An international learning collaborative phase 2 trial for haploidentical bone marrow transplant in sickle cell disease.

ABSTRACT: In the setting of a learning collaborative, we conducted an international multicenter phase 2 clinical trial testing the hypothesis that nonmyeloablative-related haploidentical bone marrow transplant (BMT) with thiotepa and posttransplant cyclophosphamide (PTCy) will result in 2-year event-free survival (no graft failure or death) of at least 80%. A total of 70 participants were evaluable based on the conditioning protocol. Graft failure occurred in 8 of 70 (11.4%) and only in participants aged <18 years; all had autologous reconstitution. After a median follow-up of 2.4 years, the 2-year Kaplan-Meier-based probability of event-free survival was 82.6%. The 2-year overall survival was 94.1%, with no difference between children and adult participants. After excluding participants with graft failure (n = 8), participants with engraftment had median whole blood donor chimerism values at days +180 and +365 after transplant of 100% (n = 58), respectively, and 96.6% (57/59) were off immunosuppression 1 year after transplant. The 1-year grade 3 to 4 acute graft-versus-host disease (GVHD) rate was 10%, and the 2-year moderate-severe chronic GVHD rate was 10%. Five participants (7.1%) died from infectious complications. We demonstrate that nonmyeloablative haploidentical BMT with thiotepa and PTCy is a readily available curative therapy for most adults, even those with organ damage, compared to the more expensive myeloablative gene therapy and gene editing. Additional strategies are required for children to decrease graft failure rates. The trial was registered at www.clinicaltrials.gov as #NCT01850108.

An ultra-stable gold-coordinated protein cage displaying reversible assembly.

Symmetrical protein cages have evolved to fulfil diverse roles in nature, including compartmentalization and cargo delivery1, and have inspired synthetic biologists to create novel protein assemblies via the precise manipulation of protein-protein interfaces. Despite the impressive array of protein cages produced in the laboratory, the design of inducible assemblies remains challenging2,3. Here we demonstrate an ultra-stable artificial protein cage, the assembly and disassembly of which can be controlled by metal coordination at the protein-protein interfaces. The addition of a gold (I)-triphenylphosphine compound to a cysteine-substituted, 11-mer protein ring triggers supramolecular self-assembly, which generates monodisperse cage structures with masses greater than 2 MDa. The geometry of these structures is based on the Archimedean snub cube and is, to our knowledge, unprecedented. Cryo-electron microscopy confirms that the assemblies are held together by 120 S-Aui-S staples between the protein oligomers, and exist in two chiral forms. The cage shows extreme chemical and thermal stability, yet it readily disassembles upon exposure to reducing agents. As well as gold, mercury(II) is also found to enable formation of the protein cage. This work establishes an approach for linking protein components into robust, higher-order structures, and expands the design space available for supramolecular assemblies to include previously unexplored geometries.

Hypophosphatasia: presentation and response to asfotase alfa.

Hypophosphatasia (HPP) is a rare bone disease with limited scientific evidence on the tolerability and safety of its novel treatment, Asfotase Alfa (AA). We report 7 HPP patients' heterogenous presentations and the significant improvement in various clinical outcomes attained with AA shedding light on this highly effective and safe therapy. INTRODUCTION: Hypophosphatasia (HPP) is a rare inherited metabolic bone disorder characterized by a deficiency in the tissue non-specific alkaline phosphatase (TNSALP) due to loss of function mutation in the ALPL gene. HPP is associated with impaired skeletal mineralization due to elevations in inorganic pyrophosphate and altered phosphate : pyrophosphate ratio. Asfotase alfa (AA) "enzyme replacement" was approved for treatment of HPP in 2015. We present 7 patients with HPP, 5 with pediatric-onset, and 2 with adult-onset, who have been treated with AA and describe the efficacy and safety in these patients. METHODS: 7 patients (4 females, 3 males) aged 19-68 years with HPP were included in this study. Diagnosis of HPP was confirmed by DNA analysis. AA was administered in doses of 6mg/kg/week with a mean follow-up of 6 months (SD= 5). RESULTS: Subjective improvement in muscle strength, muscle pain, walking ability, and walking distance with a reduction in the use of gait aids was seen "with AA in HPP patients." Muscle strength and pain improved by up to 70% from baseline as quantified subjectively by patients. Walking distance improved by up to 100%. Patients also reported improved cognition, mood, and energy levels, with up to 90% improvement in mood and 75% improvement in energy levels. 4 out of 6 patients first noted clinical signs of improvement after 3 months of being on therapy. 1 out of the 7 patients sustained a toe fracture 10 months from being on AA. AA was well-tolerated with injection site reactions being the most reported adverse effect. CONCLUSION: HPP treatment with AA in individuals with both pediatric and adult-onset forms resulted in significant subjective improvement in musculoskeletal and cognitive manifestations in addition to patients' quality of life. The drug was well tolerated in 6 patients. 1 patient discontinued therapy because of minor adverse effects with myalgias.

Multicomponent nature underlies the extraordinary mechanical properties of spider dragline silk.

Dragline silk of golden orb-weaver spiders (Nephilinae) is noted for its unsurpassed toughness, combining extraordinary extensibility and tensile strength, suggesting industrial application as a sustainable biopolymer material. To pinpoint the molecular composition of dragline silk and the roles of its constituents in achieving its mechanical properties, we report a multiomics approach, combining high-quality genome sequencing and assembly, silk gland transcriptomics, and dragline silk proteomics of four Nephilinae spiders. We observed the consistent presence of the MaSp3B spidroin unique to this subfamily as well as several nonspidroin SpiCE proteins. Artificial synthesis and the combination of these components in vitro showed that the multicomponent nature of dragline silk, including MaSp3B and SpiCE, along with MaSp1 and MaSp2, is essential to realize the mechanical properties of spider dragline silk.

How nursing practice environments limit implicit rationing of care and nurse-assessed adverse events: the role of flow at work.

BACKGROUND: The nursing practice environment is beneficial in curbing implicit rationing of nursing care and adverse patient events. However, the underlying mechanisms of these relationships remain unexplored. AIM: To test whether flow at work mediates the relationship between the nursing practice environment, implicit rationing of nursing care, and nurse-assessed adverse patient events. METHODS: This cross-sectional study involved 231 nurses from five hospitals in Port Said, Egypt. The participants completed Arabic-translated versions of the Practice Environment Scale of the Nursing Work Index, the Work-Related Flow Inventory, the Perceived Implicit Rationing of Nursing Care instrument, and the Adverse Patient Events scale. Structural equation modeling was used to test the hypothetical model. RESULTS: The favorable nursing practice environment positively predicted nurses' flow at work (ß = 0.64, p < 0.001), while inversely predicting implicit rationing of nursing care (ß = -0.23, p = 0.014) and adverse patient events (ß = -0.35, p < 0.001). Nurses' flow at work inversely predicted implicit rationing of nursing care (ß = -0.30, p = 0.002) and adverse patient events (ß = -0.29, p = 0.002). Moreover, nurses' flow at work acted as a mediator, linking the nursing practice environment to the rationing of nursing care and adverse patient events, with 500 bootstrap results for the indirect effects (ß = -0.24, p = 0.001, 95% CI: -0.43 to -0.09; and ß = -0.44, p = 0.003, 95% CI: -0.79 to -0.16, respectively). CONCLUSION: Nurses working in a favorable nursing practice environment are more likely to experience flow at work, limiting implicit rationing of nursing care and adverse patient events. IMPLICATIONS FOR NURSING MANAGEMENT: Nursing administrators should strive to create a healthy nursing practice environment to foster nurses' flow and thereby reduce the frequency of implicit rationing of nursing care and adverse patient events.

Exploring the relationship between nursing students' knowledge and attitudes towards climate change and their psychological distress: a cross-national investigation.

BACKGROUND: Climate change poses a worldwide challenge with anticipated exacerbation in the future, resulting in irreversible consequences. Nursing students may be vulnerable to experiencing psychological effects associated with climate change. AIM: The current study aimed to investigate the relationship between nursing students' knowledge and attitudes toward climate change and their psychological distress. METHOD: This descriptive cross-sectional study recruited 377 nursing students from three universities located in Saudi Arabia, Jordan, and Egypt in the Middle East. Data collection was conducted using scales for assessing nursing students' knowledge and attitudes towards climate change and their psychological distress. Correlations were assessed and multiple regression analysis was conducted to explore the predictors of students' psychological distress. RESULTS: The current study showed that knowledge regarding climate change significantly and positively correlated to the attitude toward climate change (r = 0.213), then again, the score of psychological distress significantly and negatively correlated to the score of students' knowledge and attitude regarding climate change (r = - 0.182 and - 0.110 respectively). Regression analyses showed that academic achievement had the strongest positive impact on students' psychological distress, while knowledge regarding climate change and attitude toward climate change had negative impacts (ß = 0.381, ß=-0.205, and ß=-0.045 respectively). Moreover, knowledge and attitude regarding climate change were found to be significant predictors of students' psychological distress, collectively accounting for 18.2% of the observed variance. CONCLUSIONS/IMPLICATION FOR FUTURE PRACTICE: The findings highlight the importance of incorporating climate change into nursing education programs. By enhancing nursing students' knowledge and attitudes towards climate change, there is potential to reduce their psychological distress. This study underscores the need for curriculum reforms that integrate climate change topics, aiming to foster a well-informed and resilient future nursing workforce.

The relationship between emotional intelligence, resilience, and psychological distress among nurses in Jordan.

BACKGROUND: Nurses have significant levels of depression, anxiety, and stress as a result of their exposure to various stressors at work. Emotional intelligence and resilience are relatively new notions essential for nurses to naturalize high levels of psychological problems. The study aimed to investigate the relationship between emotional intelligence and resilience and their impact on depression, anxiety, and stress among nurses. METHODS: A cross-sectional descriptive design was used and recruited 152 nurses from three private hospitals in Jordan. Variables were measured using the Depression Anxiety Stress Scale, Brief Resilience Scale, and Brief Emotional Intelligence Scale. RESULTS: The participants' average age was 27.6 years, and they were mostly female (66.4 %). Resilience earned an average of 3.1, while emotional intelligence averaged 31.7. Depression, anxiety, and stress were all significantly higher among nurses. Also, negative relationships were found between emotional intelligence, resilience, and psychological problems (p < .001). Emotional intelligence is positively associated with resilience (r = 0.83, p < .001). In addition, emotional intelligence and resilience were both significant predictors of depression, anxiety, and stress. CONCLUSION: Emotional intelligence is critical to nurses' well-being. Higher emotional intelligence is associated with higher resilience and lower levels of depression, anxiety, and stress among nurses. Interventions and education programs emphasizing emotional intelligence and resilience are essential to improving their mental health. Healthcare organizations and governments should promote these attributes to enhance nurses' psychological wellness in challenging healthcare settings.

Mistreatment of nurses by patients and its impact on their caring behaviors: The roles of psychological detachment and supervisor positive gossip.

BACKGROUND: Mistreatment by patients is a detrimental phenomenon predominant in healthcare organizations. However, there is a lack of nursing literature regarding the underlying mechanisms and boundary conditions that exist between mistreatment by patients and its detrimental consequences. AIM: By integrating the Job Demands-Resources Model and the Conservation of Resources Theory, the study aimed to unveil the mediating role of psychological detachment from work in the relationship between mistreatment by patients and nurses' caring behaviors, as well as the moderating effect of supervisor positive gossip. METHODS: This multicenter, cross-sectional study involved 341 nurses from five hospitals across two cities in Egypt. Data were collected using an introductory information form, the Mistreatment by Patients Scale, the Psychological Detachment Scale, the Caring Behaviors Scale, and the Supervisor Positive Gossip Scale. The study's hypotheses were tested using the PROCESS macro. RESULTS: The results showed that mistreatment by patients was negatively related to nurses' caring behaviors via an increased psychological detachment. Further, higher levels of supervisor positive gossip were shown to weaken the direct effect of mistreatment by patients in increasing nurses' psychological detachment, as well as the indirect negative effect of mistreatment by patients on nurses' caring behaviors via psychological detachment. CONCLUSIONS: Mistreatment by patients contributes to nurses' psychological detachment and decreased caring behaviors; however, these negative outcomes can be mitigated by supervisor positive gossip. IMPLICATION FOR NURSING POLICYMAKING: Nursing managers should adopt strategies to deter the rise of mistreatment by patients, and utilize positive gossip to support nurses exposed to such mistreatment to alleviate its consequences.

Unusual pKa Values Mediate the Self-Assembly of Spider Dragline Silk Proteins.

Spider dragline silk is a remarkably tough biomaterial and composed primarily of spidroins MaSp1 and MaSp2. During fiber self-assembly, the spidroin N-terminal domains (NTDs) undergo rapid dimerization in response to a pH gradient. However, obtaining a detailed understanding of this mechanism has been hampered by a lack of direct evidence regarding the protonation states of key ionic residues. Here, we elucidated the solution structures of MaSp1 and MaSp2 NTDs from Trichonephila clavipes and determined the experimental pKa values of conserved residues involved in dimerization using NMR. Surprisingly, we found that the Asp40 located on an acidic cluster protonates at an unusually high pH (∼6.5-7.1), suggesting the first step in the pH response. Then, protonation of Glu119 and Glu79 follows, with pKas above their intrinsic values, contributing toward stable dimer formation. We propose that exploiting the atypical pKa values is a strategy to achieve tight spatiotemporal control of spider silk self-assembly.

Imaging of Castleman Disease.

Castleman disease (CD) is a group of rare and complex lymphoproliferative disorders that can manifest in two general forms: unicentric CD (UCD) and multicentric CD (MCD). These two forms differ in clinical manifestation, imaging appearances, treatment options, and prognosis. UCD typically manifests as a solitary enlarging mass that is discovered incidentally or after development of compression-type symptoms. MCD usually manifests acutely with systemic symptoms including fever and weight loss. As a whole, CD involves lymph nodes throughout the chest, neck, abdomen, pelvis, and axilla and can have a wide variety of imaging appearances. Most commonly, lymph nodes or masses in UCD occur in the chest, classically with well-defined borders, hyperenhancement, and possible characteristic patterns of calcification and/or feeding vessels. Lymph nodes affected by MCD, while also hyperenhancing, tend to involve multiple nodal chains and manifest alongside anasarca or hepatosplenomegaly. The polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) subtype of MCD may demonstrate lytic or sclerotic osseous lesions in addition to features typical of MCD. Since a diagnosis of CD based solely on imaging findings is often not possible, pathologic confirmation with core needle biopsy and/or surgical excision is necessary. Nevertheless, imaging plays a crucial role in supporting the diagnosis of CD, guiding appropriate regions for biopsy, and excluding other potential causes or mimics of disease. CT is frequently the initial imaging technique used in evaluating potential CD. MRI and PET play important roles in thoroughly evaluating the disease and determining its extent, especially the MCD form. Complete surgical excision is typically curative for UCD. MCD usually requires systemic therapy. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

[A particular state of shock: spontaneous rupture of a heterotopic pregnancy]. / Cas clinique. Un état de choc particulier : une rupture spontanée de grossesse hétérotopique.

Heterotopic pregnancy is a rare pathology. It is defined as the coexistence of an intrauterine pregnancy and an extrauterine pregnancy, whatever its location. The presence of an intrauterine pregnancy in a woman of childbearing age does not therefore exclude the presence of an associated ectopic pregnancy. Late diagnosis can lead to fatal consequences for the mother, subsequent fertility as well as for the intrauterine pregnancy. We report the case of a 28-year old female patient, without any notion of assisted reproduction, who was admitted to the emergency department for hemorrhagic shock. The diagnosis of ruptured tubal heterotopic pregnancy was made intraoperatively.

La grossesse hétérotopique est une pathologie rare. Elle est décrite comme la coexistence d'une grossesse intra-utérine et d'une grossesse extra-utérine, quelle que soit la localisation de cette dernière. Nous rapportons le cas d'une patiente âgée de 28 ans, sans notion de procréation médicalement assistée, qui a été admise aux soins d'urgence dans un état de choc hémorragique. Le diagnostic de grossesse hétérotopique tubaire rompue a été posé en peropératoire. Malgré la rareté de cette entité, il est fondamental pour les cliniciens de la connaître afin d'évoquer la possibilité de ce diagnostic. L'échographie endovaginale précoce est l'examen de choix pour l'aide au diagnostic. Ce dernier reste néanmoins difficile en raison d'une symptomatologie déroutante. La présence d'une grossesse intra-utérine chez une femme en âge de procréer n'exclut dès lors pas la présence d'une grossesse ectopique associée. Le diagnostic tardif peut conduire à des conséquences fatales pour la maman, pour sa fertilité ultérieure ainsi que pour l'évolution de la grossesse intra-utérine.

Complexity of Spider Dragline Silk.

The tiny spider makes dragline silk fibers with unbeatable toughness, all under the most innocuous conditions. Scientists have persistently tried to emulate its natural silk spinning process using recombinant proteins with a view toward creating a new wave of smart materials, yet most efforts have fallen short of attaining the native fiber's excellent mechanical properties. One reason for these shortcomings may be that artificial spider silk systems tend to be overly simplified and may not sufficiently take into account the true complexity of the underlying protein sequences and of the multidimensional aspects of the natural self-assembly process that give rise to the hierarchically structured fibers. Here, we discuss recent findings regarding the material constituents of spider dragline silk, including novel spidroin subtypes, nonspidroin proteins, and possible involvement of post-translational modifications, which together suggest a complexity that transcends the two-component MaSp1/MaSp2 system. We subsequently consider insights into the spidroin domain functions, structures, and overall mechanisms for the rapid transition from disordered soluble protein into a highly organized fiber, including the possibility of viewing spider silk self-assembly through a framework relevant to biomolecular condensates. Finally, we consider the concept of "biomimetics" as it applies to artificial spider silk production with a focus on key practical aspects of design and evaluation that may hopefully inform efforts to more closely reproduce the remarkable structure and function of the native silk fiber using artificial methods.

Eosinophilic Disorders of the Gastrointestinal Tract and Associated Abdominal Viscera: Imaging Findings and Diagnosis.

Eosinophilic gastrointestinal disorders (EGIDs) are inflammatory conditions of the gastrointestinal tract that are characterized by tissue eosinophilia and end-organ dysfunction or damage. Primary EGIDs are associated with atopy and other allergic conditions, whereas secondary EGIDs are associated with underlying systemic diseases or hypereosinophilic syndrome. Within the spectrum of EGIDs, eosinophilic esophagitis is the most prevalent. Eosinophilic gastroenteritis and eosinophilic colitis are relatively uncommon. Eosinophilic infiltration of the liver, biliary tree, and/or pancreas also can occur and mimic other inflammatory and malignant conditions. Although endoscopic evaluation is the method of choice for eosinophilic esophagitis, radiologic evaluation of the esophagus plays an important role in the assessment of disease severity. CT and MR enterography are the modalities of choice for demonstrating specific forms of eosinophilic gastroenteritis. CT and MRI are important in the detection of abdominal visceral involvement in EGIDs. Diagnosis is often challenging and relies on symptoms, imaging findings, histologic confirmation of tissue eosinophilia, and correlation with peripheral eosinophilia. Imaging is crucial for identifying characteristic organ-specific findings, although imaging findings are not specific. When promptly treated, EGIDs usually have a benign clinical course. However, a delayed diagnosis and associated surgical interventions have been associated with morbidity. Therefore, a radiologist's knowledge of the imaging findings of EGIDs in the appropriate clinical settings may aid in early diagnosis and thereby improve patient care. An overview of the clinical features and imaging findings of EGIDs and the eosinophilic disorders of associated abdominal viscera is provided. Online supplemental material is available for this article. ©RSNA, 2022.

Primary angiosarcoma of aorta: A systematic review.

PURPOSE: To elucidate the epidemiology, anatomical, presentation, classification, pathology, investigative modalities, management and prognosis of primary angiosarcoma of the aorta. MATERIAL AND METHODS: A systematic review of literature from the database inception to January 2021 in PubMed and Embase, CINAHL and Cochrane Library in accordance to PRISMA was conducted. Retrieval and extraction was performed by two independent reviewers. The hierarchy of the evidence was assessed through the National Institute for Health and Care Excellence Checklist. Data were subjected to pooled prevalence analysis, Kaplan-Meier survival and test of probability using log-rank analysis. This review is registered with International Prospective Register of Systematic Reviews: RD42021231314. RESULTS: 82 studies with n = 123 cases met the inclusion criterion. Abdominal (45%) aorta was the commonest anatomical site with female predominance in ascending aorta (4:1) and aortic arch (2:1). The longest survival was in the ascending aorta and the shortest in the abdominal aorta [540 (interquartile range [IQR], 7-1560 days vs. 180 (IQR, 1-5730 days)], respectively. The overall median survival was 210 days (IQR, 1-5730 days) or 7 months. Lack of metastasis (47%) was a marker of longer survival (p < 0.03) irrespective of other attributes. CONCLUSION: The pathophysiology appears to be a trend of increasing fatigue, fever and weight loss associated with segmental dysfunction of the aorta projecting occlusive or destructive phenotypes. Computed tomography angiography features of volume-occupying, bulky, polypoid (intraluminal), protrusive vegetation, hyper vascular without atherosclerotic lesions are extremely suggestive of PA of the aorta at 5th and 6th decades of life.

Effect of humble leadership on proactive work behaviour: The mediating role of psychological empowerment among nurses.

AIM: This study was designed to determine the effect of humble leadership on nurses' proactive behaviours at work through psychological empowerment. BACKGROUND: Nurse leaders' humility has a profound effect on enhancing nurses' proactive work behaviour; yet, the mechanism underlying this pattern of effect remains unknown. METHODS: A descriptive cross-sectional study was conducted in all nine Universal Health Insurance hospitals in Port Said, Egypt. Using scales for assessing leader humility, psychological empowerment and proactive behaviour at work, 316 nurses were surveyed. Regression analyses and structural equation models were used to examine the study hypothetical model. RESULTS: The results revealed that humble leadership, psychological empowerment and proactive work behaviour were significantly positively correlated. Psychological empowerment fully mediated the relationship between humble leadership and proactive work behaviour. CONCLUSION: This study emphasized the value of humble leadership in psychologically empowering nurses, which results in greater proactive work behaviour. IMPLICATION FOR NURSING MANAGEMENT: Nurse managers should lead their nursing staff with humility, which could increase the level of nurses' psychological empowerment and proactive behaviours at work, which will be reflected positively on the staffs and the organization.

[Fatal serotonin syndrome induced by ecstasy consumption]. / Le cas clinique du mois. Un cas fatal de syndrome sérotoninergique induit par la consommation d'ecstasy.

The use of amphetamines and amphetamine derivatives such as ecstasy can cause serotonin toxic syndrome, an uncommon but potentially serious adverse effect. Although most of the reported cases evolve spontaneously and favourably, in rare cases, serious complications can occur leading to the death of the patient. We report the case of a 27-year-old man admitted to our emergency department for altered consciousness with hyperthermia at 42°C after illicit drug use. The patient developed severe multivisceral failure and disseminated intravascular coagulopathy despite maximalist management focused on cooling and multiorgan supportive therapy. The patient died within hours of admission. The diagnosis is essentially based on the patient history and clinical examination. The first treatment is to stop the toxic and then, to treat the symptoms and support possible organ failures.

La consommation d'amphétamines et de ses dérivés tels que l'ecstasy peut être responsable d'un syndrome toxique sérotoninergique, effet indésirable peu fréquent mais potentiellement redoutable. Bien que la plupart des cas rapportés évoluent spontanément favorablement, dans de rares cas, de graves complications peuvent survenir pouvant mener jusqu'au décès du patient. Nous rapportons le cas d'un homme de 27 ans, admis dans notre service des urgences pour altération de l'état de conscience avec hyperthermie à 42°C après consommation de drogues illicites. Le patient a développé une défaillance multiviscérale sévère ainsi qu'une coagulopathie intravasculaire disséminée malgré une prise en charge maximaliste centrée sur le refroidissement et le traitement supportif multi-organique. Le patient est décédé quelques heures après son admission. Le diagnostic du syndrome sérotoninergique est essentiellement basé sur l'anamnèse et l'examen clinique. La prise en charge comprend l'arrêt du toxique, le traitement des symptômes et le support des potentielles défaillances organiques.

[An unusual case of central cyanosis]. / Un cas inhabituel de cyanose centrale.

We report the case of a 29-year-old man admitted to the emergency department for dyspnea and changes in mental status during a festivity. Clinically the patient presented a central cyanosis refractory to the administration of high concentration of oxygen. The consumption of poppers is increasingly used by young people for recreational purposes because they are inexpensive and easy to acquire. Methemoglobinemia is a potentially serious and little known complication of popper intoxication. This condition, known as «methemoglobinemia¼, was suspected by the emergency physician and confirmed through non-invasive measurement of methemoglobinemia in arterial blood gases. The early recognition of methemoglobinemia and prompt treatment allowed a favourable evolution of our patient avoiding the development of multi-systemic organ failure or even death.

Nous rapportons le cas d'un homme de 29 ans admis pour dyspnée et altération de l'état de conscience survenue dans le décours d'une soirée festive. Le tableau clinique est marqué par une cyanose centrale réfractaire à l'administration d'oxygène au masque à haute concentration. La consommation de poppers à usage récréatif est de plus en plus fréquente chez les jeunes adultes. C'est une substance peu coûteuse et facile d'accès, consommée, notamment, pour ses propriétés euphorisantes. La méthémoglobinémie est une complication potentiellement grave et peu connue de l'intoxication par poppers. Dans le cas présenté, la méthémoglobinémie, suspectée par le médecin urgentiste, a pu être confirmée rapidement par une mesure non invasive à la gazométrie artérielle. La reconnaissance précoce de la méthémoglobinémie et l'initiation d'un traitement efficace ont permis une évolution rapidement favorable et d'éviter une défaillance multi-systémique pouvant conduire au décès du patient.

Efficacy and safety of corpus callosotomy versus vagus nerve stimulation as long-term adjunctive therapies in children with Lennox-Gastaut syndrome: Experience of a tertiary care center.

OBJECTIVES: To compare the efficacy and safety of corpus callosotomy versus vagus nerve stimulation (VNS) as long-term adjunctive therapies in children with Lennox-Gastaut syndrome. METHODS: This retrospective study was conducted in King Fahad Medical City between 2010 and 2019. The authors identified and followed 9 patients with Lennox-Gastaut syndrome (LGS) who underwent corpus callosotomy or VNS implantation for at least 12 months; seizure frequency and major complications were monitored. Five patients with a mean age of 10.8±1.3 years had corpus callosotomy, and 4 patients with a mean age of 13.8±3.9 years were implanted with VNS stimulators. RESULTS: Reduction in seizure frequency was achieved in all 5 patients who underwent corpus callosotomy, with greater than 75% seizure reduction in more than 50% in one, and greater than 25% in 2 respectively. However, in those implanted with VNS, 2 (50%) patients achieved a reduction in seizure frequency of greater than 75% and 2 (50%) greater than 25%, respectively. No significant difference was observed between the 2 treatment groups. One patient who underwent corpus callosotomy suffered cerebrospinal fluid leakage, and swallowing difficulties in one patient who underwent VNS. CONCLUSION: Both corpus callosotomy and VNS are safe and effective as adjunctive treatments for LGS patients.

Prostatitis, the Great Mimicker of Prostate Cancer: Can We Differentiate Them Quantitatively With Multiparametric MRI?

OBJECTIVE. The purpose of this study was to investigate the diagnostic performance of semiquantitative and quantitative pharmacokinetic parameters and quantitative apparent diffusion coefficient (ADC) values obtained from prostate multiparametric MRI (mpMRI) to differentiate prostate cancer (PCa) and prostatitis objectively. MATERIALS AND METHODS. We conducted a retrospective review of patients with biopsy-proven PCa or prostatitis who underwent mpMRI study between January 2015 and February 2018. Mean ADC, forward volume transfer constant (Ktrans), reverse volume transfer constant (kep), plasma volume fraction (Vp), extravascular extracellular space volume fraction (Ve), and time to peak (TTP) values were calculated for both lesions and contralateral normal prostate tissue. Signal intensity-time curves were analyzed. Lesion-to-normal prostate tissue ratios of pharmacokinetic parameters were also calculated. The diagnostic accuracy and cutoff points of all parameters were analyzed to differentiate PCa from prostatitis. RESULTS. A total of 138 patients (94 with PCa and 44 with prostatitis) were included in the study. Statistically, ADC, quantitative pharmacokinetic parameters (Ktrans, kep, Ve, and Vp), their lesion-to-normal prostate tissue ratios, and TTP values successfully differentiated PCa and prostatitis. Surprisingly, we found that Ve values were significantly higher in prostatitis lesions. The combination of these parameters had 92.7% overall diagnostic accuracy. ADC, kep, and TTP made up the most successful combination for differential diagnosis. Analysis of the signal intensity-time curves showed mostly type 2 and type 3 enhancement curve patterns for patients with PCa. Type 3 curves were not seen in any prostatitis cases. CONCLUSION. Quantitative analysis of mpMRI differentiates PCa from prostatitis with high sensitivity and specificity, appears to have significant potential, and may improve diagnostic accuracy. In addition, evaluating these parameters does not cause any extra burden to the patients.

[Complication of a successful cardiopulmonary resuscitation]. / Cas clinique. Complication d'une réanimation cardiopulmonaire réussie.

A case of bilateral extensive subcutaneous emphysema secondary to external cardiac massage is reported. This is a rare complication. It occurs with a pneumothorax after resuscitation maneuvers. The diagnosis is based on a bundle of anamnestic, clinical and radiological arguments. This case demonstrates the importance of performing a simple prehospital thoracostomy on the finger without chest tube placement and demonstrates the need for systematic investigation of possible complications in any patient who has undergone external cardiac massage.

Un cas d'emphysème sous-cutané, extensif, bilatéral, consécutif à un massage cardiaque externe, est rapporté. Il s'agit d'une complication rare. Celle-ci survient avec un pneumothorax après les manœuvres de réanimation. Le diagnostic est basé sur un faisceau d'arguments anamnestiques, cliniques et radiologiques. Ce cas montre l'importance de la réalisation d'une thoracostomie préhospitalière simple, au doigt, sans pose de drain thoracique et démontre la nécessité de la recherche systématique de complications éventuelles chez tout malade ayant subi un massage cardiaque externe en cas de dégradation clinique.