World Journal of Pediatrics最新文献

Background: Malignant vasovagal syncope (VVS) is a unique form of cardioinhibitory VVS, characterized by prolonged asystole. To deepen the understanding of this distinct type of VVS in children, this article reviews advancements in the potential pathogenesis, diagnostic approaches, clinical significance, and treatment controversies of malignant VVS in pediatric patients.

Data sources: This article was developed by reviewing the literature and studies in databases including PubMed and Chinese Journal Full-text Database up to September 2024. Search terms included "malignant vasovagal syncope" AND "children" or "vasovagal syncope" AND "asystole" AND "children".

Results: To date, studies focused on malignant VVS in children remain limited. Theoretically, children with malignant VVS are at risk of physical or mental impairment due to this cardiac asystole, though no definite conclusions have been reached. The mechanism underlying the significant cardiac inhibition in malignant VVS remains unclear, and the treatment strategy of malignant VVS is debatable.

Conclusions: The understanding of children with malignant VVS is insufficient. Further research is required to evaluate the clinical features and the pathogenesis of pediatric VVS with cardiac asystole and to establish effective management strategies for malignant VVS. Video Abstract.

Background: Decades of mainstream SIDS research based on the Triple Risk Model and neuropathological findings have failed to provide convincing evidence for a primary CNS-based mechanism behind putative secondary dyshomeostasis (respiratory or cardiac) or impaired arousal. Newly revealed data indicate that severe metabolic acidosis (and severe hyperkalemia) is a common accompaniment in SIDS. This supports the direct effect of sepsis on vital-organ function and occurrence of secondary CNS changes accompanied by the dyshomeostasis leading to SIDS.

Data sources: Using PubMed and Google Scholar literature searches, this paper examines how metabolic acidosis and sepsis might contribute to the underlying pathophysiologic mechanisms in SIDS.

Results: The discovery of a series of non-peer-reviewed publications provided the basis for a serious examination of the role of metabolic acidosis and sepsis in SIDS. Most SIDS risk factors relate directly or indirectly to infection. This consequently elevated the position of septic or superantigenic shock and viremia in causing secondary organ failure leading to SIDS. The latter could include diaphragmatic failure, as evidenced by peripheral respiratory (muscle) arrests in experimental septic shock, as well as infectious myositis and diaphragm myopathy in sudden unexpected deaths, including SIDS. In addition, just as acidosis lowers the threshold for ventricular fibrillation and sudden cardiac arrest, it could also contribute to similarly unstable diaphragm excitation states leading to respiratory failure.

Conclusions: This paper uniquely reveals compelling evidence for a connection between metabolic acidosis, sepsis, viral infections, and sudden unexpected child deaths and provides a solid basis for further work to define which pathway (or pathways) lead to the tragedy of SIDS. It is recommended that all autopsies in sudden unexpected deaths should include pH, bicarbonate, lactate, and electrolyte measurements, as well as diaphragm histology.

Purpose: Cancer predisposition syndromes are genetic disorders that significantly raise the risk of developing malignancies. Although the malignant manifestations of cancer predisposition syndromes are well-studied, recognizing their non-malignant features is crucial for early diagnosis, especially in children and adolescents.

Methods: A comprehensive literature search was conducted using the PubMed database, focusing on non-malignant manifestations of cancer predisposition syndromes in children and adolescents. Key sources included the Clinical Cancer Research pediatric oncology series and ORPHANET. Studies that described clinical signs and symptoms affecting specific organ systems were included.

Results: Non-malignant dermatological features often serve as early indicators of cancer predisposition syndromes, including café-au-lait spots in Neurofibromatosis Type 1 and facial angiofibromas in Tuberous Sclerosis Complex. Neurological and developmental anomalies such as cerebellar ataxia in ataxia-telangiectasia and intellectual disabilities in neurofibromatosis type 1 and tuberous sclerosis complex are significant indicators. Growth and metabolic anomalies are also notable, including overgrowth in Beckwith-Wiedemann syndrome and growth hormone deficiency in neurofibromatosis Type 1. In addition, facial anomalies, ocular manifestations, hearing issues, and thyroid anomalies are prevalent across various cancer predisposition syndromes. For instance, hearing loss may be significant in neurofibromatosis Type 2, while thyroid nodules are common in PTEN hamartoma tumor syndrome and DICER1 syndrome. Cardiovascular, abdominal, musculoskeletal, pulmonary, genitourinary manifestations, and prenatal deviations further complicate the clinical picture.

Conclusions: Recognizing non-malignant features of cancer predisposition syndromes is essential for early diagnosis and management. This organ-specific overview furthers awareness among healthcare providers, facilitating timely genetic counseling, surveillance programs, and preventive measures, ultimately improving patient outcomes.

Background: An increased number of double-negative T (DNT) cells expressing the αβ T cell receptor (αβ⁺DNT cells) is one of the diagnostic criteria for autoimmune lymphoproliferative syndrome (ALPS). Moreover, these cells are expanded in a widely used murine model for lupus. However, the homeostasis of αβ⁺DNT cells remains inadequately investigated in rheumatic disorders, especially in pediatric patients.

Methods: In this cross-sectional, prospective, and observational study, children with rheumatic disorders and healthy controls were recruited to analyze the quantity and characteristics of circulating DNT cells using flow cytometry.

Results: Overall, the two study groups did not differ in their total DNT cell pool in the bloodstream. However, the number of αβ⁺DNT cells was significantly higher in rheumatic children than that in the controls, whereas the γδ⁺DNT cells remained similar. This expansion in the circulating pool of αβ⁺DNT cells was comparable across different rheumatic diseases, all showing significant differences from the controls in this regard. Moreover, no significant correlation was found between αβ⁺DNT cell numbers and disease activity.

Conclusions: These preliminary results indicate that circulating αβ⁺DNT cells are significantly expanded in children with rheumatic disorders; however, this finding appears to be a nonspecific (disease-unrelated) marker of autoimmunity. Further and larger studies are necessary to better investigate and define the role of DNT cells in pediatric rheumatic diseases.

Background: Pertussis resurgence has been reported worldwide in the past two decades. Pertussis is still endemic and difficult to control though with universal vaccination in children. The resurgence may be related to multiple variables, such as increased disease awareness and laboratory tests, waning of immunity following vaccination, and/or genetic mutations of Bordetella pertussis. For better pertussis prevention, diagnosis, and management, we called up an expert panel to develop this expert consensus to provide new concepts in diagnosis and treatment for clinical practice.

Data sources: The expert groups collected clinical evidence, summarized their clinical experiences, evaluated preliminary recommendations or guidelines, and then organized open-ended discussions to form the recommendations. This consensus was developed by reviewing the literature and studies in databases, including PubMed, Cochrane, EMBASE, the China Biomedical Database, and the Chinese Journal Full-text Database up to May 2024. The search terms included "pertussis" or "whooping cough", "children", "diagnosis", and "treatment".

Results: The burden of pertussis has also changed from infants to  school children and adults, and these age groups have consequently become the main source of infection for vulnerable population including infants and newborns. In China, a high prevalence of erythromycin-resistant Bordetella pertussis (ERBP) has been reported in the past decade. ERBP may lead to failed clinical empirical treatment with macrolides, which poses a great challenge for pertussis management and control. For better management of pertussis, a flow diagram for diagnosis and treatment of pertussis was presented in this consensus. This consensus also described the diagnostic criteria for pertussis, high-risk cases, and severe pertussis. Macrolides can still be used to treat confirmed erythromycin-sensitive B. pertussis (ESBP) infections, whereas oral trimethoprim-sulfamethoxazole therapy is the initial treatment option for children older than two months. For infants younger than two months, severe patients, or those exhibiting a high degree of sulfonamide allergy, intravenous administration of piperacillin or cefoperazone-sulbactam is advised.

Conclusions: This expert consensus provides a comprehensive guidance and a reference for the diagnosis and treatment of pertussis in children.

Background: Seroprevalence studies across various countries can contribute to achieving the elimination target for measles and rubella. However, in the Mainland of China, the concept of herd immunity remains unclear due to the lack of a nationwide serosurvey.

Methods: This systematic review and meta-analysis was conducted by retrieving literature reporting the seroprevalence of measles and rubella published between 2012 and 2023. The pooled positive rates and estimated geometric mean concentrations (GMCs) of measles and rubella immunoglobulin G antibodies were calculated.

Results: This study analysed 135 studies on measles and 77 on rubella, including data from 368,023 and 177,422 healthy individuals, respectively. Between 2010 and 2022, the overall pooled positive rates for measles and rubella antibodies were 88.8% and 79.91%, respectively. The age-specific susceptibility analysis showed that infants aged < 1 year had the lowest pooled positive rates. Other age groups had a roughly U-shaped distribution, with relatively higher positive rates and GMC of measles and rubella antibodies in young children and older age groups. However, the positive rates for both measles and rubella antibodies fell below the elimination threshold in almost all age groups other than young children aged 1-4 years, especially in recent years. In addition, antibody positivity rates varied by geographical region and decreased with economic level.

Conclusion: Our findings provide preliminary insights into herd immunity for measles and rubella, highlighting the challenges to achieving their elimination in China.