建立年幼儿童哮喘诊断标准及预测工具的探索

与其他年龄段儿童相比,年幼儿童是发生哮喘的高危年龄段.由于临床表现非特异、喘息表型多样、辅助检查难以实施等原因,年幼儿童哮喘的诊断对临床医师而言存在一定困难.近二十年来,国内外对年幼儿童诊断标准及预测工具的探索从未停止,而我国也亟需建立起年幼儿童哮喘的诊断标准.该文综述国内外建立年幼儿童哮喘诊断标准及预测工具的过程,为建立我国新的年幼儿童哮喘诊断标准及预测工具提供参考.     

两种青少年代谢综合征诊断标准在台湾地区12～19岁人群中的应用比较

目的 比较两种青少年代谢综合征(MS)诊断标准在中国台湾地区12～19岁人群MS及其组分的检出情况,为进一步制定相应的预防和治疗措施提供依据.方法 选择2005年1月～2006年12月首次参加美兆健康体检的12～19岁人群1629名进行横断面研究,应用分别由Cook(标准Ⅰ)和de Ferranti(标准Ⅱ)推荐的两种标准诊断MS,使用χ2检验比较不同性别、不同体重人群MS检出率、MS各组分检出率及MS组分数只构成的性别差异,应用Kappa值评价两种标准在台湾地区青少年中应用的一致性.结果 (1)标准Ⅰ MS检出率为4.05%(男5.84%,女1.98%),标准Ⅱ为8.35%(男10.42%,女5.95%);体重正常、高危和超重二组人中MS标准Ⅰ检出率分别为0.94%、14.20%和36.59%,标准Ⅱ检出率分别为3.61%、25.93%和53.66%;(2)标准Ⅰ的5种组分检出率为9.09%(低高密度脂蛋白胆固醇)～16.39%(高血压),标准Ⅱ的5种组分检出率0.98%(高血糖)～27.13%(高腰围);(3)满足≥3个异常组分的人中,标准Ⅰ具有3项、4项和5项MS组分组合的比例分别为2.76%、1.04%和0.25%,标准Ⅱ分别为6.69%、1.60%和0.34%;(4)MS患者异常组分最常见组合,标准Ⅰ为"肥胖+低HDL-C+高BP",标准Ⅱ为"肥胖+低HDL-C+高甘油三酯";(5)标准Ⅰ和Ⅱ诊断的一致率为94.35%,Kappa值为0.518.结论 采用两种MS诊断标准获得的12～19岁人群检出率和异常组分聚集情况差异较大,结果的高估与低估与标准推荐使用的各组分的切点值高度相关,建议制定具本国特异性切点值的儿童青少年MS诊断标准.     

What are the diagnostic criteria for intestinal neuronal dysplasia?

The incidence of isolated intestinal neuronal dysplasia (IND) has varied from 0.3% to 62% of all suction rectal biopsies in different centres. The uncertainty regarding the incidence has resulted from the considerable confusion regarding the essential diagnostic criteria. In an attempt to clarify the diagnostic criteria for IND, we examined biopsy material from the following three groups using acetylcholinesterase (AChE) histochemistry: (1) full-thickness normal colon from 23 controls; (2) suction rectal biopsies from 9 patients who had isolated IND; and (3) full-thickness biopsies from 10 patients with Hirschsprung's disease (HD) who demonstrated IND in the proximal margin of the resected segment. Our data show that hyperganglionosis is the most consistent finding in both IND associated with HD and isolated IND. Other histochemical criteria of IND were dependent upon whether the biopsy was full-thickness or a suction rectal biopsy. Where full-thickness biopsies were available, giant ganglia and ectopic ganglion cells were seen in all cases. Increases in AChE-positive nerve fibres in the mucosa was a frequent finding in patients with IND diagnosed by suction rectal biopsies. We recommend that patients suspected to have IND on suction rectal biopsy should have a full-thickness biopsy for detailed examination of the submucous and myenteric plexuses.     

Comparison of Scrub Typhus Meningitis with Acute Bacterial Meningitis and Tuberculous Meningitis

Objective

To compare scrub typhus meningitis with bacterial and tuberculous meningitis.

Methods

Children aged<15 years admitted with meningitis were screened and those who fit criteria for diagnosis of scrub typhus meningitis (n=48), bacterial meningitis (n=44) and tuberculous meningitis (n=31) were included for analysis. Clinical features, investigations and outcomes were compared between the three types of meningitis.

Results

Mean age, duration of fever at presentation, presence of headache and, altered sensorium and presence of hepatomegaly/splenomegaly were statistically significantly different between the groups. Scrub typhus had statistically significant thrombocytopenia, shorter hospital stay and a better neurological and mortality outcome.

Conclusions

Sub-acute presentation of meningitis in older age group children, and good outcome is associated with scrub typhus when compared to bacterial and tuberculous meningitis.

     

脑性瘫痪的定义、诊断标准及临床分型

在2005年中华医学会儿科学分会神经学组和2007年中国康复医学会儿童康复专业委员会制定发表的《小儿脑性瘫痪的定义、分型和诊断条件》的基础上,《中国脑性瘫痪康复治疗指南》编写委员会参考国外有关小儿脑性瘫痪诊疗指南和最新文献报道,多次组织讨论编写.以便更好地指导临床医师对小儿脑性瘫痪定义的理解,提高脑性瘫痪的诊断和分型水平,与国际接轨.     

新生儿缺氧缺血性脑病诊断标准中存在的问题

新生儿缺氧缺血性脑病(HIE) 是围生期窒息的严重并发症。国内HIE 的诊断标准和国外标准差 别很大,国内标准未将脐动脉血气和多脏器功能障碍(MOD) 指标纳入其中,容易误诊;国外标准较科学、全面,但过 于严格,可导致漏诊。目前,HIE 缺乏高度特异性和敏感性的诊断指标,诊断应结合病史、Apgar 评分、脐动脉血气、 MOD、头颅影像学及临床经过等多项指标对其进行综合评价。     

Late Otological Sequelae of Tuberculous Meningitis

An otological follow-up study in 1956 and 1959—62 of 103 children treated for and cured of tuberculous meningitis in 1949—54 is presented. Impaired hearing was observed in 63 per cent, severe loss of hearing in 15 per cent and total deafness in 7 per cent. In 36 per cent there was a hearing loss of more than 10 dB in the speech range (500—2,000 cps). The initial and the follow-up results of otological investigations were in close accordance with each other — only in 3 cases was there some improvement and in 11 some deterioration of hearing. The cases with hearing loss were evenly distributed between the different age groups excepting the youngest one — 0—12 months — which displayed a considerably lower frequency of impaired hearing as a whole but a much higher frequency of severe hearing loss. There was no correlation between hearing sequelae and the duration of meningeal symptoms prior to hospitalization or the severity of the disease on admission. There was, however, a correlation between the frequency of hearing sequelae and the daily and total doses of streptomycin given: the frequency of hearing loss was 49 per cent (severe hearing loss 22 per cent) when 50 mg/kg daily was used compared with an incidence of 15 per cent (severe hearing loss in 4 per cent) when 10—20 mg/kg of streptomycin was used intramuscularly and 1—3 mg/kg intrathecally daily.
No correlation was found between loss of hearing and neurological, mental, eye or EEG disturbances.
Disturbances of vestibular function were found in 54 per cent altogether and they were severe in 31 per cent.
Five of the 15 children with severe hearing loss used hearing aids with considerable success, 3 got along without them in ordinary schools and 10 children attended schools for the deaf.     

Late Neurological Sequelae of Tuberculous Meningitis

The neurological findings in a follow-up study of 103 children treated for and cured of tuberculous meningitis in 1949—54, are presented. Fifty children were found to be free of late neurological sequelae, 29 had minor neurological sequelae including 2 with cranial nerve palsies, nystagmus, and ataxia, 13 with mild disturbances of coordination and 10 with symptoms of upper motor neuron lesion without subjective complaints. Gross neurological sequelae were found in 22 cases, including 8 with spastic palsies, 9 with spastic palsies and convulsions, and 5 with convulsions without spastic palsies. Two children were found to have sequelae of spinal lesions; one had paraplegia and the other disturbance of sensation on the dorsum of the left foot. Fourteen children had convulsions.     

Late Ophthalmological Sequelae of Tuberculous Meningitis

One hundred and three patients who had recovered from tuberculous meningitis in 1949—54 were thoroughly re-examined ophthalmologically an average of 8 1/2 years (range 6—12 1/2 years) after recovery. One blind patient and one with severe visual impairment were found. In addition to these, a number of cases with ophthalmological sequelae of minor importance were found; partial structural and functional lesions of the optic nerve were found in 17 cases (including 1 case with associated abducens nerve palsy), nystagmus was found in 2, pupillary disturbances in 6 and scars of choroidal tubercles in 16 cases. Thus 36 cases (35 per cent) showed ophthalmological sequelae of the past illness. Permanent visual disability was found in the 2 cases mentioned above, both of them being late cases with a high block treated with trephination. Of the other sequelae of the disease only intracranial calcification showed some degree of correlation with the ophthalmological sequelae.     

Late Radiological Findings after Recovery from Tuberculous Meningitis

The results of skull x-ray examinations of 101 children recovered from tuberculous meningitis are reported. The roentgenological re-examinations were performed 6—12 1/2 years after the disease, and were repeated later in several cases. Intra-cranial calcifications were found in 26 per cent. Of these, 4 children showed extensive calcified plots, including 1 with intracerebral calcifications. The occurrence of intracranial calcified plots was not correlated with the sex, the duration of meningeal symptoms prior to hospitalization, or the treatment of the patients; nor was it correlated with increased intracranial pressure during the disease or with the late mental and otological sequelae of the disease. Positive correlations were, however, found between the development of intracranial calcifications and the age at the onset of the disease, the state on admission, trephination, occurrence of convulsions and late neurological, EEG and ophthalmological sequelae.     

Late Electroencephalographic Findings after Recovery from Tuberculous Meningitis

Eighty five children and young persons cured of tuberculous meningitis were reexamined by EEG 6—12 1/2 years after the onset of the disease.
Moderate or severe EEG changes were seen in 50 cases, i.e. 59 per cent. The most frequent abnormality found was general dysrhythmia (29 cases) combined with focal abnormalities in 9 cases. Focal abnormalities were seen in the records of altogether 14 former patients. Of these, 9 had convulsions. Repeated EEG examinations were made in 15 cases. Seven of these showed improvement and 1 progression of the disturbances. Abnormal EEGs were seen most frequently among the youngest patients (0—3 years), among patients with severe symptoms on admission and among patients treated with cortisone.
Correlation was also found between EEG changes and other sequelae: convulsions, neurological disorders, mental sub-normality, behaviour disturbances and intracranial calcifications. There were no correlations between EEG abnormalities and late ophthalmological and otological sequelae.     

Modern Treatment and Late Prognosis of Tuberculous Meningitis

Certain controversial subjects in the treatment of tuberculous meningitis are analyzed, amongst them intrathecal administration of streptomycin, the toxicity of isoniazid, long-term treatment with INH, and the etiological role of the primarily resistant strains of tubercle bacilli. The corticoid problem is given special consideration and the possibility of iatrogenic late sequelae due to corticoids is discussed at length. The importance of proper neurosurgical management in cases with increased intracranial pressure and signs of block formation and the value of preventive measures, like BCG vaccination, chemoprophylaxis and isolation are also stressed.
In addition, the methods of treatment presently employed at the Children's Hospital are presented in detail and certain therapeutical problems are discussed in this connection. The recent results, from the years 1956—62, are outlined. Finally, in the late prognosis of the survivors of tuberculous meningitis the emphasis is laid upon certain sociomedical aspects and the children are grouped into: Group A — 50 –60 per cent — fully recovered individuals with no handicaps; Group B – 30—40 per cent — individuals with minor handicaps requiring efficient rehabilitation and special training but with a favourable socio-medical prognosis when given these; and Group C — 10 per cent — severely handicapped individuals at the mercy of the domestic environment and or the community.     

儿童结核性脑膜炎的诊断和治疗

结核性脑膜炎是严重威胁儿童生命健康的神经系统感染性疾病.在小儿肺外结核病中结核性脑膜炎是最常见的,尽管目前开展了足量的化疗,结核性脑膜炎仍有高发病率和病死率的特点.虽然近年来研究者从细菌学、分子生物学及免疫学等方面对小儿结核性脑膜炎的诊断进行了研究,但均末获得突破性进展,对小儿结核性脑膜炎的诊断仍依靠综合分析患儿的临床特点以及通过辅助检查的结果判断.早期及时诊断和完善的治疗是降低结核性脑膜炎病死率及后遗症发生率的关键.     

Additional Tunisian patients with Sanjad–Sakati syndrome: A review toward a consensus on diagnostic criteria

Background and objectives

Sanjad–Sakati syndrome (SSS; OMIM 241410) is a rare autosomal recessive disorder found almost exclusively in people of Arab origin. It is characterized by congenital hypoparathyroidism, severe prenatal and postnatal growth retardation, and distinct facial dysmorphism. The molecular pathology of this syndrome was shown to be due to a mutation in the tubulin-specific chaperone E (TBCE) gene in chromosomal area 1q42-q43. We aimed to detect and confirm the common mutation responsible for SSS in Tunisian patients and review the literature in order to create a set of clinical diagnostic criteria that might provide appropriate indications for molecular testing.

脑脊液置换联合鞘内注射药物治疗中晚期儿童结核性脑膜炎的疗效

目的 探讨脑脊液置换联合鞘内注射药物治疗中晚期儿童结核性脑膜炎的疗效.方法 选择2005年1月-2010年 4月本院收治住院的64例中晚期结核性脑膜炎患儿,随机分为治疗组和对照组各32例.对照组予常规抗结核药物治疗,治疗组在常规抗结核药物治疗基础上行脑脊液置换,并鞘内注射抗结核药物,治疗8周后对比2组治疗效果.结果 治疗组治愈24例,好转 5例,无效 2例,死亡1例,总有效率为90.6%;对照组治愈17例,好转5例,无效7例,死亡2例,总有效率为68.8%.2组总有效率比较差异有统计学意义(χ2=4.729,P<0.05).结论 脑脊液置换联合鞘内射注药物治疗中晚期儿童结核性脑膜炎疗效好,且不良反应少.     

Late Psychological and Psychiatric Sequelae of Tuberculous Meningitis

The psychological and psychiatric sequelae of tuberculous meningitis in 103 children were studied by thorough clinical follow-up examinations at the Children's Hospital, University of Helsinki, and extensive correspondence with the parents and teachers of the children. Forty five per cent of the series were found to be of subnormal or borderline intelligence. The achievements of those attending school were, on the whole, comparable with those of the siblings and classmates. Only 4 children were found to be severely mentally disturbed — of these 1 has greatly improved in the last 2 years — and 17 were slightly disturbed. Mental retardation was found to be more frequent in the youngest age groups (under 3 years of age) and social re-adjustment problems more frequent in the older age groups, particularly the school-age children. The latter seemed to pose the major problems in this series, especially the cases with major intellectual, sensory or motor handicaps.