A commonly misdiagnosed rare pulmonary disease: Idiopathic pleuroparenchymal fibroelastosis

Awareness among clinicians about Idiopathic pleuroparenchymal fibroelastosis (PPFE) is lacking and by the time patients are diagnosed with it, they were seen by multiple physicians and misdiagnosed multiple times. It is a rare condition that is characterized by fibrosis of the pleura and subpleural lung parenchyma, predominantly affecting the upper lobes. Most common cause of fibrosis in other processes is collagen predominant but in PPFE fibrosis is usually caused by elastic fibers. Verhoeff-Van Gieson stain from lung biopsies in patients who presented with fibrosis in the upper pleural and parenchymal areas will help in establishing the diagnosis by demonstrating the elastic fibers. We also need to rule out the possibility of other lung parenchymal conditions like usual interstitial pneumonia, nonspecific interstitial pneumonitis, pulmonary apical cap etc. We have presented a case report on PPFE to bring attention to clinicians so that patients are diagnosed early

Two patients with daptomycin induced eosinophilic pneumonia with different presentations and treatment

We present two cases of daptomycin-induced eosinophilic pneumonia with characteristic chest x ray, CT scan and bronchoalveolar lavage (BAL) findings. It is not a commonly seen complication of Daptomycin but when it happens, it could cause a diagnostic and treatment dilemma. Patients could present acutely with hypoxic respiratory failure or a less dramatic chronic presentation is also possible. Our two patients presented with acute hypoxic respiratory failure and presentation resembles that of an infectious etiology. Diagnosis is confirmed by bronchoscopy with BAL in one of them and the other was treated empirically as the clinical and radiologic presentation was typical. Treatment includes removal of the offending agent, daptomycin. Corticosteroids are used if symptoms are severe and can result in rapid clinical improvement. There is no agreed upon dose and duration of steroids and we suggest a long taper of steroids for patients who present with severe symptoms and a short course steroid for patients with milder symptoms

Pulmonary vein thrombosis secondary to tuberculosis in a non-HIV infected patient

Tuberculosis has been suggested as an independent risk factor for thromboembolism due to a hypercoagulable state induced by changes in clotting factors, protein C and vascular endothelium. Pulmonary vein thrombosis (PVT) is a rare, potentially serious and life-threatening condition that can be caused by tuberculosis. Its rare occurrence is due to a rich network of venous collateral vessels that drain the lung. PVT can also occur following lobectomy for malignancy, lung transplantation, radiofrequency catheter ablation for atrial fibrillation, sclerosing mediastinitis and following metastatic cancer, such as liposarcoma. Pulmonary vein thrombosis is difficult to diagnose clinically and requires a combination of conventional diagnostic modalities. Systemic anticoagulation, treatment of the predisposing pathology and monitoring of the thrombosis are parts of management of pulmonary vein thrombosis. We present a case of pulmonary vein thrombosis due to tuberculosis. Our patient is a 67-year-old Caucasian male with a past medical history significant for hypertension and hyperlipidemia who came to our hospital with hemoptysis, cough and shortness of breath 1-month duration. He also noticed around 20 pounds of weight loss and night sweats but had no fever. He had no history of travel outside the USA and has never been incarcerated before, but he endorsed that his son has been in Jail before but the son has never been diagnosed with TB. He quit smoking 2 months ago. He was tachycardic, tachypneic and had decreased air entry with crepitation in the right side of the posterior chest. Chest x ray showed multiple cavitary lesions in the right upper lobe area. CT scan of the chest with contrast showed pulmonary venous partial thrombosis in the right upper lobe with multiple cavitary lesions with hilar and mediastinal lymphadenopathy. Sputum AFB was positive for mycobacterium tuberculosis and was sensitive for rifampicin. Patient was treated with systemic anticoagulation and anti-tuberculosis medications. Patient’s hospital course was complicated by development of elevated liver enzymes at which time the anti-TB medications were stopped to be restarted one by one with a follow up of his liver enzymes and liver function tests. Our case shows that presentation of patients with PVT is non-specific and needs a very high index of suspicion for proper diagnosis and management to prevent further complications as it is associated with limb and life-threatening complications. It also illustrates the importance of considering PVT in a patient with tuberculosis

Treatment of Sigmoid Volvulus by Deflation Versus Surgery: A Single Site Report

Background: Although very common in developing regions like Africa, sigmoid volvulus (SV) has a much lower incidence in the West. There is a dearth of literature on the treatment of SV in countries with limited resources. This paper reports study results using a cohort of 200 SV patients in a low-income developing country. Objectives: The aim of this study was to identify the characteristics associated with length of hospital stay (LOS) in the largest sample of sigmoid volvulus patients drawn from a single site within a single year and to examine the effects of treatment complications and previous attacks of SV on LOS. Methods: Data from charts for a retrospective sample of 200 adult SV patients hospitalized in 2012 at Gondar University Hospital, Ethiopia, were statistically analyzed to determine LOS by patient characteristics and the effects of complications on LOS. Results: Among the 200 patients diagnosed with SV, the ratio of deflation to surgery was almost 2:1 ratio to surgery. The total patient days in the study were 856 days. Patients with LOS \u3e 5 days accounted for 79% of patient days. Patients with a maximum LOS of 30 days accounted for 10.5% of total patient days. The shortest LOS (1.36 days) was among patients who underwent sigmoidoscopic decompression only and had no treatment complications. Complications associated with surgery accounted for 10.6 to 17.0 LOS days. Age, gender, gangrene and previous attacks were not significantly associated with surgery. Among the study patients, there were only five cases of failed deflation followed by surgery. All but five of the patients with gangrene had surgery. Shock, stroke, abscess, sepsis and surgery significantly prolonged LOS. Patients (66%) had not experienced previous attacks. SV affected middle aged men more than the elderly men. Females had an excess relative risk (RR=1.62) for surgery but was not statistically significant due to the small number of females (n=15) in the study. Conclusion: Deflation was an effective treatment modality, while surgery was performed only when indicated and only on the critical cases. Patients who are treated with deflation may avoid surgery which can be associated with additional complications. Avoidance of surgery and complications can substantially reduce LOS with subsequent and system costs

Ammonia vs. Lactic Acid in Predicting Positivity of Microbial Culture in Sepsis: The ALPS Pilot Study

Objective: The use of serum ammonia as a novel marker for sepsis compared to lactic acid levels in intensive care unit (ICU) patients. Design and Interventions: Single arm, prospective clinical trial to collect arterial blood samples from patients with sepsis. Serial ammonia and lactic acid levels were sent every six hours for a total of three days. Measurements and results: Compare mean levels of ammonia and lactic acid in terms of diagnosing sepsis and patient outcome, including length of stay and mortality. A total of 30 patients were enrolled in the pilot study. On admission, mean ammonia level was 35.7 μmol/L and lactic acid was 3.06 mmole/L. Ammonia levels checked at the end of day 2 (ammonia 2-4) and the beginning of day 3 (ammonia 3-1) were higher in patients who had a microbial culture-proven sepsis (p-values 0.029 and 0.002, respectively) compared to those without culture-positive sepsis. Ammonia levels did predict a longer hospital stay; ammonia level of more than 40 μmol/L had a mean hospital stay of 17.6 days vs. patients with normal levels who had a mean hospital stay of 9.62 days (p-value 0.0082). Conclusion: Elevated ammonia level can be a novel biomarker for sepsis, comparable to conventional markers. Ammonia levels have a prognostic utility as elevated levels were associated with longer hospital stay

Hypoxic respiratory failure due to hyperammonemic encephalopathy induced by concurrent use of valproic acid and topiramate, a case report and review of the literature

Valproic acid (VPA) is widely used for the treatment of epilepsy, migraine, and a variety of psychiatric symptoms, including bipolar disorder, borderline personality disorder, and alcohol withdrawal. Valproate is associated with severe idiosyncratic adverse effects, the most notable being valproate-induced hyperammonemic encephalopathy (VHE). Topiramate is also a broad-spectrum anticonvulsant that is also extensively used for migraine prophylaxis, as a mood stabilizer, and for alcohol dependency. There is increased occurrence of VHE when valproate is used with other medications like phenytoin, phenobarbital, and topiramate. Our case report is on a young patient who was on valproic acid and topiramate and developed metabolic encephalopathy with hypoxic respiratory failure. We reviewed the causes and management of the hyperammonemic encephalopathy. We believe that clinicians should be aware of possible hyperammonemic encephalopathy in any patient who is taking valproic acid and presenting with impaired consciousness and cognitive decline. We also underline the importance of early recognition and high index of suspicion of encephalopathy related to hyperammonemia

Two patients with daptomycin induced eosinophilic pneumonia with different presentations and treatment

We present two cases of daptomycin-induced eosinophilic pneumonia with characteristic chest x ray, CT scan and bronchoalveolar lavage (BAL) findings. It is not a commonly seen complication of Daptomycin but when it happens, it could cause a diagnostic and treatment dilemma. Patients could present acutely with hypoxic respiratory failure or a less dramatic chronic presentation is also possible. Our two patients presented with acute hypoxic respiratory failure and presentation resembles that of an infectious etiology. Diagnosis is confirmed by bronchoscopy with BAL in one of them and the other was treated empirically as the clinical and radiologic presentation was typical. Treatment includes removal of the offending agent, daptomycin. Corticosteroids are used if symptoms are severe and can result in rapid clinical improvement. There is no agreed upon dose and duration of steroids and we suggest a long taper of steroids for patients who present with severe symptoms and a short course steroid for patients with milder symptoms

Rasburicase induced severe hemolysis and methemoglobinemia in a Caucasian patient complicated by acute renal failure and ARDS

Rasburicase is a recombinant urate-oxidase enzyme and is a very important medication for tumor lysis syndrome. Methemoglobinemia and hemolysis are known side effects of rasburicase that result from oxidative stress caused by hydrogen peroxide, a byproduct generated during the breakdown of uric acid to allantoin. Patients with G6PD deficiency have a decreased tolerance to oxidative stress and are therefore at a greater risk of hemolysis and methemoglobinemia with rasburicase. Our patient is a 56-year-old Caucasian male with a recent diagnosis of grade 2-3a non-Hodgkin's lymphoma who presented to our emergency department with shortness of breath and dark discoloration of urine. Patient was discharged 36 hours ago from our hospital after he was given a first course of R-CHOP regimen and a dose of rasburicase. On further evaluation, patient was found to have severe anemia with hemolytic picture, hyperkalemia and acute kidney injury. He also had a discrepancy of the transcutaneous saturation (75%) and the saturation in an arterial blood gas value (99%). His methemoglobin level was found to be 11.9%. We were aware that methylene blue is a contraindication in patients with G6PD deficiency but considering patient being Caucasian and low risk for it and his deteriorating respiratory condition, it was decided to offer the treatment and patient received 1 dose of methylene blue which failed to improve his methemoglobinemia. He was also given vitamin C and 8 units of packed red blood cell throughout his stay in the hospital. Patient's hospital course was complicated by ARDS needed to be on mechanical ventilation support for 4 days and acute renal failure secondary to pigment nephropathy and acute tubular necrosis which required a hemodialysis support. Even if rasburicase induced methemoglobinemia and hemolysis are not very common complications, clinicians who prescribe and follow patients should detect this serious complication early and manage it accordingly. Our case can be used as a reminder that patients should be followed closely and given the right instructions on discharge to treat these complications which are associated with severe consequences. It is also vital to assume a diagnosis of G6PD deficiency until proven otherwise in a patient who presents with rasburicase induced hemolysis and avoid administration of methylene blue even if the patient is from a low risk ethnicity for G6PD as in our patient

Insights into SARS-CoV-2 Persistence and Its Relevance

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of coronavirus disease 2019 (COVID-19), continues to wreak havoc, threatening the public health services and imposing economic collapse worldwide. Tailoring public health responses to the SARS-CoV-2 pandemic depends on understanding the mechanism of viral replication, disease pathogenesis, accurately identifying acute infections, and mapping the spreading risk of hotspots across the globe. However, effective identification and isolation of persons with asymptomatic and mild SARS-CoV-2 infections remain the major obstacles to efforts in controlling the SARS-CoV-2 spread and hence the pandemic. Understanding the mechanism of persistent viral shedding, reinfection, and the post-acute sequalae of SARS-CoV-2 infection (PASC) is crucial in our efforts to combat the pandemic and provide better care and rehabilitation to survivors. Here, we present a living literature review (January 2020 through 15 March 2021) on SARS-CoV-2 viral persistence, reinfection, and PASC. We also highlight potential areas of research to uncover putative links between viral persistence, intra-host evolution, host immune status, and protective immunity to guide and direct future basic science and clinical research priorities

Ammonia vs. Lactic Acid in Predicting Positivity of Microbial Culture in Sepsis: The ALPS Pilot Study

Objective: The use of serum ammonia as a novel marker for sepsis compared to lactic acid levels in intensive care unit (ICU) patients. Design and Interventions: Single arm, prospective clinical trial to collect arterial blood samples from patients with sepsis. Serial ammonia and lactic acid levels were sent every six hours for a total of three days. Measurements and results: Compare mean levels of ammonia and lactic acid in terms of diagnosing sepsis and patient outcome, including length of stay and mortality. A total of 30 patients were enrolled in the pilot study. On admission, mean ammonia level was 35.7 μmol/L and lactic acid was 3.06 mmole/L. Ammonia levels checked at the end of day 2 (ammonia 2-4) and the beginning of day 3 (ammonia 3-1) were higher in patients who had a microbial culture-proven sepsis (p-values 0.029 and 0.002, respectively) compared to those without culture-positive sepsis. Ammonia levels did predict a longer hospital stay; ammonia level of more than 40 μmol/L had a mean hospital stay of 17.6 days vs. patients with normal levels who had a mean hospital stay of 9.62 days (p-value 0.0082). Conclusion: Elevated ammonia level can be a novel biomarker for sepsis, comparable to conventional markers. Ammonia levels have a prognostic utility as elevated levels were associated with longer hospital stay