1.Childhood-onset Takayasu arteritis: Unique characteristics and outcomes from a Singapore cohort.
Amanda Xin Yi YAP ; Junjie HUANG ; Kai Liang TEH ; Lena DAS ; Yun Xin BOOK ; Sook Fun HOH ; Xiaocong GAO ; Thaschawee ARKACHAISRI
Annals of the Academy of Medicine, Singapore 2025;54(9):531-541
INTRODUCTION:
Takayasu arteritis is the most common large-vessel vasculitis in childhood, but there is a lack of literature regarding childhood-onset Takayasu arteritis (c-TAK) in Southeast Asia. We aim to describe a c-TAK cohort in Singapore and highlight a unique subset that first presents with Kawasaki-like disease (KD).
METHOD:
A single-centre cohort study in Singapore of consecutive children diagnosed with c-TAK between 2002 and 2023 was performed. Demographic and clinical features, laboratory and angiographic findings, treatment, and outcomes were summarised. Disease activity was evaluated using the Paediatric Vasculitis Disease Activity Score and inflammatory markers.
RESULTS:
Twenty-three patients, fulfilling both the EULAR/ PRINTO/PReS and ACR/EULAR 2022 criteria, were recruited. The most common clinical features at diagnosis were fever (15, 65%) and neurological symptoms (11, 48%, half of which presented with stroke), while the most prevalent angiographic pattern by Hata's classification was Type V (21, 91%). Eight children (35%) initially presented with refractory KD, and these patients were significantly younger, more male-predominant, and had higher inflammatory markers at diagnosis; all of them had coronary artery involvement, but none had intracranial vascular findings. Of the entire cohort, 16 (70%) achieved inactive disease on medications with a median duration of 6 months (interquartile range [IQR]: 4-11), and 8 (35%) achieved remission off medications with a median duration of 43 months (IQR 35-60).
CONCLUSION
Our c-TAK cohort has high proportions of neurological involvement and stroke. This is also the first cohort study to describe a distinct group of patients who first presented with refractory KD.
Humans
;
Takayasu Arteritis/complications*
;
Singapore/epidemiology*
;
Male
;
Female
;
Child
;
Adolescent
;
Age of Onset
;
Mucocutaneous Lymph Node Syndrome/diagnosis*
;
Cohort Studies
;
Child, Preschool
;
Fever/etiology*
;
Stroke/epidemiology*
;
Retrospective Studies
2.Febrile infection-related epilepsy syndrome caused by hemophagocytic lymphohistiocytosis: a case report.
Xiao-Lu DENG ; Li-Fen YANG ; Xia WANG ; Hui ZHANG ; Jian HE ; Jing PENG
Chinese Journal of Contemporary Pediatrics 2025;27(7):864-869
The patient was a girl, aged 10 years, who was admitted due to fever for 5 days and pancytopenia in peripheral blood for 2 days. Bone marrow examination showed the presence of phagocytic activity, and peripheral blood tests showed pancytopenia, an increase in ferritin, a reduction in fibrinogen, increases in triglyceride and sCD25, and a reduction in natural killer cell activity, which led to the diagnosis of hemophagocytic lymphohistiocytosis (HLH). On the day of admission, the child developed convulsions and rapidly progressed to refractory status epilepticus, which was consistent with the manifestations of febrile infection-related epilepsy syndrome. HLH was controlled after active immunotherapy, with the sequela of refractory epilepsy, and her cognitive function was essentially within normal limits. This article reports the condition of febrile infection-related epilepsy syndrome caused by HLH for the first time in China, in order to improve the awareness of this disease among clinicians.
Humans
;
Lymphohistiocytosis, Hemophagocytic/complications*
;
Female
;
Child
;
Epilepsy/etiology*
;
Fever/etiology*
;
Epileptic Syndromes/etiology*
3.Clinical characteristics and prognosis in 12 children with SARS-CoV-2 Omicron variant infection-associated acute necrotizing encephalopathy.
Jin-Mei SHEN ; Rui-Hai LIU ; Zi-Pu LI ; Jian-Hong DONG ; Jian-Ning TONG ; Jing LI
Chinese Journal of Contemporary Pediatrics 2023;25(11):1170-1174
OBJECTIVES:
To study the clinical characteristics and prognosis of SARS-CoV-2 Omicron variant infection-associated acute necrotizing encephalopathy (ANE) in children .
METHODS:
A retrospective analysis was conducted on the medical data of 12 children with SARS-CoV-2 Omicron variant infection-associated ANE who were admitted to the Pediatric Intensive Care Unit, Qingdao Women and Children's Hospital from December 18 to 29, 2022. The children were divided into two groups based on outcomes: death group (7 cases) and survival group (5 cases). The clinical manifestations and auxiliary examination results were compared between the two groups.
RESULTS:
The median age of the 12 patients was 30 months, with a male-to-female ratio of 1:1. All patients presented with persistent high fever, with a median highest body temperature of 41℃. The median time from fever onset to seizure or consciousness disturbance was 18 hours. The death group had a higher proportion of neurogenic shock, coagulation dysfunction, as well as elevated lactate, D-dimer, interleukin-6, interleukin--8, and interleukin-10 levels compared to the survival group (P<0.05).
CONCLUSIONS
Children with SARS-CoV-2 Omicron variant infection-associated with ANE commonly present with persistent high fever, rapidly progressing disease, and have a high likelihood of developing consciousness disorders and multiorgan dysfunction within a short period. The occurrence of neurogenic shock, coagulation dysfunction, and significantly elevated cytokine levels suggests an increased risk of mortality.
Humans
;
Female
;
Child
;
Male
;
Infant
;
SARS-CoV-2
;
Retrospective Studies
;
COVID-19/complications*
;
Brain Diseases/etiology*
;
Prognosis
;
Fever
;
Blood Coagulation Disorders
4."Typhoidal Cells" Appear in a Woman with Hemophagocytic Syndrome Secondary To Brucellosis: A Case Report.
Wei-Qing SONG ; Xu ZHENG ; Hai-Ni LI ; Li LI ; Jiang-Shui YUAN ; Shu-Guo WANG
Chinese Medical Sciences Journal 2023;38(1):62-65
We report a case of hemophagocytic syndrome (HPS) secondary to brucellosis, in which typhoidal cells were found in bone marrow, suggesting typhoidal cells present not only in Salmonella typhi infections but also in other bacterial infections. Typhoidal cells in bone marrow can be used to quickly identify the presence of bacterial infection pending the results of bone marrow and/or blood cultures.
Female
;
Humans
;
Typhoid Fever/microbiology*
;
Lymphohistiocytosis, Hemophagocytic/etiology*
;
Brucellosis/complications*
5.Efficacy and safefy of Polymyxin B treatment for neutropenic patients suffering from refractory Gram-negative bacterial bloodstream infection.
Meng ZHOU ; Hui Zhu KANG ; Cheng Yuan GU ; Yue Jun LIU ; Ying WANG ; Miao MIAO ; Jian Hong FU ; Xiao Wen TANG ; Hui Ying QIU ; Cheng Cheng FU ; Zheng Ming JIN ; Cai Xia LI ; Su Ning CHEN ; Ai Ning SUN ; De Pei WU ; Yue HAN
Chinese Journal of Hematology 2023;44(6):484-489
Objective: To assess the efficacy and safety of polymyxin B in neutropenic patients with hematologic disorders who had refractory gram-negative bacterial bloodstream infection. Methods: From August 2021 to July 2022, we retrospectively analyzed neutropenic patients with refractory gram-negative bacterial bloodstream infection who were treated with polymyxin B in the Department of Hematology of the First Affiliated Hospital of the Soochow University between August 2021 to July 2022. The cumulative response rate was then computed. Results: The study included 27 neutropenic patients with refractory gram-negative bacterial bloodstream infections. Polymyxin B therapy was effective in 22 of 27 patients. The median time between the onset of fever and the delivery of polymyxin B was 3 days [interquartile range (IQR) : 2-5]. The median duration of polymyxin B treatment was 7 days (IQR: 5-11). Polymyxin B therapy had a median antipyretic time of 37 h (IQR: 32-70). The incidence of acute renal dysfunction was 14.8% (four out of 27 cases), all classified as "injury" according to RIFLE criteria. The incidence of hyperpigmentation was 59.3%. Conclusion: Polymyxin B is a viable treatment option for granulocytopenia patients with refractory gram-negative bacterial bloodstream infections.
Humans
;
Polymyxin B/adverse effects*
;
Retrospective Studies
;
Gram-Negative Bacterial Infections/complications*
;
Fever/drug therapy*
;
Sepsis/drug therapy*
;
Anti-Bacterial Agents/therapeutic use*
;
Bacteremia/complications*
6.Predictive factors for sequelae of bronchitis obliterans in refractory Mycoplasma pneumoniae pneumonia.
Jian Hua LIU ; Jin Rong LIU ; Xiao Lei TANG ; Hai Ming YANG ; Hui LIU ; Hui XU ; Hui Min LI ; Shun Ying ZHAO
Chinese Journal of Pediatrics 2023;61(4):317-321
Objective: To investigate the predictive factors for bronchitis obliterans in refractory Mycoplasma pneumoniae pneumonia (RMPP). Methods: A restrospective case summary was conducted 230 patients with RMPP admitted to the Department of No.2 Respiratory Medicine of Beijing Children's Hospital, Capital Medical University from January 2013 to June 2017 were recruited. Clinical data, laboratory results, imaging results and follow-up data were collected. Based on bronchoscopy and imaging findings 1 year after discharge, all patients were divided into two groups: one group had sequelae of bronchitis obliterans (sequelae group) and the other group had not bronchitis obliterans (control group), independent sample t-test and nonparametric test were used to compare the differences in clinical features between the two groups. Receiver operating characteristic (ROC) curve to explore the predictive value of Bronchitis Obliterans in RMPP. Results: Among 230 RMPP children, there were 115 males and 115 females, 95 cases had sequelae group, the age of disease onset was (7.1±2.8) years;135 cases had control group, the age of disease onset was (6.8±2.7) years. The duration of fever, C-reative protein (CRP) and lactate dehydrogenase (LDH) levels, the proportion of ≥2/3 lobe consolidation, pleural effusion and the proportion of airway mucus plug and mucosal necrosis were longer or higher in the sequelae group than those in the control group ((17±9) vs. (12±3) d, (193±59) vs. (98±42) mg/L,730 (660, 814) vs. 486 (452, 522) U/L, 89 cases (93.7%) vs. 73 cases (54.1%), 73 cases (76.8%) vs.59 cases (43.7%), 81 cases (85.3%) vs. 20 cases (14.8%), 67 cases (70.5%) vs. 9 cases (6.7%), t=5.76, 13.35, Z=-6.41, χ2=14.64, 25.04, 22.85, 102.78, all P<0.001). Multivariate Logistic regression analysis showed that the duration of fever ≥10 days (OR=1.200, 95%CI 1.014-1.419), CRP levels increased (OR=1.033, 95%CI 1.022-1.044) and LDH levels increased (OR=1.001, 95%CI 1.000-1.003) were the risk factors for sequelae of bronchitis obliterans in RMPP. ROC curve analysis showed that CRP 137 mg/L had a sensitivity of 82.1% and a specificity of 80.1%; LDH 471 U/L had a sensitivity of 62.7% and a specificity of 60.3% for predicting the development of bronchitis obliterans. Conclusions: The long duration of fever (≥10 d), CRP increase (≥137 mg/L) may be used to predict the occurrence of sequelae of bronchitis obliterans in RMPP. It is helpful for early recognition of risk children.
Child
;
Male
;
Female
;
Humans
;
Child, Preschool
;
Mycoplasma pneumoniae
;
Retrospective Studies
;
Pneumonia, Mycoplasma/complications*
;
Disease Progression
;
L-Lactate Dehydrogenase
;
Fever
7.The epidemiology and pathogeny investigation of two clusters of severe fever with thrombocytopenia syndrome disease outbreaking in Henan Province, 2022.
Yi LI ; Xiao Yang WANG ; Ya Fei LI ; Dong Xiao LI ; Xiao HU ; Lin ZHU ; Ai Guo YOU ; Hai Feng WANG ; Ying YE ; Wan Shen GUO ; Xue Yong HUANG
Chinese Journal of Preventive Medicine 2023;57(10):1719-1724
To investigate two clusters of severe fever with thrombocytopenia syndrome virus (SFTSV) in Xinyang City, Henan Province, in 2022, and analyze their causes, transmission route, risk factors, and the characteristics of virus genetic variation. Case search and case investigation were carried out according to the case definition. Blood samples from cases, family members and neighbors and samples of biological vectors were collected for RT-PCR to detect SFTSV. The whole genome sequencing and bioinformatics analysis were performed on the collected positive samples. A total of two clustered outbreaks occurred, involving two initial cases and ten secondary cases, all of which were family recurrent cases. Among them, nine secondary cases had close contact with the blood of the initial case, and it was determined that close contact with blood was the main risk factor for the two clustered outbreaks. After genome sequencing analysis, we found that the SFTSV genotype in two cases was type A, which was closely related to previous endemic strains in Xinyang. The nucleotide sequence of the SFTSV in the case was highly homologous, with a total of nine amino acid mutation sites in the coding region. It was not ruled out that its mutation sites might have an impact on the outbreak of the epidemic.
Humans
;
Severe Fever with Thrombocytopenia Syndrome/epidemiology*
;
Bunyaviridae Infections/epidemiology*
;
Thrombocytopenia/complications*
;
Phlebovirus/genetics*
;
Disease Outbreaks
;
China/epidemiology*
8.The epidemiology and pathogeny investigation of two clusters of severe fever with thrombocytopenia syndrome disease outbreaking in Henan Province, 2022.
Yi LI ; Xiao Yang WANG ; Ya Fei LI ; Dong Xiao LI ; Xiao HU ; Lin ZHU ; Ai Guo YOU ; Hai Feng WANG ; Ying YE ; Wan Shen GUO ; Xue Yong HUANG
Chinese Journal of Preventive Medicine 2023;57(10):1719-1724
To investigate two clusters of severe fever with thrombocytopenia syndrome virus (SFTSV) in Xinyang City, Henan Province, in 2022, and analyze their causes, transmission route, risk factors, and the characteristics of virus genetic variation. Case search and case investigation were carried out according to the case definition. Blood samples from cases, family members and neighbors and samples of biological vectors were collected for RT-PCR to detect SFTSV. The whole genome sequencing and bioinformatics analysis were performed on the collected positive samples. A total of two clustered outbreaks occurred, involving two initial cases and ten secondary cases, all of which were family recurrent cases. Among them, nine secondary cases had close contact with the blood of the initial case, and it was determined that close contact with blood was the main risk factor for the two clustered outbreaks. After genome sequencing analysis, we found that the SFTSV genotype in two cases was type A, which was closely related to previous endemic strains in Xinyang. The nucleotide sequence of the SFTSV in the case was highly homologous, with a total of nine amino acid mutation sites in the coding region. It was not ruled out that its mutation sites might have an impact on the outbreak of the epidemic.
Humans
;
Severe Fever with Thrombocytopenia Syndrome/epidemiology*
;
Bunyaviridae Infections/epidemiology*
;
Thrombocytopenia/complications*
;
Phlebovirus/genetics*
;
Disease Outbreaks
;
China/epidemiology*
9.Etiology and clinical characteristics of fever of unknown origin in 357 pediatric patients.
Jun XU ; Ying Zi YE ; Li Jing YE ; Shu Zhen HAN ; Xia WU ; Cui WANG ; Hui YU
Chinese Journal of Pediatrics 2022;60(1):41-45
Objective: To explore the etiologies and clinical characteristics of fever of unknown origin (FUO) and to provide clues for early diagnosis of FUO. Methods: The data about etiology, age, sex, clinical course, length of hospital stays and the expression levels of inflammatory factors in fever phase of 357 pediatric inpatients who were diagnosed with FUO in Children's Hospital of Fudan University from 1 January 2016 to 31 December 2020 were collected and retrospectively analyzed. Participants were grouped into infectious disease, inflammatory disease, malignancy and others and according to the classification of diseases and also grouped into those aged<1 year, 1-<3 years,3-<6 years, 6-<12 years and 12-<18 years. Comparisons between groups were performed using the Mann-Whitney U test, Kruskal-Wallis H test and χ² test. Results: Among the 357 patients (217 males and 140 females). The age of onset was 3.9 (1.3, 9.2) years and visiting age was 5.1 (2.0, 9.3) years. The time-consuming of diagnosis was 94 (66, 213) days. The hospital stay was 8 (6, 14) days. The most frequently identified cause of FUO was infectious diseases (163 cases, 45.7%), followed by non-infectious inflammatory diseases (133 cases, 37.2%), malignancy (21 cases, 5.9%) and others (40 cases, 11.2%). The patients at younger age were more likely to be attacked by malignancy, oncologic diagnoses, and others, nevertheless patients at older age were more likely to be attacked by non-infectious inflammatory diseases oppositely (9.8 (3.6, 11.5) vs. 3.0 (1.2, 7.0), 2.3 (1.0, 5.2), 0.9 (0.5, 1.8) years, U=41.30, 15.94, 37.08, all P<0.01);106 (65%) patients were male, and 57 (35%) patients were female. This result indicated that boys were more susceptible to infectious diseases (χ²=14.73, P<0.01). Analysis of inflammatory factors in serum among 103 patients, interleukin (IL)-6 level in 40 infectious diseases patients (9 (2, 38) ng/L) was significantly lower than those of 6 tumor patients (89 (64, 599) ng/L) and 57 non-infectious inflammatory diseases patients (25 (8, 78) ng/L, U=51.05, 15.70, both P<0.05), no significant difference was observed in IL-2, IL-4, IL-10, tumor necrosis factor α and interferon among the groups (all P>0.05). The patients grouped into those aged 1-<3 years and 3-<6 years were more likely to be attacked by infectious diseases (51.3% (59/115) and 57.1% (40/70)), while patients grouped into those aged 6-<12 years and 12-<18 years were more likely to be attacked by non-infectious inflammatory diseases (55.6% (65/117) and 72.4% (21/29)). Conclusions: Infectious disease is still the main cause of FUO in children and the boys are more susceptible to infectious diseases. However, the morbidity of non-infectious inflammatory diseases increases to number 1 in FUO of children over 6 years of age.
Aged
;
Child
;
Communicable Diseases/complications*
;
Female
;
Fever of Unknown Origin/etiology*
;
Humans
;
Length of Stay
;
Male
;
Neoplasms/complications*
;
Retrospective Studies
10."Massive hemoptysis" and shock after fever and cough.
Liang-Ji DENG ; Jie XIONG ; Li-Li ZHONG ; Xiao-Juan LIN ; Xu-Ping XIAO ; Zhi-Qun MAO
Chinese Journal of Contemporary Pediatrics 2022;24(6):705-710
A boy, aged 11 years, was admitted due to intermittent fever for 15 days, cough for 10 days, and "hemoptysis" for 7 days. The boy had fever and cough with left neck pain 15 days ago, and antibiotic treatment was effective. During the course of disease, the boy developed massive "hemoptysis" which caused shock. Fiberoptic bronchoscopy revealed a left pyriform sinus fistula with continuous bleeding. In combination with neck and vascular imaging examination results, the boy was diagnosed with internal jugular vein injury and thrombosis due to congenital pyriform sinus fistula infection and neck abscess. The boy was improved after treatment with temperature-controlled radiofrequency ablation for the closure of pyriform sinus fistula, and no recurrence was observed during the follow-up for one year and six months. No reports of massive hemorrhage and shock due to pyriform sinus fistula infection were found in the searched literature, and this article summarizes the clinical features, diagnosis, and treatment of this boy, so as to provide a reference for the early diagnosis of such disease and the prevention and treatment of its complications.
Abscess/surgery*
;
Cough
;
Fever/complications*
;
Fistula/surgery*
;
Hemoptysis/complications*
;
Humans
;
Male
;
Neck
;
Shock

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