A Case Report of Congenital Afibrinogenemia and Literature Review of Management of Post-circumcision Bleeding.

We present a case of bleeding from circumcision in a full-term newborn male resulting from a rare coagulopathy, congenital afibrinogenemia, and a review of the literature regarding the management of bleeding after circumcision. Bleeding was managed with silver nitrate, suturing, thrombin powder, AristaTM AH (absorbable hemostatic particles; Becton, Dickinson and Company, Franklin Lakes, USA), FFP (fresh frozen plasma), and cryoprecipitate. The Fibrinogen level was less than 30 mg/dl (ref 150-430 mg/dl). The diagnosis of congenital afibrinogenemia was confirmed by a gene test. The baby was found to have a heterozygous pathogenic variant (c.510+1G>T) and a heterozygous likely pathogenic variant (c.1037del) in the FGA gene.

Financing Models for Virtual Care.

Principles for determining physician compensation in the provision of virtual care are discussed along with analysis of the virtual care components included in the 2022 Ontario Physician Services Agreement (SEAMO 2022). Ministries and medical associations should continue to discuss appropriate payment for virtual advice for patients who do not have a primary care provider. They should also determine methods for payment for physician engagement in apps designed to optimize treatment of chronic diseases.

Effect of pulsed electromagnetic field as an intervention for patients with quadriceps weakness after anterior cruciate ligament reconstruction: a double-blinded, randomized-controlled trial.

BACKGROUND: The ultimate goal of anterior cruciate ligament reconstructions (ACLR) is to fulfil the return-to-play (RTP) criteria. Quadriceps muscle strength is one of the key determinants for a patient's successful return-to-play after ACLR. Quadriceps muscle atrophy can persist beyond the completion of the rehabilitation program in almost half the patients and the reason behind this is still unknown. There are emerging evidences showing that pulsed electromagnetic field (PEMF) can modulate mitochondrial activities for muscle gain. PEMF exposure on top of regular exercise training may promote muscle regeneration and tissue healing. METHODS: This is a double-blinded, randomized controlled trial to investigate the effects of PEMF treatment during the postoperative period on quadriceps muscle strength in ACL injured patient. Adult patients (aged 18-30) with a unilateral ACL injury, total quadriceps muscle volume is equal or more than 7% deficit on involved leg compared with uninvolved leg, sporting injury with a Tegner score of 7+, and both knees without a history of injury/prior surgery will be recruited. To estimate the improvement of patients, isokinetic muscle assessment, ultrasound imaging and MRI for quadriceps muscle thickness, self-reported outcomes with questionnaires, KT-1000 for knee laxity and biomechanical analysis, and Xtreme CT for bone mineral density will be performed. To investigate the mechanism of PEMF therapy on increasing quadriceps strength, samples of blood serum will be drawn before and after intervention. DISCUSSION: This is the first trial evaluating the effects of PEMF on quadriceps muscle recovery after ACLR. The proposed study addresses a huge research gap by evaluating practical use of PEMF as part of rehabilitation. The proposed study will provide much needed scientific support in the use of this noninvasive treatment modality to facilitate recovery of quadriceps strength after PEMF. TRIAL REGISTRATION: ClinicalTrials.gov NCT05184023. Registered on 5 January 2022.

Knee wobbling during the single-leg-squat-and-hold test reflects dynamic knee instability in patients with anterior cruciate ligament injury.

We propose using the single-leg squat-and-hold (SLSH) task with kinematic analysis to objectively measure dynamic knee stability after anterior cruciate ligament (ACL) injury. There are three objectives of this study: to compare the knee kinematics of ACL-deficient patients and healthy controls by capturing knee wobbling during the SLSH task, to detect kinematic changes after ACL reconstruction, and to correlate the kinematic variables with self-reported knee function. Twenty-five ACL-deficient participants and 18 healthy matched participants were recruited. The knee kinematics involving both the magnitudes and frequency of motion fluctuation was captured during SLSH by 3D motion analysis system (Vicon). Compared to the limbs of the control participants, the ACL involved limbs exhibited a greater range of flexion-extension (4.33 ± 1.96 vs. 2.73 ± 1.15; p = 0.005) and varus-valgus (2.52 ± 0.99 vs. 1.36 ± 0.42; p < 0.001). It also inhibited higher frequency of flexion-extension (4.87 ± 2.55 vs. 2.68 ± 1.23; p = 0.003) and varus-valgus (3.83 ± 2.59 vs. 1.42 ± 0.55; p < 0.001). The range of flexion-extension (4.50 ± 2.24 vs. 2.90 ± 1.01; p = 0.018), frequency of flexion-extension (4.58 ± 2.53 vs. 3.05 ± 1.80; p = 0.038) and varus-valgus (3.46 ± 2.11 vs. 1.80 ± 1.23; p = 0.022) was reduced after ACL reconstruction. Increased frequency of knee varus-valgus was correlated with lower IKDC score (r = -0.328; p = 0.034). Knee wobbling was more prominent in ACL-deficient patients, which was associated with poor knee function. SLSH task with kinematic analysis appears to be a potential assessment method for monitoring dynamic knee stability after ACL injury.

Comparison of Drug Benefits Provided by Veterans Affairs Canada and the Canadian Forces Health Services Group.

BACKGROUND: Drug benefits are provided at public expense to all actively serving Canadian Armed Forces (CAF) personnel, with ongoing drug coverage offered by Veterans Affairs Canada (VAC) for selected conditions following termination of employment. Differences in drug coverage between these programs could introduce risks for treatment disruption. OBJECTIVES: Work was undertaken to establish a process that would allow systematic comparison of the entire VAC and CAF formularies, and to identify and explain discordant listings in 14 therapeutic categories that pose risk of adverse outcomes with sudden treatment interruption. METHODS: Lists of medications were created for each program, including regular benefit and restricted use drugs, using files obtained from the claims processor in January 2015. Products were coded using the Anatomic-Therapeutic-Chemical (ATC) system. Degree of alignment within therapeutic categories was assessed based on the percentage of fifth-level ATCs that were covered in common. Discordantly listed drugs in 14 categories of concern were reviewed to identify similarities in product characteristics. RESULTS: A total of 1124 medications were identified in 80 therapeutic categories. Coverage of medications was identical in 11 categories, and overall, almost three-quarters of identified drugs (73.4%, n = 825) were covered in common by both plans. Many discordant listings reflected known differences in the programs' operating procedures. A number of discrepancies were also identified in newer therapeutic categories. CONCLUSIONS: There is significant overlap in the medications covered by the CAF and VAC drug benefit programs. Application of the ATC coding system allowed for discrepancies to be readily identified across the entire formulary, and in specific therapeutic categories of concern.

The hypocretin/orexin system in sleep disorders: preclinical insights and clinical progress.

Much of the understanding of the hypocretin/orexin (HCRT/OX) system in sleep-wake regulation came from narcolepsy-cataplexy research. The neuropeptides hypocretin-1 and -2/orexin-A and -B (HCRT-1 and -2/OX-A and -B, respectively), as we know, are intimately involved in the regulation wakefulness. The HCRT/OX system regulates sleep-wake control through complex interactions between monoaminergic/cholinergic (wake-promoting) and gamma-aminobutyric acid-ergic (sleep-promoting) neuronal systems. Deficiency of HCRT/OX results in loss of sleep-wake control or stability with consequent unstable transitions between wakefulness to nonrapid eye movement and rapid eye movement sleep. This manifests clinically as abnormal daytime sleepiness with sleep attacks and cataplexy. Research on the development of HCRT/OX agonists and antagonists for the treatment of sleep disorders has dramatically increased with the US Food and Drug Administration approval of the first-in-class dual HCRT/OX receptor antagonist for the treatment of insomnia. This review focuses on the origin, mechanisms of HCRT/OX receptors, clinical progress, and applications for the treatment of sleep disorders.

Prolonged dependence of ischemic penumbra on induced hypertension.

BACKGROUND: Pharmacological elevation of blood pressure in acute stroke due to large artery occlusion may enhance collateral blood flow and salvage penumbral tissue. The duration of viability of human penumbral tissue under such circumstances is unclear. OBJECTIVE: To describe a patient presenting with acute middle cerebral artery occlusion treated with induced hypertension who had prolonged survival of penumbral tissue with subsequent infarct extension after pressor agents were withdrawn. CONCLUSIONS: Enhanced collateral flow by elevation of blood pressure may result in prolonged survival of the ischemic penumbra which remains dependent on persistent use of pressor agents.

Sepsis alters the megakaryocyte-platelet transcriptional axis resulting in granzyme B-mediated lymphotoxicity.

RATIONALE: Sepsis-related mortality results in part from immunodeficiency secondary to profound lymphoid apoptosis. The biological mechanisms responsible are not understood. OBJECTIVES: Because recent evidence shows that platelets are involved in microvascular inflammation and that they accumulate in lymphoid microvasculature in sepsis, we hypothesized a direct role for platelets in sepsis-related lymphoid apoptosis. METHODS: We studied megakaryocytes and platelets from a murine-induced sepsis model, with validation in septic children, which showed induction of the cytotoxic serine protease granzyme B. MEASUREMENTS AND MAIN RESULTS: Platelets from septic mice induced marked apoptosis of healthy splenocytes ex vivo. Platelets from septic granzyme B null (-/-) mice showed no lymphotoxicity. CONCLUSIONS: Our findings establish a conceptual advance in sepsis: Septic megakaryocytes produce platelets with acutely altered mRNA profiles, and these platelets mediate lymphotoxicity via granzyme B. Given the contribution of lymphoid apoptosis to sepsis-related mortality, modulation of platelet granzyme B becomes an important new target for investigation and therapy.

Anesthesia considerations for robotic-assisted laparoscopic prostatectomy: a review of 1,500 cases.

Prostate cancer is the second most common cancer in American men and new surgical techniques have led to less invasive options for prostate surgery. Innovations in robotic technology have enabled robotic systems to become a more common sight in operating theatres throughout the United States. Approximately 1,500 consecutive patients scheduled for elective robotic prostatectomy (RP) were reviewed. Patient demographics were recorded and significant intraoperative and postoperative events were reviewed. At our institution the mean age for patients undergoing RP is 60.3 (41-79) years; the mean body mass index is 27.3 kg m(-2). The mean operative time, defined as the time from the start of insufflation to closure is 177.5 (81-365) min and mean blood loss is 109 (50-750) mL. There was a 1.3% incidence of postoperative anemia (hemoglobin <10 g dL) where patients required blood transfusions (15/1,500). Three patients were diagnosed with postoperative pulmonary emboli and were treated with IV heparin with no additional sequelae. The most common anesthesia-related complication was corneal abrasions, which were seen in 3% of cases. One patient required postoperative mechanical ventilation because of laryngeal edema secondary to multiple intubation attempts from an unexpectedly difficult airway. Anesthetic and perioperative complications are rare for patients undergoing robotic-assisted laparoscopic prostatectomy at our institution. Our institution has performed more robotic prostatectomies than any other institution in the world and we review our experience delivering anesthesia for the first 1,500 patients undergoing this operation.