Needle Decompression Complicated by Cardiac Injury in a Prehospital Environment.

Needle decompression is a mainstay intervention for tension pneumothorax in trauma medicine. It is used in combat and prehospital medicine when definitive measures are often not available or ideal. It can temporarily relieve increased intrathoracic pressure and treat a collapsed lung or great vessel obstruction. However, when done incorrectly, it can result in underlying visceral organ and vessel trauma. This is a case of an adult male who presented to the emergency department after sustaining multiple stab wounds during an altercation. On arrival, the patient had a 14-gauge angiocatheter inserted at the 4th intercostal space (ICS), left of the parasternal line traversing the right ventricle and interventricular septum and terminating in the left ventricle. The case emphasizes the importance of understanding the landmarks of performing needle decompression in increasing the procedure's efficacy and reducing iatrogenic complications.

Two Satellite Gene Clusters Enhance Ergot Alkaloid Biosynthesis Capacity of Aspergillus leporis.

Ergot alkaloids are fungal specialized metabolites that are important in agriculture and serve as sources of several pharmaceuticals. Aspergillus leporis is a soil saprotroph that possesses two ergot alkaloid biosynthetic gene clusters encoding lysergic acid amide production. We identified two additional, partial biosynthetic gene clusters within the A. leporis genome containing some of the ergot alkaloid synthesis (eas) genes required to make two groups of clavine ergot alkaloids, fumigaclavines and rugulovasines. Clavines possess unique biological properties compared to lysergic acid derivatives. Bioinformatic analyses indicated the fumigaclavine cluster contained functional copies of easA, easG, easD, easM, and easN. Genes resembling easQ and easH, which are required for rugulovasine production, were identified in a separate gene cluster. The pathways encoded by these partial, or satellite, clusters would require intermediates from the previously described lysergic acid amide pathway to synthesize a product. Chemical analyses of A. leporis cultures revealed the presence of fumigaclavine A. However, rugulovasine was only detected in a single sample, prompting a heterologous expression approach to confirm functionality of easQ and easH. An easA knockout strain of Metarhizium brunneum, which accumulates the rugulovasine precursor chanoclavine-I aldehyde, was chosen as expression host. Strains of M. brunneum expressing easQ and easH from A. leporis accumulated rugulovasine as demonstrated through mass spectrometry analysis. These data indicate that A. leporis is exceptional among fungi in having the capacity to synthesize products from three branches of the ergot alkaloid pathway and for utilizing an unusual satellite cluster approach to achieve that outcome. IMPORTANCE Ergot alkaloids are chemicals produced by several species of fungi and are notable for their impacts on agriculture and medicine. The ability to make ergot alkaloids is typically encoded by a clustered set of genes that are physically adjacent on a chromosome. Different ergot alkaloid classes are formed via branching of a complex pathway that begins with a core set of the same five genes. Most ergot alkaloid-producing fungi have a single cluster of genes that is complete, or self-sufficient, and produce ergot alkaloids from one or occasionally two branches from that single cluster. Our data show that Aspergillus leporis is exceptional in having the genetic capacity to make products from three pathway branches. Moreover, it uses a satellite cluster approach, in which gene products of partial clusters rely on supplementation with a chemical intermediate produced via another gene cluster, to diversify its biosynthetic potential without duplicating all the steps.

Contribution of a novel gene to lysergic acid amide synthesis in Metarhizium brunneum.

OBJECTIVE: The fungus Metarhizium brunneum produces ergot alkaloids of the lysergic acid amide class, most abundantly lysergic acid α-hydroxyethylamide (LAH). Genes for making ergot alkaloids are clustered in the genomes of producers. Gene clusters of LAH-producing fungi contain an α/ß hydrolase fold protein-encoding gene named easP whose presence correlates with LAH production but whose contribution to LAH synthesis in unknown. We tested whether EasP contributes to LAH accumulation through gene knockout studies. RESULTS: We knocked out easP in M. brunneum via a CRISPR/Cas9-based approach, and accumulation of LAH was reduced to less than half the amount observed in the wild type. Because LAH accumulation was reduced and not eliminated, we identified and mutated the only close homolog of easP in the M. brunneum genome, a gene we named estA. An easP/estA double mutant did not differ from the easP mutant in lysergic acid amide accumulation, indicating estA had no role in the pathway. We conclude EasP contributes to LAH accumulation but is not absolutely required. Either a gene encoding redundant function and lacking sequence identity with easP resides outside the ergot alkaloid synthesis gene cluster, or EasP plays an accessory role in the synthesis of LAH.

Adherence to goal-directed therapy for patients with cirrhosis hospitalized with spontaneous bacterial peritonitis.

PURPOSE: The American Association for the Study of Liver Diseases has developed a standard of care for the treatment of patients with spontaneous bacterial peritonitis (SBP). Evidence examining the adherence rate to these guideline recommendations is limited. This study aimed to determine the adherence rate to guideline-directed therapy for patients with cirrhosis hospitalized with SBP. METHODS: This institutional review board-approved retrospective cohort study conducted at a large academic hospital evaluated the adherence rate to guideline-directed therapy in adult patients with cirrhosis hospitalized with SBP. Included hospitalized patients had a documented diagnosis of cirrhosis and acute SBP. The adherence rate to guideline-directed therapy was determined by receipt of paracentesis within 24 hours of admission, request of Gram stain and culture tests, avoidance of fresh frozen plasma, receipt of albumin on days 1 and 3, receipt of empiric antibiotics within 6 hours, receipt of SBP prophylaxis, receipt of deep vein thrombosis prophylaxis, and offer of pneumococcal vaccination. RESULTS: A total of 110 patients were included. Provider adherence to goal-directed therapy was poor, with criteria met for only 10 (9.1%) patients. The therapies with the lowest adherence rates included SBP prophylaxis on discharge (54.5%), receipt of albumin on day 3 (42.7%), and offer of pneumococcal vaccination during admission (43.6%). Patients with a gastrointestinal consult were more likely than those without a consult to obtain albumin on day 1 (69.4% vs 36.8%, P = 0.001) and albumin on day 3 (52.8% vs 23.7%, P = 0.004). CONCLUSION: This study demonstrated a lack of adherence to guideline-directed therapy for the management of SBP.

Impact of Chronic Hepatitis C virus co-infection on outcomes of infective endocarditis in people who inject drugs.

Injection drug use (IDU) is a risk factor for infective endocarditis (IE) and hepatitis C virus (HCV) infection. This retrospective cohort study assessed HCV's impact on outcomes of adult people who inject drugs (PWID). Those admitted due to IE using modified Duke criteria from January 2012 through May 2018 were identified. The cohort was divided into HCV seropositive and seronegative groups. The seropositive group was further stratified according to HCV viremia. Complications and mortality during the IE hospitalization, at 10 weeks, and 1 year were compared across groups. Clinical factors were similar between the cohorts, except patients without viremia (29, 81%) required more ICU admissions than with viremia (30, 60%) (P < 0.05). There was no difference in mortality at all time periods between the groups. Although several factors affect mortality in PWID with IE, neither HCV antibody positivity nor viremia appear to increase the risk for complications or death.

Safety of direct oral anticoagulants in patients with mild to moderate cirrhosis: a systematic review and meta-analysis.

To evaluate major bleeding in cirrhosis with use of traditional anticoagulation or direct oral anticoagulants (DOACs), using a standardized definition. Anticoagulation in patients with cirrhosis is often a clinical conundrum for providers as the necessary balance between thrombotic and bleeding risk is complicated by end organ damage. Recent meta-analyses have sought to evaluate the safety and efficacy of direct oral anticoagulants in patients with liver disease. These recent analyses are limited by various bleeding definitions, broad inclusion criteria, and few indications for anticoagulation. We sought to conduct a meta-analysis using a validated definition for major bleeding and compare rates between traditional anticoagulation and DOACs in patients with cirrhosis. Articles were eligible for inclusion if the international society on thrombosis and hemostasis (ISTH) definition of a major bleed was the primary safety outcome. Additionally, only articles including patients with cirrhosis and receiving treatment with anticoagulation for an indication for stroke prevention or venous thromboembolism were eligible. Eligible articles needed a DOAC comparator group against traditional anticoagulant medication. Seven studies met inclusion criteria and compiled data for 683 patients in the meta-analysis. Pooled trial analysis demonstrated no statistically significant difference in the primary outcome of ISTH major bleeding (OR 0.55, 95%CI 0.28-1.07, I2 0%). Individual secondary outcomes of all bleeding, intracerebral hemorrhage, or gastrointestinal bleeding also demonstrated no significant difference between DOACs and traditional anticoagulation. Use of DOACs in patients with mild to moderate cirrhosis carries similar risk to use of traditional anticoagulation.

Direct Oral Anticoagulants in Select Patients With Hypercoagulable Disorders.

OBJECTIVE: To summarize the literature assessing the safety and efficacy of direct oral anticoagulants (DOACs) for the acute treatment and secondary prevention of venous thromboembolism (VTE) in select patients with hypercoagulable disorders. DATA SOURCES: An electronic PubMed literature search was conducted from January 2010 to July 2020 using the following keywords: DOAC, rivaroxaban, apixaban, dabigatran, edoxaban, thrombophilia, cancer, antiphospholipid syndrome, protein C deficiency, protein S deficiency, antithrombin deficiency, factor V Leiden, prothrombin G20210A gene mutation, congenital thrombophilia, hypercoagulable, hereditary thrombophilia, acquired thrombophilia. STUDY SELECTION AND DATA EXTRACTION: Articles were included if they reported clinical outcomes associated with cancer-associated VTE, antiphospholipid syndrome (APS), and other hereditary thrombophilias. DATA SYNTHESIS: The safety and efficacy of using a DOAC is highly dependent on the type of hypercoagulable disease state. Current trials support the use of edoxaban, rivaroxaban, and apixaban for the treatment of cancer-associated thrombosis (CAT), with apixaban being preferred because of lower bleeding rates compared with standard of care. The use of DOACs, especially rivaroxaban, have been associated with worse outcomes in patients with APS, whereas data on DOAC use in hereditary thrombophilia remains scarce and limited to low-risk patients. RELEVANCE TO PATIENT CARE AND CLINICAL PRACTICE: This review evaluates the literature assessing the safety and efficacy of DOACs in patients with various hypercoagulable disorders. CONCLUSIONS: The current body of evidence supports the use of select DOACs for the treatment of CAT. In contrast, DOAC use in patients with APS and hereditary thrombophilia should be avoided at this time.

Infective Endocarditis Guidelines: The Challenges of Adherence-A Survey of Infectious Diseases Clinicians.

BACKGROUND: Guidelines exist to aid clinicians in managing patients with infective endocarditis (IE), but the degree of adherence with guidelines by Infectious Disease (ID) physicians is largely unknown. METHODS: An electronic survey assessing adherence with selected IE guidelines was emailed to 1409 adult ID physician members of the Infectious Diseases Society of America's Emerging Infections Network. RESULTS: Five hundred fifty-seven physicians who managed IE responded. Twenty percent indicated that ID was not consulted on every case of IE at their hospitals, and 13% did not recommend transthoracic echocardiography (TTE) for all IE cases. The duration of antimicrobial therapy was timed from the first day of negative blood cultures by 91% of respondents. Thirty-four percent of clinicians did not utilize an aminoglycoside for staphylococcal prosthetic valve IE (PVE). Double ß-lactam therapy was "usually" or "almost always" employed by 83% of respondents for enterococcal IE. For patients with active IE who underwent valve replacement and manifested positive surgical cultures, 6 weeks of postoperative antibiotics was recommended by 86% of clinicians. CONCLUSIONS: The finding that adherence was <90% with core guideline recommendations that all patients with suspected IE be seen by ID and that all patients undergo TTE is noteworthy. Aminoglycoside therapy of IE appears to be declining, with double ß-lactam regimens emerging as the preferred treatment for enterococcal IE. The duration of postoperative antimicrobial therapy for patients undergoing valve replacement during acute IE is poorly defined and represents an area for which additional evidence is needed.

Genetic Reprogramming of the Ergot Alkaloid Pathway of Metarhizium brunneum.

Ergot alkaloids are important specialized fungal metabolites that are used to make potent pharmaceuticals for neurological diseases and disorders. Lysergic acid (LA) and dihydrolysergic acid (DHLA) are desirable lead compounds for pharmaceutical semisynthesis but are typically transient intermediates in the ergot alkaloid and dihydroergot alkaloid pathways. Previous work with Neosartorya fumigata demonstrated strategies to produce these compounds as pathway end products, but their percent yield (percentage of molecules in product state as opposed to precursor state) was low. Moreover, ergot alkaloids in N. fumigata are typically retained in the fungus as opposed to being secreted. We used clustered regularly interspaced short palindromic repeat (CRISPR)-CRISPR-associated protein 9 (Cas9) and heterologous expression approaches to engineer these compounds in Metarhizium brunneum, representing an alternate expression host from a different lineage of fungi. The relative percent yields of LA (86.9%) and DHLA (72.8%) were much higher than those calculated here for previously engineered strains of N. fumigata (2.6% and 2.0%, respectively). Secretion of these alkaloids also was measured, with averages of 98.4% of LA and 87.5% of DHLA being secreted into the growth medium; both values were significantly higher than those measured for the N. fumigata derivatives (both of which were less than 5.6% secreted). We used a similar approach to engineer a novel dihydroergot alkaloid in M. brunneum and, through high-performance liquid chromatography-mass spectrometry (LC-MS) analyses, provisionally identified it as the dihydrogenated form of lysergic acid α-hydroxyethylamide (dihydro-LAH). The engineering of these strains provides a strategy for producing novel and pharmaceutically important chemicals in a fungus more suitable for their production.IMPORTANCE Ergot alkaloids derived from LA or DHLA are the bases for numerous pharmaceuticals with applications in the treatment of dementia, migraines, hyperprolactinemia, and other conditions. However, extraction of ergot alkaloids from natural sources is inefficient, and their chemical synthesis is expensive. The ability to control and redirect ergot alkaloid synthesis in fungi may allow more efficient production of these important chemicals and facilitate research on novel derivatives. Our results show that Metarhizium brunneum can be engineered to efficiently produce and secrete LA and DHLA and, also, to produce a novel derivative of DHLA not previously found in nature. The engineering of dihydroergot alkaloids, including a novel species, is important because very few natural sources of these compounds are known. Our approach establishes a platform with which to use M. brunneum to study the production of other ergot alkaloids, specifically those classified as lysergic acid amides and dihydroergot alkaloids.

Outcomes Associated With Resuming Direct Oral Anticoagulant Therapy Following Admission for a Gastrointestinal Bleed.

BACKGROUND: Although direct oral anticoagulants (DOACs) carry a lower bleeding risk compared with warfarin, gastrointestinal bleeds (GIB) are a known complication. There are limited data observing outcomes associated with resuming DOACs following a GIB. OBJECTIVE: The purpose of this study was to evaluate practice patterns and clinical outcomes of patients admitted with an index GIB while receiving DOAC therapy. METHODS: This retrospective, single-system study included adult patients receiving DOAC therapy prior to admission and hospitalized with an index GIB between January 1, 2013, and October 31, 2018. Patient exclusion criteria were a history of immune thrombocytopenia purpura or inflammatory bowel disease; discharge to hospice; leaving against medical advice; or death during hospitalization. The primary objective was 90-day readmission for a recurrent GIB. RESULTS: There were 57 patients included for analysis; 37 patients had DOAC therapy held >7 days, 18 patients resumed DOAC therapy within 7 days, and 2 patients switched to warfarin. The majority of patients received rivaroxaban (59.6%) prior to admission for atrial fibrillation (71.9%), were admitted with a major GIB (66.7%), and required a blood transfusion (61.4%). The rates of recurrent GIB were 2.5% (n = 1) and 5.6% (n = 1) for those who had their DOAC held and resumed, respectively (P = 0.83). Mortality within 12 months of discharge occurred in 4 patients (10.8%) who had their DOAC held and 4 patients (22.2%) who resumed DOAC therapy (P = 0.28). CONCLUSION AND RELEVANCE: Resuming anticoagulation within 7 days of admission for an index GIB was not associated with a recurrent GIB within 90 days of discharge.

Left-sided infective endocarditis in persons who inject drugs.

PURPOSE: The purpose of this study was to describe left-sided infective endocarditis (LSIE) in persons who inject drugs (PWID) and compare that group to PWID with non-LSIE and to non-PWID with LSIE. METHODS: Retrospective single-center study of adult IE patients from 2011 to 2018. RESULTS: Of the 333 patients in our cohort, 54 were PWID with LSIE, 75 were PWID with non-LSIE, and 204 were non-PWID with LSIE. When comparing LSIE vs non-LSIE in PWID, the LSIE group was older (median age 35 vs 28.5, p < 0.01), had fewer S. aureus infections (59% vs 92%, p < 0.01), was more likely to have cardiac surgery (31% vs 13%, p < 0.01), and had a higher 10-week mortality (22% vs 5%, p < 0.01). When comparing PWID with LSIE to non-PWID with LSIE, the PWID group were younger (median age 35 vs 46, p < 0.01); had more frequent multi-valve involvement (33% vs 19%, p = 0.04), Staphylococcus aureus infections (54% vs 27%, p < 0.01), and previous IE (24% vs 8%, p < 0.01); and experienced more strokes (54% vs 31%, p < 0.01). Ten-week mortality was similar for LSIE in both PWID and non-PWID (24% vs 20%, p = 0.47). CONCLUSIONS: LSIE in PWID is not uncommon. Compared to non-LSIE in PWID, valve surgery is more common and mortality is higher. For reasons that are unclear, stroke is more frequent in LSIE in PWID than in non-PWID with LSIE but mortality is no different.

Direct oral anticoagulants and warfarin in patients with cirrhosis: a comparison of outcomes.

Anticoagulation management in patients with cirrhosis presents several challenges as a result of alterations in hemostasis. Historically vitamin k antagonists and low molecular weight heparins have been the agents of choice in this patient population. Direct oral anticoagulants (DOACs) may provide an alternative to traditional anticoagulant therapy. To evaluate the rate of major bleeding among patients receiving DOACs or warfarin with cirrhosis. A retrospective, observational, cohort study of adult patients admitted between January 2012 and July 2018 with diagnosis of cirrhosis receiving anticoagulation with DOAC or warfarin therapy was performed. Patients were stratified based on the receipt of a DOAC or warfarin. The primary endpoint was incidence of major bleeding at 90 days. Secondary endpoints included stroke or embolic event at 90 days as well as rehospitalization and mortality at 1 year. One hundred sixty-seven patients were included for analysis; of which 110 received warfarin and 57 received a DOAC. The most commonly used DOAC was apixaban (52.6%) followed by rivaroxaban (45.6%) and dabigratran (1.8%). The incidence of major bleeding was similar between warfarin and DOAC groups (9.1% vs. 5.2% p = 0.381). No difference in the rate of stroke or recurrent embolic event at 90 days was identified between the two groups (0% vs. 1.58% p = 0.341; 1.8% vs. 1.8% p = 0.731). In conclusion DOACs appear to be a safe alternative to warfarin in patients with mild to moderate cirrhosis. Further studies are warranted to confirm these findings.

Oral antibiotics for the treatment of Gram-negative bloodstream infections: A retrospective comparison of three antibiotic classes.

OBJECTIVES: Treatment of bacteraemia with oral antibiotics has the potential to reduce hospital length of stay, treatment costs and line-related complications. To date, small trials have supported the use of specific classes of antibiotics, primarily fluoroquinolones (FQs), in the treatment of Gram-negative bloodstream infections (GNBSIs). Currently, limited data exist evaluating treatment with ß-lactams (BLs) or trimethoprim/sulfamethoxazole (SXT). The purpose of this study was to compare treatment of GNBSIs across three different oral antibiotic classes. METHODS: A retrospective cohort of hospitalised patients with GNBSI receiving initial intravenous (i.v.) antibiotic therapy followed by step-down oral therapy was conducted. Patients were divided into one of three oral antibiotic treatment groups: FQ; BL; or SXT. The composite primary endpoint was treatment failure, including 30-day mortality, recurrent bacteraemia or transition back to i.v. antibiotics. Additional endpoints included secondary infections and individual components within the primary endpoint. Categorical endpoints were analysed using χ2 test or Fisher's exact test, whilst continuous variables were assessed by one-way ANOVA. RESULTS: A total of 204 patients were included in the analysis. The majority of patients received a FQ (136; 66.7%), followed by a BL (46; 22.5%) and SXT (22; 10.8%). Treatment failure occurred in 15 patients (7.4%), with no statistically significant differences between groups. Likewise, individual composite outcomes and secondary outcomes demonstrated no statistically significant differences. CONCLUSION: Transitioning to oral antibiotics to complete GNBSI treatment can offer many advantages. As FQ resistance increases, data supporting the use of a BL or SXT in GNBSI treatment will become essential.

The Effect of Preexisting Anticoagulation on Cerebrovascular Events in Left-Sided Infective Endocarditis.

BACKGROUND: Stroke is a frequent complication of infective endocarditis, especially infection involving left-sided valves. Management of anticoagulation in left-sided infective endocarditis is controversial as it is unclear whether anticoagulation impacts stroke and bleeding risk in patients with this condition. The objective of this study was to evaluate the effect of anticoagulation on stroke occurrence and bleeding complications in patients with left-sided infective endocarditis. METHODS: Patients admitted to a tertiary academic hospital with left-sided infective endocarditis between December 2011 and April 2018 were identified. Patients were stratified based on receipt of therapeutic anticoagulation prior to admission. The primary outcome measure was the rate of radiographically confirmed stroke at 10 weeks. RESULTS: Two-hundred and fifty-eight consecutive patients with left-sided infective endocarditis were identified. Patients receiving anticoagulation (n = 50) were older (median age 63 vs 52; P = .02), were more likely to have a history of atrial fibrillation (22% vs 8.2%; P < .01), more often had prosthetic valves (38% vs 13.9%; P < .01), and had a lower incidence of mitral valve involvement (40% vs 62%; P < .01), compared with patients not receiving anticoagulation. There was no significant difference in the rate of stroke, cerebrovascular hemorrhage, or mortality at 10 weeks between the two cohorts. CONCLUSIONS: Preexisting anticoagulation did not appear to have an effect on stroke, cerebrovascular hemorrhage, or mortality in patients with left-sided infective endocarditis at 10 weeks. Continuation of anticoagulation in patients with a definitive preexisting indication should be considered in patients with left-sided infective endocarditis in the absence of other contraindications.

Bleeding Risk of Therapeutic Unfractionated Heparin and Low Molecular Weight Heparin in Patients with Cirrhosis.

BACKGROUND AND OBJECTIVE: Patients with cirrhosis are simultaneously at an increased risk of bleeding and thrombosis. Studies comparing the safety of parenteral anticoagulants in this population are lacking. This study evaluated the safety of therapeutic unfractionated heparin versus low molecular weight heparin in patients with cirrhosis. METHODS: This system-wide, retrospective cohort study included adults with cirrhosis receiving unfractionated heparin or low molecular weight heparin for the treatment of acute venous thromboembolism. The primary endpoint was the incidence of major bleeding. RESULTS: Eighty-two patients were included in this study, with 52 receiving unfractionated heparin and 30 receiving low molecular weight heparin. More major bleeding occurred in the unfractionated heparin arm compared to the low molecular weight heparin arm (19.2% vs 0%, p = 0.010). CONCLUSIONS: Low molecular weight heparin may be a safer option in patients with cirrhosis treated for acute venous thromboembolism, but future studies should confirm these findings.

Treatment of Helicobacter pylori in Special Patient Populations.

Helicobacter pylori infection can lead to gastritis, gastric and duodenal ulcers, and gastric cancer. Consequently, complete eradication is the goal of therapy. First-line therapy for H. pylori infection includes clarithromycin triple therapy (clarithromycin, proton pump inhibitor [PPI], and amoxicillin or metronidazole), bismuth quadruple therapy (bismuth salt, PPI, tetracycline, and metronidazole or amoxicillin), or concomitant therapy (clarithromycin, PPI, amoxicillin, and metronidazole). However, many patients have relative contraindications to the antibiotics included in these regimens, making therapy selection difficult. Furthermore, failure of initial therapy makes selection of second-line therapy challenging due to concerns for potential resistance to agents included in the initial regimen. This review discusses H. pylori microbiology, including antibiotic resistance, and summarizes the existing evidence for first- and second-line treatment regimens that may be considered for special populations such as patients with penicillin allergies, patients with or at risk for QTc-interval prolongation, and patients who are pregnant, breastfeeding, or elderly.

Screening urinalysis demonstrates that haematuria is a frequent finding in persons with haemophilia treated at a paediatric haemophilia treatment centre.

INTRODUCTION: Haematuria is a recognized complication of haemophilia A and B (HA, HB). Adult persons with haemophilia (PWH) have a higher prevalence of renal disease than the general population. There is limited literature investigating the prevalence of haematuria in paediatric PWH. AIM: Our paediatric haemophilia treatment centre (HTC) had previously used quality improvement methods to increase the frequency of screening urinalyses at annual comprehensive visits. We retrospectively reviewed the data collected to determine the prevalence of haematuria and explore for associations in those with haematuria. METHODS: Retrospective chart review to identify the frequency of haematuria on screening urinalysis in all male PWH ≥2 years old. Haematuria was defined as ≥3 red blood cells (RBCs) per high power field. Univariate logistic regression was performed to evaluate for associations with haematuria. RESULTS: A total of 93 patients met eligibility criteria. Sixty-seven with HA (11 mild, 17 moderate, 39 severe) and 26 with HB (three mild, 16 moderate, seven severe). Forty-two of ninety-three (45%) patients were identified as having haematuria (median RBCs 7, mean RBCs 332). Of those with haematuria, 76% were identified by screening UA, as opposed to clinical symptoms, and 52% had recurrent haematuria. Older age and HA were associated with an increased likelihood of haematuria. CONCLUSIONS: Our study demonstrated that the prevalence of haematuria was high in PWH treated at our paediatric HTC. Future investigation is needed to determine the population-wide prevalence of haematuria in paediatric PWH and its impact on renal function.

Use of Cardiac MRI to Assess Antitumor Efficacy of Everolimus in Sporadic Cardiac Rhabdomyoma.

Cardiac rhabdomyoma is the most common neonatal cardiac tumor and is typically associated with tuberous sclerosis complex (TSC). Although these tumors may naturally regress, some patients require surgical resection because of cardiac instability. If not fully resected, patients may also require medical therapy to improve their hemodynamics. Everolimus, a mammalian target of rapamycin inhibitor, has shown promise in reducing rhabdomyoma in patients with TSC, but the drug's impact in patients without TSC has not been reported. Monitoring of tumor response has typically been limited to echocardiograms, which is not ideal given inherent difficulties in three-dimensional measurements. We report a case of sporadic cardiac rhabdomyoma in a neonate treated with everolimus resulting in tumor regression as documented by cardiac MRI. While on everolimus, our patient had an increased incidence of a preexisting arrhythmia, which resolved with planned cessation of therapy, suggesting that close monitoring is imperative in patients with arrhythmia.