Immune Deficiency,Thrombocytopenia and Osteomyelitis in Pediatric Patients

Surgical decompression of osteomyelitis in pediatric patients should be an essential part of therapy in most cases. The initial deferral of prompt decompression in two patients because of underlying chronic, hemorrhagic (thrombocytopenic) disorders—one with Wiskott-Aldrich syndrome and one with Gaucher''s disease—resulted in more serious and prolonged courses of the infectious processes. The complications might have been ameliorated by earlier drainage in each case. Based upon the experience gained from these two patients, we recommend early drainage, with appropriate treatment of the hemorrhagic disorder, to prevent more widespread dissemination or prolongation of osseous infection in similarly affected children.     

不同类型弱视儿童视网膜神经纤维层与预后视力恢复的相关性

摘要 目的：探讨不同类型弱视儿童视网膜神经纤维层（RNFL）与预后视力恢复的相关性。方法：选择2017年6月至2020年6月在本院眼科就诊的80例弱视患儿作为研究对象,其中屈光参差性弱视32例（A组）、斜视性弱视28例（B组）、屈光不正性弱视20例（C组）。三组患儿都进行常规检查与光学相干断层成像（OCT）,调查随访患儿的预后视力恢复情况,并进行相关性分析。结果：三组的视盘面积、盘沿面积、校正视盘面积、校正盘沿面积、等效球镜绝对值、眼轴长度等数据对比无差异（P>0.05）。B组与C组的上方、鼻侧、下方、颞侧、全周的RNFL厚度都高于A组（P<0.05）,C组高于B组（P<0.05）。随访截止时间为2021年1月,A组、B组与C组的总有效率分别为87.5 %、85.7 %和85.0 %,对比无差异（P>0.05）。Pearson线性相关分析显示预后总有效率与上方、鼻侧、下方、颞侧、全周的RNFL厚度均存在相关性（P<0.05）。结论：不同类型弱视儿童的视网膜神经纤维层结构厚度存在差异,与患儿的预后视力恢复存在相关性。     

儿科抗癫痫药物应用研究进展

儿童癫痫为小儿神经科的常见疾病,临床表现以抽搐为主。近年来,随着医疗技术的发展,以及人们对儿童癫痫的重视,国内外文献对儿科癫痫的治疗报道越来越多,目前,药物治疗仍然是抗癫痫的首选方法,除了运用新型抗癫痫药物外,也有采用中药治疗癫痫的报道,现就近年来儿童癫痫的药物治疗研究作一综述。     

Is a compromised interferon response an etiologic factor in Reye's syndrome?

Young mice injected with sublethal doses of Toximul MP8, a typical commercial polyoxyethylene ether-based emulsifier, died more frequently when infected with encephalomyocarditis virus than did control mice. Lymphocytes taken from emulsifier-injected mice responded poorly to interferon induction, unlike lymphocytes from control animals. Interferon protected control mice against viral encephalomyocarditis, but such protection was not equally demonstrable in emulsifier-injected mice. These data suggest that the enhanced lethality of encephalomyocarditis virus in emulsifier-injected mice is associated with and perhaps caused by a compromised interferon response in these animals. Since these emulsifiers are commonly found in the environment in areas where forests are sprayed with pesticides, a group of children suffering from Reye''s syndrome who lived in such areas was investigated. Blood samples were obtained from five children with influenza B-associated Reye''s syndrome during their acute illness and during convalescence. Lymphocytes obtained from these samples and from peripheral blood samples from healthy children (controls) were induced to synthesize interferon by exposure to Newcastle disease virus. The lymphocytes from the convalescent patients and from the controls responded well to induction. However, the lymphocytes obtained from patients and from the controls responded well to induction. However, the lymphocytes obtained from patients during the acute phase of Reye''s syndrome responded very poorly and produced significantly less interferon.     

Identity cards for patients infected with HIV?

Dr A C Srivastava has written to us to describe a case that raises the suggestion that people infected with the human immuno-deficiency virus (HIV) should carry identity cards. We asked two physicians, a general practitioner working with patients with the acquired immune deficiency syndrome (AIDS), and a general practitioner with a special interest in medical ethics to respond to the broad issues raised by Dr Srivastava''s letter.     

综合治疗法对不同弱视儿童治疗效果观察

目的:探讨综合治疗法对不同程度、不同年龄、不同类型的弱视患儿的临床疗效。方法:选取2012年1月~2014年1月在我院接受治疗的弱视患儿393例(671眼),根据弱视程度分为轻度组(299眼)、中度组(293眼)和重度组(79眼),3组患者均采用遮盖、精细目力训练、仪器训练及压抑疗法进行治疗,均治疗6~12个月,比较不同程度、不同年龄、不同类型的弱视患儿治疗后临床疗效。结果:治疗后,轻度、中度和重度组患儿的治愈率分别为90.97%、72.35%和17.72%,总有效率分别为100.00%、96.93%和84.81%,组间比较差异具有统计学意义(P0.05)。3~5岁、6~8岁和9~11岁患儿的治愈率分别为77.08%、74.72%和71.03%,总有效率分别为99.48%、97.36%和93.93%,3~5岁患儿总有效率显著高于9~11岁患儿(P0.05),但三个年龄段患儿治愈率差异不显著(P0.05)。屈光不正性弱视患儿、斜视性弱视患儿、屈光参差性弱视患儿的治愈率分别为85.65%、45.75%和61.54%,总有效率分别为99.34%、90.20%和95.38%,其中,屈光不正性弱视患儿经治疗的总有效率和治愈率均显著高于其他两种类型(P0.05)。结论:综合治疗法对弱视患儿的临床疗效与弱视程度、弱视类型和年龄密切相关,结合儿童弱视类型提早治疗是提高预后的关键。     

Progressive outer retinal necrosis syndrome: a comprehensive review of its clinical presentation, relationship to immune system status, and management

Progressive outer retinal necrosis (PORN) syndrome is a form of the Varicella zoster virus (VZV) chorioretinitis found almost exclusively in people with the acquired immunodeficiency syndrome (AIDS). This destructive infection has an extremely rapid course that may lead to no light perception in affected eyes within days or weeks. Attempts at its treatment have had limited success. Rhegmatogenous retinal detachments often occur after the development of atrophic retinal holes, and silicone oil temponade has been found to be the most successful reattachment procedure. Unfortunately, cataract formation is common after such surgery. PORN needs to be differentiated from acute retinal necrosis (ARN) syndrome, a necrotizing retinitis that can also be caused by VZV. PORN and ARN are found at opposite ends of the spectrum of necrotizing herpetic retinopathies (NHR), where its clinical presentation depends upon immune system status. After a brief case presentation, the distinguishing clinical characteristics of PORN, its differentiation from ARN, attempts at its treatment, the role of the immune system status on its clinical appearance and treatment, and management of complications such as retinal detachment and subsequent cataracts are discussed.     

A CMA position. Acquired immunodeficiency syndrome.

The following general principles serve as guidelines for various bodies, health care professionals and the general public. Specific aspects of infection with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) that relate to physicians'' ethical responsibilities as well as society''s moral obligations are discussed. Such matters include the need for education, research and treatment resources; the patient''s right to investigation and treatment and to refusal of either; the need to obtain the patient''s informed consent; the right to privacy and confidentiality; the importance of infection control; and the right to financial compensation in the case of occupational exposure to HIV.     

37例儿童噬血细胞综合征的临床分析

目的:分析儿童噬血细胞综合征(hemophagocytic syndrome,HPS)的病因、临床表现、实验室检查结果、治疗和预后特点。方法:回顾性分析我院收治的37例HPS患儿的临床资料。结果:37例HPS患儿(男24例、女13例),年龄2月~9岁,5例(13.5%)有明显家族史,获得性HPS32例(86.5%),包括EB病毒感染16例、巨细胞病毒感染7例,其他原因9例;所有患儿均表现为发热,肝脾肿大,外周血白细胞、血红蛋白、血小板、白蛋白、纤维蛋白原减低,TG、ALT、AST、LDH、铁蛋白升高;5例遗传性HPS患儿死亡4例,放弃治疗1例,剩余32例患儿中好转20例(62.5%),包括痊愈17例,完全缓解后继续治疗中3例,未好转12例(37.5%),其中死亡7例,病情危重放弃治疗3例,复发2例。12例未好转病例中,9例为EBV感染,1例为肾母细胞瘤,1例为幼年类风湿性关节炎合并CMV感染,1例原因不明。遗传性HPS的好转率较继发性HPS明显降低,差异有统计学意义(X2=5.30,P0.05),继发性HPS中EBV感染者的好转率较非EBV感染者低,差异有统计学意义(X2=4.80,P0.05)。结论:及时诊断儿童HPS并明确其病因,对该病的治疗及预后具有重要意义。     

Primary care for AIDS and chemical dependence.

Primary care clinicians are acquiring an increasingly important role in preventing, diagnosing, and treating both chemical dependence and human immunodeficiency virus (HIV) illness. Towards this end they need to know the epidemiology of HIV infection in chemically dependent persons and methods of educating persons at high risk for these problems. It is critical that physicians screen for alcohol and drug addiction. Health care providers should understand the risks and benefits of HIV antibody testing and include in their practices the basic components of counseling before and after testing and informed consent. Both HIV illness and addiction are chronic diseases with long-term health implications. A knowledge of patient characteristics, intensity of treatments, and treatment modalities is important in making recommendations for individualized therapy. Combining service delivery is a future challenge necessitated by today''s joint epidemics of the acquired immunodeficiency syndrome and chemical dependence.     

Abnormal Fixational Eye Movements in Amblyopia

Purpose

Fixational saccades shift the foveal image to counteract visual fading related to neural adaptation. Drifts are slow eye movements between two adjacent fixational saccades. We quantified fixational saccades and asked whether their changes could be attributed to pathologic drifts seen in amblyopia, one of the most common causes of blindness in childhood.

Methods

Thirty-six pediatric subjects with varying severity of amblyopia and eleven healthy age-matched controls held their gaze on a visual target. Eye movements were measured with high-resolution video-oculography during fellow eye-viewing and amblyopic eye-viewing conditions. Fixational saccades and drifts were analyzed in the amblyopic and fellow eye and compared with controls.

Results

We found an increase in the amplitude with decreased frequency of fixational saccades in children with amblyopia. These alterations in fixational eye movements correlated with the severity of their amblyopia. There was also an increase in eye position variance during drifts in amblyopes. There was no correlation between the eye position variance or the eye velocity during ocular drifts and the amplitude of subsequent fixational saccade. Our findings suggest that abnormalities in fixational saccades in amblyopia are independent of the ocular drift.

Discussion

This investigation of amblyopia in pediatric age group quantitatively characterizes the fixation instability. Impaired properties of fixational saccades could be the consequence of abnormal processing and reorganization of the visual system in amblyopia. Paucity in the visual feedback during amblyopic eye-viewing condition can attribute to the increased eye position variance and drift velocity.     

阿托品压抑疗法和遮盖疗法治疗学龄儿童单眼弱视的临床效果

目的:探讨阿托品不同治疗方法对于学龄儿童单眼弱势的治疗效果。方法:回顾抽取我院眼科住院治疗的90例单眼弱视学龄儿童患者,根据疗法分成压抑组(压抑疗法)和遮盖组(短时遮盖疗法)各45例,对比分析两组患儿在视力、依从性、临床疗效和不良反应方面的差异性。结果:治疗后,压抑组患儿的视力、依从率(93.33%)、总有效率(91.11%)均显著高于遮盖组,且不良反应总发生率(15.00%)显著低于遮盖组(26.67%),差异具有统计学意义(P0.05)。结论:阿托品压抑疗法治疗学龄儿童单眼弱视具有疗效显著、依从性好、视力矫正效果明显、安全性好等优点,值得临床推荐。     

AIDS-associated malignancies: research perspectives

The appearance in 1981 of a usually rare malignancy, Kaposi’s sarcoma, in homosexual men [1] was one of the first harbingers of an epidemic caused by a retrovirus, human immunodeficiency virus (HIV), which causes the acquired immunodeficiency syndrome (AIDS). Lymphoid and other malignancies were also increased, most strikingly non-Hodgkin’s lymphoma and primary central nervous system (CNS) lymphoma. Advances in molecular biology, immunology, virology, and anti-viral therapy have combined to create unique research opportunities. One developing theme is the role of viral co-infection and malignancy. Human herpes virus 8 (HHV8), Epstein-Barr virus (EBV) and papilloma virus each may have a causal role in the development of HIV-associated malignancy. New antiretroviral therapies are able to substantially reverse or delay the profound immunosuppression of HIV infection. The changes in the epidemiology of malignancies, and understanding the mechanism of action of these new therapeutics provide research opportunities to understand the pathogenesis of these malignancies. The opportunities to discover the consequences of T-cell based immunodeficiency and the interactions with specific viral pathogens will likely lead to progress in HIV treatment and new strategies for other malignancies.     

Role of the School Eye Clinic in Modern Ophthalmology

Routine sight tests for children at intervals throughout their school career are clearly important; three-quarters of those referred to the school eye clinics in this area had some ocular defect. It is probably no longer necessary for myopic schoolchildren to be treated by a consultant ophthalmologist after their initial examination.Forty-five per cent. of children referred to the school eye clinics in this area were found to have squint and/or hypermetropic/anisometropic/astigmatic refractive errors. Priority should be given to this group because of the association of amblyopia with these conditions. Their treatment requires closer association with the hospital ophthalmic department, perhaps even complete unity. Transfer of children at present seen in the school eye clinic to a hospital-based “children''s eye clinic” would also fit in with a unified health service administration structure and be better placed to indicate, evaluate, and control future developments towards the prevention of amblyopia.     

Ocular Findings in Several Metabolic Diseases

Changes in ocular findings have been noted in association with several metabolic diseases.In homocystinuria the crystalline lens in the majority of cases is subluxated inferiorly, while in Marfan''s syndrome the dislocation was upward.In cystinosis, slit-lamp examination reveals numerous gold crystal-like cystine deposits in both the cornea and bulbar conjunctiva.Patients with galactosemia have cataracts of the “oil drop” type, which usually can be seen with an ophthalmoscope even though the opacity is not dense.Eight patients with Lowe''s syndrome who were observed had cataracts, and four of them had severe glaucoma.Three of five patients with glycogen storage disease Type I had yellowish deposits in the macular and paramacular areas, thought to be due to hypercholesterolemia.     

Animal-Assisted Interventions in Children's Hospitals: A Critical Review of the Literature

There is a perception in the scientific and general communities that hospitalized children benefit from visits by animals. Animal-assisted interventions (AAI), including animal-assisted therapy and animal-assisted activities, usually involving dogs, are thus employed in pediatric hospitals. However, the actual prevalence of AAI in children's hospitals has been poorly documented in the literature. Furthermore, the evidence base for claims that children in hospital benefit from AAI is limited. There are nine existing research studies in the area, all with methodological challenges that make conclusive statements in either direction about the efficacy of AAI difficult. In this critical review we consider methodological considerations pertinent to evaluations of AAI interventions for hospitalized children. These include: definitions and terminology; cultural attitudes; children's receptivity to animals, including phobia, type of illness and health status of the child, familiar as opposed to unknown animals, and age of the child; animal welfare; zoonoses and allergies; and hospital staff attitudes toward AAI. We highlight the many difficulties involved in conducting research on AAI in pediatric settings. Given the limited information around AAI for hospitalized children, including the risks and benefits and the limitations of existing studies, future research is required. This should take into account the methodological considerations discussed in this review, so that our knowledge base can be enhanced and if and where appropriate, such interventions be implemented and rigorously evaluated.     

The epidemiology and disease burden of children hospitalized for viral infections within the family Flaviviridae in China: A national cross-sectional study

BackgroundViruses of the family Flaviviridae, including Japanese encephalitis virus (JEV), dengue virus (DENV), yellow fever virus (YFV) and hepatitis C virus (HCV), are widely distributed worldwide. JEV, DENV and YFV belong to the genus Flavivirus, whereas HCV belongs to the genus Hepacivirus. Children’s symptoms are usually severe. As a result, rates of hospitalization due to infection with these viruses are high. The epidemiology and disease burden of hospitalized children have rarely been described in detail to date. The objective of this study was to report the general epidemiological characteristics, clinical phenotype, length of stay (LOS), burden of disease, and potential risk factors for hospitalized children infected with JEV, DENV, YFV, or HCV in Chinese pediatric hospitals.MethodologyA cross-sectional study of epidemiology and disease burden of children hospitalized for Flaviviridae virus infections between December 2015 and December 2020 in China was performed. Face sheets of discharge medical records (FSMRs) were collected from 27 tertiary children’s hospitals in the Futang Research Center of Pediatric Development and aggregated into FUTang Update medical REcords (FUTURE). Information on sociodemographic variables, clinical phenotype, and LOS as well as economic burden was included in FSMRs and compared using appropriate statistical tests.FindingsThe study described 490 children aged 0–15 years hospitalized for infections with Flaviviridae viruses. Japanese encephalitis (JE) cases are the highest, accounting for 92.65% of the total hospitalization cases caused by Flaviviridae virus infection. The incidence of JE peaked from July to October with a profile of a high proportion of severe cases (68.06%) and low mortality (0.44%). Rural children had a significantly higher incidence than urban children (91.63%). Most hospitalized dengue cases were reported in 2019 when dengue outbreaks occurred in many provinces of China, although only 14 dengue cases were collected during the study period. Yellow fever (YF) is still an imported disease in China. The hospitalizations for children with hepatitis C (HC) were not high, and mild chronic HC was the main clinical phenotype of patients. Among the four viral infections, JE had the highest disease burden (LOS and expenditure) for hospitalized children.ConclusionFirst, the present study reveals that JE remains the most serious disease due to Flaviviridae virus infection and threatens children’s health in China. Many pediatric patients have severe illnesses, but their mortality rate is lower, suggesting that existing treatment is effective. Both JEV vaccination and infection control of rural children should represent a focus of study. Second, although the dual risks of indigenous epidemics and imports of DENV still exist, the prevalence of DENV in children is generally manageable. Third, YFV currently shows no evidence of an epidemic in China. Finally, the proportion of children with chronic hepatitis C (CHC) is relatively large among hospitalized children diagnosed with HCV. Thus, early and effective intervention should be offered to children infected with HCV to ease the burden of CHC on public health.     

Manitoba aboriginal kindred with original cerebro-oculo- facio-skeletal syndrome has a mutation in the Cockayne syndrome group B (CSB) gene

Cerebro-oculo-facio-skeletal (COFS) syndrome is a rapidly progressive neurological disorder leading to brain atrophy with calcification, cataracts, microcornea, optic atrophy, progressive joint contractures, and growth failure. Cockayne syndrome (CS) is a recessively inherited neurodegenerative disorder characterized by low-to-normal birth weight; growth failure; brain dysmyelination with calcium deposits; cutaneous photosensitivity; pigmentary retinopathy, cataracts, or both; and sensorineural hearing loss. CS cells are hypersensitive to UV radiation because of impaired nucleotide excision repair of UV radiation-induced damage in actively transcribed DNA. The abnormalities in CS are associated with mutations in the CSA or CSB genes. In this report, we present evidence that two probands related to the Manitoba Aboriginal population group within which COFS syndrome was originally reported have cellular phenotypes indistinguishable from those in CS cells. The identical mutation was detected in the CSB gene from both children with COFS syndrome and in both parents of one of the patients. This mutation was also detected in three other patients with COFS syndrome from the Manitoba Aboriginal population group. These results suggest that CS and COFS syndrome share a common pathogenesis.     

间歇性外斜视患儿的注意跟踪能力异常

间歇性外斜视是儿童眼科的常见疾病之一,也是目前眼科学研究的热点领域之一.本研究使用多物体跟踪实验范式,系统考察了间歇性外斜视儿童,相对于弱视儿童以及正常对照儿童,注意的容量是否存在异常.实验结果发现间歇性外斜视儿童和弱视儿童的注意跟踪能力都显著地差于正常对照儿童,而且间歇性外斜视儿童和弱视儿童的跟踪成绩基本没有差别,他们的注意跟踪功能的受损程度相近.本研究首次报道了间歇性外斜视儿童注意能力的行为异常,对进一步研究间歇性外斜视的发病机理和早期临床诊断具有潜在的参考意义.     

Rapid human immunodeficiency virus decay in highly active antiretroviral therapy (HAART)-experienced children after starting mega-HAART

Increasing numbers of patients are treated with mega-highly active antiretroviral therapy (HAART), or multiple-combination antiretroviral therapy, in an attempt to overcome the viral resistance that has contributed to treatment failure. Studies of human immunodeficiency virus (HIV) viral dynamics are used to quantify the potency of a given regimen. While mega-HAART is expected to provide potent therapy, its potency among heavily experienced HIV-infected children who have failed previous treatment is untested. HIV dynamics studies performed in children have provided minimal information on viral dynamics during mega-HAART. The present study estimates first- and second-phase viral dynamics in six children on mega-HAART, following failure of combination therapy. The first phase of viral decay was rapid, relative to rates reported in previous pediatric studies (median delta = 0.778d(-1), range = 0.583 to 1.088, half-life 1 [t1(1/2)] = 0.894d), while the second phase revealed results similar to those of previous studies (median mu = 0.026d(-1), range = -0.005 to 0.206, t2(1/2) = 9.316d). This indicates that mega-HAART can provide potent therapy among heavily experienced pediatric patients.