Recent Advances for Global Perspectives on Etiology, Pathophysiology, Clinical Presentations, and Management of Moyamoya Disease

Moyamoya disease (MMD) is a condition characterized by the gradual narrowing and blockage of blood vessels in the brain, specifically those in the circle of Willis and the arteries that supply it. This results in reduced blood flow and oxygen to the brain, leading to progressive symptoms and potential complications. The underlying pathophysiological mechanism remains elucidated. However, recent studies have highlighted numerous etiologic factors: abnormal immune complex responses, susceptibility genes, branched-chain amino acids, antibodies, heritable diseases, and acquired diseases, which may be the great potential triggers for the development of moyamoya disease. Its clinical presentation has varying degrees from transient asymptomatic events to significant neurological deficits. Moyamoya disease (MMD) shows different patterns in children and adults. Children with MMD are more susceptible to ischemic events due to decreased blood flow to the brain. Conversely, adults with MMD are more prone to hemorrhagic events involving brain bleeding. Children with MMD may experience a range of symptoms including motor impairments, sensory issues, seizures, headaches, dizziness, cognitive delays, or ongoing neurological problems. Although adults may present with similar clinical symptoms as children, they are more prone to experiencing sudden onset intraventricular, subarachnoid, or intracerebral hemorrhages. One of the challenges in moyamoya disease is the potential for misdiagnosis or delayed diagnosis, particularly when physicians fail to consider MMD as a possible cause in stroke patients. This review aims to provide a comprehensive overview of recent global studies on the pathophysiology of MMD, along with advancements in its management. Additionally, the review will delve into various surgical treatment options for MMD, as well as its rare occurrence alongside atrioventricular malformations. Exciting prospects include the use of autologous bone marrow transplant and the potential role of Connexin 43 protein treatment in the development of moyamoya disease.

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,long-term effects and potential risks, such as renal dysfunction and reproductive impacts, require further investigation. The effectiveness of hydroxyurea in young children with SCD has been demonstrated in various clinical trials and observational studies. These studies have shown a significant reduction in disease-related complications and improved quality of life. However, optimal dosing, treatment duration, and long-term outcomes are still areas of ongoing research. This review focuses on recent studies investigating the benefits, effectiveness, responses, and safety of hydroxyurea in pediatric individuals diagnosed with sickle cell disease.

The Prevalence and Clinical Manifestations of Co-Infection in Pediatric Infectious Mononucleosis: A Single-Centered, Retrospective Study

Background: Recent studies indicate that the incidence of infectious mononucleosis (IM) has increased in China. Furthermore, it has been shown that children diagnosed with IM are prone to acquiring other pathogens. However, there is limited research on the prevalence of these co-infections in children with IM. Thus, we conducted this study to determine the prevalence of coinfections and common pathogens, as well as to compare clinical manifestations in children with and without coinfections. Methods: This retrospective observational study was conducted at the Department of Pediatrics Zhongnan Hospital of Wuhan University, Wuhan, China, with data from January 2018 to January 2023. Data, including demographics, symptoms, lab results, and complications, were collected from the hospital’s electronic database and analyzed. The statistical analysis included descriptive statistics, independent samples t-tests and Mann-Whitney tests to compare the means of continuous variables. Statistical significance was determined by p-values less than 0.05. Results: The study involved 216 participants diagnosed with IM, predominantly males (61.6%) aged 0 - 4 years (50.9%). Coinfection was detected in 39.8% of children, with multiple pathogens present in 33.72% of these cases. Among coinfection cases, 40% occurred in children under 5 years old, and females made up 54.2% of these cases. Mycoplasma pneumoniae (MP) was the most prevalent pathogen, accounting for 18.1% of cases. Influenza B (IFB) and Influenza A (IFA) viruses were found in 16.7% and 13.9% of participants, respectively, indicating a notable occurrence of respiratory pathogen coinfections. Male gender, fever, tonsillopharyngitis, lower HGB levels, higher ESR, CRP, and AST levels were correlated with coinfections. Conclusion: In summary, the study revealed a high prevalence of coinfections among children diagnosed with IM, particularly involving Mycoplasma pneumoniae and influenza viruses. These coinfections were notably common in children under 5 years old and were more frequent among females. Clinical manifestations such as fever and tonsillopharyngitis, along with specific laboratory findings including lower hemoglobin levels, elevated ESR, CRP and AST levels, were found to be correlated with coinfections.

Association between Supraventricular Tachycardia and Necrotizing Enterocolitis: A Case-Control Study

Background: Necrotizing enterocolitis (NEC) is the most common and fatal gastrointestinal disease encountered in the Neonatal Intensive Care Unit. Several case reports have shown an association between supraventricular tachycardia and necrotizing enterocolitis. This study aimed to determine the association between supraventricular tachycardia and necrotizing enterocolitis. Methods: This study was conducted from April 1st, 2016 to March 31st, 2022, at the Department of Pediatrics, Zhongnan Hospital of Wuhan University, Hubei, China. The records of 74 subjects with the diagnosis of necrotizing enterocolitis (NEC) were obtained from the hospital’s medical data records. Consequently, 74 gender, gestational age, and birth weight-matched controls (babies without NEC) were recruited as controls. Results: Of the 74 cases, 47.3% of the cases were males, and 52.7% were females. Regarding the birth weight and gestational age, 77% of the cases had low birth weight (LBW) and 86.5% were premature. In terms of Apgar score, 93.2% of NEC cases had an Apgar score of >7 at five minutes. The median values of white blood cells, platelets, and hemoglobin of cases were 10.90 (8.09, 13.80), 227 (169.75, 295.50), and 155.6 (130.53, 170.95), respectively. No Association between supraventricular tachycardia and necrotizing enterocolitis (P = 1.00). Conclusion: No association between necrotizing enterocolitis and supraventricular tachycardia was found. Further multicenter-based studies examining whether there is a potential relationship exists between supraventricular tachycardia and the development of necrotizing enterocolitis are required.

Diagnosis, treatment, and prevention of 2019 novel coronavirus infection in children: experts' consensus statement

Since the outbreak of 2019 novel coronavirus infection (2019-nCoV) in Wuhan City,China,by January 30,2020,a total of 9692 confirmed cases and 15,238 suspected cases have been reported around 31 provinces or cities in China.Among the confirmed cases,1527 were severe cases,171 had recovered and been discharged at home,and 213 died.And among these cases,a total of 28 children aged from 1 month to 17 years have been reported in China.For standardizing prevention and management of 2019-nCoV infections in children,we called up an experts' committee to formulate this experts' consensus statement.This statement is based on the Novel Coronavirus Infection Pneumonia Diagnosis and Treatment Standards (the fourth edition) (National Health Committee) and other previous diagnosis and treatment strategies for pediatric virus infections.The present consensus statement summarizes current strategies on diagnosis,treatment,and prevention of 2019-nCoV infection in children.

A respiratory syncytial virus persistent-infected cell line system reveals the involvement of SOCS1 in the innate antiviral response

HEp-2 cells persistently infected with respiratory syncytial virus(RSV) are a heterogeneous mixture of viral antigen-positive and-negative variants; however, the mechanism through which viral replication becomes latent remains unclear. In this study, we investigated the potential mechanism by which RSV escapes from innate immune surveillance. Persistent-infected RSV HEp-2 cells were isolated and cell clones were passaged. The RSV-persistent cells produced viruses at a lower titer, resisted wild-type RSV re-infection, and secreted high levels of interferon-β(IFN-β), macrophage inflammatory protein-1α(Mip-1α), interleukin-8(IL-8), and Rantes. Toll-like receptor 3(TLR3), retinoic acid inducible gene-I(RIG-I), and suppressor of cytokine signaling 1(SOCS1) levels were upregulated in these cells. The silencing of TLR3 m RNA decreased the expression of SOCS1 protein and the secretion of cytokines. RSV-persistent cells are in an inflammatory state; upregulation of SOCS1 is related to the TLR3 signaling pathway, which could be associated with the mechanism of viral persistence.