Factor XIII Deficiency With Repeated Severe Postoperative Bleeding After Laparotomy: A Case Report.

Patients with factor XIII (FXIII) deficiency present with a bleeding tendency that is difficult to diagnose because their coagulation test results are normal. We herein report a case of a 74-year-old male who presented to our hospital in cardiac arrest. After resuscitation, he was found to have sigmoid volvulus and necrosis; therefore, an emergency laparotomy was performed. Intraoperative findings revealed an extensive strangulated ileus in addition to sigmoid volvulus. We performed resection without reconstruction and maintained open abdominal management (OAM) for six days. After abdominal closure, the patient experienced postoperative bleeding four times from the mesenteric transection; three of the bleeding episodes required open hemostasis. Since he had mild coagulopathy during each bleeding episode, FXIII deficiency was suspected and diagnosed. After administration of FXIII concentrate, the tendency to intraoperative bleeding improved significantly. FXIII deficiency should be considered in cases of repeated severe bleeding, even when coagulation tests reveal no major abnormalities.

Disseminated Mycobacterium abscessus infection with osteoarticular manifestations as an important differential diagnosis of inflammatory arthritis: A case report and literature review.

This case report describes a 52-year-old immunocompromised man diagnosed with disseminated Mycobacterium abscessus complex (MABC) infection. The patient had a history of malignant lymphoma and presented with fever and polyarthritis that lasted 3 weeks. Upon initial evaluation, blood and synovial fluid cultures from the swollen joints were negative. Reactive arthritis or rheumatoid arthritis was suspected as the cause of inflammatory synovitis in multiple joints. Administration of prednisolone followed by an interleukin-6 inhibitor improved the fever, but polyarthritis persisted, and destruction of the left hip joint was observed. Two months later, M. abscessus was detected in a blood culture and right shoulder joint synovium, leading to a final diagnosis of disseminated MABC infection. The joint symptoms resolved with combined antimicrobial therapy using amikacin, azithromycin, and imipenem/cilastatin. To date, 12 cases of disseminated MABC infection with osteoarticular manifestations have been reported. A total of 13 cases, including the present case, were reviewed. Seven patients had bone involvements, five had joint involvement, and the remaining one had bursa involvement. All the cases with joint involvement, except for our case, presented with monoarthritis. MABC infection is diagnosed based on the demonstration of MABC itself. Clinicians should keep disseminated MABC infection in mind as a possible cause of persistent arthritis. As demonstrated in our case, multiple replicate cultures of blood or specimens from the affected sites may be needed to detect it.

Seasonal Influence on Development of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Retrospective Cohort Study Conducted at Multiple Institutions in Japan (J-CANVAS).

OBJECTIVE: To clarify seasonal and other environmental effects on the onset of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We enrolled patients with new-onset eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and granulomatosis with polyangiitis (GPA) registered in the database of a Japanese multicenter cohort study. We investigated the relationship between environmental factors and clinical characteristics. Seasons were divided into 4 (spring, summer, autumn, and winter), and the seasonal differences in AAV onset were analyzed using Pearson chi-square test, with an expected probability of 25% for each season. RESULTS: A total of 454 patients were enrolled, with a mean age of 70.9 years and a female proportion of 55.5%. Overall, 74, 291, and 89 patients were classified as having EGPA, MPA, and GPA, respectively. Positivity for myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA was observed in 355 and 46 patients, respectively. Overall, the seasonality of AAV onset significantly deviated from the expected 25% for each season (P = 0.001), and its onset was less frequently observed in autumn. In ANCA serotypes, seasonality was significant in patients with MPO-ANCA (P < 0.001), but not in those with PR3-ANCA (P = 0.97). Additionally, rural residency of patients with AAV was associated with PR3-ANCA positivity and biopsy-proven pulmonary vasculitis. CONCLUSION: The onset of AAV was influenced by seasonal variations and was less frequently observed in autumn. In contrast, the occurrence of PR3-ANCA was triggered, not by season, but by rural residency.

Appearance of anti-MDA5 antibody-positive dermatomyositis after COVID-19 vaccination.

The direct causes of dermatomyositis, a common autoimmune disease, have not yet been accurately identified, but several studies have linked this condition to various patient-associated and environmental factors, such as viral infections and area of residence. In the present report, we describe our experience with a patient presenting with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis, which developed after vaccination against coronavirus disease 2019 (COVID-19). This patient was simultaneously diagnosed with anti-glutamic acid decarboxylase antibody-positive slowly progressive insulin-dependent diabetes (SPIDDM); her human leucocyte antigen test revealed that she expressed the DRB1*04:05 allele. This is important as this genotype is known to increase susceptibility to both anti-MDA5 antibody-positive dermatomyositis and type I diabetes. To the best of our knowledge, this is the first case of dermatomyositis complicated by SPIDDM identified after COVID-19 vaccination against COVID-19 and presenting with an underlying susceptible genotype. The patient's genetic predisposition may also be important for the development of autoimmune disease after COVID-19 vaccination.

Successful weaning of a patient with severe COVID-19 pneumonia under prolonged midazolam sedation using morphine.

The coronavirus disease 2019 (COVID-19) pandemic continues to spread around the world. In April 2021, Japan experienced a fourth wave of COVID-19 infections, which led to the breakdown of the medical system. Osaka, Japan, was particularly affected, with many severe cases and the highest number of COVID-19-associated deaths in Japan. Herein, we present a patient with severe COVID-19 infection who received prolonged midazolam (MDZ) treatment since propofol was not available due to shortage of medical resources. Moreover, the duration of mechanical ventilation was extended due to the development of a pneumothorax. When MDZ tapering was initiated, tachypnea was observed, which resulted failure in ventilator weaning. However, the use of continuous morphine infusion led a successful weaning off the ventilator. We suggest that the administration of morphine may allow for a smoother weaning process for some patients with severe COVID-19 infection.

Influence of Nutritional Management and Rehabilitation on Physical Outcome in Japanese Intensive Care Unit Patients: A Multicenter Observational Study.

BACKGROUND: There is lack of evidence regarding nutritional management among intensive care unit (ICU) patients in a population with relatively low body mass index. Therefore, we conducted an observational study to assess the nutritional management in Japanese ICUs. Also, we investigated the impact of nutritional management and rehabilitation on physical outcome. METHODS: The study population comprised 389 consecutive patients who received mechanical ventilation for at least 24 h and those admitted to the ICU for > 72 h in 13 hospitals. The primary outcomes were caloric and protein intake in ICU on days 3 and 7, and at ICU discharge. The secondary outcome was the impact of nutritional management and rehabilitation on physical status at ICU discharge. We defined good physical status as more than end sitting and poor physical status as bed rest and sitting. We divided the participants into 2 groups, namely, the good physical status group (Good group) and poor physical status group (Poor group) for analysis of the secondary outcome. Data were expressed as median (interquartile range). RESULTS: The median amount of caloric intake on days 3 and 7, and at ICU discharge via enteral and parenteral routes were 8.4 (3.1-15.6), 14.9 (7.5-22.0), and 11.2 (2.5-19.1) kcal/kg/day, respectively. The median amount of protein intake on days 3 and 7, and at ICU discharge were 0.2 (0-0.5), 0.4 (0.1-0.8), and 0.3 (0-0.7) g/kg/day, respectively. The amount of caloric intake on day 3 in the Poor group was significantly higher than that of the Good group (10.1 [5.8, 16.2] vs. 5.2 [1.9, 12.4] kcal/kg/day, p < 0.001). The proportion of patients who were received rehabilitation in ICU in the Good group was significantly higher than that of the Poor group (92 vs. 63%, p < 0.001). The multivariate analysis revealed that caloric intake on day 3 and rehabilitation in ICU were considered independent factors that affect physical status (OR 1.19; 95% CI 1.05-1.34; p = 0.005 and OR 0.07; 95% CI 0.01-0.34; p = 0.001). CONCLUSIONS: The caloric and protein intakes in Japanese ICUs were 15 kcal/kg/day and 0.4 g/kg/day, respectively. In addition, critically ill patients might benefit from low caloric intake (less than 10 kcal/kg/day) until day 3 and rehabilitation during ICU stay.

Impact of Gram stain results on initial treatment selection in patients with ventilator-associated pneumonia: a retrospective analysis of two treatment algorithms.

BACKGROUND: Ventilator-associated pneumonia (VAP) is a common and serious problem in intensive care units (ICUs). Several studies have suggested that the Gram stain of endotracheal aspirates is a useful method for accurately diagnosing VAP. However, the usefulness of the Gram stain in predicting which microorganisms cause VAP has not been established. The purpose of this study was to evaluate whether a Gram stain of endotracheal aspirates could be used to determine appropriate initial antimicrobial therapy for VAP. METHODS: Data on consecutive episodes of microbiologically confirmed VAP were collected from February 2013 to February 2016 in the ICU of a tertiary care hospital in Japan. We constructed two hypothetical empirical antimicrobial treatment algorithms for VAP: a guidelines-based algorithm (GLBA) based on the recommendations of the American Thoracic Society-Infectious Diseases Society of America (ATS-IDSA) guidelines and a Gram stain-based algorithm (GSBA) which limited the choice of initial antimicrobials according to the results of bedside Gram stains. The GLBA and the GSBA were retrospectively reviewed for each VAP episode. The initial coverage rates and the selection of broad-spectrum antimicrobial agents were compared between the two algorithms. RESULTS: During the study period, 219 suspected VAP episodes were observed and 131 episodes were assessed for analysis. Appropriate antimicrobial coverage rates were not significantly different between the two algorithms (GLBA 95.4% versus GSBA 92.4%; p = 0.134). The number of episodes for which antimethicillin-resistant Staphylococcus aureus agents were selected as an initial treatment was larger in the GLBA than in the GSBA (71.0% versus 31.3%; p < 0.001), as were the number of episodes for which antipseudomonal agents were recommended as an initial treatment (70.2% versus 51.9%; p < 0.001). CONCLUSIONS: Antimicrobial treatment based on Gram stain results may restrict the administration of broad-spectrum antimicrobial agents without increasing the risk of treatment failure. TRIAL REGISTRATION: UMIN-CTR, UMIN000026457 . Registered 8 March 2017 (retrospectively registered).