The scholars abroad have reported a group of previously normal children,who develop intractable focal epilepsy or status epilepticus after fever,followed by drug-resistant partial epilepsy and severe cognitive impairment,named febrile infection-related epilepsy syndrome (FIRES).There are unknown etiology,critical condition,poor prognosis and absent of specific changes on cerebrospinal fluid,biopsies and neuroimagings.No therapeutic agent is efficacious and ketogenic diet may be an alternative therapy in some reports.This study describes the clinical characteristics of FIRES.

Hypomelanosis of Ito is a multisystem neurocutaneous syndrome. The pathogenesis hasn′t been clear and typical cutaneous lesions are associated with variable extracutaneous abnormalities involved the central nervous system and musculoskeletal system. Its clinical manifestations include abnormal skin biopsy and chromosomal patterns and brain iconography. The treatments have to be adapted to the individual and prognosis depends on the severity of clinical manifestations and complications.

Objective To evaluate the echocardiographic features of coronary artery dilation (CAD) and coronary artery aneurysms (CAA) in Kawasaki disease dynamically.Methods Echocardiography was performed in KD cases for measurement of the diameter of the coronary artery.Results Twelve CAD and four CAA were identified in forty-one patients.In CAD group,left coronary artery (LCA) is more susceptive to dilation than right coronary artery (RCA)(P<0.05).Both LCA and RCA were involved in CAA group,the maximum diameter of coronary artery is 10mm.Six to eighteen days after treatment,LCA were regressed significantly in CAD group(P<0.05),but the diameter of RCA in CAD group and coronary artery in CAA patients have no change(P>0.05).Ten coronary artery lesion patients were followed up,8 CAD cases regressed completely,1 CAA extended,the other CAA regressed completely.Conclusions In CAD group,LCA is more susceptive to dilation than RCA,but it regressed significantly in a short time after treatment.Echocardiography is a non-traumatic method to detect the coronary artery lesions in KD patients dynamically.

Objective To investigate the clinical features of incomplete Kawasaki disease (IKD).Methods The data of 46 Kawasaki disease (KD) patients including 14 IKD patients (IKD group)and 32 classical Kawasaki disease (CKD) patients (CKD group) was analyzed retrospectively,and compared the clinical manifestation,laboratory examination result,the incidence of coronary artery lesion (CAL) and the response to intravenous immunoglobulin (IVIG) of two groups.Results There was no significant difference in gender and age between IKD group and CKD group (P> 0.05 ).In IKD group,the fever time was longer than that in CKD group [( 11.43 ± 9.12) d vs (7.12 ± 2.83 ) d, P < 0.05], but the frequency of conjunctival congestion, lymphadenectasis of neck, indurative edema of palms and soles, changes of lips and oral cavity were less than these in CKD group [57.14%(8/14) vs 93.75%(30/32),28.57%(4/14) vs 59.38%(19/32), 35.71%(5/14) vs 78.12%(25/32),42.86%(6/14) vs 75.00%(24/32)](P< 0.01 or < 0.05).There was no difference in laboratory examination result and incidence of CAL between two groups, but the incidence of IVIG nonresponse in IKD group was higher than that in CKD group [40.0%(4/10) vs 6.45%(2/31 ),P <0.05].Conclusions The frequency of conjunctival congestion, indurative edema of palms and soles, changes of lips and oral cavity are less common in IKD patients comparing with CKD patients.The laboratory examination result and the risk of CAL in IKD patients are similar to CKD ones.Moreover, IKD is not sensitive to IVIG, so the doctors should pay more attention to it.

Objective:This study was aimed to analyze the untargeted metabolomics of serum samples from children with mycoplasma pneumonia in a hospital in Beijing.Methods:A total of 50 children with mycoplasma pneumonia as the case group were recruited from Department of Pediatrics, China-Japan Friendship Hospital in Beijing from January 2019 to February 2020, and meanwhile 50 age-and gender-matched heathy children were selected and formed the control group. 2 ml venous fasting blood samples was collected from all children. Serum metabolites were quantified by using the untargeted ultra-high performance liquid chromatography/tandem mass spectrometry (UPLC-MS/MS) technique. Unsupervised principle component analysis and (orthogonal) partial least-squares-discriminant analysis were employed to identify differential metabolites between cases and controls. MBRole software was used for pathway enrichment analysis.Results:There were 27 boys and 23 girls in the case group with an average age of (6.0±3.65) years, and the control group consisted of 28 boys and 22 girls with an average age of (6.62±2.64) years. A total of 392 different metabolites were detected. Compared with the control group, 306 metabolites were decreased and 86 increased in case group. Forty-one differential metabolites with variable important in projection (VIP) values larger than 5 and P values less than 0.05 were teased out, and they mainly concentrated on phospholipid. The levels of 38 metabolites were significantly lower in the case group, yet 4 metabolites were significantly higher than that of the control group. Metabolic enrichment analysis showed that different metabolites were related to the biosynthesis of phenylalanine, tyrosine, tryptophan, unsaturated fatty acid, ammonia acyl tRNA and insulin signaling pathway, as well as the metabolism of ABC transporters. Conclusion:The serum untargeted metabolomics differed remarkably between children with mycoplasma pneumonia and healthy children.

Objective:This study was aimed to analyze the untargeted metabolomics of serum samples from children with mycoplasma pneumonia in a hospital in Beijing.Methods:A total of 50 children with mycoplasma pneumonia as the case group were recruited from Department of Pediatrics, China-Japan Friendship Hospital in Beijing from January 2019 to February 2020, and meanwhile 50 age-and gender-matched heathy children were selected and formed the control group. 2 ml venous fasting blood samples was collected from all children. Serum metabolites were quantified by using the untargeted ultra-high performance liquid chromatography/tandem mass spectrometry (UPLC-MS/MS) technique. Unsupervised principle component analysis and (orthogonal) partial least-squares-discriminant analysis were employed to identify differential metabolites between cases and controls. MBRole software was used for pathway enrichment analysis.Results:There were 27 boys and 23 girls in the case group with an average age of (6.0±3.65) years, and the control group consisted of 28 boys and 22 girls with an average age of (6.62±2.64) years. A total of 392 different metabolites were detected. Compared with the control group, 306 metabolites were decreased and 86 increased in case group. Forty-one differential metabolites with variable important in projection (VIP) values larger than 5 and P values less than 0.05 were teased out, and they mainly concentrated on phospholipid. The levels of 38 metabolites were significantly lower in the case group, yet 4 metabolites were significantly higher than that of the control group. Metabolic enrichment analysis showed that different metabolites were related to the biosynthesis of phenylalanine, tyrosine, tryptophan, unsaturated fatty acid, ammonia acyl tRNA and insulin signaling pathway, as well as the metabolism of ABC transporters. Conclusion:The serum untargeted metabolomics differed remarkably between children with mycoplasma pneumonia and healthy children.

Ulcerative colitis （UC） is a chronic non-specific inflammatory disease characterized by the damage of the epithelial barrier of the colon and the destruction of immune homeostasis. It has a long course， no recovery and high recurrence rate， and is recognized as a difficult digestive disease. MicroRNA （miRNA） has been confirmed to be specifically or differentially expressed in both UC patients and UC animal models， so miRNA can be used as markers for UC diagnosis or reference for treatment evaluation. TCM therapy has a definite therapeutic effect， a wide range of effects， and minimal side effects in the treatment of UC， so this article takes miRNA as the starting point and systematically elaborates on the mechanism of TCM regulating UC related signaling pathways by regulating the expression of miRNA. The results show that chlorogenic acid， Anchang decoction， and Fuyang huoxue jiedu formula can regulate the expressions of miR-155， miR-146a and miR-31-5p， etc.， thereby inhibiting signal transducer and activator of transcription （STAT） signal pathway transduction to improve UC. Limonin， ginsenoside Rh2， artesunate， etc. can inhibit nuclear factor-κB （NF-κB） signaling pathway conduction to improve UC by regulating the expressions of miR-214， miR- 155 and miR-19a， etc. Nitidine chloride， berberine， resveratrol， etc. can regulate the expressions of miR-31， miR-146a， miR- 146b， etc.， thereby inhibiting the Toll-like receptor 4 （TLR4） signaling pathway to improve UC. Mango polyphenolics， Compound qinbai granules， and Astragalus membranaceus polysaccharides can regulate the expressions of miR-126 and miR-193a-3p， thereby inhibiting the phosphoinositide 3-kinase/protein kinase B/mammalian target of rapamycin （PI3K/AKT/mTOR） signaling pathway to improve UC.