Cutaneous lupus erythematosus: Understanding of clinical features,genetic basis,and pathobiology of disease guides therapeutic strategies

Cutaneous features of the protean disease lupus erythematous (LE) constitute 4 of 11 diagnostic criteria for systemic lupus erythematosus (SLE) and are exhibited by approximately 3/4 of patients during the course of their disease. Because the pathogenesis of LE is multifactorial and polygenic, many of the details of the pathogenesis remain unclear. We review here the clinical features of cutaneous lupus and recent genetic data that elucidate potential candidate genes for both cutaneous lupus erythematosus (CLE) and SLE. We discuss advances in elucidating the autoimmune pathogenesis of CLE and SLE. Furthermore, promising experimental therapies based on these advances are reviewed in the context of B cell directed therapies, T cell directed therapies, disruption of B and T cell interactions, cytokine directed therapies and finally, end-effector targeted therapies.     

Autism as a disorder of prediction

A rich collection of empirical findings accumulated over the past three decades attests to the diversity of traits that constitute the autism phenotypes. It is unclear whether subsets of these traits share any underlying causality. This lack of a cohesive conceptualization of the disorder has complicated the search for broadly effective therapies, diagnostic markers, and neural/genetic correlates. In this paper, we describe how theoretical considerations and a review of empirical data lead to the hypothesis that some salient aspects of the autism phenotype may be manifestations of an underlying impairment in predictive abilities. With compromised prediction skills, an individual with autism inhabits a seemingly “magical” world wherein events occur unexpectedly and without cause. Immersion in such a capricious environment can prove overwhelming and compromise one’s ability to effectively interact with it. If validated, this hypothesis has the potential of providing unifying insights into multiple aspects of autism, with attendant benefits for improving diagnosis and therapy.     

Gossypiboma mimicking posterior urethral stricture

INTRODUCTIONForeign bodies in the urogenital tract are not uncommon. Hairpins, glass rods, umbilical tapes, ball point pen are described in lower urogenital tract. Retained gauze piece (gossypiboma) in posterior urethra may cause diagnostic dilemma. Symptoms and investigations may mimic stricture of posterior urethra.PRESENTATION OF CASETwo cases of retained gauze pieces in the urethra are described here. The micturating cystourethrogram was suggestive of posterior urethral stricture.DISCUSSIONTwo cases described here had retained gauze piece as a cause of filling defect and abnormal appearance in the micturating cystourethrogram. Gossypiboma may be a possibility where posterior urethral stricture are seen after previous surgery in paediatric age group.CONCLUSIONIn the setting of previous urogenital surgery gossypiboma should be kept in the differential diagnosis where posterior urethral stricture are seen in the paediatric age group.     

Resistant Hypertension

Hypertension affects 25 % of all adults worldwide and is a leading risk factor contributing to 62 % of all strokes and 49 % of all cases of heart disease, leading to an estimated 7.1 million deaths a year; equivalent to 13 % of total worldwide deaths. In spite of therapeutic advances, up to 30 % of hypertensive individuals fail to achieve goal blood pressure even with the use of three antihypertensive medications. Resistant hypertension (RH) is a common clinical problem faced by physicians and the incidence is increasing as the population becomes heavier and older. The diagnosis and treatment of RH, is often accompanied by other risk factors such as obesity, sleep apnea, diabetes and chronic kidney disease is important because of the associated increased end organ damage and the subsequent clinical and social impact. Pseudo resistance, lack of blood pressure control due to poor medication adherence or white coat hypertension must be excluded. A successful treatment of RH requires identification of contributing lifestyle factors and eliminating them including the use of multidrug therapy. A potential genetic causes of RH have not been well studied. African American (black) race and certain other ethnic groups are associated with higher prevalence of RH and also poor response to therapy. Studies on RH are limited, in part because of difficulties in enrolling large groups of patients and patient comorbidity, higher cardiovascular risk and other diseases, e.g. sleep apnea, diabetes and chronic kidney disease that can confound the interpretation of study results. This review provides an overview of RH, and its association with risk factors, various ethnic groups, diagnosis and treatment modalities of RH with special emphasis on the relations of the latter with race/ethnicity.     

Long-term single procedure efficacy of catheter ablation of atrial fibrillation

Background Two important limitations of the data regarding the outcomes of catheter ablation of atrial fibrillation (AF) are the short-term follow-up used in most published studies and the lack of single-procedure outcomes.Objective The objective was to report the long-term single-procedure outcomes at our center.Materials and methods The patient population was comprised of 200 consecutive patients who underwent ablation (133 men; age 56 ± 11 years). Atrial fibrillation was paroxysmal in 92 (46%). Success was defined as absence of symptomatic AF, off antiarrhythmic drug (AAD) after a single procedure.Results After a follow-up of 26 ± 11 months, the single-procedure long-term success rate was 28% with an additional 7% of patients demonstrating improvement. After including repeat procedures in 64 patients, the overall long-term success rate was 41% with 11% demonstrating improvement. Further subgroup analysis of 48 paroxysmal AF patients considered to be optimal candidates for the procedure, revealed a long-term success rate of 69% with an additional 4% demonstrating improvement. A major complication occurred in 7.9% of patients.Conclusion The results reveal that the long-term single-procedure success rate of catheter ablation of AF in a cohort of patients with predominantly non-paroxysmal AF is less than 40%. The inclusion of redo procedures resulted in an improvement in outcomes. A much higher success rate of 69% was achieved in patients with paroxysmal AF considered to be optimal candidates for this procedure. These results make it clear that further advances in the technique of catheter ablation of AF are needed to improve the safety and efficacy of this procedure. In order to be able to compare outcomes of various techniques in differing patient populations, we urge investigators to report long-term single procedure outcomes.This study was supported by The Norbert and Louise Grunwald Cardiac Arrhythmia Research Fund.     

An elevated C-reactive protein level in an inpatient rehabilitation setting after joint replacement: To act or not to act? – that is the question

C-reactive protein (CRP) is part of a battery of “routine bloods” performed by residents on patients when they are admitted into a rehabilitation unit. Generally, an elevated CRP is considered to be an indicator of an acute infective process. Numerous studies have indicated that the CRP peaks on the 2nd or 3rd day post total hip arthroplasty (THR) and total knee arthroplasty (TKR) and returns to normal by day 7. When the CRP level remains elevated, it is generally felt that infection should be excluded.We performed a prospective study on 45 consecutive patients admitted into a rehabilitation unit post hip and knee arthroplasty over a 6 months period, to evaluate the incidence of an elevated CRP on admission, to determine whether an isolated elevated CRP on admission to a rehabilitation setting should not be considered as an indicator of an infective process.We found all patients (100%) had elevated CRP''s on admission, ranging from 8.6 mg/L to 139.2 mg/L, between days 5–7 post-operatively. By day 14, CRP''s reduced, but 91% of patients still had elevated CRP''s, ranging from 2.1 mg/L to 47.3 mg/L after THR and 4.8 mg/L to 40 mg/L after TKR at day 14.These results suggest that even in uncomplicated elective joint arthroplasty, CRP''s can remain elevated up to 14 days post-procedure, in the absence of an infective process.An isolated elevated CRP on admission to a rehabilitation setting should not be considered as an indicator of an infective process, but rather part of the normal post-operative inflammatory response. The elevated CRP should be monitored and only an upward trend requires further investigation and management.