Rasburicase-induced Reds and Blues

Introduction: G6PD deficiency (G6PDD) is a genetic disorder resulting in low levels of the G6PD enzyme which plays a key role in preventing cellular damage from oxidative stress. We report a case of newly diagnosed G6PDD manifesting as methemoglobinemia (MetHb) and non-autoimmune hemolytic anemia (NIHA) following Rasburicase administration in an elderly male. Case: A 78-year-old male with a history of untreated chronic lymphocytic leukemia (CLL), congestive heart failure and hypertension presented with altered mental status and acute kidney injury. Initial labs revealed a creatinine of 6.89 mg/dL, potassium of 5.2, phosphate of 6.1, calcium of 9.0, uric acid of 13.9, leukocytosis of 68 × 109/L, and hemoglobin of 8.5 g/dL. CT head was negative. Urinalysis revealed pyuria and large leukocyte esterase. Ultrasound showed enlarged kidneys with no hydronephrosis. Due to concern for infiltrative CLL and spontaneous tumor lysis syndrome (TLS), he was treated with 6mg of Rasburicase and broad spectrum antibiotics for possible infection. The next day, the patient became hypoxic to 87% requiring non-rebreather. Imaging confirmed no evidence of pulmonary embolism, pneumonia or pulmonary edema. ABG showed a PaO2 of 280. Given the discrepancy between pulse oximetry and ABG, there was concern for MetHb secondary to G6PDD. Subsequent testing revealed elevated methemoglobin levels and low G6PD levels. Given his G6PDD, methylene blue was contraindicated. He required high flow nasal cannula and was eventually weaned down to room air. His hemoglobin also began to drop requiring multiple transfusions of PRBCs. Labs were consistent with NIHA. Dialysis was initiated for his AKI and he ultimately was discharged home. Discussion: G6PDD is the most common enzymatic deficiency which may present at birth with jaundice; however, most are asymptomatic. This case is unique given the older age of onset of the patient coupled with the rare instance of spontaneous TLS in a patient with CLL. It also illustrates the challenges with typical management strategies. Administering rasburicase vs initiating dialysis is usually considered promptly along with aggressive hydration and electrolyte repletion. However, Rasburicase administration is contraindicated in people with G6PDD due to its potential to cause MetHb and hemolysis. It is often recommended to test G6PD levels prior to administration, however this is not always feasible. Conventional treatment for MetHb includes methylene blue, likewise, this is contraindicated in patients with G6PDD because of its oxidant properties, resulting in further NIHA. Conclusion: MetHb can present in G6PDD patients along with NIHA. Treatment includes supportive therapy with supplemental oxygen, blood products and avoidance of inciting agent. G6PDD may present later in life.https://scholarlycommons.henryford.com/merf2020caserpt/1046/thumbnail.jp

Critical Care Delivery Solutions in the Emergency Department: Evolving Models in Caring for ICU Boarders

The National Academy of Medicine has identified emergency department (ED) crowding as a health care delivery problem. Because the ED is a portal of entry to the hospital, 25% of all ED encounters are related to critical illness. Crowding at both an ED and hospital level can thus lead to boarding of a number of critically ill patients in the ED. EDs are required to not only deliver immediate resuscitative and stabilizing care to critically ill patients on presentation but also provide longitudinal care while boarding for the ICU. Crowding and boarding are multifactorial and complex issues, for which different models for delivery of critical care in the ED have been described. Herein, we provide a narrative review of different models of delivery of critical care reported in the literature and highlight aspects for consideration for successful local implementation

Accelerated Critical Therapy Now in the Emergency Department Using an Early Intervention Team: The Impact of Early Critical Care Consultation for ICU Boarders

Evaluate the impact of an emergency department (ED)-based critical care consultation service, hypothesizing early consultation results in shorter hospital length of stay (LOS). DESIGN: Retrospective observational study from February 2018 to 2020. SETTING: An urban academic quaternary referral center. PATIENTS: Adult patients greater than or equal to 18 years admitted to the ICU from the ED. Exclusion criteria included age less than 18 years, do not resuscitate/do not intubate documented prior to arrival, advanced directives outlining limitations of care, and inability to calculate baseline modified Sequential Organ Failure Assessment (mSOFA) score. INTERVENTIONS: ED-based critical care consultation by an early intervention team (EIT) initiated by the primary emergency medicine physician compared with usual practice. MEASUREMENTS: The primary outcome was hospital LOS, and secondary outcomes were hospital mortality, ICU LOS, ventilator-free days, and change in the mSOFA. MAIN RESULTS: A total 1,764 patients met inclusion criteria, of which 492 (27.9%) were evaluated by EIT. Final analysis, excluding those without baseline mSOFA score, limited to 1,699 patients, 476 in EIT consultation group, and 1,223 in usual care group. Baseline mSOFA scores (±sd) were higher in the EIT consultation group at 3.6 (±2.4) versus 2.6 (±2.0) in the usual care group. After propensity score matching, there was no difference in the primary outcome: EIT consultation group had a median (interquartile range [IQR]) LOS of 7.0 days (4.0-13.0 d) compared with the usual care group median (IQR) LOS of 7.0 days (4.0-13.0 d), CONCLUSIONS: An ED-based critical care consultation model did not impact hospital LOS. This model was used in the ED and the EIT cared for critically ill patients with higher severity of illness and longer ED boarding times

Critical review of multimorbidity outcome measures suitable for low-income and middle-income country settings: perspectives from the Global Alliance for Chronic Diseases (GACD) researchers.

OBJECTIVES: There is growing recognition around the importance of multimorbidity in low-income and middle-income country (LMIC) settings, and specifically the need for pragmatic intervention studies to reduce the risk of developing multimorbidity, and of mitigating the complications and progression of multimorbidity in LMICs. One of many challenges in completing such research has been the selection of appropriate outcomes measures. A 2018 Delphi exercise to develop a core-outcome set for multimorbidity research did not specifically address the challenges of multimorbidity in LMICs where the global burden is greatest, patterns of disease often differ and health systems are frequently fragmented. We, therefore, aimed to summarise and critically review outcome measures suitable for studies investigating mitigation of multimorbidity in LMIC settings. SETTING: LMIC. PARTICIPANTS: People with multimorbidity. OUTCOME MEASURES: Identification of all outcome measures. RESULTS: We present a critical review of outcome measures across eight domains: mortality, quality of life, function, health economics, healthcare access and utilisation, treatment burden, measures of 'Healthy Living' and self-efficacy and social functioning. CONCLUSIONS: Studies in multimorbidity are necessarily diverse and thus different outcome measures will be appropriate for different study designs. Presenting the diversity of outcome measures across domains should provide a useful summary for researchers, encourage the use of multiple domains in multimorbidity research, and provoke debate and progress in the field

Measuring universal health coverage based on an index of effective coverage of health services in 204 countries and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019

Background Achieving universal health coverage (UHC) involves all people receiving the health services they need, of high quality, without experiencing financial hardship. Making progress towards UHC is a policy priority for both countries and global institutions, as highlighted by the agenda of the UN Sustainable Development Goals (SDGs) and WHO's Thirteenth General Programme of Work (GPW13). Measuring effective coverage at the health-system level is important for understanding whether health services are aligned with countries' health profiles and are of sufficient quality to produce health gains for populations of all ages. Methods Based on the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2019, we assessed UHC effective coverage for 204 countries and territories from 1990 to 2019. Drawing from a measurement framework developed through WHO's GPW13 consultation, we mapped 23 effective coverage indicators to a matrix representing health service types (eg, promotion, prevention, and treatment) and five population-age groups spanning from reproductive and newborn to older adults (≥65 years). Effective coverage indicators were based on intervention coverage or outcome-based measures such as mortality-to-incidence ratios to approximate access to quality care; outcome-based measures were transformed to values on a scale of 0–100 based on the 2·5th and 97·5th percentile of location-year values. We constructed the UHC effective coverage index by weighting each effective coverage indicator relative to its associated potential health gains, as measured by disability-adjusted life-years for each location-year and population-age group. For three tests of validity (content, known-groups, and convergent), UHC effective coverage index performance was generally better than that of other UHC service coverage indices from WHO (ie, the current metric for SDG indicator 3.8.1 on UHC service coverage), the World Bank, and GBD 2017. We quantified frontiers of UHC effective coverage performance on the basis of pooled health spending per capita, representing UHC effective coverage index levels achieved in 2019 relative to country-level government health spending, prepaid private expenditures, and development assistance for health. To assess current trajectories towards the GPW13 UHC billion target—1 billion more people benefiting from UHC by 2023—we estimated additional population equivalents with UHC effective coverage from 2018 to 2023. Findings Globally, performance on the UHC effective coverage index improved from 45·8 (95% uncertainty interval 44·2–47·5) in 1990 to 60·3 (58·7–61·9) in 2019, yet country-level UHC effective coverage in 2019 still spanned from 95 or higher in Japan and Iceland to lower than 25 in Somalia and the Central African Republic. Since 2010, sub-Saharan Africa showed accelerated gains on the UHC effective coverage index (at an average increase of 2·6% [1·9–3·3] per year up to 2019); by contrast, most other GBD super-regions had slowed rates of progress in 2010–2019 relative to 1990–2010. Many countries showed lagging performance on effective coverage indicators for non-communicable diseases relative to those for communicable diseases and maternal and child health, despite non-communicable diseases accounting for a greater proportion of potential health gains in 2019, suggesting that many health systems are not keeping pace with the rising non-communicable disease burden and associated population health needs. In 2019, the UHC effective coverage index was associated with pooled health spending per capita (r=0·79), although countries across the development spectrum had much lower UHC effective coverage than is potentially achievable relative to their health spending. Under maximum efficiency of translating health spending into UHC effective coverage performance, countries would need to reach $1398 pooled health spending per capita (US$ adjusted for purchasing power parity) in order to achieve 80 on the UHC effective coverage index. From 2018 to 2023, an estimated 388·9 million (358·6–421·3) more population equivalents would have UHC effective coverage, falling well short of the GPW13 target of 1 billion more people benefiting from UHC during this time. Current projections point to an estimated 3·1 billion (3·0–3·2) population equivalents still lacking UHC effective coverage in 2023, with nearly a third (968·1 million [903·5–1040·3]) residing in south Asia. Interpretation The present study demonstrates the utility of measuring effective coverage and its role in supporting improved health outcomes for all people—the ultimate goal of UHC and its achievement. Global ambitions to accelerate progress on UHC service coverage are increasingly unlikely unless concerted action on non-communicable diseases occurs and countries can better translate health spending into improved performance. Focusing on effective coverage and accounting for the world's evolving health needs lays the groundwork for better understanding how close—or how far—all populations are in benefiting from UHC

A Snowball Effect; From an Uncomplicated Delivery to a Rare Case of Septic Ovarian Vein Thrombophelbitis Complicated by Ureteral Obstruction and Septic Pulmonary Emboli

Learning Objective #1: Recognize septic ovarian vein thrombophelbitis SVOT as an important cause of abdominal pain in peripartum females. Learning Objective #2: Know potential complications of SVOT that may occur beyond the pelvis. CASE: A 23 years old female, G2P2 presented 11 days post normal vaginal delivery with acute onset severe lower abdominal pain and substernal chest pain. She denied any change in the postpartum vaginal discharge and physical exam was only remarkable for diffuse abdominal tenderness. Computed Tomography CT of the abdomen/Pelvis and Chest were performed. CT abdomen revealed a thrombus in the inferior vena cava extending to the right adnexa suggestive of ovarian vein thrombus, and suspicious for thrombophlebitis, in addition to severe right hydroureter secondary to external obstruction. CT chest revealed lobar segmental pulmonary embolus as well as multiple patchy nodules suspicious for septic emboli. She was initially started on Ampicillin + Sulbactam, covering against common endometritis pathogens including streptococci, gram negatives and anaerobes, despite no growth of the blood cultures. She was also anti-coagulated with unfractionated heparin. She underwent placement of percutaneous nephrostomy tube for the un-resolving unilateral hydroureter. Subsequent workup for thrombophilia was negative and the patient was discharged home on warfarin, Clindamycin and Ceftriaxone for total 6 weeks. The percutaneous nephrostomy tube was removed 8 weeks later. The patient achieved a full recovery. IMPACT: This case of complicated septic ovarian vein thrombophelbitis SVOT made me realize that high index of suspicion of the diagnosis in peripartum patients with abdominal pain. Thoughtful consideration is needed to obtain the appropriate imaging modality (CT, US with dopplers or MRI of the abdomen), as well as the appropriate anatomical site imaged (lungs or the urinary system) to diagnose complications. DISCUSSION: Septic Ovarian vein thrombophlebitis SOVT complicated by septic pulmonary emboli and ureteral obstruction is very rare with few cases reported in the literature. SOVT mostly happens in the first 10 days postpartum with an incidence of 1 in 9000 of vaginal deliveries and 1 in 800 of caesarian deliveries. Other rare causes of SOVT include pelvic inflammatory disease and endometritis, malignancy and following pelvic surgery. The etiology for developing SOVT in the peripartum is proposed to be the general hypercoagulable state of pregnancy in addition to the stasis of ovarian venous drainage postpartum. Few studies suggested prothrombotic predisposition in up to 50% of SOVT cases. The diagnosis of SOVT is achieved radiologically utilizing ultrasound with doppler, CT or MRI of the abdomen. The current management of SVOT include anticoagulation and antibiotic therapy. Complications of SOVT include extension into the renal veins and the inferior vena cava, pulmonary emboli and sepsis. Few cases of SOVT complicated by ureteral obstruction are reported

A snowball effect; From an uncomplicated delivery to a rare case of septic ovarian vein thrombophelbitis complicated by ureteral obstruction and septic pulmonary emboli.

Learning Objective #1: Recognize septic ovarian vein thrombophelbitis SVOTas animportant cause of abdominal pain in peripartum females. Learning Objective #2: Know potential complications of SVOT that may occur beyond the pelvis. CASE: A 23 years old female, G2P2 presented 11 days post normal vaginal delivery with acute onset severe lower abdominal pain and substernal chest pain. She denied any change in the postpartum vaginal discharge and physical exam was only remarkable for diffuse abdominal tenderness. Computed To-mography CTof the abdomen/Pelvis and Chest were performed. CTabdomen revealed a thrombus in the inferior vena cava extending to the right adnexa suggestive of ovarian vein thrombus, and suspicious for thrombophlebitis, in addition to severe right hydroureter secondaryto external obstruction. CTchest revealed lobar segmental pulmonary embolus as well as multiple patchy nodules suspicious for septic emboli. She was initially started on Ampicillin + Sulbactam, covering against common endometritis pathogens including streptococci, gram negatives and anaerobes, despite no growth of the blood cultures. She was also anti-coagulated with unfractionated heparin. She underwent placement of percutaneous nephrostomy tube for the un-resolving unilateral hydroureter. Subsequent workup for thrombophilia was negative and the patient was discharged home on warfarin, Clindamycin and Ceftriaxone for total 6 weeks. The percutaneous nephrostomy tube was removed 8 weeks later. The patient achived a full recovery. IMPACT: This case of complicated septic ovarian vein thrombophelbitis SVOT made me realize that high index of suspicion of the diagnosis in peripartum patients with abdominal pain. Thoughtful consideration is needed to obtain the appropriate imaging modality (CT, US with dopplers or MRI of the abdomen), as well as the appropriate anatomical site imaged (lungs or the urinary system) to diagnose complications. DISCUSSION: Septic Ovarian vein thrombophlebitis SOVT complicated by septic pulmonary emboli and ureteral obstruction is very rare with few cases reported in the literature. SOVT mostly happens in the first 10 days postpartum with an incidence of 1 in 9000 of vaginal deliveries and 1 in 800 of caesarian deliveries. Other rare causes of SOVT include pelvic inflammatory disease and endometritis, malignancy and following pelvic surgery. The etiology for developing SOVT in the peripartum is proposed to be the general hypercoagu-lable state of pregnancy in addition to the stasis of ovarian venous drainage postpartum. Few studies suggested prothrombotic predisposition in up to 50% of SOVT cases. The diagnosis of SOVT is achieved radiologically utilizing ultrasound with doppler, CTor MRI of the abdomen. The current management of SVOT include anticoagulation and antibiotic therapy. Complications of SOVT include extension into the renal veins and the inferior vena cava, pulmonary emboli and sepsis. Few cases of SOVT complicated by ureteral obstruction are reported

Novel Use of Glidescope Indirect Laryngoscopy for Insertion of a Minnesota Tube for Variceal Bleeding

BACKGROUND: With improvements in endoscopic and interventional radiologic therapies, insertion of gastroesophageal balloon tamponade catheters, commonly known as Sengstaken-Blakemore or Minnesota tubes, is a rarely performed procedure for esophageal or gastric variceal bleeding. In small hospitals or freestanding emergency departments, endoscopic or interventional radiology (IR) therapies might not be available, so patients with exsanguinating variceal bleeding must be stabilized or temporized for transport to larger hospitals. Occasionally, tamponade devices are necessary as a rescue therapy for failed endoscopic or IR therapies or can be used as definitive therapy in select cases. In addition to being rarely performed, there are multiple technical complications associated with blind insertion of tamponade catheters. DISCUSSION: We describe a novel use of indirect laryngoscopy using a Glidescope for assisting in placement of a Minnesota tube in 4 patients with exsanguinating esophageal bleeding. CONCLUSIONS: Insertion of a Minnesota tube for bleeding esophageal or gastric varices is an uncommon, technically challenging procedure that can be lifesaving, and is something emergency physicians, intensivists, and gastroenterologists should be capable of performing. Addition of indirect laryngoscopy may help to improve rapid, safe, and successful placement of these devices