Pectus excavatum is a congenital chest wall deformity, commonly identified in early childhood, creating a "sunken chest" appearance. Over time, the deformity can worsen, thus impacting cardiopulmonary function and creating significant body image disturbance in patients. The Nuss procedure is a minimally invasive technique in which a curved steel bar is introduced underneath the sternum through small bilateral thoracic incisions to correct the deformity. Most studies regarding the procedure to date focus on outcomes and complications in pediatric patients, however, few studies discuss these results in adult patients. This systematic review aims to analyze common complications and outcomes in patients over the age of 18 who have not undergone any prior intervention for pectus excavatum. The most common complications experienced in adult patients were displacement of the implanted steel bar, infection of the surgical site, pneumothorax, pleural effusion, and chronic postoperative pain. Reoperation was common in patients with a displacement of the bar, chronic pain, and bleeding. Additionally, adult patients routinely required a higher number of steel bars to be placed to correct the deformity. Despite evidence that the rate of complications increases with age, the majority of adult patients in our included studies were satisfied with the outcome of the procedure with indications of improved self-image and reduced preoperative symptoms such as dyspnea on exertion, palpitations, chest pain, and depression.
Sirtuins (SIRT) are a class of histone deacetylases that regulate important metabolic pathways and play a role in several disease processes. Of the seven mammalian homologs currently identified, sirtuin 1 (SIRT1) is the best understood and most studied. It has been associated with several neurodegenerative diseases and cancers. As such, it has been further investigated as a therapeutic target in the treatment of disorders such as Parkinson’s disease (PD), Huntington’s disease (HD), and Alzheimer’s disease (AD). SIRT1 deacetylates histones such as H1 lysine 26, H3 lysine 9, H3 lysine 56, and H4 lysine 16 to regulate chromatin remodeling and gene transcription. The homolog has also been observed to express contradictory responses to tumor suppression and tumor promotion. Studies have shown that SIRT1 may have anti-inflammatory properties by inhibiting the effects of NF-κB, as well as stimulating upregulation of autophagy. The SIRT1 activators resveratrol and cilostazol have been shown to improve Alzheimer’s Disease Assessment Scale-Cognitive Subscale (ADAS-Cog) scores in AD patients. In this review, we aim to explore the various roles of SIRT1 with regard to neuroprotection and neurodegeneration.
Birt-Hogg-Dubé syndrome (BHDS) is a rare genetic disorder characterized by cutaneous, pulmonary, and renal manifestations. We present a unique case in which a combination of multiple chronic illnesses, genetic testing, and significant family history led to a diagnosis of BHDS. A 72-year-old male patient presented to their primary care physician with a persistent cough of four months and was admitted to the emergency department after the discovery of a left hydropneumothorax. The patient's medical history was significant for recurrent spontaneous bilateral pneumothoraces diagnosed over 20 years ago, chronic obstructive pulmonary disease (COPD), and many other systemic illnesses. A combination of the patient's significant past medical and family history led to a diagnosis of BHDS. Genetic testing was also done to confirm the diagnosis. Despite benign skin lesions being the most common finding, they are not always present, as seen in our case, which can result in missed diagnosis. Due to the autosomal dominant nature of BHDS, it is vital to make an accurate diagnosis to allow for proper genetic counseling, as the development of renal cancer is the leading cause of mortality.
Varicella-zoster virus (VZV) is a member of the alpha-herpesvirus family, which can occasionally cause severe neurological complications such as encephalitis. In this case report, we discuss a rare finding of VZV encephalitis in which an immunocompetent pediatric patient, vaccinated against varicella, presented with altered mental status and no vesicular rash. A 15-year-old male presented to the Emergency Department with progressively worsening altered mental status over the past three days. The patient's mother stated that he was exhibiting frequent memory lapses as well as the sudden loss of the ability to play the piano. After admission to the pediatric general floor, lumbar puncture was performed and cerebrospinal fluid analysis returned positive for VZV, confirmed by polymerase chain reaction. The patient was then started on intravenous (IV) acyclovir at a dose of 650 mg every 8 hours to treat VZV-induced encephalitis. While the patient continued to have intermittent episodes of confusion and headaches, his overall condition improved, and by day 4, he was able to resume playing the piano and ukulele. The patient was discharged on day 8 with no home medications, and a follow-up with this primary care physician was scheduled. This patient is one of only four recorded cases of VZV encephalitis in immunocompetent children. It is extremely rare to encounter pediatric patients with this diagnosis and, as such, can elude physicians when developing differential diagnoses. If VZV is suspected, a lumbar puncture should be performed promptly, and, if confirmed, IV acyclovir should be started. Furthermore, this case highlights the need for future research with regard to VZV and potential predisposing factors in immunocompetent patients.
Peritonitis is inflammation of the peritoneum that can arise from a number of complications affecting the lining of the abdominal wall and organs. Acute abdomen and peritonitis is a rare complication in a previously healthy woman following a seemingly uncomplicated normal full-term vaginal delivery. We report such a case in a 20-year-old gravida 2 para 2 (G2P2) woman of Guatemalan descent, who presented nine days postpartum following an uncomplicated delivery, to the emergency room with acute abdomen and associated systemic inflammatory reaction. Interventional radiology paracentesis was performed, yielding a milky, purulent peritoneal fluid with no visible organisms and negative cultures. Antibiotics and paracentesis were insufficient in managing her condition, which continued to worsen over the course of several days. Given her continued deterioration despite clinical intervention, she underwent an exploratory laparotomy and peritoneal lavage along with continued broad-spectrum antibiotics. Cultures continued to be negative but operative findings included diffuse fibrinous peritonitis with no obvious abscess or perforated abdominal viscus. Following surgical laparotomy, she recovered fully without any complications. We review the available literature regarding peritonitis, discuss its management, and speculate as to its cause in this case.
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