Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH

Valproic Acid-Induced Hyperammonemia with Encephalopathy (VIHE): A Case Report

Valproic acid (VPA) is a wide spectrum antiepileptic medication indicated for seizure prophylaxis across the spectrum of epilepsy. Since coming into clinical use, VPA has also been recommended for the management of a variety of other pathologies, including, most notably, mood stabilization in the manic patient. VPA’s common adverse effects include gastrointestinal, influenza-like symptoms, headache, and difficulties with sleep; nonetheless, in rare instances, VPA has been noted to cause the severe and potentially lethal condition of hyperammonemia with encephalopathy (VIHE). VIHE is the result of a dose-independent increase in ammonia levels. Often the patient is asymptomatic; if symptoms reach clinical threshold, lethargy is most common, though seizures, focal neurologic deficits and even coma are possible. VIHE can occur in patients despite normal hepatic function, normal loading doses, chronic stable doses and normal free serum drug levels. Once the diagnosis is confirmed, the first approach for symptomatic patients is to discontinue VPA, start alternative mood stabilizer as indicated, and supplement hyperammonemia treatment with lactulose, carnitine or carglumic acid. Below is a case report of VIHE that developed in an adolescent girl with a history of Bipolar I Disorder who was hospitalized in our facility for stabilization of mania.  As demonstrated below, early diagnosis of VIHE is pivotal in reducing morbidity and ultimately can be life-saving

The impact of peripheral arterial disease on patients with mechanical circulatory support.

Background: Left ventricular assist devices (LVAD) are indicated as bridging or destination therapy for patients with advanced (Stage D) heart failure and reduced ejection fraction (HFrEF). Due to the clustering of the mutual risk factors, HFrEF patients have a high prevalence of peripheral arterial disease (PAD). This, along with the fact that continuous flow LVAD influence shear stress on the vasculature, can further deteriorate the PAD. Methods: We queried the National Inpatient Sample (NIS) database (2002-2014) to identify the burden of pre-existing PAD cases, its association with LVAD, in-hospital mortality, and other complications of LVAD. The adjusted odds ratio (aOR) and 95% confidence interval (CI) were calculated using the Cochran-Mantel-Haenszel test. Results: A total of 20,817 LVAD patients, comprising of 1,625 (7.8%) PAD and 19,192 (91.2%) non-PAD patients were included in the study. The odds of in-hospital mortality in PAD patients were significantly higher compared to non-PAD group (OR 1.29, CI, 1.07-1.55, P = 0.007). The PAD group had significantly higher adjusted odds as compared to non-PAD group for acute myocardial infarction (aOR 1.29; 95% CI, 1.07-1.55, P = 0.007), major bleeding requiring transfusion (aOR, 1.286; 95% CI, 1.136-1.456, P \u3c 0.001), vascular complications (aOR, 2.360; 95% CI, 1.781-3.126, P \u3c 0.001), surgical wound infections (aOR, 1.50; 95% CI, 1.17-1.94, P = 0.002), thromboembolic complications (aOR, 1.69; 95% CI, 1.36-2.10, P \u3c 0.001), implant-related complications (aOR, 1.47; 95% CI, 1.19-1.80, P \u3c 0.001), and acute renal failure (aOR, 1.26; 95% CI, 1.12-1.43, P \u3c 0.001). Conclusion: PAD patients can have high LVAD associated mortality as compared to non-PAD

Proprotein convertase subtilisin/Kexin type-9 (PCSK-9) inhibitors induced liver injury - a retrospective analysis.

Background: Proprotein convertase subtilisin/Kexin type 9 (PCSK-9) inhibitors induced liver dysfunction in patients with or without previous liver injury, and this is not well discussed in the previous literature. Methods: A total sample of 202 patients were retrospectively reviewed at the University of Missouri, Kansas City, from the year 2015 to 2018 based on predefined selection criteria. Inclusion criteria involved patients with dyslipidemia, with or without PCSK-9 inhibitors, liver function tests and lipid profile at baseline and at a mean of 6-month follow-up. The variables, including age, gender, and confounding factors like other medications (statin, oral antidiabetic, and antihypertensive) induced, or chronic secondary liver diseases causing liver injury were taken into consideration. Exclusion criteria included patients without dyslipidemia. Results: The mean age of the study population was 64 ± 11 years (63% males and 37% females). The lipid profile including triglyceride and cholesterol levels during 6-month followup visit showed a mean of 184 ± 260 and 163 ± 50 mg/dL as compared to that at baseline of 227 ± 603 and 181 ± 70 mg/dL, respectively. In terms of clinical efficacy, a 6-month follows-up showed a drop in triglyceride and cholesterol levels by 38 and 15 mg/dL, respectively. A liver function test at 6 months in patients taking PCSK-9 inhibitors showed an increase in alanine transaminase (ALT) and aspartate transaminase (AST) by 5.8 mg/dL (p = 0.037) and 6.2 mg/dL (p = 0.008), respectively, from baseline values. Conclusion: PCSK-9 inhibitors should be used cautiously with a follow-up liver function test

Carglumic Acid Treatment of a Patient with Recurrent Valproic Acid-induced Hyperammonemia: A Rare Case Report

Valproic acid, first manufactured as an anticonvulsant, is commonly used to treat both neurological and psychiatric conditions. A rare and deadly side effect of this medication is hyperammonemia, presenting as lethargy, confusion, seizure, and, ultimately, coma. In rare circumstances, hyperammonemia can be recurrent and devastating, especially in patients with an underlying N-acetyl glutamate synthase (NAGS) deficiency, as the valproic acid can enhance this enzyme deficiency and inhibit the conversion of ammonia into urea in the liver. For these subtypes of patients, the United States Food and Drug Administration (US FDA) has recently approved carglumic acid, a medication that can act as a scavenger by effectively increasing the levels of NAGS, ultimately enhancing the conversion of ammonia to urea. In our case report, we have mentioned a patient with treatment-resistant bipolar disorder, who presented with elevated ammonia levels secondary to valproic acid treatment. Valproic acid was the only drug that was effective in his case, so we initiated therapy to reduce his elevated ammonia levels. After a thorough evaluation, we found the patient had a genetic NAGS deficiency. Carglumic acid was initiated and proved efficacious in our patient

Outcomes of open mitral valve replacement versus Transcatheter mitral valve repair; insight from the National Inpatient Sample Database.

Background: Transcatheter mitral valve repair and replacement (TMVR) is a minimally invasive alternative to conventional open-heart mitral valve replacement (OMVR). The present study aims to compare the burden, demographics, cost, and complications of TMVR and OMVR. Methods: The United States National Inpatient Sample (US-NIS) for the year 2017 was queried to identify all cases of TMVR and OMVR. Categorical and continuous data were analyzed using Pearson chi-square and independent t-test analysis, respectively. An adjusted odds ratio (aOR) based on the ordinal logistic regression (OLR) model was calculated to determine the association between outcome variables. Results: Of 19,580 patients, 18,460 (94%) underwent OMVR and 1120 (6%) TMVR. Mean ages of patients were 63 ± 14 years (OMVR) and 67 ± 13 years (TMVR). Both cohorts were predominantly Caucasian (73% OMVR vs. 74.0% TMVR). The patients who underwent TMVR were more likely to belong to a household with an income in the highest quartile (26.1% vs. 22.0% for OMVR) versus the lowest quartile (22.1% vs. 27.8%). The average number of days from admission to TMVR was less compared to OMVR (2.63 days vs. 3.02 days, p = 0.015). In-hospital length of stay (LOS) was significantly lower for TMVR compared to OMVR (11.56 vs. 14.01 days, p=\u3c0.0001). Adjusted in-hospital mortality taking into account comorbidities showed no significant difference between the two groups (OR 1.2, 0.93-1.68, p = 0.15). Conclusion: Patients undergoing TMVR were older and more financially affluent. TMVR was more costly but was associated with a shorter hospital stay and similar mortality to OMVR

Investigating the Complications and Causes of Failure of the AngioVac System: A Post-Marketing Surveillance From the MAUDE Database

Background Aspiration thrombectomy devices, such as the AngioVac, allow the removal of thrombus, especially in patients with contraindications to anticoagulation use. The AngioVac was approved by the U.S. Food and Drug Administration to remove fresh, soft thrombi or emboli during extracorporeal bypass for up to six hours. Real-world data on the most common modes of failure and complications associated with the AngioVac are unavailable. Methods The Manufacturer and User Facility Device Experience database was queried for reports of the AngioVac device failure and adverse events from April 2013 to March 2022. Categorical variables were described as numbers, and all statistical calculations were performed with IBM SPSS Statistics, version 27.0 (IBM Corp., Armonk, NY). Results A total of 115 events were reported during the study period. After the exclusion of duplicate reports, the final cohort included 93 reports. The most common mode of failure for the AngioVac was physical damage of the device, with 13 reports (14%). The most common vessels associated with events were the superior vena cava and inferior vena cava, occurring in 23 reports (24.7%). The most common adverse clinical events were pulmonary embolism (PE), occurring in 33 reports (35.5%), and perforation, occurring in 16 reports (17.2%). Other less frequent adverse outcomes were arrhythmias, stroke, and foreign body device embedment. There were 45 deaths reported with the use of the AngioVac. Conclusions Aspiration thrombectomy devices provide promising efficacy; however, physicians should be aware of known adverse outcomes, even if they are infrequent. Based on this analysis, PE and vessel perforation were the most common adverse outcomes. Furthermore, the most common mode of failure was secondary to physical damage of the device

Chronic inflammatory demyelinating polyneuropathy as a paraneoplastic manifestation of colorectal carcinoma: What do we know?

The pathogenesis of chronic inflammatory demyelinating polyneuropathy (CIDP) remains highly debated among experts. In recent times, literature has divulged a riveting yet plausible association between colorectal carcinoma and CIDP as its paraneoplastic ramification. Initially, research suggested that chronic inflammatory demyelinating polyneuropathy (CIDP) was caused solely by macrophages. However, recent studies have insinuated towards an alternative pathogenesis, one involving autoantibodies against paranodal junction proteins. These two distinct mechanisms are the primary contenders responsible for the development of CIDP, rendering it an elusive paraneoplastic manifestation of colorectal carcinoma.</p

A Review of the Mechanism of Antagonism of N-methyl-D-aspartate Receptor by Ketamine in Treatment-resistant Depression

The biochemical processes involved in depression go beyond serotonin, norepinephrine, and dopamine. The N-methyl-D-aspartate (NMDA) receptor has a major role in the neurophysiology of depression. Ketamine, one of the prototypical NMDA antagonists, works rapidly in controlling depressive symptoms, including acutely suicidal behavior, by just a single injection. Ketamine may rapidly increase the glutamate levels and lead to structural neuronal changes. Increased neuronal dendritic growth may contribute to synaptogenesis and an increase in brain-derived neurotrophic factor (BDNF). Activation of the mechanistic target of rapamycin (mTOR), as well as increased levels of BDNF, may increase long-term potentiation and result in an improvement in the symptoms of depression. The mechanisms of ketamine’s proposed effect as an off-label treatment for resistant depression are outlined in this paper

As the COVID-19 pandemic drags on, where have all the STEMIs gone?

In the midst of current pandemic individuals with all sorts of medical ailments are afraid to venture into health-care settings and risk contracting Coronavirus disease 2019 (COVID-19). Irrespective of the inherent thromboembolic risks associated with COVID-19, this puts patients at high risk of cardiac complication