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06 June 2024, Volume 39 Issue 6 Previous Issue   

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Expert consensus on the management of dermatogenic chronic pruritus in children Group of Dermatology, Professional Committee of Child Allergology, China Maternal and Child Health Association 2024, 39(6): 401-408.  DOI: 10.19538/j.ek2024060601 Abstract ( )   Pruritus occurs in many diseases， and in children it is mostly caused by skin disorders， namely dermatogenic pruritus. Persistent chronic pruritus（CP） is difficult to treat and can have a negative impact on children's sleep， mood， cognition， school performance， social and family functions， etc.， seriously affecting the quality of life of children and their family members. There is an urgent clinical need for standardized management of CP in children. However， there is currently a lack of independent guidelines or consensus on the diagnosis and treatment of CP in children at and abroad domestic. Based on evidence-based medicine and expert opinions， this consensus formulates a management plan for dermatogenic CP in children aged 0-18 for reference by clinical doctors.

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Interpretation of the 2024 International Consensus Criteria for Pediatric Sepsis and Septic Shock HUANG Han-wu, ZHAO Zhe, WANG Yi, et al 2024, 39(6): 409-416.  DOI: 10.19538/j.ek2024060602 Abstract ( )   Sepsis is the main cause of death of children in pediatric intensive care unit， which seriously threatens the life and health of children， and septic shock is the stage of cardiovascular system damage of sepsis， which is its most serious manifestation. The standardized diagnosis of sepsis and septic shock is very important， and the lack of understanding of clinicians may delay the diagnosis and treatment， resulting in the continuous progression of the disease， multiple organ function injury and even death. In 2024， the Society of Critical Care Medicine （SCCM） Working Group on Definitions of Sepsis in Children issued a new Phoenix Sepsis Criteria for Sepsis and Septic Shock in children. There is potential to improve clinical care， epidemiological assessment and research of sepsis and septic shock in children around the world. The limitations of the current criteria for pediatric sepsis， the formulation and definition of a new Phoenix criteria for pediatric sepsis， the content of Phoenix criteria，the comparison between the new and the current criteria， the precautions and limitations of the new criteria are interpreted for clinical reference， in order to improve the standardized diagnosis of sepsis and septic shock of children by pediatricians.

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Interpretation of ELSO guidelines： routine neurological monitoring of neonatal and pediatric patients supported by extracorporeal membrane oxygenation ZHANG Yue, ZHAO Shi-guo, YANG Zi-hao, et al 2024, 39(6): 417-421.  DOI: 10.19538/j.ek2024060603 Abstract ( )   The extracorporeal life support organization （ELSO） issued a guideline for routine neurological monitoring of neonatal and pediatric supported by extracorporeal membrane oxygenation （ECMO）， with the aim of accumulating knowledge and making useful and safe recommendations for clinicians and other healthcare professionals in the assessment of brain injury in pediatric populations supporting by ECMO This paper mainly interprets the clinical recommendations in order to provide reference for pediatric clinicians in China.

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Focus on the progress of systemic autoinflammatory diseases GAO Si-hao, SONG Hong-mei 2024, 39(6): 422-424.  DOI: 10.19538/j.ek2024060604 Abstract ( )   Systemic autoinflammatory diseases （SAIDs） affect various organ systems throughout the body. The expanding spectrum of these diseases， along with new classifications， increased heterogeneity of phenotypes， and research into genetic mechanisms has advanced our understanding of basic science theories with improved precision diagnosis and treatment. Because these diseases affect multiple organs or systems， pediatricians across different specialties are required to enhance their knowledge and attention in this field.

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Progress in the treatment of systemic autoinflammatory diseases GAO Si-hao , SONG Hong-mei 2024, 39(6): 425-432.  DOI: 10.19538/j.ek2024060605 Abstract ( )   Systemic autoinflammatory diseases（SAIDs） are a group of genetic disorders characterized by recurrent or persistent inflammatory responses and multi-system involve-ment， where the innate immune system plays a major role in disease pathogenesis. Although pathway -directed targeted therapies such as JAK inhibitors， IL-1 pathway inhibitors， and TNF inhibitors have achieved good response in some SAIDs. New targets and treatment strategies， powered by the understanding of disease mechanisms， the discovery of new genes， and the proposal of new classifications， are continuously applied in clinical practice and research. This article reviews the progress in the treatment of SAIDs in the past three years， introduces the updates in SAIDs treatment， and aims to promote the recognition， diagnosis， treatment， and research of such diseases in the future.

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Research progress of inflammasome-associated autoinflammatory diseases WU Jun-feng, TANG Xue-mei 2024, 39(6): 432-438.  DOI: 10.19538/j.ek2024060606 Abstract ( )   The inflammasome is a macromolecular multi-protein complex in cells， and its activation can regulate the activation of cysteine aspartic protease-1 （CASP1）， promote the activation and secretion of IL-1β and IL-18， and cause inflammation in the body. In recent years， researchers have conducted extensive studies on the molecular composition and activation mechanisms of different inflammasomes， and a variety of inflammasome molecules have been characterized， among which the self-activating mutations of four inflamm-asome can lead to single-gene autoinflammatory diseases in humans. This review focuses on the research progress of the pathogenesis， clinical manifestations and treatment of NLRP1， CARD8， NLRP3， NLRC4 and Pyrin inflammasome-related autoinflammatory diseases.

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Progress in diagnosis and treatment of non-inflammasom-apathies HUANG Yan-yan, YANG Jun 2024, 39(6): 439-444.  DOI: 10.19538/j.ek2024060607 Abstract ( )   Non-inflammasomapathies is a group of autoinf-lammatory diseases. The clinical manifestations are complex. The common clinical manifestations are repeated fever， rash， arthritis， eye inflammation， and increased inflammatory protein in the acute phase. Genetic examination should be further improved to confirm the diagnosis. There are no guidelines for the treatment of non-inflammasomapathies. Glucocorticoids， non-steroidal anti-inflammatory drugs， immunosuppressives and biological agents have been applied， but the efficacy is different. In this review， the pathogenesis， clinical manifestations， diagnosis and treatment experience were discussed， so as to provide new insights for the understanding of these diseases.

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Research progress of typeⅠinterferonopathy YU Zhong-xun, GAO Si-hao, MA Ming-sheng 2024, 39(6): 444-448.  DOI: 10.19538/j.ek2024060608 Abstract ( )   Type I interferonopathy is a group of novel diseases characterized by excessive upregulation of type I interferons due to specific gene mutations， leading to systemic multi-systemic inflammatory reactions. This article reviews recent research progress in the pathogenesis， disease types， and therapeutic advances of type 1 interferonopathy.It highlights key pathogenic mechanisms such as abnormalities in nucleic acid sensing pathways and various new type 1 interferonopathies including ARF1 defects，STAT2 loss-of-function mutations， JAK1 gain-of-function mutations， ATAD3A mutations，and RELA mutations. Although evidence-based therapeutic strategies are currently lacking，treatments such as glucocorticoids，anti-IL-1，anti-IL-6，anti-interferon receptor therapies，and JAK inhibitors can partially alleviate disease progression.

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Neurological damage of systemic autoinflammatory diseases ZOU Li-ping 2024, 39(6): 448-453.  DOI: 10.19538/j.ek2024060609 Abstract ( )   Systemic autoinflammatory diseases （SAIDs） is a group of diseases caused by systemic inflammatory response due to dysfunction of the innate immune system. Due to genetic mutations causing changes in the encoded protein， it is also known as hereditary autoinflammatory disease. SAIDs have a wide variety of types， clinical manifestations and pathogenesis. It is classified into different types based on its clinical characteristics and pathogenesis. These diseases are more common in childhood and can affect multiple systems throughout the body. Among them， neurological damage is an important factor affecting the clinical prognosis of patients. Due to insufficient understanding of SAIDs， neurological damage often presents as non-specific symptoms and is easily misdiagnosed. Early identification， diagnosis， and initiation of treatment are of great significance for improving clinical prognosis.

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Hematological manifestations of autoinflammatory diseases REN Jia-ning, ZHU Xiao-fan 2024, 39(6): 453-456.  DOI: 10.19538/j.ek2024060610 Abstract ( )   Autoinflammatory diseases （AIDs） are a group of genetic diseases that have attracted high attention in recent years， and their main characteristics are systemic inflammatory responses caused by innate immune dysregulation， and unlike autoimmune diseases， they usually lack autoantibodies or antigen-specific T cells. AIDs are more common than juvenile-onset illness and can affect multiple systems throughout the body， with the hematologic system being the most common site of AIDs involvement， and they share many common clinical features with hematologic neoplasms， such as anemia， lymphadenopathy， and/or splenomegaly. While hematopoietic cells help create and propagate a protective inflammatory response to infection or injury， excessive inflammation can lead to many diseases of the blood， bone marrow， and lymphatic system . This article focuses on several common hematologic manifestations of AIDs in order to raise awareness among haematologists about this type of disease and to avoid misdiagnosis or mistreatment.

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Kidney injury in systemic autoinflammatory diseases WANG Da-hai, CHANG Hong 2024, 39(6): 456-462.  DOI: 10.19538/j.ek2024060611 Abstract ( )   The kidney is a major target organ of systemic autoinflammatory diseases. The clinical manifestations vary from proteinuria， nephrotic syndrome， and renal insufficiency which may end up requiring renal failure or death. The pathogenetic mechanisms are likewise quite diverse， ranging from amyloidosis caused by persistent or recurrent inflammation to inflammasome activation-mediated vasculitis/vasculopathy and several other uncommon types of glomerulonephritis. It is critical to increase the understanding and early identification of autoinflammatory diseases， as well as acquire pathology in time when renal injury occurs， to enhance disease recovery and prognosis.

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Immunodeficiency with autoinflammation in inborn errors of immunity DU Hong-qiang , ZHAO Xiao-dong 2024, 39(6): 462-466.  DOI: 10.19538/j.ek2024060612 Abstract ( )   Inborn errors of immunity （IEIs） are a type of monogenic genetic disease caused by heritable mutation in genes encoding immune molecules， with the classic clinical manifestations being recurrent infections. Monogenic autoinflammatory diseases （mAID） represent a relatively new subclass of IEI， characterized by non-infectious hyperin- flammation. In recent years， an increasing number of complex IEIs have been discovered， with some patients exhibiting both immunodeficiency and autoinflammatory manifestations. This article provides a brief summary and introduction to the clinical manifestations of these IEIs， aiming to update clinicians' understanding of IEI or mAID and reduce misdiagnosis and under-treatment.

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Clinical analysis of 68 cases of infantile pneumonia with bronchopulmonary dysplasia XIA Lei, ZHANG Ying-yan, CHENG Hui-qing, et al 2024, 39(6): 467-472.  DOI: 10.19538/j.ek2024060613 Abstract ( )   Objective    To explore the clinical characteristics of infantile pneumonia in children with different degrees and months of bronchopulmonary dysplasia. Methods    Retrospective collection of medical records of 68 cases with bronchopulmonary dysplasia （BPD） who re-entered in infancy at the Third Affiliated Hospital of Zhengzhou University from January 2020 to December 2021. Results    Among 68 cases（132 cases） who re-entered BPD were included.  Compared with the mild BPD group， the moderate-severe BPD group had a higher proportion of neonates who received repeated use of pulmonary surfactant due to neonatal respiratory distress during the neonatal period， a longer duration of mechanical ventilation （invasive and non-invasive） and a longer total duration of oxygen therapy during hospitalization. The clinical manifestations are mainly wheezing， coughing， coarse wet rales， and wheezing，the differences are statistically significant in the comparison of different months groups （P<0.05）. Respiratory pathogens are mainly Gram-negative bacillus， and the top three special pathogens are Klebsiella pneumoniae pneumoniae， Pseudomonas aeruginosa， Staphylococcus aureus; respiratory virus detected mainly respiratory syncytial virus. The average median hospitalization time of re-entered for 13 （8-21） days; 60.6% of re-entered （80/132） progressed to severe pneumonia， 28.8% （38/132） combined with respiratory failure. The children in the moderate/severe group had higher incidence of days， for hospitalization time and severe pneumonia， and the difference is statisically significant in the June-December months group（P<0.05）. Further logical regression analysis shows that moderate BPD （OR：2.842 95% CI：1.138~7.100） and severe BPD （OR：3.336 95% CI：1.085~10.259） are risk factors for respiratory failure. Conclusion    BPD， especially moderate/severe BPD premature infants， have serious pneumonia and have been hospitalized time for a long time. It is prone to severe pneumonia and respiratory failure. Special attention should be paid to respiratory infection.

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Clinical analysis of 198 cases of interstitial lung disease in children CHEN Qi-hong, GE Dan-dan, YANG yun-gang 2024, 39(6): 473-476.  DOI: 10.19538/j.ek2024060614 Abstract ( )   Objective    To analyze the clinical data of interstitial lung disease （ILD） in children， in order to improve the understanding of this disease among clinical doctors. Methods    Retrospective analysis of clinical data of pediatric patients with ILD who were hospitalized in the First Affiliated Hospital of Xiamen University  from December 2015 to April 2023， including gender， age， symptoms， bronchoscopic manifestations， imaging manifestations， lung function， primary disease， concomitant diease， disease burden and outcomes.Results    A total of 198 children were diagnosed， with 110 males and 88 females; The age of onset was 31.00 months （1.43-156.00 months）.The most common respiratory symptoms in children with ILD were cough in 130 cases （65.7%）， expectoration in 117 cases （59.1%）.The most common systemic symptoms were fever in 72 cases （36.4%）; 31 cases （15.7%） had different degrees of malnutrition. Among the etiology，134 cases（67.7%）were related to alveolar structure disorder. 59 cases（29.8%） were associated with systemic dieases. 3 cases（1.5%） were unigue to infancy. 2 cases（1.0%） were related to environmental exposure.Among them， 129 cases （96.3%） of pulmonary infection were the most common cases related to alveolar structural disorders; Systemic diseases were common in 25 cases （42.4%） of juvenile idiopathic arthritis， followed by 10 cases （16.9%） of systemic lupus erythematosus.The HRCT manifestations of the chest were ground glass shadow in 192 cases （97.0%）， subpleural patchy shadow in 162 cases （81.8%）.Under bronchoscopy， various degrees of bronchial intimal inflammation were observed in all 111 children. Among them， 16 cases （8.1%） were accompanied by tracheomalacia in different parts， 3 cases （1.5%） were accompanied by laryngeal cleft， 4 cases （2.0%） had tracheal bronchi. A total of 77 cases （38.9%） completed lung function examinations， 26 cases （33.8%） were normal， 28 cases （36.3%） had varying degrees of ventilation dysfunction， 8 cases （10.4%） had varying degrees of diffusion dysfunction， and 15 cases （19.5%） had mixed ventilation dysfunction. Conclusion    The age distribution of children with ILD is wide， and only some children have respiratory symptoms. HRCT manifestations of the chest are diverse， which helps in the early diagnosis of ILD，the control of the primary disease， and regular respiratory follow-up are the key to the diagnosis and treatment of ILD.

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A case report on congenital rubella syndrome complicated with interstitial pneumonia CHEN Jing-lun, LIANG Yu-feng, TAO Jian-ping 2024, 39(6): 477-480.  DOI: 10.19538/j.ek2024060615 Abstract ( )   Congenital rubella syndrome is a disease that usually starts in infancy and is caused by rubella infection in the mother's uterus. The main manifestations are hearing impairment， congenital heart malformation， cataract/congenital glaucoma and pigmentary retinopathy， and a few may be complicated by interstitial pneumonia. Nowadays CRS is rare，and there has been no report on cases of CRS complicated with interstitial pneumonia. This article reports a case of URS complicated with interstitial pneumonia starting in infancy who were hospitalized in the Guangzhou women and Children’s Medical Center from November 29，2020. The main clinical manifestations were interstitial pneumonia， cataract， hearing impairment， congenital heart malformation， etc. Serum rubella virus-IgM was positive， the alveolar lavage fluid was detected by high throughput seguencing and rubella virus was found， and chest CT showed uniform ground glass interstitial changes in both lungs， which confirmed the diagnosis of CRS complicated with interstitial pneumonia. This article aims to study the harm and prognosis of the disease and strengthen the attention of clinicians by analyzing the pathogenesis， clinical manifestations， laboratory indicators， imaging manifestations，treatment and prognosis of CRS complicated with interstitial pneumonia.