The changes of BPA level in 31 cases of children with aplastic anaemia and its clinical significance

Abstract. Burst-promoting activity (BPA) was measured in the sera from 31 children with aplastic anaemia (AA). BPA levels were elevated in most of the children with A A (65-2%), the mean value (137.7 ± 18.4%) being significantly higher than that in normal children (69.6 ± 9.4%), in children in the recovery period and in children with non-aplastic anaemia. There was a negative relationship between the BPA level in children with AA and the peripheral haemoglobin concentration. The BPA level was higher in those whose duration of illness was shorter than 1 year. In three cases of AA caused by chloramphenicol and benzene hexachloride and one case of congenital pure red cell AA, the BPA level was not elevated. Eleven patients received fetal liver cell suspensions intravenously (FLI). After FLI the BPA level in their sera was significantly reduced. According to these results, it appears that the elevation of BPA level is a special phenomenon of A A. The measurement of BPA in serum is helpful for differentiation between AA and other kinds of anaemia. The elevation of the BPA level in serum is a biological compensation for the haematopoietic disorder, and the measurement of BPA in the serum of patients with A A may be helpful in evaluating the haematopoietic condition.     

Cytogenetical and clinical investigations in aplastic anaemia (Fanconi's type)

Summary Cytogenetic investigations were performed in a 10 year-old girl with clinical features of Fanconi's anaemia, i.e.: growth retardation, skin pigmentation, bilateral absence of thumbs, anaemia, leukopenia, etc. A variety of structural anomalies as chromatid gaps and exchanges, chromatid and isochromatid breaks were observed. The same type of chromosome anomalies was found in the parents and in the younger sister of the proposita, the older sister being karyotypically normal.Dermatoglyphic investigations revealed in the proposita the absence of t triradius on both palms and an increased mean ridge count, increased also in all the family members. Genetic implications and possible mechanisms of chromosomal aberrations are discussed.This investigation was supported by a grant from C.N.R., Italy.Professor of Child Health.     

呼吸道合胞病毒肺炎患儿血清NRAV水平及临床意义

探讨呼吸道合胞病毒（RSV）肺炎患儿血清中抗病毒应答负性调节因子（NRAV）的表达水平及临床意义。

选取我院收治的RSV肺炎患儿100例作为RSV组,并将RSV肺炎患儿根据肺炎严重指数（PSI）分为低危组（62例）、中危组（24例）及高危组（14例）。另选取同期于本院体检的102例健康儿童作为对照组。采用荧光定量PCR法检测血清NRAV水平,用肺功能仪检测受试儿童肺功能,采用Pearson法分析血清NRAV水平与RSV肺炎患儿肺功能的相关性;采用Logistics回归分析RSV肺炎患儿病情严重程度的影响因素;采用ROC曲线分析血清NRAV水平对RSV肺炎的诊断价值。

RSV组患儿血清NRAV水平高于对照组,第1秒用力呼气容积占预计值百分比（FEV₁%）及FEV₁/用力肺活量（FVC）均低于对照组（均P＜0.05）。不同严重程度RSV肺炎患儿血清NRAV水平、FEV₁%及FEV₁/FVC比较差异有统计学意义（均P＜0.05）。低危组、中危组、高危组RSV肺炎患儿血清NRAV水平依次升高,FEV₁%及FEV₁/FVC均依次下降,组间两两比较差异有统计学意义（均P＜0.05）。RSV肺炎患儿血清NRAV水平与FEV₁%及FEV₁/FVC均呈负相关（均P＜0.05）。Logistics分析显示,血清NRAV水平升高、FEV₁%及FEV₁/FVC水平下降均是影响RSV肺炎严重程度的独立危险因素（均P＜0.05）。ROC曲线分析显示,血清NRAV水平诊断RSV肺炎的曲线下面积为0.843,敏感度为76.0%,特异度为88.2%,最佳截断值为1.14。

RSV肺炎患儿血清NRAV水平升高且随着疾病严重程度的加重而升高,其水平可反映肺功能,对RSV肺炎具有一定诊断价值。

     

低水平乙型肝炎表面抗原的检测及其临床价值评估

我们采用具有世界卫生组织( WHO) 溯源性的标准血清建立浓度标准曲线, 评估微粒子酶免疫分析法(MEIA) 和酶联免疫吸附试验( ELISA) 的检测灵敏度, 并对97 例经确认为低水平乙型肝炎表面抗原( HBsAg)的阳性结果进行检测和比对分析; 同时综合评估低水平HBsAg 患者的临床各项指标, 探究低水平HBsAg 检测的临床意义。根据MEIA、ELISA 的S/CO 值分析, 2 种方法的检测灵敏度分别为0. 095 IU/ml 和0. 378 IU/ml;ELISA 对97 例低水平HBsAg 的检出率仅为45. 4% ( 44 /97) ; 在97 例低水平HBsAg 患者中, HBV DNA≥1 ×103 拷贝/ml 占17. 5% ( 17/97) , 肝功能异常率占38. 1% ( 37 /97) , 其中明确诊断为乙型肝炎相关疾病的19 例患者中, 肝硬化占57. 9% ( 11 /19) 。因此灵敏度高、特异性好的方法有助于低水平HBsAg 的检出, 有利于对这些人群的诊断和治疗。部分低水平HBsAg 患者往往伴随病毒复制及肝功能的慢性损伤, 预后不良, 应引起临床的高度重视和关注。     

Treatment of severe aplastic anaemia with antilymphocyte globulin or bone-marrow transplantation.

Fifty-three patients with severe aplastic anaemia were admitted to this hospital between January 1976 and June 1980, of whom three arrived in terminal condition and died before treatment for their basic disease could be given. Thus 50 patients were treated and evaluated in a prospective study according to one protocol. Eighteen patients with an HLA-identical sibling underwent bone-marrow transplantation with the aim of achieving haematopoietic chimerism. Thirty-two patients without an HLA-identical sibling were given antilymphocyte globulin with or without an infusion of HLA-haplotype-identical marrow. All these 32 patients received low-dose androgens after the procedure. In the first group eight patients (44%) survived. In the two other groups, 22 patients survived (69%), of whom 20 were completely self-sustaining (63%). Engraftment and graft-versus-host disease did not occur in the group who received antilymphocyte globulin and haploidentical marrow, and the haematopoietic reconstitutions in these patients were all autologous. These results confirm the efficacy of antilymphocyte globulin in the treatment of severe aplastic anaemia and show that such treatment is at least as good as bone-marrow transplantation. Its mechanism of action remains unknown, but most patients with aplastic anaemia have a pool of haematopoietic stem cells able to repopulate the marrow after this type of treatment.     

妊娠高血压综合征患者血清瘦素水平变化及意义

目的探讨妊娠高血压综合征(妊高征)患者血清瘦素含量的变化及其在妊高征发病中的意义。方法采用放射免疫分析法(R IA)测定50例妊高征患者(HDP组,其中妊娠期高血压组12例,轻度子痫前期组20例,重度子痫前期组18例)、50例正常晚孕妇女(对照组)患者血清瘦素。结果 HDP组的血清瘦素值(24.23±7.94)μg/L显著高于对照组(10.12±3.15)μg/L(P<0.001),以子痫前期组增高明显。结论血清瘦素水平与妊高征的发生和病情程度有关。     

326例儿童缺铁性贫血与感染幽门螺杆菌关系探究

摘要：目的 分析幽门螺杆菌（H.pylori）对儿童缺铁性贫血（IDA）的影响,探讨H.pylori感染与儿童IDA的相关性,提高儿童IDA诊治率。方法 应用13C-呼气试验和胶体金法检测326例IDA患儿及211例健康儿童H.pylori感染情况,并对其结果进行统计学分析。结果 326例IDA患儿中H.pylori检测阳性217例（阳性率为66.56%）,健康组儿童H.pylori检测阳性47例（阳性率为22.27%）,两组比较差异有统计学意义（χ2=100.54,P<0.01）。2－6岁组分别与7－9岁组及10－12岁组H.pylori阳性率比较差异均有统计学意义（χ2=8.61、21.46,P<0.05）,7－9岁组较10－12组H.pylori阳性率比较差异无统计学意义（χ2=2.62,P>0.05）。其中女患儿H.pylori阳性率为78.06%（121/155）,男患儿H.pylori阳性率为56.14%（96/171）,两者比较差异有统计学意义（χ2=17.55,P<0.01）,且不同年龄组男女阳性率比较差异均有统计学意义。217例H.pylori感染的IDA患儿以轻度贫血为主,占95.85%（208/217）。结论 H.pylori感染与儿童IDA之间存在相关性。     

326例儿童缺铁性贫血与感染幽门螺杆菌关系探究

目的分析幽门螺杆菌(H.pylori)对儿童缺铁性贫血(IDA)的影响,探讨H.pylori感染与儿童IDA的相关性,提高儿童IDA诊治率。方法应用~(13)C-呼气试验和胶体金法检测326例IDA患儿及211例健康儿童H.pylori感染情况,并对其结果进行统计学分析。结果 326例IDA患儿中H.pylori检测阳性217例(阳性率为66.56%),健康组儿童H.pylori检测阳性47例(阳性率为22.27%),两组比较差异有统计学意义(X~2=100.54,P0.01)。2-6岁组分别与7-9岁组及10-12岁组H.pylori阳性率比较差异均有统计学意义(X~2=8.61、21.46,P0.05),7-9岁组较10-12组H.pylori阳性率比较差异无统计学意义(X~2=2.62,P0.05)。其中女患儿H.pylori阳性率为78.06%(121/155),男患儿H.pylori阳性率为56.14%(96/171),两者比较差异有统计学意义(X~2=17.55,P0.01),且不同年龄组男女阳性率比较差异均有统计学意义。217例H.pylori感染的IDA患儿以轻度贫血为主,占95.85%(208/217)。结论H.pylori感染与儿童IDA之间存在相关性。     

Humoral immune response in children with iron-deficiency anaemia.

The humoral immune response (as shown by plasma immunoglobulin concentrations and antibody response to diphtheria and tetanus toxoids) was evaluated in 14 children with iron-deficiency anaemia and in 24 normal controls. Mean concentrations of haemoglobin and serum iron and mean transferrin saturation were significantly lower in children with iron-deficiency anaemia than in controls. Serum immunoglobulin concentrations were within the normal range in both groups. Two weeks after immunisation with diphtheria and tetanus toxoids the concentrations of IgG increased significantly in both groups. Antibody titres in iron-deficient children were similar to those of controls before and after immunisation. The mean T-lymphocyte count was significantly lower in iron-deficient children than that in controls, but the mean B-lymphocyte counts were similar in the two groups. These observations suggest that humoral immunity in children is not affected by iron deficiency and that conventional immunisation programmes would be effective in children with iron-deficiency anaemia.     

阿维菌素中毒患者血清C反应蛋白的浓度变化及意义

目的探讨口服阿维菌素中毒患者血清C反应蛋白浓度变化与病情严重程度及预后的关系。方法将39例口服阿维菌素中毒患者分为轻度中毒组和中重度中毒组,42例健康体检者为对照组。轻、中重度中毒组患者分别于入院时及入院第3、5天检测血清C反应蛋白浓度,并进行组间比较。结果中毒组血清CRP水平明显高于对照组,差异有统计学意义（P〈0.05）;轻度中毒组血清CRP水平在入院时及入院第3、5天变化较小,各时点血清CRP水平比较无显著统计学意义（P〉0.05）;中重度中毒组血清CRP水平在入院时即显著升高,入院第3天达最高值,各时点血清CRP水平比较差异有统计学意义（P〈0.05）。结论血清C反应蛋白浓度可作为判断口服阿维菌素中毒患者病情严重程度及预后的敏感指标。     

抽动障碍患儿血清脑源性神经营养因子及微量元素锌水平检测及临床意义

目的:检测抽动障碍(TD)患儿血清中脑源性神经营养因子及微量元素锌水平.研究其临床意义.方法:按照中国精神障碍分类与诊断标准第3版中的抽动障碍分类与诊断标准选取30名患儿,选取15名年龄、性别类同的健康儿童作为对照组.应用酶联夹心免疫吸附法测定受试者血清BDNF的水平,原子吸收法测定血锌水平,与健康儿童进行比较.结果:与对照组相比,患儿血清BDNF的水平升高,差异有统计学意义(P<0.05);两者血锌水平相当,无统计学意义(P>0.05).结论:BDNF参与抽动障碍的发病,外源性BDNF的介入可能成为干预、防治TD的有效方法.抽动障碍惠儿血锌水平无明显变化.     

Spleen and liver weight changes in NZB mice with haemolytic anaemia.

Spleen and liver weights from 998 NZB mice were subjected to computerised multivariate analysis. 3-dimensional graphs relating predicted spleen and liver weights to age and bodyweight were prepared and the relationship of the fitted surface to the histology and pathological autoantibody is discussed. On the basis of spleen weight increase, red-cell destruction throughout the greater part of life seems to be mainly intrasplenic, while intrahepatic destruction does not become important until later, when it is probably a dominant feature in the terminal period of the disease. Only limited organ weight increases occur in breeding females, and it is suggested that pregnancy lessens the effect of the haemolytic disease, the 'protective' effect extending long after the cessation of breeding.     

Meckel's diverticulum: its clinical significance; a report of 54 cases

Of 54 cases of Meckel's diverticulum observed at one hospital in a 20-year period, 27 were noted incidentally and 27 caused symptoms. In the latter group, the patients were preponderantly males. The most common symptoms, in order of frequency, were hemorrhage from the bowel, intestinal obstruction, acute diverticulitis, and intussusception. All the complications except intestinal obstruction occurred in infancy and childhood more often than in later life. Intestinal obstruction was limited to adults. There was strong coincidence of complications and ectopic tissue. Surgical excision was done in all cases of diverticulum causing symptoms, and all patients recovered.     

High dosage desferrioxamine therapy in a female patient with acquired aplastic anaemia and transfusion siderosis

A 32 year old woman with severe aplastic anaemia required frequent transfusions and consequently developed hyperferrioxaemia (54 microMol/l) and hyperferritinaemia (1,700 ng/ml). For the treatment of transfusion siderosis she was given 18 high dose courses each comprising 35 g of desferrioxamine. Because of pre-existing thrombocytopenia (platelet count 5 X 10(9)/l) the iron chelating agent was given by continuous intravenous infusion over 3 1/2 days. High dose desferrioxamine had to be abandoned because of severe bone pain. The desferrioxamine infusions achieved a negative iron balance, iron loss after each infusion being 100 to 200 mg in the urine and 400 mg in the faeces. Serum iron and ferritin concentrations fell almost to normal. This report shows that faecal iron excretion must be taken into account in assessing the balance of iron input and output during desferrioxamine treatment.