儿童泛发性脓疱型银屑病32例临床与病理分析

目的 探讨儿童泛发性脓疱型银屑病的发病及治疗情况，以提高治愈率。方法 对32例儿童泛发性脓疱型银屑病的临床症状及病理检查进行分析。结果 32例患儿均有其典型的临床表现，男女发病之比为2．2：1，平均年龄6．45岁，取新脓疱作病理活检，发现角质形成细胞变性，表皮水肿，表皮内有中性粒细胞移入，棘层上部海绵样脓疱形成，真皮血管扩张，血管周围淋巴细胞及中性粒细胞浸润。结论儿童泛发性脓疱型银屑病为一种少见的疾病，临床及组织病理具有特征性。治疗应去除诱发因素，及时控制感染，可在实验室定期监控下，首选新体卡松口服，症状可得到明显改善。     

皮肤疾病

003728复发性急性泛发性发疹性脓疙病1例J李雪松…//中国实用儿科杂志一2000,15(3)一159 患儿男,7岁。全身反复出现脓疤伴发热6年余,每年冬春季发病,1周左右体温恢复正常,脓疤干燥脱屑痊愈,愈后不瘤疤痕,发作后无寻常型银屑病皮损。面部、躯干及四肢散在大面积弥漫水肿性红斑,其上有不等大非毛囊性脓疙,密集分布。脓疤内容物培养无细菌生长。病理角层下脓疤。(张桂芳) 003729创灼膏治疗7种儿童皮肤病:附53例婴儿湿疹自身对照观察刁冻艳华…//中国皮肤性病学杂志一2000,14(2)一101 应用创灼膏治疗7种儿童皮肤病%例。同时应用自制0.1%强的松…     

非艾滋病儿童马尔尼菲青霉菌病3例报告及文献复习

目的总结非艾滋病儿童播散性马尔尼菲青霉菌病(PSM)的临床特点及治疗转归。方法回顾分析3例PSM患儿的临床资料。结果 3例患儿中女1例,男2例,发病年龄分别为5月龄、1岁5月龄、3岁2月龄,均以反复发热伴咳嗽、气促,病情进行性加重,抗感染治疗无效为主要临床特征;肺部CT均显示多发异常密度影;1例患儿处于急性淋巴细胞白血病诱导化疗期,2例为先天性胆道闭锁。2例患儿经血培养及痰培养、1例经血清及肺泡灌洗液病原微生物二代测序确诊。3例患儿经两性霉素B为主的治疗后均好转。结论 PSM可发生在非艾滋病儿童,肺部是主要靶器官;在PSM流行地区,免疫低下儿童应考虑PSM可能,尽早行病原学检查。     

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我们并用阿昔洛韦与丙种球蛋白治疗急性水痘样脓疱病(Kaposi水痘样疹)8例,疗效满意,现报道如下。临床资料:2005年3～12月,中国医科大学附属第二医院皮肤科共收治Kaposi水痘样疹8例,其中男5例,女3例,年龄5～14个月,发病至入院时间为3～6d,其原发疾病均为湿疹。8例患儿发病前均有单纯疱疹接触史。入院体检:体温38.2～39.8℃,头面部、颈部可见脐凹性的水疱、脓疱,部分水疱聚集成片,伴有大片结痂。根据患儿有湿疹病史及单纯疱疹接触史,结合典型的脐凹性水泡、伴发热等症状可作出诊断[1]。患儿精神萎靡,食欲不振,心肺未见异常。所有入选病例均否…     

儿童塑型性支气管炎53例临床回顾分析

目的 探讨塑型性支气管炎（plastic bronchitis,PB）的临床特点,提高对儿童PB的认识。方法 回顾分析天津儿童医院自2012年10月至2014年1月收治的53例PB患儿临床表现、实验室、影像学和支气管镜检查,以及吸出物病理分析、治疗经过、转归等。结果 53例患儿均经电子支气管镜检查吸取或钳取肺内塑型样物质,并经病理检查确诊为PB。患儿均有发热、咳嗽病史,发热峰值均＞39℃,80%以上患儿发热时间小于1周,最短发热时间仅为半天。患儿均为首次发作,X线胸片及肺CT均示大片实变,63%合并胸腔积液。支气管镜直接去除阻塞气道的塑型性内生异物是治疗PB最有效、最直接的治疗方法。还须辅以糖皮质激素等药物治疗以及体位引流、胸部理疗等。53例患儿平均住院12.6 d,病情好转或治愈出院,随访3个月未见病情反复。结论 塑型性支气管炎为儿科急危重症,病情进展快,凡临床上急起高热,咳嗽、气促,肺部影像学急性进展,出现肺大片实变、肺不张及胸腔积液,血Ｃ反应蛋白（CRP）、降钙素原（PCT）、血沉（ESR）呈数倍升高,常规抗感染治疗无效,需警惕急性塑型性支气管炎可能,建议及早行支气管镜检查及肺泡灌洗治疗。     

儿童急性播散性脑脊髓炎十例临床分析及文献回顾

目的 急性播散性脑脊髓炎起病隐匿、临床表现多样化,该研究通过探究儿童急性播散性脑脊髓炎临床特点并文献复习,以提高对该病的诊治水平.方法 回顾性分析我科2002年至2017年收治的10例急性播散性脑脊髓炎患儿的临床表现、实验室检查、治疗及预后并进行文献复习.结果 发热7例,抽搐4例,乏力3例,嗜睡3例,视力障碍3例,意识障碍1例,头痛1例,语言障碍1例,吞咽困难1例;脑压升高4例,脑脊液常规及生化异常6例;头颅MRI均异常,以白质病变为主;脑电图异常3例;血清抗MOG抗体IgG阳性1例.10例患儿均使用大剂量糖皮质激素联合人免疫球蛋白治疗;7例患儿症状好转后出院;1例重症,因呼吸循环衰竭死亡;2例留有不同程度的后遗症.结论 儿童临床表现急性播散性脑脊髓炎表现缺乏特异性,头颅MRI影像学改变可协助诊断,早期糖皮质激素、人免疫球蛋白联合使用安全有效,早期识别并积极治疗可改善其预后.     

皮肤疾病

911259 10例小儿泛发性脓疱型银屑病的临床分析/叶志云∥临床皮肤科杂志。-1991,20(1)。-45 10例中,男女各5例,最小发病年龄出生后7天,1岁以内发病者6例,病程7天～12年。皮损初发以头皮和额部多见,逐渐播及全身皮肤,皆为粟粒状小脓疱,群集或散在分布,部份融合为脓疱,脓疱表浅,很快干燥脱屑,但又不断有新的小脓疱出现,呈周期性反复发     

儿童脓毒症继发脾脓肿两例临床分析

目的 了解儿童少见类型脾脓肿的起病情况、临床表现及治疗特点.方法 回顾性分析湖南省儿童医院确诊的2例脾脓肿患儿的临床资料.结果 两例患儿中,1例为男性小婴儿（2个月）,因发热伴气促10余天,咳嗽4d,呕吐2d,腹胀1d入院,入院后明确有脓毒症及不典型化脓性脑膜炎,在完成了治疗疗程且一般情况好转时,发现外周血白细胞明显升高,并出现肝功能损伤,B超及CT检查提示脾脓肿,经积极抗感染治疗后患儿好转出院;另1例为7岁3个月男孩,临床表现为反复发热、龟头脓疱、双下肢脓疱,病程达2月余,在当地医院多次抗炎治疗,体温稳定后又反复,且不断出现新的皮肤脓疱,B超及CT均支持脾脓肿诊断,入院后先予积极抗感染治疗,未能控制病情,最后经脾切除治疗后好转出院.结论 儿童脾脓肿临床表现各异,对于反复发热、长时间外周血白细胞高、治疗过程中出现病情反复,均需高度怀疑脾脓肿.B超可早期诊断.儿科患者以保守治疗为主,小婴儿抗感染疗程可适当延长,必要时行脾切除.     

儿童临床无肌病皮肌炎合并肺间质病变 1 例报告及文献复习

目的分析儿童起病的临床无肌病皮肌炎(CADM)合并肺间质病变(ILD)的临床特点。方法回顾1例成功治疗的CADM合并ILD患儿的临床资料,并检索中英文数据库,分析儿童CADM合并ILD的临床特点、治疗及预后。结果女性患儿,13岁,Gottron's征阳性,临床无乏力症状,肌酸激酶、肌电图均正常,胸部高分辨CT提示肺间质性病变。经积极加用糖皮质激素联合环磷酰胺治疗,有所好转。检索国内外报道儿童CADM合并ILD 3例,加该患儿共4例,起病年龄10~16岁;首发症状分别为Gottern's征3例、发热2例;4例患儿的抗核抗体及抗Jo-1抗体均为阴性,2例抗Ro-52抗体阳性。3例文献报道的患儿在发现ILD时无呼吸系统临床症状,半年内迅速进展为呼吸衰竭死亡。结论儿童CADM合并ILD可呈现为快速进展型肺间质性病变,临床疑诊为CADM的患儿应完善肺部影像学检查,如存在ILD应予糖皮质激素联合免疫抑制剂积极治疗,c改善预后。     

皮肤疾病

0353‘7小儿医院感染性播散性带状疙疹1例/黄丽华…//临床误诊误治一2003,16(1)一79 0353‘8红拼索过权苯甲睡扭胶治疗30例典儿座疮/张该…//临床皮肤科杂志一2003,32(3).一17。 3~6个月龄。I级5例,I组16例,,级9例.父母单方或双方同患痊疮18例.占60%.经治疗获痊愈.痊愈率达100%。参5(张忠) 0353‘,反盆发作的急性泛发性发疹性胶疙病1例/徐翔…//中国实用儿科杂志一2003,18(5)一286 0353,O阿雄A醋和(或)阿维A治疗儿t脓疙型银月病/陈洁//临床皮肤科杂志一2003,32(6)一354~355 13例脓疤型银屑病患儿7例用阿维A醋治疗,5例用阿维A治疗,1例先后…     

Acute generalized exanthematous pustulosis: first case associated with a Chlamydia pneumoniae infection]

Skin reactions to drugs or infections can be very important. CASE REPORT: A 14-year-old boy developed fever, a diffuse pustular rash and a respiratory distress. Chest X-rays and serology were consistent with an acute Chlamydia pneumoniae infection. A skin biopsy revealed an acute generalized exanthematous pustulosis. CONCLUSION: Acute generalized exanthematous pustulosis (AGEP) is a disease manifested by a diffuse eruption of follicular sterile pustules. Most of the cases are drug induced, but they may be secondary to viral or bacterial infections. We report the first case of AGEP in a child with an acute C. pneumoniae infection.     

Localized acute generalized exanthematous pustulosis with amoxicillin and clavulanic acid

Acute generalized exanthematous pustulosis (AGEP) is a rare skin disorder, characterized by acute development of numerous, pin-head sized, nonfollicular pustules on erythematous skin, with high fever and neutrophilia. The condition is frequently caused by hypersensitivity reaction to drugs or viral infections. Diagnosis is established according to clinical and histological criteria. Herein, we report a 17-year-old girl with localized AGEP related to the use of amoxicillin-clavulanate.     

基于规范化诊治与管理的234例儿童癫性脑病病例系列报告

目的 探讨儿童癫性脑病(EE)的病因及预后。方法 回顾性分析儿童EE的临床资料,行病因学分析,并随访其疗效及预后。结果 234例EE患儿,包括West综合征92例,Dravet综合征53例,Lennox-Gastaut综合征32例,Landau-Kleffner综合征15例,大田原综合征13例,伴睡眠期持续棘慢波的癫性脑病10例,肌阵挛-失张力癫2例,Rasmussen综合征2例,15例未能归为已知的癫综合征。病因：遗传相关67.9%（基因诊断明确为遗传性31.2%）,结构性12.0%,感染性4.7%,代谢性和免疫性各0.9%,病因不明19.2%。治疗有效率：药物33.8%（79/234）,生酮饮食47.3%（35/74）,外科手术60.0%（3/5）,迷走神经刺激术（VNS）44.4%（4/9）;综合治疗（单纯药物治疗或在药物治疗基础上行生酮饮食/外科手术/VNS治疗）有效率48.7%。预后：发病后首次智能发育评估,223例（95.3%）存在发育异常;治疗后（随访病程中位数44月）智能发育随访,215例（91.9%）存在发育异常。结论 遗传性病因是EE的首要病因,仅1/3抗癫药物治疗有效,随访发现智能发育异常率高,总体预后不良。     

Plasma levels of the von Willebrand factor-cleaving protease in physiological and pathological conditions in children

The hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are rare disorders characterized by thrombocytopenia, hemolytic anemia, and ischemic organ failure due to thrombotic occlusions in arterioles. The recent observation that a von Willebrand factor-cleaving protease (VWF-CP) is low in the plasma of patients with TTP but normal in those with HUS has potentially offered a new specific tool for differential diagnosis. In this study, the authors evaluated the plasma levels of the VWF-CP during the neonatal state and healthy childhood and in some pathological pediatric conditions. The protease was measured in 16 healthy newborns, 20 healthy children aged 5-18 years, patients with diabetes mellitus type1 ( n = 7), acute viral hepatitis ( n = 10), chronic viral hepatitis ( n = 10), transfusion-dependent β-thalassemia major ( n = 10), acute varicella infection ( n = 11), the nephrotic syndrome ( n = 11), and familial Mediterranean fever ( n = 10). Mean protease levels were significantly lower in newborns than in healthy children (50.5 ±16.1% vs. 83.3 ±16.3%)( p = .0001). In patients with acute viral hepatitis, protease levels were also significantly reduced (40.2 ±27% v s. 83.3 ±16.3% in healthy children)( p = .0001). Other patient groups had normal protease levels. In conclusion, low protease levels are far from being a specific beacon for TTP. The current paradigm that a single laboratory test may enable physicians to distinguish TTP from HUS seems to be challenged by these and other findings.     

Abnormal serum phenylalanine-tyrosine ratio and hyperferritinemia in malignant histiocytosis

Nine cases of childhood malignant histiocytosis (MH) showed an abnormally high serum phenylalanine (Phe)/tyrosine (Tyr) ratio (3.47 +/- 1.32) coincident with hyperferritinemia (50,800 +/- 33,600 ng/ml). Lactate dehydrogenase activity was also increased in these patients. These values were compared with data on sera from two groups of patients, acute leukemia cases (n = 14) and measles cases (n = 13), and with control values from normal healthy children (n = 38). The Phe/Tyr ratio was 1.57 +/- 0.54 for the acute leukemia (p less than 0.01) and 2.58 +/- 1.46 for the measles cases (NS), serum ferritin was 245 +/- 124 ng/ml for acute leukemia (p less than 0.01) and 167 +/- 117 ng/ml for measles (p less than 0.01). Accordingly, the concurrence of both abnormalities is considered to be characteristic for MH. It was also found that both serum Phe/Tyr ratio and ferritin levels reflect the disease activity, indicating that these two factors are useful prognostic indicators in the treatment of patients with MH.     

儿童副鼻窦支气管炎治疗方案探讨

目的探讨儿童副鼻窦支气管炎的治疗方案、分析对儿童慢性咳嗽的认识及诊疗对策。方法对72例儿童副鼻窦支气管炎患儿作全程疗效随访观察,72例中男59例、女13例,随机分成三组:TFH组(29例)用头孢特仑脂(富山龙) N-乙酰半胱氨酸(富露施) 核酪口服液治疗,ZFH组(17例)用阿奇霉素(希舒美) 富露施 核酪口服液治疗,对照组(26例)按常规用药。应用统计学方法分析疗效结果。结果TFH组和ZFH组疗效均优于对照组,且ZFH组优于TFH组。ZFH组和TFH组总有效率分别达100%和79.3%,与对照组比较差异均有统计学意义。以10d为一个疗程,儿童副鼻窦支气管炎的疗程大约需要6～8个疗程(2个月以上)。结论对儿童副鼻窦支气管炎应采用综合性治疗,在抗生素应用的基础上应配合应用祛痰剂和免疫增强剂。ZFH方案是治疗儿童副鼻窦支气管炎的最佳方案。     

Seroetiology of acute lower respiratory infections among hospitalized children in Beijing

BACKGROUND: Little is known of the etiology of childhood acute lower respiratory infections in China, where the use of antimicrobials is indiscriminate. Trials to change such a policy require etiologic data, especially on the bacteria most relevant to these common diseases. METHODS: One hundred consecutive infants and children from 3 months to 14 years of age with symptoms and signs compatible with acute lower respiratory infections were studied prospectively in the largest pediatric hospital in Beijing from February to May, 1997. Blood culture, thorax radiography and paired sera for 20 microbiologic assays were taken, and the course of illness was monitored uniformly. Disease severity was graded. RESULTS: In 24 cases there was evidence only of bacterial etiology, and in 5 solely viral agents were found; 3 children probably had a mixed bacterial-viral infection. Surprisingly no pneumococcal infection was detected, Mycoplasma pneumoniae (n = 21), Haemophilus influenzae type b (n = 8) and Chlamydia pneumoniae (n = 7) being the dominant bacteria. All children recovered. CONCLUSIONS: Routine use of antimicrobials for these patients seems unjustified. Serologic evidence for the H. influenzae type b etiology is encouraging in terms of vaccination, but confirmatory studies are needed.     

Molecular cytogenetic findings of acute leukemia included in the Brazilian Collaborative Study Group of Infant acute leukemia

BACKGROUND: Chromosome abnormalities often occur prenatally in childhood leukemia, characterizing an early event in leukemogenesis. The majority of the abnormalities occurring in infants involve the MLL gene on chromosome band 11q23. We describe the molecular cytogenetic findings of 207 infant acute leukemia (IAL) cases included in the Brazilian Collaborative Study Group of Infant acute leukemia. PROCEDURE: The diagnosis of Acute Lymphoblastic leukemia (ALL) or acute myeloblastic leukemia (AML) was made according to morphology and immunophenotyping classification, followed by conventional karyotyping. Samples were then screened using RT-PCR for the presence of specific chromosome translocations. FISH assay for MLL rearrangements was performed only in cases with negative or inconclusive cytogenetic or PCR results. RESULTS: The characteristics of children with IAL were as follows: 115 boys and 92 girls, age range 0-23 months, mean age 12 months, 145 ALL, and 62 AML. A statistically significant association was observed between pro-B ALL cases and MLL+ve (P=0.0001) cases and the age group 0-3 months with MLL+ve (P=0.008) cases. Two rare cases of pro-T ALL with MLL+ve were found. Other than MLL rearrangements, various other molecular aberrations were detected including TEL/AML1+ve (n=9), E2A/PBX1+ve (n=4), PML/RARA+ve (n=4), and AML1/ETO+ve (n=2). Cytogenetic analysis revealed hyperdiploidy (n=6), del(7) in two cases and del(11)(q23) in seven cases. CONCLUSIONS: Our results show that not only MLL rearrangements, but also other molecular abnormalities occur before birth and may contribute to leukemogenesis.     

儿童侵袭性真菌感染临床分析

目的 分析、总结儿童侵袭性真菌感染的临床特点,以利早期诊断,改善预后.方法 回顾分析2001年至2007年住院确诊侵袭性真菌感染的6例患儿临床资料.结果 全部患儿伴有基础疾病,并且都有发热.肺部感染4例,CT扫描出现新月征或气环征2例.肺外感染2例.半乳甘露聚糖试验1/5患儿阳性.4/6患儿为曲霉菌感染.主要以二性霉素B或二性霉素B脂质体治疗,4/6患儿治愈,1例死亡.结论 恶性血液系统疾病和严重免疫抑制是儿童侵袭性真菌感染发病的重要因素.患儿临床表现缺乏特异性,应结合影像学及实验室检查全面考虑进行诊断,曲霉菌感染常见.二性霉素B治疗儿童侵袭性真菌感染有效.     

Colonic diversion for intractable constipation in children: colonic manometry helps guide clinical decisions.

BACKGROUND: Colonic manometry helps discriminate functional and behavioral causes for childhood constipation from colonic neuromuscular disease. METHODS: Of 375 colonic manometries performed for clinical indications, 12 could not be interpreted because of chronic colonic dilation. Based on colonic manometries that showed either no contractions or an absence of the gastrocolonic response or an absence of high-amplitude propagating contractions, the authors recommended diverting colostomies or ileostomies in 12 chronically constipated children (mean age, 4 years; range, 2-14 years, 5 boys). Before study, medical treatment was ineffective in all children. These children had persistently dilated colons with pathologic diagnoses of intestinal neuronal dysplasia (n = 4), hypoganglionosis (n = 2), hollow visceral myopathy (n = 1), and normal (n = 5). RESULTS: Six to 30 months after diversion, the authors restudied all the children. Eleven of 12 diverted colons were no longer dilated. In two patients, abnormal motility involving the entire colon was unchanged from the initial study, small bowel motility was abnormal, and we recommended no further surgery. In two cases, the colon remained abnormal but small bowel motility was normal, and we recommended subtotal colectomy and ileoproctostomy. In four cases, the left colon remained abnormal, but the right colon was normal, and we recommended reanastomosis after left hemicolectomy. In four cases, motility in the diverted colons was normal, including a gastrocolonic response and high-amplitude propagating contractions, and the authors recommended reanastomosis. Defecation problems resolved in 10 of 12 when followed up 5 to 30 months after treatment. CONCLUSION: These data suggest that in some cases of intractable childhood constipation associated with colonic distention, temporary diversion improved colonic motility. Colonic manometry may be used to predict which patients will benefit from resection or reanastomosis.