RESUMEN
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening clinical syndrome in children, and the knowledge of it is still limited. Two hundred twenty-seven children with HLH in our hospital were retrospectively analyzed from January 2001 to December 2018. The age of the patients on admission ranged from 1 day to 14 years old. The 3 most common clinical manifestations include fever (98.7%), hepatomegaly (95.6%), and splenomegaly (92.1%). The decrease of high-density lipoprotein cholesterol (99.1%) is very common in children with HLH. Albumin<25 g/L, activated partial thromboplastin time >65 s, and lactose dehydrogenase >1000 U/L were independent risk factors for poor early prognosis in children with HLH, and their odds ratio values were 2.515, 3.094, and 2.378, respectively, while age >28 months was identified as a protective factor (odds ratio=0.295). Of the 227 children, 67 (29.52%) died within 30 days of onset. The mortality rate in 2013 to 2018 was significantly lower than that in 2001 to 2012 (16.35% vs. 40.65%, P=0.000). The shortening of the time from onset to admission and the reduction of time from admission to definite diagnosis could be some of the reasons for the decrease of HLH mortality in 2013 to 2018 (P<0.05, respectively). Our study suggests that early identification of risk factors for HLH, timely diagnosis and treatment are important measures to improve the short-term prognosis of HLH in children.
Asunto(s)
HDL-Colesterol/sangre , Linfohistiocitosis Hemofagocítica , Albúmina Sérica Humana/metabolismo , Adolescente , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Recién Nacido , Linfohistiocitosis Hemofagocítica/sangre , Linfohistiocitosis Hemofagocítica/mortalidad , Masculino , Tiempo de Tromboplastina Parcial , Estudios Retrospectivos , Factores de Riesgo , Tasa de SupervivenciaRESUMEN
Obstructive sleep apnea hypopnea syndrome (OSAHS) is associated with the neurocognitive deficits as a result of the neuronal cell injury. Previous studies have shown that adenosine A1 receptor (ADORA1) played an important role against hypoxia exposure, such as controlling the metabolic recovery in rat hippocampal slices and increasing the resistance in the combined effects of hypoxia and hypercapnia. However, little is known about whether ADORA1 takes part in the course of neuronal cell injury after intermittent hypoxia exposure which was the main pathological characteristic of OSAHS. The present study is performed to explore the underlying mechanism of neuronal cell injury which was induced by intermittent hypoxia exposure in PC12 cells. In our research, we find that the stimulation of the ADORA1 by CCPA accelerated the injury of PC12 cells as well as upregulated the expression of PKC, inwardly rectifying potassium channel 6.2(Kir6.2) and sulfonylurea receptor 1(SUR1) while inhibition of the ADORA1 by DPCPX alleviated the injury of PC12 cells as well as downregulated the expression of PKC, Kir6.2, and SUR1. Moreover, inhibition of the PKC by CHE, also mitigated the injury of PC12 cells, suppressed the Kir6.2 and SUR1 expressions induced by PKC. Taken together, our findings indicate that ADORA1 accelerated PC12 cells injury after intermittent hypoxia exposure via ADORA1/PKC/KATP signaling pathway.
Asunto(s)
Neuronas/metabolismo , Canales de Potasio de Rectificación Interna/metabolismo , Proteína Quinasa C/metabolismo , Receptor de Adenosina A1/metabolismo , Transducción de Señal , Apnea Obstructiva del Sueño/metabolismo , Receptores de Sulfonilureas/metabolismo , Animales , Hipoxia de la Célula , Neuronas/patología , Células PC12 , Canales de Potasio de Rectificación Interna/genética , Ratas , Receptor de Adenosina A1/genética , Apnea Obstructiva del Sueño/genética , Apnea Obstructiva del Sueño/patología , Receptores de Sulfonilureas/genéticaRESUMEN
BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is highly prevalent in children and adolescents, particularly those with obesity. NAFLD is considered a hepatic manifestation of the metabolic syndrome due to its close associations with abdominal obesity, insulin resistance, and atherogenic dyslipidemia. Experts have proposed an alternative terminology, metabolic dysfunction-associated fatty liver disease (MAFLD), to better reflect its pathophysiology. This study aimed to develop consensus statements and recommendations for pediatric MAFLD through collaboration among international experts. METHODS: A group of 65 experts from 35 countries and six continents, including pediatricians, hepatologists, and endocrinologists, participated in a consensus development process. The process encompassed various aspects of pediatric MAFLD, including epidemiology, mechanisms, screening, and management. FINDINGS: In round 1, we received 65 surveys from 35 countries and analyzed these results, which informed us that 73.3% of respondents agreed with 20 draft statements while 23.8% agreed somewhat. The mean percentage of agreement or somewhat agreement increased to 80.85% and 15.75%, respectively, in round 2. The final statements covered a wide range of topics related to epidemiology, pathophysiology, and strategies for screening and managing pediatric MAFLD. CONCLUSIONS: The consensus statements and recommendations developed by an international expert panel serve to optimize clinical outcomes and improve the quality of life for children and adolescents with MAFLD. These findings emphasize the need for standardized approaches in diagnosing and treating pediatric MAFLD. FUNDING: This work was funded by the National Natural Science Foundation of China (82070588, 82370577), the National Key R&D Program of China (2023YFA1800801), National High Level Hospital Clinical Research Funding (2022-PUMCH-C-014), the Wuxi Taihu Talent Plan (DJTD202106), and the Medical Key Discipline Program of Wuxi Health Commission (ZDXK2021007).
Asunto(s)
Consenso , Enfermedad del Hígado Graso no Alcohólico , Humanos , Enfermedad del Hígado Graso no Alcohólico/epidemiología , Enfermedad del Hígado Graso no Alcohólico/terapia , Enfermedad del Hígado Graso no Alcohólico/metabolismo , Enfermedad del Hígado Graso no Alcohólico/diagnóstico , Niño , Adolescente , Síndrome Metabólico/epidemiología , Síndrome Metabólico/diagnóstico , Síndrome Metabólico/terapia , Síndrome Metabólico/metabolismoRESUMEN
OBJECTIVE: To study the clinical effect of continuous thoracic close drainage using central venous catheter instead of repeated thoracocentesis in the treatment of tuberculous pleurisy in children. METHODS: Thirty-nine children with tuberculous pleurisy, who received continuous thoracic close drainage using central venous catheter in addition to conventional antituberculous chemotherapy, were used as the observation group and 42 children with tuberculous pleurisy who underwent repeated thoracocentesis in addition to conventional antituberculous chemotherapy served as the control group. The two groups were compared in terms of time to pleural effusion absorption, improvement in pleural thickening, length of hospital stay, and puncture-related expenses. RESULTS: Compared with the control group, the observation group had significantly faster pleural effusion absorption (8 ± 4 d vs 12 ± 6 d; P < 0.01), significantly more improvement in pleural thickening (1.50 ± 0.25 mm vs 3.10 ± 0.30 mm; P < 0.05), a significantly shorter length of hospital stay (11 ± 3 d vs 18 ± 6 d; P < 0.01), and significantly lower puncture-related expenses (269 ± 24 yuan vs 475 ± 50 yuan; P < 0.05), as well as alleviated pain. CONCLUSIONS: Continuous thoracic close drainage using central venous catheter is superior to repeated thoracocentesis in the treatment of tuberculous pleurisy in children, and it holds promise for clinical application in pediatric patients.
Asunto(s)
Catéteres Venosos Centrales , Drenaje/métodos , Tuberculosis Pleural/terapia , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , MasculinoRESUMEN
OBJECTIVE: To explore the role of endoplasmic reticulum stress in brain injury following chronic intermittent hypoxia (CIH) in weanling rats. METHODS: A total of 48 male healthy Sprague-Dawley rats (3-4-week-old, 80-100 g) were randomly divided into 4 groups: 2-week-CIH (2IH) group, 4-week-CIH (4IH) group, 2-week-control (2C) group and 4-week-control (4C) group. The morphologic changes were observed by hematoxylin-eosin (HE) staining and cell apoptosis detected by terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling assay. Then hippocampus and prefrontal cortices were collected for transcription and expression analysis of glucose regulated protein 78 (GRP78) by reverse transcription (RT)-PCR and Western blotting respectively. And the expressions of Caspase-12 mRNA and Caspase-12 protein in prefrontal cortex were analyzed by RT-PCR and immunohistochemistry. RESULTS: The neuronal apoptosis in hippocampus and prefrontal cortices in CIH exposed groups were more pronounced than those of the control groups (all P < 0.01), especially in the 4IH group (hippocampus: 8.78% ± 0.71% vs 3.26% ± 0.45%, cortices: 6.02% ± 0.32% vs 2.91% ± 0.29%). The expression levels of GRP78 mRNA (hippocampus: 0.424 ± 0.033 vs 0.326 ± 0.013 and 0.444 ± 0.028 vs 0.310 ± 0.015, cortices: 0.514 ± 0.038 vs 0.430 ± 0.017 and 0.524 ± 0.038 vs 0.439 ± 0.033) and GRP78 protein in hippocampus and prefrontal cortices (hippocampus: 0.221 ± 0.032 vs 0.178 ± 0.014 and 0.241 ± 0.019 vs 0.170 ± 0.013, cortices: 0.307 ± 0.012 vs 0.226 ± 0.022 and 0.311 ± 0.023 vs 0.225 ± 0.025), and the expression levels of Caspase-12 mRNA (0.396 ± 0.004 vs 0.323 ± 0.014, 0.417 ± 0.011 vs 0.313 ± 0.011) and Caspase-12 protein (0.334 ± 0.035 vs 0.197 ± 0.023, 0.368 ± 0.079 vs 0.215 ± 0.024) in prefrontal cortex in the IH groups all were more than those in the 2C and 4C groups (all P < 0.05). CONCLUSIONS: Chronic intermittent hypoxia can up-regulate the GRP78 transcription and expression in brain regions associated with learning and memory. This may induce the endoplasmic reticulum stress and activate the Caspase-12 mediated apoptosis signaling pathway. In the end, neuronal apoptosis occurs. All these factors may play an important role in the impairment of learning memory during the exposure of growing rats to chronic intermittent hypoxia.
Asunto(s)
Lesiones Encefálicas/metabolismo , Caspasa 12/metabolismo , Estrés del Retículo Endoplásmico , Hipoxia/metabolismo , Animales , Apoptosis , Lesiones Encefálicas/patología , Corteza Cerebral/metabolismo , Chaperón BiP del Retículo Endoplásmico , Proteínas de Choque Térmico/metabolismo , Hipocampo/metabolismo , Hipoxia/patología , Masculino , Ratas , Ratas Sprague-DawleyRESUMEN
OBJECTIVE: To investigate the positive rate of hepatitis B surface antibody (HBsAb) in children. METHODS: Blood samples from 3022 children who received a physical examination in outpatient departments from 2009 to 2011 were subjected to serological test using ELISA to measure the positive rate of HBsAb. RESULTS: The positive rate of HBsAb decreased with age (P<0.01). There was no significant difference in the positive rate of HBsAb between boys and girls (P>0.05), however the positive rate of HBsAb in boys aged one year and over was lower than in girls of the same age (P<0.01). The positive rate of HBsAb in boys aged between 3 and 4 years was higher than in girls of the same age (P<0.01). The positive rate of HBsAb decreased with age in boys, and was lower in those aged two years and over than in those aged one year and over (P<0.01). The positive rate of HBsAb also decreased with age in girls, with significant differences between different age groups (P<0.01). CONCLUSIONS: The positive rate of HBsAb decreases with age in children, so younger children have a higher risk of infection with hepatitis B virus. Serological monitoring of hepatitis B needs to be enhanced.
Asunto(s)
Anticuerpos contra la Hepatitis B/sangre , Antígenos de Superficie de la Hepatitis B/inmunología , Distribución por Edad , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Lactante , MasculinoRESUMEN
Candida haemulonii forms part of the non-albicans Candida species. The candidemia caused by C. haemulonii is extremely rare but fatal in neonates. We reported the first two neonates with C. haemulonii candidemia in China which were treated with fluconazole and reviewed two papers previously reported. Our report adds further awareness on C. haemulonii candidemia in critical neonates and points out the importance of an early diagnosis and treatment of this fatal infection.
La Candida haemulonii forma parte de la especie Candida no albicans. La candidemia por C. haemulonii es sumamente infrecuente, pero mortal, en los recién nacidos. Se informa sobre los dos primeros recién nacidos con candidemia por C. haemulonii en China tratados con fluconazol y se revisan dos artículos informados con anterioridad. Nuestro informe incrementa la sensibilización sobre la candidemia por C. haemulonii en recién nacidos críticos y resalta la importancia de un diagnóstico y un tratamiento tempranos de esta infección mortal.
Asunto(s)
Antifúngicos/uso terapéutico , Candidemia/tratamiento farmacológico , Infecciones Relacionadas con Catéteres/tratamiento farmacológico , Fluconazol/uso terapéutico , Candida/aislamiento & purificación , Candidemia/etiología , Candidemia/microbiología , Infecciones Relacionadas con Catéteres/microbiología , China , Humanos , Recién Nacido , Masculino , Resultado del TratamientoRESUMEN
Scrub typhus is caused by Orientia tsutsugamushi. Any delay in diagnosis can result in delayed treatment and severe complications, including secondary hemophagocytic lymphohistiocytosis, which is rare but potentially fatal.In this paper, the authors present 3 cases of secondary hemophagocytic lymphohistiocytosis associated with scrub typhus, successfully treated with chloramphenicol without additional antineoplastic therapy. All patients cured and achieved complete resolution.This report highlights the effectiveness of chloramphenicol without the need for chemotherapy in the treatment of scrub typhus-associated hemophagocytic lymphohistiocytosis in a pediatric population under the age of 8 years.
Asunto(s)
Antibacterianos/uso terapéutico , Cloranfenicol/uso terapéutico , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/etiología , Tifus por Ácaros/complicaciones , Tifus por Ácaros/tratamiento farmacológico , Niño , Preescolar , China , Femenino , Humanos , MasculinoRESUMEN
The most usual presentation of Plesiomonas shigelloides infection is an acute gastroenteritis, and extraintestinal manifestations are extremely rare. We reported the first neonate with P. shigelloides meningoencephalitis in China and reviewed the twelve cases previously reported. Our report adds further awareness on Plesiomonas shigelloides meningoencephalitis in neonate and points out the importance of an early diagnosis and the use of sensitive antibiotics treatment to this fatal infection.
Asunto(s)
Infecciones por Bacterias Gramnegativas , Meningoencefalitis/microbiología , Plesiomonas , Antibacterianos/uso terapéutico , China , Quimioterapia Combinada , Femenino , Infecciones por Bacterias Gramnegativas/tratamiento farmacológico , Humanos , Recién Nacido , Meningoencefalitis/diagnóstico por imagen , RadiografíaRESUMEN
La Candida haemulonii forma parte de la especie Candida no albicans. La candidemia por C. haemulonii es sumamente infrecuente, pero mortal, en los recién nacidos. Se informa sobre los dos primeros recién nacidos con candidemia por C. haemulonii en China tratados con fluconazol y se revisan dos artículos informados con anterioridad. Nuestro informe incrementa la sensibilización sobre la candidemia por C. haemulonii en recién nacidos críticos y resalta la importancia de un diagnóstico y un tratamiento tempranos de esta infección mortal.
Candida haemulonii forms part of the non-albicans Candida species. The candidemia caused by C. haemulonii is extremely rare but fatal in neonates. We reported the first two neonates with C. haemulonii candidemia in China which were treated with fluconazole and reviewed two papers previously reported. Our report adds further awareness on C. haemulonii candidemia in critical neonates and points out the importance of an early diagnosis and treatment of this fatal infection.
Asunto(s)
Humanos , Masculino , Femenino , Recién Nacido , Fluconazol/uso terapéutico , Infecciones Relacionadas con Catéteres/tratamiento farmacológico , Candidemia/tratamiento farmacológico , Candida/aislamiento & purificación , China , Resultado del Tratamiento , Infecciones Relacionadas con Catéteres/microbiología , Candidemia/etiología , Candidemia/microbiología , Antifúngicos/uso terapéuticoRESUMEN
OBJECTIVE: To exam the relationship between snoring and morbidities of multiple systems in children. STUDY DESIGN: Children with snoring were enrolled and divided into primary snorer (PS) group and obstructive sleep apnea hypopnea syndrome (OSAHS) group based on polysomnography. The healthy children served as the control group. The growth parameters, maxillofacial malformations, blood chemistry, electrocardiogram, and echocardiogram were recorded and intelligence testing was performed in the enrolled children who were ≥6 years old. RESULTS: The weight and height were similar in the control group (n = 60) and the PS group (n = 63), but lower in the OSAHS group (n = 89; P < 0.001). Occurrence of adenoidal face and dental malocclusion in the OSAHS and the PS group was significantly higher than that in the control group (P < 0.001). Compared with the control group, the OSAHS group had a lower serum high-density lipoprotein cholesterol level, higher low-density lipoprotein cholesterol level; and a possible higher pulmonary artery pressure based on the echocardiogram (P < 0.001). All the above parameters in the PS group were similar to those in the control group. Full-scale IQ and performance IQ of the OSAHS group was lower (P < 0.001), attention deficits were significantly higher in the OSAHS group (P < 0.001), but were similar in the PS group when compared to the control group. CONCLUSIONS: OSAHS in children is associated with delayed growth, maxillofacial malformations, impaired cognitive functions, abnormalities in lipid metabolism, and changes in pulmonary artery pressures. PS children also have higher incidence of maxillofacial malformations but have a normal growth and normal cognitive functions.
Asunto(s)
Apnea Obstructiva del Sueño/complicaciones , Ronquido/complicaciones , Estudios de Casos y Controles , Niño , Preescolar , Trastornos del Conocimiento/diagnóstico , Trastornos del Conocimiento/etiología , Hipertensión Pulmonar Primaria Familiar , Femenino , Trastornos del Crecimiento/diagnóstico , Trastornos del Crecimiento/etiología , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Trastornos del Metabolismo de los Lípidos/diagnóstico , Trastornos del Metabolismo de los Lípidos/etiología , Masculino , Anomalías Maxilofaciales/complicaciones , Anomalías Maxilofaciales/diagnóstico , Polisomnografía , Apnea Obstructiva del Sueño/diagnósticoAsunto(s)
Feto/diagnóstico por imagen , Hidrocefalia/diagnóstico por imagen , Ultrasonografía Prenatal/métodos , Adolescente , Femenino , Feto/anomalías , Feto/patología , Edad Gestacional , Humanos , Hidrocefalia/epidemiología , Hidrocefalia/etiología , Incidencia , Imagen por Resonancia Magnética , Embarazo , Complicaciones Infecciosas del EmbarazoAsunto(s)
Accidentes por Caídas , Fracturas Cerradas/etiología , Fracturas del Radio/etiología , Fracturas del Cúbito/etiología , Traumatismos de la Muñeca/etiología , Niño , Diagnóstico Diferencial , Fracturas Cerradas/diagnóstico por imagen , Humanos , Masculino , Fracturas del Radio/diagnóstico por imagen , Fracturas del Cúbito/diagnóstico por imagen , Traumatismos de la Muñeca/diagnóstico por imagenRESUMEN
OBJECTIVE: Obstructive sleep apnea-hypopnea syndrome (OSAHS) may cause serious morbidities, such as systemic hypertension, diabetes, and cor pulmonale. However, currently no many reports on study of OSAHS in children are available. This study aimed to explore the effects of OSAHS on children's multiple systems. METHOD: A total of 89 cases of children who came to the Sleep Treatment Center in the authors' hospital from March 2009 to December 2010 with snoring were tested with overnight polysomnography (PSG). They were classified into mild OSAHS group (n = 59, mean age of 5.71, SD = 2.46) and moderate to severe group (n = 30, mean age of 5.30, SD = 2.73) based on the PSG results, and 100 healthy children were selected as the control group (n = 100, mean age of 6 years, SD = 2.98). Data including height, weight, body mass index and blood pressure, peripheral blood routine, blood lipids, glucose and insulin, electrocardiogram and echocardiography were collected. Patients' adenoid face and abnormal occlusion were also recorded. Comparisons of the data were made among those groups. RESULT: Mild OSAHS and moderate to severe group had significantly higher prevalence of adenoid face (23.7%, 26.7%), and abnormal occlusion (74.6%, 60.0%) than that in control group (0, 40%) (P < 0.05). There were no significant differences in terms of BMI between the OSAHS group and the control group, but the weight (kg) and height (cm) in the mild OSAHS group (23.3 ± 10.1, 114.9 ± 16.2) and moderate to severe group (21.9 ± 8.4, 110.8 ± 13.3) were lower than those of the control group (31.8 ± 10.1, 136.1 ± 15.1) (all P < 0.05). Compared with the control group, the level of HDL-C (mmol/L)and insulin (mU/L) in moderate and severe group decreased [(1.20 ± 0.30) vs. (1.40 ± 0.27), 2.79 (0.84 - 16.16) vs. 4.92 (0.76 - 16.80), P < 0.05], while the LDL-C (mmol/L) increased [(2.61 ± 0.75) vs. (2.32 ± 0.62), P < 0.05]. The red blood cell counts (× 10(12)/L) and the blood platelet counts (× 10(9)/L) in the mild OSAHS (4.93 ± 0.37, 292.92 ± 75.64) and moderate and severe OSAHS group (5.23 ± 0.22, 292.50 ± 63.05) were significantly higher in contrast to the control group (4.70 ± 0.31, 255.60 ± 69.12) (all P < 0.05), systolic blood pressure (mmHg) in mild group (98.54 ± 10.44) and moderate to severe group (99.13 ± 19.13) was significantly higher compared to control group (87.88 ± 11.37), and the heart rate (beats/min) in moderate to severe group (94.43 ± 10.64) was higher than those in control group (87.12 ± 16.20) (all P < 0.05). The mild OSAHS and moderate and severe OSAHS group had decreased right ventricular internal diameter [(14.24 ± 1.64) mm, (13.17 ± 2.07) mm ], increased main pulmonary artery diameter [(17.05 ± 3.33) mm, (16.33 ± 3.14) mm] and the thickness of right ventricular wall [(3.43 ± 0.26) mm, (3.57 ± 0.20) mm] compared to control group [ (16.10 ± 2.96) mm, (14.11 ± 2.52) mm, (3.32 ± 0.25) mm] (all P < 0.05). CONCLUSION: OSAHS in children may be associated with craniofacial malformations, and may contribute to slow growth and development, elevated blood viscosity and blood pressure, metabolic abnormalities, and change cardiac structure.