Allergic eosinophilic gastroenteritis (AEG) with protein-losing enteropathy (PLE): Intestinal pathological characteristics and long-term clinical follow-up

2005 ◽  
Vol 115 (2) ◽  
pp. S242
Author(s):  
M. Chehade ◽  
S. Mofidi ◽  
M.S. Magid ◽  
A. Nowak-Wegrzyn ◽  
S.H. Sicherer ◽  
...  
Keyword(s):  
Eosinophilic Gastroenteritis ◽  
Protein Losing Enteropathy ◽  
Pathological Characteristics

Primary Intrapulmonary Thymomas: Case Report and Review of the Literature

2009 ◽  
Vol 37 (4) ◽  
pp. 1252-1257 ◽  
Author(s):  
L Gong ◽  
Y-H Li ◽  
X-L He ◽  
Q Wang ◽  
L Yao ◽  
...  
Keyword(s):  
Epithelial Membrane Antigen ◽  
Immunohistochemical Analysis ◽  
Lung Tumour ◽  
Review Of The Literature ◽  
Malignant Tumours ◽  
Pathological Characteristics ◽  
Immunological Phenotype ◽  
Type A Thymoma

Primary intrapulmonary thymomas (PIT), which are intrapulmonary tumours without an associated mediastinal component, are very rare; only 29 cases of PIT have been described in the literature since 1951. This report presents a case of PIT in a 59-year old Chinese woman with a type A thymoma (including its pathogenesis, clinical pathological characteristics, immunological phenotype, treatment and prognosis), in the context of a review of the current literature. The origin of thymomas in this unusual location remains unknown. In this case, immunohistochemical analysis demonstrated that the epithelial component was strongly positive for cytokeratin and focally reactive for epithelial membrane antigen. It is concluded that PIT should be considered when the histopathological appearance of a lung tumour is not typical of other pulmonary neoplasms. Complete resection appears sufficient in non-malignant tumours, while in cases of partial resection or malignancy, adjuvant radiotherapy should be considered. Long-term regular clinical follow-up is also warranted, due to the risk of late local recurrence.

Long-term outcome after atrioventricular valve surgery following modified Fontan operation

2010 ◽  
Vol 21 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Shaji C. Menon ◽  
Joseph A. Dearani ◽  
Frank Cetta
Keyword(s):  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Valve Surgery ◽  
Atrioventricular Valve ◽  
Term Outcome ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome

AbstractObjectiveThe objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.BackgroundAtrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.MethodsPatients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.ResultsA total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.ConclusionAtrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Pathological characteristics and clinical outcome in triple-negative breast cancer (BC) patients (PTS): Results from the NORA study

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 11014-11014 ◽  
Author(s):  
M. E. Cazzaniga ◽  
G. Mustacchi ◽  
P. Pronzato ◽  
F. Di Costanzo ◽  
A. De Matteis ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Clinical Outcome ◽  
Triple Negative ◽  
Fold Increase ◽  
Conservative Surgery ◽  
Treatment Modalities ◽  
Pathological Characteristics ◽  
Worse Prognosis

11014 Background: Different studies have recently focused the attention on the so-called triple-negative pts, defined as ER-/PR- /HER2-. Although triple-negative tumours have been reported to be more aggressive, there are limited long-term data evaluating outcome as a function of this classification. Methods: NORA is an observational study aimed at investigating treatment modalities and clinical outcome of 3515 patients (pts) with early breast cancer (EBC) treated in 77 Oncological Centres in Italy from to 2000 to 2003, whose overall results have been already published. We now compare pathological characteristics and clinical outcome of pts for whom ER and PR andHER2 tests are negative with remaining pts (OTH). Results: 123 (4.1%) of 2968 evaluable pts resulted triple-negative. Out of these 123, 76 pts (61.7% vs 63.2% of OTH) were treated with conservative surgery (CS). Pathological T stage was T1 in55.3% pts (OTH: 59.6%), 47.9% were pN+ (OTH: 45.3%), 63.2 had G3 tumours (OTH: 34.1%). Chemotherapy alone was administered in 89.4% pts, mainly anthracycline-based (54.1%). At a median follow up of 27 months, all triple-negative pts were alive, 11 (8.9%) with relapse. No difference has been observed between two groups both in DFS (HR=0.74; 95%CI: 0.39–1.40; p=0.35) and OS (HR not estimated, p=0.99). Conclusions: Our results suggest that triple-negative pts have pathological characteristics similar to what observed in the other pts, receive CS in the same percentage of the cases, but have a 3-fold increase of undifferentiated tumours. On the contrary to what observed by other Authors, our data show that triple- negative pts do not have worse prognosis. Longer follow up is required to confirm these data. No significant financial relationships to disclose.

scholarly journals Malignant Lymphoma Originating in the Jejunum of a Long-term Follow-up Case of Protein-losing Enteropathy.

1997 ◽  
Vol 36 (8) ◽  
pp. 556-560 ◽  
Author(s):  
Meiko NISHIUCHI ◽  
Masato IIDA ◽  
Yasuhiko SAIKA ◽  
Takahiro KUWASAKO ◽  
Kouichi SEKI ◽  
...  
Keyword(s):  
Malignant Lymphoma ◽  
Protein Losing Enteropathy ◽  
Long Term Follow Up

Permanent pacing post-Fontan is not associated with reduced long-term survival

2019 ◽  
Vol 29 (12) ◽  
pp. 1432-1434
Author(s):  
Sally J. Hall ◽  
Damien Cullington ◽  
John D. R. Thomson ◽  
James R. Bentham
Keyword(s):  
Sinus Rhythm ◽  
Fontan Procedure ◽  
Kidney Injury ◽  
Prognostic Indicator ◽  
Retrospective Case ◽  
Protein Losing Enteropathy ◽  
Term Survival ◽  
Long Term Survival

AbstractBackground:The Fontan procedure is the final stage of surgical palliation for a single-ventricle circulation. Significant complications are common including rhythm disturbance necessitating implantation of a permanent pacemaker. This has been widely considered a negative prognostic indicator.Methods:This single-centre, retrospective case control study involved all patients who underwent the Fontan procedure at the Leeds Congenital Heart Unit between 1990 and 2015 and have had regular follow-up in Yorkshire and Humber, United Kingdom. 167 Fontan patients were identified of which 2 were excluded for having a pre-procedure pacemaker. Of the remainder, 23 patients required a pacemaker. Outcomes were survival, early and late complications, need for further intervention and oxygen saturation in long-term follow-up.Results:There was no difference in survival (30-day survival pacemaker 92.6%, sinus rhythm 90.5%, p = 0.66, 1-year pacemaker 11.1%, sinus rhythm 10.1%, p = 1). The pacemaker group was more likely to have cerebral or renal complications in the first-year post-procedure (acute kidney injury: sinus rhythm 0.8%, pacemaker 19.1%, p = 0.002). No difference was observed in longer term complications including protein losing enteropathy (sinus rhythm 3.5%, pacemaker 0% p = 1). There was no difference in saturations between the two groups at follow-up. Paced patients were more likely to have required further intervention, with a higher incidence of cardiopulmonary bypass procedures (sinus rhythm 6.3%, pacemaker 35%, p < 0.001).Conclusions:Despite an increase in early complications and the need for further interventions, pacemaker requirement does not appear to affect long-term survival following the Fontan procedure.

Long term follow-up after pregnancy in Fontan patients

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Gribaudo ◽  
A Constantine ◽  
A Pires ◽  
I Ahmed ◽  
R Patel ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Nyha Class ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Atrial Arrhythmia ◽  
Class I ◽  
Protein Losing Enteropathy ◽  
Fontan Patients

Abstract Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

scholarly journals Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience

2019 ◽  
Author(s):  
Xiaobing Liu ◽  
Haiyun Yuan ◽  
Jimei Chen ◽  
Jianzheng Cen ◽  
Zhiqiang Nie ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Growth Potential ◽  
Total Cavopulmonary Connection ◽  
Long Term Outcomes ◽  
Protein Losing Enteropathy ◽  
Significant Difference ◽  
Cavopulmonary Connection ◽  
Extracardiac Fontan

AbstractOBJECTIVESThe modified extracardiac Fontan of direct total cavopulmonary connection (dTCPC) with entirely autologous vessels is a better solution in selected patients with functionally univentricular hearts because it combines the advantages of a tubular connection and of full growth potential. We investigated the mid-term to long-term outcomes of the physical condition of patients who had the dTCPC and assessed the growth potential of the autologous dTCPC pathway.METHODSFrom July 2005 to June 2014, 31 patients, aged 1.8–14.0 years, underwent a modified extracardiac Fontan with dTCPC at our institution. Twenty-two patients underwent a 1-stage dTCPC and 9 patients underwent a 2-stage dTCPC. The preoperative and postoperative data were reviewed retrospectively.RESULTSThere were 17 (54.8%) boys and 14 (45.2%) girls, with a median age of 6.4 years (range 1.8–14.0 years) and a mean weight of 17.8 kg (range 9–41 kg). The mean follow-up period was 6.0 years (range 2.1–10.2 years). There was 1 early death and 2 late deaths. The event-free survival rate for the 31 patients was 88.9% at 10 years during the follow-up period. Late protein-losing enteropathy, thromboembolism, arrhythmia or heart failure were not observed. There was a significant difference between the preoperative and postoperative data for body mass index and the diameters of the autologous dTCPC pathway and pulmonary artery branches, confirming that the growth potential of the autologous dTCPC pathway was realized.CONCLUSIONSThe dTCPC procedure could be performed with satisfactory midterm to long-term outcomes in selected patients. It has the potential of retaining the advantage of the extracardiac Fontan operation together with the potential for growth and the avoidance of prosthetic materials.

scholarly journals Long-term therapeutic effect of Fontan conversion with an extracardiac conduit

2019 ◽  
Vol 57 (5) ◽  
pp. 951-957 ◽  
Author(s):  
Takaya Hoashi ◽  
Masatoshi Shimada ◽  
Kenta Imai ◽  
Motoki Komori ◽  
Kenichi Kurosaki ◽  
...  
Keyword(s):  
Survival Rate ◽  
Therapeutic Effect ◽  
Survival Curve ◽  
Preoperative Evaluation ◽  
Fontan Operation ◽  
Actuarial Survival ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit

Abstract OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9–29.2) and 17.8 years (15.4–20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P &lt; 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P &lt; 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P = 0.19). CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.

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