Outcomes of critically ill patients with liver failure who require mechanical ventilation: A retrospective, single-center study.

Background and Aims: Critically ill patients with liver failure have high mortality. Besides the management of organ-specific complications, liver transplantation constitutes a definitive treatment. However, clinicians may hesitate to introduce mechanical ventilation for patients on liver transplantation waitlists because of poor prognosis. This study investigated the outcomes of intensive care and ventilation support therapy effects in patients with liver failure. Methods: This single-center study retrospectively enrolled 32 consecutive patients with liver failure who were admitted to the intensive care unit from January 2014 to December 2020. The medical records were reviewed and analyzed retrospectively for Acute Physiologic and Chronic Health Evaluation (APACHE)-II. The model for end-stage liver disease scores, 90-day mortality, and survival was assessed using the Kaplan-Meier method. Results: The average patient age was 45.5 ± 20.1 years, and 53% of patients were women. On intensive care unit admission, APACHE-II and model for end-stage liver disease scores were 20 and 28, respectively. Among 13 patients considered for liver transplantation, 4 received transplants. Thirteen patients (40.6%) were intubated and mechanically ventilated in the intensive care unit. The 90-day mortality rate of patients with and without mechanical ventilation in the intensive care unit (13, 61.5% vs. 19, 47.4%, p = 0.4905) was similar. APACHE-II score >21 was an independent predictor of mechanical ventilation requirement in patients with liver failure during intensive care unit stay. Conclusion: Although critically ill patients with liver failure are at risk of multiorgan failure with poor outcomes, mechanical ventilation did not negatively affect the 90-day mortality or performance rates of liver transplantation. Clinicians should consider mechanical ventilation-based life support in critically ill patients with liver failure who are awaiting liver transplantation.

Effect of multidrug therapy on the prognosis of Mycobacterium avium complex pulmonary disease.

Multidrug therapy for Mycobacterium avium complex pulmonary disease (MAC-PD) results in negative sputum cultures. However, the prognostic value of this treatment approach remains unclear. This study aimed to clarify whether multidrug therapy reduces the incidence of events related to MAC-PD and improves the mortality rate. Patients who met the diagnostic criteria for MAC-PD at our hospital between 2003 and 2019 were retrospectively evaluated using medical records. Events related to MAC-PD were defined as hospitalisation for haemoptysis or respiratory infection and the development of chronic respiratory failure. There were 90 and 108 patients in the multidrug and observation groups, respectively. The median observation period was 86 months. Intergroup differences in body mass index, proportion of patients with cavities, and erythrocyte sedimentation rate were not significant. However, the observation group was older with a higher mean age (multidrug group: 62 years, observation group: 69 years; P < 0.001) and had a higher proportion of male patients (multidrug group: 13/90 [14.4%], observation group: 35/108 [32.4%]; P < 0.01). Furthermore, intergroup differences in the incidence of events related to MAC-PD (multidrug group: 26.69/1000 person-years, observation group: 25.49/1000 person-years), MAC-PD-associated mortality rate (multidrug group: 12.13/1000 person-years, observation group: 12.74/1000 person-years), and total mortality (multidrug group: 24.26/1000 person-years, observation group: 29.50/1000 person-years) were not significant. Many patients relapse even after multidrug therapy, and our findings suggest that multidrug therapy has no effect in preventing the onset of respiratory events or prolonging life expectancy.

Effects and Usefulness of Inspiratory Muscle Training Load in Patients with Advanced Lung Cancer with Dyspnea.

BACKGROUND: Patients with advanced lung cancer tend to experience dyspnea. Pulmonary rehabilitation has been reported as a method for relieving dyspnea. However, exercise therapy imposes a high burden on patients, and it is difficult to sustain in many cases. Inspiratory muscle training (IMT) imposes a relatively low burden on patients with advanced lung cancer; however, its benefits have not been demonstrated. METHODS: We retrospectively analyzed 71 patients who were hospitalized for medical treatment. The participants were divided into an exercise therapy group and an IMT load + exercise therapy group. Changes in maximal inspiratory pressure (MIP) and dyspnea were examined using a two-way repeated measures analysis of variance. RESULTS: MIP variations significantly increase in the IMT load group, with significant differences between baseline and week 1, between week 1 and week 2, and between baseline and week 2. The analysis also showed that the variations in dyspnea decreased in the IMT load + exercise therapy group with significant differences between baseline and week 1 and between baseline and week 2. CONCLUSIONS: The results show that IMT is useful and has a high persistence rate in patients with advanced lung cancer who present dyspnea and cannot perform high-intensity exercise therapy.

Clinical Outcomes and Prevalence of Sarcopenia in Patients with Moderate to Severe COVID-19.

Background: The purpose of this study was to investigate the effectiveness and clinical outcomes of inpatient rehabilitation for patients with severe COVID-19 in Japan. Methods: Patients with severe COVID-19 who underwent rehabilitation during hospitalization were included. The Medical Research Council (MRC) score and short physical performance battery (SPPB), such as physical function assessment and the intensive care unit (ICU) mobility scale, the functional status score for the ICU, and Barthel index as activities of daily living (ADLs) were evaluated at admission and discharge or transfer from the hospital. The correlation between SPPB at discharge and each factor at admission were also analyzed. Furthermore, the prevalence of sarcopenia was evaluated by defining SPPB of <9 points at discharge as sarcopenia. Results: The median age of the total of 23 patients was 59 years (interquartile range (IQR): 47−67), 73.9% were male, and the median PaO2/FiO2 at admission was 172.0 (IQR: 123.0−209.0). All physical function and ADL parameters were significantly improved from the time of admission to discharge (p = 0.014 for the MRC score and p < 0.001 for all others). Moreover, SPPB at discharge significantly correlated with WBC (Spearman's rho = −0.473, p = 0.041), C-reactive protein (Spearman's rho = −0.468, p = 0.044), and exhibited a significant trend with PaO2/FiO2 (Spearman's rho = 0.429, p = 0.067) and age (Spearman's rho = 0.409, p = 0.083). Although the median Barthel index at discharge was 90 points, 47% of patients had sarcopenia as defined by an SPPB of <9 points. Conclusions: Early rehabilitation for patients with severe COVID-19 improved physical function and ADLs during hospitalization. However, 47% of patients had the same level of sarcopenia at discharge.

Improvement of catatonia-induced rapid respiratory failure with electroconvulsive therapy: A case report.

We encountered a case of sudden respiratory failure during treatment of catatonia that required intensive care. Electroconvulsive therapy (ECT) was administered in the intensive care unit while the patient was under systemic control. The catatonia symptom was relieved, and respiratory failure improved. Although a proximal venous thrombus was observed, anticoagulation therapy was continued during ECT, and the patient was successfully treated without causing a pulmonary embolism. It is crucial to monitor the patient's physical and psychological symptoms because respiratory status may deteriorate rapidly in a catatonic state.

Report of a combination of remdesivir, intravenous methylprednisolone pulse, and tocilizumab for severe coronavirus disease: 20-case series at a single institution.

Many drugs have been marketed for treating coronavirus disease 2019 (COVID-19) infection, the disease that has caused a worldwide pandemic. However, in reported clinical trials, almost 30% of patients with COVID-19 did not show any health improvement. The 28-day survival rate was 69.5% when patients who required highflow oxygen therapy (HFNC), ventilation, and extracorporeal membrane oxygenation (ECMO) management were treated with remdesivir. The mortality rate of patients receiving 6 mg dexamethasone was 27%, and that of patients treated with tocilizumab and steroids was 31%. These results are unsatisfactory, and treatment for patients with severe respiratory failure has not yet been established. In our institution, we used remdesivir, methylprednisolone (mPSL) pulse therapy, and tocilizumab in 20 patients with COVID-19 whose PaO2/FIO2 (P/F) ratio was <200, and obtained good results for this combination therapy without any adverse events. In this study, we report the possible efficacy and safety of this treatment.

Successful Rescue of Life-threatening Hemoptysis Caused by Pulmonary Tuberculosis Bridging with Extracorporeal Membrane Oxygenation.

Massive hemoptysis is a fatal complication associated with pulmonary tuberculosis (TB). It can lead to severe respiratory failure. Extracorporeal membrane oxygenation (ECMO) is a life-saving technology that is rarely indicated for bleeding disorders. We herein report a 26-year-old man who presented with severe respiratory failure caused by massive hemoptysis with pulmonary TB. Transcatheter artery embolization was successfully performed with venovenous ECMO support. The hemostatic procedure allowed concomitant anticoagulant use, and neither bleeding nor thrombotic complications occurred throughout the clinical course. Administering the appropriate hemostatic procedure with subsequent management, including anticoagulant therapy, supported ECMO application in a case of bleeding.

Tolerability of nintedanib in the elderly with idiopathic pulmonary fibrosis: A single-center retrospective study.

Idiopathic pulmonary fibrosis (IPF), a fibrosing interstitial lung disease, predominantly affects the elderly and is associated with a high mortality risk. Nintedanib, a tyrosine kinase inhibitor, significantly reduces IPF progression. However, data on the tolerability and efficacy of nintedanib in the elderly with IPF are limited. Therefore, this study aimed to examine the tolerability and efficacy of nintedanib in the elderly with IPF in a real-world setting. Medical records of 19 elderly IPF patients (≥ 75 years) and 46 non-elderly IPF patients (< 75 years) newly administered nintedanib were retrospectively analyzed. We compared the forced vital capacity (FVC) level, incidence and severity of adverse events, and continuation rates of nintedanib between the two groups. FVC and percent predicted diffusing capacity of the lung for carbon monoxide (DLco) were lower in the elderly IPF group at baseline. Although the elderly IPF patients had a significantly higher incidence of adverse events, such as diarrhea, nausea, and elevation of hepatic enzymes, the rate of discontinuation of nintedanib owing to adverse events was not different between the groups. The continuation rates of nintedanib treatment at 6 months and 1 year in the elderly IPF group were equivalent. Furthermore, there was a similar trend in the reduction of the annual FVC decline after nintedanib initiation between the groups. Our study demonstrated that nintedanib was tolerable in both the IPF patient groups in a real-world setting. Proper management of adverse events in the elderly with IPF would lead to a better clinical outcome.

Effects of inspiratory muscle training after lung transplantation in children.

Pulmonary rehabilitation is a cornerstone of management for patients after lung transplantation (LT), but the benefits of inspiratory muscle training (IMT) after LT in children are unclear. Therefore, we examined whether IMT can improve respiratory function and dyspnoea in a paediatric patient after LT.The patient was a 13-year-old boy who underwent double LT. However, mild physical activity such as walking triggered dyspnoea for the patient. The patient underwent IMT with the intensity of approximately 30% of his maximal inspiratory pressure (MIP) for 2 months.The patient's MIP was increased by approximately 60% after 2 months, and his forced vital capacity as a percent of the predicted normal value increased from 74.6% to 83.4%, with improvement of dyspnoea.IMT may help improve dyspnoea after LT in children with respiratory muscle weakness and a decline in respiratory function.

CCR4-positive peripheral T-cell lymphoma presenting as eosinophilic pneumonia and developing from prolonged pustular psoriasis.

A 29-year-old woman with chronic, prolonged pustular psoriasis was admitted to our hospital because of high-grade fever and a systemic skin rash. General examination revealed a whole-body skin rash and superficial lymphadenopathy. Peripheral blood examination showed unclassified cells positive for CD3, CD4, and T-cell receptor αß, and negative for CD20 and CD56. Soon after administration, she developed acute respiratory failure and required artificial ventilation. Bronchoalveolar lavage fluid showed increased numbers of eosinophils and abnormal lymphocytes of the same phenotype in peripheral blood and skin. She was diagnosed with eosinophilic pneumonia, and her respiratory failure was improved by corticosteroid therapy. Based on the histological findings of skin, lymph node, and bone marrow biopsies, a diagnosis of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), with positivity for CC chemokine receptor 4 was made. She received chemotherapy followed by allogeneic stem cell transplantation, which resulted in complete remission of her PTCL-NOS. She remained alive and disease-free 6 years later. This is the first reported case of PTCL-NOS developing during the clinical course of pustular psoriasis. The clinical manifestations of PTCL-NOS are complex, but an accurate diagnosis and appropriate therapy may produce a good clinical outcome in patients with PTCL-NOS.

Guidelines for the diagnosis and treatment of acute encephalopathy in childhood.

The cardinal symptom of acute encephalopathy is impairment of consciousness of acute onset during the course of an infectious disease, with duration and severity meeting defined criteria. Acute encephalopathy consists of multiple syndromes such as acute necrotizing encephalopathy, acute encephalopathy with biphasic seizures and late reduced diffusion and clinically mild encephalitis/encephalopathy with reversible splenial lesion. Among these syndromes, there are both similarities and differences. In 2016, the Japanese Society of Child Neurology published 'Guidelines for the Diagnosis and Treatment of Acute Encephalopathy in Childhood', which made recommendations and comments on the general aspects of acute encephalopathy in the first half, and on individual syndromes in the latter half. Since the guidelines were written in Japanese, this review article describes extracts from the recommendations and comments in English, in order to introduce the essence of the guidelines to international clinicians and researchers.

Efficacy of hypothermia therapy in patients with acute encephalopathy with biphasic seizures and late reduced diffusion.

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic seizures and impaired consciousness. The efficacy of hypothermia/normothermia therapy in patients with AESD has rarely been reported on. METHODS: We enrolled 15 patients with AESD admitted to Yamaguchi University Hospital and Yamaguchi-ken Saiseikai Shimonoseki General Hospital between 2005 and 2019 and retrospectively evaluated the long-term efficacy of hypothermia therapy compared to that of non-hypothermia therapy. We compared the long-term sequelae of patients with AESD treated with or without hypothermia therapy. We used the Pediatric Cerebral Performance Category (PCPC) scale and intelligence tests including the Wechsler Intelligence Scale for Children, Tanaka-Binet Intelligence Scale, and Enjoji Infantile Developmental Scale to evaluate neurological sequelae and mental disability. The preventive effect of hypothermia therapy was assessed based on the development of post-encephalopathic epilepsy (PEE). RESULTS: There was no significant between-group difference in the PCPC score (p = 0.53). The subjects with severe mental disability in the hypothermia therapy group were 0 (0%), while those in the non-hypothermia group were 2 (29%); however, the difference was not significant. Notably, there were no patients with onset of PEE in the hypothermia therapy group, while there were 4 (57.1%) in the non-hypothermia group (p = 0.03). CONCLUSIONS: Our study suggests that hypothermia therapy may be effective in the long-term sequelae of AESD in terms of preventing the development of PEE. We propose that hypothermia therapy could contribute to improve the quality of life in these patients by preventing the subsequent onset of PEE.

Elevated quinolinic acid levels in cerebrospinal fluid in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a rare neurodegenerative disorder caused by a persistent infection with aberrant measles virus. Indoleamine-2, 3-dioxygenase (IDO) initiates the increased production of kynurenine pathway (KP) metabolites quinolinic acid (QUIN), which has an excitotoxic effect for neurons. We measured serum IDO activity and cerebrospinal fluid (CSF) levels of QUIN. The CSF QUIN levels were significantly higher in SSPE patients than in controls, and increased according as neurological disability in a patient studied. Elevation of CSF QUIN and progression of SSPE indicate a pathological role of KP metabolism in the inflammatory neurodestruction.

Using SpO2 Recovery Index after a 6-Minute Walk Test to Predict Respiratory-Related Events in Hospitalized Patients with Interstitial Pneumonia.

Although the prognostic factors of interstitial pneumonia (IP) patients have been reported, IP has poor prognosis. Hospitalized patients with IP have severely impaired pulmonary diffusion capacity and prominent desaturation. We hypothesized that determining oxygen saturation recovery (SpO2 recovery index) after the 6-minute walk test (6MWT) can provide additional prognostic information regarding rehospitalization for respiratory-related events. We evaluated 73 IP patients at our hospital for demographic characteristics, pulmonary function tests and 6MWT. The Kaplan-Meier method was used to estimate rehospitalisation for respiratory-related events using SpO2 recovery index. Cox regression analysis revealed a relationship between SpO2 recovery index and rehospitalisation. The optimum cutoff value of SpO2 recovery index was 4% (sensitivity, 71.4%; specificity, 79.2%). SpO2 recovery index was most closely related to pulmonary diffusion capacity (r = 0.684, P < 0.001). In a multivariable model, it was the strongest independent predictor of rehospitalisation for respiratory-related events (hazard ratio, 0.3; 95% confidence interval, 0.10-0.90; P = 0.032). In this study, we estimated pulmonary diffusion capacity using SpO2 recovery index values obtained from 6MWT. A SpO2 recovery index of <4% can be useful in predicting rehospitalisation for respiratory-related events.

Twenty-year Follow-up of the First Bilateral Living-donor Lobar Lung Transplantation in Japan.

Patients with end-stage lung disease can undergo living-donor lobar lung transplantation (LDLLT), with survival rates improving every year. We herein report the 20-year follow-up findings of the first patient who underwent LDLLT in Japan. A 24-year-old woman with primary ciliary dyskinesia became ventilator-dependent after severe respiratory failure and right-sided heart failure following repeated respiratory infections. In 1998, she underwent LDLLT and received her sister's right lower lobe and her mother's left lower lobe. Although the patient required 21 hospitalizations and developed unilateral bronchiolitis obliterans syndrome, she is in good physical condition and lives without restriction at 20 years after undergoing LDLLT.

A case of acute encephalophathy with residual neurological sequelae induced by immunoglobulin A vasculitis.

Immunoglobulin A vasculitis (IgAV) occasionally induces central nervous system (CNS) involvement, which is usually transient with no sequelae except for hemorrhagic stroke. It is thought to be useful to measure serum and cerebrospinal fluid (CSF) cytokine levels for better understanding the pathological condition in encephalopathy, but there have been no reports in acute encephalopathy with IgAV. We describe an 8-year-old boy with IgAV who had neurological sequelae after complication of acute encephalopathy, focusing on the cytokine profiles and unique biphasic findings of magnetic resonance imaging. He presented with status epilepticus and mildly intensified area in the occipital lobe on the fluid-attenuated inversion recovery view. Arterial spin labeling (ASL) revealed the reduction of cerebral blood flow in the left hemisphere. On day 5 of illness, these abnormal findings disappeared, but delayed hyperintensity lesions on diffusion-weighted images newly emerged. Furthermore, CSF interleukin (IL)-6 levels markedly increased without elevated levels of IL-10 during the acute phase of disease. He suffered from long-lasting hemiparesis and intellectual impairment. In conclusion, acute encephalopathy with IgAV could cause neurological sequelae by prolonged seizure, and elevated IL-6 in CSF and laterality of cerebral blood flow in ASL might be useful to predict the prognosis of CNS dysfunction of IgAV.

Virtual bronchoscopic navigation as an aid to CT-guided transbronchial biopsy improves the diagnostic yield for small peripheral pulmonary lesions.

BACKGROUND AND OBJECTIVE: Virtual bronchoscopic navigation (VBN) entails the provision of a virtual display of the bronchial routes that lead to small peripheral pulmonary lesions (PPL). It has been predicted that a combination of computed tomography (CT)-guided transbronchial biopsy (CT-TBB) with VBN might improve the diagnostic yield for small PPL. This study sought to investigate that prediction. METHODS: A total of 100 patients with small PPL (<20 mm) were enrolled for CT-TBB and randomly allocated to either a VBN+ or VBN- group (50 subjects per group). Group results were then compared in terms of diagnostic yield, whole procedure time, times at which the first CT scan and biopsy were taken and the number of lung biopsy specimens retrieved. RESULTS: The diagnostic yield for small PPL was significantly higher in the VBN+ group versus VBN- group (84% vs 58%, respectively (P = 0.013)), with no significant difference in (whole) examination time between groups (VBN+: 32:53 (32 min and 53 s) ± 12:01 vs VBN-: 33:06 ± 10:08 (P = NS)). However, the time periods between commencing the examination and either the first CT scan or first biopsy were significantly shorter for the VBN+ group, while the net biopsy time tended to be longer for this group with a significantly higher number of specimens collected (VBN+: 3.54 ± 1.07 specimens vs VBN-: 2.98 ± 1.06 specimens (P = 0.01)). CONCLUSION: Combining VBN with CT-TBB significantly improved the diagnostic yield for small PPL.

Signet Ring Cell Carcinoma of Unknown Primary Origin Detected Incidentally by Lymph Node Purification for Thyroid Carcinoma.

A 63-year-old woman underwent thyroidectomy for papillary thyroid adenocarcinoma and cervical lymph node resection. Pathological analyses revealed the presence of signet cell carcinoma in a resected lymph node, which were apparently different from the pathological findings of thyroid carcinoma. No evidence of a primary tumor could be found elsewhere despite detailed examinations, including esophagogastroduodenoscopy, colonoscopy, capsule endoscopy, CT scan, and fluorodeoxyglucose-positron emission tomography. Two and half years later, the patient developed multiple bone metastases and the pathological findings confirmed the presence of signet cell carcinoma. The primary origin remained undetermined. Metastatic signet ring cell carcinoma of unknown primary origin is extremely rare.