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Background: Cardiovascular diseases are the leading cause of death among patients on hemodialysis, with approximately 40% of the cardiovascular deaths linked to acute coronary syndrome. We aimed to investigate the incidence and risk factors of acute coronary syndrome in patients undergoing hemodialysis. Methods: Patients undergoing hemodialysis were prospectively enrolled from January 2018. Data regarding hospitalization due to acute coronary syndrome were collected at 3-month intervals through December 31, 2021. Cox regression model was used to estimate the association between baseline factors and incident acute coronary syndrome during follow-up. Results: Patients' mean age was 66 years, 48% were men, and 16% had a history of coronary artery disease at enrolment. Over a median follow-up of 1,187 days, 85 patients were hospitalized due to acute coronary syndrome. Left main or triple vessel disease was identified in 67 patients. Risk factors associated with incident acute coronary syndrome included aging, male sex, smoking, low diastolic blood pressure, and baseline comorbidities, in addition to dialysis factors including low urea clearance, central venous catheter use, and history of dialysis access dysfunction. After multivariate analysis, age, diabetes, hyperlipidemia, smoking, and frequent interventions for vascular access remained significant risk factors. Conclusions: A high acute coronary syndrome incidence was observed in our cohort, with traditional risk factors playing a consistent role with that in the general population. A history of frequent dialysis access dysfunction was also associated with incident acute coronary syndrome.
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BACKGROUND: The clinical presentations of abusive head trauma can abruptly worsen, so the occurrence of seizures and changes of EEG can be variable according to patients' conditions. Since the changes of EEG background waves reflect the cortical function of children, we aimed to find out whether the timing of EEG background, epileptiform discharges and seizure patterns were associated with the outcomes of patients with AHT. MATERIAL AND METHODS: Using seizure type and acute stage electroencephalographic (EEG) characteristics to assess adverse neurological outcomes in children with seizures secondary to abusive head trauma (AHT). Children who were hospitalized with AHT at a tertiary referral hospital from October 2000 to April 2010 were evaluated retrospectively. A total of 50 children below 6 years of age admitted due to AHT were included. KOSCHI outcome scale was used to evaluate the primary outcome and neurological impairment was used as secondary outcome after 6 months discharge. RESULTS: Children with apnea, cardiac arrest, reverse blood flow and skull fracture in clinic had a higher mortality rate even in the no-seizure group (3/5 [60%] vs. 3/45 [6.7%], odds ratio [OR] = 11; 95% CI = 2.3-52; p = 0.025). Seizure occurrence reduced mostly at the second day after admission in seizure groups; but children with persistent seizures for 1 week showed poor neurological outcomes. The occurrence of initial seizure was frequency associated with younger age; focal seizure, diffuse cortical dysfunction in acute-stage EEG, and low Glasgow Coma Scale (GCS) score were significantly related to poor outcomes after 6 months. Diffuse cortical dysfunction was also associated with motor, speech, and cognitive dysfunction. CONCLUSIONS: Diffuse cortical dysfunction in acute-stage EEG combined with low GCS score and focal seizure may related to poor outcomes and neurological dysfunctions in children with AHT.
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Phosphofurin Acidic Cluster Sorting Protein 2 (PACS2)-related early infantile developmental and epileptic encephalopathy (EIDEE) is a rare neurodevelopmental disorder. EIDEE is characterized by seizures that begin during the first three months of life and are accompanied by developmental impairment over time. In this article, we present three patients with EIDEE who experienced neonatal-onset seizures that developed into intractable seizures during infancy. Whole exome sequencing revealed a de novo heterozygous missense variant in all three patients in the p.Glu209Lys variant of the PACS2 gene. We conducted a literature review and found 29 cases to characterize the seizure patterns, neuroimaging features, the usage of anticonvulsants, and the clinical neurodevelopmental outcomes of PACS2-related EIDEE. The seizures were characterized by brief, recurring tonic seizures in the upper limbs, sometimes accompanied by autonomic features. Neuroimaging abnormalities were observed in the posterior fossa region, including mega cisterna magna, cerebellar dysplasia, and vermian hypoplasia. The long-term prognosis ranges from low-average intelligence to severe developmental retardation, emphasizing the importance of early recognition and accurate diagnosis by pediatric neurologists to provide personalized patient management.
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Food allergy is one of the most common chronic non-infectious diseases in many countries and regions, which affects 2%-4% of children and adults.Its prevalence is on the rise worldwide.In 2022, the Global Allergy and Asthma European Network (GA 2LEN) proposed recommendations on managing food allergy to people at different age groups.This review aims to interpret the recommendations, clinical practice, precautions, evidence gaps and research priorities of food allergy management based on the GA 2LEN guideline 2022, thus providing reference for clinical management of food allergy.
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Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.
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Asthma is a common chronic respiratory disease in children.Adolescent asthma is characterized by high prevalence rate, high mortality and poor disease control.The optimal adolescent asthma management is crucial to improve asthma control, as well as reduce the burden of asthma including mortality.The key of the management in adolescents with asthma is the self-management, which involves individual, family and social aspects.The imbalance between physical development and psychological development in adolescents leads to many challenges in the self-management in adolescents with asthma.This review elaborates the current situation, influence factors and promotional strategies of self-management in adolescents with asthma so as to achieve the optimal asthma self-management.
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Anti-glutamic acid decarboxylase (anti-GAD) antibodies are associated with different types of syndromes. However, few studies have investigated the correlation between anti-GAD antibody titers with clinical severity and outcomes in children with encephalitis/encephalopathy. In this single-center retrospective cohort study, we consecutively enrolled hospitalized children who had encephalitis and/or encephalopathy with positive anti-GAD antibodies in serum and/or cerebrospinal fluid (CSF) from February 2010 to October 2021. Thirty-seven patients were included and divided into high-titer and low-titer groups. The patients with high anti-GAD antibody titers were associated with initial symptoms of language difficulty and ataxia. The level of titers was not associated with severity or outcomes. Anti-GAD antibody titers decreased after immunotherapy, however, the clinical response to immunotherapy was variable. A transient elevation in anti-GAD antibody titers during immunotherapy was noted. Further studies are warranted to investigate the role of anti-GAD antibodies in the pathogenesis and immune mechanisms of encephalitis/encephalopathy.
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Antipyretic-analgesics are currently one of the most prescribed drugs in children.The clinical application of antipyretic-analgesics for children in our country still have irrational phenomenon, which affects the therapeutic effect and even poses hidden dangers to the safety of children.In this paper, suggestions were put forward from the indications, dosage form/route, dosage suitability, pathophysiological characteristics of children with individual differences and drug interactions in the symptomatic treatment of febrile children, so as to provide reference for the general pharmacists when conducting prescription review.
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With the emergence of new respiratory virus, it is more apparent for the vulnerability of population to respiratory viral infection.Non-pharmaceutical interventions (NPIs) for respiratory virus infection have become the main way to prevent corona virus disease 2019.Some studies had proven its effectiveness.In addition, the NPIs also significantly reduced the incidence and hospitalization rate of other respiratory disease in children.NPIs for respiratory virus infection in children have its particularity and challenge.In daily life, it is important to guide children how to do the NPIs, so as to protect susceptible children and reduce the disease burden in children′s health system.Therefore, the aerosol transmission, the specificity of the NPIs in children, and the impact on childhood respiratory diseases are described in this article, to improve the prevention of common respiratory diseases in children.
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Hypoxemia is a common complication of pneumonia, asthma, and bronchopulmonary dysplasia in children.Rapid identification of hypoxemia is of great significance for the disposal and management of critical children.Pulse oximetry is recognized by the World Health Organization as the best way to monitor hypoxemia in children, and it can monitor pulse oxygen saturation noninvasively and continuously.Based on the related literature at home and abroad, combined with the clinical needs of pediatrics, the " Expert consensus on clinical application of pulse oximetry in children" is formulated to improve the understanding of pediatricians and nurses on the application in pediatric clinical practice, principle, operation techniques, and limitations of pulse oximetry.
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The clinical data of a cystic fibrosis (CF) child with allergic bronchopulmonary aspergillosis (ABPA) and hemoptysis in the Department of Respiratory Disease Ⅰ, Beijing Children′s Hospital, Capital Medical University in May 2021 were retrospectively analyzed.Meanwhile, relevant literature was reviewed to analyze the clinical characteristics, diagnosis and treatment of CF patients with ABPA.This patient was a 15 years and 4 months old boy and complained of recurrent cough with sputum.The test showed increased blood eosinophils, total serum IgE higher than 500 IU/mL, positive aspergillus fumigatus specific IgE and IgG antibodies.Chest CT revealed central bronchiectasis and high-density mucus thrombus, and the patient was initially diagnosed with ABPA.Further examinations suggested the sweat chloride concentration was 89 mmol/L, and the genetic results showed a compound heterozygous mutation of CFTR (c.2909G>A from his father, c.3310G>T from his mother). Then, he was diagnosed with CF complicated with ABPA and treated with glucocorticoid and antifungal therapy.The disease was repeated after drug withdrawal.Due to hemoptysis, the right upper lobe lobectomy was performed.Unfortunately, ABPA occurred again 2 years later.The child is being followed up at present.CF is a rare monogenetic disease with poor prognosis.It is difficult to treat CF patients with ABPA and the disease repeats easily.Early identification and treatment will improve the prognosis.
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Asthma is the most common chronic respiratory disease in children and requires long-term medical management.Adolescence has unique physical and psychological characteristics, which challenge the management of asthma in adolescents.A personalized transition strategy can achieve a successful asthma transition from childhood into adulthood and improve long-term outcomes of asthma.In this paper, the characteristics of adolescence, burden of adolescent asthma, challenges to adolescent asthma, adolescent asthma management system, and successful transition of adolescent asthma were reviewed.
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Objective:To analyze the clinical manifestations, genetic variations, diagnosis and treatment of children with inherited thrombophilia(IT).Methods:Retrospective study.Children with IT treated in Department of Respiratory Diseases 1 of Beijing Children′s Hospital, Capital Medical University from October 2016 to August 2021 were included in the study and followed up.Results:A total of 5 children met the inclusion criteria, with 3 boys and 2 girls; the age of diagnosis ranged from 7 years to 13 years and 6 months.There were 2 cases of protein C deficiency, 1 case of congenital protein S deficiency, 1 case of activated protein C resistance and 1 case of congenital afibrinogenemia.All 5 cases had pulmonary embolism, 2 cases had deep venous thrombosis of lower limbs, and 1 case had cardiac thrombosis and arterial embolism.The level of protein C was significantly decreased in 1 case, and the level of protein S in 1 case was significantly decreased in the laboratory test of thrombophilia; 2 cases were positive for antiphospholipid antibodies in the acute phase, but negative after 3-6 months of re-examination.Genetic analysis showed 2 cases of PROC gene mutation, 1 case of PROSI gene mutation, 1 case of F5 gene mutation, and 1 case of FGA gene mutation.All children were treated with anticoagulation drugs for long-term, including 4 patients with Warfarin and 1 patient with Rivaroxaban.The follow-up time ranged from 3 months to 5 years.During the follow-up, 1 patient experienced thrombosis recurrence due to infection incentives 1 month after discontinuing anticoagulant drugs on his own. Conclusions:The clinical manifestations of children with IT are the same as those of adults, mainly including venous thromboembolism(VTE); there are limitations in laboratory detection of thrombophilia, and gene analysis is of great significance.Children diagnosed with IT need long-term anticoagulant therapy to reduce the recurrence of VTE.
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Objective:To explore the effect of Bushen Huoxue Decoction on ventricular remodeling and myocardial nuclear factor-kappaB (NF-κB) protein in rats with chronic heart failure.Methods:60 male SD rats were randomly divided into sham operation group (15 rats) and experimental group (45 rats). The rats of the experimental group was established CHF model by ligating the left anterior descending coronary artery combined with exhaustive swimming and starvation. Rats with chronic heart failure were randomly divided into model group, Bushen Huoxue group and lisinopril group.The Bushen Huoxue group was perfused with 15.75 g/(kg·d) Bushen Huoxue Decoction, the lisinopril group was perfused with 1.8 mg/(kg·d) of lisinopril suspension, and the sham operation group and model group were perfused with equal volume of distilled water. After 4 weeks of administration, the general mental state of rats was observed. The left ventricular internal systolic diameter (LVIDs) and internal diastolic diameter (LVIDd) were measured by cardiac color Doppler ultrasound, and the left ventricular ejection fraction (LVEF) and short axis shortening fraction (LVFS) were calculated. The expression of NF-κB protein in rat myocardium was detected by Western blot, and the morphology of left ventricular myocytes was observed by hematoxylin eosin staining.Results:Compared with the model group, the myocardial fibers of rats in Bushen Huoxue group and lisinopril group were arranged orderly, with few pyknosis, a small amount of inflammatory cell infiltration. Compared with the model group, the levels of LVIDs [(6.00±0.58)mm vs. (6.99±0.90)mm] and LVIDd [(3.96±0.51)mm vs. (5.14±0.57)mm] significantly decreased, LVEF [(54.48±6.75)% vs. (30.28±4.85)%] and LVFS [(33.86±4.27)% vs. (26.10±4.96)%] significantly increased, as well as the expression of myocardial NF-κB (1.06±0.10 vs. 1.58±0.29) protein significantly decreased ( P<0.05). Conclusion:Bushen Huoxue Decoction can resist ventricular remodeling,improve cardiac function and treat heart failure of CHF rats and the possible mechanism might be it could down-regulate myocardial NF-κB expression.
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Mucus plugs is a feature of fatal asthma, which is the abnormally thick mucus accumulated in the airway of patients.The study of airway mucus plugs in asthmatic patients began 100 years ago.In addition to the manifestation of primary disease, asthma patients with mucus plugs may have acute exacerbation of asthma or poor treatment effect.At this time, we should pay attention to the existence of mucus plugs.Multidetector computed tomography can be used to identify and evaluate mucus plugs.This article briefly describes the generation of airway mucus, and introduces the formation mechanism, clinical characteristics, identification and scoring system of mucus plugs in asthmatic patients, and reviews the differential diagnosis and treatment of mucus plugs.
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To achieve a sensitive and accurate method in body temperature measurement of cattle, this study explores the uses of infrared thermography (IRT), an anemometer, and a humiture meter as a multiple sensors architecture. The influence of environmental factors on IRT, such as wind speed, ambient temperature, and humidity, was considered. The proposed signal processes removed the IRT frames affected by air flow, and also eliminated the IRT frames affected by random body movement of cattle using the frame difference method. In addition, the proposed calibration method reduced the impact of ambient temperature and humidity on IRT results, thereby increasing the accuracy of IRT temperature. The difference of mean value and standard deviation value between recorded rectal reference temperature and IRT temperature were 0.04 °C and 0.10 °C, respectively, and the proposed system substantially improved the measurement consistency of the IRT temperature and reference on cattle body temperature. Moreover, with a relatively small IRT image sensor, the combination of multiple sensors architecture and proper data processing still achieved good temperature accuracy. The result of the root-mean-square error (RMSE) was 0.74 °C, which is quite close to the accurate result of the IRT measurement.
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Temperatura Corporal , Termografía , Animales , Bovinos , Humedad , Rayos Infrarrojos , Temperatura , VientoRESUMEN
Anaphylaxis is a hypersensitivity reaction that is rapid in onset, multi-system involved and can be fatal.Adrenaline is the first-line treatment of anaphylaxis.Adrenaline autoinjector is an important device in emergent situation.In European Academy of Allergy and Clinical Immunology anaphylaxis guideline updated in 2021, adrenaline autoinjector is systemically described and recommended.The following interpretation is focused on adrenaline autoinjector, including advantages, indications for prescription, pharmacokinetic data, dose and long-term management, aimed to enhance understanding of this device and standardize future application.
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Objective:To summarize the clinical features of children with autosomal dominant hyper-IgE syndrome (AD-HIES) and the differential diagnosis of hyper-IgE syndrome and allergic diseases as well.Methods:All clinical data, including general information, clinical features, and genetic changes, from 7 children with AD-HIES who were diagnosed in Beijing Children′s Hospital Affiliated to Capital Medical University from April 2016 to June 2020 were analyzed retrospectively.The diagnostic criteria are based on the National Institutes of Health′s (NIH)′s hyper-IgE syndrome score and combined with the results of gene detection, shown as follows: (1) NIH score over 40, with signal transducer and activator of transcription 3 gene ( STAT3) pathogenic mutation; (2) NIH score between 20 and 40, with reported STAT3 pathogenic mutation; (3) NIH score less than 20 points was excluded. Results:There were 3 males and 4 females.The onset age of 7 cases was within 2 months after birth, and the mean age at diagnosis was 3 years old.All seven cases had recurrent skin or lung infections, with 4 cases having skin and lung infections, 1 case of skin abscesses at the BCG vaccination site, and 2 cases without skin infection suffering from recurrent pneumonia.The mean onset age of skin abscess in 5 cases was 1.5 years, and pus culture of 3 cases were Staphylococcus aureus.Four cases developed bullae and 6 cases had lung infections.Four cases had otitis media, and oral thrush was seen in 4 cases.One case of skin and lung infection developed liver abscess and sepsis.Seven cases had eczema, which was disco-vered in the neonatal period for 6 cases.Four cases had the symptoms of eczema for the first visit.Two cases had food allergy, and 1 case had recurrent wheezing within 1 year old.The serum IgE level and blood eosinophil count in 7 children were elevated.All children had heterozygous pathogenic mutations in STAT3.Six patients had de novo mutations.There were 6 different mutation sites.The 4 mutation sites were reported: c.1145G>A, c.1144C>T, and c. 1699A>G were missense mutations, and c. 1139+ 5G>A was splicing mutation.Two mutation sites had not been reported: c.1031A>C was missense mutation, and c. 2050G>T was nonsense mutation.The pathogenic grade of them were likely pathogenic, and the NIH score of 2 cases were above 40 score, which was consistent with the clinical diagnosis of hyper-IgE syndrome. Conclusions:Eczema is a common and early clinical manifestation of hyper-IgE syndrome, along with elevated IgE levels and eosinophil counts that need to be differentiated from allergic diseases.On the contrary, it often had recurrent skin abscesses or pneumonia, which was prone to bullae.The clinical manifestations of young children were atypical, and genetic testing was helpful for early diagnosis.