Objective To investigate the value of magnetic resonance imaging-proton density fat fraction(MRI-PDFF)in evaluating hepatic steatosis in patients with chronic hepatitis B virus(HBV)infection.Methods The patients,aged>16 years,who visited the outpatient service or were hospitalized in Beijing Tsinghua Changgung Hospital from January 2018 to December 2022 and were diagnosed with chronic HBV infection were enrolled,and all patients underwent MRI examination of the liver in our hospital.The patients were divided into groups based on the presence or absence of liver cirrhosis,and the consistency in PDFF between different hepatic segments was compared between groups.The Kappa consistency test and intraclass correlation coefficient(ICC)were used for consistency analysis.Results A total of 76 patients treated with nucleoside analogues were enrolled,among whom 23(30.26％)had liver cirrhosis.For all patients,the simple arithmetic average of PDFF fluctuated between 1.49％and 30.93％.According to MRI-PDFF≥5％as the diagnostic criterion for fatty liver disease,there were 29 patients(38.16％)with fatty liver disease among all patients.For all 76 patients,the simple arithmetic average of PDFF was lower than the weighted average of PDFF for the whole liver,and there was no significant difference between the simple arithmetic average of PDFF,the weighted average of PDFF,and the PDFF values of the left and right lobes of the liver(F=0.39,P=0.76).The consistency test showed that the PDFF values of each hepatic segment and the left and right lobes of the liver had strong consistency with the weighted average and simple arithmetic average of PDFF,with an ICC of>0.75,but the consistency between the PDFF value of the right lobe and the weighted average of PDFF was higher than that between the PDFF value of the left lobe and the weighted average of PDFF.In the consistency test of differentiating fatty liver disease in patients with liver cirrhosis,there was poor consistency between the PDFF value of segment Ⅶ and the weighted average of PDFF(Kappa=0.39),with moderate consistency for the left lobe and the Ⅰ,Ⅱ,Ⅲ,Ⅴ,Ⅵ,and Ⅷ segments.For the patients with liver cirrhosis,the lowest consistency was observed between the PDFF value of Ⅶ segment and the weighted average of PDFF for the whole liver,and the highest consistency was observed between the PDFF value of Ⅵ segment and the weighted average of PDFF for the whole liver.For the patients without liver cirrhosis,the lowest consistency was observed between the PDFF value of Ⅱ segment and the weighted average of PDFF for the whole liver,and the highest consistency was observed between the PDFF value of Ⅴ segment and the weighted average of PDFF for the whole liver.Conclusion MRI-PDFF is more comprehensive in evaluating hepatic steatosis in patients with chronic HBV infection,and for the patients with liver cirrhosis,there is poor consistency between the PDFF value of each segment and the weighted average of PDFF.

Acute necrotizing encephalopathy (ANE) is a subtype of acute encephalopathy presented with disturbance of consciousness and symmetric bilateral thalamic necrosis in neuroradiology.Patients with ANE had a high mortality or severe neurological sequela.ANE usually secondary to virus infectious disease, in which influenza is a common etiology.During the 3 years of the worldwide pandemic of severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection, ANE has become a severe complication and cause of death in children, which has aroused much concern.Here is a review of the research progress of epidemiology, pathogenesis, diagnosis, treatments and prognosis of ANE, in order to improve the knowledge of clinicians on this disease.

Cystic fibrosis (CF) is a multisystem disease caused by CFTR gene mutations, affecting the lungs, digestive system, and reproductive tract.Infectious lung disease is the major cause of exacerbation and mortality in most patients.There are specific pathogen spectrums in infectious lung disease and antibiotics application principles in CF patients.In this article, the application of antibiotics in clinical guidelines for care of children with CF in Royal Brompton Hospital (2020) was introduced.

Objective:To investigate the feasibility of nebulizing injectable Tobramycin for the treatment of Pseudomonas aeruginosa (Pa) in pediatric cystic fibrosis (CF) patients. Methods:The clinical data of 3 CF children with Pa infection who were treated by nebulizing injectable Tobramycin in Shenzhen Children′s Hospital from September 2017 to March 2021 were retrospectively analyzed.The efficacy and safety were explored.The nebulized injectable Tobramycin (160 mg/dose) was given twice daily after airway clearance.After one-month treatment course, oral Ciprofloxacin would be given [30 mg/(kg·d)] to patients for another 1 month if Pa was still positive in repeat sputum cultures.Results:There were 2 males and 1 female in 3 cases recruited.The youngest patient was 1-year-old when receiving Tobramycin treatment.After airway clearance and inhalation of injectable Tobramycin, all had improvements in respiratory symptoms and chest CT scan.Two cases took additional oral Ciprofloxacin as Pa was still positive after the 1-month treatment course of Tobramycin.Pa turned to negative in all 3 cases after treatment for 3 months to 1.5 years.Besides, after treatment all the 3 patients had normal liver and renal functions, and normal hearing in multiple follow ups.One patient had a normal brainstem auditory evoked potential in the reexamination.Conclusions:Nebulizing injectable Tobramycin would be a reasonable alternative to inhaled Tobramycin solution for treating pediatric CF patients with Pa in view of the present condition in mainland China.However, it is still worth further study and discussion.

Objective:To investigate the etiological characteristics and changes of plastic bronchitis(PB)in children from 2010 to 2019 at Shenzhen Children′s Hospital, and provide reference basis for improving the understanding of PB etiology.Methods:The clinical data of children diagnosed with infectious-associated PB at Shenzhen Children′s Hospital from Jan 2010 to Dec 2019 were retrospectively analyzed, and the etiological characteristics and changes were summarized.Results:There were 94 cases of mycoplasma pneumoniae, 38 cases of influenza virus, 41 cases of adenovirus, 16 cases of mixed infection, 11 cases of bacteria, and 57 cases of unclear etiology in 266 infectious-associated PB children.The distribution of PB in each age group: 15 cases were infants, 63 cases were toddlers, 112 cases were preschoolers, and 76 cases were school-age children.Adenovirus was the main pathogen of PB in infants and toddlers(60.0%, 28.6%), and mycoplasma pneumoniae(34.8%, 60.5%) as well as influenza virus(13.4%, 22.4%) were the main pathogen in preschool and school-age children, with statistically significant difference( P<0.001). From 2010 to 2019, the annual positive rates of pathogens were 62.5%, 60.0%, 66.7%, 74.1%, 64.0%, 50.0%, 93.3%, 57.1%, 75.0%, and 84.7%, respectively.PB was caused by mycoplasma pneumoniae infection every year.From 2016 to 2019, PB caused by mycoplasma pneumoniae infection increased year by year, while PB caused by adenovirus infection increased every other year. Conclusion:Mycoplasma pneumoniae was the most common pathogen of PB, followed by adenoviruses and influenza viruses, while bacteria, fungi and other viruses were relatively rare.In the infant group, adenovirus infection was predominant, while in preschool and school-age children group, mycoplasma pneumoniae and influenza virus infection were predominant.

In recent years, with being gradually developed, three-dimensional (3D) reconstruction based on pathology and medical imaging technology has shown certain value in the diagnosis and treatment of breast cancer. And with its advantages of providing the spatial location, morphological structure and 3D structure relationships with the surrounding tissues and organs, 3D reconstruction technology has played a key role in the early diagnosis, surgical treatment, and accurate evaluation of the treatment effect after surgery of breast cancer. Although the application of 3D reconstruction technology based on pathology and medical imaging is still inadequate, with the continuous development of science and technology, 3D reconstruction technology will play an increasingly important role in the diagnosis, personalized treatment and prognosis assessment of breast cancer.

Objective:To investigate the etiology, clinical characteristics and outcome of severe pneumonia-associated hemophagocytic lymphohistiocytosis, and to analyze the risk factors for mortality.Methods:Clinical data of patients with severe pneumonia-associated hemophagocytic lymphohistiocytosis admitted to Shenzhen Children′s Hospital from February 2009 to February 2019 were retrospectively analyzed.The data included clinical characteristics, etiology, clinical manifestations, laboratory data, treatment and outcomes of the patients.The clinical characteristics and laboratory data of the survival group and the death group were compared by independent sample t-test. Results:(1) Clinical characteristics: the patients were aged from 3 months to 8 years and 7 months, including 15 males and 15 females.Severe pneumonia-associated hemophagocytic lymphohistiocytosis accounted for 2.74% (30/1 096 cases) of severe pneumonia in the same period.(2) Etiology: Mycoplasma pneumoniae infection was found in 8 cases (8/30 cases, 26.67%), virus infection in 7 cases (7/30 cases, 23.33%, including 5 cases with adenovirus infection, 1 case with EB virus infection, and 1 case with cytomegalovirus infection), Mycoplasma pneumoniae complicated with adenovirus infection in 4 cases (4/30 cases, 13.33%), bacterial infection in 3 cases (3/30 cases, 10%), and fungal infection in 2 cases, Mycobacterium tuberculosis infection in 1 case.The pathogens were not identified in 5 patients.(3) Clinical manifestations: fever and hepatomegaly were present in all patients.Besides, 86.67% (26/30)patients had fever duration more than 10 days, 83.33% (25/30 cases) patients had cough, 76.66% (23/30 cases) patients had splenomegaly, and 33.33% (10/30 cases) patients had nervous system symptoms.Laboratory data showed varying degrees of reduction of binary and ternary systems in 80.00%(24/30 cases) of the patients.Liver function impairment was found in half of the patients, and serum ferritin and lactate dehydrogenase levels were elevated in all patients.(4) The mortality rate was 30.00% (9/30 cases). The differences in age, hypertriglyceridemia and high serum ferritin levels between the survival and death groups were significant (all P<0.05). Conclusions:Severe pneumonia-associated hemophagocytic lymphohistiocytosis is a disease with a high mortality rate.Patients with Mycoplasma pneumoniae and adenovirus pneumonia are more likely to suffer from secondary hemophagocytic lymphohistiocytosis.Younger age, hypertriglyceridemia and high serum ferritin levels are indicative of poor prognosis.

Objective:To summarize the clinical characteristics of adenovirus (HAdVs) pneumonia in children.Methods:The clinical manifestations, laboratory results, imaging features and treatment courses of 541 children with HAdVs pneumonia treated in Shenzhen Children′s Hospital from January 2017 to August 2019 were retrospectively studied.Results:The number of children diagnosed with HAdVs pneumonia increased year by year.A total of 541 patients were included in this study (214 females and 327 males, median age 31 months). The majority of patients (89.3%, 483/541 cases) were less than 6 years of age.Cough (98.3%, 532/541 cases) was the most common symptom, followed by fever (93.7%, 507/541 cases), tachypnea (36.6%, 198/541 cases) and wheeting (32.5%, 176/541 cases). Thirteen point three percent patients (72/541 cases) had existing primary diseases.Severe HAdVs pneumonia was observed in 33.8% of patients (183/541 cases), the most common intrapulmonary complication were pleural effusion(18.6%, 101/541 cases) and respiratory failure (7.0%, 38/541 cases). In co-infection cases, 46.9% of the patients (254/541 cases) suffered from co-infection with mycoplasma pneumoniae, 21.6% of the patients (117/541 cases) suffered from bacterial co-infection.Ninety-eight point five percent of the patients (533/541 cases) were cured, and 1.5% of the patients (8/541 cases) died or discharged voluntarily.Convulsions [ P=0.026, OR=7.312 (95% CI: 1.267-42.191)] and bacterial co-infections [ P=0.030, OR=2.724 (95% CI: 1.101-6.741)] were independently correlated with the occurrence of severe HAdVs pneumonia. Conclusions:Pre-school aged children are at higher risks of developing HAdVs pneumonia.The majority of patients presents with fever and cough as primary manifestations.Tachypnea and wheezing are also common clinical features of HAdVs pneumonia patients.Approximately a third of children with HAdVs pneumonia may develop into severe pneumonia.The presentation of convulsion and the exis-tence of bacterial co-infections may help to predict the development of severe HAdVs pneumonia in pediatric patients.

Objective: To summarize the clinical features of immunodeficiency diseases with interstitial lung disease (ILD) as major clinical manifestations and to improve understanding etiology of ILD. Methods: The clinical features and clinical clues for diagnosis of six cases with immunodeficiency presented with ILD in Shenzhen Children′s Hospital from January 2014 to December 2016 were retrospectively analyzed. Results: The patients′ age ranged from 3 months to 5 years and 9 months, 5 cases were male. All cases had cough and tachypnea, 3 cases had lung infection and respiratory failure, 2 cases had chronic hypoxia and one had clubbing. Three cases had skin rashes; 5 cases had failure to thrive. Chest CT scan showed diffuse ground glass opacity in all the 6 cases, and 2 cases had cystic changes and one had "crazy-paving" pattern. Five patients were suspected to have surfactant dysfunction and genetic testing was performed before diagnosis of immunodeficiency, of which the results were negative. With human immunodeficiency virus antibody test or immunologic laboratory testing and/or immune genetic panel, acquired immune deficiency syndrome was confirmed in one case, hyper-IgM syndrome was confirmed in two cases and hyper-IgE syndrome in one case, Wiskott-Aldrich syndrome in one and STAT3 gain of function genetic mutation in another. All cases had clinical clues indicative of underlying immunocompromise. Conclusions The clinical features of immunodeficiency diseases with ILD are cough, tachypnea or hypoxia, respiratory failure with infection, diffuse ground glass opacity in Chest CT imaging. With thorough medical history and immunology screening, there would be clinical clues indicative of underlying immunocompromise. Screening for immunodeficiency disease should be emphasized in the differential diagnosis of ILD, otherwise it may lead to misdiagnosis or unnecessary testing.

Objective To investigate the clinical features and outcomes of pulmonary surfactant protein C gene (SFTPC) 218 site mutation in children with pulmonary interstitial disease.Methods In this retrospective study,the clinical data,outcomes and influencing factors of 7 cases of SFTPC gene 218 site mutations in infants with interstitial lung disease in three hospitals from January 2013 to December 2016 were analyzed.Results Seven cases were full-term children,4 cases had the onset within 3 months after birth,2 cases after 1 year old,1 case within 3 months to 1 year,clinical manifestations of these cases were cough,shortness of breath,dyspnea,and limited growth and development,could not maintain life without additional oxygen supplementation,blood gas analysis showed hypoxemia,4 cases had clubbing.Chest CT showed diffuse ground glass-like change in both lungs.Three cases were positive for cytomegalovirus (CMV)-IgM or CMV-DNA.The mutations in 7 cases were exon 3,5 of which were SFTPC gene c.218T＞C,p.lle73Thr (heterozygous mutation),and 2 cases were SFTPC gene c.218T＞A,p.lle73Asn (homozygous mutation),1 case combined with ABCA3 gene mutations.Four patients were treated with prednisone alone,one with prednisone plus hydroxychloroquine,and two with symptomatic treatment.Three patients died,3 patients improved,and 1 patient was lost to follow-up.Conclusions The severity and prognosis of the children with SP-C 218 site mutation may be affected by many factors.Some children who received glucocorticoid alone do not have a good response.