hepatobiliary disease
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2021 ◽  
Author(s):  
Wafaa Ahmed ◽  
Baljinder Sidhu ◽  
Devinder Bansi ◽  
Panagiotis Vlavianos ◽  
Christopher Wadsworth ◽  
...  

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S241-S241
Author(s):  
Hernando Salazar ◽  
Catherine Ye ◽  
Brian Schott ◽  
Kristin R Riddle ◽  
Diana Finkel

Abstract Background Endophthalmitis (EO) is an ocular emergency characterized by intraocular inflammation, usually in response to infection. While most cases are exogenous, gram negative (GN) EO account for 10-24% of all cases, and endogenous EO (EEO) account for 2-8% of all cases. Risk factors for EEO include diabetes mellitus (DM), IV drug use, and indwelling catheters. Major sources of infection are urinary tract infections (UTI), liver abscesses, pneumonia, and bacteremia. Common pathogens include K. pneumoniae, P. aeruginosa, and H. influenzae. Outcomes are poor, with only 20% of patients achieving improved visual acuity, and 30-40% requiring enucleation. Methods Retrospective analysis was performed on patients diagnosed with EO (n=89) at University Hospital in Newark from January 2016 to December 2020 using ICD-10 codes H44.0-H44.009, H44.1, and H44.19. Patients included were 18 years of age or older with culture proven GN endogenous EO (GNEEO) (n=7). Outcomes included anatomical success, functional success, and mortality at 28 days and 3 months. Results 7 of 89 patients met criteria for GNEEO (median age 67, 4 males, 71.4% Hispanic/Latino). Comorbidities included hepatobiliary disease (57.1%) and DM (42.9%). All 7 patients presented with ocular symptoms and 3 had non-ocular symptoms. Primary sources of infection included UTI, prostate abscess, and pneumonia/empyema. Eye cultures identified Pseudomonas in 4 patients and Klebsiella in 3 patients. Mean antibiotic length was 17.7 days with 6 patients receiving intravitreal antibiotics. Enucleation was performed in 3 patients. 2 patients had functional success and 4 had anatomical success, with 0 mortality at 28 days and 3 months. Table 1. Ocular symptoms on presentation of cases of gram negative endogenous endophthalmitis Table 2. Positives cultures obtained from cases of gram negative endogenous endophthalmitis Conclusion Although rare, GNEEO causes significant morbidity, with only 2 recovering visual acuity and 3 requiring enucleation. Risk factors, sources of infection, and microbes were all consistent with those in previous reports. Hepatobiliary disease and DM were the most prominent risk factors while sources of infection included UTI and empyema. Eye cultures were positive for K. pneumoniae and P. aeruginosa, two common pathogens previously identified. This case series highlights the importance of prompt recognition and initial treatment of GNEEO with empiric coverage that includes vancomycin and ceftazidime. Disclosures All Authors: No reported disclosures


2021 ◽  
pp. 134-154
Author(s):  
Carrie A. Schroeder

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Adele D’Amico ◽  
Antonella Longo ◽  
Fabiana Fattori ◽  
Michele Tosi ◽  
Luca Bosco ◽  
...  

Abstract Background X-linked myotubular myopathy (XLMTM) is a rare congenital myopathy resulting from pathogenic variants in the MTM1 gene. Affected male subjects typically present with severe hypotonia and respiratory distress at birth and they often require intensive supportive care. Long-term survivors are often non-ambulant, ventilator and feeding tube–dependent and they generally show additional organ manifestations, indicating that myotubularin does play a vital role in tissues other than muscle. For XLMTM several therapeutic strategies are under investigation. For XLMTM several therapeutic strategies are under investigation including a study of intravenous MTM1 gene transfer using a recombinant AAV8 vector of which has some concerns arises due to hepatotoxicity. Results We report prospective and retrospective clinical data of 12 XLMTM patients collected over a period of up to 10 years. In particular, we carried out a thorough review of the data about incidence and the course of hepatobiliary disease in our case series. Conclusions We demonstrate that hepatobiliary disease represents a common comorbidity of XLMTM that seems irrespective to age and diseases severity. We recommend to carefully explore and monitor the hepatobiliary function in XLMTM patients. We believe that a better understanding of the pathogenic mechanisms that induce hepatobiliary damage is essential to understand the fatal events that may occur in the gene therapy program.


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Jayan George ◽  
Alok Mathew ◽  
Edward Robinson ◽  
Edward Botsford ◽  
Rohan Ardley ◽  
...  

Abstract Aims 1. Understand the confidence levels in caring for general surgical patients of current foundation year one (FY1) doctors in who have had foundation interim year one (FiY1) post in a tertiary centre (TC) and a district general hospital (DGH). 2. Determine whether there is a difference in the experience of the FiY1 post in a TC or DGH. Methods FY1s doctors were surveyed throughout August – December 2020. Nine questions used Likert scales (1 to 5: not at all confident to very confident) over some common general surgical themes. Data was initially inputted using Google Docs and analysed in Microsoft Excel. Results 20% (6/30) from a DGH and 80% (24/30) from a TC Confidence levels were higher for FY1s who had a FiY1 post in a TC as opposed to a DGH in dealing with acute general surgical patients, appendicitis and scrubbing in theatre. Confidence levels were the same in both a TC and DGH for FY1s in managing those with hepatobiliary disease, prescribing fluids for resuscitation and maintenance as well as speaking to a family when a patient is dying and speaking to a patient when their diagnosis is unknown. Conclusions FY1s who had a FiY1 post in a TC had better confidence levels in more areas as opposed to a DGH. The experience of the FiY1 post should be more standardised to ensure the growth of confidence across all areas identified in the survey.


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Jayan George ◽  
Alok Mathew ◽  
Edward Robinson ◽  
Rohan Ardley

Abstract Aims 1. Understand the confidence levels of current foundation year one (FY1) doctors. 2. Understand whether an interim foundation year one (FiY1) post improves confidence. Methods FY1s were surveyed throughout August – December 2020. Nine questions used Likert scales (1 to 5: not at all confident to very confident) over some common general surgical themes. Data was initially inputted using google docs and analysed in Microsoft Excel. Results 32.1% (9/28) of FY1s completed the survey. 33.3% (3/9) did not undertake a FiY1 post where 66.7% (7/9) did. Confidence levels were mostly the same between FY1s who had a FiY1 post and those that did not for dealing with patients with hepatobiliary disease, non-specific abdominal pain, appendicitis, speaking to a family when a patient is dying prescribing fluids for maintenance as well as resuscitation. Confidence levels were higher for FY1s who did not have a FiY1 post compared to those that did in managing the deteriorating patient. Confidence levels were lower for FY1s who did not have a FiY1 post compared to those that did in speaking to a patient when their diagnosis is unknown. Conclusions FY1s who have had a FiY1 post in a district general hospital have more confidence in most areas but not all. This data shows the value of the FiY1 post however work needs to be done to improve the experience to the role.


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Jayan George ◽  
Alok Mathew ◽  
Edward Botsford ◽  
Ahmed Al-Mukhtar

Abstract Aims 1. Understand the confidence levels of current foundation year one (FY1) doctors. 2. Understand whether a foundation interim year one (FiY1) post improves confidence. Methods FY1s were surveyed throughout August – December 2020. Nine questions used Likert scales (1 to 5: not at all confident to very confident) over some common general surgical themes. Data was initially inputted using Google Docs and analysed in Microsoft Excel. Results 28.6% (30/105) of FY1s completed the survey. 20% (6/30) did not undertake a FiY1 post but 80% (24/30) did. Confidence levels were similar between FY1s who had a FiY1 post and those that did not for: dealing with acute general surgical patients, non-specific abdominal pain, appendicitis and prescribing fluids for maintenance as well as resuscitation. Confidence levels were higher for FY1s who did not have a FiY1 post compared to those that did in managing those with hepatobiliary disease. Confidence levels were lower for FY1s who did not have a FiY1 post compared to those that did in scrubbing for theatre, speaking to a family when a patient is dying and speaking to a patient when their diagnosis is unknown. Conclusions FY1s who have had a FiY1 post in a tertiary centre have more confidence in most areas but not all. This data shows the value of the FiY1 post however work needs to be done to improve the experience to the role.


2021 ◽  
Author(s):  
Adele D'Amico ◽  
Antonella Longo ◽  
Fabiana Fattori ◽  
Michele Tosi ◽  
Luca Bosco ◽  
...  

Abstract BACKGROUND: X-linked myotubular myopathy (XLMTM) is a rare congenital myopathy resulting from pathogenic variants in the MTM1 gene. Affected male subjects typically present with severe hypotonia and respiratory distress at birth and they often require intensive supportive care. Long-term survivors are often non-ambulant, ventilator and feeding tube–dependent and they generally show additional organ manifestations, indicating that myotubularin does play a vital role in tissues other than muscle. For XLMTM several therapeutic strategies are under investigation.A recent clinical trial delivering the MTM1 gene using a recombinant AAV8 vector to XLMTM patients showed encouraging results in term of efficacy, but some concerns about safety arised due to three fatal events related to hepatotoxicity in boys that were exposed to the higher dose.RESULTS: We report prospective and retrospective clinical data of 12 XLMTM patients collected over a period of up to 10 years. In particular, we carried out a thorough review of the data about incidence and the course of hepatobiliary disease in our case series.CONCLUSIONS: We demonstrate that hepatobiliary disease represents a common comorbidity of XLMTM that seems irrespective to age and diseases severity. We recommend to carefully explore and monitor the hepatobiliary function in XLMTM patients. We believe that a better understanding of the pathogenic mechanisms that induce hepatobiliary damage is essential to understand the fatal events that may occur in the gene therapy program.


Author(s):  
Y. Bandara ◽  
W. A. Bayton ◽  
T. L. Williams ◽  
T. Scase ◽  
N. H. Bexfield

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