Anasarca: not a nephrotic syndrome but dermatomyositis

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract, and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of JDM, generalized edema has rarely been reported. Here, we report a 3.5-year-old boy with JDM presenting with generalized edema. The diagnostic criteria of JDM rely on typical clinical manifestations that include: severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum skeletal muscle enzymes, and myopathic electromyographic pattern. Our patient initially received methylprednisolone and intravenous immunoglobulin (IVIG) without significant improvement, so he was given azathioprine and a prolonged course of oral prednisolone. We conclude that JDM should be suspected in patients presenting with anasarca in the absence of laboratory parameters of other causes of generalized edema and an appearance of heliotrope rash with muscle weakness. Also, we suggest that muscle magnetic resonance imaging (MRI) should be considered among the diagnostic tools of JDM.     

幼年型皮肌炎特异性抗体研究进展

幼年型皮肌炎（juvenile dermatomyositis，JDM）是以近端肌无力和皮疹为主要临床表现的自身免疫疾病，亦可累及多系统、多脏器。肌炎特异性抗体（myositis-specific autoantibodies，MSA）与JDM患者的并发症及预后高度相关。抗Mi-2抗体阳性患者预后较好，临床症状典型；抗MDA5抗体阳性患者多伴发弥漫性间质性肺疾病及皮肤溃疡，肌炎症状轻；抗NXP2抗体阳性患者易合并钙质沉着，该抗体与胃肠出血及穿孔相关；抗TIF1-γ抗体阳性患者有弥漫、顽固的皮损表现；抗SAE抗体在儿童中检出率较低，相关报道较少。该文综述了5种MSA亚型JDM患者的临床表型特点，为JDM患儿的临床治疗和随访管理提供依据。     

Disease patterns of juvenile dermatomyositis from western India

A retrospective assessment of clinical characteristics, complications/ associations, laboratory investigations, treatment modalities and outcome in an inceptional cohort of 22 (male-13) children with juvenile dermatomyositis (JDM) receiving treatment at Jaslok Hospital, Mumbai during 1997–2012 was performed. Mean age at diagnosis was 7.52 ± 3.99 years. Typical skin rash and muscle weakness were present in all children. Common complications included cutaneous ulcers (27.27%), dysphagia (22.72%) and calcinosis (18.18%).All patients presented with at least one of the serum muscle enzymes elevated. Absence of mortality and cardio-pulmonary complications and a monocyclic course in 72.7% of our patients are at variance from Western series.     

小儿皮肌炎的临床特征

目的探讨皮肌炎（JDM）患儿的临床特点和疗效。方法回顾性分析1990年1月～2004年1月住本院15例小儿JDM的临床特点,对其血清肌酶、肌肉活检的病理改变及患儿对糖皮质激素的疗效和预后资料进行分析。结果JDM患儿均有对称性近端肌无力;最常见皮肤改变是眼镜状红斑和高春征;患儿均有肌酶升高,肌电图示呈肌源性损害;肌肉活检示肌炎改变,8例经激素治疗1个月肌酶恢复正常,2.5个月肌力改善。结论掌握小儿JDM临床特征对诊断有重要意义,早期诊断、合理治疗可改善预后。     

幼年皮肌炎120例临床特点及治疗随访分析

目的 研究幼年皮肌炎(JDM)的临床特征、治疗效果以及转归.方法 回顾性分析2003年12月-2011年3月在北京儿童医院住院JDM患儿120例,分析其起病情况、临床表现、实验室检查及辅助检查、治疗方法、随访和预后.结果 120例患儿男55例,女65例;发病年龄1～14岁,平均年龄7岁.患儿均有典型的皮损及不同程度的肌肉症状,83例(69%)患儿有内脏受累,最常受累系统为呼吸系统(48%).所有患儿肌酶增高,肌电图均表现为肌源性损害.120例患儿均采用糖皮质激素治疗,均在早期加用甲氨蝶呤,有肺损害及重症患儿加用环孢素或环磷酰胺.早期治疗效果及远期预后均较好,120例在急性期死亡7例,死于肺部受累并感染致呼吸衰竭5例,并巨噬细胞活化综合征2例.结论 JDM是一种少见疾病,以肌无力和皮肤损害为突出表现,其皮损具有特征性,各脏器功能评估对诊断和判断疾病严重性非常有益;糖皮质激素联合免疫抑制剂治疗JDM安全有效,且预后较好.     

自体外周血干细胞移植治疗难治性学龄前儿童风湿病

目的探讨自体外周血干细胞移植（auto-PBHSCT）治疗难治性学龄前儿童风湿病的可行性、安全性和有效性。方法幼年皮肌炎（JDM）、幼年系统性红斑狼疮（JSLE）、幼年类风湿关节炎（JRA）各1例,均为男性患儿,年龄分别为3、6,6岁,病程分别为14、3．6、22个月,常规治疗中病情进展,库欣征明显,患病后患儿身高均无增长。JDM患儿肌力Ⅱ级,吞咽肌和呼吸肌受损,明显Gottron’s征和向阳性皮疹;JSLE患儿表现为蝶形红斑、蛋白尿、贫血,脑MRI显示脱髓鞘病变,系统性红斑狼疮疾病活动性指数（SLEDAI）≥12分;JRA患儿双膝、踝、腕、肘严重多关节炎。以环磷酰胺（CTX）＋粒细胞集落刺激因子（G-CSF）进行外周造血干细胞动员,经CliniMACS细胞分选仪分选CD34‘细胞。预处理方案：JSLE和JRA为卡氮芥（BCNU）＋足叶乙甙（VP16）＋阿糖胞苷（Ara-C）＋马法兰（MEL）及抗胸腺球蛋白（ATG）;JDM为CTX＋Mel及ATG。回输CD34^＋细胞数分别为9．45×10^6／kg、5．46×10^6／kg和9．60×10^6／kg。观察移植治疗前后风湿病状态和免疫学指标的变化。结果3例患儿移植后分别于＋9d、＋13d、＋11d粒细胞≥0．5×10^9／L,＋14d、＋18d和＋13d血小板≥20×10^9／L。CIM处于低水平,CD4／CD8倒置。JDM移植后1个月皮疹消失,肌力Ⅴ级,移植后2个月血清肌酶、肌电图恢复正常。JSLE移植后3个月皮疹消退,无蛋白尿,脑MRI病变吸收,移植后8个月自身抗体转阴性,SLEDAI为2～3分。JRA移植后3周关节炎好转,移植后3个月无关节肿胀及活动受限。均停用激素和免疫抑制药物,库欣征消退,18个月身高增长10～15cm,均已上小学或学前班,随访25～27个月无复发。结论auto-PBHSCT治疗难治性学龄前儿童风湿病近期疗效显著,安全性及远期疗效有待进一步观察。     

Juvenile dermatomyositis: An atypical presentation

Juvenile dermatomyositis (JDM) is a rare disease of unknown etiology that results in inflammation of the connective tissue of striated muscle, subcutaneous tissue, nail beds, and skin. Although JDM is a rare disease, occurring in 3 per 1 million children, it is the most common idiopathic, inflammatory, myopathic disease of childhood. A case example is provided that describes an atypical presentation of JDM. Information about the disease, the usual and unusual clinical manifestations, the diagnostic processes, and therapeutic management plans for JDM are presented. Results of a comparison between this case and the literature are discussed, along with implications for nurse practitioners.     

注意缺陷多动障碍患儿药物治疗前后microRNA表达量与临床症状的关系

目的 探讨注意缺陷多动障碍（ADHD）儿童药物治疗前后microRNA表达量与临床症状的关系。方法 选取2017年5月至2018年10月初诊为ADHD儿童80例为研究对象，将愿意接受药物治疗的儿童随机分为盐酸哌甲酯治疗组（n=31）和盐酸托莫西汀治疗组（n=33），不愿接受治疗的作为未治疗组（n=16），随访中盐酸哌甲酯组脱落10例，盐酸托莫西汀组脱落13例。另随机选取同时期行健康体检儿童60例作为健康对照组。ADHD儿童在首诊、随访3个月、6个月时进行SNAP-V评分，并采集ADHD及健康对照组儿童血清样本以荧光定量PCR法检测miR-4655-3p和miR-7641的相对表达量。结果 重复测量方差分析结果显示，注意力不足症状SNAP-V评分在两治疗组和未治疗组中，以及两种miRNA相对表达量在两治疗组和健康对照组中均存在分组与时间因素差异，且分组与时间因素均存在交互作用（P < 0.05）。多动冲动症状SNAP-V评分在两治疗组和未治疗组中存在时间因素差异（P < 0.05），而分组因素差异无统计学意义，且时间因素与分组因素无交互作用（P > 0.05）。经药物治疗的ADHD儿童注意力不足症状SNAP-V评分与miRNA-4655-3p和miRNA-7641相对表达量均呈负相关（分别r=-0.314、-0.495，P < 0.05）。结论 药物治疗可显著改善ADHD儿童的临床症状；血清中miR-4655-3p和miR-7641的表达水平可能作为ADHD的诊断及疗效评估的分子指标。     

Decreased serum adiponectin: an early event in pediatric nonalcoholic fatty liver disease

OBJECTIVE: To evaluate the relative concentrations of cytokines in pediatric nonalcoholic fatty liver disease (NAFLD). STUDY DESIGN: Thirty children were evaluated at a fasting morning visit to a pediatric research unit. RESULTS: Compared with normal-weight children (n = 12) and children who were overweight (n = 11), children who had presumed NAFLD (elevated Alanine aminotransferase [ALT] with negative work-up) (n = 7) had significantly lower mean serum adiponectin levels (P = .004). Adiponectin negatively correlated with body mass index (r = -0.60, P = .001), insulin (r = -0.74, P < .001), glucose (r = -0.52, P = .004), and ALT (r = -0.53, P = .003). There was no difference between normal-weight, obese, and presumed NAFLD subjects in mean serum tumor necrosis factor alpha and interleukin-6 and -8 concentrations nor in tumor necrosis factor alpha and interleukin-8 and -10 levels in an ex vivo lipopolysaccharide-stimulated system. CONCLUSIONS: Serum adiponectin is reduced in children with elevated ALT, similar to adults. However, children with presumed NAFLD do not have elevated pro-inflammatory cytokine levels. This suggests that depressed adiponectin plays a more proximal role than elevated levels of circulating pro-inflammatory cytokines in the development of NAFLD in children.     

儿童型肌炎/皮肌炎临床和病理研究

目的　儿童型多发性肌炎 /皮肌炎 (JPM /DM)目前在国内外文献中的大宗病例报道不多。为深入探讨两病的特征 ,本文着重研究其临床及病理特点 ,以进一步认识两病。方法　回顾性分析 4 2例JPM/DM (2 4例JPM ,1 8例JDM )患儿的临床资料 ,总结其临床表现、血清肌酶学、肌电图、肌肉病理的特点。结果　该病临床上主要有肌无力、肌萎缩 ,CK等血清肌酶增高 ,肌电图主要呈肌源性损害。JDM大多还有典型的皮肤损害 ,部分病例可出现皮下钙质沉积。JPM/DM的肌肉病理均显示免疫炎性改变。JPM组肌内膜炎发生率较JDM组高 (70 .8%vs 38.9% ,P <0 .0 5 ) ,JDM组肌束周萎缩及血管病变的发生率较JPM组高 (72 .2 %vs 4 1 .7% ,94 .4 %vs 5 4 .2 % ,P <0 .0 5或 0 .0 1 )。结论　JPM和JDM的临床和病理有所不同 ,JPM的病理改变可表现为散在肌纤维变性坏死、肌内膜炎改变为主的特征 ,部分病例微血管病变较明显 ;而JDM组的典型病理为广泛微血管病变和肌束周萎缩     

Effect of asthma intervention on children with undiagnosed asthma

OBJECTIVE: To measure the effect of an asthma intervention on the functional status and morbidity of children with undiagnosed asthma. STUDY DESIGN: Data from a randomized trial were used to compare outcomes at baseline and follow-up for children with undiagnosed and diagnosed asthma. We studied 510 symptomatic children with diagnosed asthma (diagnosed) and 299 children with symptoms but no diagnosis (undiagnosed). Baseline functioning and morbidity were similar for undiagnosed and diagnosed patients classified as moderate-severe. RESULTS: There were fewer undiagnosed reported allergies, seasonal symptoms, and other respiratory diagnoses (all P < 0.01). Among the moderate-severe, functional status, for example, symptom-days ( P = .02), symptom-nights ( P < .01), and days of restricted activity ( P < .01), was significantly reduced at follow-up for the undiagnosed in the intervention group but not for undiagnosed control subjects. Findings were similar for children with diagnosed asthma. CONCLUSIONS: Children with undiagnosed asthma were generally nonatopic, although some had symptoms at a level comparable to children with a diagnosis. The intervention successfully improved functional status for children with undiagnosed asthma as well as for children with diagnosed asthma. These results can be applied to ongoing discussions related to case detection.     

Serum citrulline levels correlate with enteral tolerance and bowel length in infants with short bowel syndrome

OBJECTIVE: To determine if serum levels of CIT (a nonprotein amino acid synthesized by the intestine) correlate with total parenteral nutrition (PN)-independence in children with short bowel syndrome (SBS). STUDY DESIGN: We prospectively obtained serum amino acid profiles over a 24-month interval from all infants with SBS 3 weeks to 4 years of age. Remaining small intestine length was recorded at surgery, and percent enteral calories tolerated (enteral calories divided by enteral plus parenteral calories x 100) was determined in 24 infants with SBS and 21 age-matched controls (blood drawn for non-gastrointestinal symptoms). RESULTS: Mean CIT for controls was 31 +/- 2 micromol/L. In patients with SBS (n = 24), serum CIT correlated linearly with percent enteral calories (R = 0.85; P <.001) and with bowel length (R = 0.47; P < or =.03). CIT level in patients with SBS weaned off PN was 30 +/- 2 micromol/L; in those subsequently weaned off PN, 20 +/- 2 micromol/L; and in those who would remain PN-dependent, 11 +/- 2 micromol/L ( P < or =.01). Serum CIT > or =19 micromol/L had 94% sensitivity and 67% specificity for being off or coming off total PN. CONCLUSIONS: Serum CIT level >19 micromol/L in children with SBS is associated with development of enteral tolerance and may be a useful predictive test.     

幼年型皮肌炎常见肌炎抗体与临床表型的关联分析

背景 目前成人和儿童皮肌炎的肌炎抗体谱与临床表型谱的分析受单纯与复合抗体、是否已治疗等混杂因素影响。目的 探讨幼年型皮肌炎(JDM)肌炎特异性抗体(MSA)与临床表型的相关性。设计 病例系列报告。方法 JDM诊断参照Bohan/Peter标准或2017年EULAR/ACR标准。纳入复旦大学附属儿科医院(我院)风湿科2011年开始建立的JDM随访系统中2017年3月至2020年4月行欧蒙免疫印迹法检测16种肌炎抗体的连续病例。从我院HIS系统中和风湿科JDM随访系统中采集来我院就诊时的临床数据:发病年龄,诊断时病程,随访病程,体温,皮肤、骨和关节、肺、肝、肾表现,实验室检查,MAS,重叠SLE,儿童肌力(CMAS)评分。主要结局指标 单纯和复合抗-MDA5抗体患儿临床表型特点。结果 符合本文纳入标准的103例JDM进入分析,男54例(52.4%),诊断JDM后未经治疗前行特异性抗体谱检测60例。初诊时CMAS评分中位数为33(23.0,44.7)。主要临床表现:发热、皮肤溃疡、皮肤钙化、关节痛或关节炎、间质性肺炎、合并重症肺炎、吞咽困难、血尿和IgA肾病、合并巨噬细胞活化综合征。103例JDM患儿中,肌炎抗体阳性68例(66.0%),其中MSA阳性64例,肌炎相关性抗体(MAA)阳性24例,MSA+MAA阳性20例。抗-MDA5抗体组、抗-NXP2抗体组、抗-TIF-1γ抗体组及抗体全阴性组4组间发病年龄和初诊时病程比较差异有统计学意义。抗-TIF-1γ抗体组发病年龄更小,初诊时病程更长。抗-MDA5抗体组更易发生皮肤溃疡、关节炎/关节痛,更多合并间质性肺炎和发热。4组间CMAS评分、ALT、AST、LDH、α-羟丁酸脱氢酶(HBDH)、CK、Fer差异有统计学意义,抗-NXP2抗体组CMAS评分最低,CK、LDH和HBDH最高,抗-MDA5抗体组ALT、AST最高、CK最低,Fer最高;抗-TIF-1γ抗体组ALT、AST、LDH、Fer最低;抗体全阴性组ALT、AST、CK、LDH、HBDH未显示有意义的特点。初治单纯抗-MDA5组(n=9)HBDH、Fer基本正常,初治复合抗-MDA5组(n=10)HBDH、Fer显著增高。结论 抗-MDA5、抗-NXP2和抗-TIF-1γ抗体阳性JDM患儿呈现了足以鉴别的临床表型和实验室检查结果,未治疗情况下单纯与复合抗-MDA5抗体阳性JDM患儿HBDH和Fer有明显的鉴别意义,抗体全阴性患儿临床表型和实验室结果无明显特征。     

幼年型皮肌炎42例临床回顾分析

目的　探讨幼年型皮肌炎的临床特点。方法　对 4 2例幼年型皮肌炎进行临床分析。结果　首发症状中单纯皮疹占 33 3% ,皮疹伴肌炎占 4 0 5 % ,单纯肌炎占 16 7%。后期钙质沉着占 2 6 2 %。实验室检查乳酸脱氢酶 (LDH)、天冬氨酸转氨酶 (AST)、磷酸肌酸激酶 (CPK)升高分别为 94 7%、83 4 %、6 2 2 % ,类风湿因子 (RF)和抗核抗体 (ANA)阳性率分别为 37 5 %、2 5 0 %。内脏损害中心脏、肺、胃肠道分别占 5 9 5 %、5 7 1%、19 1% ,无一例伴发恶性肿瘤。肌电图显示肌源性改变占 91 7% ,肌活检异常占 91 7%。结论　幼年型皮肌炎以皮肤损害为首发症状 ,后期可引起广泛钙质沉着 ;实验室检查中肌酶升高频率LDH >AST >CPK ;RF阳性率高于ANA ;内脏损害中以心脏和肺损害多见。     

Discordant erythrocyte sedimentation rate and C-reactive protein in children with inflammatory bowel disease taking azathioprine or 6-mercaptopurine

BACKGROUND: Inflammatory bowel disease (IBD) is characterized by periods of relapse and remission. Treatment is aimed at reducing symptoms during relapse and prolonging the duration of remissions. 6-Mercaptopurine (6-MP) and its prodrug azathioprine (AZA) are commonly used to prolong clinical remissions. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are two widely used laboratory markers of inflammation. The authors observed an unexplained discordance between ESR and CRP in children with asymptomatic IBD who were being treated with AZA or 6-MP. OBJECTIVE: To characterize children with IBD in remission treated with 6-MP or AZA who have persistently elevated ESR but normal CRP. METHODS: All patients seen in Pediatric Gastroenterology Clinic between January 1, 1995, and December 31, 2002, with Crohn disease or ulcerative colitis who received AZA or 6-MP continuously for at least 6 months were identified and their medical records reviewed. RESULTS: One hundred twenty patients met the eligibility criteria. Twelve had an ESR >18 mm/hour on at least three occasions during at least 12 consecutive months with a simultaneous CRP <0.8 mg/dL. Eleven of these 12 had no signs or symptoms of active disease and had Pediatric Crohn Disease Activity Index scores <10 for at least 12 consecutive months while the ESR was elevated. Disease duration was similar in the 11 children with asymptomatic disease and with discordant ESR and CRP and in 108 children with concordant ESR and CRP (69.2 + 22.5 months v 54.3 +/- 40.1 months, P = 0.0709). Duration of AZA or 6-MP therapy was greater in the 11 children with asymptomatic disease and discordant ESR and CRP than in those with or without symptoms and with concordant ESR and CRP (58.1 +/- 16.4 months v 36.6 +/- 24.1 months, P = 0.0043). There were no differences between the groups with respect to diagnosis, location of disease, or age at onset of symptoms. The mean corpuscular volume (MCV) was somewhat larger in the children with discordant ESR and CRP than in the children with concordant ESR and CRP (91.4 +/- 6.97 fL v 87.0 +/-7.07 fL, respectively, P = 0.0373); however, in both groups, the MCV was in the normal range. There were no significant differences in hematocrit, white blood cell count, serum albumin, total serum protein, or estimated serum globulin between the groups. CONCLUSIONS: The results suggest that among children treated with AZA or 6-MP, CRP may be a more reliable indirect indicator of inflammation than ESR. This report alerts clinicians that some children taking AZA or 6-MP may have persistent elevation of the ESR with a normal CRP and have no clinical evidence of active disease.     

儿童吉兰-巴雷综合征需要辅助呼吸的危险因素分析

目的 研究儿童吉兰-巴雷综合征（Guillain-Barré syndrome,GBS）需要辅助呼吸的危险因素。 方法 回顾性收集107例GBS患儿的临床资料。根据是否需要辅助呼吸,分为需要辅助呼吸组（n=16）和不需要辅助呼吸组（n=91）。比较两组患儿的临床资料,采用多因素logistic回归分析法分析GBS患儿需要辅助呼吸的危险因素。 结果 107例GBS患儿中,16例患儿需要辅助呼吸,发生率为15.0%。需要辅助呼吸组面肌和/或延髓肌无力比例高于不需要辅助呼吸组,起病到病情高峰期天数短于不需要辅助呼吸组,首次就诊休斯功能分级量表得分高于不需要辅助呼吸组（均P<0.05）。面肌和/或延髓肌无力、起病到病情高峰期天数短是GBS患儿需要辅助呼吸的危险因素（分别OR=5.053、1.239,P<0.05）。 结论 面肌和/或延髓肌无力和起病到病情高峰期天数的缩短可能增加GBS需要辅助呼吸的风险。 引用格式:     

Characteristics of juvenile dermatomyositis in Japan

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM , Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.     

Features of Kawasaki disease at the extremes of age

AIM: The diagnosis of Kawasaki disease (KD) in those outside the typical age range (6 months-4 years) is often delayed, potentially worsening prognosis. The features of KD in childrenor=5 years were compared with those presenting within the more typical age distribution. METHODS: Korean children with complete diagnostic criteria for KD were grouped according to their age at presentation: Group A (or=5 years). The clinical features, laboratory findings and outcome in each group were compared. RESULTS: Of 136 children presenting to a single centre between 1999 and 2003, 10 children were in Group A, 114 in Group B and 12 in Group C. The mean total fever duration was 8 days in Group C and 6.2 days in Group A (P=0.03). All children in Group C had cervical lymphadenopathy, compared with 50% of Group A and 64% of Group B (P=0.01). Coronary artery lesions were commoner in older children (Group C, 42%) compared with Group B (17%, P=0.05). All children had an equivalent leukocytosis, but Group C had significantly higher neutrophil counts (P=0.001). Group A had significantly lower mean haemoglobin (P=0.003) and total protein (P=0.002) at presentation and a more marked thrombocytosis 1 week after intravenous immunoglobulin therapy (P<0.05). CONCLUSION: The clinical and laboratory phenotype of KD varies with age. Older children may have a more marked inflammatory response and worse outcome. Younger children who are treated appropriately may not have a chance to higher risk of coronary artery lesions.     

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??Idiopathic inflammatory sympathies are a group of rare autoimmune disorders??characterized by inflammation in striated muscle. Dermatomyositis is the most common subtype in children.Though a number of diagnostic criteria have been proposed??the diagnosis of dermatomyositis is based on a combination of clinical??laboratory and electromyography findings.Typical features are subacute-onset?? proximal??symmetric muscle weakness??pathognomonic skin rashes.Muscle biopsy demonstrated inflammatory infiltrates confined to perimysium??often around blood vessels??and perifascicular muscle fiber atrophy. The uses of magnetic resonance imaging of skeletal muscles??detection of myositis-specific autoantibodies and nailfold capillaroscopy have become useful additions to our diagnostic test.     

The relationship of body mass index and blood pressure in primary care pediatric patients

OBJECTIVE: To determine whether an association of overweight, or risk of overweight, and blood pressure can be detected in children in the pediatric primary care practice setting. STUDY DESIGN: We examined electronic medical record (EMR) data from primary care practices on 18,618 children age 2 to 19 years. Each child was classified on the basis of age- and sex-specific body mass index (BMI) percentile as normal weight (BMI < 85th percentile), at risk for overweight (BMI > or = 85th and < 95th percentile), or overweight (BMI > or = 95th percentile). BMI Z-score and height Z-score were computed. Systolic and diastolic blood pressures were compared among age-sex-BMI groups. RESULTS: Among children in primary care pediatric practices, 16.7% were at risk of overweight and 20.2% were overweight. With increasing BMI status there was a significant increase in both systolic blood pressure (P < .001) and diastolic blood pressure (P < .001). The association of higher blood pressure with increasing BMI status was present in all age groups. CONCLUSIONS: Clinical data from pediatric primary care practices verify the high prevalence of childhood overweight. The effect of overweight on blood pressure is present in childhood and can be detected even in children as young as 2 to 5 years.