Rejected

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic cerebral small-vessel disease, which is characterized by migraine, recurrent ischemic strokes, psychiatric disorder, progressive cognitive decline, and occasionally intracerebral hemorrhage (ICH). ICH events have been reported in a high proportion of East Asian CADASIL patients with R544C mutation in exon 11 of NOTCH3; however, whether any other specific NOTCH3 mutation determines the ICH phenotype has yet to be explored.

Lung injury from smoke inhalation manifests as airway and parenchymal damage, at times leading to the acute respiratory distress syndrome. From the beginning of this millennium, the approach to mechanical ventilation in the patient with ARDS was based on reduction of tidal volume to 6 milliliters/kilogram of ideal body weight, maintaining a ceiling of plateau pressure, and titration of driving pressure (plateau pressure minus PEEP). Beyond these broad constraints, there is little specification for the mechanics of ventilator settings, consideration of the metabolic impact of the disease process on the patient, or interaction of patient disease and ventilator settings. Various studies suggest that inhomogeneity of lung injury, which increases the risk of regional lung trauma from mechanical ventilation, may be found in the patient with smoke inhalation. We now appreciate that energy transfer principles may affect optimal ventilator management and come into play in damaged heterogenous lungs. Mechanical ventilation in the patient with inhalation injury should consider various factors. Self-injurious respiratory demand by the patient can be reduced using analgesia and sedation. Dynamic factors beginning with rate management can reduce the incidence of potentially damaging ventilation. Moreover, preclinical study is underway to examine the flow of gas based on the ventilator mode selected, which may also be a factor triggering regional lung injury.

Headache is the most common presenting symptom of spontaneous subarachnoid hemorrhage and managing this acute pain can be challenging. The aim of this study was to describe the course of headaches and factors associated with analgesic failure in patients with spontaneous subarachnoid hemorrhage.

The Delta (B.1.617.2) SARS-CoV-2 variant was the predominant UK circulating strain between May and November 2021. We investigated whether COVID-19 from Delta infection differed from infection with previous variants in children.

Endometriosis (EM) is a hormone-dependent gynecological disease associated with chronic pelvic pain and altered immuno-inflammatory processes. It shares some cancer-like characteristics such as increased proline biosynthesis and activated glutaminolysis. Both proline and glutamine are interconvertible metabolically, and studies have shown their roles in cancer cell metabolic reprogramming, redox homeostasis, occurrence/development of endometrial carcinoma, and its further progression toward the malignant state. So based on this, we hypothesized that the circulatory proline to glutamine ratio (PQR) would be altered in EM and may serve as an indicative biomarker to improve the clinical diagnosis of EM. In present study, the circulatory-PQR levels were estimated for 39 EM patients and 48 age matched healthy female subjects using 800 MHz NMR spectroscopy. Among 39 EM patients, 15 were in the clinical stages I to II and referred to here as moderate EM (MEM) patients and 24 were in the clinical stages III to IV and referred here as severe EM (SEM) patients. The circulatory-PQR levels were significantly increased in EM patients (0.99 ± 0.13 μM in MEM; 1.39 ± 0.22 μM in SEM) compared to normal control (NC) subjects (0.52 ± 0.05 μM in NC). Further, the circulatory PQR levels exhibit the highest diagnostic potential with area under receiver operating characteristic (AUROC) curve values equal to 0.87 ± 0.04 [95%CI = 0.79-0.96] for MEM and 0.89 ± 0.04 [95% CI = 0.82-0.96] for SEM. These results suggested that circulatory-PQR has significant potential to serve as a noninvasive biomarker for diagnostic/prognostic screening of EM and further underscored the importance of these two nonessential amino acids (proline and glutamine) in cancer metabolism.

The scope of dermatologic adverse events to ibrutinib has not been systemically described. We sought to determine the incidence and severity of ibrutinib-associated dermatologic toxicities and provide management recommendations. We conducted a systematic literature search of clinical trials and cohorts investigating ibrutinib monotherapy for cancer or chronic graft-versus-host disease through June 2020. Thirty-two studies with 2,258 patients were included. The incidence of all-grade toxicities included cutaneous bleeds (24.8%; 95%CI, 18.6%-31.0%), mucocutaneous infections (4.9%; 95%CI, 2.9%-7.0%), rash (10.8%; 95%CI. 6.1% – 15.5%), mucositis (6%; 95%CI, 3.6%-8.5%), edema (15.9%; 95%CI, 11.1%-20.6%), pruritus (4.0%; 95%CI, 0.0%-7.9%), xerosis (9.2%; 95%CI, 5.5%-13.0%), nail changes (17.8%; 95%CI, 4.1%-31.5%), and hair changes (7.9%; 95%CI, 0.0%-21.3%). The incidence of high-grade toxicities included mucocutaneous infection (1.3%; 95%CI, 0.5%-2.2%), rash (0.1%; 95%CI, 0.0%-0.2%), mucositis (0.1%; 95%CI, 0.0%-0.3%), and edema (0.1%; 95%CI, 0.0%-0.2%). It is imperative that clinicians familiarize themselves with ibrutinib-associated dermatologic toxicities to learn how to manage them, prevent discontinuation, and improve patient outcomes.

Uremic calciphylaxis is a rare disease that affects patients with chronic end-stage renal disease. It is a pathology of the microvessels of the dermis and hypodermis which are calcified and whose thrombosis leads to skin necrosis. Calciphylaxis lesions can be distal and axial. They lead to pain, infection and are associated with denutrition and in high mortality rate (40-80% at 1 year). This general review describes the clinical and para-clinical presentations of calciphylaxis. It summarizes the current knowledge on its pathogenesis and the therapeutical options that can be proposed to improve the management and attempt to reduce the mortality of patients with uremic calciphylaxis.

Hemifacial spasm (HFS) can be associated with Chiari malformation type I (CM1), but the treatment paradigm for these concurrent conditions has not been well-defined. We sought demographical differences between patients with HFS with and without CM1 and explored optimal surgical treatments for these patients. A systematic review of peer-reviewed literature identified 8 studies with 51 patients with CM1 and HFS. A patient from the authors' institution is presented as a case illustration. Of the 51 patients, the average age was 39.4 years, 63% (32/51) were female, 73% (37/51) underwent microvascular decompression (MVD) as a primary intervention, and 16% (8/51) underwent suboccipital decompression (SOD). After primary MVD, 83.7% (31/37) had complete resolution of their symptoms and 10.8% (4/37) had either recurrent CM1 symptoms or new-onset CM1 symptoms. Three (8.1%) required reoperation with suboccipital decompression to address new CM1-related symptoms. All patients who underwent SOD first had complete or near-complete resolution of symptoms. In 3 patients (37.5%) with near-complete resolution, the residual symptoms had insignificant impact on their quality of life. These data suggest that concomitant CM1 should be among the differential diagnosis in younger patients who present with HFS, particularly those who are female or who present with history suggesting tussive headaches. For patients who present with HFS and headache with CM1, SOD instead of MVD may be the preferred surgery to address concurrent symptoms. In patients with HFS and CM1 without headache, optimal treatment is less clear, but SOD as initial surgery may obviate the need for future reoperation.

Greater trochanteric pain syndrome may often mimic pain generated from other sources. However, it is most commonly caused by gluteus medius and gluteus minimus tendinopathy or tear. The purpose of this technical report was to: 1) describe the ultrasound-guided fascial plane block technique targeting the superior gluteal nerve in the plane between gluteus medius/gluteus minimus to treat moderate-to-severe chronic refractory greater trochanteric pain syndrome; 2) anatomically correlate the procedure with cadaveric dissections demonstrating the structures being imaged and the tissues along the needle trajectory; 3) demonstrate the feasibility of the technique with serial dissection of one cadaveric specimen following injection with color dye.

Pain is a global, complex health problem that includes physical, emotional and social components. The pain management process has many goals, including patient satisfaction, reducing clinical complications, and lowering costs. The physician describes the pain medications in terms of the proven cause and classification of the severity of the pain. The combination of celecoxib and tramadol was recently approved by the FDA in October 2021 for the treatment of acute pain in adults.