小儿骨髓异常增生综合征的治疗

我们对１２例骨髓异常增生综合征患儿进行了分型治疗。９例ＲＡＥＢ型以小剂量Ａｒａ－ｃｉｃｕｂｏ ｙ ，１０－３０ｍｇ／ｄ，肌注或静滴，１个疗程。     

儿童慢性再生障碍性贫血与骨髓异常增生综合征的临床特点比较

目的：对儿童慢性再生障碍性贫血（CAA）与骨髓异常增生综合征（MDS）的临床特点进行比较分析,探讨两种疾病之间的差异,帮助临床鉴别诊断。方法：回顾性分析2007年9月至2010年9月收治的23例CAA患儿和9例MDS患儿的临床资料,对所有病例的血常规、网织红细胞计数、乳酸脱氢酶、血清铁蛋白等水平及骨髓细胞学检查、骨髓CD34+计数、骨髓染色体检查、骨髓荧光原位杂交（FISH）检查结果进行对比分析。结果：MDS组中性粒细胞、网织红细胞、血清铁蛋白、乳酸脱氢酶水平均高于CAA组;MDS组骨髓原始细胞计数及三系血细胞的病态造血现象与CAA组差异有显著性;MDS组骨髓CD34+细胞计数水平较CAA组高,FISH异常的检出率亦较CAA组高。结论：CAA和MDS患儿实验室检查、骨髓细胞形态学、细胞遗传学检查结果存在显著不同,三者联合检测对鉴别CAA与MDS有重要意义。     

18例小儿骨髓增生异常综合征治疗探讨

骨髓增生异常综合征(MDS),是造血干细胞异常的克隆性疾病。儿童发病率为3.4/10万,目前尚无特效疗法。我科对18例MDS患儿,根据临床不同分型采取不同方案治疗,现报道如下。 资料与结果 一、临床资料 MDS患儿18例,女2例,男16例;年龄8月～16岁,平均6.3岁。病人均系原发型初治MDS患儿,经临床、血象、骨髓涂片、骨髓染色体检查及祖细胞培养确诊。FAB分型如下,难治型贫血(RA)12例,难治型贫血伴原始细胞增多(RAEB)2例,转化中的难治型贫血伴原始细胞增多(RAEB-T)2例,慢性粒单核细胞白血病(CMML)2例。     

骨髓增生异常综合征诊断治疗探讨──附25例报告

本文对25例骨髓异常增生综合征（MDS）的诊断治疗探讨。均具有中度贫血，发热，出血症状及浅表淋巴结轻──中度肿大，肝脾轻──中度种中，外周血象血红蛋白平均50g／L，＜90g／L者占89％；红细胞增均1．78×10(12)／L，白细胞＜4×109／L者占52．3％，血小板平均62×109／L，骨髓表现增生活跃或明显活跃者占88％，增生减低者占12％，粒：红≤1者占65％，红系增生活跃占68％，减低占32％，并且大多数表现为成熟红细胞大小不等。巨幼样变及核的畸形，粒系统增生活跃者28％，减低者72％，多表现为核的不规则，核浆发育不平衡，多见双核粒细胞，巨核系统大部分受抑制，出现小淋巴样巨核细胞，部分表现为巨核细胞，血小板罕见。其中，难治性贫血（RA）16例；环状铁粒幼细胞性贫血（RA—S）1例；原始细胞过多难治性贫血（RAEB）6例；转化中难治性贫血伴有原始细胞增多（RAEB-T）1例；急性淋巴细胞性白血病（ALL）1例。提示：本组病例骨髓原始淋巴细胞增多占相当比例，MDS一旦转化为急性白血病，则病情迅速恶化，存活期明显缩短。我们体会到MDS应与再障相鉴别：其关键是MDS具有各种代表DNA复制紊乱的现象和骨髓中原始细胞增多之特点而再障则不具备。     

小儿骨髓增生异常综合征诊疗建议

小儿骨髓增生异常综合征（ＭＤＳ）诊疗建议自１９５年于中国实用儿科杂志发表以来,经几年的实践,对本病的基础和临床研究均取得了新的进展,此次经全国小儿血液病学术会议讨论,再次修订如下。一、诊断（一）ＭＤＳ诊断标准：１临床表现以贫血症状为主,可兼有发热、...     

小儿骨髓增生异常综合征

小儿骨髓增生异常综合征现已取代了“小儿白血病前期”的命名。本病小儿罕见。其诊断在于在外周血和骨髓发现异常的血细胞以及其他的临床异常,且分为个亚型。本症染色体核型异常的发生率高达40～60%。本文对本症的骨髓细胞遗传学、体外培养及治疗做了较为详细的介绍。     

联合促分化治疗小儿骨髓增生异常综合征10例

用全反式维甲酸（ATRA）、维生素D化合物、干扰素（α－IFN）和小剂量阿糖胞苷（Ara－c）联合治疗小儿骨髓增生异常综合征（MDS）10例,总有效率为50％,结果表明,联合促分化治疗较单药治疗效果好,且毒副作用小,患儿能够耐受。     

小儿骨髓增生异常综合征的临床研究

目的 探讨骨髓增生异常综合征（ＭＤＳ）的临床特点、诊断临床特点、诊断及鉴别诊断。方法全部患者儿均检测常规血液学及骨髓细胞形态学指标,部分患儿行骨髓病理、免疫组化、干细胞培养及染然体核型检查。并以不同方案进行治疗和追踪。以ＳＰＳＳ软件行统计学处理。结果 ３８例中难治性贫血（ＲＡ）占５８％,难治性贫血伴环形铁粒幼红细胞（ＲＡＳ）增多,占３％,难治性贫血伴原始细胞增多（ＲＡＥＢ）占２６％,转变中的ＲＡＥ     

THE EFFECTS OF PHARMACOLOGICAL TREATMENT ON PULMONARY FUNCTION IN CHILDREN WITH EXERCISE-INDUCED ASTHMA

Abstract. Bolme, P., Eriksson, M., Freyschuss, U. and Winbladh, B. (Department of Paediatrics, Karolinska Institute, St. Göran's Children's Hospital, Stockholm, Sweden). The effects of pharmacological treatment of pulmonary function in children with exercise-induced asthma. Acta Paediatr Scand, 69: 165, 1980.—The effects of two therapeutic regimens were investigated in eleven boys and eight girls (8–13 years) with a history of exercise-induced asthma (EIA), who reacted with subjective signs of EIA and a decrease in FEY_1.0 exceeding 10% after an exercise test. A test program (TP) including complete spirometry, bicycle ergometer and treadmill exercise tests preceded and followed by dynamic spirometry at one and five min after exercise, was performed before and after each treatment period. In 12 patients with sporadic medication before the trials, continuous peroral treatment with a combination of a ß₂-stimulating drug and a xanthine derivative for three weeks did not significantly improve TP data. In 13 children (six from the above group) who were already on continuous treatment as above, addition of disodium cromoglycate (DSCG) inhalations for 3–4 weeks improved the response to acute administration of a β -receptor stimulatory aerosol but did not influence EIA. Seven of the children continued their DSCG treatment for one year. Minor improvement of EIA provoked by cycling but not by treadmill was seen after this. The ventilatory effort in relation to working intensity was lowered. No significant differences were found between treadmill running and cycling in provoking asthma.     

CYTOTOXIC TREATMENT IN CHILDREN WITH IDIOPATHIC NEPHROTIC SYNDROME

Abstract. Herin, P. and Eriksson, M. (Department of Paediatrics, Karolinska Institutet, St. Göran's Children's Hospital, Stockholm, Sweden). Cytotoxic treatment in children with idiopathic nephrotic syndrome. Acta Paediatr Scand, 69:315, 1980.—The purpose of this retrospective study was to investigate the therapeutic value of steroids and cytotoxics in children with idiopathic nephrotic syndrome which developed during the period 1968–1977. Thirty-eight patients were followed. They were divided into three morphological groups based on renal biopsy findings, i.e., minimal changes (MCNS, n=34), focal segmental glomerulosclerosis (FSGS, n=2) and atypical undefined changes (Undef., n=2). In the latter two groups therapy was less successful than in the MCNS-group. This study conclusively demonstrates that cytotoxic therapy is of value in prolonging the duration of the remission and increasing the responsiveness to steroids in children with MCNS.     

PARVOVIRUS B19 INFECTION REMINISCENT OF MYELODYSPLASTIC SYNDROME IN THREE CHILDREN WITH CHRONIC HEMOLYTIC ANEMIA

The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.     

CEREBELLAR-ATAXIC SYNDROME IN CHILDREN AND ADOLESCENTS WITH HYPOTHYROIDISM UNDER TREATMENT

ABSTRACT. Animal experiments and observations on quantitative growth of human cerebellum suggest a critical period when its development is particularly vulnerable to hypothyroidism. Sixty-seven patients aged 7–24 years with hypothyroidism under long-term treatment were examined for ataxic symptoms. These were found in 24 of 39 patients (60%) hypothyroid before or during the third month of life. Only 4 of 18 patients (20 %) hypothyroid later had cerebellar symptoms. Such symptoms could be evidence for the onset of hypothyroidism before or during the 3rd month of life. Seventeen (80%) of mentally retarded patients had cerebellar symptoms compared with 11 (30%) of 45 attending normal school. Even retrospecitvely, these data might permit a more accurate prognosis of further mental development in hypothyroid children.     

A DOUBLE-BLIND DRUG TRIAL OF TREATMENT IN YOUNG CHILDREN WITH WAKING PROBLEMS

A double-blind trial using trimeprazine tartrate was carried out in 22 children with severe waking problems. On parental verbal reports sleep was significantly improved on the drug compared with the original baseline and the placebo, but diaries kept by the parents showed that this improvement was clinically only moderate, with many wakeful nights still occurring. Taking the drug produced no permanent effect on sleep patterns and a follow-up of 14 children 6 months later showed persisting sleep problems in the majority.     

MVPP CHEMOTHERAPY COMBINED WITH RADIOTHERAPY IN THE TREATMENT OF HODGKIN'S DISEASE IN CHILDREN

ABSTRACT. Thirty-four children with Hodgkin's disease were treated during the years 1969–75. After radiotherapy, 7–15 cycles of MVPP were given within 24–53 months. In order to avoid chronic leukopenia, leukocyte counts were made frequently during chemotherapy, and the drug doses adjusted accordingly. A complete remission was obtained in 32 of the 34 children. Two patients died because of progressive disease. Twelve of the 32 survivors have been followed for at least 5 years, and a further 12 for at least 3 years. Three children are still on chemotherapy, whereas the remaining 29 being followed are in continued complete remission.