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Objective:To investigate the value of endoscopic retrograde cholangiopancreatography(ERCP)and related techniques in the diagnosis and treatment of chronic pancreatitis in children.Methods:The clinical data of 16 children with chronic pancreatitis diagnosed in the Department of Gastroenterology at Beijing Children′s Hospital from January 2021 to January 2022, who had ERCP indications were analyzed retrospectively, including age, sex, operation time, postoperative complications and follow-up data.Results:Thirty nine ERCP operations were performed in 16 children, with the age of (7.20±2.51) years old, and the minimum weight of 13 kg.There were 7 males and 9 females.The clinical manifestations were upper abdominal pain in all cases, 3 patients with dyspnea, 1 patient with gastrointestinal bleeding, and 1 patient with diabetes.In terms of etiology, 16 cases were diagnosed by ERCP including, 7 cases with pancreatic divisum, 1 case with abnormal pancreaticobile duct confluence.Among the 16 children, 11 had gene examination with 9 gene mutations (including 7 cases SPINK1 mutation, 1 case PRSS mutation, and 1 case CFTR mutation).The operation time was 30 to 65 minutes, and the median was 43 minutes.The operation time was negative correlation with age, while there was no correlation with the anatomical structure abnormality or the gene mutation.Among the 16 children, 15 were placed with pancreatic duct stents, with a success rate of 93.8%.Three children had postoperative pancreatitis, the rest had hyperamylasemia.Postoperative complications were not related with the age, the anatomical structure abnormality or the gene mutation.All children had been followed up for more than one year.All children have not suffered from pancreatitis again, and the body mass index had increased significantly after operation than before( P<0.05).The ERCP operation was performed 2 to 4 times in children after stent replacement, and the time of stent replacement ranged from 3 months to 12 months. Conclusion:The main causes of chronic pancreatitis in children are anatomical abnormalities or gene mutations.ERCP and related techniques are minimally invasive, safe and effective in the treatment of chronic pancreatitis.
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Objective:To compare the efficacy of combination therapy on cyclic vomiting syndrome(CVS)in children, and improve the efficacy of CVS treatment in the future.Methods:This study retrospectively analyzed patients′ medical records of CVS, which were admitted to Digestive Department of Beijing Children′s Hospital from 2012 to 2019.The treatment regimen was A(Cyproheptadine+ Doxepin+ Valproate), B(Propranolol+ Cyproheptadine), or C(Propranolol+ Amitriptyline). Meanwhile, the patients should take drugs more than three months.The clinical data of 42 cases were analyzed retrospectively, and the treatment effect after discharge was followed up by telephone until October, 2020.Results:Among the 42 cases, 17 were male and 25 were female, whose mean age of onset was (4.65±3.23) years, and the age of diagnosis was (6.79±3.58) years.The main accompanied symptoms were abdominal pain and upper gastrointestinal bleeding.Forty-two patients were moderate/severe CVS.The regimens A, B and C were observed in 7, 11, and 24 patients, respectively.The age at improvement was(8.17±4.12)years.The course of treatment was(1.37±0.96)years.The age at follow-up was(10.32±4.03)years.During the 1-year follow-up, 35 cases were effective, and the efficiency was 83.3%.Among them, 23 cases had no paroxysmal vomiting and 7 cases had no effect.There was no significant difference in therapy effects among group A, B and C. Between the effective group and non-effective group, there were statistical differences in the personal history of hiatus hernia( P=0.024), the weight at follow-up ( P=0.042), and the course of medication( P=0.020). Conclusion:The combination regimen has a higher effective rate in the treatment of CVS.There was no significant difference among the three regimens in the treatment of CVS.For children with refractory CVS, who can not be treated with combination therapy, individualized therapy should be further developed.
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Objective:To evaluate the efficacy and safety of infliximab(IFX)in pediatric patients with ulcerative colitis(UC).Methods:The clinical data of 17 UC patients who received IFX treatment at Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2021 were retrospectively analyzed, and the pediatric ulcerative colitis activity index(PUCAI)and laboratory data were compared before and after treatment to evaluate the efficacy and safety of IFX.Results:A total of 17 UC patients were included, and among them there were 9 boys and 8 girls.The age of onset was 12.1(10.7, 12.8)years old, and median age at IFX initiation was 12.5(11.8, 13.6)years old.The duration of IFX medication was 46.1(17.4, 56.9)weeks, and the times of IFX injections was 8.0(4.5, 10.5). The mean PUCAI score of the 17 UC patients at start of IFX treatment was (50.6±21.2) points, and the Mayo endoscopic score showed: ten severe activity, six moderate activity, and one mild activity.IFX efficacy analysis: the clinical response rate was 87.5%(14/16), and the clinical remission rate was 56.3%(9/16) at the 14th week.The sustained response rate was 81.8%(9/11), and the sustained remission rate was 36.4%(4/11) at the 30th week.At the 14th week of IFX treatment, PUCAI score[2.5(0, 10.0)points vs.50.0(41.3, 70.0)points] and white blood cell count[5.7(4.8, 8.6)×10 9/L vs.8.7(6.4, 13.5)×10 9/L] significantly decreased(all P<0.05), hemoglobin[(113.8±20.4)g/L vs.(99.3 ± 19.4)g/L] and albumin level[42.2(40.0, 44.4)g/L vs.36.6(28.6, 40.2)g/L] significantly increased compared with those before IFX treatment, and the differences were statistically significant(all P<0.05). The Mayo endoscopic scores at 14 weeks of IFX treatment in 12 active UC patients showed: only 2 patients achieved mucosal healing, 5 patients had reduced from severe to moderate mucosal inflammation, and 5 patients had no improvement.Seven patients had 10 adverse events, 2 cases had 4 times acute infusion reactions, and 5 cases had 6 times infections. Conclusion:IFX is effective and relatively safe in inducing and maintaining clinical remission in pediatric UC patients.
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Objective:To evaluate the efficacy and safety of infliximab(IFX)in pediatric patients with ulcerative colitis(UC).Methods:The clinical data of 17 UC patients who received IFX treatment at Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2021 were retrospectively analyzed, and the pediatric ulcerative colitis activity index(PUCAI)and laboratory data were compared before and after treatment to evaluate the efficacy and safety of IFX.Results:A total of 17 UC patients were included, and among them there were 9 boys and 8 girls.The age of onset was 12.1(10.7, 12.8)years old, and median age at IFX initiation was 12.5(11.8, 13.6)years old.The duration of IFX medication was 46.1(17.4, 56.9)weeks, and the times of IFX injections was 8.0(4.5, 10.5). The mean PUCAI score of the 17 UC patients at start of IFX treatment was (50.6±21.2) points, and the Mayo endoscopic score showed: ten severe activity, six moderate activity, and one mild activity.IFX efficacy analysis: the clinical response rate was 87.5%(14/16), and the clinical remission rate was 56.3%(9/16) at the 14th week.The sustained response rate was 81.8%(9/11), and the sustained remission rate was 36.4%(4/11) at the 30th week.At the 14th week of IFX treatment, PUCAI score[2.5(0, 10.0)points vs.50.0(41.3, 70.0)points] and white blood cell count[5.7(4.8, 8.6)×10 9/L vs.8.7(6.4, 13.5)×10 9/L] significantly decreased(all P<0.05), hemoglobin[(113.8±20.4)g/L vs.(99.3 ± 19.4)g/L] and albumin level[42.2(40.0, 44.4)g/L vs.36.6(28.6, 40.2)g/L] significantly increased compared with those before IFX treatment, and the differences were statistically significant(all P<0.05). The Mayo endoscopic scores at 14 weeks of IFX treatment in 12 active UC patients showed: only 2 patients achieved mucosal healing, 5 patients had reduced from severe to moderate mucosal inflammation, and 5 patients had no improvement.Seven patients had 10 adverse events, 2 cases had 4 times acute infusion reactions, and 5 cases had 6 times infections. Conclusion:IFX is effective and relatively safe in inducing and maintaining clinical remission in pediatric UC patients.
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Objective:To evaluate the efficacy and safety of Adalimumab(ADA) in the treatment of pediatric Crohn′s disease (CD).Methods:The clinical data of 20 CD patients treated with ADA at Beijing Children′s Hospital, Capital Medical University from September 2016 to September 2021 were retrospectively analyzed.The disease activity status and mucosal inflammation in CD patients were evaluated using the Pediatric Crohn′s Disease Activity Index (PCDAI) and Crohn′s Disease Endoscopic Severity Index(CDEIS). Data were compared between groups using the rank sum test or Fisher′ s exact test. Results:A total of 20 CD patients were recruited, including 12 males and 8 females.The mean age at diagnosis of CD was (9.5 ± 4.9) years old, ranging from 0.9-15.1 years old.The mean age of the first use of ADA was (10.4 ± 4.8) years old, ranging from 1.2-16.7 years old.The median duration of CD symptoms before ADA treatment was 0.9 (0.4, 1.7) years.The mean PCDAI score of 20 CD patients before ADA treatment was (28.5±19.8) points (range: 0-65.0 points). Of the 20 cases, 8 cases (40.0%) had severe disease activity, and 4 cases (20.0%) were in remission.A total of 15 CD patients underwent CDEIS assessment.The results showed that 9 patients had moderate to severe disease activity, and the symptoms were improved in 1 case under endoscopy.Ten patients (10/20 cases, 50.0%) received Infliximab (IFX) treatment preceding ADA treatment.IFX discontinuation was due to the loss of response(8/10 cases, 80.0%) and allergic reactions (2/10 patients, 20.0%). After 6 weeks of ADA treatment, the median PCDAI score of the 20 CD patients was 5.0 (0, 10.0) points, which was significantly lower than that before ADA treatment ( P<0.001). The clinical remission rate and clinical response rate of 16 patients with active CD treated with ADA for 6 weeks were 62.5% (10/16 cases) and 87.5% (14/16 cases), respectively.There were no significant differences in the clinical remission rate and clinical response rate between the patients who did not receive IFX and those who had previously received IFX(all P> 0.05). The median ADA treatment period was 5.5 (2.6, 17.8) months.During the follow-up period, 6 patients (6/20 cases, 30.0%) suffered from clinical recurrence of CD.At the end of the follow-up visit, seventeen patients(17/20 cases, 85.0%) maintained clinical remission, one had primary non-response and two experienced secondary non-response.Adverse events were reported in 7 patients, mainly including pneumonia (4 cases) or upper respiratory tract infection (2 cases). No tumor or other serious adverse events were recorded. Conclusions:ADA has good efficacy in inducing and maintaining clinical remission in pediatric CD patients, and does not cause serious adverse events.
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Objective:To investigate the clinical characteristics of Epstein-Barr(EB)virus associated gastroenteritis, aiming to improve the diagnosis and treatment.Methods:The clinical data of 8 patients diagnosed as EB virus associated gastroenteritis in Department of Gastroenterology, Beijing Children′s Hospital were retrospectively analyzed from January 2017 to December 2021 including the clinical data, laboratory examination, endoscopic and imaging findings, medications and clinical prognosis.Results:There were eight cases with the male: female ratio 6: 2, the average age 7.46 years, and history 20 d to 3 years.Main clinical manifestations were abdominal pain(7/8), diarrhea(4/8), bloody stools(3/8), fever(2/8), hematemesis(1/8), with the high load of serum EB virus DNA in 4 cases.The endoscopic features were rough mucosa, edema, erosion, boundary clear shallow ulcer.The EB virus-encoded small RNA(EBER)was positive in situ hybridization(8/8).The frequently affected sites were upper gastrointestinal tract(3/8), small intestine(2/8)and colon(4/8).The common complications were sepsis(3/8)and mild malnutrition(3/8).All patients became better by the enteral nutrition and antiviral therapy.Conclusion:The EB virus associated gastroenteritis is rare with the serious complications, the non-specific clinical manifestations.The clinical doctors should improve the understanding of this disease in order to improve the diagnosis and therapy.Clinicians should pay more attention to the test of EB virus in serum and membrane, then carry out the accurate treatment.
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Objective:To evaluate the clinical efficacy and safety of Infliximab (IFX) in pediatric Crohn′s disease (CD).Methods:The efficacy of IFX therapy in 30 patients suffering from active CD who were not completely improved with traditional medicine and enteral nutrition or had intolerance to the medicine in Beijing Children′s Hospital Affiliated to Capital Medical University from December 2014 to December 2019 were retrospectively analyzed.Pediatric Crohn′s Disease Activity Index (PCDAI), blood biochemistry indices, mucosal healing, nutritional status, and adverse reactions were compared and evaluated.Results:Thirty active CD cases, with 18 males and 12 females, were enrolled, and the average age was (8.63±4.76) years old.Three cases who didn′t complete 3 times of IFX injection and 1 case who lost to be followed up were excluded.A total of 26 cases of CD in active period were enrolled in this study on efficacy.The clinical remission and response rate of 26 cases were 61.5% and 84.6%, respectively, at 14-week of IFX therapy.The clinical remission and response rate of 21 cases were 71.4% and 85.7%, respectively, at 30-week.The clinical remission and response rate of 15 cases were 86.7% and 93.3%, respectively, at 54-week.At week 14 th, PCDAI score [(9.56±8.05) scores vs.(29.02±10.86) scores] decreased compared with before treatment ( t=7.339, P<0.05). The levels of erythrocyte sedimentation rate [(15.54±10.26) mm/1 h vs.(33.77±21.30) mm/1 h] and C-reactive protein [(4.79±12.94 ) mg/L vs.(16.33±23.43) mg/L] were obviously decreased, and the hemoglobin [(126.27±16.51) g/L vs.(110.58±16.45) g/L], hematocrit [(37.03±3.95)% vs.(33.52±4.32)%], and albumin levels [(42.30±3.03) g/L vs.(37.13±5.68) g/L] were remarkably increased compared with those before treatment ( t=3.932, 1.993, -3.398, -3.060, -4.009, all P<0.05). Height for age Z score and body mass index Z score were increased after IFX treatment, without statistically significant differences (all P>0.05). Conclusions:IFX therapy had good clinical efficacy in controlling inflammatorys and inducing clinical remission in pediatric CD.
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Objective:To summarize clinical and genetic features of infantile-onset inflammatory bowel disease(IO-IBD) patients.Methods:The clinical data of 48 IO-IBD patients from Beijing Children′s Hospital, Capital Medical University, including age of onset, family history, clinical manifestations and drug efficacy were retrospectively analyzed.Based on target gene panel, next generation sequencing (NGS) was performed for 43 patients, and further compared clinical features between gene mutation and non-gene mutation IO-IBD groups.Results:Among the 48 IO-IBD patients, 41 cases suffered from Crohn′s disease (CD) and 7 cases were ulcerative colitis (UC). The median age of onset was 2.00(0.24-6.00) months, and 45.8%(22/48 cases) of patients′ onset age was within 1 month.Diarrhea (48/48 cases, 100%), fever (45/48 cases, 93.8%) and hematochezia (37/48 cases, 77.1%) were the main clinical symptoms.Perianal lesions and rashes were common extraintestinal manifestations, accounting for 43.8%(21/48 cases) each.Among 41 CD patients, 73.2%(30/41 cases) had predominantly colonic disease (L2 type), and disease behavior was mainly nonstricturing and nonpenetrating (B1 type) (33/41 cases, 80.5%). Among 7 UC patients, 57.1%(4/7 cases) had predominantly pancolonic (E4 type). The surgical rate of the 48 IO-IBD patients was 12.5%(6/48 cases), the clinical remission rate was 50.0%(24/48 cases), and the mortality rate was 25.0%(12/48 cases). Among the 43 IO-IBD patients, 23 (23/43 cases, 53.5%) had meaningful gene mutations, of which 22 cases had mutations in interleukin-10 receptor A ( IL-10 RA) and 1 case with mutation in TTC37.A total of 11 mutation sites were detected in 22 patients with IL-10 RA mutations, including one novel mutation site c. 635G>C (p.R212P); 19 cases c. 301C>T (p.R101W) and 8 cases c. 537G>A (p.T179T) that were common mutation sites.Compared with non-gene mutation IO-IBD group, patients in gene mutation IO-IBD group had earlier onset age [0.3 (0.1-1.0) months vs.(6.27±5.64) months, P<0.001], higher proportion of malnutrition [14 cases(60.9%) vs.5 cases(25.0%), P=0.018], oral ulcers [14 cases(60.9%) vs. 30 cases(15.0%), P=0.006] and perianal lesions [17 cases(73.9%) vs.3 cases(15.0%), P<0.001], and the lower rate of clinical remission[7 cases(30.4%) vs.15 cases(75.0%), P=0.004]. Conclusions:IO-IBD patients had a high rate of monogenic mutation, and IL-10 RA gene mutation was the common mutation.IO-IBD patients developing with gene mutation were characterized by early age of onset, higher incidence of malnutrition, oral ulcers, perianal lesions, and the lower clinical remission rate.
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From November, 2010 to February, 2018, 11 children with variants of Guillain-Barré syndrome (GBS) were treated in Xiangya Hospital of Central South University. Clinical manifestations included cranial nerve involvement in 9 cases, limb weakness in 9 cases, ataxia in 8 cases, abnormal sensation in 7 cases, weakened or disappeared tendon reflex in all cases, albuminocytologic dissociation of cerebrospinal fluid in 6 cases, and neurogenic changes in electromyography in 8 cases. Immune globulin and steroids were given to 5 cases and 1 case, respectively, while the combination of immune globulin with steroids was given to 1 case, and symptomatic treatment was given to 4 cases. All treatments were effective, remaining no apparent side effect for nervous system. The diagnosis of GBS variants was based on clinical manifestation. Patients who had cranial nerve involvement, ataxia and weakened or disappeared tendon reflex should be paid attention. Albuminocytologic dissociation of cerebrospinal fluid and electromyography were helpful to diagnosis. GBS variants were self-limited disease. Immunotherapy can ease the patients' critical condition.
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Child , Humans , Electromyography , Guillain-Barre Syndrome , Immunoglobulins, Intravenous , ImmunotherapyABSTRACT
Objective@#To investigate the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES).@*Methods@#The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed. The basic information, clinical manifestations, electroencephalogram, imaging examination, treatment and prognosis were analyzed.@*Results@#Of the 12 patients, 7 were male and 5 were female. The age of onset was (7.0±3.7)years (1.3 year to 13 years). The average hospitalization time (34-86 days, median 52 days). Twelve patients were healthy before the disease, and had fever before convulsion. The interval between fever and seizure was (3.5±1.7)days (1-7 days). The status epilepticus and consciousness deficit were the main clinical manifestations. The electrogram of 8 patients showed status epilepticus when admitted. 12 patients had disturbance of consciousness; the acute episodes were focal seizures (100%, 12/12) and generalized tonic-clonic seizures (41.7%, 5/12). All patients used 3-5 antiepileptic drugs (median 4), all treated with hormones and gamma globulin. 4 patients with ketogenic diet (KD) were treated within 2 weeks of onset, and the average duration from onset to electroencephalogram (EEG) improvement was (19.2±5.0)days. In 8 patients who did not use KD within 2 weeks of onset, the average duration from onset to EEG improvement was (29.9±9.6)days.@*Conclusions@#FIRES is more common in normal children with school age. The main manifestation is refractory status epilepticus in the days after acute fever, focal episodes of seizures, anti-epileptic drug resistance. Early initiation of KD produces a favorable prognosis.
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Objective@#To explore the clinical features and genetic characteristics of myoclonic-atonic epilepsy (MAE) caused by SLC6A1 gene mutation.@*Methods@#The clinical data of a patient with SLC6A1 gene mutation from Xiangya Hospital of Central South University was collected. The related literatures were reviewed from Wanfang Data, China National Knowledge Infrastructure, PubMed (until July 2019) by using keywords " SLC6A1" and " myoclonic-atonic epilepsy" . The characteristics of SLC6A1 gene mutation and the clinical phenotype of children with myoclonic-atonic epilepsy were summarized.@*Results@#A 8 year and 8 months old girl was enrolled in the study. Her first attack happened at the age of 19 months, and multiple seizure types including myoclonic-atonic, atonic and absence were observed. The seizures were well controlled by valproate (VPA), but she has mild-moderate intellectual disability. Whole exome-sequencing study identified a de novo nonsense variant of c. 46G>T(p.Glu16*)in SLC6A1 gene. A total of 27 cases including the present case with SLC6A1 gene mutation were analyzed. 22 mutations were identified, including 11 missense mutations, 5 nonsense mutations, 3 frameshift mutations, 2 splicing mutation and 1 with chromosome microdeletion. Among them 26 patients had more one type of seizures, 20 cases had absence seizures, 17 cases had atonic seizures, 14 cases had myoclonic seizures, 11 cases had myoclonic-atonic seizures, 4 cases had generalized tonic-clonic seizures, 3 cases had eyelid myoclonias, 2 cases had nonconvulsive status epilepticus and 2 cases had tonic seizure. 24 patients had described intelligence assessment. Among them, 18 had developmental delay before epilepsy onset, 11 had developmental regression after onset. There were 9 cases with autistic features, 4 cases with attention deficit hyperactivity disorder and 3 cases with ataxia. The seizures of 17 cases were controlled, 4 cases had partial seizure control, 3 cases had no significant improvement, and other 3 cases were unclear.@*Conclusions@#The main clinical feature of MAE patients with SLC6A1 gene mutations is absence and atonic seizures, cognition before epilepsy onset can be impaired, and some patients had behavioral problems, such as autistic features or attention deficit hyperactivity disorders. VPA is recommend as first-line treatment.
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Objective To investigate the clinical features,diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES).Methods The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed.The basic information,clinical manifestations,electroencephalogram,imaging examination,treatment and prognosis were analyzed.Results Of the 12 patients,7 were male and 5 were female.The age of onset was (7.0 ± 3.7)years (1.3 year to 13 years).The average hospitalization time (34-86 days,median 52 days).Twelve patients were healthy before the disease,and had fever before convulsion.The interval between fever and seizure was (3.5 ± 1.7) days (1-7 days).The status epilepticus and consciousness deficit were the main clinical manifestations.The electrogram of 8 patients showed status epilepticus when admitted.12 patients had disturbance of consciousness;the acute episodes were focal seizures (100％,12/12) and generalized tonic-clonic seizures (41.7％,5/12).All patients used 3-5 antiepileptic drugs (median 4),all treated with hormones and gamma globulin.4 patients with ketogenic diet (KD) were treated within 2 weeks of onset,and the average duration from onset to electroencephalogram (EEG) improvement was (19.2 ± 5.0)days.In 8 patients who did not use KD within 2 weeks of onset,the average duration from onset to EEG improvement was (29.9 ± 9.6) days.Conclusions FIRES is more common in normal children with school age.The main manifestation is refractory status epilepticus in the days after acute fever,focal episodes of seizures,anti-epileptic drug resistance.Early initiation of KD produces a favorable prognosis.
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Objective To explore the clinical features and genetic characteristics of myoclonic-atonic epilepsy (MAE) caused by SLC6A1 gene mutation.Methods The clinical data of a patient with SLC6A1 gene mutation from Xiangya Hospital of Central South University was collected.The related literatures were reviewed from Wanfang Data,China National Knowledge Infrastructure,PubMed (until July 2019) by using keywords "SLC6A1" and "myoclonic-atonic epilepsy".The characteristics of SLC6A1 gene mutation and the clinical phenotype of children with myoclonic-atonic epilepsy were summarized.Results A 8 year and 8 months old girl was enrolled in the study.Her first attack happened at the age of 19 months,and multiple seizure types including myoclonic-atonic,atonic and absence were observed.The seizures were well controlled by valproate (VPA),but she has mild-moderate intellectual disability.Whole exome-sequencing study identified a de novo nonsense variant of c.46G ＞ T(p.Glu16 *)in SLC6A1 gene.A total of 27 cases including the present case with SLC6A1 gene mutation were analyzed.22 mutations were identified,including 11 missense mutations,5 nonsense mutations,3 frameshift mutations,2 splicing mutation and 1 with chromosome microdeletion.Among them 26 patients had more one type of seizures,20 cases had absence seizures,17 cases had atonic seizures,14 cases had myoclonic seizures,11 cases had myoclonic-atonic seizures,4 cases had generalized tonic-clonic seizures,3 cases had eyelid myoclonias,2 cases had nonconvulsive status epilepticus and 2 cases had tonic seizure.24 patients had described intelligence assessment.Among them,18 had developmental delay before epilepsy onset,11 had developmental regression after onset.There were 9 cases with autistic features,4 cases with attention deficit hyperactivity disorder and 3 cases with ataxia.The seizures of 17 cases were controlled,4 cases had partial seizure control,3 cases had no significant improvement,and other 3 cases were unclear.Conclusions The main clinical feature of MAE patients with SLC6A1 gene mutations is absence and atonic seizures,cognition before epilepsy onset can be impaired,and some patients had behavioral problems,such as autistic features or attention deficit hyperactivity disorders.VPA is recommend as first-line treatment.
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Objective To summarize the clinical data of the children with inflammatory bowel diseases (IBD),including Crohn's disease (CD) and ulcerative colitis (UC),and to analyze and compare the clinical features of very early-onset IBD (VEO-IBD) and late-onset IBD (LO-IBD).Methods A retrospective analysis of the clinical data of 184 cases of IBD hospitalized children diagnosed at Beijing Children's Hospital,Capital Medical University from January 2000 to December 2014.According to their ages of onset,the patients were divided into VEO-IBD group (＜6 years old) and LO-IBD group (6-16 years old);the patients with CD were divided into VEO-CD group (＜ 6 years old) and LO-CD group (6-16 years old);UC were divided into VEO-UC group (＜ 6 years old) and LO-UC group (6-16 years old).The clinical features among each group were analyzed and compared.Results A total of 184 IBD patients were included in the study,77 cases(41.8％) were VEO-IBD and 107 cases(58.2％) were LO-IBD.Comparison between VEO-CD group and LO-CD group indicated that abdominal pain was more common in LO-CD group (P ＜ 0.05),while diarrhea and hematochezia were more common in VEO-CD group (all P ＜ 0.05).In addition,comparison between VEO-UC group and LO-UC group indicated that abdominal pain was more common in LO-UC group(P ＜ 0.001),while diarrhea,fever,and oral ulcers were more common in VEO-UC group (all P ＜0.05).Both VEO-CD and LO-CD group were mainly ileocolonic[15/27 cases (55.6％),20/47 cases (42.6％)],non-narrow,non-penetrating [20/27 cases (74.1％),30/47 cases (63.8％)] and moderate-to-severe activity[23/27 cases(85.2％),37/47 cases (78.7％)].The incidence of perianal lesions in the VEO-CD group was as high as 51.9％ (14/27 cases),which was significantly higher than that in the LO-CD group (9/47 cases,19.1％) (P ＜ 0.05).Left-sided UC and severe UC were more common in VEO-UC group(all P ＜ 0.05),while pancolitis and mild UC were more common in LO-UC group (all P ＜ 0.05).The incidence of intestinal perforation in the VEO-UC group was significantly higher than that in the LO-UC group (P ＜0.05).The incidence of surgical rate,intestinal obstruction,and intestinal perforation in the LO-CD group were significantly higher than those in the LO-UC group (all P ＜ 0.05).Conclusions Compared between VEO-IBD and LO-IBD,VEO-IBD patients are more severe,with perianal lesions more common,and the incidence of intestinal perforation is higher.
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Objective@#To investigate the clinical features, treatment strategies and long term outcomes of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.@*Methods@#The data of clinical features, auxiliary examinations, treatments and prognosis in children with anti-NMDAR encephalitis in Xiangya Hospital of Central South University from March 2014 to October 2017 were collected and retrospectively analyzed. A total of 71 patients were enrolled, including 33 males and 38 females. The youngest age of onset was 4 months old, and the age of onset was (9±4) years. The first-line immunotherapy treatment for anti-NMDAR encephalitis was short course corticosteroid (high-dose impulse therapy and oral maintenance therapy for 1 month in acute period) and (or) immunoglobulin. The clinical evaluation was performed 2 weeks after first-line immunotherapy treatment. The second-line immunotherapy treatment, including rituximab and (or) cyclophosphamide, would be started if the symptoms did not improve significantly and the modified Rankin scale (mRS) score ≥3. All patients were followed up and evaluated for prognosis. T-test, Mann-Whitney U, Chi square test and Fisher′s exact probability method were used for comparison between good outcome group and poor outcome group, first-line immunotherapy group and first-line immunotherapy combined with second-line immunotherapy group.@*Results@#The more common clinical manifestations were psychiatric symptoms (n=61, 86%), dyskinesia (n=55, 77%) and convulsions (n=51, 72%). Two cases (3%) had tumors. Electroencephalogram (EEG), cerebro-spinal fluid (CSF) and brain magnetic resonance imaging (MRI) studies were abnormal in 83% (59/71), 39% (27/69) and 38% (27/71) patients, respectively. For the treatment regimens, all the 71 patients underwent first-line immunotherapy, resulting in improvement within 14 days in 40 cases (56%), and 1 case (1%) died. The rest 30 cases (42%) received second-line immunotherapy. The patients were followed up for 5.0-41.8 months, with a median of 19.3 months. At the last follow-up, 49 cases (69%) recovered completely, 15 cases (21%) had mild disability, 6 cases (8%) had severe disability, 1 case (1%) died and 3 cases (4%) had relapse. There were significant differences between the groups with good prognosis and poor prognosis on admission to pediatric intensive care unit (PICU) and consciousness disorder (10/64 vs. 5/7, 39/64 vs. 7/7, P=0.047, 0.004). There were significant differences between first-line immunotherapy group and the first-line combined second-line immunotherapy group on admission to PICU, consciousness disorder, sleep disorder and first mRS score (12% (5/41) vs. 33% (10/30), 44% (18/41) vs. 93% (28/30), 56% (23/41) vs. 90% (27/30), 3 (1-5) vs. 4 (3-5), respectively; χ2=4.645, 18.555, 9.560, Z=5.184, P=0.031, <0.01, 0.002, <0.01, respectively).@*Conclusions@#Anti-NMDAR encephalitis can occur in all ages of children. The most common clinical manifestations are psychotic symptoms, dyskinesia and convulsions. Paraneoplastic cases are less common in children. Immunotherapy is effective. The second-line immunotherapy should be given after the failure of first-line therapy (mRS score≥3).
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Objective@#To investigate the antibiotic resistance of Helicobacter pylori(Hp) isolates cultured from endoscopic gastric mucosal samples and influencing factors for antibiotic resistance in children.@*Methods@#From April 2013 to February 2016, Hp cultured from mucosa samples of the gastric antrum and the body of stomach was investigated in 246 patients with 13C breath test positive examined by gastroscopy.Resistance to Amoxicillin, Clarithromycin, Metronidazole, Tetracycline was tested for Hp by using E-test.The clinical data were collected from the patients, and the relationship among age, gender, endoscopic diagnosis, histological performance, eradication number factors and antibiotic resistance rate were analyzed.@*Results@#Of 246 specimens, 174 cases (70.7%) were positive.The overall antibiotic resistance rates of isolates obtained were 96.55% (168/174 cases), 57.47% (100/174 cases), 4.02% (7/174 cases), 1.15%(2/174 cases) with respect to Clarithromycin, Metronidazole, Tetracycline and Amoxicillin.An antibiotic resistance rate was 43.10% (75/174 strains), among which the Clarithromycin resistance rate was 93.33% (70/75 strains), and the Metronidazole resistance rate was 6.67% (5/75 strains). Double resistance rate was of 54.02%(94/174 strains), among which the resistances rate of Clarithromycin+ Metronidazole was 52.30%(91/94 strains), and the resistances rate of Clarithromycin+ Tetracycline was 1.72%(3/94 strains). Triple resistance rate of Clarithromycin+ Metronidazole+ Tetracycline was 1.15%(2/174 strains). Quadruple resistance rate of Clarithromycin+ Metronidazole+ Amoxicillin+ Tetracycline was 1.15%(2/174 strains). Factor analysis showed that the resistance rate of Clarithromycin in children who had failed in eradication therapy [98.7%(148/150 cases)] was higher than that in children who had not undergone eradication therapy [83.3%(20/24 cases)], and the difference was statistically significant(χ2=14.610, P<0.05). There was no significant difference in the relationship between Hp resistance to Metronidazole and Clarithromycin and the age, gender, endoscopic diagnosis and histological manifestations(all P>0.05).@*Conclusions@#Hp resistance rate to Clarithromycin, Metronidazole is very high in children, but it is relatively low to Amoxicillin and Tetracycline, and multiple antibiotic resistance is at high prevalence.
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Objective To investigate the diagnostic analysis and pathogenic characteristics of bacterial meningitis (BM) in children in Midwest area in China.Methods Clinical and pathogenic data of inpatients with BM were analyzed retrospectively at Xiangya Hospital,Central South University and Children's Hospital of Chongqing Medical University,including age,gender,index of peripheral inflammation,cerebrospinal fluid routine,biochemistry,culture,drug sensitivity and image change.Results In total,446 cases with BM were analyzed in this study.The male-to-female ratios was 1.67:1.00.Peripheral blood detected abnormal ratios of white blood cell (WBC),C reactive protein(CRP)and procalcitonin (PCT) which were 67.7％ (302/446 cases),64.9％ (268/413 cases) and 71.7％ (279/389 cases),respectively.Cerebrospinal fluid detected abnormal ratios of WBC count,glucose and protein,which were 76.5％ (332/434 cases),71.2％ (306/430 cases) and 91.7 ％ (397/433 cases),respectively;79.4％ of the patients revealed abnormal head CT/MRI image.Bacterial culture positive rate was 33.6％ (150/446 cases),and the positive rates of abnormality in peripheral blood WBC,CRP,PCT or cerebrospinal fluid WBC,glucose and protein were 34.9％ (116/332 cases),34.8％ (138/397 cases),36.6％ (112/306 cases),respectively,which were higher than the normal patients,who were 29.4％ (30/102 cases),25.0％ (9/36 cases),21％ (26/124 cases),respectively.The most common pathogenic bacteria were Streptococcus pneumoniae,Escherichia coli and coagulase negative staphylococcus.Infants within one year presented dominantly with infection of Escherichia coli,accounting for 26.6％ (29/109 cases).Children above 1-year-old presented dominantly with Streptococcus pneumoniae,accounting for 61.0％ (25/41 cases).A drug-resistance phenomenon was common among pathogenic bacteria.Conclusion Abnormality in peripheral blood WBC,CRP,PCT or cerebrospinal fluid WBC,glucose and protein,and subdural effusion/empyema can help the early diagnosis and differential diagnosis of BM.The most common pathogenic bacteria in Midwest area of China are Streptococcus pneumoniae,Escherichia coli and coagulase negative staphylococcus,which suggests recommend the application of Streptococcus pneumoniae vaccine.Drug-resistance phenomenon to pathogens is very common.For the treatment strategy,it should be made and adjusted timely according to the drug sensitivity and common hospital antimicrobial spectrum.
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Objective Explore the sodium citrate anticoagulation in the continuity of plasma adsorption closed-circuit circulation of anticoagulation therapy method and effect. Methods Line into the continuity of plasma adsorption treatment of 156 cases of acute drug poisoning patients were randomly(random number) divided into two groups, 78 cases in each group, respectively adopt low molecular heparin (group A), sodium citrate anticoagulation (group B). Contrast analysis of two groups after the therapy began 30 min, 3 h, 6 h before the filter in patients with pressure, transmembrane pressure, pressure drop, at the same time to compare two groups of 10 min before the start of treatment, after treatment began to 3 h, 6 h platelet, coagulation time live enzymes, vein in the body of free Ca2+, Na+and HCO3- 24 h and internal bleeding. Results Two groups in gender, age, clinical diagnosis, blood purification time comparative differences had no statistical significance (P>0.05);Two groups of 30 min after the start of treatment, 3 h, 6 h patients before pressure, transmembrane pressure, filter pressure drop compared differences were no statistical significance (P>0.05); Part of coagulation treatment after low molecular heparin group live enzymes the sodium citrate group significantly prolonged (P<0.01);Platelets, HCO3- the two groups after treatment, intravenous free Ca2+ and Na+ differences had no statistical significance (P>0.05). Conclusions In the continuous plasma adsorption treatment process using sodium citrate anticoagulation with clinical feasibility, safety.
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Objective To summarize the clinical features of global developmental delay (GDD) children,and to explore the relationship between severity of GDD and social-culture factors.Methods Sign the informed consent before enrollment.Collect clinical data in detail about 100 GDD children (GDD group) and 95 children with normal development (healthy control group),and analyze their regular clinical data,physical examination,intellectual disability test,electroencephalography (EEG) and cranial imaging test.Spearman rank correlation was used to analyze the differences of social and cultural factors between GDD group and healthy control group,such as maternal reproductive age,parental education level and family economic status.At the same time,we compared the lag degree of total developmental quotient and the degree of developmental retardation of five energy areas with the above factors.Results Significant associations were found between GDD and maternal/paternal education,economic level of family,but no sgnificant association was found between maternal age and GDD.And analysis in the relationship between severity of delay in all domains of the child's developmental status about language and social-culture factors,we only obtained the severity of delay in abilities about language is related with maternal education.Spearman rank correlation statistics explains that if there are the lower level of education with mothers,the delay of language domain will more severe (rs =-0.505,P ＜ 0.05).Conclusions Significant associations were found between GDD and maternal/paternal education,economic level of family.The higher maternal education was an important protective factor against risk of GDD.Improving the cognition of parents in child health care,early be diagnosed,early be intervened,is the most important for children with language development.
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Objective To investigate the correlation between catheter indwelling time and catheter-related bloodstream infection in patients with ICU. Methods Chosen 60 cases of peripheral arterial catheterization and indwelling arterial catheters because of illness need more than 7 days of age, from September 2015 to January 2016 who were treated in respiratory and critical care medicine. Numbered in the order of entry (1-60), andnumber of singular werein the control group and number of even were in the experimental group. In the control group, the catheter was removed and the catheter was replaced at 7 days after the catheter was inserted,the experimental group continued to use the catheter for 7 days until the patient did not need to remove the catheter. Microbial culture was carried out in two groups of catheter tip,The bacterial setting rate of catheter tip culture was compared between the two groups. Results The control group of bacterial colonization rate was 7.14％ (2/28), the experimental group of bacterial colonization rate was 10.34％ (3/29), the difference between the two groups was not statistically significant (χ2=0.000, P>0.05). Conclusions Nursing care of patients with indwelling arterial catheters just follow the principle of maximum aseptic artery puncture and disinfection treatment, and patients without clinical symptoms need not to prevent catheter-related infection by peripheral arterial catheter replacement.