Myelin oligodendrocyte glycoprotein antibody-associated disease in children: Are there MRI predictors of relapse?

Background and PurposeMyelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is increasingly recognized in children. Some children have isolated disease while others relapse. The study evaluates clinical, demographic and imaging features children with positive anti-MOG antibodies comparing to previously reported findings and correlate patterns on MR imaging with a relapsing course in MOGAD.Material and methodsAll pediatrics patients with serum anti-MOG antibodies were reviewed. Demographic, clinical, and imaging data were evaluated. Patients with a relapsing course were compared to those with a single event. We assessed initial MR images of the brain, orbits and spine obtained at the onset of clinical symptoms, whether performed at our institution or elsewhere.ResultsThirty patients were included, fourteen with a single event and sixteen with more than one clinical event. The mean age was 8.1 years, with a mean follow-up of 58 months (range of 0.67 to 238 months). The relapsing patients had a mean of 3.5 relapses (range 2–12). 55% of patients had long segment optic nerve lesions, 53% of patients had cortical or peripheral white matter lesions, and 46% of patients had thalamic lesions. 43% of patients had spinal cord lesions, with 39% involving the central cord and 26% with long segment involvement. The imaging features between the groups were not statistically significant.ConclusionThere were no distinguishing features in relapsing versus non-relapsing patients. In the absence of any predictive characteristics for future relapse, patients should have regular clinical and imaging follow up.     

Pathogenesis of histologic variations of appendiceal mucinous neoplasms

BackgroundIn order for peritoneal metastases from a primary appendiceal mucinous neoplasm to occur, the wall of the appendix must perforate to allow mucus with tumor cells access to the peritoneal spaces. With progression the peritoneal metastases show a broad spectrum of tumor biology varying from indolent to aggressive activity.MethodsThe histopathology of peritoneal tumor masses was determined from the clinical material resected at the time of cytoreductive surgery (CRS). All groups of patients were treated by a uniform strategy that involved complete CRS and perioperative intraperitoneal chemotherapy. Overall survival was determined.ResultsFrom a database of 685 patients, four histologic subtypes were identified and long-term survival determined. Four hundred and fifty patients (66.0%) had low-grade appendiceal mucinous neoplasm (LAMN), 37 patients (5.4%) had mucinous appendiceal adenocarcinoma of intermediate subtype (MACA-Int), 159 patients (23.2%) had mucinous appendiceal adenocarcinoma (MACA), and 39 patients (5.4%) had a mucinous appendiceal adenocarcinoma with positive lymph nodes (MACA-LN). The mean survival of the four groups was 24.5, 14.8, 11.2 and 7.4 years, respectively (p < 0.0001). These four subtypes of mucinous appendiceal neoplasms were shown to have distinct survival estimates.ConclusionsThe estimated survival of these four histologic subtypes in patients having a complete CRS plus HIPEC is of value to the oncologist managing these patients. A mutations and perforations hypothesis was offered in an attempt to explain the broad spectrum of mucinous appendiceal neoplasms that exist. Inclusion of MACA-Int and MACA-LN as standalone subtypes was thought to be necessary.     

Background rates of adverse events of special interest for COVID-19 vaccine safety monitoring in the United States, 2019–2020

BackgroundThe U.S. Food and Drug Administration (FDA) Biologics Effectiveness and Safety (BEST) Initiative conducts active surveillance of adverse events of special interest (AESI) after COVID-19 vaccination. Historical incidence rates (IRs) of AESI are comparators to evaluate safety.MethodsWe estimated IRs of 17 AESI in six administrative claims databases from January 1, 2019, to December 11, 2020: Medicare claims for adults ≥ 65 years and commercial claims (Blue Health Intelligence®, CVS Health, HealthCore Integrated Research Database, IBM® MarketScan® Commercial Database, Optum pre-adjudicated claims) for adults < 65 years. IRs were estimated by sex, age, race/ethnicity (Medicare), and nursing home residency (Medicare) in 2019 and for specific periods in 2020.ResultsThe study included >100 million enrollees annually. In 2019, rates of most AESI increased with age. However, compared with commercially insured adults, Medicare enrollees had lower IRs of anaphylaxis (11 vs 12–19 per 100,000 person-years), appendicitis (80 vs 117–155), and narcolepsy (38 vs 41–53). Rates were higher in males than females for most AESI across databases and varied by race/ethnicity and nursing home status (Medicare). Acute myocardial infarction (Medicare) and anaphylaxis (all databases) IRs varied by season. IRs of most AESI were lower during March–May 2020 compared with March–May 2019 but returned to pre-pandemic levels after May 2020. However, rates of Bell’s palsy, Guillain-Barré syndrome, narcolepsy, and hemorrhagic/non-hemorrhagic stroke remained lower in multiple databases after May 2020, whereas some AESI (e.g., disseminated intravascular coagulation) exhibited higher rates after May 2020 compared with 2019.ConclusionAESI background rates varied by database and demographics and fluctuated in March–December 2020, but most returned to pre-pandemic levels after May 2020. It is critical to standardize demographics and consider seasonal and other trends when comparing historical rates with post-vaccination AESI rates in the same database to evaluate COVID-19 vaccine safety.     

Bone graft tuberculosis outbreak in USA: Is it a concern in India?

Globally, 25% of the population is infected with tuberculosis, which poses a leading cause of death worldwide. The transmission of tuberculosis (TB) during organ transplant is reported in the literature whereas only one report has been published on the transmission of TB, during bone allograft transplantation. In the US, in May 2021, an outbreak of TB occurred in patients undergoing spine surgery with bone allograft. This bone graft was retrieved from 80 years deceased donor with latent TB, which was not diagnosed earlier. The recipients were started with a long course of anti-tuberculous drugs. This review narrates the pathway of TB spread among transplant recipients and the strategies to be followed while performing organ or tissue transplantation.     

First Japanese case of disseminated blastomycosis imported from North America: A case report

Blastomycosis is a fungal infectious disease that can occur in both immunocompromised and immunocompetent populations endemic in North America, with no previous reports in Japan.A 26-year-old Japanese female patient with no relevant medical history presented intermittent left back pain and an abnormal shadow in the left upper lung field eight months ago at a local clinic. She was referred to our hospital for further evaluation and treatment. The patient currently lives in Japan, but until two years ago had spent several years in New York, Vermont and California. Chest computed tomography revealed a 30 mm mass with a cavity in the left pulmonary apex. The specimens obtained by transbronchial biopsy showed periodic acid-Schiff stain (PAS)-positive and Grocott-positive yeast-like fungi scattered among the granulomas, with no malignant findings, and the initial pathology did not lead to a definitive diagnosis. She was empirically started on fluconazole because of onset of multiple subcutaneous abscesses and was referred to the Medical Mycology Research Center. Although antibody tests could not diagnose the disease, blastomycosis was suspected based on the pathology of the skin and lung tissue at the Medical Mycology Research Center, and Blastomyces dermatitidis was identified by ITS analysis of the rRNA region. Her symptoms and CT findings gradually improved with fluconazole. We reported the first Japanese case of blastomycosis with pulmonary and cutaneous involvement in Japan. As the number of overseas travelers is expected to continue increasing, we would like to emphasize the importance of travel history interviews and information of blastomycosis.     

不同严重程度脓毒症患者血栓弹力图特点及预测价值北大核心CSCD

目的探讨血栓弹力图在脓毒症患者病情严重程度以及预后等方面的相关性以及预测价值。方法回顾性分析2013年1月1日-2019年12月31日期间于上海市交通大学附属瑞金医院急诊ICU及急诊内科病房收治的147例脓毒症患者,入院24 h内行炎症指标及血栓弹力图检测,同时行APECHEⅡ评分,根据APACHEⅡ评分分为轻症组和重症组,观察不同组间血栓弹力图的差异;在脓毒症患者中分为死亡组和存活组,评价对脓毒症患者预后的判断价值。结果重度的脓毒症较轻度的脓毒症其α角减小(P=0.015)、K时间延长(P=0.015),最大血块强度增强(P=0.045)。脓毒症死亡组的K时间明显大于存活组的K时间。当K时间≥2.2 min时(敏感度为77.27%,特异度为95.20%)脓毒症发生死亡的风险最大(P<0.01);结论重度脓毒症患者表现明显的低凝状态,K时间对脓毒症患者的预后具有预判价值。     

Non-traumatic bilateral rectus sheath hematoma during septic disseminated intravascular coagulation

A non-traumatic abdominal wall hematoma is rare, and occurs occasionally due to coughing, physical activity, or antithrombotic/anticoagulant therapy. The condition is usually unilateral; however, rare bilateral cases have been reported. Here, we report a rare case of a non-traumatic bilateral rectus sheath hematoma. The patient was a 60-year-old woman who was urgently admitted to our hospital due to the occurrence of pneumonia during postoperative chemotherapy for breast cancer. Because she exhibited disseminated intravascular coagulation, a therapy with antibacterial agents, thrombomodulin alpha, and catecholamines was initiated. During hospitalization, hemorrhagic shock due to hematomas in both rectus abdominis muscles was observed without any discernible cause. Subsequent emergency angioembolization was successful, and abdominal computed tomography performed 3 months after the onset of the rectus sheath hematoma confirmed a reduction in the hematoma size.     

近20年妊娠急性脂肪肝患者预后变化和死亡原因分析

目的 分析近20年我科诊治的妊娠急性脂肪肝(AFLP)患者的临床特征。方法 2002年1月～2011年12月北京佑安医院收治AFLP患者25例(A组),2012年1月～2022年1月收治28例(B组),对比分析两组临床表现、并发症、病死率变化和死亡原因。结果 A组和B组血清总胆红素水平分别为 (239.8±104.2)μmol/l和【(161.9±107.7)μmol/l, P=0.052】, A组肝功能异常率为12.0%,显著低于B组的42.9%(P=0.006);A组剖宫产率为76.0%,显著低于B组的100.0%(P=0.020);A组与B组DIC(40.0%对3.6%,P=0.001)和失血性休克发生率(24.0%和3.6%,P=0.028)存在显著性差异,而产后出血(48.0%对25.0%,P=0.081)无显著性差异;B组产妇病死率为3.6%而胎儿无死亡,显著低于A组的24.0%和12.1%(P<0.05)。结论 DIC和大出血是10年前AFLP产妇的主要死亡原因,近10年AFLP母婴病死率明显下降,但肝衰竭进展仍较常见,仍需早期识别和处理。     

Sinusoidal obstruction syndrome associated with disseminated toxoplasmosis involving the liver after allogeneic hematopoietic stem cell transplantation: A case report

Sinusoidal obstruction syndrome (SOS) is a fatal complication after hematopoietic stem cell transplantation (HSCT). Only a few complications after HSCT have been reported as risk factors for SOS, including sepsis. Here, we report the case of a 35-year-old male diagnosed with Philadelphia chromosome-positive acute lymphoblastic leukemia who underwent peripheral blood HSCT from a human leukocyte antigen-matched unrelated female donor in remission. Graft-versus-host disease prophylaxis contained tacrolimus, methotrexate, and low-dose anti-thymoglobulin. The patient was treated with methylprednisolone for engraftment syndrome from day 22. On day 53, he presented worsening fatigue, breathlessness, and abdominal pain in the right upper quadrant that had persisted for 4 days. Laboratory tests showed severe inflammation, liver dysfunction, and positive for Toxoplasma gondii PCR. He died on day 55. An autopsy showed SOS and disseminated toxoplasmosis. Hepatic infection with T. gondii was identified in zone 3 of the liver, which overlapped with the pathological features of SOS. In addition, the timing of the exacerbation of hepatic dysfunction coincided with the onset of systemic inflammatory symptoms and T. gondii reactivation. This rare case of toxoplasmosis is the first to suggest that hepatic infection with T. gondii is strongly associated with SOS after HSCT.