共查询到20条相似文献,搜索用时 62 毫秒
1.
2.
3.
幼年型特发性关节炎尤其是少关节炎型主要以骨关节疼痛为主要表现,有的局限于某个大关节,有的可表现为多关节疼痛、肿胀,甚至存在关节腔积液,临床上常常以此作为诊断该病的主要标准而开始治疗。我们将近几年在外院诊断为幼年型特发性关节炎,并进行相关治疗,由于疗效不佳转来本院,在本院经反复检查最后确诊为急性白血病的7例病例总结分析如下。 相似文献
4.
5.
关节疼痛在小儿急性白血病的特点──附29例临床分析 总被引:1,自引:0,他引:1
目的:探讨关节疼痛在小儿急性白血病(AL)的表现特点,减少临床误诊。方法:分析近13年来在我院确诊以关节疼痛为特点的急性白血病的临床表现、伴随症状、实验室检查及误诊情况。结果:29例患儿中27例为急性淋巴细胞性白血病(ALL),2例为急性非淋巴细胞性白血病(ANLL)。关节疼痛具有:多发生在大关节,游走性,局部无红热等特点;常伴随发热、贫血、肝脾肿大等。ESR、CRP可明显升高;6例早期外周血无幼稚细胞。误诊11例,主要误诊为风温热式风湿性关节炎。结论:小儿AL的关节疼痛以ALL为常见,具有以大关节游走性无红热等特点。重视小儿AL中关节疼痛的特点可减少误诊。 相似文献
6.
治疗相关白血病1例报告 总被引:1,自引:0,他引:1
目的探讨急性淋巴细胞白血病患者长期化疗后发生治疗相关骨髓增生异常综合征白血病(t-MDS/AML)的临床特征及预后。方法观察一例儿童急性淋巴细胞白血病经化疗后演变为幼年慢性粒-单核细胞白血病(JMML)的临床演变过程及治疗结果并进行相关文献复习。结果1例急性淋巴细胞白血病患儿经3年正规化疗停药后3月,外周血白细胞进行性增高并出现原始粒、单核细胞。各项检查支持幼年慢性粒-单核细胞白血病诊断。根据2000年WHO造血组织和淋巴组织肿瘤分类方法,归于MDS/MPD一类疾病。患者迅速进展为AML-M4b,治疗无效死亡。结论t-MDS比原发性MDS病情进展更快,预后更差。t-AML临床预后不良,骨髓移植及大剂量化疗仅对部分患者有效。应进一步加强儿童ALL患者的合理用药,根据不同危险度分型,选取相应治疗强度化疗方案,以减少t-MDS/AML发生的可能。 相似文献
7.
目的 探讨重组人型肿瘤坏死因子受体抗体融合蛋白(益赛普)治疗幼年特发性关节炎的临床疗效.方法 45例幼年特发性关节炎患儿分为试验组和对照组.试验组使用益赛普12.5 mg皮下注射,每周2次,治疗8周;对照组使用常规抗风湿药物.观察患儿关节压痛、肿胀等临床症状的改善情况,并检测红细胞沉降率和C反应蛋白.结果 试验组益赛普起效迅速,与对照组比较有明显效果(P<0.05),试验组治疗后红细胞沉降率、C反应蛋白明显下降(P<0.05).结论 益赛普治疗幼年特发性关节炎短期疗效非常明显,可作为治疗难治性幼年特发性关节炎诱导缓解的首选药物. 相似文献
8.
益赛普治疗幼年特发性关节炎的临床研究 总被引:1,自引:0,他引:1
目的 探讨重组人型肿瘤坏死因子受体抗体融合蛋白(益赛普)治疗幼年特发性关节炎的临床疗效.方法 45例幼年特发性关节炎患儿分为试验组和对照组.试验组使用益赛普12.5 mg皮下注射,每周2次,治疗8周;对照组使用常规抗风湿药物.观察患儿关节压痛、肿胀等临床症状的改善情况,并检测红细胞沉降率和C反应蛋白.结果 试验组益赛普起效迅速,与对照组比较有明显效果(P<0.05),试验组治疗后红细胞沉降率、C反应蛋白明显下降(P<0.05).结论 益赛普治疗幼年特发性关节炎短期疗效非常明显,可作为治疗难治性幼年特发性关节炎诱导缓解的首选药物. 相似文献
9.
目的探讨急性淋巴细胞白血病患儿化疗后可逆性脑病的临床和影像学特点。方法回顾分析2015年9月1日至2018年9月1日住院时发生脑病的急性淋巴细胞白血病患儿的临床资料。结果研究期间共收治新发急性淋巴细胞白血病582例,9例患儿发生10次可逆性脑病(1例患儿发生2次),其中男6例、女3例,脑病发生中位年龄6.55岁(3.9~12.5岁)。最常见的神经系统临床表现是抽搐,其次是肌无力和感觉异常。7例患儿曾接受培门冬治疗;5例患儿在脑病发生前有急性高血压病史;6例患儿在脑病发生时有低钠血症,部分有低纤维蛋白原血症。头颅磁共振成像检查均提示T1和T2信号异常,累及部位多见于顶枕叶。结论联合化疗、化疗药物鞘内注射和急性高血压是可逆性脑病发生的高危因素;监测血压、血钠、纤维蛋白原,以及头颅磁共振成像检查有助于早期发现可逆性脑病。 相似文献
10.
11.
Prof. Ulrich Wahn 《Pediatric allergy and immunology》1998,9(3):116-124
There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis. 相似文献
12.
OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (= 18 months) and those aged 16-20 years. The majority was unvaccinated (58/87). A proportion (11/87) demonstrated vaccine failure, most likely primary failures. CONCLUSION: This changing measles epidemiology suggests lowering of herd immunity. 'Catch up' vaccinations in July- October 1997 given to school children aged 12-18 years (200 000 individuals or 82% of cohort), may have helped contain the outbreak. These results substantiated the need for a two-dose policy and 'catch-up' immunization program. 相似文献
13.
金宇 《中国实用儿科杂志》2012,27(6):412-415
孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%~5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相 相似文献
14.
EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE 总被引:1,自引:0,他引:1
Vipul N. Mankad 《Fetal and pediatric pathology》2001,20(1):1-13
During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes. 相似文献
15.
Takechi T Maeda A Hisakawa H Wakiguchi H Takeuchi T Sonobe H Ohtsuki Y 《Pediatric hematology and oncology》2002,19(7):459-465
A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I. 相似文献
16.
LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER 总被引:2,自引:0,他引:2
Shao-Ming Wu Ellen Werber Leschek Owen M. Rennert Wai-Yee Chan 《Fetal and pediatric pathology》2000,19(1):21-40
Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea. 相似文献
17.
18.
This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions. 相似文献
19.
E Olafsdottir B Ellertsen A Berstad G Fluge 《Acta paediatrica (Oslo, Norway : 1992)》2001,90(6):632-637
The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children. 相似文献
20.
Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.
Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft. 相似文献
Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft. 相似文献