Maffucci’s syndrome is a rare congenital nonhereditary syndrome with less than 300 cases having been reported in the United States. It is characterized by multiple enchondromas, hemangiomas, and rarely lymphangiomas. Enchondromas may undergo malignant transformation to chondrosarcomas. Surveillance plays a vital role in detecting early malignant transformation. Fluorodeoxyglucose (FDG) PET/CT, although falling out of favor, may be utilized as an imaging modality by physicians to determine such transformation, allowing for timely management and intervention. In this report, we share our experience with such a case.
While various modalities exist for the diagnosis of pulmonary embolism (PE), CT pulmonary angiography (CTPA) is the most widely used and can establish the diagnosis quickly and reliably.We report a patient who presented with syncope who developed pulseless electrical activity (PEA) arrest in the emergency department.Given the presence of acute renal injury, CTA was felt to be contraindicated.A ventilationperfusion lung (VQ) scan demonstrated low probability for PE; however, echocardiography revealed evidence for right heart strain.Subsequent cardiac magnetic resonance imaging (CMR) unexpectedly revealed a saddle PE.This case highlights the potential role for MR for the diagnosis of PE when high clinical suspicion is discordant with results of conventional imaging.
The improved life expectancy of patients with cystic fibrosis (CF) has led to a change in the impact of liver disease on the prognosis of this population. Liver transplantation has emerged as the procedure of choice for patients with CF and features of hepatic decompensation and for intractable variceal bleeding as a major manifestation. We retrospectively reviewed the United Network for Organ Sharing database to analyze the outcomes of 55 adults and 148 children with CF who underwent liver transplantation, and we compared them to patients who underwent transplantation for other etiologies. We additionally compared the benefits of liver transplantation among patients who underwent transplantation for cystic fibrosis-related liver disease (CFLD) and those who remained on the waiting list. The 5-year survival rates for children and adults undergoing liver transplantation were 85.8% and 72.7%, respectively (P = 0.016). A multivariate Cox regression analysis comparing pediatric and adult CF patients to patients who underwent transplantation for other etiologies noted lower 5-year survival rates (P < 0.0001). However, compared to those remaining on the waiting list, pediatric transplant recipients with CF (hazard ratio = 0.33, 95% confidence interval = 0.16-0.70, P = 0.004) and adult transplant recipients with CF (hazard ratio = 0.25, 95% confidence interval = 0.11-0.57, P = 0.001) gained a significant survival benefit. In conclusion, long-term outcomes in patients with CFLD are acceptable but are inferior in comparison with the outcomes of those undergoing transplantation for other etiologies. Despite such observations, a survival benefit was noted in transplant patients versus those who remained on the waiting list.
Abstract Background A paradoxical inverse relationship between body mass index, morbidity and mortality in patients with ischemic heart disease has been noted; but the underlying mechanisms remain unclear. Given that coronary resistance arteries are the primary regulators of myocardial blood flow, we examined the effects of obesity and medication on dilator function in coronary microvessels. Methods Bradykinin-induced coronary dilation was assessed by videomicroscopy in ex vivo coronary arterioles obtained from 64 consecutive patients undergoing heart surgery. Multi-variable linear regression and logistic regression were used to investigate the effects of obesity (BMI ≥ 30 kg/M 2 ) and the influences of medications on vessel responses. Results In isolated, pressurized (80 mmHg) coronary arterioles of obese and non-obese patient the active (73±4 vs. 79±13 μm) and passive (111 ± 5.5 vs. 118 ± 5.0 μm) diameters were similar. Bradykinin elicited substantial dilation in coronary arterioles, with a similar magnitude in obese and non-obese patients (to 10 -8 M: 55 ± 5% vs. 46 ± 5%, P = 0.20), but with significantly enhanced sensitivity in obesity (EC50: 8.2x10 -9 M vs. 1.9x10 -8 M, respectively, P = 0.03). When adjusted for other risk factors and medications, obesity and statins were determined to be the only positive predictors of enhanced dilation, as assessed with multiple regression analysis. Moreover, obese patients with or without statin exhibited significantly increased coronary dilation to bradykinin, when compared to non-obese patients without statin therapy. Conclusions Obesity and statin therapy are independently associated with an enhanced dilator function of coronary arterioles in patients undergoing heart surgery, which may offer a potential mechanism for the better cardiovascular outcome described earlier as the obesity paradox.
Non-renal transplant recipients who subsequently develop ESRD and undergo kidney transplantation are medically and immunologically complex due to comorbidities, high cumulative exposure to immunosuppressants, and sensitization to alloantigen from the prior transplant. Although prior non-renal transplant recipients are one of the fastest growing segments of the kidney wait list, minimal data exist to guide the use of antibody induction therapy (IT+) at the time of kidney after lung (KALu), heart (KAH), and liver (KALi) transplant.This retrospective cohort study used national registry data to examine IT use and survival after kidney transplantation. Separate multivariate Cox regression models were constructed to assess patient survival for IT+ and IT- KALu (n=232), KAH (n=588), and KALi (n=736) recipients.Use of IT increased during the study period. The percentage of patients considered highly sensitized (panel reactive antibody ≥20%) was not statistically significant between IT+ and IT- groups. IT+ was not associated with improvement in 1- and 10-year patient survival for KALu (P=0.20 and P=0.22, respectively) or for KAH (P=0.90 and P=0.14, respectively). However, IT+ among KALi was associated with inferior patient survival at 1 and 10 years (P=0.04 and P=0.02, respectively).Use of IT for kidney transplantation among prior non-renal transplant recipients may not offer a survival advantage in KALu or KAH. However, due to limited power, these findings should be interpreted cautiously. IT+ was associated with inferior outcomes for KALi. Use of IT should be judicially reconsidered in this complex group of recipients.
The disparity between the number of patients waiting for kidney transplantation and the limited supply of kidney allografts has renewed interest in the benefit from kidney transplantation experienced by different groups. This study evaluated kidney transplant survival benefit in prior nonrenal transplant recipients (kidney after liver, KALi; lung, KALu; heart, KAH) compared to primary isolated (KA1) or repeat isolated kidney (KA2) transplant. Multivariable Cox regression models were fit using UNOS data for patients wait listed and transplanted from 1995 to 2008. Compared to KA1, the risk of death on the wait list was lower for KA2 (p < 0.001;HR = 0.84;CI = 0.81-0.88), but substantially higher for KALu (p < 0.001; HR = 3.80;CI = 3.08-4.69), KAH (p < 0.001; HR = 1.92; CI = 1.66-2.22), and KALi (p < 0.001; HR = 2.69; CI = 2.46-2.95). Following kidney transplant, patient survival was greatest for KA1, similar among KA2, KALi, KAH, and inferior for KALu. Compared to the entire wait list, renal transplantation was associated with a survival benefit among all groups except KALu (p = 0.017; HR = 1.61; CI = 1.09-2.38), where posttransplant survival was inferior to the wait list population. Recipients of KA1 kidney transplantation have the greatest posttransplant survival and compared to the overall kidney wait list, the greatest survival benefit.
Chronic kidney disease among prior heart transplant recipients is a growing problem that is likely to place an increased demand on a limited supply of kidney allografts. Allocation of the limited resource of kidneys for transplantation requires consideration of the demands of fair distribution and optimizing patient and graft survival. The aim of this study was to compare the kidney transplant outcomes among recipients of kidney after prior heart transplantation (KAH, n=456) with kidney transplantation in other clinical settings.A retrospective cohort study using United Network for Organ Sharing registry data (1995-2008) was performed comparing renal allograft survival among KAH recipients with patients who underwent simultaneous kidney-heart transplant (SKH, n=252), primary kidney transplant alone (KA1, n=112,882), or repeat kidney transplant alone (KA2, n=14,070).The annual number of KAH recipients more than quadrupled during the study period from 24 in 1995 to 99 in 2008. In a multivariable analysis using Cox regression, allograft survival among KAH recipients was not different from SKH (P=0.16, hazards ratio [HR]=0.79, confidence interval [CI]=0.57-1.10), and KA2 (P=0.11, HR=0.86, CI=0.72-1.04), but it was inferior to KA1 (P<0.001, HR=0.66, CI=0.55-0.80). Patient death accounted for 75.2% of KAH kidney loss. Kidney quality as measured by living or deceased donors (P=0.62) and standard criteria or extended criteria (P=0.87) was not associated with survival; however, there was a trend toward improved survival (P=0.08) among recipients of a preemptive transplant.Kidney graft survival among prior heart transplant recipients is inferior to KA1 but similar to other clinical scenarios. Preemptive transplantation with an extended criteria or living donor kidney should be encouraged.
