RESUMEN

We present a case of a nine-year-old female patient who presented with recurrent syncope and was ultimately diagnosed with pulmonary hypertension (PH) secondary to pulmonary artery thrombi in the context of anti-phospholipid syndrome (APS). Extensive investigations including imaging studies revealed PH. Thromboembolic workup confirmed multiple pulmonary artery thrombi, and anti-phospholipid antibody testing confirmed APS. The patient received anticoagulation therapy tailored to APS management. Follow-up assessments demonstrated significant improvement in PH leading to cessation of syncope episodes. In this case, we underscore the importance of considering rare causes of syncope in the pediatric age group, particularly autoimmune disorders. Timely recognition and appropriate management are crucial for favorable outcomes in such cases. This report contributes to understanding the diverse clinical presentations of APS and emphasizes the need for a comprehensive diagnostic approach in patients with unexplained syncope.

RESUMEN

Background: Pancreatic ductal carcinoma (PDC) is a challenging diagnosis with a particularly poor prognosis, even after curative surgery (median survival: <30 months). The prognosis of borderline resectable pancreatic cancer (BR-PDC) is even worse. We describe a patient with BR-PDC who achieved stable disease with metronomic chemotherapy after refusing surgery. Case presentation: A 75-year-old woman was presented with jaundice and epigastric pain. Imaging confirmed a mass in the pancreatic head encasing the superior mesenteric vein, with obstruction of the pancreatic and bile ducts. After stenting to relieve the obstruction, Fine needle aspiration (FNA) confirmed the diagnosis of PDC. The patient refused surgery and radiation therapy but agreed for chemotherapy. After the second cycle of mFOLFIRINOX - complicated by febrile neutropenia - she refused further IV therapy. Genomic profiling revealed KIT amplification. Therefore, she was started on imatinib with dramatic improvement both clinically and biochemically reflected in carbohydrate antigen 19-9 drop. However, that response was short-lived at 3 months. Therefore, capecitabine was added at a low dose of 1 g bid on an alternate weekly basis. The patient did well and she is currently alive with a stable disease as of 2 years after diagnosis. Conclusion: Metronomic chemotherapy, especially capecitabine added to the targeted therapy, imatinib, is a potentially useful treatment for PDC where no other options are available, especially those harbouring no mutation in the dominant four genes. Indeed, the absence of mutation with KIT amplification could be a potential marker for improved outcomes with targeted and metronomic therapy, which deserves further evaluation in a clinical trial setting.

RESUMEN

PURPOSE: To assess Blood Oxygen Level-Dependent (BOLD) Magnetic Resonance Imaging (MRI) for noninvasive preoperative prediction of Microvascular Invasion (MVI) in Hepatocellular Carcinoma (HCC). MATERIALS AND METHODS: In this prospective, institutional review board approved study, 26 patients (21 men and 5 women age range, 34-77 years with mean age of 61 years) with HCC were evaluated preoperatively with liver MRI including baseline and post oxygen (O2) breathing BOLD MRI. Post processing of MRI data was performed to obtain R2* values (1/s) and correlated with histopathological assessment of MVI. Statistical analysis was performed to assess correlation of baseline R2*, post O2 R2* and R2* ratios to presence of MVI in HCC by binary logistic regression analysis. RESULTS: MVI was present in 15/26 (58%) of HCC on histopathology. The mean R2* values ± SD at baseline and post O2 with and without MVI were 35 ± 12, 36 ± 12, 38 ± 10, 42 ± 17. The R2* values between the groups with and without MVI were not significantly different statistically. CONCLUSION: BOLD MRI is unable to accurately predict MVI in HCC. The noninvasive preoperative MRI detection of MVI in HCC remains elusive.

Asunto(s)

RESUMEN

AIM: To determine predictors of clinical outcome following percutaneous transluminal angioplasty (PTA) in elderly patients with below knee atherosclerotic lesions causing intermittent claudication (IC) or critical limb ischaemia (CLI). MATERIALS AND METHODS: Over 7.5 years, 76 patients (CLI 72%, n = 55) underwent below knee PTA. The composite end-point of interest was major adverse clinical outcome (MACO) of the treated limb at follow-up which was defined as clinical failure, need for subsequent endovascular or surgical revascularization or amputation. Actuarial freedom from MACO was assessed using Kaplan-Meier curves and multivariable Cox proportional hazards regression. RESULTS: IC was improved in 95% at mean 3.4 years (range 0.5-108 months). Successful limb salvage and ulcer healing were seen in 73% with CLI. Most failures were in the CLI group (27% CLI vs. 5% IC), with an amputation rate of 16% for CLI vs. 5% for IC and persistent ulceration in 24% of CLI. Significant independent predictors of MACO were ulceration (hazard ratio 4.02, 95% CI = 1.55-10.38) and family history of atherosclerosis (hazard ratio 2.53, 95% CI = 1.1-5.92). CONCLUSION: Primary below knee PTA is a feasible therapeutic option in this elderly population. Limb ulceration and family history of atherosclerosis may be independent predictors of adverse outcome.

Asunto(s)

RESUMEN

PURPOSE: To determine the clinical outcomes and success rates after percutaneous subintimal angioplasty (SIA) in patients with lower-limb occlusive lesions causing intermittent claudication (IC) or critical limb ischemia (CLI) at midterm to long-term follow-up. The secondary aim was to elicit factors predictive of a successful outcome. MATERIALS AND METHODS: Between January 1999 and June 2006, 75 consecutive patients (45 men; age range, 46-91 years; CLI in 79%) underwent SIA of iliac and infrainguinal (84%) occlusions. Outcomes were determined on an intent-to-treat basis. The composite endpoint of interest was major adverse clinical outcome (MACO) of the treated limb at follow-up, which was defined as the development of IC, CLI, or need for subsequent endovascular or surgical revascularization. Actuarial freedom from MACO was assessed via Kaplan-Meier curves and multivariable Cox proportional-hazards regression. RESULTS: SIA was performed on 75 lesions, with an initial procedure success rate of 83% (n = 62). Procedure failure was caused by heavily calcified lesions (n = 5) and failure of reentry (n = 8). A total of 56.3% of patients with claudication were free from ipsilateral claudication at follow-up (mean, 32 months; range, 1-64 months), and those with CLI had a 79.7% limb salvage rate at a mean follow-up of 30.7 months (range, 0.5-91 months). On Cox regression analysis, the following variables were identified as independent predictors of MACO within the limb treated with SIA: ABI after SIA (hazard ratio, 0.21; 95% CI, 0.05-0.89; P = .035) and number of patent runoff vessels (ie, /=2; hazard ratio, 0.29; 95% CI, 0.15-0.59; P = .001). CONCLUSIONS: SIA is a feasible therapeutic option for occlusive atherosclerotic lesions in IC and CLI and is the evolving preferred strategy in CLI and perhaps IC with long-segment occlusions.

Asunto(s)

RESUMEN

PURPOSE: The clinical importance of angiographically detected asymptomatic lower-limb stenoses and occlusions is unknown. This study aims to (i) assess the clinical outcome of asymptomatic lesions in the lower limb, (ii) identify predictors of clinical deterioration, and (iii) determine which asymptomatic lower-limb lesions should be treated at presentation. MATERIALS AND METHODS: All 918 patients undergoing peripheral angiography with or without angioplasty over a period of 7.5 years (January 1999 through June 2006) at a single institution were retrospectively evaluated. One hundred twenty-two patients (54% men; mean age, 70.3 years; age range, 41-91 y) with angiographic stenoses (> or =50%) or occlusions on the asymptomatic leg were included. The composite endpoint of interest was major adverse clinical outcome (MACO) of the asymptomatic limb at clinical follow-up, which was defined as the development of intermittent claudication (IC), critical limb ischemia (CLI), or need for subsequent endovascular or surgical revascularization. Actuarial freedom from MACO was assessed with Kaplan-Meier curves and multivariable Cox proportional-hazards regression. RESULTS: During a 4.2-year mean follow-up in 122 patients with significant concomitant asymptomatic disease, 32.8% of patients developed symptoms (13.9% with IC, 18.9% with CLI); 42.5% of these cases required revascularization. Cox regression revealed two independent predictors of MACO on the asymptomatic side: contralateral below-knee amputation (BKA; hazard ratio, 2.93; 95% CI, 1.21-7.10; P = .01) and statin treatment (hazard ratio, 3.56; 95% CI, 1.56-8.13; P = .003). CONCLUSIONS: Asymptomatic peripheral angiographic stenoses and occlusions become symptomatic in one third of patients, necessitating treatment in 13.9% overall. Previous contralateral BKA and statin use were independent predictors of adverse outcome in this population. Close clinical follow-up and appropriate risk factor modification are recommended.

Asunto(s)