Definitions, Etiologies, and Outcomes of Acute on Chronic Liver Failure: A Systematic Review and Meta-analysis.

BACKGROUND & AIMS: Acute-on-chronic liver failure (ACLF) is a major public health concern. We aimed to assess the definitions, etiologic spectrum, organ failure (OF), and outcomes of ACLF globally. METHODS: Three databases were searched for studies on ACLF from 1990 until September 2022. Information regarding definitions, acute precipitants, underlying chronic liver disease (CLD), OF, and mortality were extracted. Meta-analyses were performed for pooled prevalence rates (95% confidence interval [CI]) using random-effects model for each definition of ACLF. RESULTS: Of the 11,451 studies identified, 114 articles (142 cohorts encompassing 210,239 patients) met the eligibility criteria. Most studies (53.2%) used the European Association for the Study of the Liver (EASL) definition, followed by Asia-Pacific Association for the Study of the Liver (APASL) (33.3%). Systemic infection was the major acute precipitant, and alcohol use was the major cause of CLD in EASL-defined studies, whereas alcohol was both the major acute precipitant and cause of CLD in APASL-defined studies. Liver failure was the major OF in APASL-based studies, whereas renal failure was predominant in EASL-based studies. Thirty-day mortality varied across definitions: APASL: 38.9%, 95% CI, 31.2%-46.9%; EASL: 47.9%, 95% CI, 42.2%-53.5%; and NACSELD: 52.2%, 95% CI, 51.9%-52.5%. Diagnostic overlap between definitions ranged from 7.7% to 80.2%. Meta-regression suggested that the World Health Organization region influenced 30-day mortality in studies using EASL definition. CONCLUSIONS: Heterogeneity in the definition of ACLF proposed by different expert societies and regional preferences in its use result in differences in clinical phenotype and outcomes. A uniform definition would enhance the comparability and interpretation of global data.

Thromboelastography-guided vs. standard-of-care or on-demand platelet transfusion in patients with cirrhosis and thrombocytopenia undergoing procedures: A randomized controlled trial.

PURPOSE: To compare the efficacy and safety of a thromboelastography (TEG)-guided platelet transfusion strategy to empirical or on-demand transfusions in patients with cirrhosis and severe thrombocytopenia (platelet counts <50 x109/L) undergoing high-risk invasive procedures. MATERIALS AND METHODS: This was a single-center, single-blinded, randomized controlled trial. Patients with cirrhosis and severe thrombocytopenia undergoing high-risk invasive procedures were randomized into three groups- TEG group: transfusions based on TEG parameters; SOC group: 3 units of random donor platelets pre-procedure; On-demand group: transfusions based on procedural adverse effects /clinician's discretion. The primary outcome was periprocedural platelet transfusion in each arm. RESULTS: Eighty-seven patients were randomized (29 in each group) with no significant differences in demographics/coagulation profile/procedures. The median platelet count was 33 x109/L (IQR: 26-43). Percutaneous liver biopsy was the most common procedure (46, 52.9%). Significantly lower number of patients in the TEG group received platelets (4 cases, 13.8%; 95%CI: 3.9-31.7) compared to SOC (100%; 95%CI: 88.1-100) (p<0.001). Four patients in the on-demand group received platelets (13.8%; 95%CI: 3.9-31.7). Minor (WHO grade 2) procedure-related bleeding occurred in 3 (10%; 95%CI: 2.2-27.4) patients in the TEG-guided transfusion group, compared to 1 (3.4%; 95%CI: 0.1-17.8) each in SOC and on-demand groups, respectively (p=0.43) although our sample size was underpowered for comparison of outcomes such as post-procedural bleeding. No bleeding-related mortality was observed in any of the three groups. CONCLUSION: Thromboelastography-guided transfusion reduces prophylactic transfusions in patients with cirrhosis and severe thrombocytopenia undergoing high-risk invasive procedures. (CTRI/2021/05/033464).

Acute bilateral sensorineural hearing loss as presentation of leptomeningeal metastases.

We describe a rare occurrence of bilateral acute severe sensorineural hearing loss in a middle-aged man that heralded the diagnosis of metastatic gastric cancer.

Knowledge and Skills in Cardiopulmonary Resuscitation and Effect of Simulation Training on it among Healthcare Workers in a Tertiary Care Center in India.

Aim and background: High-quality cardiopulmonary resuscitation (CPR) is associated with improved patient outcomes, but healthcare workers (HCWs) may be frequently undertrained. This study aimed to assess baseline knowledge and skills among HCWs about basic and advanced life support and the effect of simulation-based training on it. Methods: It was a single-center prospective quasi-interventional study among resident doctors and nurses at a Tertiary Center in New Delhi, India. A questionnaire-based assessment was done to assess baseline knowledge. The participants then underwent simulation-based training followed by questionnaire-based knowledge assessment and skill assessment. A repeat questionnaire-based assessment was done 6 months post-training to assess knowledge retention. Results: A total of 82 HCWs (54 doctors and 28 nurses) were enrolled. The participants scored 22.28 ± 6.06 out of 35 (63.65%) in the pre-training knowledge assessment, with low scores in post-cardiac arrest care, advanced life support, and defibrillation. After the training, there was a significant rise in scores to 28.32 ± 4.08 out of 35 (80.9%) (p < 0.01). The retention of knowledge at 6 months was 68.87% (p < 0.01). The participants scored 92.61 ± 4.75% marks in skill assessment with lower scores in chest compressions and team leadership roles. There was a positive correlation (r = 0.35) between knowledge and skills scores (p < 0.01). Conclusion: There is a progressive decrease in baseline knowledge of HCWs with the further steps in the adult chain of survival. The simulation training program had a positive impact on the knowledge of HCWs. The training programs should focus on defibrillation, advanced life support, post-cardiac arrest care, and leadership roles. How to cite this article: Agarwal A, Baitha U, Ranjan P, Swarnkar NK, Singh GP, Baidya DK, et al. Knowledge and Skills in Cardiopulmonary Resuscitation and Effect of Simulation Training on it among Healthcare Workers in a Tertiary Care Center in India. Indian J Crit Care Med 2024;28(4):336-342.

Clinical and radiological spectrum of anti-myelin oligodendrocyte glycoprotein (MOG) antibody encephalitis: single-center observational study.

OBJECTIVE: The objective was to describe the clinical presentations, radiologic features, and outcomes of patients with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibody (MOG). BACKGROUND: During the past decade, the spectrum of the myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD) has expanded. Recently, patients with MOG antibody encephalitis (MOG-E) who do not fulfill the criteria for ADEM have been reported. In this study, we aimed to describe the spectrum of MOG-E. METHODS: Sixty-four patients with MOGAD were screened for encephalitis-like presentation. We collected the clinical, radiological, laboratory, and outcome data of the patients who presented with encephalitis and compared it with the non-encephalitis group. RESULTS: We identified sixteen patients (nine males and seven females) with MOG-E. The median age of the encephalitis population was significantly lower than the non-encephalitis group (14.5 years (11.75-18) vs. 28 years (19.75-42), p = 0.0004). Twelve out of sixteen patients (75%) had fever at the time of encephalitis. Headache and seizure were present in 9/16 (56.2%) and 7/16 (43.75%) patients, respectively. FLAIR cortical hyperintensity was present in 10/16 (62.5%) patients. Supratentorial deep gray nuclei were involved in 10/16 (62.5%) patients. Three patients had tumefactive demyelination, and one patient had a leukodystrophy-like lesion. Twelve of 16 (75%) patients had a good clinical outcome. Patient with leukodystrophy pattern and other with generalized CNS atrophy showed a chronic progressive course. CONCLUSION: MOG-E can have heterogeneous radiological presentations. FLAIR cortical hyperintensity, tumefactive demyelination, and leukodystrophy-like presentations are novel radiological presentations associated with MOGAD. Though majority of MOG-E have a good clinical outcome, few patients can have chronic progressive disease even on immunosuppressive therapy.

Prevalence and determinants of post-stroke sleep disorders: a cross-sectional hospital-based study.

INTRODUCTION: Post-stroke sleep disorders (PSSD) are an important part of post-stroke disability. PSSD is neglected as a part of stroke rehabilitation. We aimed to study the prevalence and determinants of PSSD in a hospital based, single center setting. METHODS: In a cross-sectional study, adult patients (≥ 18 years) with stroke (one month to one year after the onset), were enrolled in the study. Demographic, clinical, radiological, and motor and functional disabilities were assessed. Sleep quality was assessed with Pittsburg Sleep Quality Index (PSQI) and STOP BANG questionnaire (for obstructive sleep apnea [OSA]). Patients with poor sleep quality (PSQI > 5) were analyzed for risk factors. RESULTS: A total of 103 patients were recruited in the study period (January 2021 to June 2022). The self-reported prevalence of PSSD was 16% which increased to 72% when the PSQI was administered. High risk of OSA was present in 33%. In bivariate analysis, factors associated with PSQI > 5 were involvement of ≥ 2 lobes, lower body mass index (BMI), worse modified Rankin Scale (mRS), Hamilton Depression Rating Scale (HAM-D), Hamilton Anxiety Rating Scale (HAM-A) and Stroke Specific Quality of Life (SSQoL). In multivariate analysis, only depression was associated with PSQI > 5 (OR: 1.3 (1.0; 1.7); p-value = 0.03). CONCLUSION: PSSD had a prevalence of 72%. In multivariate analysis, the factor associated with PSQI > 5 was worse HAM-D score.

Brain imaging inspired by outer space.

Medicine has many vividly named signs. We have compiled a list of radiological cerebral signs inspired by phenomena in outer space. These range from the well-known 'starry sky' appearance of neurocysticercosis or tuberculomas, to various lesser known signs including the 'starfield' pattern of fat embolism; 'sunburst' sign of meningiomas; 'eclipse' sign of neurosarcoidosis; 'comet tail' sign of cerebral metastases; 'Milky Way' sign of progressive multifocal leukoencephalopathy; 'satellite' and 'black hole' sign of intracranial haemorrhage; 'crescent' sign of arterial dissection and 'crescent moon' sign of Hirayama disease.

Publication of neurology clinical trials registered with clinical trial registry of India: A cross-sectional study.

OBJECTIVES: Unbiased and full disclosure of trial results is vital to evidence-based medicine. Non-publication and selective publication leads to publication bias and unrealistic risk-benefit ratio. In the present study, we aim to determine the publication rate of clinical trials related to neurology registered with the Clinical Trial Registry of India (CTRI), compare the characteristics of published and unpublished trials, and evaluate the adherence of investigators to ethics-approved criteria and outcomes. MATERIALS AND METHODS: A cross-sectional search using the keyword "neurology" was carried out in CTRI registry. Two independent investigators searched Pubmed, Medline, Scopus, and Google Scholar for published manuscripts. The final literature search occurred in November 2021. RESULTS: Out of 325 trials, 102 trials were published (31.4%). Ninety-one trials were beyond 3 years of expected time of trial completion and were still unpublished. Randomized trials had a slightly higher publication rate than non-randomized ones (56% vs. 46%, p = .223); however the difference was not statistically significant. Majority of trials sponsored by pharmaceutical companies were not published, while majority of those sponsored by non-pharmaceutical institutions were published (34.5% vs. 69.3%, p < .001). Feedback to CTRI about trial status was particularly poor (31.5% - informed vs. 68.5% - not informed, p < .001). 52 (50.9%) and 65 (63.7%) of the 102 published trials had changed the registered inclusion and exclusion criteria, respectively, in the CTRI registry compared to those in the published manuscript. In 29 (28.3%) of the 102 trials, the primary outcome did not match with that registered in the CTRI and in 73 (57.8%) trials, the secondary outcomes did not match. CONCLUSION: A large proportion of neurology registered trials are still unpublished, with a majority of pharmaceutical company-sponsored trials not being published. There is scope for improving the provisions in CTRI for enlisting trial results, that may prevent publication bias and also ensure the investigators adhere to the pre-specified ethics approved trial procedures and outcomes.

Classification and comparative analysis of psychogenic nonepileptic seizures (PNES) semiology based on video-electroencephalography (VEEG).

BACKGROUND: Multiple classification systems for psychogenic nonepileptic seizures (PNES) based on semiological features have been described. We sought to compare the efficiency of four PNES classification systems. METHODS: We retrospectively analyzed medical and video-electroencephalography (VEEG) records of patients with PNES with at least one typical event recorded on VEEG. Semiology of PNES events was stringently classified using Hubsch, Dhiman, Wadwekar, and Asadi-Pooya's classification systems. RESULTS: We studied 248 patients with PNES (78% females, mean age 23.1 ±â€¯10.3 years) and reviewed 498 PNES events. Using Hubsch's scheme, we classified events into: dystonic attacks with primitive gestural activity (5.2%), paucikinetic attacks with preserved responsiveness (9.7%), pseudosyncope (59.8%), hyperkinetic prolonged attacks (16.2%) and axial dystonic prolonged attacks (1.6%), and unclassified (7.5%). Using Dhiman's classification, events were: abnormal motor (hypermotor [10.4%]/ partial motor [12.7%]), dialeptic type (58.6%), mixed patterns (17.3%), and unclassified (1%). Using Wadwekar's classification: dystonic attacks with primitive gestural activity (5.2%), paucikinetic attacks with preserved responsiveness (9.6%), pseudosyncope with/without hyperventilation (65.1%), hyperkinetic prolonged attacks involving limbs ±â€¯trunk (18.5%), and axial dystonic prolonged attacks (1.6%). Using Asadi-Pooya's classification, events were: hypermotor (30.1%), non-motor (62.9%), and mixed (7.0%). All events could be classified via Wadwekar and Asadi-Pooya systems. CONCLUSION: In our study, pseudosyncope/dialeptic/non-motor semiology emerged as most frequent. Most of our patients with PNES had stereotyped semiology. All events could be classified using the schemes by Asadi-Pooya and Wadweker et al. Dhiman et al. scheme could classify 99% and 7.5% remained unclassified using Hubsch et al. scheme.

Guillain-Barre syndrome complicating chikungunya virus infection.

Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease. While outbreaks have been earlier reported from India and other parts of the world, the recent outbreak in India witnessed more than 1000 cases. Various systemic and rarely neurological complications have been reported with CHIKV. We report two cases of Guillain-Barré syndrome (GBS) with CHIKV. GBS is a rare neurological complication which may occur after subsidence of fever and constitutional symptoms by several neurotropic viruses. We describe two cases of severe GBS which presented with rapidly progressive flaccid quadriparesis progressing to difficulty in swallowing and breathing. Both required mechanical ventilation and improved partly with plasmapharesis. The cases emphasize on (1) description of the rare complication in a setting of outbreak with CHIKV, (2) acute axonal as well as demyelinating neuropathy may occur with CHIKV, (3) accurate identification of this entity during outbreaks with dengue, both of which are vector borne and may present with similar complications.

One-Year Outcomes of Long Coronary Drug-Eluting Stents (≥40 MM) in Patients With Diffuse Coronary Artery Disease: Findings From a Tertiary Care Hospital in North India.

Background and objective Diffuse coronary artery disease (CAD) is associated with extensive involvement of coronary arteries, necessitating the use of long (≥40 mm) drug-eluting stents (DES) based on the lesion length. However, these long DES can lead to complications such as in-stent restenosis (ISR) and stent thrombosis. This study aimed to assess the safety, efficacy, and one-year clinical outcomes of using long DES in patients with diffuse CAD undergoing PCI at a tertiary care hospital in north India. Methodology Patients with diffuse CAD undergoing PCI with long DES between January 2017 and June 2022 were included in the study. Baseline characteristics were recorded, and patients were followed up telephonically or in the outpatient department (OPD) at one, three, six, and 12 months following the PCI. The primary endpoint was the target lesion failure (TLF) rate, with secondary endpoints constituting all-cause mortality, major adverse cardiovascular events (MACE), subacute stent thrombosis, and ISR. Results A total of 200 patients were recruited and followed up for one year. The median age of the patients was 58 years (range: 48.25-63 years), and 82% were men. The most frequently stented artery was the left anterior descending (LAD, 48%), followed by the right coronary artery (RCA, 36%). A total of 388 stents (mean: 1.94 ±0.79) were implanted, including both long and short stents. The mean length and diameter of long stents were 43.64 ±3.58 mm and 3 ±0.37 mm, respectively. At the one-year follow-up, patients undergoing PCI with long DES ≥40 mm had an overall TLF rate of 5%, all-cause mortality of 6% (12 patients), MACE of 6% (12 patients), subacute stent thrombosis of 4% (eight patients), and ISR of 1% (two patients). A large proportion of patients (90%) had an uneventful follow-up of up to a year. At the one-year follow-up, all 10 (5%) patients with a primary outcome had a smaller stent diameter than those without a primary outcome (2.5 ±0.25 mm vs. 3.03 ±0.35 mm, p=0.015). Conclusions Our results suggest that using extremely long stents (>40 mm) for diffuse coronary lesions is safe, efficacious, and associated with relatively low event rates. In addition, the stent diameter has a substantial correlation with the primary outcome. Further studies with larger sample sizes as well as longer follow-up periods are required to validate our findings.

YAP1∷KMT2A fusion-positive sarcoma: An emerging soft tissue tumor entity with morphological features resembling a sclerosing epithelioid fibrosarcoma.

ABSTRACT: Sclerosing epithelioid fibrosarcoma (SEF) and low-grade fibromyxoid sarcoma (LGFMS) constitute a morphological continuum with certain overlapping histopathological features and MUC 4 immunopositivity. SEF is characterized by EWSR1 gene rearrangement, is relatively more aggressive and shows a limited response to chemotherapy. Lately, a subset of tumors with morphological features of SEF, but lacking MUC4 immunostaining and EWSR1 gene rearrangement have been observed. We report the first case of YAP1∷KMT2A-positive sarcoma from the Indian subcontinent along with a review of similar tumors reported previously. A case of 31-year-old male presented with a 3 cm painful lump in the right suboccipital region. Histopathological examination of the excised specimen revealed a cellular tumor composed of atypical spindle and epithelioid cells, exhibiting moderate nuclear pleomorphism, arranged in cords, embedded in a dense collagenous/hyalinized stroma. By immunohistochemistry, the tumor cells were diffusely positive for cyclin D1 and negative for MUC4, desmin, myogenin, ß-catenin, STAT6, myoD1, SMA, and S100P. By fluorescence in-situ hybridization, EWSR1 gene rearrangement was negative. Next-generation sequencing (NGS) revealed YAP1exon5∷KMT2Aexon4 fusion. Given a positive resection margin, he underwent a revision surgery involving wide local excision of the lesion including the outer table of the occipital bone followed by image-guided radiation therapy. Over 2 years of his follow-up, the patient is alive with no evidence of recurrence. Thus, YAP1∷KMT2A positive sarcomas have distinct molecular and overlapping histopathological features with SEF, with relatively less aggressive disease course. Documentation of additional similar tumors with long-term follow-up is required.

The Pitfalls of NIHSS: Time for a New Clinical Acute Stroke Severity Scoring System in the Emergency?

Stroke is the second leading cause of death and a major cause of disability worldwide. Stroke severity scales serve as reliable means to track a patient's neurological deficit, predict outcome, and guide treatment decisions in clinical practice. The National Institute of Health Stroke Scale (NIHSS) was introduced over 30 years ago, marking a significant milestone in the field of stroke. Over the years, there have been notable advancements in acute stroke care. Despite several modifications made to NIHSS, none has yet succeeded in effectively capturing all the complex effects of a stroke. This review focuses on the pitfalls of NIHSS and emphasizes the need for a quick and comprehensive clinical and upgraded version of the stroke severity rating scale.