Cystic lung diseases are a group of disorders that appear similar on radiological studies on chest computed tomography. Each disorder is characterized by its own etiology, pathophysiology, course of progression and manifestation. Lymphangioleiomyomatosis (LAM) is one of the cystic lung diseases that can either be hereditary or sporadic. The sporadic form is a rare disease with no accurate prevalence reported but is believed to be less than 10 per million. LAM is associated with inappropriate activation of mammalian target of rapamycin (mTOR) signaling which regulates cellular growth. The sporadic form is almost confined to premenopausal female population and estrogen is believed to play an important role in the pathogenesis. Pregnancy and use of estrogen based oral contraceptives can aggravate symptoms of already existing LAM. Here we describe a case of LAM that was previously treated as asthma and was diagnosed after exacerbation of respiratory symptoms after pregnancy. We offer a review of the medical literature regarding the etiology, clinical course, diagnosis and treatment of LAM.
Introduction The role of obesity in cardiovascular mortality is controversial. The obesity paradox has been widely attributed to smoking in the underweight. Large-scale studies analyzing the outcomes of peripheral arterial disease (PAD) in patients with a higher body mass index (BMI) while accounting for confounders such as smoking are lacking. Method The 2016 National Inpatient Sample (NIS) was used to identify all admissions with a primary discharge diagnosis of PAD. A secondary diagnosis of obesity or elevated BMI was used to segregate the admissions into two groups. Propensity scores were calculated to match and control both groups for age, smoking, and diabetes, amongst other confounders. A multivariate logistic and linear regression analysis was performed to calculate the odds ratio for in-hospital mortality, amputation, need for intervention (angioplasty or bypass), acute kidney injury, hospital charges, and length of stay. Non-obesity-related PAD admissions were selected as the reference groups. Results Among 248,288 PAD-related admissions, 41,618 had a secondary diagnosis of obesity. After calculating propensity scores for 1-1 matching, 41,589 admissions in the PAD and obesity group were compared to a similar number of admissions in the reference population. Patients with a concomitant diagnosis of obesity had lower odds of amputation (OR=0.90, 95% CI=0.84-0.95, p<0.001), need for intervention (OR=0.66, 95% CI=0.62-0.69, p<0.0001), and in-hospital mortality (OR=0.81, 95% CI=0.74-0.87, p<0.0001). On the contrary, the odds of having acute kidney injury were higher with elevated BMI (OR=1.30, 95% CI=1.26-1.34, p<0.0001). Conclusion Despite increasing the risk of hypertension, diabetes, and hypertriglyceridemia, the obesity paradox continues to exist with a better short-term prognosis in patients with PAD. Future studies looking into the pathophysiology behind this phenomenon are needed.
Intracardiac blood cysts (ICBC) are cardiac pseudoneoplasm commonly seen in infants below two months of age. ICBC typically resolve spontaneously; however, they can sometimes persist in adults and can cause detrimental consequences. A 47-year-old female presented to our facility with complaints of chest pain and was found to have an incidental subvalvular chordal mitral apparatus echolucent mass on transthoracic echocardiogram (TTE). A stress echocardiography was performed, which revealed transient left ventricle outflow tract (LVOT) obstruction in the absence of anginal symptoms. A cardiac magnetic resonance imaging (MRI) showed no evidence of increased mass enhancement confirming the diagnosis of a benign blood cyst of mitral apparatus. The cyst was treated conservatively with carvedilol to prevent worsening of exertional LVOT obstruction. A follow-up study done at six months showed stable exertional hemodynamics. There is no general consensus while managing ICBC. In asymptomatic, non-surgical patients stress echocardiography can offer valuable information by assessing the hemodynamic implications resulting from the cyst.
Objective:Rare disease Background:Initially presumed as nonpathogenic, the bacterial genus aerococcus now includes 7 distinct virulent and avirulent species. Aerococcus urinae first isolated in 1992 is an uncommon cause of urinary tract infection (UTI) and is seen in only 0.15% to 0.8% of cases. A. urinae associated invasive bacteremia and systemic infection are extremely rare entities. Less than 50 cases of A. urinae associated with infective endocarditis (IE) have been reported in the literature, with the prevalence being 3 per 1 million. Case Report:A 59-year-old male presented to our hospital with exertional dyspnea and new-onset atrial flutter. Prior to his current admission patient was treated for A. urinae associated UTI with levofloxacin for 10 days. A transthoracic echocardiogram revealed severe aortic regurgitation with aortic valve endocarditis, which was subsequently confirmed on transesophageal echocardiogram. Blood cultures displayed gram-positive cocci in clusters, ultimately identified as A. urinae. The patient was treated with intravenous vancomycin and underwent surgical aortic valve replacement along with patch repair for underlying aortic wall ulcer. Conclusions:To the best of our knowledge, this is the first-ever reported case of A. urinae associated IE complicated by an aortic wall ulcer. Male gender, age >65 years, and preexisting urinary tract pathology have all been implicated as risk factors for aerococcus infection. A. urinae is almost always sensitive to penicillin, carbapenem, and aminoglycosides.
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