Full Workup Research Articles

A new prognostic score for predicting survival in patients treated with robotic stereotactic radiotherapy for brain metastases

The study aimed to analyze potential prognostic factors in patients treated with robotic radiosurgery for brain metastases irrespective of primary tumor location and create a simple prognostic score that can be used without a full diagnostic workup. A retrospective analysis of 142 patients with 1–9 brain metastases treated with stereotactic radiosurgery (1–4 fractions) was performed. Volumes of all lesions were calculated using linear dimensions of the tumors (CC, LR, AP) and 4/3*π*(CC/2)*(LR/2)*(AP/2) formula. Kaplan–Meier method and log-rank test were used to analyze survival. Variables significantly associated with overall survival in univariate analysis were included in Cox multivariate analysis. The validity of the model was tested with the bootstrap method. Variables from the final model were used to construct a new prognostic index by assigning points according to the impact of a specific variable on overall survival. In the multivariate analysis, four factors: Karnofsky Performance Status (p = 0.000068), number of brain metastases (p = 0.019), volume of the largest lesion (p = 0.0037), and presence of extracerebral metastases (p = 0.0017), were independent predictors of survival. Total scores ranged from 0 to 12 points, and patients were divided into four groups based on median survival of each subgroup: 0–1 points—18.8 months, 2–3 points—16.9 months, 4–5 points—5.6 months, and ≥ 6 points—4.9 months (p < 0.001). The new prognostic index is simple to calculate. It has a strong prognostic value in a heterogeneous population of patients with a various number of brain metastases, but its value requires confirmation in another cohort.

Ventriculoatrial Shunting: An Escape Option in Patients with Idiopathic Normal Pressure Hydrocephalus Failing Ventriculoperitoneal Drainage

Ventriculoperitoneal (VP) shunting is widely accepted as the gold-standard treatment for idiopathic normal pressure hydrocephalus (iNPH). However, a restricted group of patients experience only minimal or no improvement after the operation. In such cases, the question whether the diagnosis was incorrect or the shunt is malfunctioning remains unanswered. We retrospectively collected data on a 10-year series of VP-shunted patients with iNPH showing transient or minimal improvement of symptoms within 3weeks from surgery. A full workup (including noninvasive diagnostic, cognitive, and invasive tests) was performed. After ruling out mechanical malfunction, we performed a tap test followed by a Katzman test 2 weeks later. The confirmed persistence of disturbance of cerebrospinal fluid dynamics was treated by shunt revision and, if found working, by its replacement into the atrial cavity. Twenty patients were diagnosed with shunt insufficiency. At surgery, the distal end of the shunt was easily extruded and found working in all cases. It was then repositioned into the right atrium (the first 8 patients of the series also underwent failed contralateral abdominal replacement). Early postoperative clinical improvement was always confirmed. In 1 case, shunt overdrainage was corrected by valve upregulation. According to our experience, inadequate distal end placement of a shunt might be one of the reasons needing investigation in patients with iNPH failing improvement after surgery. In such situations, the conversion to a ventriculoatrial shunt proved to be a low-cost and successful treatment option.

Severity of upper limb discrepancies affect the postural balance in children with obsteric brachial plexus palsy (pilot study)

Ventriculoperitoneal (VP) shunting is widely accepted as the gold-standard treatment for idiopathic normal pressure hydrocephalus (iNPH). However, a restricted group of patients experience only minimal or no improvement after the operation. In such cases, the question whether the diagnosis was incorrect or the shunt is malfunctioning remains unanswered.We retrospectively collected data on a 10-year series of VP-shunted patients with iNPH showing transient or minimal improvement of symptoms within 3 weeks from surgery. A full workup (including noninvasive diagnostic, cognitive, and invasive tests) was performed. After ruling out mechanical malfunction, we performed a tap test followed by a Katzman test 2 weeks later. The confirmed persistence of disturbance of cerebrospinal fluid dynamics was treated by shunt revision and, if found working, by its replacement into the atrial cavity.Twenty patients were diagnosed with shunt insufficiency. At surgery, the distal end of the shunt was easily extruded and found working in all cases. It was then repositioned into the right atrium (the first 8 patients of the series also underwent failed contralateral abdominal replacement). Early postoperative clinical improvement was always confirmed. In 1 case, shunt overdrainage was corrected by valve upregulation.According to our experience, inadequate distal end placement of a shunt might be one of the reasons needing investigation in patients with iNPH failing improvement after surgery. In such situations, the conversion to a ventriculoatrial shunt proved to be a low-cost and successful treatment option.

PH Roundtable: Conundrums and Controversies in PH-ILD: To Treat or Not to Treat—Identifying Optimal Treatment Candidates

PH Roundtable: Conundrums and Controversies in PH-ILD: To Treat or Not to Treat—Identifying Optimal Treatment Candidates

68Ga-DOTATOC PET/CT to detect immune checkpoint inhibitor-related myocarditis

BackgroundImmune checkpoint inhibitor (ICI)-related myocarditis is a rare but potentially fatal adverse event that can occur following ICI exposure. Early diagnosis and treatment are key to improve patient outcomes. Somatostatin...

Single Center Experience with Isolated Spinal Cord Neurosarcoidosis

Isolated spinal cord neurosarcoidosis is extremely rare. The potential implications of long-term immunosuppressant therapy make correct diagnosis imperative. However, there are challenges inherent in isolated spinal cord involvement that require a multidisciplinary approach. Here we present the largest series of definite and possible isolated spinal neurosarcoidosis and discuss our institutional experience in managing this rare but morbid condition. A retrospective review was performed to identify all neurosarcoidosis cases starting from 2002 to 2020 at our institution. Patients were screened for cases of isolated spinal neurosarcoidosis. A descriptive analysis was performed for each case. A total of 64 cases of neurosarcoidosis were identified. The spine was involved in 26 (40.6%) patients. Only 4 (6.3%) cases had isolated spinal cord involvement. A full medical and imaging workup was performed in determining isolated spinal cord involvement. Three patients subsequently underwent surgical biopsy, and 1 did not undergo biopsy because of patient preference. One of the patients who underwent biopsy had an initial nondiagnostic biopsy and had a repeat biopsy. Corticosteroids were employed in all cases with additional immunosuppressive agents for maintenance therapy and refractory cases. All showed radiographic improvement and were clinically stable to improved. Isolated spinal cord involvement of neurosarcoidosis is rare and can present challenges in diagnosis. A biopsy can be performed when necessary. However, a biopsy of the spinal cord carries inherent risks and may not always be possible or result in a nondiagnostic sample. In the setting of high clinical suspicion, maximal medical therapy is still employed.

Impact of bone deformities and labral and cartilage lesions on early functional results of arthroscopic treatment of femoroacetabular impingement

IntroductionFemoroacetabular impingement (FAI) is a frequent cause of inguinal pain. Treatment failure rates range between 2.9% and 13.2%. The aim of the present study was to assess the impact of preoperative bone deformities (BD), labral lesions (LL) and cartilage lesions (CL) on clinical results of arthroscopic treatment of FAI. Material and methodA prospective operational study included patients undergoing hip arthroscopy for FAI. All patients had full radiographic work-up and clinical assessment on Non-Arthritic Hip Score (NAHS), preoperatively and at 1year. Hips with Tönnis grade>1, coxa profunda [VCE (vertical center edge angle)>35°] or borderline dysplasia (VCE<25°) were excluded. The Czerny classification was used for the labrum and the Beck classification for the cartilage. The aim of the study was to assess the impact of preoperative BD, LL and CL on clinical results of arthroscopic treatment of FAI. The study hypothesis was that type of lesion does not influence early functional results at 1year, whatever the technique used for the labrum. ResultsOne hundred and ninety-seven patients were included. Mean preoperative NAHS was 59.1±17.5. There were 145 patients with labral suture (73.6%), 42 with labral debridement (21.3%) and 10 with conservative treatment (5.1%). At 1year, mean NAHS was 88.1±15.3: i.e., a significant improvement (p<2.2×10−16). Improvement was also significant in the debridement, non-operative and suture subgroups. BD showed significant correction in the overall population (alpha angle 48.2° postoperatively versus 66.7° preoperatively; crossing sign in 14.5% versus 62.9% of cases). There were no significant differences in functional scores according to extension or type of labral or cartilage lesion. At follow-up, 3 patients (1.5%) required repeat arthroscopy. ConclusionThe present study showed that early functional results of arthroscopic treatment of FAI were unaffected by the severity of bone deformity (alpha and VCE angles), or extension or type of labral or cartilage lesion. Regardless of BD, LL and CL, 1-year clinical progression was satisfactory when all bone deformities were treated by the arthroscopic procedure. Level of evidenceIV; prospective non-comparative study.

Osteosarcoma of the maxilla presenting as a chronic pyogenic abscess: A case report.

IntroductionOsteosarcoma of the maxilla is recorded as the least common of all bone malignancies. It exhibits a clinical behavior and natural history distinct from their counterparts of the trunk and extremities. Transformation from a chronic pyogenic abscess of the maxilla is even more unusual.Case summaryA 70 year old lady presented to our hospital with a hard, fixed and tender bony swelling in her left cheek. She had initially presented to a different hospital with a similar presentation which was excised after imaging and post excision was found to be a chronic pyogenic abscess. The swelling reappeared within one year and on re-excision was found to be a low grade paraosteal osteosarcoma of the hard palate. CECT and PET-CT work-up at our hospital showed a left maxillary sinus growth with prominent neck lymph nodes along with mediastinal lymphadenopathy and pulmonary metastasis. Final histopathology revealed ulcerated stratified squamous epithelium mucosa overlying a lesion suggestive of osteosarcoma.DiscussionComplete surgical excision with negative margins continues to be the mainstay of treatment, but osteosarcomas of maxillofacial region pose difficulties in obtaining tumour free margins because of their complex anatomy around the cranium. Surgery may be complemented by radiotherapy with or without chemotherapy. Small size of the tumour and low-grade histology have been assumed to reflect a better prognosis.ConclusionOsteosarcoma of maxillofacial region has variable appearance clinically as well as radiologically posing a diagnostic challenge for clinicians. Any chronic abscess or recurrent cheek swelling thus necessitates further suspicion and requires a full work-up to rule out this high risk malignancy.

Abstract P172: Pulmonary Valve Papillary Fibroelastoma In A Patient With Chronic Chest Pain

Primary cardiac tumors are rare entity, and the most common benign cardiac neoplasms include myxomas, lipomas and papillary fibroelastomas (PFE) with PFE accounting for 8% of the tumors. A 53-year-old female presented with chronic chest pain and exertional shortness of breath. She first sought evaluation four years ago, when her pulmonologist referred her to thoracic surgery after an incidental finding of a 3.9 cm anterior mediastinal mass on CT-scan (Figure 1A-B). The patient described the pain as a constant pressure and squeezing sensation in her chest and worsening shortness of breath while laying on her sides, relieved only when a pillow was propped up under her chest. The pain did not significantly reduce her mobility or capacity to work. Initial evaluation, including transthoracic echocardiogram, stress testing, carotid ultrasound, and pulmonary function testing - was performed and found to be unremarkable. Further evaluations revealed a hiatal hernia, leading her symptoms to be attributed to GERD as causing her persistent non-exertional chest pain. The patient remained in good health despite her symptoms persisting. She later re-sought attention for chest pain on exertion and hoarseness three years later. She again underwent a full cardiopulmonary workup, yielding benign findings on EKG, troponins, and echocardiogram. Due to no alarming symptoms at the time, she was subsequently discharged. The patient continued to endorse chest pain and a follow-up MRI revealed a now 5 cm anterior mediastinal mass, thought to be a thymic cyst. Over the next few months, the patient continued to endorse worsening chest pain and exertional shortness of breath. She followed up with her cardiologist and a subsequent TEE was significant for a pulmonic valvular mass. She was admitted to the hospital for further investigation. During her hospital stay, the patient underwent surgery to resect the anterior mediastinal mass likely to be a thymic cyst and the pulmonary artery mass which was determined to be a pulmonary valve papillary fibroelastoma in the histopathology exam (Figure 2). The surgery was successful with no complications postoperatively. The patient was discharged with no further chest pain and with improvement of the exertional shortness of breath.

Balloon pulmonary angioplasty protocol in a Portuguese pulmonary hypertension expert center

IntroductionBalloon pulmonary angioplasty (BPA) has emerged as a promising therapeutic option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not eligible for pulmonary thromboendarterectomy (PEA) or who have recurrent or persistent pulmonary hypertension after surgery. There is no standardized technique for BPA and, its complexity and high risk of severe complications, requires skills and appropriate training and should be reserved for expert CTEPH centers, as a complementary intervention to medical and surgical therapy. ObjectiveThe purpose of this document is to describe the BPA protocol used at a high-volume center nationwide, validated by its results. MethodsThe present protocol includes technical details, definition of outcomes and complications, as well as patient full diagnostic work-up and treatment algorithm, before and after BPA. ResultsThe technical, hemodynamic, and clinical results of the application of this protocol will be subject of a later publication where they will be described in detail. In conclusion, we present a percutaneous intervention protocol in the treatment of pulmonary hypertension in the context of chronic pulmonary thromboembolism, validated by its clinical, hemodynamic, and technical results.

Epidemiology and Management of Acquired Hemophilia A in a Single Hospital System

Introduction: Acquired Hemophilia A (AHA) is an uncommon autoimmune disease that occurs when autoantibodies form against intrinsic factor VIII. Cases are typically identified due to isolated prolongation of the partial thromboplastin time (PTT) or unexplained bleeding diathesis, prompting further evaluation with PTT mixing studies and the Bethesda assay. AHA is historically associated with a high mortality rate; however, when appropriately diagnosed and treated, modern mortality from acute bleeding is approximately 4.5% (Knoebl et al Journal of Thrombosis and Haemostasis 2012). This study aims to explore the epidemiology, management, and outcomes in cases of AHA found within our hospital system to guide future care.Methods: The study design involves identifying all diagnosed cases of AHA within our hospital system using diagnosis codes and use of specific laboratory tests. The study design was presented to the University of Illinois College of Medicine at Peoria Internal Review Board and granted exempt status. 1,931 patients were identified, of which 6 patients met criteria for diagnosis of AHA.Results: The initial analysis reveals that the study population had a median age of 79.5 years old (high: 90; low: 61). All the patients presented with bleeding symptoms and 5 of 6 had major bleeding requiring transfusion. 5 of the 6 patients also developed soft tissue bleeding with hematoma or hemarthrosis. Underlying diagnosis of cancer was the suspected etiology in 4 of 6 cases. The median initial Bethesda assay value was 106.5 Bethesda units (high: 180; low: 33) and the median initial factor VIII activity assay result was less than 1%. The median PTT mixing study result was 51.5 seconds (high: 66.7; low: 44). In 3 of the 6 cases, patient survival was less than 1 year from onset of bleeding symptoms. 1 patient died prior to initiation of a bypassing agent. All the remaining patients were treated with the bypassing agent recombinant factor VIIA (NovoSeven ®). 2 of those 5 cases required management with a second line bypassing agent and in both cases porcine recombinant factor VIII (Obizur ®) was used and successfully achieved hemostasis. Remission with complete eradication of the acquired factor inhibitor was achieved in 4 of 5 cases receiving a bypassing agent with subsequent immunosuppressive therapy. Prednisone was used in all cases, with two using a second immunosuppressant (cyclophosphamide and rituximab). Of the 4 patients achieving remission, all eventually had recurrence of bleeding. Only 1 patient is still alive and none of the other patients survived to 5 years from initial presentation. 4 of those 5 patients died from direct complications of bleeding.Conclusions: Despite having access to electronic patient data from one of the largest hospital systems in Illinois, only 6 diagnoses were confirmed. We suspect that AHA is significantly underdiagnosed and that many patients developing factor inhibitors die before a full workup can be performed. The EACH2 registry changed how we view AHA; however, that analysis of that database must continue to be supported by population data from centers not included in that study (Knoebl et al Journal of Thrombosis and Haemostasis 2012). Furthermore, analysis of outcomes with newer bypassing agents and more sophisticated use of these agents is ongoing. We plan to publish the full results of our study, including a cost-benefit analysis of these 6 cases. We hope that our patient data can be compounded with other recent studies to help to shape future management decisions and guidelines. DisclosuresNo relevant conflicts of interest to declare.

The role of colchicine treatment on reproductive outcome in women with Familial Mediterranean Fever

Öz&#x0D; Amaç: Bu çalışma, Ailevi Akdeniz Ateşi (AAA) tanısı alan ve kolşisin tedavisi verilen kadınların üreme sonuçlarını karşılaştırmak amacıyla planlandı.&#x0D; Gereç ve Yöntem: AAA tanılı 40 infertil kadın kolşisin tedavisi alıp almadığına bakılmaksızın iki gruba ayrıldı. 29 AAA hastası düzenli kolşisin tedavisi alırken, 11 hasta kolşisin tedavisi almıyordu. Hastalara tüp bebek kliniğimize başvurmadan önce AAA tanısı kondu. Tüm hastalar, hormonal profili, semen analizi ve histerosalpingografi (HSG) dahil olmak üzere baseline kısırlık incelemesine tabi tutuldu. Katılımcılara standart antagonist protokole göre ovulasyon stimülasyonu başlandı. Çalışmamızın birincil sonuç ölçütleri, klinik gebelik yüzdesi, abortus oranları, devam eden gebelik, canlı doğum ve term doğum oranları olarak belirlendi.&#x0D; Bulgular: HSG'de üç tür problem tespit ettik; (i) peritubal adezyon (ii) periovaryan adezyon ve (iii) hidrosalpinks. HSG'deki anormal bulgular nedeniyle 11 hastaya laparoskopi yapıldı. Kolşisin grubunda 8 olguda adezyon, kolşisin verilmeyen hastalarda ise 3 olguda adezyon tespit edildi. Kolşisin grubunda 8 vakada hidrosalpinks tespit edilirken, kolşisin almayanlarda sadece 1 vakada tespit edildi. Douglas poşundaki asit her iki grupta da ultrasonografi veya laparoskopi ile tespit edildi. Kolşisin almayan hastalarda, kolşisin alan hastalara göre klinik gebelik anlamlı derecede yüksek bulundu. Düşük oranları açısından iki grup arasında anlamlı fark yoktu. Kolşisin grubunda erken doğum oranları anlamlı olarak düşük bulunurken, zamanında doğum oranları kolşisin grubunda kontrol grubuna göre anlamlı olarak yüksek bulundu.&#x0D; Sonuç: Term gebeliklerin sayısı üzerindeki olumlu etkisi dışında, kolşisin tedavisinin üreme sonuçları üzerinde herhangi bir olumsuz etkisi tespit edemedik.&#x0D; &#x0D; Abstract&#x0D; Aim: This study was planned to compare the reproductive outcome of women diagnosed with Familial Mediterranean Fever (FMF) who were given colchicine treatment and those who were not. &#x0D; Material and Method: Forty infertile women with FMF were divided into two groups whether they received colchicine treatment or not. While 29 FMF patients received regular colchicine treatment 11 patients did not receive colchicine treatment. The patients were diagnosed with FMF before applying to our IVF clinic. All patients were subjected to full infertility work-up, including hormonal profile, semen analysis and hysterosalpingography (HSG). All women with FMF were treated according to a standard antagonist protocol. The primary outcome measures were the percantage of clinical pregnancy (CPR), miscarraige rates, ongoing pregnancy, live birth and term birth rates. &#x0D; Results: We detected three types of problems in HSG; (i) peritubal adhesion (ii) periovarian adhesion and (iii) hydrosalpinx. Eleven patients underwent laparoscopy due to abnormal findings in HSG. Adhesion was detected in 8 cases in the colchicine group, while adhesion was detected in 3 cases in the patients who were not given colchicine. Hydrosalpinx was detected in 8 cases in the colchicine group, while it was detected in only one case in those who did not take colchicine. Ascites in the Douglas pouch was detected ultrasonographically or by laparoscopy in both groups. The CPR was found to be significantly higher in patients who did not receive colchicine compared to patients who received colchicine. There was no significant difference between two groups in terms of miscarraige rates. While preterm birth rates were found to be significantly lower in the colchicine group term birth rates were significantly higher in colchicine group compared to control group. &#x0D; Conclusion: Except for the positive effect on the number of term pregnancies we could not detect any negative effect of the colchicine treatment on the reproductive outcome.

A novel index using inflammatory markers improves the diagnosis of hemophagocytic lymphohistiocytosis in patients with hematologic malignancies.

7563 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome that may accompany hematologic malignancies (HM). The diagnosis of HLH in patients with HM (HM-HLH) is confounded by a number of factors: the most commonly used HLH-2004 diagnostic criteria are derived from studies in infants while the Hscore used in adults is not specific for HMs; moreover, most parameters in these scoring systems may reflect features of the underlying HM rather than HLH associated inflammation; and finally specific diagnostic cutoff values for laboratory abnormalities in HM-HLH have not been defined. We therefore conducted a study to optimize the HLH-2004 laboratory thresholds for the diagnosis of HM-HLH. Methods: A multi-center retrospective study in adult patients with HM in whom testing for HLH was performed. HM-HLH was defined as fulfillment of 5/8 HLH-2004 diagnostic criteria. We established the optimal diagnostic cutoff levels for HLH-2004 laboratory parameters using receiver operating curves (ROC) and combined the best performing parameters into a combined index, using binary logistic regression. We then created a clinical decision tree using a Classification and Regression Tree (CART) analysis with all available parameters, using cross validation. We also determined the prognostic value of our combined diagnostic tool. Results: 225 adults were analyzed (112 with HM-HLH per HLH-2004 and 113 with HM only). 35% of patients were evaluated for HLH routinely upon HM diagnosis. Soluble CD25 (sCD25) and ferritin best discriminated HM-HLH from HM, with an area under the curve (AUC) of 0.83 for each. ROC analysis demonstrated an optimal cutoff of &gt; 4190 U/mL for sCD25 (sensitivity/specificity 91%/69%) and an optimal cutoff of &gt; 2636 ng/ml for ferritin (sensitivity/specificity 64%/86%) for HM-HLH. We term the combination of elevated sCD25 and ferritin using optimized cutoff levels the ‘optimized HLH inflammatory’ (OHI) index. This OHI index was highly specific for the diagnosis of HM-HLH (specificity of 92%, sensitivity 79%). CART analysis demonstrated that OHI index positivity was sufficient to diagnose HM-HLH. In patients without a positive OHI index an Hscore &gt; 168 and either splenomegaly or triglycerides &gt; 279 ng/dL can still diagnose HM-HLH. By following this decision pathway, approximately 92% of patients were accurately classified based on HLH-2004. Furthermore, the OHI was better (odds ratio (OR) 7.9; 95% confidence interval (CI) 4.2-14.6) than Hscore &gt;169 (OR 5.5; CI 3.9-9.6) and &gt; 5/8 HLH-2004 (OR 5.3; CI 3-9.3) at predicting mortality at 1 year. Conclusions: The OHI index derived here is a simple tool that can accurately diagnose HLH and predict mortality in patients with hematologic malignancies. Some patients may not need full HLH workup before intervening with therapy that is HLH directed and not only malignancy directed.

Internal Jugular Vein Thrombosis: Atypical Presentation of COVID-19

Background: COVID-19 is associated with elevated inflammatory markers and a hypercoagulability state leading to venous thromboembolism. We present an atypical presentation of a young female with Internal Jugular Vein (IJV) thrombosis, a cause attributable to the disease. Case: A 47-year-old female with a past medical history of diabetes mellitus, hypertension, bronchial asthma, morbid obesity, uterine cancer and iron deficiency anemia (IDA) presented to the hospital with neck swelling and pain on the right side for the last week. The patient had a low-grade fever of 100.3 degrees F at home with neck discomfort, productive cough and sore throat. She was seen in the Emergency Department about 1 week back for cough and was discharged. A computerized tomography (CT) scan of the neck and soft tissue with contrast showed the right IJV almost entirely thrombosed from its origin at the skull base to the right subclavian vein and mild pulmonary edema suspected by diffuse ground glass opacity within the lungs. The patient was receiving intravenous iron therapy for IDA through a porta-catheter, placed 7 months ago. The right central vein subcutaneous porta-catheter access was removed, begun on systemic anticoagulation with Warfarin. SARS-CoV-2 RT-PCR was not done in this visit. On follow up, Anti-SARS-CoV-2 antibodies were positive. A repeat CT scan of the neck after 6 months of anticoagulation was done, and the thrombus was resolved. Conclusion: We would like to emphasize more about the hypercoagulability in SARS-CoV-2 patients and broaden our vision for all possibilities of thrombosis, like the IJV thrombosis in our patient. We recommend a full hypercoagulability workup and imaging for prompt diagnosis and management of thromboembolic events in SARS-CoV-2 patients. There should be a low threshold for the SARS-CoV-2 PCR test and a vigilant attitude while we discover more of the disease.

Assessing the risk of venous thromboembolism (VTE) in ambulatory patients with cancer: Rationale and implementation of a pharmacist-led VTE risk assessment program in an ambulatory cancer centre.

ObjectivesThe objectives of this paper were to identify and compare clinical prediction models used to assess the risk of venous thromboembolism (VTE) in ambulatory patients with cancer, as well as review the rationale and implementation of a pharmacist-led VTE screening program using the Khorana Risk Score model in an ambulatory oncology centre in Sault Ste. Marie, Ontario, Canada.Data SourcesPubMed was used to identify clinical practice guidelines and review articles discussing risk prediction models used to assess VTE risk in ambulatory patients with cancer.Data SummaryThree commonly used VTE risk prediction models in ambulatory patients with cancer: the Khorana Risk Score, Vienna Cancer and Thrombosis Study (CATS) and Protecht Score, were identified via literature review. After considering guideline recommendations, site-specific factors (i.e. laboratory costs, time pharmacists spent calculating VTE risk) and evidence from the CASSINI and AVERT trials, a novel pharmacist-led VTE risk assessment program using the Khorana Risk Score was developed during a fourth-year PharmD clinical rotation at the Algoma District Cancer Program (ADCP) [ambulatory cancer care centre]. ADCP patients with a Khorana Risk Score of were referred to the hematologist for a full VTE workup. Considering limitations, inclusion and exclusion criteria of the CASSINI and AVERT trials, the hematologist and pharmacy team decided on appropriate initiation of thromboprophylaxis with a direct oral anticoagulant (DOAC).ConclusionsThe Khorana Risk Score was the chosen model used for the pharmacist-led VTE risk assessment program due to its user-friendly scoring algorithm, evidence from validation studies and clinical trials, as well as ease of integration into pharmacy workflow. More research is needed to determine if pharmacist-led VTE risk assessment programs will impact patient outcomes, such as morbidity and mortality, secondary to cancer-associated thrombosis.

Pembrolizumab Induced-Encephalitis in An Elderly Patient with Nasal Angiosarcoma: A Case Report

Introduction New immunotherapies are increasingly being used as treatment for different types of malignancies. Immunotherapeutic agents such as immune checkpoint inhibitors (ex. pembrolizumab) have been associated with various side effects, including encephalitis. Pembrolizumab, a monoclonal antibody which mechanism of action consists of activity against programmed cell death antigen (PD-1) has been associated with encephalitis. Methods We present the case of a 76-year-old man with past medical history of angiosarcoma of the nose that was treated with radiation, chemotherapy with Taxol. Due to the patient's high tumor mutation burden, he began treatment with pembrolizumab. After six months of treatment with pembrolizumab, he presented to the emergency department with acute changes in mentation. The patient was confused and wanted to go to vote in the middle of the night. He was waking up every 30 minutes and exhibited disorganized thought process and visual hallucinations. Full delirium work-up was done without any major findings. The following tests were in the normal range or negative: thyroid function tests, prolactin, LH, cosytropin stimulation test, ACTH, UA, CBC and CMP. Brain MRI excluded evidence of acute intracranial findings, acute ischemia or brain metastases. Psychiatry was consulted. Pembrolizumab was stopped. Recommendation was made to start aripiprazole 5mg daily. Patient improved after implementing these measures. Results We postulate that the patient developed encephalitis triggered by pembrolizumab in the context of angiosarcoma of the nose. Autoimmune encephalitis is a rare adverse effect of immune checkpoint inhibitors but it may be severe and potentially lethal. The etiology is not known and diagnosis can be challenging. We postulate that the patient developed encephalitis triggered by pembrolizumab in the context of angiosarcoma of the nose. Conclusions This case highlights the need of psychiatrists to understand the behavioral symptoms that could be attributed to immunotherapy agents in patient undergoing cancer treatment. Early identification and treatment of the behavioral symptoms are important in order to evaluate and manage appropriately, especially in the elderly population. It is important to consider autoimmune encephalitis secondary to immunotherapy as a differential for psychiatric disorders. Funding No funding

Neonates and Young Infants With COVID-19 Presented With Sepsis-Like Syndrome: A Retrospective Case Controlled Study.

Objective: We aimed to describe the presentations and biochemical characteristics of sepsis-like syndrome (SLS) in infants aged <2 months who tested positive for SARS-CoV-2-in comparison to those in the same age group who were SARS-CoV-2-negative.Background: COVID-19 presents with a spectrum of manifestations, and children seem to have a favorable clinical course compared to other age groups. Limited data are available for symptomatic infants.Design: This was a case-controlled single-institution retrospective study on infants aged <2 months admitted with SLS between 1 April 2020 and 1 July 2020. These infants were divided into 2 groups: Group 1 (n = 41), infants with positive nasal/oropharyngeal swab polymerase chain reaction (PCR) results for SARS-CoV-2; and Group 2 (n = 40), infants with negative PCR results for SARS-CoV-2 (control group). Details between both groups were reviewed and analyzed.Outcome: The clinical and laboratory data for SARS-CoV-2 -positive infants who presented with SLS may differ from those for infants with SLS who tested negative for SARS-CoV-2.Results: Overall, 105 infants were admitted with clinical sepsis: 41 were SARS-CoV-2-positive, and 64 were negative. Fever was present in 90% of SARS-CoV-2-positive infants vs. 80% of the negative group. SARS-CoV-2-positive infants had a higher incidence of nasal congestion and cough (39 and 29%, respectively) compared to the SARS-CoV-2-negative group (20 and 3%, respectively) (P < 0.05). Poor feeding and hypoactivity occurred more frequently in the SARS-CoV-2-negative group (58 and 45%, respectively) than in the SARS-CoV-2-positive group (22 and 12%, respectively, P < 0.004). Sepsis workup, including lumbar puncture, was performed in 67% and partial septic workup was performed in 23% of the SARS-CoV-2-positive infants. Full sepsis workup was performed in 92% of the SARS-CoV-2-negative group. Cerebrospinal fluid (CSF) cultures were negative in 26/27SARS-CoV-2-positive infants (an infant had Klebsiella meningitis). All the SARS-CoV-2-negative infants had negative CSF cultures. Blood culture was negative in both groups. Urine culture showed bacterial growth in 9 infants with SARS-CoV-2-negative sepsis.Conclusions: Our study showed that respiratory symptoms (cough and nasal congestion) were more prominent in the SARS-CoV-2-positive group, while poor feeding and hypoactivity were reported more frequently in the negative group. However, the clinical differentiation between COVID-19 disease and sepsis in such age groups is difficult. Therefore, screening young infants with SLS for SARS-CoV-2- is necessary during this pandemic.

Inborn Error of Immunity or Atopic Dermatitis: When to be Concerned and How to Investigate

Around 20% of all children worldwide suffer from atopic dermatitis. Therefore, eczematous skin lesions and elevated serum immunoglobulin E (IgE) levels are common findings. Inborn errors of immunity (IEI) may be missed in the context of atopic dermatitis, and management and prognosis of these conditions can be substantially different. Children suffering from IEIs such as hyper-IgE syndromes, Wiskott-Aldrich syndrome, immunodysregulation polyendocrinopathy enteropathy X-linked syndrome, Omenn syndrome, the atypical complete DiGeorge syndrome, and skin barrier disorders like Comèl-Netherton syndrome and severe dermatitis-multiple allergies and metabolic wasting syndrome may present with additional red flags, which should raise a clinical suspicion for an underlying IEI. These red flags may include eczematous skin lesion manifesting prior to two months of life, disseminated or recurrent viral, bacterial, or fungal infections, mucocutaneous candidiasis, purpura, chronic diarrhea, or abnormalities in development or of connective tissue. A differential blood count, as well as a lymphocyte subset analysis, total immunoglobulin levels, and vaccination titers can help the clinician to decide whether a patient with eczematous skin lesions and elevated serum IgE should be referred to a clinical immunologist for a full immunological work-up and broad genetic analysis.

Treatment of Hepatitis C with Glecaprevir/Pibrentasvir in a Patient with Concurrent Stricturing Crohn’s Disease on Adalimumab

A 22-year-old male with long standing, active Crohn’s disease on Adalimumab had presented with increasing levels of his transaminases. A full workup was conducted and the patient was found to have hepatitis C (HCV) based on a positive HCV antibody, polymerase chain reaction (PCR) and genotyping. He was started on a regimen of Glecaprevir/Pibrentasvir with excellent response defined by complete normalization of his transaminitis and an undetectable PCR at the end of 8 weeks of treatment and achieved sustained viral response at 12 weeks of treatment. This is the first case reporting the use of a combination of Glecaprevir/Pibrentasvir and Adalimumab in a patient with HCV and Crohn’s disease. Key words: Hepatitis C, Glecaprevir/Pibrentasvir, Crohn’s Disease

The yield of CSF molecular testing in febrile neonates.

We retrospectively examined the yield of a cerebrospinal fluid (CSF) multiplex real-time PCR assay of febrile young infants undergoing a full sepsis work-up. Eighty infants were included in the study: Forty-nine (61%) neonates and 31 (39%) 29-90day-old patients were included in the study. A viral pathogen was detected in 59% (47/80) of the samples, human enterovirus in 53% (42/80) and Human parechovirus in 6% (5/80). The CSF of nearly half of the subjects with CNS infection was without pleocytosis; all CSF cultures were negative. Multiplex PCR CSF testing enhances the diagnosis of pathogen-specific viral CNS infection among febrile young infants.