Management of High-Grade Coronary Artery Disease and Concomitant Glanzmann Thrombasthenia.

In the present case report, we describe the management of severe coronary artery disease in a patient with Glanzmann thrombasthenia. To the best of our knowledge, there are no established guidelines for revascularization in this setting, and we pose novel discussion points regarding the nuanced care of this patient. (Level of Difficulty: Intermediate.).

Aspiration fluid cytology during single operator cholangioscopy with targeted biopsy to improves the diagnostic yield in indeterminate biliary strictures.

INTRODUCTION: Sensitivities of various sampling methods to detect malignant biliary strictures remain suboptimal. Irrigation during digital single operator cholangioscopy (dSOC) is done routinely for visualization of the duct. The aim of this study was to evaluate improvement of the sensitivity for detecting malignant biliary strictures when adding aspiration fluid cytology (AFC) from the irrigated fluid during cholangioscopy to cholangioscopic biopsy (CBx). METHODS: We conducted a retrospective analysis of patients at a tertiary medical center who underwent CBx for evaluation of their biliary strictures. We included patients who had aspiration of fluid from the bile duct after CBx and were sent for cytology from January 2017 to October 2017. Diagnosis was made on the basis of final pathology or follow-up over 9 months. RESULTS: Fifty-six patients had CBx obtained, out of which 35 patients had AFC in conjunction. Twenty-two (62%) patients were male and the average age was 65 years. Considering atypical cells as benign, the sensitivity, specificity, positive and negative predictive values (PPV, NPV) for CBx were 62.5%, 100%, 100%, and 76% respectively. When CBx combined with AFC, the above statistics went up to 81.25%, 100%, 100%, and 86.36% respectively. When atypical cells were considered malignant, the sensitivity, specificity, PPV and NPV for CBx were 81.25%, 84.21%, 81.25%, 84.21% and increased to 93.75%, 78.94%, 78.94%, and 93.75% respectively after adding AFC results. CONCLUSION: For patients with biliary stricture, addition of AFC dSOC guided biopsies, significantly improves the sensitivity for detecting malignancy.

A Case of Waardenburg-Shah Syndrome Type 4 Presenting with Bilateral Homochromatic Blue Irises from Pakistan.

Waardenburg syndrome (WS) is a rare genetic disorder. It is caused by multiple mutations affecting the melanocytes, leading to a multitude of skin, hair, and eye symptoms. It is an autosomal dominant disease with four subtypes, each presenting with varying degrees of sensorineural hearing loss along with a constellation of other symptoms. Hirschsprung disease is unique to Waardenburg-Shah syndrome subtype 4 and is not associated with any other subtype. We present a case of this subtype 4 that presented with a bilateral sensorineural hearing loss, mutism, delayed milestones, white forelock, Hirschsprung disease, and bilateral blue homochromatic irises, a finding which is not typical for this subtype. This is the first case of WS with homochromatic irises and the fourth case to be reported from Pakistan.

Conversion Disorder- Mind versus Body: A Review.

In this article, the authors accentuate the signs and symptoms of conversion disorder and the significance of clinical judgment and expertise in order to reach the right diagnosis. The authors review the literature and provide information on the etiology, prevalence, diagnostic criteria, and the treatment methods currently employed in the management of conversion disorder. Of note, the advancements of neuropsychology and brain imaging have led to emergence of a relatively sophisticated picture of the neuroscientific psychopathology of complex mental illnesses, including conversion disorder. The available evidence suggests new methods with which to test hypotheses about the neural circuits underlying conversion symptoms. In context of this, the authors also explore the neurobiological understanding of conversion disorder.

Insight into delirium.

Delirium is a common and serious disorder with high morbidity and mortality. It occurs in 35 to 80 percent of critically ill, hospitalized patients. It may lead to mortality if not detected early. Studies show that delirium causes death in as many as 22 to 76 percent of patients who are hospitalized with delirium. It is often unrecognized and inadequately treated, and this may lead to poor outcome, including long-term care, longer length of stay in hospital, and high impact on health and social expenditure. There may be many reasons that delirium goes undetected, such as not understanding full pathophysiology and not having enough diagnostic tools to detect delirium in the early phase. A clear understanding of neurochemical equilibrium and pathways of the brain will help the clinician to understand the signs and symptoms of delirium. Pathophysiology of delirium is complex, and multiple theories have been proposed to explain its exact pathophysiology, but none of these mechanisms have been fully understood. Early detection of delirium and reduction of modifiable risk factors, along with better management, can result in better outcomes. This article discusses the pathophysiology and parts of brain involved in delirium as well as mood and psychotic symptoms of delirium.