A child with Winchester syndrome responding to oral betamethasone and methotrexate.

Winchester syndrome (WS) is a rare genetic disorder with a handful of cases reported to date. We report a 14-year-old male who presented with growth retardation, contracture of left lower limb due to thick indurated skin, hypertrichosis, and bilateral corneal opacity. There was complete improvement in joint contracture with oral betamethasone pulse and weekly oral methotrexate.

Parasitic larvae in bronchoalveolar lavage fluid in a non-immunocompromised patient.

Strongyloides stercoralis is responsible for a significant human parasitic infection known as strongyloidiasis. In addition, pulmonary strongyloidiasis is one of the most critical signs of disseminated strongyloidiasis. In this instance, S. stercoralis was unexpectedly discovered in bronchoalveolar lavage fluid.

Cytohistological correlation and risk stratification of salivary gland lesions using the Milan System for Reporting Salivary Gland Cytopathology: A tertiary care centre experience.

INTRODUCTION: Salivary gland neoplasms account for approximately 5% of head and neck tumours. The cytomorphology of fine needle aspiration material helps determine the preoperative assessment and risk stratification. The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) was developed to improve communication between the laboratory and treatment provider. AIMS AND OBJECTIVE: In the present study, we stratified all salivary gland lesions according to the MSRSGC and evaluated each category's concordance and risk of malignancy (ROM). MATERIALS AND METHODS: This was a 5 year retrospective study. First, all cases were assigned to one of the six MSRSGC categories. Then, following cytohistological correlation, the concordance rates and ROM were calculated based on the final histopathology report. RESULTS: A total of 354 cases were identified, with ages ranging from 2 to 88 years and the commonest age group was the 3rd to 4th decades. Categories I, II, III, IVA, IVB, V, and VI comprised 5.37% (19/354), 26.84% (95/354), 1.13% (04/354), 51.41% (182/354), 1.98% (07/354), 1.13% (04/354), and 12.15% (43/354) of the cases, respectively. The overall concordance rate between the cytological and histopathological diagnoses was 81.25% (65 out of 80 cases), with the sensitivity, specificity, positive predictive value, and negative predictive value at 43.48%, 96.49%, 83.33%, and 80.88%, respectively. CONCLUSION: The MSRSGC provides a better objective and structured way to communicate with the health care provider. In our study, the overall concordance rate was observed in 62/80 cases, with maximum concordance seen in categories III, IVB, and V of the MSRSGC.

Use of Pediatric Injectable Medicines Guidelines and Associated Medication Administration Errors: A Human Reliability Analysis.

BACKGROUND: In a recent human reliability analysis (HRA) of simulated pediatric resuscitations, ineffective retrieval of preparation and administration instructions from online injectable medicines guidelines was a key factor contributing to medication administration errors (MAEs). OBJECTIVE: The aim of the present study was to use a specific HRA to understand where intravenous medicines guidelines are vulnerable to misinterpretation, focusing on deviations from expected practice (discrepancies) that contributed to large-magnitude and/or clinically significant MAEs. METHODS: Video recordings from the original study were reanalyzed to identify discrepancies in the steps required to find and extract information from the NHS Injectable Medicines Guide (IMG) website. These data were combined with MAE data from the same original study. RESULTS: In total, 44 discrepancies during use of the IMG were observed across 180 medication administrations. Of these discrepancies, 21 (48%) were associated with an MAE, 16 of which (36% of 44 discrepancies) made a major contribution to that error. There were more discrepancies (31 in total, 70%) during the steps required to access the correct drug webpage than there were in the steps required to read this information (13 in total, 30%). Discrepancies when using injectable medicines guidelines made a major contribution to 6 (27%) of 22 clinically significant and 4 (15%) of 27 large-magnitude MAEs. CONCLUSION AND RELEVANCE: Discrepancies during the use of an online injectable medicines guideline were often associated with subsequent MAEs, including those with potentially significant consequences. This highlights the need to test the usability of guidelines before clinical use.

Association Between Treatments and Short-Term Biochemical Improvements and Clinical Outcomes in Post-Severe Acute Respiratory Syndrome Coronavirus-2 Inflammatory Syndrome.

OBJECTIVES: To 1) analyze the short-term biochemical improvements and clinical outcomes following treatment of children with post-severe acute respiratory syndrome coronavirus-2 inflammatory syndrome (multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2) admitted to U.K. PICUs and 2) collate current treatment guidance from U.K. PICUs. DESIGN: Multicenter observational study. SETTING: Twenty-one U.K. PICUs. PATIENTS: Children (< 18 yr) admitted to U.K. PICUs between April 1, 2020, and May 10, 2020, fulfilling the U.K. case definition of pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Routinely collected, deidentified data were analyzed. Propensity score and linear mixed effects models were used to analyze the effect of steroids, IV immunoglobulin, and biologic agents on changes in C-reactive protein, platelet counts, and lymphocyte counts over the course of PICU stay. Treatment recommendations from U.K. clinical guidelines were analyzed. Over the 6-week study period, 59 of 78 children (76%) received IV immunoglobulin, 57 of 78 (73%) steroids, and 18 of 78 (24%) a biologic agent. We found no evidence of a difference in response in clinical markers of inflammation between patients with multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 who were treated with IV immunoglobulin, steroids, or biologics, compared with those who were not. By the end of the study period, most patients had received immunomodulation. The 12 patients who did not receive any immunomodulators had similar decrease in inflammatory markers as those treated. Of the 14 guidelines analyzed, the use of IV immunoglobulin, steroids, and biologics was universally recommended. CONCLUSIONS: We were unable to identify any short-term benefit from any of the treatments, or treatment combinations, administered. Despite a lack of evidence, treatment guidelines for multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 have become very similar in advising step-wise treatments. Retaining clinical equipoise regarding treatment will allow clinicians to enroll children in robust clinical trials to determine the optimal treatment for this novel important condition.

Acute Kidney Injury in Pediatric Inflammatory Multisystem Syndrome Temporally Associated With Severe Acute Respiratory Syndrome Coronavirus-2 Pandemic: Experience From PICUs Across United Kingdom.

OBJECTIVES: To study the prevalence, evolution, and clinical factors associated with acute kidney injury in children admitted to PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. DESIGN: Multicenter observational study. SETTING: Fifteen PICUs across the United Kingdom. PATIENTS: Patients admitted to United Kingdom PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 between March 14, 2020, and May 20, 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Deidentified data collected as part of routine clinical care were analyzed. All children were diagnosed and staged for acute kidney injury based on the level of serum creatinine above the upper limit of reference interval values according to published guidance. Severe acute kidney injury was defined as stage 2/3 acute kidney injury. Uni- and multivariable analyses were performed to study the association between demographic data, clinical features, markers of inflammation and cardiac injury, and severe acute kidney injury. Over the study period, 116 patients with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 were admitted to 15 United Kingdom PICUs. Any-stage acute kidney injury occurred in 48 of 116 patients (41.4%) and severe acute kidney injury in 32 of 116 (27.6%) patients, which was mostly evident at admission (24/32, 75%). In univariable analysis, body mass index, hyperferritinemia, high C-reactive protein, Pediatric Index of Mortality 3 score, vasoactive medication, and invasive mechanical ventilation were associated with severe acute kidney injury. In multivariable logistic regression, hyperferritinemia was associated with severe acute kidney injury (compared with nonsevere acute kidney injury; adjusted odds ratio 1.04; 95% CI, 1.01-1.08; p = 0.04). Severe acute kidney injury was associated with longer PICU stay (median 5 days [interquartile range, 4-7 d] vs 3 days [interquartile range, 1.5-5 d]; p < 0.001) and increased duration of invasive mechanical ventilation (median 4 days [interquartile range, 2-6 d] vs 2 days [interquartile range, 1-3 d]; p = 0.04). CONCLUSIONS: Severe acute kidney injury occurred in just over a quarter of children admitted to United Kingdom PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. Hyperferritinemia was significantly associated with severe acute kidney injury. Severe acute kidney injury was associated with increased duration of stay and ventilation. Although short-term outcomes for acute kidney injury in pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 appear good, long-term outcomes are unknown.

Giant fibroepithelial polyp of the thigh and retroperitoneal fibromatosis in a young woman: a rare case.

We present a case of 20-year-old woman who presented with a large pedunculated skin covered mass lesion arising from the left thigh, measuring 40 × 25 cm, with no history of pain or skin ulceration and a feeling of a lump with dragging pain in the left side of the abdomen for about 7 years. Subsequently, ultrasound, contrast-enhanced computed tomography, and magnetic resonance imaging of abdomen and left thigh region were carried out. The lesion was broad-based toward the left upper thigh with a central core of interspersed fat supplied by branches of the superficial and deep femoral arteries. Another lesion was seen in the left retroperitoneum anterior to the psoas muscle in a left paravertebral location encasing the left common iliac vessels extending into the left pelvic cavity and inguinal region inferiorly. The lesion showed dense post-acoustic shadowing on ultrasound, mild enhancement on contrast-enhanced computed tomography, and appeared hypointense on T1- and T2-weighted images. A left thigh lesion was excised, whereas incisional biopsy was done for the left retroperitoneal lesion. The diagnosis of a giant fibroepithelial polyp arising from the left thigh and left retroperitoneal fibromatosis was made. This is the first report of such a giant fibroepithelial polyp arising from the thigh with associated retroperitoneal fibromatosis.

Bridging the gaps: A prospective analysis of root causes for rejection and incompleteness in blood requisition forms.

INTRODUCTION: Blood request form (BRF) stands as a pivotal document in ensuring safe and effective blood transfusions within healthcare settings. Incomplete or erroneous data on BRF can heighten risk of adverse reactions and compromise patient safety. Aim of study was to assess level of completion of BRFs by clinicians and to evaluate root cause analysis (RCA) of incompleteness of BRFs and factors leading to their rejection. MATERIALS AND METHODS: This prospective study was carried out from February 2024 to April 2024 on BRFs received in the blood centre. They were audited and RCA for factors leading to their incompleteness and rejection were analysed. RESULTS: Total number of BRFs received in blood centre was 14,468. 13,358 (92.3%) BRFs were accepted and 1,110 (7.7%) BRFs were rejected. 12,804 (95.85%) of accepted BRFs were incomplete. Weight was the most common missing parameter (89% {n = 11403}) while name of the requesting clinician was least common (2.5% {n-318}). 3.52% n = 510) BRFs were rejected due to mismatch in name and patient registration number on BRF and samples. 0.14% n = 21) BRFs were rejected due to hemolysed samples. RCA for incompleteness of BRFs showed that main reason was manpower (61-83%) while environment was least common (17-67%). RCA for rejection of BRFs showed that environment was most common cause (13.3-80.15%) while manpower was least common (9-19.85%). CONCLUSION: Regular audits and personnel training, and quality assurance measures can help identify and address deficiencies in BRF completion to enhance patient safety and reduce incidence of transfusion-related errors and complications.

Augmenting complex and dynamic performance through mindfulness-based cognitive training: An evaluation of training adherence, trait mindfulness, personality and resting-state EEG.

Human performance applications of mindfulness-based training have demonstrated its utility in enhancing cognitive functioning. Previous studies have illustrated how these interventions can improve performance on traditional cognitive tests, however, little investigation has explored the extent to which mindfulness-based training can optimise performance in more dynamic and complex contexts. Further, from a neuroscientific perspective, the underlying mechanisms responsible for performance enhancements remain largely undescribed. With this in mind, the following study aimed to investigate how a short-term mindfulness intervention (one week) augments performance on a dynamic and complex task (target motion analyst task; TMA) in young, healthy adults (n = 40, age range = 18-38). Linear mixed effect modelling revealed that increased adherence to the web-based mindfulness-based training regime (ranging from 0-21 sessions) was associated with improved performance in the second testing session of the TMA task, controlling for baseline performance. Analyses of resting-state electroencephalographic (EEG) metrics demonstrated no change across testing sessions. Investigations of additional individual factors demonstrated that enhancements associated with training adherence remained relatively consistent across varying levels of participants' resting-state EEG metrics, personality measures (i.e., trait mindfulness, neuroticism, conscientiousness), self-reported enjoyment and timing of intervention adherence. Our results thus indicate that mindfulness-based cognitive training leads to performance enhancements in distantly related tasks, irrespective of several individual differences. We also revealed nuances in the magnitude of cognitive enhancements contingent on the timing of adherence, regardless of total volume of training. Overall, our findings suggest that mindfulness-based training could be used in a myriad of settings to elicit transferable performance enhancements.

Primary Amelanotic Malignant Melanoma of the Tongue.

Background: Primary malignant melanoma rarely occurs in the oral cavity. The tongue is a particularly unusual primary site; lesions may be pigmented or amelanotic. Primary malignant melanoma is frequently mistaken for squamous cell carcinoma. Case Report: A 27-year-old male presented with a large, painless, ulceroproliferative mass on the dorsal surface of the tongue for 6 months. Squamous cell carcinoma was suspected, and the lesion was biopsied. Histopathology was compatible with primary amelanotic malignant melanoma. The patient had no cutaneous lesions consistent with malignant melanoma, and no definitive metastatic lesions were found. Ultrasound and computed tomography did not reveal any evidence of regional draining lymph node metastasis or suspicious lesions anywhere else in the body. The patient underwent composite resection of the tongue tumor and bilateral neck lymph node dissection, had an uneventful postoperative recovery, but was lost to follow-up. Conclusion: Primary oral amelanotic malignant melanoma is a highly aggressive, potentially fatal tumor and because of its rarity, presents a diagnostic challenge. The ideal treatment modality for primary malignant melanoma of the tongue is poorly defined, but surgery is regarded as the most effective course of therapy.

Evaluation of comorbidities in the SARS-CoV-2-related mortalities: A retrospective observation from a dedicated COVID-19 care hospital.

Since its discovery in Wuhan, China, in December 2019, the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has spread rapidly around the world with being declared a pandemic in March 2020 by the World Health Organization (WHO). Comorbidities are one of the most concerning clinical considerations for mortality. Materials and Methods: This was a retrospective observational study conducted at the All India Institute of Medical Sciences in Patna, Bihar, from June 2020 to December 2020. It included adult patients who succumbed to coronavirus disease 2019 (COVID-19) during this period, and their relevant information, such as demographic information hospital stay duration, haematological parameters and comorbidities, was gathered. Results: The mean age of the study group was 63.5 (11.8) years. Of 150 cases, 126 men (about 84 per cent) and 24 women were involved (16 per cent). In our study, hypertension (HTN) was shown to be the most frequent comorbidity at 68.7% (103/150), followed by diabetes mellitus (DM) at 61.3% (92/150). The most prevalent haematological disorder identified in our investigation was anaemia and leucocytosis. Conclusion: The categorisation of patients who would need extra measures including early hospitalisation, heightened monitoring and intense therapy would be made easier by identifying patient traits and conditions.

Clinical Management of Hospitalized Patients With High-Consequence Infectious Diseases in England.

Infectious disease physicians in England have been diagnosing and managing occasional cases of viral hemorrhagic fever since 1971, including the United Kingdom's first case of Ebola virus disease in 1976. Specialist isolation facilities to provide safe and effective care have been present since that time. Following the emergence of Middle East respiratory syndrome (MERS) in 2012, and the avian influenza A (H7N9) outbreak in 2013, and the 2014-2016 Ebola virus disease outbreak in West Africa, clinical and public health preparedness and response pathways in England have been strengthened for these types of diseases, now called high-consequence infectious diseases (HCIDs). The HCID program, led by NHS England and Public Health England between 2016 and 2018, helped to deliver these enhancements, which have since been used on multiple occasions for new UK cases and outbreaks of MERS, mpox, avian influenza, and Lassa fever. Additionally, HCID pathways were activated for COVID-19 during the first 3 months of 2020, before the pandemic had been declared and little was known about COVID-19 but HCID status had been assigned temporarily to COVID-19 as a precaution. The HCID program also led to the commissioning of a network of new airborne HCID treatment centers in England, to supplement the existing network of contact HCID treatment centers, which includes the United Kingdom's only 2 high-level isolation units. In this case study, the authors describe the airborne and contact HCID treatment center networks in England, including their formation and structures, their approach to safe and effective clinical management of patients with HCIDs in the United Kingdom, and challenges they may face going forward.

Hidradenoma papilliferum at vaginal vault - A rare cause of vault growth.

ABSTRACT: Hidradenoma papilliferum (HP) is a rare benign tumor of the apocrine gland and is commonly seen at the anogenital region. A 32-year-old post-hysterectomy woman presented with excessive vaginal discharge and itching. Small polypoidal mass was noted at vault. Excision of the lesion was done, and histopathology confirmed HP. The patient is symptom free following excision. This case report highlights the presence of pluripotent cells in the vagina and their differentiation into any cell lineage.

Gastric Xanthoma: A Rare Case Report.

Xanthomas are characterized by the presence of foamy lipid-laden macrophages. The gastrointestinal tract is an uncommon site for xanthoma, with the stomach being the most favored location. They have been associated with various premalignant and malignant conditions of the stomach. We present a case of a 21-year-old female patient with dyspepsia of 4 months duration. Her lipid profile was mildly altered. On upper gastrointestinal endoscopy, multiple discrete yellow patches were found in the antrum, diagnosed as gastric xanthoma on microscopy. Various published literature has emphasized the frequent association of gastric xanthomas with gastritis, gastric atrophy, intestinal metaplasia, and gastric cancer. Hence, there is a necessity for early recognition, treatment of any coexistent pathology, and close clinical follow-up.

Microfilaria in cervicovaginal Pap smears: A common parasite hiding in an unusual site: Short series of four cases with literature review.

In India, filariasis is a severe and significant public health problem. It has been reported to be present in any potential site. However, cervicovaginal Pap smear cytology rarely contains microfilariae despite the widespread occurrence. The occurrence of microfilariae in cervicovaginal smears has seldom been reported, even as an accidental finding. In our retrospective study, four cases of clinically asymptomatic filariasis were diagnosed on a routine cervicovaginal Pap smear from January 2019 to July 2022. All microfilariae were characterized as Wuchereria bancrofti. In the present study, the majority of the cases present vaginal discharge, and the diagnosis was made by cytopathology. Therefore, it is crucial to consider and look for microfilariae in areas where they are not endemic.

Significance of immune-inflammatory markers in predicting clinical outcome of COVID-19 patients.

Background: The epidemic of coronavirus disease 2019 (COVID-19) has been rapidly spreading on a global scale affecting many countries and territories. There is rapid onset of generalized inflammation resulting in acute respiratory distress syndrome. We, thus, aimed to explore the potential of immune-inflammatory parameters in predicting the severity of COVID-19. Materials and Methods: Age, neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR), platelet-to-lymphocyte ratio (PLR), Lactate Dehydrogenase (LDH), C-reaction protein (CRP), and procalcitonin (PCT) of 611 patients with laboratory-confirmed COVID-19 were investigated and compared. Patients were divided on the basis of severity and survival into two groups. Data were expressed as mean or median values and percentages. The receiver operating characteristic curve was applied to determine the optimal cut-off values of these biomarkers. Results: The median age was 50 years and the male to female ratio was 3.7:1. The mean NLR, LMR, PLR, LDH, CRP, and Procalcitonin for the non-severe group were 4.16, 10.8, 133.7, 666.1, 49.9, and 0.15, respectively. In the severe group mean values of the above-mentioned immune-inflammatory markers were 17.8, 4.69, 268.2, 1277, 158.6, and 3.05, respectively. Elevated levels were significantly associated with disease severity. In ROC curve analysis, NLR had the largest area under the curve at 0.923 with the highest specificity (0.83) and sensitivity (0.88). Conclusion: This study shows that NLR, PLR, LDH, CRP, and Procalcitonin may be a rapid, widely available, useful predictive factor for determining the severity of COVID-19 patients.

Neural and cognitive correlates of performance in dynamic multi-modal settings.

The endeavour to understand human cognition has largely relied upon investigation of task-related brain activity. However, resting-state brain activity can also offer insights into individual information processing and performance capabilities. Previous research has identified electroencephalographic resting-state characteristics (most prominently: the individual alpha frequency; IAF) that predict cognitive function. However, it has largely overlooked a second component of electrophysiological signals: aperiodic 1/ƒ activity. The current study examined how both oscillatory and aperiodic resting-state EEG measures, alongside traditional cognitive tests, can predict performance in a dynamic and complex, semi-naturalistic cognitive task. Participants' resting-state EEG was recorded prior to engaging in a Target Motion Analysis (TMA) task in a simulated submarine control room environment (CRUSE), which required participants to integrate dynamically changing information over time. We demonstrated that the relationship between IAF and cognitive performance extends from simple cognitive tasks (e.g., digit span) to complex, dynamic measures of information processing. Further, our results showed that individual 1/ƒ parameters (slope and intercept) differentially predicted performance across practice and testing sessions, whereby flatter slopes and higher intercepts were associated with improved performance during learning. In addition to the EEG predictors, we demonstrate a link between cognitive skills most closely related to the TMA task (i.e., spatial imagery) and subsequent performance. Overall, the current study highlights (1) how resting-state metrics - both oscillatory and aperiodic - have the potential to index higher-order cognitive capacity, while (2) emphasising the importance of examining these electrophysiological components within more dynamic settings and over time.