Retrospective Review Study Research Articles

Factors associated with patency loss and actuarial patency rate following post-cholecystectomy bile duct injury repair: long-term follow-up.

Several factors may contribute to bile duct injury (BDI) repair failure. The objective of our study was to evaluate factors that contribute to the loss of patency and influence the actuarial patency rate of BDI repairs in an 11-year period. Retrospective review study of patients who underwent a hepaticojejunostomy for a type E Bismuth-Strasberg BDI (2008-2019). The outcomes are the following: primary patency attained, loss of primary patency, and actuarial primary patency rate. Logistic regression for loss of patency and Cox regression for actuarial patency rate were used. Seventy-nine patients (age 42.3 ± 15.8years, 81% female) were studied. Most common index operation was open cholecystectomy (60.8%). Most common Bismuth-Strasberg lesion was E4 (38%). Primary patency was 93.4%. Mean follow-up was 36 ± 34months. Ten-year actuarial patency was 53.9%. Factors associated with loss of patency were vasculobiliary injury, biliary stents, and 90-day biliary complications (univariate); number of surgeries before repair and postoperative cholangitis (univariate and multivariate) (p < 0.05). Factors that impacted actuarial patency rate were (univariate analysis) 90-day biliary complications; postoperative cholangitis and index treatment period stenosis (p < 0.05). No factors impacted actuarial patency rate in multivariate analysis. Postoperative cholangitis is associated with loss of patency and had potentially detrimental effect on the actuarial patency rate in BDI repair.

Added Value of Digital over Analog PET/CT: More Significant as Image Field of View and Body Mass Index Increase.

The digital PET/CT scanner with digital photon-counting technique promises a shorter scan time, improved small-lesion detectability, and reduced radiation dose for the PET and CT portions of the exam while improving image quality. Methods: In this single-institution retrospective review study, 84 participants who had undergone PET/CT on both analog and digital scanners were analyzed. The aim was to evaluate the impact of image field of view (FOV) and body mass index (BMI) on the digital compared with the analog PET/CT scanners. The participants were categorized into different groups based on their BMI. Total scan times, 18F-FDG doses, and dose-length products (DLP) were collected and compared. Image quality was also assessed by certified nuclear medicine physicians and graded on a scale from 1 to 5. Results: In the skull-to-mid-thigh FOVs, the digital scanner had a scan time shorter by 37% (P < 0.001), a 18F-FDG dose lower by 16% (P < 0.001), but only an 8% reduction in DLP (P = 0.2). In the head-to-toe FOV cases, the digital scanner showed reductions in scan time (33%; P < 0.001), 18F-FDG dose (13%; P < 0.001), and DLP (19%; P < 0.001). When BMI was accounted for, the digital scanner had a scan time shorter by 33% (P < 0.001), as well as a reduced DLP (P < 0.001) and 18F-FDG dose (P < 0.001), with the most prominent changes being in the overweight and obese participants. Image quality was also improved by the digital scanner, with a score of 4.5, versus 4.0 for the analog scanner. Conclusion: The digital scanner has a shorter scan time and lower DLP, requires a lower 18F-FDG dose, and provides improved image quality when compared with the analog scanner. The most impactful difference in scan time, DLP, and 18F-FDG dose were observed in obese and overweight participants.

Nature of adverse events with opioids in hospitalised patients: a post-hoc analysis of three patient record review studies.

ObjectiveOpioids are increasingly prescribed and frequently involved in adverse drug events (ADEs). The underlying nature of opioid-related ADEs (ORADEs) is however understudied. This hampers our understanding of risks related to opioid use during hospitalisation and when designing interventions. Therefore, we provided a description of the nature of ORADEs.DesignA post-hoc analysis of data collected during three retrospective patient record review studies (in 2008, 2011/2012 and 2015/2016).SettingThe three record review studies were conducted in 32 Dutch hospitals.ParticipantsA total of 10 917 patient records were assessed by trained nurses and physicians.Outcome measuresPer identified ORADE, we described preventability, type of medication error, attributable factors and type of opioids involved. Moreover, the characteristics of preventable and non-preventable ORADEs were compared to identify risk factors.ResultsOut of 10 917 patient records, 357 ADEs were identified, of which 28 (8%) involved opioids. Eleven ORADEs were assessed as preventable. Of these, 10 were caused by dosing errors and 4 probably contributed to patients’ death. Attributable factors identified were mainly on patient and organisational levels. Morphine and oxycodone were the most frequently involved opioids. The risk for ORADEs was higher in elderly patients.ConclusionsOnly 8% of ADEs identified in our sample were related to opioids. Although the frequency is low, the risk of serious consequences is high. We recommend to use our findings to increase awareness among physicians and nurses. Future interventions should focus on safe dosing of opioids when prescribing and administering, especially in elderly patients.

Evaluation of Secondary Post-Tonsillectomy Bleeding among Children in Saudi Arabia: Risk Factor Analysis.

Tonsillectomy is one of the most widely recognized surgeries for tonsillitis. It is the default surgery option for obstructive sleep apnea, recurrent tonsillitis, and recurrent peritonsillar abscess. However, there are limited risk factor analyses in Saudi Arabia related to tonsillectomy. The goal of study is to evaluate and analyze the risk factors for secondary post-tonsillectomy bleeding in Saudi Arabia. This retrospective review study was directed at King Abdullah Specialized Children's Hospital, Riyadh, Saudi Arabia. Univariate and multivariate investigations were performed to decide the risk factors. A total of 713 children were included. Post-tonsillectomy bleeding occurred in only 5.3% of tonsillectomies. There was no seasonal variation impact on post-tonsillectomy bleeding rate (P = .8). The multivariate analysis showed a significant association between post-tonsillectomy bleeding and both age (odds ratio [OR] = 1.156; 95% CI: 1.007-1.326; P = .039) and sleep disorder breathing/obstructive sleep apnea (OR = 3.581; 95% CI: 1.454-8.820; P = .006). This study revealed that age, sleep disorder/obstructive sleep apnea, and longer hospital stay after tonsillectomy are significant risk factors for post-tonsillectomy bleeding. This study provides an important baseline for further local studies in the future.

Facility-Level Case Report of Nursing Care Processes for Patients With Suspected 2019 Novel Coronavirus Disease in Shanghai, China

IntroductionCoronavirus disease emerged in Wuhan, China, on December 31, 2019, and spread rapidly worldwide. Few studies have described the nursing care provided to patients in isolation between suspicion of having the disease and a confirmed diagnosis. The purpose of this study was to describe the treatment of, and nursing care processes for, patients suspected, but not yet confirmed, of having coronavirus disease at 1 facility in Shanghai, China. MethodsFor this retrospective facility case review and patient health record study, data were collected on all patients with suspected coronavirus disease who were treated between January 22, 2020, and February 29, 2020, at 1 hospital. The facility’s nursing care processes were described in detail. ResultsA total of 119 patients were suspected of having coronavirus disease on the basis of the screening criteria. Nine (7.6%) patients had confirmed coronavirus disease and were transferred to a higher level of care. The remaining 110 (92.4%) were treated and discharged. No cross-infection between patients and hospital staff or other patients was detected. The patients’ symptoms included fever (n = 98, 82.4%), cough (n = 79, 66.4%), dizziness (n = 28, 23.5%), headache (n = 26, 21.8%), fatigue (n = 26, 21.8%), myalgia (n = 16, 13.4%), rhinorrhea (n = 6, 5.0%), diarrhea (n = 5, 4.2%), severe nasal congestion (n = 4, 3.4%), and dyspnea (n = 1, 0.8%). DiscussionCoronavirus disease is very contagious. Nurses need to understand the symptoms and treatment of the disease as well as nursing procedures, and learn how to cut off transmission routes, control transmission sources, and use protective equipment correctly to prevent transmission of the disease within the hospital.

Single center experience from Egypt about fulminant hepatic failure in children

Aim: To assess fulminant hepatic failure (FHF) in children and its interrelating factors. Methods and materials: A retrospective review study of 24 patients less than 17 years old who presented to the National Liver Institute with FHF over a period of two years was done. FHF was defined as the presence of acute liver failure with or without encephalopathy without pre-existing liver disease, within 8 weeks of the onset of clinical liver disease. Hepatitis A virus (HAV) IgM antibodies, Hepatitis B virus surface antigen (HBsAg), IgM-anti Hepatitis B core antigen (HBc), Hepatitis E virus (HEV) IgM and Hepatitis C virus (HCV) RNA in nested PCR were done in all patients. Special investigations like alpha fetoprotein, serum copper, blood culture, drug levels and other metabolic studies were carried out whenever indicated. Detailed clinical evaluations and routine hepatic laboratory profile were done. Results: Acute hepatitis-A virus was the commonest cause. Severe coma was significantly present among non-survivals and no ascites. Patients who survived had got significantly lower level of prothrombin time, they also had significantly lower rate of descent of prothrombin time. Conclusion: Hepatitis A virus was the commonest etiology of FHF. The peak level of total serum bilirubin, the rate of change of the prothrombin time/day and ammonia level were significant predictors of mortality.

Absolute lymphocyte count is a prognostic marker in Covid-19: A retrospective cohort review.

IntroductionPrognostic factors are needed to aid clinicians in managing Covid‐19, a respiratory illness. Lymphocytopenia has emerged as a simply obtained laboratory value that may correlate with prognosis.MethodsIn this article, we perform a retrospective cohort review study on patients admitted to one academic hospital for Covid‐19 illness. We analyzed basic demographic, clinical, and laboratory data to understand the relationship between lymphocytopenia at the time of hospital admission and clinical outcomes.ResultsWe discovered that lymphocyte count is lower (P = .01) and lymphocytopenia more frequent by an odds ratio of 3.40 (95% CI: 1.06‐10.96; P = .04) in patients admitted to the Intensive Care Unit (ICU), a marker of disease severity, relative to those who were not. We additionally find that patients with lymphocytopenia were more likely to develop an acute kidney injury (AKI), a marker of organ failure, during admission by an odds ratio of 4.29 (95% CI: 1.35‐13.57; P = .01).ConclusionThis evidence supports the hypothesis that lymphocytopenia can be an early, useful, and easily obtained, prognostic factor in determining the clinical course and disease severity of a patient admitted to the hospital for Covid‐19.

Neutrophilic drug reactions

Neutrophilic drug reactions are unique eruptions that can affect hospitalized patients and share a common pathophysiology with neutrophils as the key mediators of inflammation. They range in clinical presentation from papules and plaques to bullae and erosions to pustules. Although there is some overlap in presentation, each has distinguishing features that aid the clinician in differentiation from one another and from other drug hypersensitivity reactions. Much of the data on these reactions are from case reports and series or retrospective review studies. There are limited prospective observational studies dedicated to these adverse drug reactions. We review the more common and life-threatening neutrophilic drug reactions, their proposed mechanism of action, and their management.

Nutritional Status among Children Receiving Ashwagandhadhi Churna with milk in Residential Homes, Tamil Nadu, India

Background: Children of residential homes (RH) have a high rate of health problems including malnutrition. Ayurveda has several drugs to alleviate malnutrition, Ashwagandhadhi being one of the most commonly used drug. This study evaluated the changes in nutritional status of children in RH consuming Ashwagandhadhi churna (AC) for 1-3 years.&#x0D; Methods: This was a retrospective descriptive record review study of children consuming AC regularly for 1-3 years of two RH of Tamilnadu during 2018-2019. The children of age 6-9 and 10-13 years received a dosage of 2 and 4g of AC respectively in milk once a day. Socio-demographic details, anthropometric measurements and clinical details were assessed. Data at three time points with an interval of three months were extracted and analysed using Friedman’s tests.&#x0D; Important observations and Results: Out of 289 children, 173, who had complete details of three assessments, were included for analysis. Irrespective of the years of consumption of AC, at the first assessment, 61.6% were stunted or severely stunted; 71.2% underweight or severely underweight. Significant improvement in height-for-age and weight-for-age were observed in children consuming AC for 1 and 2 years. BMI did not show significant difference in 3 years of consumption. Males of 10 to 13 years having 1 and 2 years of consumption and females of both age groups having 2 years of consumption showed difference in BMI.&#x0D; Conclusion: Approximately three in ten children were malnourished in RH. Consumption of AC over a period of time improved the nutritional status of these children. Further large scale trials could prove the efficacy of AC in alleviating malnutrition.

Conjugated Hyperbilirubinemia as a Predictor of Mortality and Morbidity Following Cardiac Surgery

Purpose: Liver disease is common among patients with congenital heart disease (CHD) and is multifactorial in nature. Intraoperative hypoxia or low cardiac output are hypothesized to cause liver injury, as are the use of total parenteral nutrition or inotropic infusions. Therefore, conjugated bilirubin, a marker of liver disease, is a candidate marker of morbidity and mortality in neonates and infants after cardiac surgery. The purpose of our study is to describe the characteristics of patients with conjugated hyperbilirubinemia and investigate the association of bilirubin with post-operative outcomes. Methods: This was a retrospective chart review study of neonates ( < 30 days of age) and infants (≥30 days through < …

Emergency admissions of ambulatory care sensitive conditions at a Japanese local hospital: An observational study.

BackgroundEmergency admissions of ambulatory care sensitive conditions (ACSCs) are a key marker of quality of primary care and are used nationwide in some countries including the UK and Australia. There are still little data on ACSCs available in Japan. This study aimed to provide the descriptive data of the current state of ACSCs at a local hospital in Japan.MethodsThis is an observational study of retrospective chart review. The study setting is Funabashi Futawa Hospital (FFH), an acute care private hospital in Funabashi City, Japan. We analyzed the shares of admissions of ACSCs out of all admissions at FFH from April 2014 to March 2015.Results5380 charts were reviewed. Emergency admissions were 3275 cases (61%) of all admissions. Emergency admissions for ACSCs accounted for 946 cases (28.9%) of all emergency admissions. Chronic ACSC is the most common subcategory. Urinary tract infection accounted most for admissions of acute ACSCs (138 cases, 4.2% of all emergency admissions). Asthma accounted most for admissions of chronic ACSCs (139 cases, 4.2%). Pneumonia accounted most for admissions of vaccine‐preventable ACSCs (99 cases, 3.0%).ConclusionsThis is one of the first reports describing ACSCs in Japan. Most common reasons for ACSCs were similar to the previous data from the UK, except COPD ranked lower and asthma ranked higher, respectively. Comparison among different facilities (horizontal) and over time in the same institution (longitudinal), as well as analyzing relationships between other markers of primary care quality and ACSCs, will be necessary in the future.

Tuberculosis in Health Care Workers and the Impact of Implementation of Hospital Infection-Control Measures.

Background: Most studies that have evaluated the impact of infection-control measures (ICM) reported a decrease in latent tuberculosis (TB) and not in TB. The objective of this study was to evaluate the impact of ICM on TB incidence among Health Care Workers (HCW's). Methods: We conducted a retrospective record review study in a general, tertiary care, university-affiliated hospital. All TB case reports among HCWs in the hospital from 2005 to 2018 were reviewed. The TB incidence was measured before and after 2012 to evaluate the impact of ICM implemented. Findings: In total, there were 53 TB cases. The number of TB cases before and after the implementation of ICM was 42 (incidence: 100.0 cases/100,000 HCWs/year) and 11 (incidence: 26.2 cases/100,000 HCWs/year), respectively (p < .0001). Conclusions/Application to Practice: TB incidence among HCWs reduced significantly after the implementation of ICM. The establishment of ICM, such as written TB infection control plan, monitoring, screening, training, and education, can reduce TB incidence.

Association between Foramen Size and Febrile Seizure Status in the Pediatric Population: A Two-Center Retrospective Analysis.

Objective Febrile seizures have been shown to occur in 2 to 5% of children between the ages of 6 months and 5 years, making them the most common seizures of childhood. Multiple risk factors for febrile seizures have been identified; however, no investigation has been conducted to explore foramen size and associated venous drainage as a potential risk factor for experiencing febrile seizures. Of particular interest are the parietal foramen and the condylar canal, which conduct the parietal emissary vein and the occipital emissary vein, respectively. Emissary veins lack valves, allowing them to play a crucial role in selective brain cooling via a bidirectional flow of blood from the head’s evaporating surface. Narrowed cranial apertures conducting these veins may lead to reduced cerebral venous outflow and delayed brain cooling, creating favorable conditions for a febrile event. This study seeks to explore the association between cranial aperture area and febrile seizure status. Methods A retrospective cross-sectional medical record review study from January 2011 to December 2017 was conducted at a 500-bed academic hospital and a 977-bed private hospital in Lubbock, Texas, United States. A total of 101 complex febrile seizure patients were compared with a similarly aged group of 75 trauma patients representing the normal population. Parietal foramen area and condylar canal area were electronically measured and defined as having “normal” or “below normal” area. Statistical Analysis Independent t -tests were used to compare foramen and canal areas by febrile seizure status. Logistic regression analyses were conducted to determine the association of small cranial aperture area with febrile seizure status. Results Below normal parietal foramen area had a strong association with febrile seizures in our patient population. Male sex, white race, and complete vaccination status were also found to have significant associations with febrile seizure status. Conclusion Our findings indicated that narrowed parietal foramen may be considered as a risk factor for febrile seizure development.

Echocardiographic and Clinical Correlates of Ejection Fraction Among 2000 Patients with Heart Failure in Western Saudi Arabia.

IntroductionHeart failure (HF) affects about 320,000 Saudi individuals and is associated with a considerable negative impact on the patients’ quality of life. In literature, there is a lack of data about the echocardiographic abnormalities of HF patients in Saudi Arabia.Aim of WorkTo describe the echocardiographic findings of HF patients in Western Saudi Arabia.MethodologyThis was a retrospective record review study conducted on 2000 patients with chronic HF in Saudi Arabia. Demographic, clinical and echocardiographic data were collected and compared among patients with HF with reduced ejection fraction (HFrEF), ie, EF≤40%; HF with mid-range EF (HFmrEF), ie, EF=41–49%; and HF with preserved EF (HFpEF), ie, EF≥50%.ResultsAmong the 2000 patients studied, females constituted 46.3% of the sample. About 52% of females had HFpEF, whilst 70% of males had HFrEF (p<0.0001). Diastolic dysfunction occurred in 98% of HFpEF versus 78% of HFrEF (p<0.0001). Patients with HFrEF had higher left-ventricular diastolic (LVd) volume (1536 versus 826), higher left-ventricular systolic (LVs) volume (1660 vs 772), higher left atrial volume (1344 vs 875), higher aortic root dimension (1144 vs 929) and lower fractional shortening (FS) (267 vs 1213) than patients with HFpEF (p<0.0001).ConclusionHFpEF was more common among females and was associated with higher rates of diastolic dysfunction and higher FS. HFrEF was prevalent among males and associated with higher LVd, LVs, left atrium volume and aortic root dimensions.

Pattern of ophthalmic referral from primary healthcare centres to secondary and tertiary hospitals in Qassim Province, Saudi Arabia

Background: References of patients with ocular diseases are an indirect indicator that reflects integration of primary eye care into primary health care system. The study reviewed the ophthalmic references from four randomly selected primary health care centres to ophthalmic unit of secondary and tertiary hospitals in Qassim Province of Saudi Arabia.Methods: This was a retrospective health record review study was undertaken at four PHC centres in Qassim region during the year 2017. All cases referred for eye care from January 2015 until December 2016 were included. Prevalence of ophthalmic reference, profile of ophthalmic patients and ophthalmic diagnosis were analysed.Results: From primary health care centres, 423 eye cases from 1,387,996 population of catchment area were referred. The annual prevalence of eye reference was 1.5 per 10,000. Vision screening and diabetic retinopathy evaluation comprised of 55.5% of total reference.Conclusions: Poor reference pattern from primary health care centers to secondary and tertiary eye centre suggest urgent and strong need of implementing primary eye care in the primary health care centres of Saudi Arabia. Referral of simple eye cases are increasing the workload of ophthalmologists and can delay urgent referrals like cases of diabetic retinopathy screening.

Oral manifestations of Langerhans cell histiocytosis with unusual histomorphologic features

Langerhans cell histiocytosis (LCH) is a bone marrow-derived immature myeloid dendritic cell proliferative disorder with diverse clinical manifestations commonly involves bone, skin, lymph node and lung. Oral involvement is uncommon. Intraoral lesions can be the first sign of either a localized LCH or clinically undiagnosed systemic LCH, predates systemic manifestations of LCH, or an early indicator of recurrence in known cases. Clinically, it can be mistaken for primary oral and dental inflammatory, infectious and neoplastic lesions. Histologically, diagnostic challenges may arise because of the nature of oral and dental specimens, different tissue reaction patterns and variations in histomorphology of LCH. We performed a retrospective review study over 10 years. We searched for diagnosed cases of LCH. We retrieved and reviewed cases of LCH with oral involvement. We found 54 cases of LCH, four (7.4%) with oral involvement. The age range was between 1 and 27 years with an average age of 13.7 years. They were males. They were clinically confused with abscess, cysts, infection, granulation tissue and other neoplastic lesions. Histologically, they showed different histopathologic features including different patterns of necrosis, granulomas, allergic-like inflammation, superimposed infection, stomatitis, cyst and sinus formation, foreign body giant cell reaction, and foci mimicking lymphomas and metastasis. Certain cytologic features were helpful hints. In doubtful cases, immunohistochemistry helped confirm the diagnosis. Because of the multiple fragmented nature of oral specimens with different tissue reaction patterns, the diagnostic Langerhans cells may be missed or misinterpreted. Oral LCH may be confused with infectious, inflammatory, benign and malignant neoplastic lesions because of its variable clinical presentations and its heterogeneous histomorphologic features. Pathologists have an important role in guiding clinicians to the correct diagnosis and patients' management. They should be familiar with the different histomorphologic patterns to avoid pitfalls. Attention to certain morphologic features and immunohistochemistry should help resolve challenging cases.

A multicenter chart review study of patients with metastatic pancreatic ductal adenocarcinoma receiving liposomal irinotecan after gemcitabine-based therapy.

e16733 Background: Real-world data allows healthcare decision-makers to assess and manage therapeutic and economic options for patients, including those who would and would not have met eligibility criteria for randomized control trials (RCT) and are instead managed under usual care. This retrospective multi-academic center chart review study describes real-world characteristics and outcomes of US patients receiving liposomal irinotecan for the management of metastatic pancreatic ductal adenocarcinoma (mPDAC). Methods: Patients with mPDAC treated with liposomal irinotecan were eligible. Initiation of liposomal irinotecan defined index date; covariates assessed included clinical characteristics and treatment patterns; real-world overall survival (rwOS) was assessed via Kaplan-Meier methodology. The target enrollment is 300 patients. The study centers included were Memorial Sloan Kettering Cancer Center, Cedars-Sinai Medical Center, Emory Winship Cancer Institute, Houston Methodist Cancer Center, Henry Ford Cancer Institute, and University of Pittsburgh Medical Center. Results: Data on 26 patients were available for initial analyses. Mean age was 68 years; 58% were female and 65% Caucasian. 54% of patients had stage IV disease at first diagnosis, and 17%, 65%, and 17% had index ECOG score of 0, 1, and 2, respectively. Common genetic mutations include KRAS (40%) and TP53 (40%). Prior to liposomal irinotecan, treatments received for metastatic disease include gemcitabine+nab-paclitaxel (77%) and fluorouracil (5-FU)/leucovorin (LV)+irinotecan+oxaliplatin (19%). Patients had received 0 (12%), 1 (23%), and ≥2 (65%) lines of therapy in the metastatic setting prior to liposomal irinotecan. Mean duration of liposomal irinotecan use was 3.0 months; liposomal irinotecan was mostly received with 5-FU (23%) or 5-FU/LV (69%). Median rwOS was 4.9 months (95% CI: 3.0, 6.3). Conclusions: Real-world data of the first 26 patients in this study show patients treated with liposomal irinotecan are older, sicker, and have had more lines of therapy than previously reported in RCT data.

EP18 Use of anakinra for haemophagocytic lymphohistiocytosis in adults

Abstract Background Haemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening syndrome characterised by severe immune activation. The primary form occurs in early childhood and is fatal without treatment. Secondary HLH can occur at any age, usually in the setting of autoimmune disease, malignancy and infections. Macrophage activation syndrome is a form of HLH occurring in patients with juvenile idiopathic arthritis or other rheumatological conditions. The 2004 paediatric HLH treatment protocol is an established regime of etoposide, dexamethasone and ciclosporin. In recent years, studies have suggested a role for Anakinra in paediatric patients with HLH. We describe our recent experience with Anakinra in adult patients with secondary HLH. Methods We performed a retrospective case review study of four adults diagnosed with HLH and treated with anakinra between 2014 and 2018. Results Three males and one female with mean age of 35 (18-53) fulfilled the 2004 HLH diagnostic criteria. Three patients had confirmed secondary HLH with the primary trigger being CMV infection, a connective tissue disease (CTD) &amp; T-cell lymphoma and adult-onset Still’s disease. The aetiology of the fourth patient was uncertain although viral infection was likely. Common presenting features were fever, hyperferritinaemia and anaemia. Three patients were neutropaenic and two pancytopaenic. The peak mean ferritin level was 50358µg/L, (10052 - 126808). All patients were treated with anakinra 100mg subcutaneously per day. Ferritin fell rapidly, and at day 7 following treatment, the mean was 3688µg/L (645 - 8957). Three patients achieved initial clinical remission. The first stopped anakinra after 14 days of treatment and has done well, the second has remained on treatment. The third achieved initial clinical remission, stopped anakinra after two months but relapsed and died from atypical pneumonia. The fourth with CTD and T-cell lymphoma had a large drop in ferritin but did not achieve remission and died (Table 1). Conclusion HLH is a rare life threatening disorder. Similar to paediatric studies, anakinra appears to reduce ferritin rapidly in adults with secondary HLH. Anakinra may be effective in improving the clinical outcome in adults with non-malignancy associated secondary HLH. However, consistent with previous reports, patients with malignancy associated HLH had a worse survival. Disclosures L. Sammut None. S. Murryam None. N. Vincent None. B. Davidson None. C.J. Edwards Honoraria; C.J.E has received support to attend meetings and received honoraria from Amgen.

The Incidence and Nature of Adverse Medical Device Events in Dutch Hospitals: A Retrospective Patient Record Review Study.

Despite widespread use of medical devices and their increasing complexity, their contribution to unintended injury caused by healthcare (adverse events, AEs) remains relatively understudied. The aim of this study was to gain insight in the incidence and types of AEs involving medical devices (AMDEs). Data from two patient record studies for the identification of AEs were used. Identification of AMDEs was part of these studies. Patient records of 6894 admissions of a random sample of 20 hospitals in 2011/2012 and 19 hospitals in 2015/2016 were reviewed for AMDEs by trained nurses and physicians. In 98.7% of the admissions, a medical device was used. Adverse events involving medical devices were present in 2.8% of the admissions, with 24% of the AMDEs being potentially preventable. Of all AEs, in 40%, medical devices were involved. Of all potentially preventable AEs, in 44%, medical devices were involved. Implants were most often involved in potentially preventable AMDEs. Medical devices are substantially involved in potentially preventable AEs in hospitals. Research into AMDEs is of great importance because of the increasing use and complexity of medical devices. Based on patient records, most improvements could be made for placement of implants and prevention of infections related to medical devices. Safety and safe use of medical devices should be a subject of attention and further research.

Incorporation of genetic testing significantly increases the number of individuals diagnosed with familial hypercholesterolemia

It is estimated that less than 10% of cases of familial hypercholesterolemia (FH) in the United States have been diagnosed. Low rates of diagnosis may in part be attributable to affected patients not meeting the clinical diagnostic criteria of the Dutch Lipid Clinic Network (DLCN), Simon Broome, or US MEDPED diagnostic criteria. The objective of this study was to assess the utility of incorporating genetic testing into a patient's evaluation for FH. We retrospectively reviewed patients seen in the Advanced Lipids Disorders Clinic at Johns Hopkins Hospital between January 2015 and May 2018. Between June 2018 and December 2018, patients were consented to a prospective registry. DLCN, Simon Broome, and MEDPED criteria were applied to each patient, before and after genetic testing. Genetic testing included sequencing and deletion duplication analysis of four genes (LDLR, PCSK9, APOB, and LDLRAP1). The retrospective review and prospective study identified 135 adult probands who were seen in our clinic for evaluation of heterozygous FH. Twenty-nine individuals (21%) were heterozygous for a pathogenic or likely pathogenic monogenic variant. Before genetic testing, using the DLCN criteria, 35 (26%) individuals met criteria for a definite diagnosis of FH. Thirty patients (22%) met criteria using Simon Broome, and 29 (21%) patients met criteria using US MEDPED before genetic analysis. Depending on the criteria, incorporating genetic testing identified 11-14 additional patients with FH. Incorporating genetic testing diagnosed almost 50% more patients with definite FH in comparison to classification solely on clinical grounds.