Background: Emerging evidence suggests that patients with coronary artery disease carry an increased risk of developing malignancy, with deleterious effects on long-term prognosis. Our aim was to ascertain whether baseline plasma lipid levels during acute coronary syndrome (ACS) are associated with malignancy in long-term. Methods: This study included 589 patients admitted with ACS to three centers and discharged alive. Plasma lipid levels were assessed on the first morning after admission. Patients were followed for 17 years or until death. Results: Five hundred seventy-one patients were free from malignancy at enrollment, of them 99 (17.3%) developed the disease during follow-up and 75 (13.1%) died due to it. Compared to patients without malignancy, those with malignancy showed lower plasma levels of total cholesterol (TC), low-density lipoprotein (LDL), and triglycerides (TG). The groups showed similar statin use rates at any time in follow-up. The incidence rate of neoplasia and neoplastic mortality was higher in patients with baseline TC or LDL values ≤ median; they showed 85 and 72% increased incidence rate of developing malignancy and 133 and 122% increased incidence rate of neoplastic death respectively. No differences were observed relative to HDL and TG levels. In survival analysis using Cox regression with parsimonious models, patients with baseline TC or LDL values > median, respectively, showed risks of 0.6(95% CI 0.4–0.9; p = 0.01) and 0.6(95%CI 0.4–0.9; p = 0.02) for malignancy onset, and 0.5(95% CI 0.3–0.8; p = 0.005) and 0.5(95% CI 0.3–0.8; p = 0.004) for neoplastic death. Similar results were obtained using competitive risk analysis with parsimonious models. Conclusions: This long-term prospective study of an unselected real-world patient sample showed that neoplasia onset and mortality are independently associated with low plasma TC and LDL levels at admission for ACS.

Background: Neurofibromatosis type 1 (NF1) is a rare multisystem disorder characterized by variable expressivity and increased tumor risk. Café-au-lait macules (CALMs) are a hallmark of the disease, often representing one of the earliest clinical manifestations and allowing a clinical NF1 diagnosis if six or more are present. In this study, we aimed to investigate the prognostic value of CALMs at birth in NF1 patients. Methods: We conducted a retrospective study in patients aged ≥ 4 years presenting with CALMs at our Institution between 2020 and 2021, with a minimum follow-up of four years. We retrospectively collected data on CALMs at birth and other clinical manifestations associated with NF1. Results: Among 208 patients evaluated, including 147 with a confirmed diagnosis of NF1, 110 did not show CALMs at birth, and 98 had at least one. The absence of CALMs at birth did not correlate with a lower likelihood of NF1. In contrast, the CALM number at birth directly correlated with the likelihood of NF1, up to 95% in patients with ≥5 macules. Additionally, a higher number of CALMs correlated with a greater prevalence of plexiform neurofibromas (p < 0.001). Conclusions: Our findings suggest that a higher number of CALMs may indicate a more severe form of NF1, with an increased risk of plexiform neurofibromas. These results emphasize the importance of a comprehensive evaluation of patients with CALMs, especially in case of multiple lesions, aiming at implementing early NF1 diagnosis, follow-up strategies, and overall patient management.

We report a child with massive, chronic idiopathic pericardial effusion (PE), initially detected incidentally and asymptomatically. Despite multiple treatment attempts, including Nonsteroidal anti-inflammatory drugs, corticosteroids, colchicine, and repeated pericardiocentesis, the effusion persisted and recurred. Advanced imaging revealed a stable but severe PE over the years, with no hemodynamic compromise. Cytokine analysis of the pericardial fluid showed markedly elevated interleukin (IL)-6 levels, suggesting an underlying inflammatory process despite the absence of classic clinical signs. Based on this finding, the patient was successfully treated with anakinra, an IL-1 receptor antagonist, resulting in sustained remission for 24 months. This case highlights the potential role of biologic therapy in selected pediatric patients with chronic idiopathic PE, even when traditional markers of inflammation are absent. Cytokine profiling may help identify candidates for anti-IL-1 treatment, providing a valuable therapeutic option where standard approaches fail.

Myocarditis is a significant cause of cardiac morbidity in children, with viral infections, including Parvovirus B19 (PVB19), among the most implicated agents. Recent epidemiological reports suggest a resurgence of PVB19 infections post-COVID-19, raising concerns about associated myocarditis. We aimed to retrospectively analyze clinical, laboratory, and imaging data of patients with PVB19-associated myocarditis in 2024. We included 5 pediatric patients admitted with acute myocarditis and positive blood PVB19-DNA. Median age was 6.4 years; 80% were male. Most patients presented with cardiogenic shock; two required extracorporeal membrane oxygenation. Echocardiography revealed severely reduced left ventricular ejection fraction (median 25%), atrial enlargement, and mitral insufficiency. No direct correlation was found between PVB19 DNA load and disease severity. Immunomodulatory therapy, including IVIG (intravenous immunoglobulin) and corticosteroids, was administered in most cases, with variable outcomes. Three patients had viral coinfections, raising concern for a potential link with worse outcomes. Following the SARS-CoV-2 pandemic, an apparent increase in pediatric myocarditis has been observed. The 2024 outbreak of PVB19 infections was associated with an unusual number of fulminant myocarditis cases, particularly in infants. However, our small sample precludes definitive conclusions. Larger multicenter studies are needed to clarify the role of PVB19 and coinfections in pediatric myocarditis.

Background and Clinical Significance: Scurvy, caused by chronic vitamin C deficiency, is re-emerging in Western countries, particularly among pediatric patients with highly selective diets. While its musculoskeletal and mucocutaneous manifestations are well-known, its association with pulmonary arterial hypertension (PAH) is rare and poorly understood. Ascorbic acid and iron are essential cofactors for prolyl hydroxylases (PHD), which regulate Hypoxia-Inducible Factors. Their combined deficiency may trigger a “pseudohypoxic” state, leading to pulmonary vascular remodeling and vasoconstriction. Case Presentation : A 30-month-old female presented with a one-month history of limping, lower limb pain, and gingival hypertrophy. Dietary history revealed an almost exclusive cow’s milk-based intake. Physical examination showed diffuse petechiae, pallor, and right knee edema. Laboratory findings confirmed scurvy (undetectable vitamin C), severe iron-deficiency anemia (Hb: 72 g/L; ferritin: 22 mcg/L; RDW: 30%), folate deficiency, and hyperhomocysteinemia. Notably, elevated copper and vitamin B12 levels suggested a state of metabolic dysregulation. Echocardiography revealed moderate PAH phenotype (estimated sPAP: 47–50 mmHg) and a hyperdynamic contractility. A “perfect storm” mechanism was hypothesized, involving iron–ascorbate-dependent PHD impairment, high-output state, and oxidative-stress-induced hepcidin dysregulation (suggested by elevated copper). Following intravenous vitamin C and multivitamin supplementation, pulmonary pressures normalized within one week. Conclusions: PAH phenotype in scurvy represents a reversible metabolic disruption of pulmonary vascular tone rather than a structural disease. This case underscores the synergistic role of vitamin C, iron, and folate in vascular homeostasis. Clinicians should maintain high suspicion for scurvy in children with selective diets and unexplained PAH, as nutritional restoration is curative.