scholarly journals Liver Transplantation for Hepatic Epithelioid Hemangioendothelioma: The Canadian Multicentre Experience

2008 ◽  
Vol 22 (10) ◽  
pp. 821-824 ◽  
Author(s):  
Carmine G Nudo ◽  
Eric M Yoshida ◽  
Vincent G Bain ◽  
Denis Marleau ◽  
Phil Wong ◽  
...  

INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare entity. At the present time, there is no standardized effective therapy. Liver transplantation (LT) has emerged as a treatment for this rare tumour.OBJECTIVE: To evaluate the outcome of liver transplantation for HEHE at eight centres across Canada.METHODS: The charts of patients who were transplanted for HEHE at eight centres across Canada were reviewed.RESULTS: A total of 11 individuals (eight women and three men) received a LT for HEHE. All LTs were performed between 1991 and 2005. The mean (± SD) age at LT was 38.7±13 years. One patient had one large liver lesion (17 cm × 14 cm × 13 cm), one had three lesions, one had four lesions and eight had extensive (five or more) liver lesions. One patient had spleen involvement and two had involved lymph nodes at the time of transplantation. The mean duration of follow-up was 78±63 months (median 81 months). Four patients (36.4%) developed recurrence of HEHE with a mean time to recurrence of 25±25 months (median 15.6 months) following LT. The calculated survival rate following LT for HEHE was 82% at five years.CONCLUSIONS: The results of LT for HEHE are encouraging, with a recurrence rate of 36.4% and a five-year survival rate of 82%. Further studies are needed to help identify patients who would benefit most from LT for this rare tumour.

Neurosurgery ◽  
2004 ◽  
Vol 55 (2) ◽  
pp. 349-357 ◽  
Author(s):  
Vivek R. Deshmukh ◽  
Kris A. Smith ◽  
Harold L. Rekate ◽  
Stephen Coons ◽  
Robert F. Spetzler

Abstract OBJECTIVE: Pineocytomas are associated with the most favorable prognosis of all pineal tumors. However, a subset of pineocytomas may have a predilection for recurrence and therefore behave aggressively. PATIENTS AND METHODS: Records of nine patients (five men, four women; mean age, 44 yr; range, 24–63 yr) with histologically diagnosed pineocytomas consecutively treated between 1990 and 2003 were reviewed retrospectively to identify factors predictive of aggressiveness. Eight patients presented with hydrocephalus and four with tectal compression. Three patients underwent gross total resection, and six underwent subtotal resection or biopsy. RESULTS: Three local recurrences necessitated reoperation. One recurrence involved the obex of the fourth ventricle. The mean time to recurrence was 3.5 years (range, 1–7 yr). There was no correlation between histological features and tumor recurrence. Patients undergoing radiosurgery showed stable or attenuated local disease (mean follow-up, 19.3 mo; range, 6–36 mo). Mean radiographic follow-up was 34 months (range, 6 mo to 10 yr). Mean clinical follow-up was 36 months (range, 1 mo to 10 yr). CONCLUSION: A subset of pineocytomas demonstrates the potential for symptomatic recurrence. We advocate an attempt at gross total tumor resection for all symptomatic patients with tectal plate compression, reserving radiosurgery for small, subtotally resected, or recurrent lesions. Patients must be followed closely for recurrence. Radiosurgery seems to be beneficial for local tumor control. Further investigation is needed to identify histological markers for pineocytomas that behave aggressively.


2021 ◽  
pp. 1-7
Author(s):  
Naomi Vather-Wu ◽  
Matthew D. Krasowski ◽  
Katherine D. Mathews ◽  
Amal Shibli-Rahhal

Background: Expert guidelines recommend annual monitoring of 25-hydroxyvitamin D (25-OHD) and maintaining 25-OHD ≥30 ng/ml in patients with dystrophinopathies. Objective: We hypothesized that 25-OHD remains stable and requires less frequent monitoring in patients taking stable maintenance doses of vitamin D. Methods: We performed a retrospective cohort study, using the electronic health record to identify 26 patients with dystrophinopathies with a baseline 25-OHD ≥30 ng/mL and at least one additional 25-OHD measurement. These patients had received a stable dose of vitamin D for ≥3 months prior to their baseline 25-OHD measurement and throughout follow-up. The main outcome measured was the mean duration time the subjects spent with a 25-OHD ≥30 ng/mL. Results: Only 19% of patients dropped their 25-OHD to <  30 ng/ml, with a mean time to drop of 33 months and a median nadir 25-OHD of 28 ng/mL. Conclusions: These results suggest that measurement of 25-OHD every 2–2.5 years may be sufficient in patients with a baseline 25-OHD ≥30 ng/mL and who are on a stable maintenance dose of vitamin D. Other patients may require more frequent assessments.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S441-S442
Author(s):  
Xing Tan ◽  
Andrew M Skinner ◽  
Benjamin Sirbu ◽  
Larry H Danziger ◽  
Dale N Gerding ◽  
...  

Abstract Background There is a paucity of data assessing outcomes of alternate fidaxomicin strategies in patients with recurrent Clostridioides difficile infection (rCDI). The objective of our study is to evaluate a tapered-pulsed (T-P) fidaxomicin regimen that was administered immediately following a course of CDI treatment with initial symptom resolution in patients with multiple rCDI. Methods We reviewed the characteristics and outcomes of 46 consecutive patients who received T-P fidaxomicin between January 1, 2014-June 30, 2019 in a specialty CDI clinic. The first episode in which fidaxomicin T-P was administered was analyzed. Failure was defined as the persistence of diarrhea and/or the need for additional CDI treatment at any time on T-P fidaxomicin. Sustained clinical cure (SCC) was defined as resolution of diarrhea without recurrence. Recurrence was defined as the return of diarrhea requiring retreatment with CDI therapy after completion of T-P fidaxomicin. Both SCC and recurrence were evaluated at 30 and 90 days after completion of T-P fidaxomicin. Results The mean±SD age of the 46 patients was 63.2±19.9 years, 71.7% were female, and the mean±SD CDI episodes within the past year was 3±1.4 . Most patients (73.9%) had previously failed a vancomycin tapered and/or pulsed regimen. Prior to administering T-P fidaxomicin, a treatment regimen was given to ensure resolution of symptoms. The CDI treatment most commonly used (58.7%) was vancomycin. The T-P fidaxomicin regimen used consisted of 200 mg given once daily for 7 days followed by 200 mg every other day for a median (min-max) duration of 33 (6-120) days. Two patients (4%) failed to respond to T-P fidaxomicin; 34 (74%) and 28 (61%) achieved SCC at 30 and 90 days, respectively. Among the 44 patients that successfully completed the T-P fidaxomicin regimen, recurrence developed in 10 (22.7%) and 16 (36.4%) of patients at 30 and 90 days, respectively, with a median (min-max) time to recurrence of 20 (3-87) days (Figure 1). Four patients with recurrence had received subsequent systemic antibiotics. Figure 1. Course of CDI therapy and follow-up Conclusion A tapered-pulsed fidaxomicin strategy may be effective in patients with multiply rCDI who are refractory to other treatments, including a vancomycin tapered and pulsed regimen. Disclosures Larry H. Danziger, PharmD, Merck (Speaker’s Bureau)


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ryo Yamazaki ◽  
Osamu Nishiyama ◽  
Sho Saeki ◽  
Hiroyuki Sano ◽  
Takashi Iwanaga ◽  
...  

AbstractSome patients with idiopathic pulmonary fibrosis (IPF) undergo recurrent acute exacerbations (AEs). This study aimed to elucidate the risk factors for recurrent AEs of IPF (AE-IPF). Consecutive patients with IPF admitted for their first AE-IPF between January 2008 and December 2018 were retrospectively recruited. Of 63 patients admitted for an AE-IPF and discharged alive, 9 (14.3%) developed a recurrence of AE within 1 year. The mean time to recurrence was 233 ± 103 days. Total doses (mg/month and mg/kg/month) of corticosteroids administered over day 1 to 30 after the AE were significantly higher in patients without recurrences of AE-IPF (5185 ± 2414 mg/month, 93.5 ± 44.0 mg/kg/month) than the doses in patients with recurrences (3133 ± 1990 mg/month, 57.2 ± 37.7 mg/kg/month) (p = 0.02 and p = 0.03, respectively). However, no differences were observed between the total doses of corticosteroids administered over days 31 to 60, 61 to 90, 91 to 120, and 151 to 180 after the AE. Furthermore, differences between the administration rates of immunosuppressive and antifibrotic treatments administered to the 2 patient groups were not significant. An increased total dose of corticosteroid administered over day 1 to 30 after an AE-IPF was associated with a decreased risk of subsequent recurrence of AE-IPF within 1 year after the first AE.


2021 ◽  
pp. 000313482110234
Author(s):  
Angela Sickels ◽  
Keyur B. Shah ◽  
Brianna Ruch ◽  
Adrian Cotterell ◽  
Inna Tchoukina ◽  
...  

Background Combined heart-liver transplantation (CHLT) is the only curative option for patients with concomitant pathology affecting the heart and liver. In some cases, the native livers of familial amyloidosis (FA) patients may be suitable for domino transplantation into other recipients. Methods Retrospective analysis (2013 to 2019) of all CHLT at our center was performed. Continuous data were presented as mean with standard deviation and discrete variables as percentages. Results Familial amyloidosis was the indication for CHLT in 5 out of 6 patients. The mean recipient age was 55 ± 5.62 years. Two patients were bridged with total artificial heart. The mean model for end-stage liver disease score at transplant was 17.17 ± 3.7. Two explanted livers were used for transplantation in a domino fashion. The median intensive care and hospital stays were 5.5 and 19 days, respectively. Complications included renal failure (1), groin abscess (1), pulmonary embolism (1), and cardiac rejection (1). Patient and graft survival for both organs was 100% at a median follow-up of 59 (range 20-76) months. Discussion Combined heart-liver transplantation for FA achieves excellent outcomes. The possible use of livers explanted from patients with FA for domino liver transplantation can contribute to the liver donor pool.


2021 ◽  
Vol 28 (Supplement_1) ◽  
Author(s):  
J Borrego Rodriguez ◽  
C Palacios Echevarren ◽  
S Prieto Gonzalez ◽  
JC Echarte Morales ◽  
R Bergel Garcia ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. INTRODUCTION CRH in patients with ischemic heart disease is recommended by the different clinical practice guidelines with an IA level of evidence, with an important role in reducing cardiovascular mortality and hospital readmissions during follow-up. OBJECTIVE The goal of this study is to show the 4-year clinical results of a population of patients who participated in an CRH program after an Acute Coronary Syndrome (ACS). METHODS Between May/2014 and September/2017, 221 patients who had recently presented an ACS completed the 12 weeks of phase II of the CRH program at our center. In May/2020 we collected epidemiological, clinical and echocardiographic information at the time of the acute cardiovascular event; and we evaluate the current vital status of the patients and the incidence of readmissions for: angina, HF, new ACS, or arrhythmic events. RESULTS Of the 221 patients, 182 were men (82%). The mean age of our population was 58.3 ± 7.8 years. 58% (129 patients) suffered from ST-elevation ACS. The mean time of hospital stay was 6.20 ± 2.9 days. An echocardiogram was performed at discharge, which showed an average LVEF of 56 ± 6%. Eight patients (4%) developed early Ventricular Fibrilation (VF) during the acute phase of ACS. Among the classic CVRF, smoking (79%) was the most prevalent, followed by dyslipidemia (53%) and hypertension (47%). The mean time from hospital discharge to the start of phase II RHC was 42 ± 16 days. The overall incidence of events was 9%: 10 patients suffered reinfarction during follow-up, and 7 were readmitted for unstable angina, all of whom underwent PCI; no patient was admitted for HF; and none of the 8 patients with early VF had a new tachyarrhythmia, registering a single admission for VT during follow-up. None of the patients had sustained ventricular tachyarrhythmias during exercise-training. At the mean 4.5-year follow-up, 218 patients were still alive (98%). CONCLUSION The incidence of CV events in the follow-up of our cohort was low, which can be explained by the fact that it is a young population, with an LVEF at low limits of normality at discharge, which is one of the most important predictors in the prognosis after an ischemic event. As an improvement, we must shorten the time until the start of phase II of the program. CRH shows once again its clinical benefit after an ACS, in consonance with the existing evidence. Abstract Figure. Outcomes of a CRH program.


2021 ◽  
Vol 9 (6) ◽  
pp. 232596712110108
Author(s):  
Andrea Bardos ◽  
Sanjeeve Sabhrawal ◽  
Graham Tytherleigh-Strong

Background: Sternal fractures are rare, and they can be treated nonoperatively. Vertical sternal fractures have rarely been reported. Purpose: To describe the management and surgical treatment of a series of elite-level athletes who presented with symptomatic nonunions of a vertical sternal fracture. Study Design: Case series; Level of evidence, 4. Methods: Patients with an established symptomatic nonunion of a vertical sternal fracture, as diagnosed by computed tomography (CT) or magnetic resonance imaging (MRI), underwent open reduction and internal fixation using autologous bone graft and cannulated lag screws. The patients were assessed preoperatively and at the final follow-up using the Rockwood sternoclavicular joint (SCJ) score; Constant score; and shortened version of the Disabilities of the Arm, Shoulder and Hand (QuickDASH) scores. Bony union was confirmed on postoperative CT scan. Results: Five patients (4 men and 1 woman) were included; all were national- or international-level athletes (rugby, judo, show-jumping, and MotoGP). The mean age at surgery was 23.4 years (range, 19-27 years), the mean time from injury to referral was 13.6 months (range, 10-17 months), and the mean time from injury to surgery was 15.8 months (range, 11-20 months). The mean follow-up was 99.4 months (range, 25-168 months). There was a significant improvement after surgery in the mean Rockwood SCJ score (from 12.6 to 14.8 [ P < .05]), Constant score (from 84 to 96.4 [ P < .05]; 80% met the minimal clinically important difference [MCID] of 10.4 points), and QuickDASH (from 6.8 to 0.98 [ P < .05]; 0% met the MCID of 15.9 points). Four of the patients were able to return to sport at their preinjury level, and 1 patient retired for nonmedical reasons. All of the fractures had united on the postoperative CT scan. There were no postoperative complications. Conclusion: Vertical fractures of the sternum are very rare and tend to behave clinically like an avulsion fracture injury to the capsuloligamentous structure of the inferior SCJ. The requirement of advanced imaging to diagnose this injury means that the actual incidence and natural history are not known. For high-demand athletes, early identification, surgical reduction, and fixation are likely to achieve the best outcome.


2009 ◽  
Vol 65 (suppl_6) ◽  
pp. ons100-ons109 ◽  
Author(s):  
Giuseppe Mirone ◽  
Salvatore Chibbaro ◽  
Luigi Schiabello ◽  
Serena Tola ◽  
Bernard George

Abstract Objective: En plaque sphenoid wing meningiomas are complex tumors involving the sphenoid wing, the orbit, and sometimes the cavernous sinus. Complete removal is difficult, so these tumors have high rates of recurrence and postoperative morbidity. The authors report a series of 71 patients with sphenoid wing meningiomas that were managed surgically. Methods: The clinical records of 71 consecutive patients undergoing surgery for sphenoid wing meningiomas at Lariboisière Hospital, Paris, were prospectively collected in a database during a 20-year period and analyzed for presenting symptoms, surgical technique, clinical outcome, and follow-up. Results: Among the 71 patients (mean age, 52. 7 years; range, 12–79 years), 62 were females and 9 were males. The most typical symptoms recorded were proptosis in 61 patients (85.9%), visual impairment in 41 patients (57.7%), and oculomotor paresis in 9 patients (12.7%). Complete removal was achieved in 59 patients (83%). At 6 months of follow-up, magnetic resonance imaging scans revealed residual tumor in 12 patients (9 in the cavernous sinus and 3 around the superior orbital fissure). Mean follow-up was 76.8 months (range, 12–168 months). Tumor recurrence was recorded in 3 of 59 patients (5%) with total macroscopic removal. Among the patients with subtotal resection, tumor progression was observed in 3 of 12 patients (25%; 2 patients with grade III and 1 patient with grade IV resection). Mean time to recurrence was 43.3 months (range, 32–53 months). Conclusion: Surgical management of patients with sphenoid wing meningiomas cannot be uniform; it must be tailored on a case-by-case basis. Successful resection requires extensive intra- and extradural surgery. We recommend optic canal decompression in all patients to ameliorate and/or preserve visual function.


Author(s):  
David P. Bray ◽  
Bryan E. Buster ◽  
Joseph W. Quillin ◽  
Robert H. Press ◽  
Bree R. Eaton ◽  
...  

Abstract Introduction Atypical meningiomas (AM) are meningiomas that are more aggressive than their grade-I counterparts and have a higher rate of recurrence. The effect of adjuvant radiotherapy (ART) on AM of the skull base is not defined. Methods A retrospective review of all AM's of the skull base primarily resected at our institution from 1996 to 2018 was completed. ART was defined as radiotherapy (RT) that occurred within 6 months of initial resection, regardless of Simpson's grade. Minimum time length of follow-up after resection was 2 years. Statistical analysis was performed using SAS. Results There were a total of 59 skull base–located (SBL) AMs resected at our institution from 1996 to 2018. The average age of our cohort was 53.2 years. Gross total resection, defined as Simpson's grades I to III resection, was achieved in 36 (61%) of cases. Thirty-five of 59 (59%) patients received ART. Recurrence was observed in 14 patients (24%), and mean time to recurrence was 63.8 months. Patients who received ART had a lower observed rate of recurrence (8 vs. 46%); however, time to recurrence was not significantly different between the two populations. Conclusion We observe that AM in the skull base location have higher recurrence rates than we would expect from grade-I meningioma. These data suggest that ART may offer benefit to the overall observed frequency of recurrence of SBL AM; however, the time to recurrence between patients who received ART and those who did not was not statistically significant in survival analysis.


2017 ◽  
Vol 11 (1) ◽  
pp. 1041-1048 ◽  
Author(s):  
Mehmet Bekir Unal ◽  
Kemal Gokkus ◽  
Evrim Sirin ◽  
Eren Cansü

Objective: The main objective of this study is to evaluate the availability of lateral antebrachial cutaneous nerve (LACN) autograft for acute or delayed repair of segmented digital nerve injuries. Patients and Methods: 13 digital nerve defects of 11 patients; treated with interposition of LACN graft that harvested from ipsilateral extremity were included in the study. Mean follow up period was 35, 7 months. The mean time from injury to grafting is 53, 3 days. The results of the mean 2PDT and SWMT values of injured /uninjured finger at the end of follow up period were evaluated with Paired T test. The correlation between the defect length and the difference of 2PDT, SWMT values between the uninjured and injured finger at the end of follow up period; were evaluated with Pearson - correlation analysis. Results: The mean value of our 2PDT and SWMT results are ~5,923, ~3, 52, respectively in which can be interpreted between the normal and diminished light touch. The defect length and difference percentage of SWMT values is positively and significantly correlated statistically. Mean length of interposed nerve grafts was 18.5 mm. The age of the patient and the mean values of 2PDT and SWMT with the difference % of 2PDT and % of SWMT are not statistically correlated. Conclusion: Based on results regarding sensory regaining at recipient side and negligible sensory deficit at harvesting side, we suggest that lateral antebrachial cutaneous nerve might be a valuable graft option for digital nerve defects.


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