How does sickle cell disease affect the peri-operative outcome in patients undergoing total knee arthroplasty? A large-scale, National Inpatient Sample-based study.

INTRODUCTION: In view of the vaso-occlusive pathophysiology affecting osseous micro-circulation, sickle cell disease (SCD) is well known to present with diverse skeletal and arthritic manifestations. With prolonged life-expectancy over the past decades, there has been a progressive increase in the proportion of SCD patients requiring joint reconstructions. Owing to the paucity of evidence in the literature, the post-operative complication rates and outcome in these patients following total knee arthroplasty (TKA) are still largely unknown. METHODS: Based on the National Inpatient Sample (NIS) database (using ICD-10 CMP code), patients who underwent TKA between 2016 and 2019 were identified. The cohort were classified into two groups: A-those with SCD; and B-those without. The data on patients' demographics, co-morbidities, details regarding hospital stay including expenditure incurred, and complications were analyzed and compared. RESULTS: Overall, 558,361 patients underwent unilateral, primary TKA; among whom, 493 (0.1%) were known cases of SCD (group A). Group A included a significantly greater proportion of younger (60.14 ± 10.87 vs 66.72 ± 9.50 years; p < 0.001), male (77.3 vs 61.5%; p < 0.001); and African-American (88.2 vs 8.3%B; p < 0.001) patients, in comparison with group B. Group A patients were also at a significantly higher risk for longer duration of peri-operative hospital stay (p < 0.001), greater health-care costs incurred (p < 0.001), and greater need for alternative step-down health-care facilities (p < 0.001) following discharge. Among the SCD patients, 24.7%, 20.9% and 24.9% developed acute chest syndrome, pain crisis and splenic sequestration crisis, respectively during the peri-operative period. Group A patients had a statistically greater incidence of acute renal failure (ARF; p = 0.014), need for blood transfusion (p < 0.001) and deep vein thrombosis (DVT; p = 0.03) during the early admission period. CONCLUSION: The presence of SCD substantially lengthens the duration of hospital stay and enhances health care-associated expenditure in patients undergoing TKA. SCD patients are at significantly higher risk for systemic complications including acute chest syndrome, pain crisis, splenic sequestration crisis, acute renal failure, higher need for blood transfusions and deep venous thrombosis during the initial peri-operative period following TKA.

Eosinophilic Esophagitis: The Role of Steroids and the Dose, Duration, and Delivery of Steroid Therapy.

Eosinophilic esophagitis (EoE) is a chronic immune-mediated condition characterized by the eosinophil infiltration of the esophagus (>15 per high power field). Recently, there has been an increase in both the incidence and prevalence of the disease. The common modalities of treatment are dietary modification, proton pump inhibitors, and steroids. However, the United States Food and Drug Administration has not approved any drugs for the treatment of EoE. This review has discussed the role of steroids in the treatment of EoE, focusing on the various formulations of the drug, its dosage, drug delivery, and duration of therapy. The study also covers the common outcomes of steroid therapy and its side effects.

A Case of T-Cell Large Granulocyte Lymphocytic Leukemia in Rheumatoid Arthritis.

T-cell large granulocyte lymphocytic (TLGL) leukemia is a lymphoproliferative disorder involving clonal expansion of cytotoxic T-cells and subsequent cytopenia, most notably neutropenia, as well as splenomegaly. TLGL leukemia is commonly associated with autoimmune disorders, most commonly rheumatoid arthritis (RA). We present a case of a 54-year-old female with a past medical history of seropositive RA who was lost to follow-up and thus was not on any active treatment for RA for several years. She returned to the clinic with worsening joint pain, swelling, and stiffness involving multiple joints. Screen laboratory work revealed an absolute neutrophil count (ANC) of 0.19 K/uL, indicating severe neutropenia. This finding prompted further workup, for which our patient was ultimately diagnosed with TLGL leukemia. Proper treatment and control of inflammation in RA are important not only to preserve joint function and vitality but also to prevent rare sequela of untreated autoimmune disorders, as was the case in our patient.

Persistent Heart Failure Despite Medical Therapy Leading to a Diagnosis of Cardiac Amyloidosis.

Cardiac amyloidosis is restrictive cardiomyopathy, commonly classified as either light-chain amyloidosis (AL) or transthyretin amyloidosis (ATTR), which can be further subdivided into wild-type (systemic senile amyloidosis) and hereditary ATTR amyloidosis. Advanced-stage, silent, and clinically undiagnosed amyloidosis has a poor prognosis, with a survival rate of six months and up to five years. We present a 72-year-old female with a past medical history of heart failure, with preserved ejection fraction, atrial fibrillation, systemic lupus erythematosus (SLE), and stage 3b chronic kidney disease, who presented with persistent shortness of breath, lower extremity pitting edema, jugular venous distension, and dyspnea despite optimal medical therapy. The patient was diagnosed with preserved heart failure in the past and was on guideline-directed medical therapy for over five years with no history of cardiac disease in the family. The patient's previous echocardiogram revealed an ejection fraction of 65%. In order to determine the etiology of the patient's cardiomyopathy, she underwent cardiac magnetic resonance imaging (CMR), monoclonal gammopathy testing, and a Technetium pyrophosphate (99mTc-PYP) scintigraphy, of which the latter two were unrevealing. The CMR revealed increased wall thickness and multiple segments of midmyocardial to subendocardial late gadolinium enhancement, suggestive of infiltrative disease. Due to inconclusive testing, the patient underwent an endomyocardial biopsy and was determined to have wild-type, systemic senile amyloidosis, which held a poor prognosis. The patient was started on tafamidis, a new Food and Drug Administration (FDA)-approved therapy for systemic senile amyloidosis, and was discharged on the new medication, with frequent follow-up visits scheduled. Current treatment guidelines for cardiac amyloidosis include loop diuretics and spironolactone. Medications such as beta-blockers, angiotensin-converting enzyme inhibitors, and calcium channel blockers are not clinically effective. There are currently new medications on the horizon, such as tafamidis, which stabilizes the transthyretin tetramer and reduces the formation of amyloid. This case highlighted that patients who have persistent symptoms of heart failure, despite guideline-directed medical therapy, and without a history of genetic cardiac conditions, may also have a diagnosis of cardiac amyloidosis. Cardiac amyloidosis is often misdiagnosed or diagnosed late in the disease course; therefore, there is a need for increasing awareness of early diagnosis and treatment, including new FDA-approved medications for a better chance of survival.

A Rare Case of Splenic Infarct Secondary to Mobile Cardiac Echodensity.

Lambl's excrescences (LE) are mobile filiform lesions, mostly found on the left-sided heart valves. Histologically, they have a mesenchymal origin with a single endothelial layer. They have the potential to detach, resulting in catastrophic thromboembolic events. Their rarity often leads to them being misdiagnosed as vegetations of endocarditis with patients failing to improve on conventional therapy. A 48-year-old female with a history of hypertension presented to the Emergency Department with a one-week history of sharp left upper quadrant pain. She was vitally stable; the only lab abnormality was revealed to be a mildly elevated white cell count. CT abdomen revealed a splenic infarct involving 25% of the parenchyma. Patients had no history of abdominal trauma, coagulation disorders, exogenous estrogen use or IV drug abuse. Subsequent investigations failed to reveal any cause of hypercoagulability. An extensive cardiac workup revealed no arrhythmias, but transesophageal echocardiogram showed a mobile echo density on the ventricular side of the aortic valve attached at the coaptation zone, approximately 2.7 cm long and 0.1 cm wide, suggesting a very prominent Lambl's excrescence. In the absence of any other findings, the patient's splenic infarct was determined to be secondary to an embolic event from the aortic valve lesion. Rivaroxaban was initiated and the patient subsequently improved. Existing literature describes most LEs as being asymptomatic and discovered incidentally on echocardiograms. This case illustrates the need to develop a criterion for prompt identification of LEs and differentiating them from the vegetations of endocarditis. It also brings forth the question of prophylactic treatment of these lesions while highlighting the lack of guidelines regarding the management of embolic phenomena secondary to LE.

Patient Characteristics, Length of Stay, Cost of Care, and Complications in Super-Obese Patients Undergoing Total Hip Arthroplasty: A National Database Study.

Background: The purpose of this study was to compare postoperative complication rates in super-obese (SO) patients with a body mass index (BMI) ≥ 50 kg/m2 undergoing total hip arthroplasty (THA) versus non-super-obese (NSO) patients undergoing THA. Methods: In this retrospective study using the National Inpatient Sample (NIS) database, 1,646 cases of THA in SO (BMI ≥ 50 kg/m2) patients were reviewed. We used International Classification of Diseases (ICD)-10 codes to assess postoperative variables including length of stay, cost of care (cost of inpatient hospitalization), and medical and surgical complications among SO patients undergoing THA compared to NSO patients before being discharged. Results: A comparison of demographic variables showed there were more women in both groups and nearly 17.2% of SO patients were diabetic patients, 11.1% of SO patients were tobacco users, and 74.8% of the SO patients were whites (African American, 15.1%; Hispanic, 2.9%). The mean length of stay was 3.43 days in the SO group and 2.32 days in the NSO group, and this difference was statistically significant. The cost of care was $79,784.64 for the SO group, which was significantly higher than $66,821.75 for the NSO group. The SO group also showed higher odds of developing medical complications such as anemia (odds ratio [OR], 1.555; 95% confidence interval [CI], 1.395-1.734; p < 0.001), acute renal failure (OR, 3.375; 95% CI, 2.816-4.045; p < 0.001), pneumonia (OR, 2.319; 95% CI, 1.241-4.331; p = 0.014), and need for blood transfusion (OR, 1.596; 95% CI, 1.289-1.975; p < 0.001). The SO patients also showed a higher risk of several postoperative surgical complications such as periprosthetic fractures, infection, and wound dehiscence. Conclusions: Postoperative complication rates in SO patients were higher than those in the NSO group. Length of stay and cost of care were higher, whereas the mean age was lower for the SO group. Therefore, THA in SO patients should be undertaken only after careful consideration and preferably in a tertiary facility capable of handling all medical and surgical in-hospital complications.

Presentation of Sjogren Syndrome as Nodular Pulmonary Amyloidosis.

Sjogren syndrome is an autoimmune disorder that leads to dryness in the eyes and mouth. Nodular pulmonary amyloidosis is a localized amyloid deposition pathology commonly seen with monoclonal lymphoproliferative disorders. We present a patient who came in with dyspnea and was found to have nodular pulmonary amyloidosis on biopsy. Commonly associated lymphoproliferative pathologies were ruled out and on further workup, the patient was found to have Sjogren syndrome. This case demonstrates pulmonary nodular amyloidosis as a rare presentation of Sjogren syndrome in the setting of relatively well-controlled symptoms. Detection of pulmonary nodular amyloidosis should prompt evaluation of associated conditions such as malignancy and autoimmune disorders to guide further management.

Thrombotic Thrombocytopenic Purpura After Ad6.COV2.S Vaccination.

Thrombotic thrombocytopenic purpura (TTP) is caused by the deficiency of ADAMTS13, a von Willebrand factor cleaving protease, which results in thrombotic microangiopathy. It is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis leading to organ damage. It has an extremely high mortality rate if left untreated, making early diagnosis and treatment of the utmost importance. We report a case of TTP that developed after vaccination with Ad26.COV2.S COVID vaccine. We present a case of a 50-year-old African American female who presented with dyspnea one week after receiving the first dose of Ad26.COV2.S vaccine. Initial labs showed anemia, thrombocytopenia, and markers of intravascular hemolysis. The suspicion for thrombotic thrombocytopenic syndromes (TTS), vaccine-induced thrombotic thrombocytopenia (VITT), TTP, and Immune thrombocytopenic purpura (ITP) was high based on the history and laboratory results. Computed tomography (CT) of the chest and ultrasound of bilateral lower extremities did not show any evidence of thrombosis. The absence of thrombosis in the presence of a high PLASMIC score increased the suspicion of TTP over the other differentials. Diagnosis of TTP was confirmed when the ADAMTS13 level was low with an elevated autoantibody inhibitor level. The patient underwent treatment with corticosteroids, plasmapheresis, and rituximab with improvement in symptoms and platelet count. TTP and VITT are the possible differential diagnosis for a patient presenting with anemia, thrombocytopenia, and signs of hemolysis after vaccination with Ad26.COV2.S. It is necessary to differentiate these two clinical entities as the management varies based on the diagnosis.

Treatment of Fatty Liver Disease: The Present and the Future.

Non-alcoholic fatty liver disease (NAFLD) progressing to non-alcoholic steatohepatitis (NASH), cirrhosis, end-stage liver disease (ESRD), and hepatocellular carcinoma (HCC) is emerging as a global epidemic. Obesity, diabetes, and metabolic syndrome are some of the leading risk factors for NAFLD. The most prevalent treatment to stop the progression is aimed at dietary modification and lifestyle changes. Bariatric surgery is indicated for patients with morbid obesity with NAFLD. The progression of NAFLD to NASH and HCC can be arrested at various stages of pathogenesis by the already prevalent drugs and the emerging newer molecular and genetic targets. This review article analyzed various preclinical animal trials and clinical trials and has summarized various groups of drugs that can be life-altering in patients diagnosed with NAFLD. This study also discusses the obstacles in taking these clinical trials to bedside treatment.

Sickle Cell Disease and the Respiratory System: A Tangential Perspective to the Hematopulmonological Dilemma.

Sickle cell disease (SCD) is a genetically inherited hematological condition that predominantly affects the African-American subset of the population. It leads to the precipitation of multi-systematic manifestations throughout the course of the life of the patient leading to an increased rate of inpatient admissions and decreased quality of life. This article has reviewed some of the most common pulmonary complications of SCD with a brief overview of the clinical features and their management and has also highlighted the fatality of the complications placing a strong focus on screening, monitoring, and the treatment of the disease. The article has also discussed the management of SCD from a pulmonological perspective rather than hematological alone.

Role, Effectiveness, and Outcome of Decompressive Craniectomy for Cerebral Venous and Dural Sinus Thrombosis (CVST): Is Surgery Really an Option?

Cerebral venous and dural sinus thrombosis (CVST) is predominantly a disease of young people. It accounts for 0.5% of all strokes, and patients usually have good outcomes. However, a minority of patients may present with elevated intracranial pressure characteristics in a serious illness type and may die from brain herniation if not treated promptly. Decompressive craniectomy (DC) is the only treatment modality that can prevent death in such cases of imminent brain herniation. Unfortunately, due to the condition's rarity and ethical concerns, randomized controlled trials are not available. This review assessed the available literature on cerebral venous and dural sinus thrombosis in different age groups and decompressive craniectomy in cerebral venous and dural sinus thrombosis. It revealed that decompressive surgery is extremely effective when done early and for the correct indications with patients achieving excellent functional outcomes post-surgery. Decompressive surgery is recommended in rapidly deteriorating patients with computed tomography (CT) scan evidence of basal cisterns effacement, a mass effect from haemorrhage and/or infarction, and significant midline shift.

Intra-operative fractures in primary total knee arthroplasty - a systematic review.

BACKGROUND: One of the rare complications of primary total knee arthroplasty is intra-operative fracture. Intra-operative fracture during revision knee arthroplasty has been well-documented but there is limited literature on fractures occurring during primary knee arthroplasty. We conducted a systematic review of the literature to compare and contrast the various studies to clearly define the predisposing factors, incidence, and characteristics of the fracture itself and to arrive at a consensus on the management and prevention of intra-operative fractures during primary knee arthroplasty. METHODS: The PubMed/Medline, Cochrane, Scopus and Embase databases were searched using keywords "intra-operative fracture", "distal femoral fracture", "tibial fracture", "patella fracture" and "primary total knee arthroplasty". A total of 158 articles were retrieved and after further filtration and exclusion processing, 10 articles that evaluated intra-operative fractures in primary total knee arthroplasty were included for the review. RESULTS: The reported incidence of intra-operative fractures varied from 0.2% to 4.4%. A higher incidence in female patients with a male to female ratio of 0.4 was reported. Posterior stabilized (PS) total knee arthroplasty was associated with higher risk of intra-operative femoral fractures by many authors in this review. Timing of occurrence and location of the intra-operative fractures can vary widely, with femoral fractures occurring more commonly during bone preparation, trialing and impaction of the final implant and tibial fractures occurring during preparation for the tibial keel and impaction of the tibial component. CONCLUSIONS: Intra-operative fractures during primary total knee arthroplasty are rare with higher risk associated with osteoporosis, rheumatoid arthritis, advanced age, female gender, chronic steroid use, metabolic bone disorders, PS type of femoral implant and difficult surgical exposure of the knee joint due to severe deformities. A plethora of management options have been utilized according to surgeon preference. Standard principles of fracture fixation and arthroplasty principles should be followed to achieve stable internal fixation and any unstable fracture site should be bypassed with the utilization of stemmed components. Satisfactory radiographic and functional outcome can be expected with appropriate treatment.