associated with malnutrition (aOR 5.63, 3.37–9.40), sepsis (aOR 4.00, 2.24–7.14), ascites (aOR 3.43, 1.63–7.19), and peritonitis (aOR 2.91, 1.17–7.22). LOS was 4.61 days longer on average (not significant; p = 0.185), and total hospital charge was $5218 higher (β = 19,116.8; p = 0.95). In the matched cohort, the mortality association attenuated but persisted (aOR 1.38, 1.27–1.49; p < 0.001); peritonitis remained significant (aOR 1.10, 1.04–1.17), intestinal obstruction (aOR 4.91, 3.44–7.05) and iron deficiency anemia (aOR 3.54, 1.62–7.74) became significant, while ascites and gastrointestinal bleeding were not significant. Conclusions: Hypoglycemia in GIST, although uncommon, marks a higher-risk inpatient trajectory with increased mortality and several complications; these signals largely persist after balancing severity proxies. Resource-use differences were directionally higher but not statistically significant. Recognition of hypoglycemia may aid risk stratification and inpatient management in GIST. Full article

Cyclosporiasis, caused by Cyclospora cayetanensis, is a rare opportunistic infection, particularly in immunosuppressed patients with inflammatory bowel disease (IBD). Its clinical presentation may mimic IBD, with chronic diarrhea and anemia resistant to standard therapy. We report the case of a 24-year-old woman with ulcerative colitis (UC) and a history of liver transplantation, treated with vedolizumab and immunosuppressants. Despite endoscopic remission, she experienced persistent abdominal pain, diarrhea, and iron deficiency anemia. Escalation of biologic therapy was ineffective. After exclusion of bacterial and viral causes, stool testing identified Cyclospora cayetanensis. Treatment with nitazoxanide led to rapid clinical and laboratory improvement. Biologic therapy was temporarily discontinued and later resumed without recurrence of symptoms. This case shows that chronic diarrhea in IBD patients may not always result from the underlying disease. Immunosuppression increases the risk of opportunistic infections. Early diagnosis and specific treatment can improve outcomes and allow safe continuation of IBD therapy. Full article

Alpha-thalassemia is most often caused by large deletions within the α-globin gene cluster which reduce or abolish α-globin chain synthesis. Several common deletions are well described, but atypical structural variants remain underdiagnosed. In this study, we report three novel large heterozygous deletions of the α-globin cluster. The variants were identified in unrelated patients who presented with persistent microcytosis and hypochromia in the absence of iron deficiency or structural hemoglobin variants. A stepwise molecular diagnostic approach was applied. It combined commercial deletion screening assays, Sanger sequencing, Multiplex Ligation-dependent Probe Amplification (MLPA), and targeted Next-Generation Sequencing (NGS) with the Devyser Thalassemia panel. MLPA detected three deletions ranging from ~17 kb to ~360 kb. All involved the critical HS-40 regulatory region and both HBA1 and HBA2 structural genes, consistent with α⁰-thalassemia alleles. Next-Generation Sequencing confirmed the extent of each deletion and refined their genomic boundaries. Comparative genomic mapping showed that these deletions are distinct from classical variants such as --SEA or --MED, indicating novel structural configurations. Clinically, all patients displayed a carrier phenotype, with normal HbF levels (<1%) and normal or slightly reduced HbA2 values. This study broadens the mutational spectrum of α⁰-thalassemia and demonstrates the diagnostic value of combining MLPA and NGS in patients with unexplained microcytosis. By enabling accurate distinction from iron-deficiency anemia and other microcytic disorders, these findings have direct translational implications for improving diagnostic precision and genetic counseling in clinical practice. Full article

Immune dysregulation is being increasingly recognized as a leading sign of a wide spectrum of inborn errors of immunity (IEIs). Therefore, patients with IEIs are frequently managed in non-immunological settings, including hematology and oncology units, during the diagnostic process or follow-up. The most relevant hematological signs associated with IEIs comprise autoimmune cytopenia (AIC), lymphoproliferative diseases (LPD), malignancies, hemophagocytic lymphohystiocitosis (HLH), bone marrow failure (BMF), myelodysplastic syndromes (MDS), and peripheral or tissue eosinophilia. The prognosis of patients with IEIs can significantly improve when a molecular diagnosis is established, as it can allow the use of targeted treatments, guide appropriate follow-up strategies and, in some cases, support the rationale for hematopoietic stem cell transplantation or gene therapy. Therefore, there is an urgent need to recognize the warning signs suggestive for an underlying IEI among patients presenting with common hematological features and to ensure an appropriate diagnostic approach. As a general rule, clinicians should always provide a clinical alert in the presence of two or more IEI-associated hematological signs, as well as a positive familial history for IEI or hematologic immune dysregulation, a personal history of severe infections, and other signs of immune dysregulation. Concerning AIC, an increased likelihood of IEI is characteristic of patients with treatment refractoriness, autoimmune hemolytic anemia, or multilineage cytopenia. In the case of LPD, the main elements of suspicion are represented by the chronic or recurrent disease course, the persistence of Epstein–Barr Virus (EBV) infection, and the development of lymphoproliferation in atypical localizations. Among patients with malignancy, clinicians should investigate for IEI those with rare neoplasia, virus-associated tumors, and an association with syndromic features, while patients with HLH should always receive an immunological assessment when a clear rheumatologic trigger, underlying malignancy, or well-recognized cause is not evident. The case of MDS and BMF is complex, as new monogenic entities are continuously being described. However, it is pivotal to consider the presence of monocytopenia, warts, vasculitis, and neurological disease, as well as specific cytogenetic abnormalities, such as chromosome 7 monosomy, as warning sings for IEIs. Finally, the main red flags for IEIs in patients with eosinophilia are skeletal/facial abnormalities, recurrent abscesses, refractory eczema, organomegaly, or thrombocytopenia. Full article

Background and Objectives: Retinopathy of prematurity (ROP) persists as a major global cause of preventable childhood blindness. While early diagnosis and timely intervention can significantly mitigate visual loss, research is increasingly focused on identifying novel prognostic factors, with hematological markers emerging as a promising avenue for refining ROP risk prediction. This study aimed to assess the association of hemoglobin levels, red blood cell count, platelet count, and blood transfusions with the risk of developing ROP. Materials and Methods: We conducted a retrospective study involving 140 preterm infants (gestational age ≤ 34 weeks) admitted to a neonatal intensive care unit between 2021 and 2024. Hematological parameters were monitored sequentially during the first 28 days of life, and ROP screening was performed in accordance with international guidelines. Statistical analyses evaluated associations between hematological markers and the risk of developing ROP. Results: Anemia prevalence was significantly higher in infants who developed ROP (83.1%) compared with those who did not (60.3%), conferring an increased risk of ROP (OR = 3.239; p = 0.001). Red blood cell transfusions were linked to a higher likelihood of developing ROP (OR = 3.088; p = 0.001), while platelet transfusions showed a similar association (OR = 2.807; p = 0.027). Platelet counts were significantly lower on days 7, 14, and 21 in the ROP group, and thrombocytopenia was associated with an elevated risk of disease (OR = 3.542; p = 0.001). Conclusions: Early hematological imbalances (anemia, thrombocytopenia) and the requirement for blood product transfusions are significantly associated with an increased risk of ROP. Integrating the monitoring of these specific parameters into existing ROP screening protocols could enhance early identification of vulnerable preterm infants, enabling more targeted surveillance and potential preventative strategies. Full article

Early diagnosis of cystic fibrosis (CF) through newborn screening (NBS) improves clinical outcomes, but in countries like India, delayed diagnosis increases morbidity, mortality, and likely underestimates infant deaths from CF. We performed a retrospective study at a single center in south India from 2017 to 2025 reviewing children diagnosed with CF before one year of age. Patient demographic, clinical, and genetic data were analyzed to characterize early clinical features and identify factors linked to mortality. Of 56 infants diagnosed with CF, 59% survived (median current age 55 months) while 41% died (median age of death 5 months). Key clinical indicators included sibling death with CF-like symptoms, rapid weight loss, and persistent respiratory or nutritional complications. Mortality risk under one year was significantly linked to hypoalbuminemia (OR 9.7), severe malnutrition (OR 4.4), severe anemia (hemoglobin < 7 g/dL) requiring blood transfusions (OR 3.0), and peripheral edema (OR 4.2). A triad of anemia, hypoalbuminemia, and edema was found to strongly predict death (OR 4.2). Integrating clinical checklists of these manifestations into primary healthcare may improve prompt referrals for earlier diagnosis and treatment. Continued education and advocacy for NBS are essential to reduce potentially preventable CF-related deaths in young children. Full article

Background/Objectives: Heart failure (HF) is a leading cause of hospitalization in older adults, with significant sex differences in presentation, treatment, and outcomes. Transitional care models may benefit women more, yet they often receive less follow-up. This study assessed whether the clinical impact of the UMIPIC multidisciplinary HF management program differs by sex. Methods: This prospective, multicenter, observational cohort study included HF patients enrolled in the UMIPIC program or followed through conventional care in the RICA registry. Outcomes (30-day and one-year mortality and readmissions) were compared between groups, stratified by sex. Multivariate Cox models adjusted for age, HF phenotype, comorbidities, and baseline therapy. Results: A total of 5644 HF patients were included, with 2034 (36%) managed in UMIPIC and 3610 (64%) receiving conventional care. Women represented 55% of UMIPIC patients and were older, with higher prevalence of hypertension, anemia, and HF with preserved ejection fraction (HFpEF) compared to conventional care. At 30 days, women in UMIPIC had lower all-cause mortality (4.0% vs. 8.0%), cardiovascular mortality (2.0% vs. 6.0%), and readmissions (9.0% vs. 18.0%; all p < 0.01); these benefits persisted at one year. In multivariate analysis, UMIPIC enrollment remained protective (HR: 0.79; 95% CI: 0.71–0.87; p < 0.001). In men, UMIPIC patients were older with more comorbidities and higher HFpEF prevalence. They also showed lower 30-day mortality (2.0% vs. 8.0%; p < 0.05) and readmissions (8.0% vs. 18.0%; p < 0.01), with benefits maintained at one year. UMIPIC enrollment remained independently associated with reduced one-year mortality in men (HR: 0.79; 95% CI: 0.71–0.88; p < 0.001). Conclusions: The UMIPIC multidisciplinary care model reduced one-year mortality and readmissions in both women and men with HF, supporting integrated care strategies to improve outcomes in this high-risk population. Full article

Autism spectrum disorder (ASD) is a complex neurodevelopmental disease of multifactorial etiologies, manifesting as persistent challenges in social interactions, restrictive interests, and repetitive behaviors. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzymopathy affecting red blood cell function. Although G6PD enzyme deficiency is known for its role in hemolytic anemia, emerging studies have suggested a potential association between G6PD deficiency and neurodegenerative and neurodevelopmental disorders, including autism. This narrative review explores the possible connection between G6PD deficiency and autism by analyzing relevant literature from the PubMed and Scopus databases. Current evidence points to plausible biological links, particularly oxidative stress and folate metabolism, warranting further investigation into G6PD deficiency as a potential risk modifier in ASD. Moreover, further research is necessary to elucidate the nature of this relationship and its implications for clinical practice. Full article

Background and Clinical Significance: Obstructive sleep apnea (OSA) is a common comorbidity in patients with cardiac and metabolic disorders. The coexistence of central sleep apnea with Cheyne–Stokes breathing (CSA-CSB) in heart failure patients, especially those with preserved ejection fraction (HFpEF), represents a diagnostic and therapeutic challenge. Data on continuous positive airway pressure (CPAP) failure and successful adaptation to servo-ventilation (ASV) in the context of complex comorbidities remain limited. Case Presentation: We present the case of a 74-year-old male with a history of type 2 diabetes mellitus, paroxysmal atrial fibrillation, HFpEF, essential hypertension, and bladder carcinoma. He was referred for pre-operative OSA screening, reporting excessive daytime sleepiness, insomnia, and witnessed apneas. Initial respiratory polygraphy revealed severe sleep-disordered breathing with dominant CSA-CSB and moderate OSA. Laboratory investigations also revealed severe iron-deficiency anemia, which was managed with parenteral iron supplementation. The patient underwent CPAP titration, which led to modest improvement and residual high apnea–hypopnea index (AHI). After persistent symptoms and an inadequate CPAP response, an ASV device was initiated with significant clinical and respiratory improvement, demonstrating normalization of hypoxic burden and optimal adherence. Conclusions: CSA-CSB in HFpEF patients with anemia poses unique therapeutic difficulties. This case highlights the importance of individualized diagnostic and therapeutic strategies, including transitioning to ASV in CPAP-refractory cases, which can lead to improved adherence, reduced hypoxia, and better overall outcomes in high-risk patients. Full article

Introduction: Renal malacoplakia is a rare chronic granulomatous disease, often associated with immunosuppression and persistent Gram-negative infections, particularly Escherichia coli. Case Presentation: We present a case involving a 31-year-old woman with hypertension, gestational diabetes, and prior uterine curettage after labor induction for preeclampsia at 23 weeks. She developed urinary sepsis post-procedure. Imaging revealed bilateral nephromegaly, while laboratory tests showed acute kidney injury (KDIGO stage III), anemia, and thrombocytopenia. Blood and urine cultures grew Escherichia coli. Renal biopsy confirmed malacoplakia, demonstrating PAS-positive Michaelis–Gutmann bodies and Von Hansemann cells. The patient responded to prolonged antibiotic therapy and supportive care. Discussion and Conclusion: This case highlights the importance of considering renal malacoplakia in patients with atypical urinary tract infections and nephromegaly, particularly in obstetric settings. Histopathological confirmation is essential, and timely treatment with intracellularly active antibiotics can lead to favorable outcomes. Early diagnosis is critical to prevent irreversible renal damage. Full article

Background: Rapid loss of residual kidney function (RKF) is associated with unfavorable outcomes. We conducted an RCT to compare the effects on RKF preservation of incremental HD between once-weekly HD (1-WHD) and twice-weekly HD (2-WHD). Methods: ESKD patients with an eGFR of 5–10 mL/min/1.73 m² and urine output of ≥800 mL/day were randomly assigned to receive either once-weekly HD (1-WHD) or twice-weekly HD (2-WHD) for 12 months. Patients in the 1-WHD group were prescribed once-weekly HD combined with low-protein diet (0.6 g/kg/day) supplemented with keto-analogues (KAs) 0.12 g/kg/day. In the 2-WHD group, patients received twice-weekly HD with a regular-protein diet. Primary outcomes were changes in RKF by renal clearance and urine volume. Nutritional status, muscle parameters, and quality of life (QoL) were also assessed. Results: A total of 30 incident HD patients were randomized. Baseline RKF, urine volume, and demographic were not different between groups. After 3 months, urine volume was significantly higher in the 1-WHD group than in the 2-WHD group (1921 ± 767 mL/day vs. 1305 ± 599 mL/day, p = 0.02), and these significant findings persisted throughout the entire study period. For RKF, 1-WHD also had a lesser decline in urinary urea (CUrea) and creatinine clearance (CCr) than 2-WHD, with statistically significant differences observed from months 6–12. By month 6, the 1-WHD group exhibited significantly higher CUrea and CCr compared to the 2-WHD group, with CUrea at 3.2 ± 2.3 vs. 1.7 ± 1.0 mL/min (p = 0.03) and CCr at 5.9 ± 3.6 vs. 3.8 ± 1.4 mL/min (p = 0.04), respectively. Serum albumin levels, skeletal muscle mass, anemia status, metabolic parameters, protein-bound uremic toxins, and QoL scores were comparable between the two groups. Conclusions: Incremental HD, starting with once-weekly HD combined with protein restriction supplemented with KAs, appears to better preserve RKF among incident HD patients compared to twice-weekly HD with a regular-protein diet. This HD regimen was also associated with safety in metabolic and nutritional profiles. Full article