Addison’s disease (AD), also known as primary adrenal insufficiency, is a rare autoimmune disorder affecting males and females equally. The most common cause of AD is autoimmune adrenalitis and other causes include metastatic cancers, tuberculosis and acquired immunodeficiency syndrome. AD presents with a wide variety of signs and symptoms and thus, making a diagnosis challenging. The common symptoms of this disease include weakness and fatigability, orthostatic hypotension, nausea, vomiting, diarrhea, anorexia and weight loss. Addison’s disease often presented with other autoimmune disorders, such as autoimmune polyglandular syndrome. We herein report a case of a patient who presented in a hospital emergency department, with Addisonian crisis and predominant neuropsychiatric manifestation. On review of the patient's history, combined with biochemical testing, a diagnosis of Addison’s disease was established. This type of presentation is relatively uncommon.
Objective Various options exist for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined. Design We performed a retrospective review of all non‐Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression. Results Between January 2002 and December 2015, there were 182 patients identified. Sixteen patients were lost to follow‐up and 3 patients died, unrelated to cardiac surgery. Of the remaining 163 patients, the median age was 12.2 years (interquartile range 6.4‐16.4), median weight was 39.0 kg (interquartile range 19.9‐59.3), and the median body surface area was 1.23 m2 (interquartile range 0.79‐1.64). Ninety‐three (57%) patients had tetralogy of Fallot. The median follow‐up was 5.4 years (interquartile range 2.9‐8 years). There were no operative or cardiac‐related deaths. Thirty‐eight patients (23%) required reintervention. The rate of freedom from reintervention was 93.2% (95% CI 86.7%‐96.6%) at 5 years and 48.4% (95% CI 34.9%‐60.6%) at 10 years. Age < 10 years, weight < 39 kg, and body surface area <1.2 m2 at the time of valve placement, as well as valve size ≤25 mm were significantly associated with need for earlier reintervention. Conclusions The Freestyle root in the RVOT is associated with excellent survival and low midterm need for reintervention. Its longevity is comparable to published data on homografts and other bioprosthetic valves.
Iftikhar et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Background: Endoscopic procedures are frequently performed to rule out any disease process which eventuates with either a positive or negative outcome. Association of patient demographics, clinical features with endoscopic outcomes are of paramount importance for better understanding and practice of endoscopy. Objectives: This study aimed to determine the frequency of positive endoscopic findings in patients with gastrointestinal symptoms compared to those with negative findings. As a secondary objective, the association between relevant demographics, symptoms, laboratory investigations and procedural outcomes of the participants and positive endoscopic findings were also assessed. Methods: A retrospective analysis of all the patients who underwent esophagogastroduodenoscopy from January 2017 to December 2017 was conducted. A total of 1066 endoscopy records were retrieved and using a convenience sampling technique, relevant data were manually entered to the questionnaires. Records with incomplete or inconsistent data were disregarded as per the exclusion criteria, resulting in 1011 patient records ultimately utilized for the study. All data were entered and analyzed using IBM SPSS 23.0. Results: The study comprised a total of 1011 patients where 52.2% were females and 47.8% males while the mean age of patients was 42.16 ± 15.45. Positive endoscopic findings were observed in 88.1%, where the most common finding was gastritis (49.1%). Approximately 14.5% had positive investigation findings for HBsAg and 23.1% were seropositive for anti-HCV antibody.
Laurence-Moon-Bardet-Biedl syndrome (LMBBS) is a rare autosomal recessive (AR) disorder. It is characterized predominantly by hypogonadism, polydactyly, retinitis pigmentosa, obesity, and mental retardation. Herein, we present a classic case of LMBBS with generalized body edema, abdominal distension, and positive fluid thrill in a 32-year-old male. LMBBS patients are friendly with a happy predisposition, proper management, and regular examinations should be done in order to maintain healthy organ function and to avoid an early death. Renal failure is the most common cause of mortality in LMBBS patients.
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