Adverse Effects Related to Corticosteroid Use in Sepsis, Acute Respiratory Distress Syndrome, and Community-Acquired Pneumonia: A Systematic Review and Meta-Analysis.

Objectives: We postulate that corticosteroid-related side effects in critically ill patients are similar across sepsis, acute respiratory distress syndrome (ARDS), and community-acquired pneumonia (CAP). By pooling data across all trials that have examined corticosteroids in these three acute conditions, we aim to examine the side effects of corticosteroid use in critical illness.

Data Sources: We performed a comprehensive search of MEDLINE, Embase, Centers for Disease Control and Prevention library of COVID research, CINAHL, and Cochrane center for trials.

Glycogen synthase downregulation rescues the amylopectinosis of murine RBCK1 deficiency.

Longer glucan chains tend to precipitate. Glycogen, by far the largest mammalian glucan and the largest molecule in the cytosol with up to 55 000 glucoses, does not, due to a highly regularly branched spherical structure that allows it to be perfused with cytosol. Aberrant construction of glycogen leads it to precipitate, accumulate into polyglucosan bodies that resemble plant starch amylopectin and cause disease.

Ppp1r3d deficiency preferentially inhibits neuronal and cardiac Lafora body formation in a mouse model of the fatal epilepsy Lafora disease.

Mammalian glycogen chain lengths are subject to complex regulation, including by seven proteins (protein phosphatase-1 regulatory subunit 3, PPP1R3A through PPP1R3G) that target protein phosphatase-1 (PP1) to glycogen to activate the glycogen chain-elongating enzyme glycogen synthase and inactivate the chain-shortening glycogen phosphorylase. Lafora disease is a fatal neurodegenerative epilepsy caused by aggregates of long-chained, and as a result insoluble, glycogen, termed Lafora bodies (LBs). We previously eliminated PPP1R3C from a Lafora disease mouse model and studied the effect on LB formation.

Dominant mutation causes intellectual disability with remitting epilepsy.

Mis-secreted glycoproteins (LGI1, reelin) are emerging causes of epilepsy. LMAN2L belongs to a glycoprotein secretion chaperone family. One recessive missense mutation predicted to impair the chaperone's interaction with glycoproteins was reported in a family with intellectual disability (ID) and remitting epilepsy.

Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease.

Lafora disease (LD) is a fatal progressive epilepsy essentially caused by loss-of-function mutations in the glycogen phosphatase laforin or the ubiquitin E3 ligase malin. Glycogen in LD is hyperphosphorylated and poorly hydrosoluble. It precipitates and accumulates into neurotoxic Lafora bodies (LBs).

SGK1 (glucose transport), dishevelled2 (wnt signaling), LC3/p62 (autophagy) and p53 (apoptosis) proteins are unaltered in Lafora disease.

Glycogen forms through the concerted actions of glycogen synthase (GS) which elongates glycogen strands, and glycogen branching enzyme (GBE). Lafora disease (LD) is a fatal neurodegenerative epilepsy that results from neuronal accumulation of hyperphosphorylated glycogen with excessively long strands (called polyglucosans). There is no GBE deficiency in LD.

CAOS-Episodic Cerebellar Ataxia, Areflexia, Optic Atrophy, and Sensorineural Hearing Loss: A Third Allelic Disorder of the ATP1A3 Gene.

We describe the molecular basis of a distinctive syndrome characterized by infantile stress-induced episodic weakness, ataxia, and sensorineural hearing loss, with permanent areflexia and optic nerve pallor. Whole exome sequencing identified a deleterious heterozygous c.2452 G>A, p.

[Patient positioning on the operating table in neurosurgery: sitting or lying].

Efficacy and safety of microvascular decompression of trigeminal nerve depending on the position on the operating table were assessed in 200 neurosurgical patients in retrospective observational study It was shown that efficacy doesn't depend on positioning. Lying position eliminates probability of such complications as postural hypotension, hypotension during surgery, tension pneumocephalus and peripheral nerves injury. Sitting position increases risk of air venous embolism by 25 times.

[Spinal neurosurgical intervention in a patient with end-stage renal disease (ESRD)].

The article presents the description of clinical observation, in which spinal neurosurgical intervention was successfully made in a patient with ESRD, receiving chronic hemodialysis for 20 years. In this context, we discuss the various clinical and tactical aspects of the management of patients with this severe co-pathology: infusion-transfussion therapy, peculiarities of anesthetic management and intra-operation monitoring, diagnostics and correction of hemostasis disorders, hemodialysis organization.

[Thromboelastography as a method for preoperative assessment of hemostasis state in neurosurgical patients on long term aspirin therapy].

The hemostasis state was assessed by routine tests and TEG in 169 patients on long term aspirin therapy. According to TEG results all the patients were divided into three groups: normo-, hypo- and hypercoagulation. The aspirin therapy was interrupted in normo- and hypercoagulation groups, but the surgery was not postponed.

[Bilateral bispectral index monitoring during surgery for posterior cranial fossa tumors].

The analysis of the results of bilateral monitoring of the depth of anesthesia in 22 patients with neurosurgical pathology of the posterior cranial fossa using the technique bispectral index (BIS). The results showed that more than half of the observations (at 19 and 22 patients) during the main phase of the operation (removal of the tumor) were observed differences in the BIS index values between the right and left side. In 1/3 of patients, these differences were driven by higher values of BIS on the side of surgery.

[Hemostatic disorders caused by chronic use of valproic acid in neurosurgical patients. Thromboelastographic monitoring].

The paper gives the results of analyzing the hemostatic system in 26 patients with various neurosurgical diseases on the basis of routine laboratory biochemical tests and thromboelastographic indicators. In all the patients, the pattern of the disease contained an epilepsy syndrome that required mono- or combination therapy with valproic acid. Laboratory indicators of clinical hypocoagulation were found to develop during the use of valproic acid, and its monotherapy in particular.

[Effectiveness and safety of using 6% hydroxyethyl starch 130/04 and 4% modified liquid gelatin in neurosurgical patients with massive intraoperative blood loss].

The results of using three infusion solutions, such as 6% hydroxyexyethyl starch (HES) 200/0.5 (HaesSteril), HES 130/0.4 (Voluven), and 4% modified gelatin solution (Gelofusin), were compared in 33 patients with neurosurgical diseases of the brain (supratentorial meningovascular tumors) and predictable massive intraoperative blood loss during acute isovolemic hemodilution and compensation for later blood loss.

[Reducing the frequency of fresh frozen donor plasma transfusion on the basis of the results of thromboelastographic study in neurosurgical patients with intraoperative blood loss].

The paper gives the results of a study of the time course of changes in the major laboratorily determined hemostatic parameters, as well as the main characteristics of a thromboelastographic curve in 95 neurosurgical patients who developed significant intraoperative blood loss at surgery. The patients were divided into 2 comparable groups: 1) a decision on fresh frozen donor plasma transfusion was taken only on the basis of laboratory parameters; 2) this was done on the basis of thromboelastographic data. In Group 2, the frequency of fresh frozen donor plasma transfusion proved to be 4 times less than that in Group 1.

[Thromboelastography as a method for preoperative screening for the hemostatic system in neurosurgical patients].

The paper analyzes the use of a method of thromboelastography (TEG) as a screening technique of diagnosing hemostatic disorders in risk-group neurosurgical patients: hemostatic disorders detectable from the data of routine laboratory tests; the administration of anticoagulants and desaggresants, the use of anticonvulsants causing impairments in the hemostatic system, hematological diseases, and hepatic cirrhosis. As compared with the routine laboratory tests, TEG is shown to diagnose hemostatic disorders accurately and promptly and to monitor the efficiency of their therapy.

[Effect of the combined hypertonic colloidal solution HyperHeas on hemodynamic and oxygen transport parameters, intracranial pressure, and cerebral oxygenation].

The paper gives the results of a clinical study of the effect of the new combined hypertonic colloidal and hypertonic solution HyperHaes (Frezenius-Cabi) on the parameters of systemic hemodynamics (invasive evaluation by means of a Swan-Ganz catheter), systemic oxygen transport, intracranial pressure (ICP) (lumbar spinal fluid pressure), and cerebral oximetry (INVOS 5100) in neurosurgical patients. The paradoxical effect was found as acute blood pressure lowering and elevated ICP on the beginning of solution infusion (the vasodilator effect of a distinctly hyperosmolar agent). In all other respects, HyperHaes is an ideal agent for volumetric compensation in neurosurgical patients.

[Prompt massive operative blood loss during removal of a giant parietooccipital tumor in a neurosurgical patient with the single kidney].

A clinical case of successful use of 4% modified liquid gelatin (Helofusin, B. Braun, Germany) at a single-stage infusion volume of 4.5 liters (!) is described in a neurosurgical patient with the single during removal of a giant parietooccipal tumor complicated by rapid massive operative blood loss.

[Stepwise surgical treatment in a patient with concomitant cerebrovascular pathology].

The paper describes a relatively rare clinical case of stepwise surgical treatment in a patient with concomitant cerebrovascular pathology: thrombosis of the left internal carotid artery + critical stenosis of the right internal carotid artery + arteriovenous malformation of the right occipital lobe. The patient underwent open right carotid endarterectomy left extra-intracranial microvascular anastomotic application, and histoacryl endovascular embolization of the major afferents and arteriovenous malformation stroma in the stepwise fashion. Management policy, possible complications, and ways of their prevention in these patients are discussed.

[Combined surgical treatment for carotid paraganglioma].

The paper gives a clinical example of embolization of the vascular network of carotid paraganglioma with subsequent en-block tumor removal with bifurcation of the common carotid artery and with recovery of great blood flow along the internal carotid artery through alloprosthesis of the latter. The positive effects of a combination of endovascular embolization of the vascular network of the tumor with its subsequent removal are noted. Indications for endovascular embolization and its procedure, intraoperative evaluation of cerebral collateral circulation are discussed.

[Intraoperative anaphylactic/anaphylactoid reaction to infusion of a modified liquid gelatin].

A clinical observation of an extremely rare but highly severe complication, i.e. anaphylatic/anaphylactoid reaction to the infusion of a synthetic colloid plasma-expander (Gelofusion) is described in the paper.