Balloon pulmonary angioplasty under awake veno-arterial extracorporeal membrane oxygenation in a patient with class III obesity with chronic thromboembolic pulmonary hypertension complicated with multiple serious comorbidities.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a chronic disease that can rapidly deteriorate into circulatory collapse when complicated by comorbidities. We herein describe a case involving a 43-year-old woman with class III obesity (body mass index of 63 kg/m2) and severe CTEPH associated with total occlusion of the left main pulmonary artery who subsequently developed circulatory collapse along with multiple comorbidities, including acute kidney injury, pulmonary tuberculosis, and catastrophic antiphospholipid syndrome. The patient was successfully treated with two sessions of rescue balloon pulmonary angioplasty with veno-arterial extracorporeal membrane oxygenation (V-A ECMO) support under local anesthesia without sedation, at cannulation and during the V-A ECMO run, to avoid invasive mechanical ventilation. This case suggests the potential usefulness of rescue balloon pulmonary angioplasty under awake V-A ECMO support for rapidly deteriorating, inoperable CTEPH in a patient with class III obesity complicated with multiple comorbidities.

Safety and tolerability of OP-724 in patients with haemophilia and liver cirrhosis due to HIV/HCV coinfection: an investigator-initiated, open-label, non-randomised, single-centre, phase I study.

OBJECTIVE: Patients with haemophilia and HIV who acquire hepatitis C virus (HCV) after receiving contaminated blood products can experience accelerated progression of liver fibrosis and a poor prognosis, making liver disease a prominent cause of mortality among these patients. In the current study, we aimed to evaluate the safety and tolerability of the potential antifibrotic agent OP-724-a CREB-binding protein/ß-catenin inhibitor-in this patient subset. DESIGN: In this single-centre, open-label, non-randomised, phase I trial, we sequentially enrolled patients with cirrhosis following HIV/HCV coinfection classified as Child-Pugh (CP) class A or B. Five patients received an intravenous infusion of OP-724 at doses of 140 or 280 mg/m2 for 4 hours two times weekly over 12 weeks. The primary endpoint was the incidence of serious adverse events (SAEs). Secondary endpoints included the incidence of AEs and improved liver stiffness measure (LSM), as determined by vibration-controlled transient elastography. This study was registered at ClinicalTrials.gov (NCT04688034). RESULTS: Between 9 February 2021 and 5 July 2022, five patients (median age: 51 years) were enrolled. All five patients completed 12 cycles of treatment. SAEs were not observed. The most common AEs were fever (60%) and gastrointestinal symptoms (diarrhoea: 20%, enterocolitis: 20%). Improvements in LSM and serum albumin levels were also observed. CONCLUSION: In this preliminary assessment, intravenous administration of 140 or 280 mg/m2/4 hours OP-724 over 12 weeks was well tolerated by patients with haemophilia combined with cirrhosis due to HIV/HCV coinfection. Hence, the antifibrotic effects of OP-724 warrant further assessment in patients with cirrhosis. TRIAL REGISTRATION NUMBER: NCT04688034.

Rapid Progression of Aortitis Caused by Methicillin-Sensitive Staphylococcus Aureus in a Patient With Pneumonia: A Case Report.

Infected aortic aneurysm is a rare but fatal disease that occurs through various mechanisms. In this report, we describe the case of a patient who was hospitalized for acute pneumonia and developed an infected aortic aneurysm in the descending aorta during the hospitalization. A 73-year-old Japanese man presented to the emergency department with a chief complaint of fever. He had a history of chronic renal failure due to nephrosclerosis and was on regular hemodialysis three times a week. The patient presented with an elevated inflammatory response, anemia, and low platelet counts after various tests. Computed tomography (CT) showed ground-glass opacity in the left lung with a small amount of pleural effusion, leading to a diagnosis of pneumonia. The patient was admitted to the hospital on the same day, and a course of antibiotics (ceftriaxone [CTRX]) was started. On the fourth day of hospitalization, methicillin-susceptible Staphylococcus aureus (MSSA) was detected in the blood sample, which was collected from the patient on the day of admission. The patient was treated for MSSA pneumonia and bacteremia, and the antibiotics were changed to cefazolin (CEZ). Treatment with antimicrobials resulted in a negative blood culture retest on day 5 and improvement of the inflammatory response. On the 12th day, improvements in pneumonia and pleurisy were observed on the CT scan; however, an abnormal bulge was seen on the dorsal side of the descending thoracic aorta with suspected partial vessel wall disruption, suggesting a ruptured infected aortic aneurysm. Despite treatment with antibiotics, the thoracic descending aortic aneurysm continued to dilate with progressing rupture, and the patient died on the 25th day of hospitalization. This is the first report of an infected aneurysm caused by Staphylococcus aureus, despite a negative blood culture. Patients at high risk might develop infected aneurysms, and the possibility of rapid dilation should always be considered.

Characteristics and prognosis of patients with COVID-19 and hematological diseases in Japan: a cross-sectional study.

The Japanese Society of Hematology performed an observational cross-sectional study to clarify the morbidity, prognosis, and prognostic factors in patients with COVID-19 with hematological diseases (HDs) in Japan. The study included patients with HDs who enrolled in our epidemiological survey and had a COVID-19 diagnosis and a verified outcome of up to 2 months. The primary endpoints were characteristics and short-term prognosis of COVID-19 in patients with HDs. A total of 367 patients from 68 institutes were enrolled over 1 year, and the collected data were analyzed. The median follow-up among survivors was 73 days (range, 1-639 days). The 60-day overall survival (OS) rate was 86.6%. In the multivariate analysis, albumin ≤ 3.3 g/dL and a need for oxygen were independently associated with inferior 60-day OS rates (hazard ratio [HR] 4.026, 95% confidence interval (CI) 1.954-8.294 and HR 14.55, 95% CI 3.378-62.64, respectively), whereas 60-day survival was significantly greater in patients with benign rather than malignant disease (HR 0.095, 95% CI 0.012-0.750). Together, these data suggest that intensive treatment may be necessary for patients with COVID-19 with malignant HDs who have low albumin levels and require oxygen at the time of diagnosis.

Successful initiation of hemodialysis for a hemophilia A patient with factor VIII inhibitor: a case report and literature review.

We report the first case of hemophilia A with factor VIII (FVIII) inhibitor who received hemodialysis via an arteriovenous (AV) fistula. Hemophilia A is a congenital deficiency of blood coagulation FVIII that is characterized by prolonged bleeding. Approximately 30% of patients with hemophilia develop allogeneic antibodies of FVIII. The inhibitors decrease the hemostatic effect of replacement therapy; thus, the prophylaxis strategy should be well designed. Prophylactic treatment with invasive procedures is needed to prevent excessive bleeding in patients with hemophilia undergoing hemodialysis. On the contrary, hemodialysis requires attention to the development of intracircuit coagulation during dialysis. Peritoneal dialysis or hemodialysis with a long-term tunneled central venous catheter has mainly been selected as the dialysis modality for patients with hemophilia and end-stage renal disease requiring renal replacement therapy because hemodialysis with an arteriovenous fistula may result in bleeding from the puncture site after each hemodialysis session. In our patient, hemodialysis was safely performed without any anticoagulant agents, and replacement therapy with FVIII concentrates prevented bleeding after puncture of the AV fistula.

Efficacy and Safety of Single-dose Pegfilgrastim for CD34 + Cell Mobilization in Healthy Volunteers: A Phase 2 Study.

BACKGROUND: Pegfilgrastim, a long-acting form of granulocyte-colony stimulating factor, with a convenient single-injection dosage, is being investigated for peripheral blood stem cell (PBSC) mobilization in healthy volunteers. However, data on the adequate dose of pegfilgrastim for PBSC mobilization are limited. This phase 2, single-arm study evaluated the efficacy and safety of pegfilgrastim for PBSC mobilization in healthy volunteers. METHODS: The study comprised 2 phases: pilot (steps 1-3, dose escalation, a single subcutaneous dose of 3.6, 7.2, and 10.8 mg pegfilgrastim, respectively) and evaluation (step 4, efficacy and safety assessments). The primary endpoint was the proportion of subjects who achieved mobilization of ≥20 × 10 6 /L cluster of differentiation 34 positive (CD34 + ) cells. RESULTS: Thirty-five subjects (6 each in steps 1 and 2 and 23 in step 4) were included. In the pilot phase, step 3 with a 10.8 mg dose was not conducted due to favorable outcomes in step 2 (desired CD34 + cell count), at 7.2 mg pegfilgrastim, which was identified as the optimal dose for the evaluation phase. In the evaluation phase, successful CD34 + mobilization was achieved in all 23 subjects. The mean peripheral blood CD34 + cells count peaked on day 5. Back pain, thrombocytopenia, transient elevations of alkaline phosphatase, and lactate dehydrogenase were the most common adverse events. All adverse events were mild, and none led to study discontinuation. CONCLUSIONS: A single-dose pegfilgrastim successfully mobilized an optimal number of CD34 + cells and was well tolerated. Pegfilgrastim could be an alternative option for PBSC mobilization in healthy volunteers. The trial was registered at www.clinicaltrials.gov (NCT03993639).

Whole-genome CRISPR screening identifies molecular mechanisms of PD-L1 expression in adult T-cell leukemia/lymphoma.

ABSTRACT: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive T-cell malignancy with a poor prognosis and limited treatment options. Programmed cell death ligand 1(PD-L1) is recognized to be involved in the pathobiology of ATLL. However, what molecules control PD-L1 expression and whether genetic or pharmacological intervention might modify PD-L1 expression in ATLL cells are still unknown. To comprehend the regulatory mechanisms of PD-L1 expression in ATLL cells, we performed unbiased genome-wide clustered regularly interspaced short palindromic repeat (CRISPR) screening in this work. In ATLL cells, we discovered that the neddylation-associated genes NEDD8, NAE1, UBA3, and CUL3 negatively regulated PD-L1 expression, whereas STAT3 positively did so. We verified, in line with the genetic results, that treatment with the JAK1/2 inhibitor ruxolitinib or the neddylation pathway inhibitor pevonedistat resulted in a decrease in PD-L1 expression in ATLL cells or an increase in it, respectively. It is significant that these results held true regardless of whether ATLL cells had the PD-L1 3' structural variant, a known genetic anomaly that promotes PD-L1 overexpression in certain patients with primary ATLL. Pevonedistat alone showed cytotoxicity for ATLL cells, but compared with each single modality, pevonedistat improved the cytotoxic effects of the anti-PD-L1 monoclonal antibody avelumab and chimeric antigen receptor (CAR) T cells targeting PD-L1 in vitro. As a result, our work provided insight into a portion of the complex regulatory mechanisms governing PD-L1 expression in ATLL cells and demonstrated the in vitro preliminary preclinical efficacy of PD-L1-directed immunotherapies by using pevonedistat to upregulate PD-L1 in ATLL cells.

Virological outcomes of various first-line ART regimens in patients harbouring HIV-1 E157Q integrase polymorphism: a multicentre retrospective study.

BACKGROUND: Integrase strand transfer inhibitors (INSTIs) are recommended as first-line ART for people living with HIV (PLWH) in most guidelines. The INSTI-resistance-associated mutation E157Q, a highly prevalent (2%-5%) polymorphism of the HIV-1 (human immunodeficiency virus type 1) integrase gene, has limited data on optimal first-line ART regimens. We assessed the virological outcomes of various first-line ART regimens in PLWH with E157Q in real-world settings. METHODS: A multicentre retrospective observational study was conducted on PLWH who underwent integrase genotypic drug-resistance testing before ART initiation between 2008 and 2019 and were found to have E157Q. Viral suppression (<50 copies/mL) rate at 24 and 48 weeks, time to viral suppression and time to viral rebound (≥100 copies/mL) were compared among the first-line ART regimens. RESULTS: E157Q was detected in 167 (4.1%) of 4043 ART-naïve PLWH. Among them, 144 had available clinical data after ART initiation with a median follow-up of 1888 days. Forty-five started protease inhibitors + 2 NRTIs (PI group), 33 started first-generation INSTI (raltegravir or elvitegravir/cobicistat) + 2 NRTIs (INSTI-1 group), 58 started once-daily second-generation INSTI (dolutegravir or bictegravir) + 2 NRTIs (INSTI-2 group) and eight started other regimens. In the multivariate analysis, the INSTI-2 group showed similar or favourable outcomes compared with the PI group for viral suppression rates, time to viral suppression and time to viral rebound. Two cases in the INSTI-1 group experienced virological failure. CONCLUSIONS: The general guideline recommendation of second-generation INSTI-based first-line ART for most PLWH is also applicable to PLWH harbouring E157Q.

Massive Gastric Dilatation and Multi-Organ Ischemia Due to Superior Mesenteric Artery Syndrome: A Rare Case Report.

BACKGROUND Superior mesenteric artery (SMA) syndrome, a rare condition in which the SMA and aorta occlude the third duodenal portion, can cause serious complications. We present the case of an 83-year-old Japanese man who presented with shock because of massive gastric dilatation due to SMA syndrome and developed multi-organ ischemia. CASE REPORT The day before admission, the patient had visited the emergency department with abdominal pain and was sent home following spontaneous symptom resolution, but experienced abdominal pain flare-up. His history included diabetes mellitus, hypertension, gastric ulcer, prostatic hypertrophy, esophageal hiatal hernia, and esophageal cancer. Plain computed tomography showed gastric dilatation and obstruction of the duodenal third portion by the SMA, leading to SMA syndrome diagnosis. Since a nasogastric tube could not be manually inserted into the stomach and the gastric dilatation could not be decompressed, the tube was inserted endoscopically. Endoscopy revealed mechanical obstruction of the gastric cardia and gastric mucosal ischemia. He was admitted to intensive care, and blood pressure was maintained with vasopressors and blood transfusion. The next day, contrast-enhanced computed tomography performed for persistently elevated lactate levels revealed extensive ischemia affecting multiple gastrointestinal organs. Surgery and other treatments were considered too risky due to the patient's advanced age and condition. Best supportive care was administered after discussion with the family, and he died on the second day of hospitalization. CONCLUSIONS SMA syndrome with extensive ischemia and infarction is rare. Given this, clinicians should remain vigilant for its potential complications.

Overwhelming Post-splenectomy Infection Caused by Escherichia coli 20 Years After Splenectomy: A Case Report.

Post-splenectomy patients are at increased risk of infection. This complication is called overwhelming post-splenectomy infection (OPSI), which is uncommon but has high mortality. We describe a case of a man in his 80s who presented with septic shock with purpura fulminans caused by pyelonephritis. He had undergone a splenectomy in his 50s and had been taking prednisolone for the past six months for suspected immunoglobulin G4 (IgG4)-related disease. He was admitted to the intensive care unit but died the day after admission. OPSI is generally caused by encapsulated bacteria. However, in the present case, the causative agent was Escherichia coli, a bacterium that typically causes urinary tract infections. Post-splenectomy patients are known to have compromised bacterial clearance, and accumulation of bacteria such as E. coli can induce acute sepsis after splenectomy. Thus, physicians must have a high index of suspicion when treating splenectomy patients for the possibility that they may rapidly deteriorate to severe conditions such as OPSI, and the patients must be informed about the risk of severe infections, which can be fatal.

Dominant-negative type of IKZF1 deletion showed a favorable prognosis in adult B-cell acute lymphoblastic leukemia.

IKZF1 deletion is a recurrent genomic alteration in B-cell acute lymphoblastic leukemia (B-ALL) and is divided into dominant-negative (DN) and loss of function (LOF) deletions. The prognostic impact of each deletion has not been fully elucidated. We retrospectively analyzed 117 patients with adult B-ALL including 60 patients with BCR::ABL1-positive B-ALL and 57 patients with BCR::ABL1-negative B-ALL by the fluorescence in situ hybridization (FISH) method for IKZF1 deletion and multiplex PCR for the 4 most common IKZF1 deletions (∆4-7, ∆2-7, ∆2-8, and ∆4-8). Samples, in which IKZF1 deletion was detected by FISH but a specific type of deletion was not identified by the PCR, were categorized as "other." Patients were classified into a DN group that had at least 1 allele of ∆4-7 (n = 23), LOF and other group (n = 40), and wildtype group (n = 54). DN type IKZF1 deletions were found in 33.3% of BCR::ABL1-positive cases and 5.2% of BCR::ABL1-negative cases. LOF and other type IKZF1 deletions were found in 43.4% of BCR::ABL1-positive cases and 24.6% of BCR::ABL1-negative cases. Patients with the DN group showed significantly higher overall survival (OS) than that of the LOF and other and WT groups (P = 0.011). Multivariate analysis including age, WBC counts, complex karyotype, and DN type IKZF1 deletion showed that the DN type of IKZF1 deletion (HR = 0.22, P = 0.013) had a positive impact and age ≥ 65 (HR = 1.92, P = 0.029) had a negative impact on OS. The prognostic impact of IKZF1 deletion depends on the type of deletion and DN type of IKZF1 deletion showed better prognosis in adult B-ALL patients.Clinical trial registration This study was part of a prospective observational study (Hokkaido Leukemia Net, UMIN000048611). It was conducted in compliance with ethical principles based on the Helsinki Declaration and was approved by the institutional review board of Hokkaido University Hospital (#015-0344).

Ultrasound vector flow imaging during veno-arterial extracorporeal membrane oxygenation in a thoracic aorta model.

In veno-arterial extracorporeal membrane oxygenation (VA-ECMO) treatment, the mixing zone is a key hemodynamic factor that determines the efficacy of the treatment. This study aimed to evaluate the applicability of a novel ultrasound technique called vector flow imaging (VFI) for visualizing complex flow patterns in an aorta phantom under VA-ECMO settings. VFI experiments were performed to image aortic hemodynamics under VA-ECMO treatment simulated in an anthropomorphic thoracic aorta phantom using a pulsatile pump (cardiac output: 2.7 L/min) and an ECMO pump with two different flow rates, 0.35 L/min and 1.0 L/min. The cardiac cycle of hemodynamics in the ascending aorta, aortic arch, and descending aorta was visualized, and the spatio-temporal dynamics of flow vectors were analyzed. VFI successfully visualized dynamic flow patterns in the aorta phantom. When the flow rate of the ECMO pump increased, ECMO flow was more dominant than cardiac output in the diastole phase, and the speed of cardiac output was suppressed in the systole phase. Vortex flow patterns were also detected in the ascending aorta and the arch under both ECMO flow rate conditions. The VFI technique may provide new insights into aortic hemodynamics and facilitates effective and safe VA-ECMO treatment.

Spontaneous Improvement of Aortitis Associated with Severe COVID-19 Infection-A Case Report.

Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.

Calcineurin inhibitor inhibits tolerance induction by suppressing terminal exhaustion of donor T cells after allo-HCT.

Calcineurin inhibitor-based graft-versus-host disease (GVHD) prophylaxis is standard in allogeneic hematopoietic stem cell transplantation (HCT) but fails to induce long-term tolerance without chronic GVHD (cGVHD) in a considerable number of patients. In this study, we addressed this long-standing question in mouse models of HCT. After HCT, alloreactive donor T cells rapidly differentiated into PD-1+ TIGIT+ terminally exhausted T cells (terminal Tex). GVHD prophylaxis with cyclosporine (CSP) suppressed donor T-cell expression of TOX, a master regulator to promote differentiation of transitory exhausted T cells (transitory Tex), expressing both inhibitory receptors and effector molecules, into terminal Tex, and inhibited tolerance induction. Adoptive transfer of transitory Tex, but not terminal Tex, into secondary recipients developed cGVHD. Transitory Tex maintained alloreactivity and thus PD-1 blockade restored graft-versus-leukemia (GVL) activity of transitory Tex and not terminal Tex. In conclusion, CSP inhibits tolerance induction by suppressing the terminal exhaustion of donor T cells, while maintaining GVL effects to suppress leukemia relapse.

A Case of Refractory Gastric Antral Vascular Ectasia Treated Successfully With Distal Gastrectomy and Billroth II Reconstruction.

Gastric antral vascular ectasia is a rare cause of upper gastrointestinal bleeding and an important cause of transfusion dependence. Although surgery should be considered when patients with gastric antral vascular ectasia become transfusion-dependent even after endoscopic treatment, surgery for such patients with cirrhosis on dialysis has not been reported. Our patient, a 62-year-old man with a history of cirrhosis and chronic kidney failure, experienced recurrent bloody stool. Upper endoscopic findings indicated a diagnosis of gastric antral vascular ectasia; therefore, we initiated therapy with argon plasma coagulation. Anemia developed, and despite a second argon plasma coagulation treatment, it remained difficult to control. During the six weeks of hospitalization, the patient received more than 40 units of red blood cells. The gastroenterologist determined that further treatment with argon plasma coagulation would increase the risk of gastric perforation; therefore, we performed distal gastrectomy with Billroth II reconstruction. The patient was discharged from the hospital 15 days after surgery and had no signs of anemia for more than one year after discharge. The case of our patient shows that although endoscopic therapy is the usual treatment for gastric antral vascular ectasia, surgery should be considered when anemia is difficult to control.

Outcome of extracorporeal membrane oxygenation use in severe accidental hypothermia with cardiac arrest and circulatory instability: A multicentre, prospective, observational study in Japan (ICE-CRASH study).

AIM: To elucidate the effectiveness of extracorporeal membrane oxygenation (ECMO) in accidental hypothermia (AH) patients with and without cardiac arrest (CA), including details of complications. METHODS: This study was a multicentre, prospective, observational study of AH in Japan. All adult (aged ≥18 years) AH patients with body temperature ≤32 °C who presented to the emergency department between December 2019 and March 2022 were included. Among the patients, those with CA or circulatory instability, defined as severe AH, were selected and divided into the ECMO and non-ECMO groups. We compared 28-day survival and favourable neurological outcomes at discharge between the ECMO and non-ECMO groups by adjusting for the patients' background characteristics using multivariable logistic regression analysis. RESULTS: Among the 499 patients in this study, 242 patients with severe AH were included in the analysis: 41 in the ECMO group and 201 in the non-ECMO group. Multivariable analysis showed that the ECMO group was significantly associated with better 28-day survival and favourable neurological outcomes at discharge in patients with CA compared to the non-ECMO group (odds ratio [OR] 0.17, 95% confidence interval [CI]: 0.05-0.58, and OR 0.22, 95%CI: 0.06-0.81). However, in patients without CA, ECMO not only did not improve 28-day survival and neurological outcomes, but also decreased the number of event-free days (ICU-, ventilator-, and catecholamine administration-free days) and increased the frequency of bleeding complications. CONCLUSIONS: ECMO improved survival and neurological outcomes in AH patients with CA, but not in AH patients without CA.

Three-stage total knee arthroplasty combined with deformity correction and leg lengthening using Taylor spatial frames and conversion to internal fixation for severe intra- and extra-articular deformities and hypoplasia in a patient with hemophilic knee arthropathy: A case report.

BACKGROUND: Hemophilic arthropathy is a cause of severe knee deformity, because chronic synovitis due to repeated hemarthrosis affects the area of the epiphyseal plates in juvenile cases. Total knee arthroplasty (TKA) is the standard treatment for end-stage knee arthropathy. However, it is difficult to perform one-stage TKA in patients with severe intra- and extra-articular deformities. CASE PRESENTATION: We reported a case of hemophilic arthropathy in a 55-year-old male with leg length discrepancy of 4 cm, limited range of knee motion (-40° extension and 85° flexion), intra-articular deformity (medial proximal tibial angle: 69°; mechanical lateral distal femoral angle: 79°), extra-articular deformity at the distal femoral metaphyseal (30° valgus and 45° flexion deformity), and varus malalignment (% mechanical axis: 33%). We planned a three-stage TKA. Firstly, we performed gradual correction and lengthening of the distal femur using Taylor spatial flame. Six months after surgery, we performed conversion surgery from external fixation to internal fixation. Finally, we performed TKA with rotating hinged type implant. Two years after surgery, physical examination showed a normal gait, leg length discrepancy of 2 cm (the right leg was shorter), improvement in the range of knee motion (0° extension and 100° flexion). CONCLUSION: To the best of our knowledge, this presents the first combination of three-stage TKA with correction of femoral deformity and leg lengthening using a Taylor spatial frame and conversion to internal fixation in a patient with hemophilic knee arthropathy and severe intra- and extra-articular deformities.

Spinal cord infarction after withdrawal of veno-arterial extracorporeal membrane oxygenation for cardiogenic shock: A case report.

BACKGROUND: Spinal cord infarction is a rare central nervous system angiopathy that impairs motor, sensory, and autonomic nerves and occurs due to various reasons. This study reports a case of spinal cord infarction in a patient following myocardial infarction that was managed by veno-arterial extracorporeal membrane oxygenation (VA-ECMO). CASE SUMMARY: A 78-year-old Japanese man visited the emergency department with a complaint of chest tightness. He had a history of hypertension, dyslipidemia, diabetes, chronic renal failure, and postoperative bladder cancer. Myocardial infarction was diagnosed after ST elevation in lead aVR was identified by electrocardiogram during the visit, and cardiopulmonary arrest occurred twice during our examination and treatment. After percutaneous coronary intervention with an intra-aortic balloon pump and VA-ECMO, the patient was admitted to the intensive care unit. His circulation stabilized, and he was withdrawn from the intra-aortic balloon pump on day 3 of illness and from VA-ECMO on day 4. However, his consciousness remained impaired. When the patient's consciousness improved on day 14, lower limb weakness was identified. Magnetic resonance imaging conducted on the following day revealed spinal cord infarction in the 5th to 12th thoracic vertebrae. CONCLUSION: Spinal cord infarction due to VA-ECMO is extremely rare but has a poor neurological prognosis upon onset. Necessary countermeasures include conducting regular neurological examinations and high blood pressure maintenance, which is very difficult in VA-ECMO patients. Therefore, patient care will benefit from the experiences reported in such cases.

Development, implementation, and refinement of a comprehensive postcardiac arrest care training course in Japan.

Aim: In Japan, no training course is dedicated to postcardiac arrest care (PCAC), including venoarterial extracorporeal membrane oxygenation (VA-ECMO); thus, faculty members of the Japanese Circulation Society developed an original, comprehensive PCAC training course. This report reviews the development, implementation, and refinement of this PCAC training course. Methods: We examined the preserved data from the Japanese Circulation Society PCAC training courses between 2014 and 2020. Data related to the learning content and number of the attendees and instructors were collected and summarized. Results: Sixteen courses were held between August 2014 and February 2020, before the coronavirus disease 2019 (COVID-19) pandemic. A total of 677 health care providers participated: 351 doctors, 225 nurses, 62 perfusionists, five emergency medical professionals, and two pharmacists. Thirty-two attendees' data were missing. The core learning contents of all the courses included a standardized postcardiac arrest algorithm, targeted temperature management, VA-ECMO cannulation skills, and postcannulation management. Concerning curriculum evolution, extracorporeal cardiopulmonary resuscitation simulation, postarrest neurological examination and monitoring, and ultrasound-guided Seldinger technique training were added in the 4th, 5th, and 13th courses, respectively. Conclusion: The Japanese Circulation Society PCAC training course has been developed and refined to provide an organized, comprehensive opportunity for health care providers to acquire specific knowledge and skills in PCAC and VA-ECMO.