Pulmonary lymphomatoid granulomatosis in a 4-year-old girl:A case report

BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.

Expert consensus on the diagnosis, treatment, and prevention of respiratory syncytial virus infections in children

Background Respiratory syncytial virus(RSV)is the leading global cause of respiratory infections and is responsible for about 3 million hospitalizations and more than 100,000 deaths annually in children younger than 5 years,representing a major global healthcare burden.There is a great unmet need for new agents and universal strategies to prevent RSV infections in early life.A multidisciplinary consensus development group comprising experts in epidemiology,infectious diseases,respiratory medicine,and methodology aims to develop the current consensus to address clinical issues of RSV infections in children.Data sources The evidence searches and reviews were conducted using electronic databases,including PubMed,Embase,Web of Science,and the Cochrane Library,using variations in terms for"respiratory syncytial virus","RSV","lower respiratory tract infection","bronchiolitis","acute","viral pneumonia","neonatal","infant""children",and"pediatric".Results Evidence-based recommendations regarding diagnosis,treatment,and prevention were proposed with a high degree of consensus.Although supportive care remains the cornerstone for the management of RSV infections,new monoclonal antibodies,vaccines,drug therapies,and viral surveillance techniques are being rolled out.Conclusions This consensus,based on international and national scientific evidence,reinforces the current recommendations and integrates the recent advances for optimal care and prevention of RSV infections.Further improvements in the management of RSV infections will require generating the highest quality of evidence through rigorously designed studies that possess little bias and sufficient capacity to identify clinically meaningful end points.

Factors controlling fetal echocardiography determine the diagnostic accuracy of isolated ventricular septal defect

Background:Fetal echocardiography (FECG) is a key screening tool for prenatal cardiac abnormalities.Herein,we examined the ultrasonic factors determining prenatal ultrasonic diagnosis of isolated ventricular septal defect (IVSD).Methods:The diagnostic role of ultrasonic factors was investigated in patients in middle or late pregnancy,diagnosed with IVSD by FECG and confirmed using postnatal echocardiography.Results:One hundred and six patients with IVSD were enrolled;the majority had perimembranous VSD.The combined imaging mode of 2 dimentionalechocardiography (2DE) and color doppler flow imaging (CDFI) showed the highest rate (56.6%) of IVSD detection,while CDFI was more efficient than 2DE (32.1% vs.11.3%).The single-view mode was more efficient than multiple-view mode (75.5% vs.24.5%).The highest efficient mode to detect IVSD was achieved using combined imaging mode on the single view of the left ventricular outflow tract view (LVOTV) (28.3%).FECG correctly classified 71.7% of fetal IVSD.There was a significant difference of accuracy rate in classifying IVSD among the three different imaging modes (x2=7.141,P<0.05).The single imaging mode of CDFI and the mode of CDFI combined with 2DE correctly classified 75.9% and 75.0% of fetal IVSD,respectively.LVOTV was the most accurate view of fetal IVSD classification (85.2%;x2=15.782,P<0.05).There was no difference in accuracies of IVSD classification among multiple-view modes (x2=2.343,P>0.05) or between single-view mode and multiple-view mode (x2=0.32,P>0.05).Conclusion:Single LVOTV in CDFI or CDFI combined with 2DE of FECG were the most effective diagnostic modes for fetal IVSD diagnosis.

Geographic variations in the incidence of congenital hypothyroidism in China: a retrospective study based on 92 million newborns screened in 2013–2018

Background:Although congenital hypothyroidism(CH)has been widely studied in Western countries,CH incidence at different administrative levels in China during the past decade remains unknown.This study aimed to update the incidence and revealed the spatial pattern of CH incidence in the mainland of China,which could be helpful in the planning and implementation of preventative measures.Methods:The data used in our study were derived from 245 newborns screening centers that cover 30 provinces of the Chinese Newborn Screening Information System.Spatial auto-correlation was analyzed by Global Moran I and Getis-Ord Gi statistics at the provincial level.Kriging interpolation methods were applied to estimate a further detailed spatial distribution of CH incidence at city level throughout the mainland of China,and Kulldorff space scanning statistical methods were used to identify the spatial clusters of CH cases at the city level.Results:A total of 91,921,334 neonates were screened from 2013 to 2018 and 42,861 cases of primary CH were identified,yielding an incidence of 4.66 per 10,000 newborns screened(95%confidence interval[CI]:4.62–4.71).Neonates in central(risk ratio[RR]=0.84,95%CI:0.82–0.85)and western districts(RR=0.71,95%CI:0.69–0.73)had lower probability of CH cases compared with the eastern region.The CH incidence indicated a moderate positive global spatial autocorrelation(Global Moran I value=0.394,P<0.05),and the CH cases were significantly clustered in spatial distribution.A most likely city-cluster(log-likelihood ratio[LLR]=588.82,RR=2.36,P<0.01)and 25 secondary city-clusters of high incidence were scanned.The incidence of each province and each city in the mainland of China was estimated by kriging interpolation,revealing the most affected province and city to be Zhejiang Province and Hangzhou city,respectively.Conclusion:This study offers an insight into the space clustering of CH incidence at provincial and city scales.Future work on environmental factors need to focus on the effects of CH occurrence.

Forkhead box M1 transcription factor:a novel target for pulmonary arterial hypertension therapy

Background Forkhead box M1(FoxM1),a member of forkhead family,plays a key role in carcinogenesis,progression,invasion,metastasis and drug resistance.Based on the similarities between cancer and pulmonary arterial hypertension,studies on the roles and mechanisms of FoxM1 in pulmonary arterial hypertension have been increasing.This article aims to review recent advances in the mechanisms of signal transduction associated with FoxM1 in pulmonary arterial hypertension.Data sources Articles were retrieved from PubMed and MEDLINE published after 1990,including-but not limited to FoxM1 and pulmonary arterial hypertension.Results FoxM 1 is overexpressed in pulmonary artery smooth muscle cells in both pulmonary arterial hypertension patients and animal models,and promotes pulmonary artery smooth muscle cell proliferation and inhibits cell apoptosis via regulating cell cycle progression.Multiple signaling molecules and pathways,including hypoxia-inducible factors,transforming growth factor-β/Smad,SET domain-containing 3/vascular endothelial growth factor,survivin,cell cycle regulatory genes and DNA damage response network,are reported to cross talk with FoxM1 in pulmonary arterial hypertension.Proteasome inhibitors are effective in the prevention and treatment of pulmonary arterial hypertension by inhibiting the expression and transcriptional activity of FoxM1.Conclusions FoxM1 has a crucial role in the pathogenesis of pulmonary arterial hypertension and may represent a novel therapeutic target.But more details of interaction between FoxM1 and other signaling pathways need to be clarified in the future.