PXE-PDE is a rare clinicopathological entity with few cases reported. It affects more often elderly women and is characterized by asymptomatic bilateral and symmetrical yellowish papules localized predominantly on the neck and supraclavicular regions. It is clinically similar to Pseudoxanthoma Elasticum.
The authors report a case of a 64-year-old woman presenting asymptomatic, yellowish, non-follicular papules, affecting the occipital and the posterior region of the neck for 1 year. The patient denied pruritic or inflammatory changes, marked solar exposition or trauma on the affected areas. Routine laboratory studies: thoracic x-ray and ophthalmologic examination were normal.
The histopathologic examination of a biopsy of one of the cutaneous lesions showed an absence of elastic fibers in the papillary dermis.The diagnosis of Pseudoxanthoma Elasticum—like Papillary Dermal Elastolysis (PXE-PDE) was made.
Of great importance is the differential diagnosis with Pseudoxanthoma elasticum (PXE), but we have also to consider other elastolytic disorders: mid-dermal elastolysis (MDE), linear focal elastosis (LFE) and white fibrous papulosis of the neck (WFPN).
Until know, there is no effective treatment for this pathology.
Complete atrioventricular block conferred a worse outcome during hospitalisation, including a higher incidence of cardiogenic shock, ventricular arrhythmias and death.
Wellens' syndrome is characterized by an electrocardiographic pattern of T-wave changes associated with a critical stenosis of the left anterior descending artery (LAD), which progresses to an extensive anterior myocardial infarction in the majority of cases. For this reason, its recognition and early treatment are extremely important. We report 2 cases of Wellens' syndrome: an 83-year-old male presenting with ill-characterized chest pain, biphasic T waves in V1-V3 during an asymptomatic period, negative cardiac biomarkers, and a 64% stenosis in LAD with a fractional flow reserve of 0.96 who fared well on medical therapy, and an 67-year-old male with typical angina pain, biphasic T waves in V2-V4 during asymptomatic periods, anterior ST-segment elevation at 2 min of effort during an exercise stress test, positive high-sensitivity cardiac troponin, and an 80% stenosis in the proximal LAD who was submitted to percutaneous coronary intervention which rendered him asymptomatic. The spectrum of Wellens' syndrome is very wide, and knowledge and high clinical suspicion for its diagnosis, especially in its rarest presentation of biphasic T waves, is key to avoiding catastrophic consequences.
Introduction:A growing body of evidence shows the prognostic value of oxygen uptake effi ciency slope (OUES), a cardiopulmonary exercise test (CPET) parameter derived from the logarithmic relationship between O 2 consumption (VO 2 ) and minute ventilation (VE) in patients with chronic heart failure (CHF). Objective: To evaluate the prognostic value of a new CPET parameter -peak oxygen uptake effi ciency (POUE) -and to compare it with OUES in patients with CHF. Methods: We prospectively studied 206 consecutive patients with stable CHF due to dilated cardiomyopathy -153 male, aged 53.3±13.0 years, 35.4% of ischemic etiology, left ventricular ejection fraction 27.7±8.0%, 81.1% in sinus rhythm, 97.1% receiving ACE-Is or ARBs, 78.2% beta-blockers and 60.2% spironolactone -who performed a fi rst maximal symptom-limited treadmill CPET, using the modified Bruce protocol. In 33% of patients an cardioverterdefi brillator (ICD) or cardiac resynchronization therapy device (CRT-D) was implanted during follow-up.Peak VO 2 , percentage of predicted peak VO 2 , VE/VCO 2 slope, OUES and POUE were analyzed. OUES was calculated using the formula VO 2 (l/min) = OUES (log 10 VE) + b. POUE was calculated as pVO 2 (l/min) / log 10 peakVE (l/min). Correlation coeffi cients between the studied parameters were obtained. The prognosis of each variable adjusted for age was evaluated through Cox proportional hazard models and R2 percent (R2%) and V index (V6) were used as measures of the predictive accuracy of events of each of these variables. Receiver operating characteristic (ROC) curves from logistic regression models were used to determine the cut-offs for OUES and POUE. Results: pVO 2 : 20.5±5.9; percentage of predicted peak VO 2 : 68.6±18.2; VE/VCO 2 slope: 30.6±8.3; OUES: 1.85±0.61; POUE: 0.88±0.27. During a mean follow-up of 33.1±14.8 months, 45 (21.8%) patients died, 10 (4.9%) underwent urgent heart transplantation and in three patients (1.5%) a left ventricular assist device was implanted. All variables proved to be independent predictors www.revportcardiol.org
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