早期急性白血病与类风湿性关节炎的鉴别诊断探讨

骨关节肌肉症状是许多小儿急性白血病（ＡＬ）的早期表现，有可能误诊为类风湿性关节炎（ＲＡ）。本文比较了误诊为ＲＡ的ＡＬ患儿与ＲＡ患儿的早期和确诊时的临床和实验室表现，发现ＡＬ组骨关节肌肉疼痛剧烈，关节不中轻微肿大，常为短暂性，胸骨压痛常见，ＲＡ组关节固定性持续肿大，单纯胸骨压痛罕见。ＡＬ组Ｈｂ的下降速度快，幅度大也与ＲＡ组有别；血小板减少和持续下降有利于ＡＬ的诊断，ＲＡ组则正常和增加而无下降。强调有     

关节疼痛在小儿急性白血病的特点──附29例临床分析

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。     

儿童急性淋巴细胞白血病和幼年特发性关节炎的初步鉴别

部分急性淋巴细胞白血病患儿以骨骼肌肉表现为首发症状，这些患儿中有一部分会被误诊为幼年特发性关节炎，如何早期区分这些患儿对于及时治疗、改善预后很有意义。该文根据病史及常规的实验室检查和影像学检查，提出在疾病早期如何通过分析有关节症状患儿关节肿痛、血象及影像学特点初步鉴别儿童急性淋巴细胞白血病和幼年特发性关节炎，降低误诊率。     

急性白血病并弥散性血管内凝血患儿凝血与纤溶功能变化的意义

目的观察急性白血病（AL）并弥散性血管内凝血（DIC）患儿抗凝血酶活性（AT）、D-二聚体等的变化及其关系，以探讨患儿凝血、纤溶功能变化。方法选择AL未并DIC患儿27例（AL组），AL并DIC患儿25例（观察组），健康儿童36例作为健康对照组。均抽取其清晨空腹静脉血1．8mL，离心分离血清。应用发色底物法、免疫学胶乳比浊法及凝固法分别检测AT、D-二聚体、纤维蛋白原水平、活化部分凝血活酶时间、凝血酶原时间。采用SPSS 10．0软件将AL组和观察组结果与健康对照组进行统计学比较。结果AL组患儿PT延长、D-二聚体水平明显升高，与健康对照组比较，差异均有显著性意义（Pa〈0．01）；观察组D-二聚体水平明显增高，AT明显减低，与健康对照组和AL组比较，差异均有显著性意义（Pa〈0．01）；AL组各项检测指标阳性率较低（18．5％-66．7％），观察组D-二聚体、AT阳性率明显升高，且在DIC发病的不同临床阶段均保持在较高水平（80％-100％），D-二聚体检测阳性率在DIC早期高达100％。结论AL并DIC患儿存在凝血、纤溶系统的激活，各凝血指标特别是AT和D-二聚体的检测可作为DIC诊断和预后评估的指标之一。     

以肝、神经系统外表现为首发症状的肝豆状核变性25例临床分析

为研究肝豆状核变性的肝、神经系统外表现，对25例以肝、神经系统外表现为首发症状的肝豆状核变性（HLD）患儿进行临床分析。结果发现以肾脏损害为首发症状者15例，以溶血性贫血为首发症状者7例，以骨关节症状为首发症状者3例；确诊时伴有肝脏肿大21例，肝功能异常13例，脾脏肿大7例，有神经或精神改变4例；88％患儿病初误诊为肾脏疾病、溶血性贫血、类风湿性关节炎等。提示对原因不明的肾脏损害、溶血性贫血、骨关节症状，同时伴有肝脏肿大和／或肝功能异常的患儿，需考虑HLD的可能。     

先天性无痛症并骨关节病理损害6例

目的探讨儿童先天性无痛症并发骨关节病理损害的发病机制、临床诊断及外科治疗。方法本组6例患儿以不同程度的骨关节病理损害而入院。年龄3～12岁。股骨远端、胫骨近端各2例,髋关节、足部各1例。其中3例在入院前误诊为骨肉瘤。本组患儿根据病史、临床表现及X线表现确诊。治疗主要采用局部制动及抗炎治疗,同时指导家长做好护理。结果6例患儿均随访2～6个月。2例患儿出院后因看护不当而过早下床,关节损害加重,甚至出现局部感染、关节脱位。另外4例患儿经石膏制动至少观察2个月,关节损害处均有不同程度修复。结论明确先天性无痛症并骨关节病理损害诊断是关键,只要耐心仔细询问病史,认真观察和分析患儿的临床表现及X线表现,是不难作出正确诊断的。     

急性白血病患儿血清肿瘤坏死因子a的活性变化及临床意义

用放射免疫方法测定了2O例正常儿童及40例急性白血病（AL）患儿化疗前后血清肿瘤坏死因子（TNF－a）浓度。结果；在AL诊断时，TNF－a水平显著升高1当患儿获得部分缓解至完全缓解时，TNF－a水平呈梯形降低，其最低值仍高于健康儿童（P＜O．01～0．05）；22例急性淋巴细胞白血病（ALL）患儿TNF－a水平在任何阶段都比18例急性非淋巴细胞白血病（ANLL）患儿低（P＜0．o1）；TNF－a水平和临床疗效之间显示负相关性。因此，作者们认为测量TNF－a水平可作为一项判断AL疗效和预后的临床指标。     

脑性瘫痪86例临床漏误诊分析

目的分析小儿脑性瘫痪（简称脑瘫）的早期表现、临床诊断和鉴别方法。方法对2002年10月～2004年10月来我院康复科就诊后，被确诊的小儿脑性瘫痪86例漏诊和误诊儿的首次就诊情况进行临床回顾性分析和总结。结果86例患儿首诊时均被漏诊或误诊，使患儿失去了早期康复治疗的时机。结论提高和加强对小儿脑瘫早期临床表现的认识，减少漏、误诊率，是提高脑瘫患儿早期全面康复的有效措施。     

骨关节痛为早期表现的小儿白血病15例分析

目的提高对以骨关节痛为早期表现的小儿白血病的认识,避免误诊误治。方法对以骨关节痛为早期表现的小儿白血病15例进行回顾分析。结果本组15例均经骨髓细胞学检查确诊为小儿白血病,其中ALL12例;发病以学龄儿童为主,男女之比为2∶1;多数患儿伴不规则发热及肝脾肿大,所有病例外周血象均有一项或多项异常;骨关节X线检查多无异常;多数病例较长时间被误诊误治。结论以骨关节痛为早期表现的小儿白血病,其早期症状可单一不典型,若对其认识不足、警惕性不高,易引起误诊误治。临床上凡遇骨关节痛诊断为风湿或类风湿性关节炎而经抗风湿效果不佳者,应高度警惕,耐心细致的体格检查,反复血涂片和及时的骨髓检查,对于早期确诊小儿白血病有极其重要的意义。     

HA117编码蛋白、P-gp和bcl-2在儿童急性白血病中的表达及意义

目的 探讨HA117编码蛋白、B细胞白血病/淋巴瘤-2（bcl-2）和P-糖蛋白（P-gp）在急性白血病（AL）患儿骨髓单个核细胞中的表达及临床意义.方法 通过免疫组织化学（SP）方法检测37例AL患儿中HA117编码蛋白、bcl-2、P-gp的表达情况,以15例非恶性血液病患儿为对照.结果 （1）AL组患儿HAll7编码蛋白、bcl-2及P-gp的阳性表达率分别为64.86％、62.16％和37.84％;AL组HA117编码蛋白表达阳性率明显高于对照组（P =0.033）;三种蛋白在急性髓细胞白血病组和急性淋巴细胞白血病组表达阳性率差异无显著性.（2）HA117编码蛋白在初诊组表达阳性率明显高于对照组（P =0.021）,而完全缓解组与对照组比较差异无显著性（P =0.098）.（3）AL患儿HAll7编码蛋白与bcl-2的表达水平呈正相关性（P=0.0001,r=0.593）.结论 新基因HA117可能参与了儿童AL的耐药机制,对儿童AL的预后有一定的预示作用,并在肿瘤的发生、发展和耐药中可能与抗凋亡基因bcl-2存在协同作用.     

Osteoarticular manifestations as initial presentation of acute leukemias in children and adolescents in Bahia, Brazil

OBJECTIVE: This study was to determine the prevalence and characteristics of the osteoarticular manifestations on initial clinical presentation of acute leukemias (ALs) on childhood in the state of Bahia, Brazil. MATERIALS AND METHODS: This retrospective study assessed the medical records of 406 patients with AL from January 1995 to December 2004. RESULTS: Acute lymphocytic leukemia (ALL) was diagnosed in 313 (77.1%) patients and acute myeloid leukemia (AML), in 93 (22.9%) patients, including 241 males (59.4%) and 165 females (40.6%). Age ranged from 9 months to 15 years (average: 6.18 y). The most common presenting features were fever (18.5%), musculoskeletal diffuse tenderness (15.0%), pallor (11.4%), and leg tenderness (5.7%). Prior referral to our center, the most frequent initial diagnosis was anemia (15.8%), leukemia (15.0%), amygdalitis (3.7%), and rheumatic fever (2.7%). Osteoarticular manifestations were found on 54.7% of the patients with AL, with a higher frequency among patients between 1 and 9 years of age (58.7%, P=0.0007). The presence of joint tenderness (16.2% in ALLx5.4% in AML), arthritis (26.6% in ALLx9.7 in AML), bone tenderness (26.1% in ALLx16.1% in AML), limb tenderness (49.5% in ALLx25.8% in AML), and antalgic gait (32.8% in ALLx9.7% in AML) had higher prevalence on ALL. The large joints, chiefly the knees (10.6%), ankles (9.4%), elbows (4.4%), and shoulders (3.6%) were more often affected. CONCLUSIONS: AL should be considered on the differential diagnosis of osteoarticular symptoms of unknown etiology in children.     

24 cases of human parvovirus B19 infection in children]

From January 1, 1987 through December 31, 1990, twenty-four pediatric patients with human parvovirus B19 (HPV B19) infection were seen. In every case the diagnosis was established by a positive capture immunoassay for IgM antibodies against the HPV B19. Four patients had hematologic manifestations, including one case of transient bone marrow aplasia revealing hereditary spherocytosis, one case of autoimmune hemolytic anemia with beta-thalassemia, and two cases of peripheral thrombocytopenia. Eight patients had skin lesions, with a morbilliform rash in six cases, erythema nodosum in one case, and Gianotti-Crosti syndrome in one case. No patients had erythema infectiosum. Seven patients developed joint manifestations: Henoch-Sch?nlein purpura in two cases, arthralgia in four cases, and polyarticular disease progressing to severe rheumatoid arthritis in a thirteen-year-old girl. Unremarkable symptoms of viral disease were seen in three patients. A five-month-old infant developed severe acute myocarditis. One patient with hepatitis A had acute liver failure. This study confirms the broad spectrum of clinical manifestations of HPV B19 infection. There were a number of unusual findings, including the high rate of joint manifestations (29%) and the severe course of some hematologic and myocardial manifestations. These results raise the question of whether the HPV B19 may be involved in the genesis of chronic juvenile arthritis.     

Febrile neutropenia as the presenting sign of appendicitis in an adolescent with acute myelogenous leukemia

The diagnosis and management of a surgical abdomen in patients with acute leukemia is quite difficult because of the complications and treatment of disease itself. A 13-year-old boy with acute myelogenous leukemia developed 2 episodes of febrile neutropenia during induction therapy. The second one was treated with a 5-day course of parenteral antimicrobial therapy, but the patient then presented with right lower quadrant abdominal tenderness, guarding, and rebound tenderness. Abdominal ultrasonography and computed tomography revealed appendicitis. Conservative medical management was unsuccessful, and appendectomy was performed 5 days after appendicitis was diagnosed. The patient's clinical manifestations resolved 5 days later. The case illustrates that fever may be the first manifestation of appendicitis in a child with acute myelogenous leukaemia who is neutropenic. Surgery is acceptable as first-line treatment in such cases.     

Septic arthritis of the elbow in children: The role of sonography

Twelve children with suspected septic arthritis of the elbow were prospectively studied with plain-film radiography and ultrasound. Imaging data were correlated with clinical history and final diagnosis. Joint effusion was seen by sonography in six patients, four of whom underwent ultrasound-guided joint aspiration, confirming the diagnosis of septic arthritis in two patients and excluding it in two. Of nine patients whose plain films showed only soft tissue swelling, seven had one or more significant findings with sonography: joint effusion (without fat pad elevation on lateral plain films) in three patients, periosteal reaction in two, and epitrochlear mass in three. Sonography confirmed soft tissue swelling alone in two patients, thus excluding the diagnosis of septic arthritis and obviating unnecessary attempts at joint aspiration. Sonography of the elbow is an informative, easily performed examination, which is capable of showing both intra- and extra-articular abnormalities not apparent by plain radiography. Both the demonstration of pathologic changes and the failure to show joint fluid may affect clinical management.     

Parvovirus B19 in the acute arthropathies and juvenile rheumatoid arthritis

OBJECTIVE: To evaluate the prevalence of recent parvovirus B19 infection in a cohort of children presenting with acute arthropathy and to determine the prevalence of a subsequent diagnosis of juvenile rheumatoid arthritis in this cohort. METHOD: In this prospective study, parvovirus B19 IgM antibody was investigated in 75 patients who were referred to our clinic with acute joint complaints and also in 75 healthy controls. One patient in each group was excluded due to neuroblastoma and acute lymphoblastic leukaemia. The characteristics of parvovirus B19 IgM positive patients who were accepted as parvovirus B19 arthropathy were further evaluated. All the patients were followed up for at least 6 weeks and the patients with chronic progression of joint complaints were followed for at least 6 months to determine their progress. The cases of juvenile rheumatoid arthritis in this chronic group were identified. RESULTS: Parvovirus B19 IgM was detected in 16 of 74 patients (21.6%) with acute arthropathy compared with 3 of 74 (4.1%) in the healthy control group (chi(2) = 8.67; P = 0.003). The parvovirus B19 positive patients with arthropathy were more likely to become chronic (P = 3.7 x 10(-7)) and to be diagnosed as juvenile rheumatoid arthritis (P = 0.03) than the parvovirus B19 IgM negative group with arthropathy. Additional joint destruction developed in one case who was parvovirus B19 IgM positive in whom juvenile rheumatoid arthritis was diagnosed during follow up. CONCLUSION: These data support the hypothesis that parvovirus B19 infection may be associated with the onset of juvenile rheumatoid arthritis in a proportion of patients.     

Conduite à tenir devant une monoarthrite chez l’enfant

Joint swelling is common in childhood. The pattern of presentation, the duration and the location may reveal sometimes monoarthritis. A detailed clinical history, thorough clinical examination, and sometimes complementary tests are needed to reach the correct diagnosis. The single most important investigation in a child with acute monoarthritis is joint aspiration to rule out septic arthritis that may destroy the joint within few hours. Serum inflammatory markers, antinuclear antibody, tuberculosis testing, and imaging (in specific cases) play an important role in making the diagnosis. This article presents the clinical approach to the diagnosis of monoarthritis as well as the different causes of monoarthritis in children.     

Preschool sarcoidosis mimicking juvenile rheumatoid arthritis: The significance of gallium scintigraphy and skin biopsy in the differential diagnosis

Preschool sarcoidosis occurring in children less than 6 years old is rare and characterized by the triad of skin, joint and eye manifestations without any pulmonary lesion. Because of similar clinical manifestations, the diagnosis of preschool sarcoidosis and juvenile rheumatoid arthritis (JRA) is confusing. A girl with preschool sarcoidosis, initially diagnosed and treated as having JRA, is reported here. Ophthalmologic examinations revealed posterior involvement of the eye. A gallium scintigram of the head showed panda appearance. Biopsy of the cutaneous lesion demonstrated non-caseating granuloma. Gallium scanning may be an important clue to correct diagnosis.     

Children with partial IgA deficiency: clinical characteristics observed in the pediatric rheumatology clinic

Literature is lacking on partial IgA deficiency. In this study, the authors propose to describe the clinical manifestations of patients with partial IgA deficiency. Methods. The authors conducted a retrospective chart review of 13 patients with partial IgA deficiency followed at the pediatric rheumatology clinic at Robert Wood Johnson Medical School. They looked for the presence of rashes, joint pain, joint swelling, and morning stiffness. The authors also examined charts for a history of frequent infections, allergies, and the presence of elevated antinuclear antibody. Results. Eleven out of the 13 patients complained of joint pain, joint swelling, or morning stiffness. Six patients carried a diagnosis of a definitive rheumatic disease. Four patients suffered from frequent infections and 2 patients reported allergies. Conclusion. Partial IgA deficiency appears to be associated with rheumatic diseases and complaints of joint pain, joint swelling, and morning stiffness. A larger study is needed to confirm these results.     

幼年特发性关节炎关节外表现诊治进展

幼年特发性关节炎(JIA)是一组原因不明,以慢性关节炎为主要特征且临床异质性较强的儿童风湿性疾病。患儿病情多数预后较好,但部分患儿可有关节外重要组织器官受累,如心血管系统损害、肺胸膜病变、肾淀粉样变性和葡萄膜炎等。如果诊治延误可致近期和/或远期重要脏器功能损害,导致病情加重或遗留严重后遗症,引起生活质量下降甚至危及生命...     

小儿急性阑尾炎的诊治体会

目的 探讨儿童急性阑尾炎的诊治特点。方法 回顾1998年6月～2003年6月期间四川大学华西医院收治的940例小儿急性阑尾炎临床资料，总结其临床特点及处理经验。结果 本组940例，发热、腹痛、右下腹固定压痛及白细胞升高为最主要表现，939例经手术及病理检查证实诊断，术后均痊愈，其中18例有白血病、血液系统疾病及其他原发疾病的患儿，确诊后也经手术治疗痊愈。另1例有原发白血病的患儿经内科治疗缓解后离院，预后不详。结论 发热、腹痛、右下腹固定压痛及白细胞升高仍是诊断小儿急性阑尾炎的最主要依据，并且小儿阑尾炎一经诊断应尽早处理，年龄越小，越应积极手术。对合并其他原发疾病的阑尾炎患儿，在充分准备的情况下仍可进行外科治疗，以防严重并发症发生。