Medicine
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Hemophagocytic lymphohistiocytosis (HLH) is a highly fatal, hyperinflammatory syndrome in adults triggered by an underlying illness in most cases. As such, suspicion of HLH dictates further investigation to identify the HLH trigger and determine treatment. HLH is clinically challenging due to diverse presentations and underlying triggers, provider unfamiliarity, and bleeding complications. ⋯ Major hemorrhage occurred in 41% of patients. On multivariable analysis the odds ratios (OR) for major hemorrhage were increased for patients with intensive care unit level care (OR 10.47, P = .005), and disseminated intravascular coagulation in the first week of admission (OR 10.53, P = .04). These data are incorporated into a framework to encourage early HLH recognition with the HScore, facilitate trigger identification, identify those at risk for hemorrhage, and minimize low-yield or erroneous testing.
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The aim of the present study was to evaluate the incidence of undiagnosed pulmonary arterial dilatation using the gated computed tomography (CT) images acquired in patients with an otherwise normal Tc-sestamibi single-photon-emission CT (SPECT)/CT myocardial perfusion study. This was a retrospective review of 200 consecutive patients (100 men, mean age 58.7 years) who underwent a myocardial perfusion Tc-sestamibi SPECT/CT study with normal perfusion and with gated CT images acquired for coronary calcium scoring. The CT images were reviewed using a previously validated mean main pulmonary artery diameter (mPAD) measurement method which has been correlated with pulmonary arterial hypertension (PAH). ⋯ Incidental pulmonary arterial dilatation (mPAD ≥ 29.5 mm) can be detected in a large number of patients with normal myocardial perfusion scintigraphy and correlates with multiple different comorbidities. The mPAD can be measured in all patients undergoing gated imaging as part of a myocardial perfusion study, and PAH may be considered as an alternative explanation for symptoms in some patients without perfusion deficits. The data to make this potential diagnosis is already being acquired and represents an opportunity to add value to the interpretations of otherwise negative myocardial perfusion studies.
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Fenestrated atrial septal defect (ASD) occlusion has been performed in patients complicated with severe pulmonary hypertension (PH). Nevertheless, the persistent interatrial residual shunting in the fenestration might increase the risk of paradoxical embolism. Percutaneous closure of fenestrated ASD occluder (ASO) has not yet been reported. ⋯ This case illustrates that percutaneous closure of the residual shunting in fenestrated ASO was feasible and safe for short- and long-term.
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Mitochondrial respiratory chain complex I deficiency is one of common mitochondrial disorders. However, the information is relatively little about the features of Chinese patients. In this study, the clinical, biological, and genetic analyses were performed in the children with respiratory chain complex I deficiency, in order to further understand the characteristics of the disease. ⋯ The prevalence of isolated complex I deficiency was higher in the patients with mtDNA mutations (79.4%) than in the patients without mtDNA mutations (54.5%). Patients with nuclear DNA mutations are more likely to develop early onset in mitochondrial respiratory chain complex I deficiency. The patients with complex I deficiency of peripheral leukocytes may be more likely to be caused by mtDNA mutation.
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The objectives are to evaluate the application of computed tomography venography (CTV) in the diagnosis of iliac vein compression syndrome (IVCS), and to assess the factors related to the incidence and development of IVCS and the recurrence of varicose veins. Imaging data of 120 patients with chronic venous disease (CVD) of the lower extremity and 68 subjects without CVD (control) were retrospectively reviewed by radiologists blinded to the groups. CTV, conventional venography, and Doppler ultrasound were compared in the diagnosis and contributing factors for IVCS were also analyzed. ⋯ CTV is accurate for the diagnosis and severity evaluation of IVCS. IVCS might be a contributing factor for varicose vein recurrence. Iliac vein stent implantation as a safe and effective interventional therapy promotes the healing of venous ulcer caused by IVCS.