Excess deaths among adults with sickle cell disease in 2020 compared to prior years.

In 2020, there was a 20% increase in excess deaths in the USA due to COVID infections but also to changes in the healthcare system due to the pandemic. We hypothesized that people living with sickle cell disease (SCD) may be vulnerable to these changes as SCD can lead to rapid decompensation. We examined all deaths of people with SCD at our center in 2020. Cause of death was determined, clinical variables, and healthcare utilization, and the presence of COVID infection, sepsis, and acute organ failure during the death event was obtained from the electronic medical record. Deaths in 2020 were compared to deaths in 2017-2019. In 2020, deaths increase 244% (22 vs 9), but acute or previous COVID infections were identified in only 36% of 2020 deaths. People who died in 2020 were more likely to have developed acute organ failure during the death event (70.6% vs 21.1%, p = 0.003) compared to prior years. They were also more likely to have a history of stroke and more frequent hematology clinic visits. Deaths in 2020 doubled compared to prior years and COVID infection could not account for all of this excess mortality. People who died in 2020 may have had more severe disease as suggested by having more clinic visits and higher rates of stroke and were more likely to develop organ failure during the death event. This demonstrates that people with SCD may be especially vulnerable to delays in care. Larger multicenter studies should be conducted to examine this further.

Two Patients With Severe COVID Pneumonia Treated With the Seraph-100 Microbind Affinity Blood Filter.

We present 2 patients with rapidly escalating oxygen requirements from severe acute respiratory syndrome coronavirus 2 infection (COVID-19) treated with the Seraph100 Microbind Affinity Blood Filter under Emergency Use Authorization from the US Federal Drug Administration. The Seraph100 is an extracorporeal hemoperfusion filter previously demonstrated to remove viral particles and pro-inflammatory cytokines from the blood. Treatment with the Seraph100 filter was associated with a rapid improvement in oxygenation and both patients were discharged from the hospital without supplemental oxygen.

Resolution of a Mobile Right Atrial Thrombus Complicating Acute Pulmonary Embolism With Low-Dose Tissue Plasminogen Activator in a Patient With Recent Craniotomy.

Right heart thrombus in transit (RHTT) is a rare, severe form of venous thromboembolism that carries a high mortality rate. The optimal treatment for RHTT has not been well established. Thrombolysis is a therapeutic modality for RHTT but carries the risk of bleeding complications including intracranial hemorrhage. Low-dose thrombolysis has been shown to be effective in treating submassive pulmonary emboli without an increased risk in bleeding complications, but it has not been studied in patients with RHTT. Here, we discuss the case of a 74-year-old male with lung cancer and recent craniotomy with metastasectomy 30 days prior to admission presenting with RHTT and bilateral pulmonary emboli (PE). He was treated successfully with low-dose thrombolysis, despite his relative contraindication to thrombolytics. To our knowledge, this is the first reported case of low-dose alteplase (tissue plasminogen activator [tPA]) used to treat an in-transit PE in the setting of recent craniotomy with metastasectomy.

Concentration of voxelotor in sickle cell disease can be estimated using electrophoresis and high-performance liquid chromatography.

OBJECTIVES: Voxelotor can increase hemoglobin levels in patients living with sickle cell disease (SCD). A clinician who is monitoring voxelotor response may want to know whole-blood voxelotor concentration, but this cannot be measured in most clinical settings. However, voxelotor has been demonstrated to cause "peak splitting" in common methods of hemoglobin measurement such as capillary zone electrophoresis (CZE) and high-performance liquid chromatography (HPLC). We hypothesized that we could use the size of the peak split to estimate the whole-blood concentration. METHODS: Blood from people with SCD was dosed with known concentrations of voxelotor, and multiparameter regression was used to derive the relationship of voxelotor concentration to the degree of peak splitting observed. To validate these equations, 21 patients started on voxelotor at 1500 mg/d had blood samples drawn at days 0, 14, 30, and 60. Samples were sent out for gold standard voxelotor concentration testing. The derived equations were then used to calculate voxelotor concentration. RESULTS: Calculated concentrations correlated strongly with measured concentrations for both CZE (R2 = 0.83, P < .001) and HPLC (R2 = 0.76, P < .001). Voxelotor concentration also had a significant effect on increases in hemoglobin (R2 = 0.40, P < .001). CONCLUSIONS: Thus, peak splitting CZE and HPLC can be used to estimate voxelotor concentration.

Pleural Uptake Patterns in F18Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) Scans Improve the Identification of Malignant Pleural Effusions.

BACKGROUND: The confirmation of malignant pleural effusions (MPE) requires an invasive procedure. Diagnosis can be difficult and may require repeated thoracentesis or biopsies. F18Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) can characterize the extent of malignant involvement in areas of increased uptake. Patterns of uptake in the pleura may be sufficient to obviate the need for further invasive procedures. METHODS: This is a retrospective review of patients with confirmed malignancy and suspected MPE. Patients who underwent diagnostic thoracentesis with cytology and contemporaneous FDG-PET were identified for analysis. Some underwent confirmatory pleural biopsy. The uptake pattern on FDG-PET underwent blinded review and was categorized based on the pattern of uptake. RESULTS: One hundred consecutive patients with confirmed malignancy, suspected MPE and corresponding FDG-PET scans were reviewed. MPE was confirmed in 70 patients with positive pleural fluid cytology or tissue pathology. Of the remaining patients, 15 had negative cytopathology, 14 had atypical cells and 1 had reactive cells. Positive uptake on FDG-PET was noted in 76 patients. The concordance of malignant histology and positive FDG-PET occurred in 58 of 76 patients (76%). Combining histologically confirmed MPE with atypical cytology, positive pleural FDG-PET uptake had a positive predictive value of 91% for MPE. An encasement pattern had a 100% PPV for malignancy. CONCLUSION: Positive FDG-PET pleural uptake represents an excellent method to identify MPE, especially in patients with an encasement pattern. This may eliminate the need for additional invasive procedures in some patients, even when initial pleural cytology is negative.

Dilated and unreactive pupils and burst-suppression on electroencephalography due to buproprion overdose.

Burst-suppression pattern on electroencephalography (EEG) occurs upon dissociation of the cortex from underlying brain structures. Unless the pattern is a physiologic consequence of administered sedatives, this electroencephalographic pattern is indicative of a poor neurologic outcome and high mortality. We report a case of a 29-year-old female thought to be brain dead based on initial physical examination and EEG findings of burst-suppression, who was later found to have supratherapeutic serum levels of bupropion. This is the second documented case of burst-suppression pattern on EEG in a patient who overdosed on bupropion. We propose that burst-suppression in the setting of bupropion toxicity may revert with drug clearance.

Taken to heart-arrhythmic potential of heart-leaf sida, a banned ephedrine alkaloid: a case report.

BACKGROUND: Ephedra and ephedrine alkaloids were commonly used in herbal supplements before being prohibited by the European Commission and US Food and Drug Administration. However, ongoing, unknowing use by consumers can lead to potential adverse cardiovascular effects, such as arrhythmias. CASE SUMMARY: A 65-year-old-man with a history of idiopathic pulmonary fibrosis status post-right single lung transplant was admitted for dizziness and resting tachycardia. Electrocardiogram showed a narrow complex, long R-P tachycardia with upright P-waves in lead V 1. An initial workup suggested an arrhythmia associated with the consumption of an herbal supplement containing heart-leaf sida, a banned botanical ephedrine alkaloid. After the supplement was discontinued, the patient's heart rate abruptly decreased without other intervention. Electrocardiogram showed a change in P-wave morphology in lead V 1 from upright to biphasic (+/-) after conversion to normal sinus rhythm. Thus, a diagnosis of atrial tachycardia originating at or near the donor right superior pulmonary vein was favoured. DISCUSSION: Atrial tachycardia can be precipitated by the proarrhythmic effects of ephedrine alkaloids, especially in patients with underlying risk factors and susceptible atrial anatomical substrate post-lung transplantation. Despite being banned by the European Union and the USA, ephedrine alkaloids continue to be used in over-the-counter herbal supplements and may go undetected by consumers. Ongoing vigilance for ephedrine alkaloids, more rigorous regulation, and active patient education can help reduce potential cardiovascular adverse events.

A Multicenter Evaluation of the Seraph 100 Microbind Affinity Blood Filter for the Treatment of Severe COVID-19.

The Seraph100 Microbind Affinity Blood Filter (Seraph 100) (ExThera Medical, Martinez, CA) is an extracorporeal therapy that can remove pathogens from blood, including severe acute respiratory syndrome coronavirus 2. The aim of this study was to evaluate safety and efficacy of Seraph 100 treatment for COVID-19. DESIGN: Retrospective cohort study. SETTING: Nine participating ICUs. PATIENTS: COVID-19 patients treated with Seraph 100 (n = 53) and control patients matched by study site (n = 53). INTERVENTION: Treatment with Seraph 100. MEASUREMENTS AND MAIN RESULTS: At baseline, there were no differences between the groups in terms of sex, race/ethnicity, body mass index, and need for mechanical ventilation. However, patients in the Seraph 100 group were younger (median age, 54 yr; interquartile range [IQR], 41-65) compared with controls (median age, 64 yr; IQR, 56-69; p = 0.009). Charlson comorbidity index scores were lower in the Seraph 100 group (2; IQR, 0-3) compared with the control group (3; IQR, 2-4; p = 0.006). Acute Physiology and Chronic Health Evaluation II scores were also lower in Seraph 100 subjects (12; IQR, 9-17) compared with controls (16; IQR, 12-21; p = 0.011). The Seraph 100 group had higher vasopressor-free days with an incidence rate ratio of 1.30 on univariate analysis. This difference was not significant after adjustment. Seraph 100-treated subjects were less likely to die compared with controls (32.1% vs 64.2%; p = 0.001), a difference that remained significant after adjustment. However, no difference in mortality was observed in a post hoc analysis utilizing an external control group. In the full cohort of 86 treated patients, there were 177 total treatments, in which only three serious adverse events were recorded. CONCLUSIONS: Although this study did not demonstrate consistently significant clinical benefit across all endpoints and comparisons, the findings suggest that broad spectrum, pathogen agnostic, blood purification can be safely deployed to meet new pathogen threats while awaiting targeted therapies and vaccines.

A Case Series of Catheter-Directed Thrombolysis With Mechanical Thrombectomy for Treating Severe Deep Vein Thrombosis.

Two cases of extensive symptomatic deep vein thrombosis without phlegmasia cerulea dolens were successfully treated with an endovascular technique that combines catheter-directed thrombolysis and mechanical thrombectomy.

Demographic and Clinical Variables Associated With 30-day Re-Intubation Following Surgical Aortic Valve Replacement.

BACKGROUND: A retrospective study was performed to evaluate factors associated with 30-d re-intubation following surgical aortic valve repair. We hypothesized a significant increase in the odds of re-intubation among patients with preoperative comorbidities. METHODS: The American College of Surgery National Surgical Quality Improvement Program database from 2007 to 2016 was used to evaluate demographic and clinical factors associated with 30-d re-intubation following surgical aortic valve repair. Multivariable logistic regression was used to report factors associated with 30-d re-intubation while controlling for various patient characteristics. RESULTS: The study population consisted of 5,766 adult subjects who underwent surgical aortic valve repair, of whom 258 (4.47%) were re-intubated within 30 d of surgery. The mean ± SD age was 69 ± 12.98 y, and 3,668 (63.6%) were male. The prevalence of diabetes mellitus, shortness of breath, poor functional status, COPD, congestive heart failure, hypertension, and bleeding disorder was higher among subjects who were re-intubated compared to those who were not (P < .05). Age, severe COPD, congestive heart failure, and bleeding disorder were associated with this outcome. CONCLUSIONS: Age, COPD, congestive heart failure, and bleeding disorder were associated with 30-d re-intubation in this surgical cohort. If surgical aortic valve repair is deemed non-emergent, patients should be optimized preoperatively and receive careful postoperative planning to reduce the risk of postoperative complications.

The Natural History of a Patient With COVID-19 Pneumonia and Silent Hypoxemia.

A patient who declined all interventions, including oxygen, and recovered highlights the importance of treating the individual instead of clinical markers and provides a time course for recovery from pneumonia and severe hypoxemia.

Active ghrelin levels across time and associations with leptin and anthropometrics in healthy ache Amerindian women of Paraguay.

Active (acylated) ghrelin is a peptide hormone secreted primarily by the stomach, positively associated with fasting, orexigenic, and promotes growth hormone secretion. It is therefore important to energy intake management. The objective of this pilot research was to (1) compare active ghrelin with previous measurements of leptin and anthropometrics; (2) assess the consistency of active ghrelin across time in this population; (3) extend our understanding of potential population variation in active ghrelin. Two serum samples separated by 10 days at the same time between meals were collected from healthy Ache women (n = 12, mean age 32.2 +/- 14.0 SD) to determine consistency over time, associations with leptin, and anthropmetric values. Mean active ghrelin was 72.9 +/- 23.0 pg/ml, highly correlated (r(2) = 0.95, P < 0.0001) between collections, and showed no paired mean differences (P < 0.18). There was no significant correlation with leptin, age, or anthropometric measures. Active ghrelin appears to be consistent over time in this population, perhaps reflecting regimented meal schedules and less interpopulation variation compared to leptin.

Nephrogenic Systemic Fibrosis in a Patient With Multiple Inflammatory Disorders.

The risk of developing nephrogenic systemic fibrosis in patients with end stage renal disease may increase with exposure to gadolinium-based contrast dyes during magnetic resonance imaging.

The Top 10 Pulmonary Pearls for Primary Care Physicians.

Pulmonary diseases are commonly encountered by primary care physicians in the outpatient setting. Despite their prevalence, many physicians may be unfamiliar with the diagnosis and appropriate management of these disorders. The evidence-based pearls in this article will help primary care physicians navigate important topics in pulmonary medicine and guide their decision to refer their patients to a pulmonary specialist.

Tension hydropneumothorax as the initial presentation of Boerhaave syndrome.

Boerhaave syndrome, a rare yet frequently fatal diagnosis, is characterized by the spontaneous transmural rupture of the esophagus. The classic presentation of Boerhaave syndrome is characterized by Mackler's triad, consisting of chest pain, vomiting, and subcutaneous emphysema. However, Boerhaave syndrome rarely presents with all the features of Mackler's triad; instead, the common presentation of Boerhaave syndrome includes chest or epigastric pain, severe retching and vomiting, dyspnea, and shock. These symptoms are typically misdiagnosed as cardiogenic in origin. Due to its atypical presentation, rarity, and mimicry of emergent conditions, diagnosis of Boerhaave syndrome is often delayed, resulting in a high mortality rate at the time of diagnosis and with a subsequent exponential increase in mortality if treatment is delayed by greater than 48 hours. Here, we report two atypical presentations of Boerhaave syndrome presenting as tension hydropneumothorax and review ten previously reported cases of Boerhaave syndrome presenting as tension hydropneumothorax. This review serves to raise clinician awareness about the expansive and elusive ways by which esophageal perforation may present, and thereby facilitate timely and potentially life-saving diagnosis.

Tracheal epithelial-myoepithelial carcinoma associated with sarcoid-like reaction: A case report.

Epithelial-myoepithelial carcinomas are rare tumors that primarily originate in the salivary glands but have also been found in the tracheobronchial tree. We report the first case of epithelial-myoepithelial carcinoma associated with sarcoidosis. A 61 year old Hispanic man presented with altered mental status and hypercalcemia. Imaging revealed diffuse intra-thoracic and intra-abdominal lymphadenopathy. A diagnostic bronchoscopy was performed where an incidental tracheal nodule was discovered and biopsied. Pathology was consistent with epithelial-myoepithelial carcinoma. Lymph node biopsy demonstrated non-caseating granulomas consistent with sarcoidosis. Patient underwent tracheal resection of the primary tumor with primary tracheal reconstruction. Hypercalcemia subsequently normalized with clinical improvement. Repeat CT imaging demonstrated complete resolution of lymphadenopathy. Our findings are suggestive of a possible paraneoplastic sarcoid-like reaction to the epithelial-myoepithelial carcinoma with associated lymphadenopathy and symptomatic hypercalcemia.

Displasia ectodérmica: presentación de un caso / Ectodermal dysplasia: a caso report

La displasia ectodérmica es una enfermedad genética muy rara, la cual se encuentra caracterizada por manifestaciones odontoestomatológicas imponentes; presenta aspectos interesantes desde el punto de vista del diagnóstico, así como la rehabilitación conjunta ortodóntica, protésica y estética. En el Servicio de Ortodoncia de la Clínica Estomatológica INPE concurrió una paciente de 9 años de edad con manifestaciones clínicas de displasia ectodérmica, principalmente anodoncia, hipoplasia de dientes anteriores y malposición dentaria, con dientes de aspecto de conos y anomalías de las cúspides, y con afectación sicológica de la paciente. Con un tratamiento conjunto de ortodoncia y del estomatólogo general integra,l se logró restituir la maloclusión, y al mismo tiempo, con el tratamiento estético, se devolvió a la paciente a la vida socialmente útil y afectiva en vísperas de cumplir los 15 años(AU)