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目的探讨肿瘤患儿化疗相关感染早期临床表现与感染严重程度的关系,寻找早期提示重症感染的线索。方法 2005年10月至2009年1月在中山大学附属第一医院儿科化疗并发生化疗相关感染的肿瘤患儿136例,共发生感染346例次,比较重症感染与轻症感染患儿早期临床表现的差异。结果重症感染133例次,轻症感染213例次。重症感染患儿早期伴畏寒、寒战、精神欠佳、末梢循环不良、粒细胞缺乏的患儿明显多于轻症感染患儿,且热峰及发热6～12hC-反应蛋白(CRP)值均较轻症感染患儿高;接受强化疗患儿中,感染早期伴畏寒、寒战、精神欠佳、末梢循环不良、中性粒细胞计数≤0.5×109/L、CRP≥40mg/L、热峰≥39.5℃的患儿发生重症感染的机会大于无上述表现者,差异有统计学意义(P<0.05)。结论接受强化疗及感染早期伴畏寒、寒战、精神欠佳、末梢循环不良、中性粒细胞计数≤0.5×109/L、CRP≥40mg/L、热峰≥39.5℃患儿易发展为重症感染。     

Reduced vascular reactivity in diabetic children and its relation to diabetic control

The vascular reactivity in response to ischaemia was studied by a non-invasive method of measuring transcutaneous oxygen tension used at 37 degrees C, in 28 children with type I diabetes mellitus. Postischaemic hyperaemia was significantly reduced in the diabetic children compared with 34 healthy children. The degree of reduction in vascular reactivity correlated to both short-term and long-term diabetic control. Among tested variables, urinary glucose excretion during the night preceding the test showed the highest coefficient of correlation to the peak of the postocclusive reactive hyperaemia (r = -0.59, p less than 0.01). This peak was only weakly correlated to triglycerides and glycosylated haemoglobins and showed no significant correlation to fasting plasma glucose or to the duration of the disease. It was weakly correlated to the total daily insulin dosage but not to the dosage of insulin/kg body weight. Multiple linear regression analysis revealed that urinary glucose excretion, plasma glucose, haemoglobin A1, serum triglyceride and serum cholesterol, duration of diabetes, and insulin dosage per kg body weight together explained 54% of the variation in vascular reactivity. The reduced vascular reactivity found in diabetic children could not be explained by the usual parameters of carbohydrate control alone. This new non-invasive method is able to reveal a reduction in vascular function in diabetic children and should therefore be of value in attempts to find the causes of vascular dysfunction in diabetics.     

Plasma amino acids in relation to metabolic control in insulin-dependent diabetic children

The influence of metabolic control (estimated by blood glucose, 3-hydroxybutyrate and glycosylated hemoglobin levels) on plasma amino acids was determined in a group of 56 insulin-dependent diabetic children. A multiple correlation analysis revealed significant positive partial correlations between most amino acids and blood glucose. Alanine, methionine, tyrosine, phenylalanine and arginine showed negative partial correlations to the 3-hydroxybutyrate level. The results are consistent with the postulate that ketone body inhibition of muscle proteolysis is one of the factors regulating substrate flows during insulin deficiency.     

Borrelia arthritis in Swedish children: clinical manifestations in 10 children

Since 1986, serology against Borrelia burgdorferi has been performed on patients with arthritis admitted to the Division of Pediatric Rheumatology, S:t Göran's Hospital, Stockholm. We studied, retrospectively, the period 1986–88. Among 300 children with arthritis, 10 had positive titers against B. B. burgdorferi. Other causes of arthritis were excluded. The onset of Borrelia arthritis was throughout the year. Only 3 of the 10 children remembered an actual tick bite. No other manifestations of Lyme borreliosis were present simultaneously. The typical clinical picture was a relapsing unilateral arthritis of the knee.     

Abnormalities of in vitro lymphocyte response to mitogens in diabetic children during acute ketoacidosis.

Cell-mediated immunity was evaluated in 11 children with diabetes mellitus; six children were evaluated during ketoacidosis and five were evaluated with ketonuria in the absence of acidosis. Five of the six ketoacidotic children had at least one positive delayed-hypersensitivity skin test. Lymphocytes from two ketoacidotic patients were unresponsive to phytohemagglutinin and pokeweed mitogen, and lymphocytes from these two patients plus a third patient were unresponsive to concanavalin A. Lymphocytes from all six patients responded to these three mitogens after one week of therapy. In the five diabetic children without ketoacidosis, lymphocyte responses were normal to all three mitogens. Similarly, the addition of glucose to normal plasma did not alter the lymphocyte transformations of three healthy nondiabetic controls. These data suggest that cell-mediated immunity may be transiently defective in children with acute diabetic ketoacidosis.     

The prevalence of microalbuminuria in diabetic children and adolescents and its relation to puberty

The albumin excretion rate (AER) was studied in two groups of diabetic children and adolescents. Twenty-four-hour AER was studied in 75 children with diabetes for 5 years, in 49 children with diabetes for 10 years, in 55 children with diabetes for 10-20 years and in 21 age matched healthy controls. Overnight AER was studied in 129 diabetic children and adolescents with a duration of diabetes varying from 1-14 years. Diabetics exhibited a wide range of AER-values and when expressed per body surface area, diabetic children had significantly higher AER compared to controls. Log transformed AER-values were significantly correlated to age and body surface area in diabetics but not in controls. In the diabetics, log AER was also correlated to systolic and diastolic blood pressure but not to HbA1c. 20% of the diabetics had AER values exceeding the upper value for healthy controls which was 18.5 micrograms/min. 31/35 of them were older than 12 years. In both groups of diabetics, 5% had AER-values exceeding those reported to be predictive for later development of overt nephropathy, the youngest being 16 years old. When comparing diabetic children 0-12 years (i.e. before the maximal growth spurt of puberty) to those older than 12 years, at the same duration of diabetes, the latter group had significantly higher AER-values. No sex difference was found in either age group. It is concluded that after puberty diabetic patients also show evidence of incipient diabetic nephropathy. Thus, routine screening for microalbuminuria is recommended also in pediatric diabetes care after 12 years of age.     

HIV infection in hemophiliac children: clinical manifestations and therapy

The effects of human immunodeficiency virus (HIV) disease, with its progressive and multifaceted manifestations, have not yet been fully expressed in the hemophilic population. However, even at this phase of the disease, the effects of the viral illness on the growing hemophilic child are extremely complex and have only recently been recognized and studied. The management of these unfortunate children is difficult and demands understanding and insight to obtain maximum benefit from the limited therapeutic armamentarium at hand. This unsatisfactory situation will continue at least for the near future, requiring innovative research and meticulous medical care in order to help those affected. This review addresses current knowledge pertaining to hemophilic children infected with HIV-1 and covers information available on the epidemiology, clinical manifestations, and management of HIV and its complications.     

Age related clinical manifestations of HIV infection in Indian children

AIM: To determine the various clinical manifestations of HIV infection in children as per the age. SETTING: Pediatric and Perinatal HIV clinic in a tertiary pediatric hospital over a period of 7 years.Study design: Retrospective and prospective analysis. METHODS AND MATERIALS: Clinical manifestations of 317 HIV infected children were noted and commonest clinical symptoms were determined. The various clinical manifestations as per age were analysed. RESULTS: The mean age of presentation of HIV infection was 4.5+/-2.9 years. Predominant mode of transmission was vertical (83 per cent). Most of the children (75 per cent) were in Class B or C on presentation. There was no significant difference in the clinical symptoms and mode of transmission of HIV. The predominant clinical features seen were hepato-splenomegaly (51.1 per cent), lymphadenopathy (48.6 per cent) and tuberculosis (43.4 per cent). Patients with vertical transmission presented at an early mean age of 4.1+/-2.7 years as compared to those who acquired it by other means, which was statistically significant. PCP pneumonia was the earliest manifestation in toddlers and hepatosplenomegaly, lymphadenopathy, chronic diarrhea was seen in pre-schoolers. Systemic organ dysfunction due to HIV was seen in older children. CONCLUSIONS: Hepatosplenomegaly, lymphadenopathy and opportunistic infections together in a child may be suggestive of HIV infection. High suspicion and early diagnosis will lead to early management and decrease in the incidence of HIV related morbidity.     

Multiple hymenoptera stings in children: clinical and laboratory manifestations

Multiple hymenoptera stings are a cause of rhabdomyolysis, elevated liver enzymes, clotting abnormalities, kidney injury, and even death. However, the progression of the clinical and laboratory findings has been described mainly in sporadic case reports. We report the clinical and laboratory manifestations of multiple hymenoptera stings in six children who were hospitalized and referred for a nephrology evaluation and follow-up over a 12-year period. One patient succumbed 13 h after the stinging accident. In the five surviving patients, we found somewhat similar pattern of clinical and laboratory course: rhabdomyolysis with elevated liver enzymes are the earliest manifestations, followed by kidney injury and anemia. An asymptomatic phase of several days between the stinging accident and severe kidney injury can occur. There was a strong seasonal association, with all six events occurring in August or September. In children with multiple hymenoptera stings, a somewhat predictable clinical and laboratory course is expected and an initial laboratory evaluation is needed, and even in asymptomatic children, a repeated laboratory evaluation is highly recommended.     

无脑回畸形患儿的临床表现及基因分析

目的探讨无脑回畸形患儿的临床特征及其致病基因。方法回顾分析2例无脑回畸形患儿的临床表现、实验室检查及基因检测结果,并复习相关文献。结果 2例无脑回畸形患儿均为男性,分别为7个月、3岁4个月。均以癫痫发作入院,发作时意识丧失。实验室检查无明显异常;脑电图均提示有癫痫波;头部MRI均提示无脑回畸形。基因分析发现,1例患儿的PAFAH1B1基因存在IVS3-1GA杂合突变,功能分析证实该位点突变导致m RNA水平外显子4缺失,患儿父母均未见突变;另1例患儿PAFAH1B1基因存在c.274 AG突变(p.K 92 E),其父母未见异常,该突变未见报道。结论无脑回畸形患儿可能合并癫痫发生,PAFAH1B1基因突变是导致无脑回畸形的常见原因。     

Relationship of maternal autoimmune response to clinical manifestations in children with congenital complete heart block

OBJECTIVE: To study the autoimmune response in mothers of children with congenital heart block (CHB) diagnosed at different ages and with different clinical manifestations. PATIENTS AND METHODS: Clinical data and sera for the determination of immunological tests were available from 104 mothers of 113 children born between 1950 and 2000 and diagnosed with CHB before the age of 16 y. Prenatal diagnosis was performed in 74 (65%) children of 65 mothers, and 39 (35%) children had postnatal diagnosis of CHB. Maternal antibodies to 52 kd and 60 kd SS-A, and to 48 kd SS-B were determined by time-resolved fluoroimmunoassay (TR-FIA) and to antinuclear antibodies (ANA) by immunoflurescense (IF). RESULTS: Out of the 65 mothers of children with in utero diagnosed CHB, 88% had antibodies to 52 kd SS-A and 83% had ANA. Antibodies to 60 kd SS-A and 48 kd SS-B were less frequently present, in 48% and in 54% of the mothers, respectively. Seven (11%) of the mothers were negative by all immunoassays. Of the 13 mothers of children with in-infancy diagnosed CHB, one mother had high-titer ANA. After 1 y of age, CHB was diagnosed in 26 children; at 1 to 6 y in 16 and after 7 y in 10 children; 1/16 and 1/10 patients had positive antibodies. In all twin pregnancies (n = 4) and in all families with recurring cases of CHB (n = 5), maternal antibodies were positive in at least one assay. The titer of 48 kd anti-SS-B antibodies was significantly higher in children with cutaneous neonatal lupus (98.1 vs 41.0; p = 0.02). All mothers whose children died before the age of 4 y (n = 8) and 85% (11/13) of mothers whose children developed cardiomyopathy had elevated antibody titers in at least one assay. However, we could not find any prognostic value of maternal antibody levels or specificities on the clinical outcome of the children with CHB. CONCLUSIONS: Although rare, late detection or postnatal progression of CHB in antibody-mediated CHB should be taken into consideration. Maternal antibody levels or specificities have prognostic effect neither on the clinical outcome of the child with CHB nor on the risk of reappearance in the same family.     

婴幼儿和儿童食物过敏的发病机理及临床表现

食物变态反应 (ｆｏｏｄａｌｌｅｒｇｙ) ,一般称食物过敏反应 ,即人体对食物抗原产生的超敏反应。近年来 ,过敏性疾病在全世界范围内呈上升趋势 ,食物过敏作为临床最早表现出来的过敏反应症状 ,在婴幼儿发病率高于成人。食物过敏为食物不良反应的一种。但无论是食物过敏反应还是其他原因引起的食物不良反应 ,在临床表现上难以区别 ,所以有时会出现一些概念混乱或用词不当。 1995年欧洲变态反应和临床免疫学会建议根据这些疾病的发病机制来分类 ,将有关名词统一起来[1] 。一、分类食物不良反应是指食物或食物添加剂引起的一切异常反应。分为…     

儿童慢性肾衰竭的早期临床表现及诊断

慢性肾衰竭(CRF)是指由多种肾脏疾患引起的慢的肾功能减退,也是儿科肾脏疾病中的危重表现。目前其发生率尚无确切的资料报告,中华儿科学会肾脏学组(儿肾学组)调查1990年1月至2002年12月期间我国91家医院确诊的CRF共1658例,占因泌尿系统疾病住院患儿的1.31%。Hottori等报告儿童终末期肾病(ESRD)的发病率为22/百万,且每年新增病例4/百万。可见CRF在儿童泌尿系统疾病中占有相当的比例,不可忽视。1儿童CRF主要临床状由于儿童肾脏功能有较强的代偿能力,儿童CRF起病隐匿,除原发疾病外多数以肾外某个系统首发症状而就诊,如果缺乏对CRF的…     

Idiopathic hypercalciuria in children. Classification, clinical manifestations and outcome

Between 1981 and 1983, 49 children aged 2 to 15 years were diagnosed as having idiopathic hypercalciuria (IH). They were divided into 3 groups based on their response to dietary manipulation: group I (32/49) had absorptive hypercalciuria; group II (8/49) had renal hypercalciuria and group III (6/49) had sodium-dependent hypercalciuria. Response to diet was more reliable than Pak's test in differentiating between the three groups. A control group (CG) of 45 healthy, age matched children determined baseline levels for all metabolic parameters. At the time of presentation IH children did not differ from the CG in height or weight. Fifty percent of IH children had first degree relatives with urolithiasis. Yet, only 16% of the IH children had urolithiasis, the majority presenting with gross hematuria and urinary tract infections (UTI). With few exceptions the clinical symptoms resolved when urine calcium excretion was controlled. Severe calcium restriction in a few patients produced osteoporosis and delayed bone age although growth velocity was unaffected. Thiazide therapy in a few patients produced some metabolic derangements. The authors conclude that IH in childhood is a benign disease which may present with UTI or hematuria. They further propose a new classification method based on response to dietary manipulation.