Juvenile polyarteritis: results of a multicenter survey of 110 children

OBJECTIVE: To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected. STUDY DESIGN: Pediatricians were asked to classify their patients into one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients. RESULTS: The girl:boy ratio was 56:54, with a mean age of 9.05 +/- 3.57 years. The cases were classified as: 33 (30%) cutaneous PAN; 5 (4.6%) classic PAN associated with hepatitis B surface antigen (HBs Ag); 9 (8.1%) microscopic polyarteritis of adults associated with antineutrophil cytoplasmic antibodies (ANCA); and 63 (57.2%) systemic PAN. Cutaneous PAN was disease confined to the skin and musculoskeletal system. All patients with HBs Ag-associated classic PAN were diagnosed with renal angiograms. Antiviral treatment was administered in most cases. Microscopic PAN patients had pulmonary-renal disease, in combination or separately. ANCA was present in 87%, and 2 patients progressed to end-stage renal failure. Patients classified with systemic PAN had multiple system involvement, almost all had constitutional symptoms, and all had elevated acute phase reactants. Corticosteroids and cyclophosphamide were the first choices of immunosuppressive treatment. The overall mortality was 1.1%. CONCLUSIONS: There were remarkable differences among pediatric patients with PAN, with different clinical manifestations and overall better survival and lower relapse rates when compared with adults.     

Polyarteritis nodosa and deafness A human temporal bone study

Summary Temporal bone changes were described in a 66 year old woman with polyarteritis nodosa who became deaf 7 months before death. Polyarteritis nodosa of the left internal auditory artery was demonstrated with fibrosis and bone formation involving the cochlea and vestibular system. Endolymphatic hydrops of the basal turn of the cochlea was also present, as well as a chronic perforation of the free wall of the saccule.Supported by USPHS grant NS-06606 from the National Institute of Neurological and Communicative Disorders and StrokeThe author is indebted to Mrs. Ada Dörr for invaluable technical assistance.     

二维-彩色超声多普勒在大动脉炎诊断中的应用探讨

目的探讨二维-彩色多普勒超声诊断大动脉炎的应用价值。方法回顾性分析16例大动脉炎患者的病变段血管超声声像图表现。结果超声表现均为病变血管管壁不均匀增厚,管腔不同程度的狭窄。结论二维-彩色多普勒超声可以直观的显示大动脉炎受累血管的情况,并准确的判定狭窄程度和范围,是诊断大动脉炎的有效方法之一,可作为临床诊断大动脉炎的常规方法。     

Attenuation of diabetic nephropathy by Chaihuang-Yishen granule through anti-inflammatory mechanism in streptozotocin-induced rat model of diabetics

Ethnopharmacological relevance

Traditional Chinese medical herbs have been used in China for a long time to treat different diseases. Based on traditional Chinese medicine (TCM) principle, Chaihuang-Yishen granule (CHYS) was developed and has been employed clinically to treat chronic kidney disease including diabetic nephropathy (DN). The present study was designed to investigate its mechanism of action in treatment of DN.

Materials and methods

Diabetic rats were established by having a right uninephrectomy plus a single intraperitoneal injection of STZ. Rats were divided into four groups of sham, diabetes, diabetes with CHYS and diabetes with fosinopril. CHYS and fosinopril were given to rats by gavage for 20 weeks. Samples from blood, urine and kidney were collected for biochemical, histological, immunohistochemical and molecular analyses.

Results

Rats treated with CHYS showed reduced 24 h urinary protein excretion, decreased serum TC and TG levels, but CHYS treatment did not affect blood glucose level. Glomerular mesangial expansion and tubulointerstitial fibrosis in diabetic rats were significantly alleviated by CHYS treatment. Moreover, CHYS administration markedly reduced mRNA levels of NF-κB p65 and TGF-β1, as well as decreased protein levels of NF-κB p65, MCP-1, TNF-α and TGF-β1 in the kidney of diabetic rats.

Conclusions

CHYS ameliorates renal injury in diabetic rats through reduction of inflammatory cytokines and their intracellular signaling.     

A new chromone from Cassia nodosa

A new chromone named as 5,4′-dihydroxy-7-methyl 3-benzyl chromone (1) along with three known flavonoid compounds as unsubstituted flavone, kaempferol-3-o-rhamnoside and quercetin-3-o-arabinoside have been isolated from the leaves of Cassia nodosa. Their structures have been established by means of chemical and spectral evidences (IR, UV, ¹H-NMR, ¹³C-NMR and mass spectra).     

潘文教授对多囊卵巢综合征中医病机的认识

潘文教授认多囊卵巢综合征的中医病机不外"虚""实"两端,虚者多由肾虚、脾虚所致,实者多由瘀血、痰湿、肝郁所致,临床单纯表现为实证或虚证者较少,往往二者兼夹致病。     

多发性大动脉炎的辨证论治体会

文章介绍了多发性大动脉炎的辨证论治体会,认为可采用散寒祛湿、益气活血;清热化湿、活血通脉;清热凉血、解毒通脉;益气涤痰、开窍通络;温阳散寒、化瘀通脉;健脾补肾、温阳通脉;补肾益精、养血通脉;活血化瘀、疏经通络;回本扶元、气血双补等方法论治多发性大动脉炎.     

罗丹明6G-PAN能量转移荧光猝灭法测定痕量锌

目的：建立一种荧光法测定头发中痕量锌的新方法。方法：在无水乙醇介质中,Zn^2＋与1-（2-吡啶偶氮）-2萘酚（PAN）形成配合物PAN-Zn^2＋,其吸收光谱与罗丹明6G（Rh6G）的发射光谱有效重叠,在λex/λem=543 nm/558 nm处发生能量转移,使Rh6G荧光猝灭,从而建立痕量锌的荧光猝灭测定新方法。结果：在优化实验条件下,在13～360 ng/ml浓度范围内,荧光猝灭程度与Zn^2＋浓度呈现良好的线性关系（r=0.9991）。方法的检出限为4.05 ng/ml;相对标准偏差为0.88%～2.72%（n=11）;样品加标回收率为97.5%～102.5%。结论：方法加入试剂简单,稳定性好,灵敏度高,操作简便,用于头发中痕量锌的测定,结果满意。     

A case of posterior reversible encephalopathy during polyarteritis nodosa vasculitis

Posterior reversible encephalopathy is a distinctive syndrome associated with different diseases and drugs. Disease evolution is frequently favorable with an adequate treatment. Damage typically involves parietal-occipital lobes even if a more anterior diffusion has been described. Here, we report the case of a woman affected by Polyarteritis Nodosa, who suddenly complicated with decreased consciousness and seizures, during an acute hypertensive state. MRI imaging showed increased T2 and FLAIR signal in posterior regions. Her neurological evolution was positive, according to arterial pressure correction, although the systemic vasculitis was still ongoing, hence affecting final prognosis.     

Diagnostic uncertainty in the inflammatory myopathies

A group of case histories with appropriate muscle biopsy findings is presented to demonstrate some atypical presentations of the inflammatory myopathies. Differential diagnostic possibilities are considered in presentations of idiopathic polymyositis, statin myotoxicity, the inflammatory component with the dysferlinopathies, treated dermatomyositis, a necrotizing myopathy with pipe-stem microvascular change, an inflammatory myopathy with abundant macrophages, inclusion-body myositis, and the differential diagnosis of problems with eosinophilic infiltration in the muscle biopsy. Attention is given to the role of membrane attack complex deposition in the microvasculature and the role of major histiocompatibility complex-1-expressing muscle fibers indicating activation of the endoplasmic reticulum stress response.