Lamberteskesen6118
Patients with chronic idiopathic hypoparathyroidism may develop neurological complications, including calcification of the basal ganglia and other areas of the brain. In Fahr's syndrome, intracranial calcification is associated with an underlying disorder such as hypo or hyperparathyroidism. We report the case of a 37-year-old gentleman, with a history of bilateral cataract surgery and seizures, who presented with a new episode of seizure and was found to have severe hypocalcemia and bilateral symmetric intracranial calcification due to previously diagnosed primary hypoparathyroidism. He had symptoms and signs mimicking ankylosing spondylitis (AS), but with negative radiological and serological findings, not fitting into the diagnosis of axial spondyloarthropathies (SpA), as per standard criteria. Patients with long-standing idiopathic hypoparathyroidism can have severe calcification of soft tissues and bones, including vertebrae and paravertebral soft tissues, causing inflammatory back pain and stiffness. It is vital to report such cases as their occurrence is rare, and physicians should be aware of the possibility while evaluating patients with inflammatory back pain. Treatment in these cases is directed towards hypocalcemia and underlying primary pathology rather than spondyloarthropathy.A spontaneous spinal epidural hematoma (SSEH) is a rare condition of intraspinal bleeding in the epidural space. It is often associated with disorders of anticoagulation, intraspinal tumors, vascular malformations, and pertinent to this case, anticoagulation therapy. This surgical emergency requires early diagnosis and management in order to minimize permanent neurologic deficits. We report the case of a 72-year-old female with a past medical history of paroxysmal atrial fibrillation treated with rivaroxaban who presented to the emergency department with acute-onset, midline, lower back pain with no known trauma or injury to the area. At the time of emergency department (ED) admission, the patient was fully ambulatory and alert and oriented. However, within hours, she developed bilateral lower extremity motor paralysis and diminished sensation with urinary and bowel incontinence. SSEHs are rare, progressive neurologic emergencies that can present with non-specific lower back pain. This condition presents a diagnostic challenge that can result in permanent neurologic defects if not recognized early. Emergency physicians regularly encounter patients with both acute lower back pain and atrial fibrillation. This case can contribute to the possibility of SSEH to a differential diagnosis.Trauma is the leading cause of morbidity and mortality for those under 45 years of age in the United States with half of the deaths in trauma being attributed to hemorrhagic shock. The use of enhanced care teams (ECTs) that include physicians in selective prehospital settings has allowed the delivery of advanced critical care interventions in the field. We present a unique case where a young driver involved in a motor vehicle accident was trapped under the weight of his vehicle, causing extended extrication time. An ECT from the closest trauma center was able to deliver massive transfusion and definitive airway care while the patient was being extricated. While previous literature regarding the benefit of ECTs has been debated, this case suggests a unique niche where rapid deployment of an ECT to the scene made a pronounced difference in survival of the patient.Takotsubo syndrome (TTS), also called broken heart syndrome, is an acute and transient cardiac wall motion abnormality of the left ventricle. The patient prototype is a post-menopausal woman with myocardial infarction-like symptoms (angina pectoris, breathlessness, palpitations, etc.) who has experienced sudden emotional or physical stress. Although prognosis is generally considered relatively benign, both complications and recurrence rates are not insignificant. Pathophysiological mechanisms underlying TTS are not entirely understood, but the sympathetic system over-activity has a leading role. Moreover, since emotional stress frequently triggers TTS and since precedent diagnosis of psychiatric disorders sometimes coexists, the psychological response to stress could be another potential therapeutic target. Indeed, this article aims to explore the association between underlying depression and anxiety disorders and TTS, as well as to find ideal therapeutic options useful to treat and prevent TTS. Thus in our review, we considered case reports, case-control studies, and review articles from PubMed. Papers dealing with Takotsubo syndrome and anxiety disorder or depression were selected. We included papers published since 2010 and whose abstract was in English. We concluded that anxiety disorders, but not depression, are associated with a higher occurrence of TTS. There is a link between anxiety, TTS, and inflammation leading to increased sympathetic activity. Nevertheless, patients with pre-admission psychiatric disorders have a higher risk of recurrent TTS. Importantly, the use of selective serotonin reuptake inhibitors (SSRIs) could be a potential therapeutic aid in preventing TTS's recurrence in selected patients.Atypical hemolytic uremic syndrome (aHUS) is a rare but life-threatening pediatric disease caused by uncontrolled activation of the alternative complement pathway related to genetic mutations and carries a worse prognosis. In the last decade, a monoclonal antibody against complement C5, eculizumab, has dramatically improved the disease outcomes. The complement mutations in aHUS are detected only in 60%-70% of cases in previous studies. We report a severe presentation of aHUS diagnosed in a two-month-old child who presented with seizures, renal failure with anuria, and microangiopathic hemolytic anemia and required peritoneal dialysis soon after admission. The patient was clinically diagnosed having aHUS and was started on eculizumab on day 4 of hospital admission. The genetic study for major known complement mutations causing aHUS was reported negative. He had a major episode of disease relapse associated with seizures four weeks after eculizumab therapy and required prolonged peritoneal dialysis over more than two months at the time of initial admission. He developed dilated cardiomyopathy and oro-motor dysfunction as complications of aHUS. At five-year follow-up, the patient has stage 3 chronic kidney disease (CKD), proteinuria, hypertension, and required G-tube for feeds. Gemcitabine This report discussed the long-term outcome of an infant diagnosed with aHUS and tested negative for common complement mutations on eculizumab therapy. More research is needed to identify novel genes and antibodies contributing to aHUS. While the eculizumab is expensive, and the duration of treatment is not definite, the clinical severity of the disease, relapses, and presence of long-term renal complications are essential factors to decide treatment continuation.Background and objectives Recent experimental and clinical evidence supporting early debridement for open fractures has been questioned. Therefore, this systematic review and meta-analysis aimed to summarize and evaluate the current evidence regarding the timing of surgical debridement of open tibial fractures. Methods A systematic review and meta-analysis were conducted on studies compared the infection rate following early versus late debridement of open tibial fractures. We performed an online, bibliographic, search through the period from January 2000 to June 2020 in five bibliographic databases Cochrane Central Register of Controlled Trials (CENTRAL), Medline via PubMed, Web of Science, Scopus, and EBSCO host. Results Nine retrospective studies and six prospective studies were included in the present meta-analysis study. The pooled effect estimate showed no statistically significant difference between early and late debridement regarding the overall infection rate (RD 0.02, 95% CI [0 - 0.04], p = 0.94); elayed versus early debridement.Langerhans cell histiocytosis (LCH), formally referred to histiocytosis X, is a histiocytic disorder with unknown etiology. The pathogenesis is believed to originate from myeloid dendritic cells and is now considered an inflammatory myeloid neoplasm within the revised 2016 Histiocyte Society classification. Pulmonary Langerhans cell histiocytosis (PLCH) is a rare and isolated form of LCH with a strong affiliation with smoking in adults of 20-40 years of age. Characteristic CT chest and histologic findings are instrumental in the early recognition and management of a disease. We herein report a case of a Caucasian smoker female with a significant history of interstitial lung disease (ILD) presented with recurrent and progressive worsening dyspnea. History of ILD and recurring respiratory symptoms raised suspicion of PLCH. CT chest and pathological findings confirmed the diagnosis, and discontinuation of smoking resulted in favorable clinical outcomes.Background Open rhinoplasty, including the transcolumellar approach, is commonly performed as it has fewer side effects and provides superior anatomical control to the surgeons compared to closed rhinoplasty. However, the postoperative scar outcomes, such as scar appearance, vary depending on the type of suture used in wound closure, and the optimal suture type is not firmly established. Objective To compare the impact of catgut versus polypropylene sutures on the postoperative transcolumellar scar outcomes and patient satisfaction following open rhinoplasty. Methods This retrospective cohort study, including 100 patients who underwent transcolumellar open rhinoplasty, was conducted at otolaryngology department of King Abdulaziz University Hospital, Riyadh, KSA. The patients were divided into two groups the propylene suture group (group 1), which included 15 males and 35 females with a mean age of 31.5 years and underwent surgery using propylene sutures, and the catgut suture group (group 2), which included 10 males and 40 females with a mean age of 30.5 years and underwent surgery using catgut sutures. The postoperative transcolumellar scar outcomes, as determined by visual analogue scale (VAS) and Stony Brook Scar Evaluation Scale (SBSES) scores, and patient satisfaction, as assessed using a self-assessment scale, were compared between groups. Results The scars were unnoticeable in the majority of both groups 88% in group 1 and 86% in group 2. The VAS and SBSES scores did not significantly differ between groups. Patients' satisfaction rates were also comparable and did not significantly differ between groups (p = 0.341). Conclusion Both catgut and polypropylene sutures lead to similar outcomes and patient satisfaction rates in terms of postoperative rhinoplasty transcolumellar scars. Thus, catgut may be the optimal suture for closing transcolumellar incisions following open rhinoplasty.Cornelia de Lange (CdLS) is a rare congenital disorder with multifactor etiology. The syndrome features a wide variety of physical and cognitive hallmarks such as distinctive facial appearance, small stature, bone, and gastrointestinal abnormalities. Two cases of patients clinically diagnosed with CdLS are reported. Both cases were diagnosed and treated at the St. Marina University Hospital, Varna, Bulgaria and were referred for autopsy after death. The first case was of a female patient, aged 7 and the second of a 17-years-old female. Both patients had a family history, severe features, and complications associated with CdLS. In both cases, the liver had normal anatomical proportions with a slightly flattened shape especially pronounced in the second case where the liver had a pyramidal shape with sharp edges. The kidneys in both patients were with a flattened pyramidal shape, with the tip located at the hilum and a base toward the lateral abdominal side. Both patients also had a pyramidal shaped spleen, again with the tip located at the hilum, with the second patient having multiple accessory spleens along the splenic artery.