Rapid establishment of a COVID-19 convalescent plasma program in a regional health care delivery network.

BACKGROUND: COVID-19 convalescent plasma (CCP) represents an appealing approach to the treatment of patients with infections due to SARS-CoV-2. We endeavored to quickly establish a sustainable CCP transfusion program for a regional network of health care facilities. STUDY DESIGN AND METHODS: A regional collaborative group was activated to address the issues necessary to implementing a CCP transfusion program and making the program sustainable. A wide range of health care providers including physicians (critical care, infectious disease, transfusion medicine), nurses, pharmacists, laboratorians, and information technology (IT) specialists were required to make the program a success. RESULTS: The CCP implementation team initially consisted of four members but quickly grew to a group of nearly 20 participants based on different issues related to program implementation. Overall, six major implementation "themes" were addressed: (a) registration of individual hospitals and principal investigators with a national investigational new drug research protocol; (b) collaboration with a regional blood donor center; (c) targeted recruitment of convalesced donors; (d) IT issues related to all aspects of CCP ordering, distribution, and transfusion; (e) prioritization of patients to receive CCP; and (f) evaluation of CCP products including antibody characteristics and patient response to therapy. CONCLUSION: Within 4 weeks of initiation, CCP was successfully transfused at multiple hospitals in our regional health care delivery system. A program infrastructure was established that will make this program sustainable into the future. This approach has broader implications for the success of multi-institutional programs requiring rapid implementation.

Needle in a Haystack: Acute Intermittent Porphyria, an Often-missed Differential Diagnosis of Abdominal Pain.

Acute intermittent porphyria (AIP) is a rare disease that arises due to deficiency of the biosynthetic enzyme porphobilinogen deaminase (PBGD) involved in heme synthesis. Acute attacks can present with abdominal pain and neurological symptoms, although vague in nature. Recurrent hospitalizations for idiopathic intermittent abdominal pain should warrant investigation for AIP. Posterior reversible encephalopathy (PRES) presents with visual disturbances and seizure-like activity and can be, although rarely, associated with AIP. It is noteworthy to know that antiepileptic medication used in management of PRES can in turn worsen AIP.

Metaxalone-induced Immune Hemolytic Anemia.

Drug-induced immune hemolytic anemia (DIIHA) is a relatively uncommon cause of anemia, and its diagnosis can be challenging. Although beta-lactam antimicrobial agents are often associated with DIIHA, any medication can potentially cause it. We describe a patient presenting with yellow skin discoloration and orange-colored urine after starting metaxalone for treatment of lumbosacral sprain. Laboratory studies were consistent with warm hemolytic anemia. Symptoms improved remarkably after discontinuation of metaxalone, coupled with initiation of glucocorticoids and rituximab.

Catastrophic Perforation in Streptococcus Pneumoniae Aortitis: Complications of Infective Endocarditis in the Current Antimicrobial Era.

Infectious aortitis is an uncommon but life-threatening cause of aortitis. Given the lack of specific symptoms, establishing the diagnosis is often a challenge. When it is associated with an endovascular infection, such as infective endocarditis, blood cultures may be diagnostic although often limited by low positive predictive value. Imaging studies may reveal characteristic findings, with computerized tomography angiography being the most sensitive. Management includes prompt initiation of antimicrobial therapy followed by surgical intervention, keeping in mind that operative mortality is high due to weakened arterial wall integrity. Here we describe a 25-year-old woman without relevant medical history, who presented to the hospital with subacute onset of fever, back pain and malaise, and was found to have infectious aortitis secondary to Streptococcus pneumoniae endocarditis. Despite appropriate antimicrobial coverage and surgical repair attempts, she succumbed to aortic perforation after a complicated and prolonged hospitalization.

Polypill for cardiovascular disease prevention: Systematic review and meta-analysis of randomized controlled trials.

BACKGROUND: Cardiovascular disease is the leading cause of death worldwide. Although many pharmacological agents exist, drug compliance and therapeutic goal achievement continue to be suboptimal. This meta-analysis aims to study the effectiveness of polypills in controlling blood pressure, dyslipidemia and in reducing future cardiovascular events. METHODS: We conducted a systematic search of electronic databases using pre-specified terms. Randomized clinical trials (RCT) comparing polypills (statin, antihypertensive agents, with or without aspirin) with the standard of care were included. Outcomes of interest were changes in [systolic blood pressure (SBP), diastolic blood pressure (DBP)] mmHg, [total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C)] mg/dl, cardiovascular (CVD) mortality, and major adverse cardiovascular events (MACE). RESULTS: A total of 18 RCTs with 26,483 participants were included. The population had 55% males, with a mean age of 61.8 ± 7 years, and a mean BMI of 26.7 ± 4.2 kg/m2. The mean follow-up was 15.0 ± 20 months. Compared with standard of care, polypill use was associated with a significant reduction of SBP (Mean Difference [MD] -6.39; [95%CI -9.21, -3.56] p < 0.001), DBP (MD -4.19, [95%CI -5.48, -2.89; p < 0.001], TC (MD -24.95, [95%CI -33.86, -16.04]; p < 0.001), and LDL-C (MD -27.92, [95%CI -35.39, -20.44]; p < 0.001). Polypill use was also associated with a significant reduction of CVD mortality (RR = 0.78; 95% CI (0.61, 0.99); P = 0.04) and MACE [RR = 0.76;95% CI (0.64, 0.91); P = 0.002]. CONCLUSION: This meta-analysis showed that compared to standard of care, polypill use was associated with a significant reduction of SBP, DBP, TC, LDL-C, and a significant reduction in fatal and non-fatal cardiovascular events.

Evaluation of the effectiveness of an automated sepsis predictive tool on patient outcomes.

PURPOSE: To evaluate the effectiveness of a multidisciplinary, hospital-wide program as part of an electronic sepsis alert tool. MATERIALS AND METHODS: We used data from 15 hospitals about adult patients with severe sepsis or septic shock. Nine intervention hospitals implemented an Epic sepsis prediction tool, education, and standardized order sets (six control hospitals did not). A difference-in-difference approach evaluated their effectiveness: 1) pre-implementation period (January 1, 2016-November 15, 2018) and 2) implementation period (November 16, 2018-June 30, 2019). RESULTS: Outcomes included mortality, receipt of the SEP-1 bundle of care, broad spectrum antibiotic use, ICU stay, and length of stay of 6926 patients. The difference of 6.7 percentage points between the intervention and control groups in SEP-1 bundle completion was not statistically significant (p = 0.105). The increase over time for antibiotic administration ≤1 h of time zero was not larger for hospitals in the intervention group (11.7%) compared to the control-group (7.6%, p = 0.084). Differences among hospitals in both groups were not statistically different for mortality (p = 0.174), ICU stays (p = 0.174), and length of stay (p = 0.652) from pre- to implementation period. CONCLUSIONS: The intervention to facilitate timely sepsis care did not improve patient outcomes among those with severe sepsis or septic shock.

Acute motor axonal neuropathy following SARS-CoV-2 infection in the third trimester of pregnancy.

Neurological complications following SARS-CoV-2 infections are increasingly recognized. Despite that, sequelae from SARS-CoV-2 infection in gravid women are uncommonly reported. We present a case of acute motor axonal neuropathy, a variant of Guillain-Barre syndrome, in a 26-year-old primigravida at 34 weeks' gestation. She presented with worsening diplopia, dysphagia, and lower extremity sensory and motor deficits after testing positive for SARS-CoV-2 2 weeks earlier. Due to rapidly progressive symptoms, she required endotracheal intubation and mechanical ventilation. Initially she was diagnosed with Miller Fisher syndrome, but serology and electromyography studies were consistent with acute motor axonal neuropathy. Spontaneous preterm delivery, supportive care, and intravenous immunoglobulin therapy resulted in clinical improvement. Steady recovery was achieved by a prolonged rehabilitation program.

POEMS Syndrome - A Diagnostic Dilemma with Challenging Presentation.

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.

Cardiac Sarcoma Presenting as Tamponade.

Sarcomas are a broad group of neoplasm that originate from the mesenchymal layer and represent about 1% of malignancy in the adult population. We describe a 46-year-old woman with no prior medical history who presented with worsening dyspnea. Physical examination was significant for jugular venous distension and bilateral lower extremity edema. Chest Xray showed cardiomegaly. Echocardiography showed a normal ejection fraction of 65%, pericardial effusion with tamponade physiology and three epicardial masses. Patient had pericardiocentesis performed followed by pericardial window. Biopsy of pericardial mass showed morphologic and immunophenotypic findings supportive of diagnosis of malignant soft tissue sarcoma. Though this type of cancer is rare, malignancy should be included as one of the differential diagnoses of new-onset pericardial effusion in a young patient. Early diagnosis and referral to a specialized sarcoma center for treatment is recommended.

Hugh-Stovin syndrome: the 'incomplete Behcet's disease'. A case study of a young adult with recurrent pulmonary embolism and pulmonary arterial aneurysms.

Hugh-Stovin Syndrome (HSS) is characterized by recurrent thrombophlebitis and multiple pulmonary and/or bronchial artery aneurysms indistinguishable from the cardiovascular features seen in Behcet's disease (BD). Our case describes a 30-year-old male with recurrent pulmonary embolism and bilateral pulmonary aneurysms. Autoimmune, hypercoagulable, and infectious work up were negative. Elevated inflammatory markers and absence of the typical clinical findings seen in BD led to the diagnosis of Hugh-Stovin syndrome (HSS). Immunosuppression using steroids and azathioprine led to clinical response. Anticoagulation was continued based on risk/benefit ratio.

Recurrent neutropenia and chronic diarrhea following thymectomy: the good, the bad, and the ugly.

Good syndrome (GS) is a rare paraneoplastic syndrome seen before or after diagnosis of thymoma, and its treatment, and is characterized by hypogammaglobulinemia. Rarely, pure white cell aplasia (PWCA) can also be seen which can present as recurrent neutropenia. We describe a 64-year-old man with recurrent sinus infections and previous thymectomy for stage 1 type B2 thymoma presenting with chronic diarrhea and recurrent neutropenia necessitating serial hospitalizations despite repeated antimicrobial treatment. Immunoglobulin levels, including IgM, IgA, IgD, and IgE were undetectable. Flow cytometry also showed absent B cells. Patient was initiated on immunoglobulin replacement therapy with consequent significant clinical improvement. Despite thymectomy, patients can develop thymoma-associated paraneoplastic syndromes, including GS.

Terlipressin effect on hepatorenal syndrome: Updated meta-analysis of randomized controlled trials.

BACKGROUND AND AIM: Hepatorenal syndrome (HRS) is a fatal complication of liver cirrhosis with a limited pharmacological option. Terlipressin is a vasoconstrictor that is approved in many countries but not yet in the United States. This is a meta-analysis of randomized controlled trials (RCTs) to review terlipressin effect on HRS and the safety profile. METHODS: We searched electronic databases for RCTs comparing terlipressin versus placebo in addition to albumin in patients with type 1 or 2 HRS. Primary outcome was HRS reversal. Secondary outcomes were change in serum creatinine (Cr), requirement for renal replacement therapy (RRT) at 30 days of randomization, and 90-day survival. Risk ratios (RRs) and mean differences (MD) were calculated with 95% confidence intervals (CIs) using a random-effects model. RESULTS: We identified eight RCTs with a total of 974 patients, and median follow up of 100 days. Mean age was 55 ± 10 years, 61% were males. Alcoholic liver disease represented 56%. Compared with placebo, terlipressin was associated with a significantly higher likelihood of HRS reversal (RR 2.08; 95% CI [1.51, 2.86], P < 0.001), significantly lower serum Cr (MD -0.64; 95% CI (-1.02, -0.27), P < 0.001], and a trend toward less RRT requirements (RR 0.61; 95% CI [0.36, 1.02], P = 0.06). There was no difference in survival at 90 days between groups (RR 1.09; 95% CI (0.84, 1.43), P = 0.52). Major adverse effects (AEs) were gastrointestinal cramps, discomfort, and respiratory distress. CONCLUSION: In patients with liver cirrhosis complicated by HRS, terlipressin was associated with significant HRS reversal and decrease in serum Cr. No survival benefit was detected at 90 days.

Acute diverticulitis masquerading as unilateral sciatica-like symptoms.

A 66 year-old female presented with Systemic Inflammatory Response Syndrome (SIRS), severe left thigh pain, and localized edema. Non-contrast Computed Tomography (CT) suggested the presence of air in the left thigh and no evidence of an acute intra-abdominal process. Blood cultures grew an anaerobic gram-negative microorganism identified as Bacteroides fragilis. Repeat CT imaging with intravenous (IV) contrast revealed acute diverticulitis and the presence of a retroperitoneal abscess with extension to the thigh muscle. Along with antimicrobial therapy, surgical intervention was needed. The patient required a sigmoid resection with end-colostomy which led to clinical improvement.

COVID-19 infection and its deadly cytokine storm in a young obese adult.

SARS-CoV-2 is a novel coronavirus that was initially described in Wuhan China in December 2019. In the USA (US), the person to be diagnosed with the novel Coronavirus infection (COVID) was on 19 January 2020. On 18 March 2020, a 31-year-old morbidly obese African American woman presented with severe dyspnea with associated hypoxemia, fever and bilateral interstitial pulmonary ground glass infiltrates consistent with viral pneumonitis. Nasopharyngeal PCR testing was positive for SARS-CoV-2. Despite initiation of hydroxychloroquine and azithromycin along with supplemental oxygen therapy, rapid disease progression consistent with cytokine release syndrome ensued, leading to initiation of mechanical ventilatory support. Anti-Interleukin (IL)-6 receptor monoclonal antibody (tocilizumab) was administered. Acute respiratory distress syndrome (ARDS) leads to refractory hypoxemia and demise. Severe morbid obesity as well as race may be unidentified risk factors for the development of severe Illness in patients with COVID-19.

A case of anti-VGKC antibody encephalitis and prolonged encephalopathy despite spontaneous resolution of imaging abnormalities.

Anti-voltage-gated potassium channel (anti-VGKC) antibody encephalitis is a common form of autoimmune encephalitis (AE). AE is usually associated with autoimmune diseases or paraneoplastic phenomena such as seen in small cell lung cancer. Clinical presentation can include memory impairment, seizures, and psychiatric symptoms. We report a case of a 72-year-old male with non-small lung cancer in remission who presented with erosive gastritis and acute severe encephalopathy. Anti-VGKC antibody limbic encephalitis was diagnosed. Spontaneous resolution of encephalitis-associated changes on brain Magnetic Resonance (MR) with concomitant decreased circulating antibody levels were observed despite lack of overall cognitive improvement. ABBREVIATIONS: AE: autoimmune encephalitis; AMPAR: antibody limbic encephalitis - anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis; Anti-VGKC encephalitis: anti-Voltage-gated potassium channel antibody encephalitis; CRP: c-reactive protein; CT: computed tomography; EEG: electroencephalography; ESR: erythrocyte sedimentation rate; GCS: Glasgow Coma Scale; MRImaging: Magnetic resonance imaging; NMDA-R encephalitis: Anti-N-methyl D-aspartate receptor encephalitis; PCR: polymerase chain reaction.

Acute kidney injury on chronic kidney disease: from congestive heart failure to light chain deposition disease and cast nephropathy in multiple myeloma.

Acute on chronic renal failure is a common but notably broad diagnosis. We present a 64-year-old man with a history of diastolic heart failure and chronic kidney disease, admitted for an elevated creatinine. History and physical examination were suggestive of decompensated heart failure; however, the careful interpretation of urinalysis rendered the diagnosis of multiple myeloma. On renal biopsy, the patient was found to have light chain deposition disease with cast nephropathy. Combination lesions in multiple myeloma are rare and require diligent histopathology for detection, including light microscopy, immunofluorescence and electron microscopy. These patients portray different demographics, renal manifestations, oncologic characteristics and outcomes, and hence, further studies isolating these combined lesions are warranted. Abbreviations: CKD: chronic kidney disease; CN: cast nephropathy; CT: computerized tomography; EDD: electron-dense deposits; EM: electron microscopy; IF: immunofluorescence; FLC: free light chain; LC: light chain; LCDD: light chain deposition disease; MIDD: monoclonal immunoglobulin deposition disease; MM: multiple myeloma; LM: light microscopy; NGS: nodular glomerulosclerosis; κ: kappa; λ: lambda.

Bone marrow involvement in sarcoidosis: an elusive extrapulmonary manifestation.

Sarcoidosis is a granulomatous disease with various extrapulmonary manifestations. We describe a 51-year-old African American woman with a history of cutaneous sarcoidosis admitted with bicytopenia. Suspicion for systemic sarcoidosis was established after contrast-enhanced computerized tomography of the chest, abdomen and pelvis showed a pulmonary nodule, diffuse lymphadenopathy and hepatosplenomegaly. Cytopenias in sarcoidosis, when present, may reflect bone marrow infiltration. Hence, biopsy was obtained and bone marrow sarcoidosis was diagnosed. This manifestation, in spite of ethnic and gender predilection, is rarely seen. As with other forms of sarcoidosis, treatment comprises of corticosteroids. Abbreviations: ANCA: Antineutrophil cytoplasmic antibody; BM: Bone marrow; BMS: Bone marrow sarcoidosis; CT: Computerized tomography; HIV: Human immunodeficiency virus; HLA: Human leukocyte antigen; MRI: Magnetic resonance imaging.

Invasive Group B streptococcus: multiloculated perinephric abscesses treated with percutaneous drainage.

Group B streptococcus infections (GBSI) are commonly associated with neonates and pregnant women, but may also affect nonpregnant adults. Among its spectrum of manifestations, perinephric abscess (PA) is exceedingly rare. Comorbid conditions such as diabetes mellitus (DM) and immunosuppression increase the risk of GBSI. We describe a 61-year-old Vietnamese man with compensated alcoholic cirrhosis, who presented with acute encephalopathy following subacute, progressive abdominal pain. He was afebrile and hemodynamically stable. Laboratory data were remarkable for leukocytosis, thrombocytopenia, azotemia, and pyuria. He was found to have two right-sided PA measuring 15 × 10 × 11 cm and 4.6 × 2.7 × 7.8 cm, requiring interval placement of multiple percutaneous drains. Culture from abscesses revealed beta-hemolytic Group B streptococcus (GBS). His course was complicated by contiguous spread to abdominal wall and paraspinal musculature, as well as a new diagnosis of type 2 DM. Along with drainage, a prolonged course of intravenous antimicrobial treatment led to abscess resolution. Given the rising number of unusual clinical presentations of GBSI, this bacteria should be considered as a part of the microbiological differential diagnosis of PA, especially in conditions leading to immunosuppression.

Invasive Saccharomyces cerevisiae infection: a friend turning foe?

We report a very rare case of acute pyelonephritis in a 51-year-old female with a history of chronic kidney disease (CKD) and diabetes caused by a normally benign and a well-known human commensal organism, Saccharomyces cerevisiae that is very often prescribed as a probiotic in modern medical practice. The causal role of S. cerevisiae was confirmed by its isolation in blood, urine, stool as well as vaginal swabs thus proving its virulent nature in suitable situations.