Pulmonary arterial hypertension in systemic sclerosis: a national inpatient analysis.

OBJECTIVES: We aim to study the prevalence and epidemiology of pulmonary arterial hypertension in SS, and the impact of PAH on SSc hospitalizations in the United States population. METHODS: We utilized the National Inpatient Sample (NIS) from 2016-2019 to obtain adult hospitalizations with the primary/secondary diagnosis of SSc and coexistent PAH (SSc-PAH). Epidemiological variables, mortality rates, and secondary outcomes were studied including pulmonary embolism, atrial flutter, atrial and ventricular fibrillation, pneumonia, sepsis, cardiac arrest and cardiac & renal failure, and ventilator requirement. Healthcare burden was estimated from total hospital charges (THC) and length of stay (LOS). Statistical analysis was performed on STATA 16.1, using linear and logistic regression analyses. RESULTS: Out of 126,685 adult systemic sclerosis hospitalizations, 16.89% had PAH (SSc-PAH). The SSc-PAH group had significantly more females (85.4 % vs. 83.8%) and higher mean age (64.85±13.29 vs. 62.56±14.51). More African Americans were in this group than in the control group (19.5% vs. 14.6, p-value<0.001) while Whites (61.3% vs. 65.6%, p<0.001) and Asians (18.0 % vs. 2.8%, p<0.001) were less common. Charlson comorbidity index was higher for the SSc-PAH population (3.42 vs. 2.94, p-value<0.001). SSc-PAH group had a higher adjusted odds ratio (aOR) for mortality (aOR: 1.39, p<0.001), increased LOS (6.64 vs. 6.0 days, p<0.001) increased THC ($83,813 vs. $71,016, p <0.001). For the SSc-PAH group, there were also significantly higher odds of cardiac failure (aOR 3.13), ventilator requirement (aOR 2.15), cardiac arrest (aOR 1.39), kidney failure (aOR 1.63), pulmonary embolism (aOR 1.84), atrial flutter (aOR 1.86) atrial fibrillation (aOR1.56) and pneumonia (aOR 1.22). No significant difference in ventricular fibrillation, sepsis, or respiratory failure was noted. CONCLUSION: Pulmonary arterial hypertension in SSc is associated with worse outcomes in terms of mortality and morbidity, and higher healthcare burden compared to SSc without PAH. Also, PAH disproportionately affects White, African American & Asian populations. There remains a pressing need to continue efforts for early diagnosis and management of PAH in SSc patients.

Trends in outcomes and racial disparities in adult hospitalizations for immune thrombocytopenia over a decade.

Immune thrombocytopenia (ITP) is a diagnosis of exclusion characterized by a low platelet count in patients for whom other etiologies have been ruled out. It occurs due to autoimmune-mediated platelet destruction and thrombopoietin deficiency. ITP is a rare hematologic disorder in adults, and scarce information exists on the hospitalization outcomes among these patients. To address this knowledge gap, we conducted a nationwide population-based study from 2010 to 2019 using the National Inpatient Sample. We found a trend toward an increase in the annual admissions for ITP (from 392.2 to 417.3, p = 0.07). There was a decrease in mortality exclusively for White patients over the period studied (p = 0.03), which was not seen in Black or Hispanic patients. There was an increase in total charges adjusted for inflation for all subgroups (p < 0.01). Length of stay decreased during the decade analyzed (p < 0.01) for the total population and most subgroups. The rates of epistaxis and melena increased (p < 0.01), while rates of intracranial hemorrhage and hematemesis did not change significantly. Advances have been made in the ITP management over the past decade. However, this has not resulted in a decrease in the number of hospitalizations or total healthcare charges during hospitalization. Furthermore, a decrease in mortality was observed in White patients but not in other races. Prospective studies are needed to better characterize the financial burden of the disease, as well as to investigate racial variability in access to care, disease behavior, and response to treatment.

Azacitidine-Induced Pneumonitis in a Patient With Acute Myeloid Leukemia and Hyperleukocytosis.

Chemotherapy-related toxicity is a complex aspect of oncologic care. Pulmonary toxicity, in particular, poses a significant challenge, as it can have diverse presentations and can closely mimic other common complications of cancer treatment, such as infections. Azacitidine is an agent widely employed in high-risk myelodysplastic syndrome and acute myeloid leukemia. We present a case of azacitidine-induced pneumonitis, a rare adverse effect, in a 70-year-old patient with acute myeloid leukemia (AML) and hyperleukocytosis. After discontinuation of the drug and introduction of steroids, the patient had complete resolution of symptoms, highlighting the importance of identifying and addressing chemotherapy-induced pneumonitis.

Role of t-tubule remodeling on mechanisms of abnormal calcium release during heart failure development in canine ventricle.

The goal of this work was to investigate the role of t-tubule (TT) remodeling in abnormal Ca2+ cycling in ventricular myocytes of failing dog hearts. Heart failure (HF) was induced using rapid right ventricular pacing. Extensive changes in echocardiographic parameters, including left and right ventricular dilation and systolic dysfunction, diastolic dysfunction, elevated left ventricular filling pressures, and abnormal cardiac mechanics, indicated that severe HF developed. TT loss was extensive when measured as the density of total cell volume, derived from three-dimensional confocal image analysis, and significantly increased the distances in the cell interior to closest cell membrane. Changes in Ca2+ transients indicated increases in heterogeneity of Ca2+ release along the cell length. When critical properties of Ca2+ release variability were plotted as a function of TT organization, there was a complex, nonlinear relationship between impaired calcium release and decreasing TT organization below a certain threshold of TT organization leading to increased sensitivity in Ca2+ release below a TT density threshold of 1.5%. The loss of TTs was also associated with a greater incidence of triggered Ca2+ waves during rapid pacing. Finally, virtually all of these observations were replicated by acute detubulation by formamide treatment, indicating an important role of TT remodeling in impaired Ca2+ cycling. We conclude that TT remodeling itself is a major contributor to abnormal Ca2+ cycling in HF, reducing myocardial performance. The loss of TTs is also responsible for a greater incidence of triggered Ca2+ waves that may play a role in ventricular arrhythmias arising in HF.NEW & NOTEWORTHY Three-dimensional analysis of t-tubule density showed t-tubule disruption throughout the whole myocyte in failing dog ventricle. A double-linear relationship between Ca2+ release and t-tubule density displays a steeper slope at t-tubule densities below a threshold value (∼1.5%) above which there is little effect on Ca2+ release (T-tubule reserve). T-tubule loss increases incidence of triggered Ca2+ waves. Chemically induced t-tubule disruption suggests that t-tubule loss alone is a critical component of abnormal Ca2+ cycling in heart failure.

Learning curve for the transsphenoidal endoscopic endonasal approach to pituitary tumors.

INTRODUCTION: The transsphenoidal endoscopic approach is a relatively new procedure compared to the microscopic approach in pituitary adenoma resection. The endoscopic approach has shown to significantly decrease the rate of complications, time in the operating room and hospital, and patient post-op discomfort. However, this procedure requires the surgeon to make use of different visual and tactile clues that must be developed with experience. Therefore, it is important to understand the learning curve that the surgeon must overcome to become proficient with the endoscopic approach. METHODS: Retrospective review of a single-surgeon consecutive series of 78 patients undergoing endoscopic pituitary tumor surgery from 2006 to 2012 at Rush University Medical Center has been used in this study. Patients were grouped according to an early (n = 9) and late group (n = 68) determined by a significant difference in outcomes. Our primary outcome measures were: duration of operation, CSF leak, hospital length of stay, visual field improvement, diabetes insipidus, panhypopituitarism, and subtotal resection. RESULTS: There was a significant reduction in OR time and intraoperative CSF leaks between the early and late groups. There was no difference in hospital LOS or visual field improvement between the groups. With regards to complication rates, there was no difference found for DI, panhypopituitarism, lumbar drain placement, sinusitis, or subtotal resection between the two groups. CONCLUSION: This study indicates that there may be a learning curve of approximately 9 cases before a surgical team can decrease OR times and reduce the rate of intraoperative CSF leaks for the endonasal endoscopic approach to pituitary adenoma resection.