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Objective:To explore the clinical charecteristics, imaging features, therapy and prognosis of stroke in children, and provide help for clinical treatment.Methods:The clinical data of 49 children with stroke were collectedand retrospectively analyzed in the Children′s Hospital of Soochow University from January 1, 2019 to December 31, 2019.Results:A mong the 49 children with stroke, 35 were male and 14 were female, aged 1-178 (65.69 ± 55.22) months; the specific etiologies were cerebrovascular malformation, craniocerebral trauma, tumor, vitamin K deficiencies, infectious diseases, rheumatic immune diseases, hemophilia and congenital heart disease. The first symptoms of stroke were disturbance of consciousness, hemiplegia, convulsions, vomiting and headache. The arterial ischemic stroke (18 cases) were mainly caused by craniocerebral trauma and cerebrovascular malformation. The hemorrhagic stroke (31 cases) were mainly caused by arteriovenous malformation, vitamin K deficiency and tumor. The surgical rate in the arterial stroke group was significantly lower than that in the hemorrhagic stroke group.Conclusions:Traumatic cerebral infarction and intracranial arteriovenous malformation are the main causes of arterial ischemic stroke and hemorrhagic stroke in children. Early diagnosis and treatment can significantly improve prognosis.
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Clinical phenotype and gene characteristics of a patient diagnosed with Imerslund-Gr?sbeck syndrome (IGS) in Department of Neurology, Children′s Hospital of Soochow University in December 2018 were analyzed retrospectively, and literature review was conducted.The 16 years and 5 months old boy was admitted to the hospital with symptoms of weakness of lower limbs for 2 weeks.He had a history of megaloblastic anemia and isolated proteinuria.Genetic metabolism of hematuria showed methylmalonic academia.Genetic analysis revealed a compound heterozygous AMN gene mutation[c.742C>T(p.Q248 *) and c. 761G>A(p.G254E)]. These two mutations were derived from his parents respectively, which had been reported before.Symptoms of the patient improved after intramuscular administration of hydroxycobalamin and oral betaine.Review of the literature indicated that the clinical manifestations of AMN gene-related IGS were mostly megaloblastic anemia and isolated proteinuria, and the older children might suffer from neurological symptoms such as movement disorders, dementia, delirium or psychosis.The clinical phenotype of this disease was variable, which might easily lead to misdiagnosis.The patient presented with a special phenotype of mild reversible peripheral neuropathy, which expanded the clinical phenotype of pathogenic genes of AMN gene.In addition, peripheral neuropathy caused by vitamin B 12 metabolic disorders is reversible, and it is suggested to measure vitamin B 12, test related genes and treatment with vitamin B 12 in peripheral neuropathy of unknown etiology.
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Objective:To analyze the associations between attention deficit hyperactivity disorder (ADHD) and serum vitamin B levels in children.Methods:A total of 103 ADHD children who were diagnosed in the Department of Child and Adolescent Healthcare of Children′s Hospital of Soochow University from September 2018 to April 2019 were selected as the ADHD group, and 89 children of the same age who underwent routine physical examinations served as the healthy control group.The serum levels of vitamin B, including vitamin B 1, vitamin B 2, vitamin B 6, vitamin B 9, and vitamin B 12, were measured by the methods of electrochemistry.Weiss Functional Impairment Rating Scale-Parent Report (WFIRS-P) was applied to analyze the correlation between social function scores and vitamin B levels in ADHD children. Results:The levels of vitamin B 9[(12.55±2.22) nmol/L vs.(13.26±2.54) nmol/L] and vitamin B 12 [(278.54±32.00) ng/L vs.(288.90±31.32) ng/L] in ADHD children were significantly lower than those in healthy children( t=-2.064, -2.261, all P<0.05). No significant difference was observed in serum levels of vitamin B 1, vitamin B 2 and vitamin B 6 between the 2 groups (all P>0.05). Correlation analysis displayed that only vitamin B 12 level was significantly and negatively correlated with social function in the learning/school dimension ( r=-0.208, P=0.035), and no significant correlation was found between other vitamin B levels and social function (all P>0.05). Conclusions:The serum levels of vitamin B 9 and B 12 in ADHD children were obviously lower than those in healthy children of the same age.Serum vitamin B 12 deficiency had an influence on the social function of the learning/school in ADHD children.Attention to the monitoring and timely supplementation of vitamin B in childhood, especially vitamin B 9 and B 12, may be of positive significance in the prevention of the occurrence and development of ADHD to some extent.
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A retrospective analysis was performed on the clinical data of a case of late infantile neuronal ceroid lipofuscinosis admitted in the Department of Neurology, Children′s Hospital Affiliated to Soochow University in 2017.The patient was a 4-year-old girl, who was hospitalized because of " 2 convulsions in 1 day" at 2 years and 10 months old.Since then, she had convulsions and progressive regression of motor, language and intelligence frequently.The clinical feature of the patient included unstable walking, drunkenness, easy falling, shaking hands with objects, hypophrasia, prostation, lack of eye language communication, inability to take care of herself, and intelligence quotient of 1-year-old child.Brain magnetic resonance imaging(MRI) showed cerebellar atrophy; the electroencephalogram suggested spike slow wave and multi-spike slow wave burst with high amplitude.Gene detection: 2 heterozygous variants of TPP1 gene, namely c. 230-1G>C and c. 1027G>A(p.e343k), and they were " possibly pathogenic" . Patients with intractable epilepsy, ataxia, motor language retardation, and cerebellar atrophy on MRI should undergo gene detection should in time, so as to make clear diagnosis and guide prenatal diagnosis and genetic consultation as early as possible.
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Objective@#To explore the factors affecting the prognosis of children with status epilepticus(SE).@*Methods@#A retrospective review was performed on children and the outcomes were measured by Glasgow Outcome Score (GOS). GOS=5 was defined as a good outcome, and GOS<5 as a bad outcome.@*Results@#(1)Two hundred and ninety-eight children (163 girls and 135 boys) with SE were enrolled.The ages of the patients ranged from 2 months and 7 days to 14 years and 5 months, and 106 cases were at 1-3 years old.There were 154 cases (51.68%) with previous history of convulsion, including 98 cases of epilepsy (63.63%) and 241 new onset SE (80.87%). The most common cause of SE in children was febrile seizure (92 cases, accounting for 30.87%), followed by long-term symptom (81 cases, accounting for 27.18%). A total of 109 cases (41.90%) were treated with Diazepam in time after the convulsion, and 151 cases (58.10%) were treated with antiepileptic drugs such as Phenobarbital sodium or Chloral hydrate after the convulsion.Based on GOS scale, the near-term prognosis was as follows: 254 cases(85.23%) of children with GOS=5, and the remaining 44 cases of GOS<5 points.(2) Recent overall prognostic factors: single factor analysis showed that a total of 12 factors were associated with poor prognosis (P<0.05), including age, Pediatric Intensive Care Unit (PICU), tracheal intubation, previous history of convulsions, developmental delay, new onset of SE, fever, comprehensive onset, refractory SE, electroencephalogram (EEG) abnormality, neuroimaging abnormality and etiology.Multiple factor Logistic regression analysis showed that PICU monitoring was required (OR=4.306, 95%CI: 1.554-11.933, P=0.005), developmental delay (OR=3.054, 95%CI: 1.157-8.066, P=0.024), and abnormal cranial image (OR=2.110, 95%CI: 1.121-3.970, P=0.021) was an independent risk factors for short-term poor prognosis.Comprehensive onset (OR=0.330, 95%CI: 0.139-0.782; P=0.012), and the cause of febrile convulsion (OR=0.688, 95%CI: 0.480-0.987, P=0.042) were prognostic non-risk factor.(3)Recent prognostic factors in PICU monitoring group: the hospitalization time of PICU, developmental delay, the atypical attack type, electroencephalogram abnormality, neuroimaging abnormality, and the etiology of these 6 factors were correlated with the prognosis of PICU group, among which prolonged PICU (OR=36.912, 95%CI: 5.165-263.787, P=0.000) and developmental delay (OR=14.403, 95%CI: 1.725-120.273, P=0.014) were independent risk factors for poor outcome of PICU monitoring group.@*Conclusions@#PICU monitoring, developmental backwardness and abnormal head image are the independent risk factors for poor prognosis.PICU residence time and developmental delay are the independent risk factors for poor short-term prognosis in PICU enrolled children.
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Objective To explore the factors affecting the prognosis of children with status epilepticu(s SE). Methods A retrospective review was performed on children and the outcomes were measured by Glasgow Outcome Score(GOS). GOS=5 was defined as a good outcome,and GOS<5 as a bad outcome. Results (1)Two hundred and ninety-eight children(163 girls and 135 boys)with SE were enrolled. The ages of the patients ranged from 2 months and 7 days to 14 years and 5 months,and 106 cases were at 1-3 years old. There were 154 cases(51. 68% )with pre-vious history of convulsion,including 98 cases of epilepsy(63. 63% )and 241 new onset SE(80. 87% ). The most common cause of SE in children was febrile seizure(92 cases,accounting for 30. 87% ),followed by long-term symp-tom(81 cases,accounting for 27. 18% ). A total of 109 cases(41. 90% )were treated with Diazepam in time after the convulsion,and 151 cases(58. 10% )were treated with antiepileptic drugs such as Phenobarbital sodium or Chloral hy-drate after the convulsion. Based on GOS scale,the near-term prognosis was as follows:254 cases(85. 23% )of chil-dren with GOS=5,and the remaining 44 cases of GOS<5 points.(2)Recent overall prognostic factors:single factor analysis showed that a total of 12 factors were associated with poor prognosis(P<0. 05),including age,Pediatric In-tensive Care Unit(PICU),tracheal intubation,previous history of convulsions,developmental delay,new onset of SE, fever,comprehensive onset,refractory SE,electroencephalogram( EEG)abnormality,neuroimaging abnormality and etiology. Multiple factor Logistic regression analysis showed that PICU monitoring was required( OR=4. 306,95% CI:1. 554-11. 933,P=0. 005),developmental delay(OR=3. 054,95% CI:1. 157-8. 066,P=0. 024),and abnormal cranial image(OR=2. 110,95% CI:1. 121-3. 970,P=0. 021)was an independent risk factors for short-term poor prognosis. Comprehensive onset(OR=0. 330,95% CI:0. 139-0. 782;P=0. 012),and the cause of febrile convulsion (OR=0. 688,95% CI:0. 480-0. 987,P=0. 042)were prognostic non-risk factor.(3)Recent prognostic factors in PICU monitoring group:the hospitalization time of PICU,developmental delay,the atypical attack type,electroencepha-logram abnormality,neuroimaging abnormality,and the etiology of these 6 factors were correlated with the prognosis of PICU group,among which prolonged PICU(OR=36. 912,95% CI:5. 165-263. 787,P=0. 000)and developmental delay(OR=14. 403,95% CI:1. 725-120. 273,P=0. 014)were independent risk factors for poor outcome of PICU monitoring group. Conclusions PICU monitoring,developmental backwardness and abnormal head image are the inde-pendent risk factors for poor prognosis. PICU residence time and developmental delay are the independent risk factors for poor short-term prognosis in PICU enrolled children.
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Objective To explore the risk factors for childhood death from pneumococcal meningitis.Methods The data of 32 hospitalized children were retrospectively analyzed,who were diagnosed as pneumococcal meningitis and enrolled in the Affiliated Children′s Hospital of Soochow University from November 201 0 to December 201 5.The subjects were divided into the death group and survival group according to their prognosis.The clinical characteristics and laboratory data were compared between 2 groups.Results Between the death group and survival group,there were significant statistically differences in shock within 24 hours after admission(63.6% vs 1 4.3%,P =0.01 3),as well as endotracheal tube intubation(1 00.0% vs 23.8%,P <0.001 ),the levels of cerebrospinal fluid(CSF)IgG[(491 .27 ± 203.53)mg/L vs (267.24 ±1 88.07)mg/L,P =0.006],IgM[(1 1 5.72 ±79.1 9)mg/L vs (32.80 ±28.52)mg/L, P =0.006],IgA[59.52(1 5.51 ,75.69)mg/L vs 1 8.77(9.33,27.54)mg/L,P =0.023],CSF leukocyte[330.00 (1 50.00,380.00)×1 06 /L vs 870.00 (403.00,6 1 60.00)×1 06 /L,P =0.009 ],CSF protein [(4 047.00 ± 1 942.1 6)mg/L vs (2 470.62 ±1 259.94)mg/L,P =0.009],CSF adenosine deaminase (ADA)[35.20(1 8.90, 87.20)U /L vs 8.80(3.05,23.78)U /L,P =0.001 ],serum sodium[(1 30.21 ±2.85)mmol/L vs (1 32.83 ±3.69) mmol/L,P =0.049],serum lactic acid (LA)[4.40 (2.60,5.70)mmol/L vs 2.40 (1 .75,4.50)mmol/L,P =0.01 3],serum C -reactive protein (CRP)[(95.87 ±65.40)mg/L vs (1 65.61 ±83.05)mg/L,P =0.022],serum lactate dehydrogenase (LDH)[81 3.40(465.20,2 31 0.70)U /L vs 359.20(257.85,405.90)U /L,P =0.001 ], platelet[(1 63.82 ±1 64.86)×1 09 /L vs (295.71 ±1 30.29)×1 09 /L,P =0.01 9]and positive rate of blood culture (90.9% vs 47.6%,P =0.023)between the death group and survival group.Conclusions The risk factors associated with mortality in pediatric SPM include shock within 24 hours after admission,endotracheal intubation,hyponatremia, thrombocytopenia,as well as high serum LA level,high serum LDH level,lower serum CRP level or cultures of blood and CSF double positive.
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ObjectiveTo investigate the clinical features and prognosis of children with rotavirus gastroenteritis and convulsion.MethodsClinical data of children with rotavirus gastroenteritis hospitalized from January 2010 to December 2013 were retrospectively analyzed. Subjects were divided into the seizure group and no seizure group according to the presence of seizure in the course and compared between the two groups.ResultsThere were no signiifcant differences in sex, age, and the average duration of hospitalization between two groups (allP>0.05). The family history, history of seizures, the levels of serum sodium, calcium, lactate, standard bicarbonate concentration (SB), actual bicarbonate concentration (AB), carbon dioxide content (TCO2) and pH were statistically signiifcant between two groups (allP>0.05). During the follow-up period (outpatient telephone follow-up), the recurrence of seizure in two groups was signiifcant different (P<0.05) and only one (0.54%) child in seizure group developed epilepsy.ConclusionThis study showed that rotavirus gastroenteritis with convulsion is a benign clinical course.
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Objective To investigate the effect of B7 Homology 3(B7H3)on brain damage of S .Pneumococcal(SP)meningitis . Methods SP meningitis was established by intracerebral ventricular injection of SP suspension on wild-type BALB/C mice .48 mice were divided into 4 groups and received following injections :NS(CON group) ,recombinant murine B7H3alone(B7H3 group) ,SP group ,SP+B7H3 group .At 18 ,48 ,72 h post infection ,mice were conducted neurobehavior score ,then they were anesthetized and killed by cervical vertebra dislocation ,brains were collected .The mRNA expressions of NSE and S100b were detected by real-time PCR .Results Compared with CON group ,the scores of recombinant murine B7H3 group had no significant change at 18 ,48 ,72 h after infection of SP(P>0 .05);at different time points the scores of SP group were decreased significantly than the CON group (P0 .05) .At 18h ,48h ,72h ,post infection mRNA expressions of NSE ,S100b in SP group increased compared with CON group(P<0 .05);the mRNA expressions of NSE ,S100b increased furtherly in SP+B7H3 group compared with SP group(P<0 .05) .Conclusion B7H3 upregulates the mRNA expressions of NSE and S100b ,and promotes the progress of SP meningitis in mice .