Deficiency of adenosine deaminase 2; special focus on central nervous system imaging

Purpose

To increase the knowledge of central nervous system (CNS) imaging features in deficiency of adenosine deaminase 2 (DADA2) by examining magnetic resonance imaging (MRI) studies of a relatively large number of patients.

Synthesis of Polyacrylonitrile‐block‐Polystyrene Copolymers by Atom Transfer Radical Polymerization

Summary: The synthesis of polyacrylonitrile‐block‐polystyrene (PAN‐b‐PS) copolymers by atom transfer radical polymerization (ATRP) is reported. Chain extension of bromine terminated PAN macroinitiators with styrene was performed using a CuBr/N,N,N′,N″,N″‐pentamethyldiethylenetriamine catalyst system and 2‐cyanopyridine as a solvent. The first‐order kinetic plots of styrene consumption showed a significant curvature, indicating a progressive decrease in the concentration of active species during copolymerization. The loss of the bromide end group was mainly ascribed to the elimination of HBr, as shown by ¹H NMR spectroscopy. By varying the molar ratio of either the catalyst or the monomer to the initiator, a series of PAN‐b‐PS copolymers were prepared, with polydispersities as low as 1.3, and molar compositions ranging from 8.6/91.4 to 35.5/64.5.

¹H NMR spectra of PAN‐b‐PS in DMF‐d₇ at 80 °C.     

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.     

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.     

Dramatic regression of mesenteric abnormalities demonstrated on angiography following prednisolone and cyclophosphamide combination therapy in a patient with polyarteritis nodosa associated with Sjögren’s syndrome

Abstract

A 63-year-old woman, who had been followed for Sjögren’s syndrome, was admitted due to cryoglobulinemia, leukocytoclastic vasculitis, and mononeuritis multiplexa. In spite of the administration of 60 mg prednisolone, fecal occult blood was strongly positive. The colonoscopy showed multiple colonic ulcers, and a diagnosis of polyarteritis nodosa (PAN) was made because abdominal angiography revealed markedly serpentine and narrowed superior and inferior mesenteric arteries. After steroid pulse therapy and daily oral administration of cyclophosphamide were initiated, her symptoms improved and abdominal angiographic findings were finally normalized. Although there are only three case reports on improvements in abdominal angiographic findings of PAN in the literature, our case and previously reported cases suggest that improvements in angiographic findings may reflect a good prognosis of PAN.     

Alteration of Connexin43 expression in a rat model of obesity-related glomerulopathy

It is accepted that alteration of connexin43 (Cx43) expression in glomeruli is a common pathological response in several forms of kidney diseases. To date, however the change of the Cx43 expression in obesity-related glomerulopathy (ORG) has not been reported. In this study, the alteration of Cx43 expression in the glomeruli of rat with ORG was defined. Five-week-old rats were fed with high-fat diet for 18 weeks to establish the ORG model, then the histological change of glomeruli, the foot process effacement of podocyte, the markers for podocyte injury (nephrin,podocin and WT1) and Cx43 expression in glomeruli were examined respectively. The results demonstrated metabolic disorder, hyperinsulinemia, systemic inflammation and microalbuminuria in ORG rats. There was significant hypertrophy, glomerular expansion and inflammatory cell infiltration in the kidney of ORG rats compared to the control group. Significant foot process effacement of the podocyte in the glomeruli, nephrin loss and density reduction were shown in the ORG rats, and Cx43 expression was significant upregulated in glomeruli of ORG rats compared to the control group. The results indicate the correlation of overexpressed Cx43 with the obesity related renal inflammation and suggest that Cx43 might be a potential target in the development of obesity related glomerulopathy.     

Spontaneous renal laceration as the presenting feature of polyarteritis nodosa in a patient with familial Mediterranean fever after hepatitis A infection

We report a life-threatening spontaneous renal laceration with no history of bleeding diathesis or any trauma in a patient with FMF after acute hepatitis A virus (HAV) infection. Right nephrectomy was inevitable and histological investigation of the removed right kidney revealed a polyarterits nodosa (PAN). This case underlines the possibility that simultaneous PAN and immunsupressive treatment besides colchicine should be considered for patients with FMF. Also, patients with FMF who are not immune may be vaccinated for HAV which could be a predisposing mechanism for vasculitic hemorrhage.     

Targeting podocyte-associated diseases

Injury to the podocytes is the initiating cause of many renal diseases, leading to proteinuria with possible progression to end-stage renal disease. Podocytes are highly specialized cells, with an important role in maintaining the glomerular filtration barrier and producing growth factors for both mesangial cells and endothelial cells. With their foot processes they cover the glomerular basement membrane, and form slit diaphragms with neighboring podocytes.Human podocytopathies include focal and segmental glomerulosclerosis, minimal change disease, membranous nephropathy, collapsing glomerulopathy and diabetic nephropathy. Research in the last two decades has demonstrated great progress in understanding the molecular mechanisms leading to podocytopathies. These include single gene defects in slit diaphragm proteins, but also discovery of apoptotic, enzymatic and other pathways involved in podocyte injury. With this progress, a great number of animal models is now available to study either specific podocytopathies, e.g. in mouse models with single gene mutations, or more general podocyte injury patterns, such as the lipopolysaccharide or protamine sulfate model of foot process effacement.In this review, the morphology of the glomerulus will be discussed, with a focus on the podocyte, its interactions with surrounding cells, and the highly differentiated slit diaphragm separating the apical from the basal membrane. We also provide an overview of human podocytopathies and animal models to study these diseases. In the last part we discuss targeted therapies addressing pathways and proteins affected in podocyte injury.     

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。