Benign posttraumatic encephalopathy

Twenty-eight post-injury states were assessed in 27 children (in one child two attacks occurred) aged 1-9 years. The states could be classed as so called benign posttraumatic encephalopathy. The mild head injury was followed after a latent period (proved in 22 attacks) of 5 min. to 14 hours by transient brain disorder (in 17 cases quantitative, qualitative and combined alteration in consciousness, in 7 children cortical blindness, in 2 epileptic seizure and in two brain stem disturbance). The duration of symptoms lasted 5 minutes to 48 hours. After recovery the children had no difficulties; the mean of follow-up was 3.3 years. The EEG showed predominantly a normal picture or only abnormal background activity of bioccipital slow waves. In the history and clinical picture we found after the attack most often perinatal abnormality and minimal brain dysfunction (15x, 57%) and various forms of migraine (4x, 16%). It indicates that in the pathogenesis of benign posttraumatic encephalopathy apparently an important part is played by pretraumatic cerebral affection.     

Randomised controlled trial of treatment of unilateral visual impairment detected at preschool vision screening

Objectives To test the efficacy of treatment for unilateral visual loss detected by preschool vision screening and the extent to which effectiveness varies with initial severity.Design Randomised controlled trial of full treatment with glasses and patching, if required, compared with glasses only or no treatment. Masked assessment of best corrected acuity after one year of follow up.Setting Eight UK eye departments.Participants 177 children aged 3-5 years with mild to moderate unilateral impairment of acuity (6/9 to 6/36) detected by screening.Results Children in the full and glasses treatment groups had incrementally better visual acuity at follow up than children who received no treatment, but the mean treatment effect between full and no treatment was equivalent to only one line on a Snellen chart (0.11 log units; 95% confidence interval 0.050 to 0.171; P < 0.0001). The effects of treatment depended on initial acuity: full treatment showed a substantial effect in the moderate acuity group (6/36 to 6/18 at recruitment) and no significant effect in the mild acuity group (6/9 to 6/12 at recruitment) (P = 0.006 for linear regression interaction term). For 64 children with moderate acuity loss the treatment effect was 0.20 log units, equivalent to one to two lines on a Snellen chart. When all children had received treatment, six months after the end of the trial, there was no significant difference in acuity between the groups.Conclusions Treatment is worth while in children with the poorest acuity, but in children with mild (6/9 to 6/12) unilateral acuity loss there was little benefit. Delay in treatment until the age of 5 did not seem to influence effectiveness.     

Somnolence after Prophylactic Cranial Irradiation in Children with Acute Lymphoblastic Leukaemia

A transient cerebral disturbance characterized by somnolence of varying degree is described in children after cranial irradiation given as part of central nervous system (C.N.S.) prophylaxis for acute lymphoblastic leukaemia in remission.Out of 28 such children receiving cranial irradiation as part of the Medical Research Council protocol for C.N.S. prophylaxis 11 (39%) developed pronounced symptoms of somnolence, anorexia, and lethargy some six weeks after the completion of cranial irradiation, and a further 11 (39%) developed these features in mild form. In all cases the symptoms were transient, no focal neurological abnormality was detected, and all children made a spontaneous and complete recovery. E.E.G. studies on five somnolent children showed similar abnormal activity of diffuse and patchy distribution over both hemispheres. Indirect evidence is presented to support the concept that this syndrome represents a transient radiation encephalopathy, analogous to acute transient radiation myelopathy, caused by temporary disturbance of myelin synthesis.     

Experimental reproduction of ELEM

An experiment to gain insight into the minimum toxic dose of fumonisins was conducted by feeding ponies rations with known fumonisin concentrations. Naturally contaminated corn screenings (CS) were blended with pellets, corn, and molasses to formulate individual daily diets. One group of 4 ponies was fed a ration with fumonisin B₁ (FB₁) varying from <1 ppm to 22 ppm. A second group of 5 ponies was fed a ration at varying rates containing 8 ppm FB₁ for 180 days. A panel of clinical chemistry parameters was evaluated twice weekly for both groups. One pony in the first group died of equine leukoencephalomalacia (ELEM) after 225 days of which the final 55 days' diet contained 22 ppm FB₁. Approximately 9 days prior to death, this animal experienced elevated liver chemistry values. All 5 ponies in the second group experienced mild, transient, clincal signs; were euthanized at 180 days; and had mild, histopathological brain lesions.     

Specific and Evolving Resting-State Network Alterations in Post-Concussion Syndrome Following Mild Traumatic Brain Injury

Post-concussion syndrome has been related to axonal damage in patients with mild traumatic brain injury, but little is known about the consequences of injury on brain networks. In the present study, our aim was to characterize changes in functional brain networks following mild traumatic brain injury in patients with post-concussion syndrome using resting-state functional magnetic resonance imaging data. We investigated 17 injured patients with persistent post-concussion syndrome (under the DSM-IV criteria) at 6 months post-injury compared with 38 mild traumatic brain injury patients with no post-concussion syndrome and 34 healthy controls. All patients underwent magnetic resonance imaging examinations at the subacute (1–3 weeks) and late (6 months) phases after injury. Group-wise differences in functional brain networks were analyzed using graph theory measures. Patterns of long-range functional networks alterations were found in all mild traumatic brain injury patients. Mild traumatic brain injury patients with post-concussion syndrome had greater alterations than patients without post-concussion syndrome. In patients with post-concussion syndrome, changes specifically affected temporal and thalamic regions predominantly at the subacute stage and frontal regions at the late phase. Our results suggest that the post-concussion syndrome is associated with specific abnormalities in functional brain network that may contribute to explain deficits typically observed in PCS patients.     

Differential Pathological and Immune Responses in Newly Weaned Ferrets Are Associated with a Mild Clinical Outcome of Pandemic 2009 H1N1 Infection

Young children are typically considered a high-risk group for disease associated with influenza virus infection. Interestingly, recent clinical reports suggested that young children were the smallest group of cases with severe pandemic 2009 H1N1 (H1N1pdm) influenza virus infection. Here we established a newly weaned ferret model for the investigation of H1N1pdm infection in young age groups compared to adults. We found that young ferrets had a significantly milder fever and less weight loss than adult ferrets, which paralleled the mild clinical symptoms in the younger humans. Although there was no significant difference in viral clearance, disease severity was associated with pulmonary pathology, where newly weaned ferrets had an earlier pathology improvement. We examined the immune responses associated with protection of the young age group during H1N1pdm infection. We found that interferon and regulatory interleukin-10 responses were more robust in the lungs of young ferrets. In contrast, myeloperoxidase and major histocompatibility complex responses were persistently higher in the adult lungs; as well, the numbers of inflammation-prone granulocytes were highly elevated in the adult peripheral blood. Importantly, we observed that H1N1pdm infection triggered formation of lung structures that resembled inducible bronchus-associated lymphoid tissues (iBALTs) in young ferrets which were associated with high levels of homeostatic chemokines CCL19 and CXCL13, but these were not seen in the adult ferrets with severe disease. These results may be extrapolated to a model of the mild disease seen in human children. Furthermore, these mechanistic analyses provide significant new insight into the developing immune system and effective strategies for intervention and vaccination against respiratory viruses.     

Effectiveness of adenotonsillectomy in children with mild symptoms of throat infections or adenotonsillar hypertrophy: open,randomised controlled trial

Objective To assess the effectiveness of adenotonsillectomy in children with mild symptoms of throat infections or adenotonsillar hypertrophy.Design Open, randomised controlled trial.Setting 21 general hospitals and three academic centres in the Netherlands.Participants 300 children aged 2-8 years requiring adenotonsillectomy.Intervention Adenotonsillectomy compared with watchful waiting.Main outcome measures Episodes of fever, throat infections, upper respiratory tract infections, and health related quality of life.Results During the median follow up period of 22 months, children in the adenotonsillectomy group had 2.97 episodes of fever per person year compared with 3.18 in the watchful waiting group (difference -0.21, 95% confidence interval -0.54 to 0.12), 0.56 throat infections per person year compared with 0.77 (-0.21, -0.36 to -0.06), and 5.47 upper respiratory tract infections per person year compared with 6.00 (-0.53, -0.97 to -0.08). No clinically relevant differences were found for health related quality of life. Adenotonsillectomy was more effective in children with a history of three to six throat infections than in those with none to two. 12 children had complications related to surgery.Conclusion Adenotonsillectomy has no major clinical benefits over watchful waiting in children with mild symptoms of throat infections or adenotonsillar hypertrophy.     

血清硫氧还蛋白1与脑外伤患者的白质恢复的相关性分析

为探讨血清硫氧还蛋白1 (thioredoxin 1, Trx1)含量与脑外伤后白质恢复的相关性关系,本研究选取60例脑外伤患者,根据格拉斯哥昏迷指数(Glasgow coma scale, GCS)将患者分为3组(轻度,中度和重度脑外伤患者),每组20例,采用核磁共振成像(magnetic resonance imaging, MRI)检测各组患者头颅白质情况,并通过ELISA方法检测各组患者血清Trx1水平,最后利用SPSS软件Pearson方法检测血清硫氧还蛋白1含量与脑外伤后白质恢复的相关性。结果显示,轻度脑损伤患者的GCS分值明显高于中度和重度脑外伤患者;轻度脑外伤患者的Trx1含量明显高于中度和重度患者;轻度和中度脑外伤患者的白质恢复情况明显优于重度患者;脑损伤患者的血清Trx1含量和白质恢复程度呈正相关。本研究初步结论表明脑外伤患者的白质恢复情况与血清Trx1存在正相关性关系,这将为脑外伤的治疗提供新思路。     

支原体肺炎患儿血清中相关炎性因子的表达变化情况及其临床意义探讨(英文)

目的:探讨支原体肺炎(Mycoplasma Pneumoniae Pneumonia,MPP)患儿血清中细胞因子IL-8,IL-12的表达水平及hs-CRP、IgG和血清补体(C)的变化及其临床意义。方法:收集MPP患儿50例,分为重症组、轻症组。健康儿童42例作为对照组;用ELISA法测定MPP患儿急性期、恢复期及对照组儿童血清IL-8、IL-12的水平,用血浆蛋白分析仪速率散射比浊法测定hs-CRP,Ig和C含量。结果:在急性期和恢复期MPP患儿血清IL-12含量明显低于正常对照组(P0.05);而血清IL-8含量在急性期明显高于正常对照组(P0.01)。重症组患儿血清中IL-12明显低于轻症组,而血清中IL-8较轻症组高(P均0.01)。急性期MPP患儿血清IgM,IgG与对照组相比明显升高(P均0.01);而IgA明显降低(P0.05)。急性期MPP患儿hs-CRP、C3、C4与对照组比较显著升高(分别为P0.01、P0.01、P0.05)。重症组患儿血清中IgM,IgG与轻症组相比明显升高(P均0.01);IgA与轻症组相比明显降低(P0.05);重症组患儿血清中hs-CRP、C3、C4与轻症组相比明显升高(P均0.01)。结论:检测相关血清炎性细胞因子对判定MPP患儿的病情和预后有较高的临床应用价值。     

Reduced Ventral Cingulum Integrity and Increased Behavioral Problems in Children with Isolated Optic Nerve Hypoplasia and Mild to Moderate or No Visual Impairment

Objectives

To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified.

Patients and Methods

Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years) underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years) underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex) was performed.

Results

Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05) than controls (4 had scores in the clinically significant range). Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = −0.52, p<0.02, r = −0.46, p<0.049 respectively). There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores.

Conclusions

Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic radiations of children with mild to moderate or no visual impairment raises questions as to the pathogenesis of these changes which will need to be addressed by future studies.     

泌尿系造影联合彩色多普勒对小儿先天性肾积水的诊断价值评估

摘要 目的：探讨泌尿系造影联合彩色多普勒对小儿先天性肾积水的诊断价值。方法：选取2018年11月～2021年11月在本院治疗的88例先天性肾积水患儿为研究对象,所有患儿均完善静脉肾盂造影及彩色多普勒超声检查,以病理诊断结果为金标准,对比两种检查方法对小儿先天性肾积水的诊断价值。结果：彩色多普勒超声检查结果显示,肾积水轻度、中度、重度患儿分别为10例、39例、39例,不同病情程度患儿比较,重度组收缩期峰值速度（PSV）、舒张期最小流速（EDV）均低于中度组和轻度组,重度组血流阻力指数（RI）高于中度组和轻度组（P<0.05）,但轻度组与中度组PSV、EDV、RI比较差异无统计学意义（P>0.05）。与病理学诊断检查结果对比,彩色多普勒超声对中度、重度先天性肾积水患儿具有较高的诊断效能,其准确度分别为90.91%、93.18%,与病理诊断kappa值分别为0.795、0.862,具有较高的一致性;但对轻度肾积水诊断效能较低,kappa值为0.629,一致性一般。静脉肾盂造影对轻度先天性肾积水患儿具有较高的诊断效能,准确度为96.59%,与病理诊断kappa值为0.824,具有较高的一致性;但对中度、重度肾积水诊断效能较低,kappa值分别为0.583、0.565,一致性一般。彩色多普勒超声联合静脉肾盂造影诊断准确率高达94.32%,明显高于两检查方法单独应用（P<0.05）。结论：不同病情程度的先天性肾积水患儿具有不同超声征象,彩色多普勒超声对中、重度肾积水患儿具有较好的诊断价值,而静脉肾盂造影诊断轻度肾积水患儿的效能较好,将二者联合可提高对先天性肾积水的诊断准确率。     

Substantial sulfatide deficiency and ceramide elevation in very early Alzheimer's disease: potential role in disease pathogenesis

In addition to pathology in the gray matter, there are also abnormalities in the white matter in Alzheimer's disease (AD). Sulfatide species are a class of myelin-specific sphingolipids and are involved in certain diseases of the central nervous system. To assess whether sulfatide content in gray and white matter in human subjects is associated with both the presence of Alzheimer's disease (AD) pathology as well as the stage of dementia, we analyzed the sulfatide content of brain tissue lipid extracts by electrospray ionization mass spectrometry from 22 subjects whose cognitive status at time of death varied from no dementia to very severe dementia. All subjects with dementia had AD pathology. The results demonstrate that: (i) sulfatides were depleted up to 93% in gray matter and up to 58% in white matter from all examined brain regions from AD subjects with very mild dementia, whereas all other major classes of lipid (except plasmalogen) in these subjects were not altered in comparison to those from age-matched subjects with no dementia; (ii) there was no apparent deficiency in the biosynthesis of sulfatides in very mild AD subjects as characterized by the examination of galactocerebroside sulfotransferase activities in post-mortem brain tissues; (iii) the content of ceramides (a class of potential degradation products of sulfatides) was elevated more than three-fold in white matter and peaked at the stage of very mild dementia. The findings demonstrate that a marked decrease in sulfatides is associated with AD pathology even in subjects with very mild dementia and that these changes may be linked with early events in the pathological process of AD.     

Unexpected death of a 12 year old boy with monosomy 1p36

Monosomy 1p36 may result in a clinically recognizable chromosomal microdeletion syndrome. We report the unexpected death of a 12 year old boy with mildly dysmorphic facial features, short stature at 138 cm (3rd centile), moderate mental retardation and a history of seizures, obesity, transient muscle weakness of the right arm and leg and episodes of transient atonic hemiparesis of the right side of the body. Despite the relatively few congenital anomalies and normal karyotype, the 1p36 deletion was suspected on clinical grounds and was demonstrated by fluorescent in situ hybridisation (FISH). Two months after diagnosis and following a short history of a mild upper airway infection, high fever and severe diarrhea, the patient had a massive circulatory shock and asystolia, resulting in deep coma, brain edema, apallic syndrome and death. To our knowledge there has been no previous report of episodes of transient unilateral muscle weakness and atonic hemiparesis, circulatory shock and sudden death associated with monosomy 1p36.     

Immunization with Live Measles Virus Vaccine

One hundred and fifty children who had no measurable serum measles antibody were vaccinated with live measles virus. All showed post-vaccinal serum antibody levels generally considered sufficient to prevent measles.One hundred and thirty-nine (93%) of the infants and children demonstrated one or more symptoms following vaccination. In view of their generally mild and limited nature, and of the protection subsequently afforded, this procedure would seem to be a real advance in preventive medicine.     

Muscarinic Receptors in Amygdala Control Trace Fear Conditioning

Intelligent behavior requires transient memory, which entails the ability to retain information over short time periods. A newly-emerging hypothesis posits that endogenous persistent firing (EPF) is the neurophysiological foundation for aspects or types of transient memory. EPF is enabled by the activation of muscarinic acetylcholine receptors (mAChRs) and is triggered by suprathreshold stimulation. EPF occurs in several brain regions, including the lateral amygdala (LA). The present study examined the role of amygdalar mAChRs in trace fear conditioning, a paradigm that requires transient memory. If mAChR-dependent EPF selectively supports transient memory, then blocking amygdalar mAChRs should impair trace conditioning, while sparing delay and context conditioning, which presumably do not rely upon transient memory. To test the EPF hypothesis, LA was bilaterally infused, prior to trace or delay conditioning, with either a mAChR antagonist (scopolamine) or saline. Computerized video analysis quantified the amount of freezing elicited by the cue and by the training context. Scopolamine infusion profoundly reduced freezing in the trace conditioning group but had no significant effect on delay or context conditioning. This pattern of results was uniquely anticipated by the EPF hypothesis. The present findings are discussed in terms of a systems-level theory of how EPF in LA and several other brain regions might help support trace fear conditioning.     

A rare subclinical or mild type of Becker muscular dystrophy caused by a single exon 48 deletion of the dystrophin gene

In the material of 227 families with Becker muscular dystrophy (BMD), we found nine non-consanguineous families with 17 male individuals carrying a rare mutation—a single exon 48 deletion of the dystrophin gene—who were affected with a very mild or subclinical form of BMD. They were usually detected thanks to accidental findings of elevated serum creatine phosphokinase (sCPK). A thorough clinical analysis of the carriers, both children (12) and adults (5), revealed in some of them muscle hypotonia (10/17) and/or very mild muscle weakness (9/17), as well as decreased tendon reflexes (6/17). Adults, apart from very mild muscle weakness and calf hypertrophy in some, had no significant abnormalities on neurological assessments and had good exercise tolerance. Parents of the children carriers of the exon 48 deletion are usually unaware of their children being affected, and possibly at risk of developing life-threatening cardiomyopathy. The same concerns the adult male carriers. Therefore, the authors postulate undertaking preventive measures such as cascade screening of the relatives of the probands. Newborn screening programmes of Duchenne muscular dystrophy (DMD)/BMD based on sCPK marked increase may be considered.     

小儿肺热咳喘口服液联合三联吸入雾化治疗法对哮喘急性发作的影响

目的:探讨小儿肺热咳喘口服液联合三联吸入雾化治疗方案对哮喘患儿的治疗效果以及对肺功能的影响作用。方法:将我院自2017年1月至2018年11月间收治的哮喘患儿210例作为研究对象,按照随机数字表法分为两组各105例,研究组患儿在布地奈德、沙丁胺醇、异丙托溴铵三联吸入雾化治疗的基础上给予小儿肺热咳喘口服液进行治疗,对照组患儿仅给予三联雾化吸入治疗,对比观察两组患儿的疗效和预后。结果:研究组临床治疗后总有效率为95.24%,明显高于对照组77.14%(P0.05);治疗后研究组咳嗽消失时间、呼吸困难消失时间和急性发作随诊时间均明显短于对照组(P0.05),两组肺部喘鸣音消失时间比较差异无统计学意义(P0.05);治疗前患儿第一秒用力呼气量(forced expiratory volume in one second,FEV1)、最大肺活量(forced vital capacity,FVC)及FEV1/FVC值、呼气峰流速值(peak expiratory flowrate,PEF)对比无统计学意义(P0.05),治疗后1 d、3 d、7 d以上指标水平均明显升高,且在治疗后7 d,研究组明显高于对照组(P0.05)。治疗期间研究组有1例出现轻度腹泻,3例食欲减退,并发症的发生率为3.81%(4/105),对照组治疗期间2例出现轻度腹泻,4例食欲减退,并发症的发生率为5.71%(6/105),两组比较无统计学意义(P0.05)。结论:使用小儿肺热咳喘口服液联合三联吸入雾化法治疗儿童哮喘急性发作,可改善患儿临床症状和肺功能,疗效显著,可推广使用。     

Occurrence of Clostridium difficile in fecal samples of HIV-infected children in Poland

The prevalence of Clostridium difficile and its toxins (A and B) in HIV-positive children in Poland was investigated in a group of 18 children, aged 6 months to 8 1/2 years. Stool samples were tested using an antigen detection method for toxin A/B, cytotoxicity-neutralization and culture. In 3 cases (17%) C. difficile toxins were detected in both stool samples and strains recovered from culture. The three strains isolated were shown by PCR methods to contain toxins A and B genes. All children had been treated previously with antimicrobial and antiviral agents. All three C. difficile-positive children had mild diarrhea that resolved without specific therapy. Further studies involving a large number of children and molecular analyses of isolated C. difficile strains are necessary to determine the frequency and rate of carriage of C. difficile strains among HIV-positive children in Poland.     

Clinical trial of the Oka strain of live attenuated varicella vaccine on healthy children

Clinical and serological follow-up studies were made on 257 healthy children who had received live varicella vaccine (strain Oka) in Showa Hospital. Good antibody responses were shown with a seroconversion rate of 98.4% (253/257) by the immune adherence hemagglutination test. Mild adverse reactions were observed in 11 of the vaccinated children. During observation periods of 6 months to 4 years, 6 of the 253 children who were successfully vaccinated contracted mild varicella, while all 4 vaccinees who showed no primary immune response contracted mild to moderate clinical varicella. It is concluded that this vaccine is highly immunogenic and causes few clinical reactions in normal children.     

Efficacy of a small single dose of oral dexamethasone for outpatient croup: a double blind placebo controlled clinical trial.

OBJECTIVE--To assess the efficacy of a single dose of oral dexamethasone 0.15 mg/kg in children with mild croup not admitted to hospital. DESIGN--Double blind, randomised, placebo controlled clinical trial. SETTING--The emergency department of a tertiary paediatric hospital. SUBJECTS--100 children aged 4-122 months presenting with mild croup. INTERVENTION--A single oral dose of dexamethasone 0.15 mg/kg or placebo. MAIN OUTCOME MEASURE--Return to medical care with ongoing croup. RESULTS--Baseline characteristics of the two treatment groups were similar. Eight children (all from the placebo group) returned to medical care with ongoing croup, one being admitted. There was no reported difference in duration of croup symptoms, duration of viral symptoms, or rate of return to medical care for other reasons. CONCLUSION--Oral dexamethasone in a dose of 0.15 mg/kg is effective in reducing return to medical care with ongoing croup in children with mild croup.