Adding Insult to Injury: Sleep Deficiency in Hospitalized Patients.

Sleep deficiency is a common problem in the hospital setting. Contributing factors include preexisting medical conditions, illness severity, the hospital environment, and treatment-related effects. Hospitalized patients are particularly vulnerable to the negative health effects of sleep deficiency that impact multiple organ systems.

Monitoring and Management of Respiratory Function in Pompe Disease: Current Perspectives.

Pompe disease (PD) is a neuromuscular disorder caused by a deficiency of acid alpha-glucosidase (GAA) - a lysosomal enzyme responsible for hydrolyzing glycogen. GAA deficiency leads to accumulation of glycogen in lysosomes, causing cellular disruption. The severity of PD is directly related to the extent of GAA deficiency - if no or minimal GAA is produced, symptoms are severe and manifest in infancy, known as infantile onset PD (IOPD).

Adding Insult to Injury: Sleep Deficiency in Hospitalized Patients.

Sleep deficiency is a common problem in the hospital setting. Contributing factors include preexisting medical conditions, illness severity, the hospital environment, and treatment-related effects. Hospitalized patients are particularly vulnerable to the negative health effects of sleep deficiency that impact multiple organ systems.

Expanding small-molecule target space to mRNA translation regulation.

Multiple layers of regulation are in place on mRNA translation to ensure that cells respond in a fast manner to environmental cues in a tissue-specific and mRNA-selective manner. Here, we discuss mRNA translation regulatory mechanisms and potential drug-intervention targets. Taking on a new scientific rational of translation regulation and consequently a new target space, we have developed a unique discovery platform that is able to identify selective small molecule drugs that affect translation of specific proteins.

Pneumopericardium, pneumomediastinum and air travel: A case report in a patient with Gardner syndrome.

Onboard aircraft medical emergencies are on the rise as commercial air traffic is increasing. However, thoracic injury secondary to air travel is extremely rare and, most reported injuries are cases of pneumothoraces. Spontaneous pneumomediastinum and pneumopericardium have been barely reported in the medical literature as a complication of air travel.

A Case of Laryngeal Tuberculosis, Endobronchial Tuberculosis and Pulmonary Tuberculosis Coexistent in an Immunocompetent Host.

Historically associated with poor prognosis seen in advanced disease, laryngeal tuberculosis (LTB) now represents only 1% of all cases of tuberculosis (TB). The incidence of LTB has decreased drastically with the introduction of anti-tubercular drugs. LTB can be primary or secondary to pulmonary tuberculosis.

Outbreak of Electronic-Cigarette-Associated Acute Lipoid Pneumonia - North Carolina, July-August 2019.

On September 6, 2019, this report was posted as an MMWR Early Release on the MMWR website (https://www.cdc.gov/mmwr).

Left-sided Catamenial Pneumothorax: A Rare Clinical Entity.

Catamenial pneumothorax (CP) is an extremely rare pulmonary pathology seen in women of reproductive age, typically occurring within 72 hours from the onset of menstrual bleeding. Multiple theories have been proposed to explain the etiopathogenesis of CP; however, the exact underlying mechanism remains elusive. More than 90% of reported cases in the literature describe a right-sided presentation of pneumothorax.

From Bouveret's Syndrome to Gallstone Ileus: The Journey of a Migrating Stone!

Bouveret's syndrome, first described in 1896, is an unusual cause of gastric outlet obstruction secondary to large gallstone impaction in the proximal duodenum after migration through a cholecystoduodenal fistula. Stone migration has been previously described after endoscopic or surgical fragmentation. However, this is the first reported case, in our knowledge, where the stone migrated after oral contrast administration a few days after the onset of symptoms, causing a distal gallstone ileus.

Bouveret's Syndrome: Literature Review.

It was in 1896 that Bouveret's syndrome acquired its name after the French physician Leon Bouveret, who published two case reports in Revue de Medecin. Bouveret's syndrome describes gastric outlet obstruction secondary to an impacted gallstone. The gallstone reaches the small bowel through a bilioenteric fistula as a consequence of chronic inflammation and adherence between the biliary system and the bowels which increase the intraluminal pressure and leads to secondary wall ischemia and wall perforation with gallstone passage into the bowel.

A Case of Transudative Chylothorax: A Diagnostic Dilemma.

Chylothorax is a type of pleural effusion characterized by the presence of chyle in the pleural space with triglyceride levels >110 mg/dL or evidence of chylomicrons in pleural fluid. Chylous effusion is typically exudative in nature with lymphocytic predominance. Transudative chylothorax is a rare finding which has been associated with only a limited range of clinical settings.

Relevance of B-Lines on Lung Ultrasound in Volume Overload and Pulmonary Congestion: Clinical Correlations and Outcomes in Patients on Hemodialysis.

Background: Volume overload in patients on hemodialysis (HD) is an independent risk factor for cardiovascular mortality. B-lines detected on lung ultrasound (BLUS) assess extravascular lung water. This raises interest in its utility for assessing volume status and cardiovascular outcomes.

Structural Basis for the Inhibitory Effects of Ubistatins in the Ubiquitin-Proteasome Pathway.

The discovery of ubistatins, small molecules that impair proteasomal degradation of proteins by directly binding to polyubiquitin, makes ubiquitin itself a potential therapeutic target. Although ubistatins have the potential for drug development and clinical applications, the lack of structural details of ubiquitin-ubistatin interactions has impeded their development. Here, we characterized a panel of new ubistatin derivatives using functional and binding assays.

Intercostal Artery Laceration: Rare Complication of Thoracentesis and Role of Ultrasound in Early Detection.

Hemothorax is a rare but potentially fatal postthoracentesis complication. Early clinical signs may be nonspecific resulting in diagnostic delay. A high index of suspicion is vital for early diagnosis and intervention to avoid further bleeding.

Fungal Bezoar: A Rare Cause of Ureteral Obstruction.

A 52-year-old male, with diabetes mellitus and alcoholic liver disease, presented to the Emergency Room for right flank pain of 3 days' duration, associated with dysuria. Physical examination revealed right flank tenderness with fever and hypotension; laboratory findings showed acute kidney injury and large blood and leucocytes in the urine. A CT abdomen and pelvis showed hydronephrosis of the right collecting system of a horseshoe kidney with air and hyperdense debris in the renal pelvis.

Polyubiquitin-Photoactivatable Crosslinking Reagents for Mapping Ubiquitin Interactome Identify Rpn1 as a Proteasome Ubiquitin-Associating Subunit.

Ubiquitin (Ub) signaling is a diverse group of processes controlled by covalent attachment of small protein Ub and polyUb chains to a range of cellular protein targets. The best documented Ub signaling pathway is the one that delivers polyUb proteins to the 26S proteasome for degradation. However, studies of molecular interactions involved in this process have been hampered by the transient and hydrophobic nature of these interactions and the lack of tools to study them.

Left Lung Torsion: Complication of Lobar Resection for an Early Stage Lung Adenocarcinoma.

Lobar torsion is a fatal but fortunately rare occurrence following lung resection. Early clinical signs and radiographic features may be nonspecific resulting in diagnostic delay. A high index of suspicion is vital for early diagnosis and intervention to avoid further parenchymal necrosis and deadly gangrene.

Base-CP proteasome can serve as a platform for stepwise lid formation.

26S proteasome, a major regulatory protease in eukaryotes, consists of a 20S proteolytic core particle (CP) capped by a 19S regulatory particle (RP). The 19S RP is divisible into base and lid sub-complexes. Even within the lid, subunits have been demarcated into two modules: module 1 (Rpn5, Rpn6, Rpn8, Rpn9 and Rpn11), which interacts with both CP and base sub-complexes and module 2 (Rpn3, Rpn7, Rpn12 and Rpn15) that is attached mainly to module 1.

Chemical synthesis of phosphorylated ubiquitin and diubiquitin exposes positional sensitivities of e1-e2 enzymes and deubiquitinases.

Modification of ubiquitin by phosphorylation extends the signaling possibilities of this dynamic signal, as it could affect the activity of ligases and the processing of ubiquitin chains by deubiquitinases. The first chemical synthesis of phosphorylated ubiquitin and of Lys63-linked diubiquitin at the proximal, distal or both ubiquitins is reported. This enabled the examination of how such a modification alters E1-E2 activities of the ubiquitination machinery.

Abnormal cytoplasmic extensions associated with active αIIbβ3 are probably the cause for macrothrombocytopenia in Glanzmann thrombasthenia-like syndrome.

Mutations in the ITGA2B or ITGB3 genes that encode for the αIIbβ3 platelet integrin usually cause Glanzmann thrombasthenia, a severe autosomal recessive bleeding disorder characterized by absence of platelet aggregation, but normal platelet number and size. Several rare mutations cause a Glanzmann-like syndrome which manifests macrothrombocytopenia and usually displays autosomal dominant inheritance. The exact mechanism causing Glanzmann-like syndrome is unknown.

Disassembly of Lys11 and mixed linkage polyubiquitin conjugates provides insights into function of proteasomal deubiquitinases Rpn11 and Ubp6.

Protein homeostasis is largely dependent on proteolysis by the ubiquitin-proteasome system. Diverse polyubiquitin modifications are reported to target cellular proteins to the proteasome. At the proteasome, deubiquitination is an essential preprocessing event that contributes to degradation efficiency.

Natural and artificial mutations in αIIb integrin lead to a structural deformation of a calcium-binding site.

The platelet integrin αIIbβ3 is widely accepted as a structural and a functional model of the broad integrin protein family. The four calcium-binding sites in the αIIb subunit contribute to biogenesis and stability of the protein. Mansour et al.

Unique disulfide bonds in epidermal growth factor (EGF) domains of β3 affect structure and function of αIIbβ3 and αvβ3 integrins in different manner.

The β3 subunit of αIIbβ3 and αvβ3 integrins contains four epidermal growth factor (EGF)-like domains. Each domain harbors four disulfide bonds of which one is unique for integrins. We previously discerned a regulatory role of the EGF-4 Cys-560-Cys-583 unique bond for αIIbβ3 activation.

Intravenous iron therapy and risk for progressive loss of kidney function in patients with chronic kidney disease.

Background: Intravenous (IV) iron is used in the treatment of anemia in patients with chronic kidney disease (CKD). Several lines of evidence have brought up potential concerns regarding the effect of IV iron on the kidney, specifically the possibility of IV iron leading to renal injury and hastening the progression of CKD.

Methods: We performed a retrospective analysis of 77 patients to assess the rate of change in kidney function prior to and after IV iron infusion.

Thyroid hormone abnormalities and frailty in elderly patients with chronic kidney disease: a hypothesis.

Thyroid hormones play a crucial role in the metabolic activities of adults, affecting almost every organ system. All types of thyroid diseases are encountered in the elderly. As symptoms and signs of thyroid diseases may overlap with what is considered to be "normal aging," the presence of a thyroid disorder may go undiagnosed in the elderly.