The First Case Report of Schnitzler Syndrome Presenting with Eye Pain

ABSTRACT

Schnitzler syndrome is a rare, auto inflammatory condition known to manifest with bone pain, urticarial rash, fevers, relapsing arthralgia, and fatigue. In this case report, we describe a patient who was diagnosed with Schnitzler Syndrome that had initially presented with a unilateral pressure-type headache with a sensation of a ‘dagger’ stabbing into the back of the eye. He also had an associated ipsilateral redness of the conjunctiva, eyelid swelling, subtle optic disc elevations bilaterally and facial flushing - but with no visual acuity, pupillary, or lacrimatory changes. Anterior segment, fundoscopy, intraocular pressures and extraocular muscle movements were otherwise normal.     

Predicting relapse in schizophrenia: the development and implementation of an early signs monitoring system using patients and families as observers, a preliminary investigation

Recognition of prodromal symptoms of schizophrenia offers the potential of early intervention to avert relapse and re-hospitalization (Carpenter & Heinrichs, 1983). The present study investigated how a strategy to detect prodromal signs might be effectively applied in the clinical setting. A standard monitoring system was developed involving completion of a new early signs scale (ESS) measuring changes in key symptoms phenomenologically (self-report) and behaviourally (observer report). The ESS was subject to rigorous psychometric evaluation and tested in a prospective pilot investigation. The ESS reliably identified early signs and predicted relapse with an overall accuracy of 79%. Several different patterns of relapse were identified. Observer reports compensated for loss of insight in some patients. In two cases where early signs indices were detected, prompt increases in medication appeared to arrest relapse and avert readmission. The ESS offers itself as a reliable, valid and administratively feasible measure and demonstrates considerable potential as a cost-effective procedure for secondary prevention.     

Emerging drugs for prevention of T-cell mediated rejection in liver and kidney transplantation

Introduction: Acute and chronic graft rejection continues to be an important problem after solid organ transplantation. With the introduction of potent immunosuppressive agents such as calcineurin inhibitors, the risk of rejection has been significantly reduced. However, the adverse effects of life-long immunosuppression remain a concern, and there exist a fine balance between over-immunosuppression and risk of rejection.

Areas covered: In this review, the current standard of care in immunosuppressive therapy, including the use of steroids, calcineurin inhibitors, mycophenolate prodrugs and mammalian target of rapamycin inhibitors, will be discussed. Newer immunosuppressive agents showing promising early data after liver and kidney transplantation will also be explored.

Expert Opinion: Currently, calcineurin inhibitors continue to be a vital component of immunosuppressive therapy after solid organ transplantation. Although minimization and avoidance strategies have been developed, the ultimate goal of inducing tolerance remains elusive. Newer emerging agents should have potent and specific immunosuppressive activity, with minimal associated side effects. An individualized approach should be adopted to tailor immunosuppression according to the different needs of recipients.     

Unique changes in interstitial extracellular matrix composition are associated with rejection and cyclosporine toxicity in human renal allograft biopsies

Renal allograft loss from chronic rejection or cyclosporine toxicity (CsAT) is characterized by progressive interstitial fibrosis, yet the protein composition of these lesions is unknown. The normal tubular basement membrane (TBM) contains laminin (LM), collagen IV (containing collagen IV alpha chain 1 [COL4A1] and COL4A2), thrombospondin (TSP), and fibronectin (FN). Only TSP and FN extend beyond the TBM into the interstitial space. Very scanty amounts of interstitial collagens (I and III) are detected in the interstitium. In a pilot study of human renal allograft biopsy specimens, three patterns of extracellular matrix (ECM) composition were identified. Pattern 1 showed no change in ECM composition; pattern 2 showed generalized accumulation of collagens I and III in the interstitium; and pattern 3 showed new expression of COL4A3 and LM-beta2 in the proximal TBM. Criteria were established for the clinicopathological diagnosis of CsAT and rejection. These diagnoses were correlated with the ECM composition in 22 renal allograft biopsy specimens. Control groups were examined in a similar manner and included native kidney biopsy specimens from patients with other allografts (n = 7), renal biopsy specimens from patients with glomerular disease (n = 9), and renal allograft biopsy specimens from patients without clinicopathological evidence of renal disease. These data show that rejection is associated with pattern 3 and CsAT is associated with pattern 2. Thus, detection of ECM composition may be a useful adjunct to standard microscopy in distinguishing rejection from CsAT in renal allograft biopsy specimens. These data suggest that interstitial fibrosis associated with rejection and CsAT result from different pathogenic mechanisms.     

开放性结肠直肠切除术后的腹内游离气体

立位Ｘ线胸片可敏感而特异地显示膈下游离气体 ,对开放性结肠直肠切除术后空腔脏器出现破损是一项有效的检查 ,但由于剖腹术后气腹自然吸收所需要的时间未知 ,其作用尚未被确定。本次试验的目的即在于确定这一时间。　　 3个月内连续 92例接受了连续性结肠直肠切除术的病人 (8例拒绝合作 ,9例采用了腹腔镜技术 )中的 75例参与了这次试验 (平均年龄 6 2 1岁 ,男∶女 =1 3∶1) ,手术指征分别为直肠癌 6例 ,憩室病 2例 ,肠道炎症 2例 ,放疗引起狭窄 3例 ,功能性疾患 (慢性运输性便秘 ,直肠脱垂 ) 2例 ,其它 (家族性腺瘤息肉、乙状结肠扭转、短…     

树突状细胞与肝脏疾病

免疫反应的产生首先是由抗原提呈细胞(antigenpresenting cells,APC)捕获抗原,经其加工处理后将抗原信息传递给T,B淋巴细胞,从而引发一系列的特异性免疫应答.APC包括树突状细胞(dendritic cells,DC)、巨噬细胞(MΦ)、B细胞等,其中DC是人体内最具潜能的抗原提呈细胞(APC),能在体内外直接激活纯真(naive)T细胞,提呈抗原给MHC-Ⅰ类限制性CD8+和MHC-Ⅱ类限制性CD4+T淋巴细胞,诱导特异性免疫应答[1-6].     

Computed tomography appearances of the lung parenchyma in pulmonary hypertension

Computed tomography (CT) is a valuable tool in the workup of patients under investigation for pulmonary hypertension (PH) and may be the first test to suggest the diagnosis. CT parenchymal lung changes can help to differentiate the aetiology of PH. CT can demonstrate interstitial lung disease, emphysema associated with chronic obstructive pulmonary disease, features of left heart failure (including interstitial oedema), and changes secondary to miscellaneous conditions such as sarcoidosis. CT also demonstrates parenchymal changes secondary to chronic thromboembolic disease and venous diseases such as pulmonary venous occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH). It is important for the radiologist to be aware of the various manifestations of PH in the lung, to help facilitate an accurate and timely diagnosis. This pictorial review illustrates the parenchymal lung changes that can be seen in the various conditions causing PH.