scholarly journals Laparoscopic Revisional Surgery After Failed Heller Myotomy for Esophageal Achalasia: Long-Term Outcome at a Single Tertiary Center

Author(s):  
Giovanni Capovilla ◽  
Renato Salvador ◽  
Luca Provenzano ◽  
Michele Valmasoni ◽  
Lucia Moletta ◽  
...  

Abstract Background Laparoscopic Heller myotomy (HM) has gained acceptance as the gold standard of treatment for achalasia. However, 10–20% of the patients will experience symptom recurrence, thus requiring further treatment including pneumodilations (PD) or revisional surgery. The aim of our study was to assess the long-term outcome of laparoscopic redo HM. Methods Patients who underwent redo HM at our center between 2000 and 2019 were enrolled. Postoperative outcomes of redo HM patients (redo group) were compared with that of patients who underwent primary laparoscopic HM in the same time span (control group). For the control group, we randomly selected patients matched for age, sex, FU time, Eckardt score (ES), previous PD, and radiological stage. Failure was defined as an Eckardt score > 3 or the need for re-treatment. Results Forty-nine patients underwent laparoscopic redo HM after failed primary HM. A new myotomy on the right lateral wall of the EGJ was the procedure of choice in the majority of patients (83.7%). In 36 patients (73.5%) an anti-reflux procedure was deemed necessary. Postoperative outcomes were somewhat less satisfactory, albeit comparable to the control group; the incidence of postoperative GERD was higher in the redo group (p < 0.01). At a median 5-year FU time, a good outcome was obtained in 71.4% of patients in the redo group; further 5 patients (10.2%) obtained a long-term symptom control after complementary PD, thus bringing the overall success rate to 81.6%. Stage IV disease at presentation was independently associated with a poor outcome of revisional LHD (p = 0.003). Conclusions This study reports the largest case series of laparoscopic redo HM to date. The procedure, albeit difficult, is safe and effective in relieving symptoms in this group of patients with a highly refractory disease. The failure rate, albeit not significantly, and the post-operative reflux are higher than after primary HM. Patients with stage IV disease are at high risk of esophagectomy.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1511.2-1511
Author(s):  
M. G. Lazzaroni ◽  
F. Crisafulli ◽  
I. Debeni ◽  
C. Nalli ◽  
L. Andreoli ◽  
...  

Background:A possible increase in neurodevelopmental (ND) and learning disorders (LD) in the offspring of mothers affected by SLE have been suggested in some studies, along with the identification of different possible risk factors. Azathioprine (AZA) is commonly used during pregnancy, based on its non-teratogenicity and extended experience in women with different diseases. However, a few small studies suggested an association between in utero exposure to AZA and possible increased frequency of ND/LD in children, indirectly derived from increased request of supportive educational services.Objectives:To evaluate the medium-long term outcome in terms of ND/LD in children of school age (≥6 years) born to SLE women treated with AZA during pregnancy, as compared to that of children born to SLE mothers not treated with AZA during pregnancy.Methods:Data from our Pregnancy Clinic registry were collected for prospectively followed pregnancies of SLE women treated with AZA (cases) and compared to pregnancies of SLE women not treated with AZA (controls), that were matched for age at pregnancy, presence of renal involvement and aPL positivity. SLE patients (cases and controls) were interviewed by phone to collect data about their children, focusing on the presence of ND/LD certified by Neuropsychiatrists.Results:Data were collected for 14 SLE mothers in the AZA group and 31 in the control group, with similar age at pregnancy (30.3±5.21 vs 31.4±4.70 years, p:0.45) and frequency of renal involvement (50.0% vs 44.1%, p:0.77), aPL positivity (33.3% vs 29.4%, p:0.76) and anti-Ro/SSA positivity (27.8% vs. 26.5%, p:0.55). A SLE flare during pregnancy was more frequently recorded in the AZA group (27.8% vs. 2.94%, p:0.02). Other medications included HCQ (55.6% vs. 70.6%, p:0.36) and corticosteroids (100% vs 79.4%, p:0.08).We collected data for 18 children in the AZA group and 34 children in the control group, that had a similar mean age at the time of the interview (12.7±4.80 vs. 12.9±5.61 years, p:0.91). The two groups had also similar gestational age (37.4±2.20 weeks vs. 38.0±1.29 weeks, p:0.23), birth weight (3003±433 g vs 3011±453 g, p:0.95) and rate of male sex (61.1% vs 44.1%, p:0.38).We recorded similar frequency of ND/LD in the two groups. In particular, a ND was present in 2/18 (11.1%) of children exposed to AZA vs. 2/34 (5.88%) in the control group (p:0.60). A LD was present in 1/18 cases (5.56%) and 6/34 controls (17.6%) (p:0.40).Conclusion:The medium-long term outcome of children born to SLE mothers in the whole cohort was characterized by the presence of ND in 4/54 (7.69%) and LD in 7/52 (13.5%). ND/LD do not seem to be related to in utero exposure to AZA.Disclosure of Interests:None declared


Medicina ◽  
2021 ◽  
Vol 57 (1) ◽  
pp. 54
Author(s):  
Mario Luciano ◽  
Luca Steardo ◽  
Gaia Sampogna ◽  
Vito Caivano ◽  
Carmen Ciampi ◽  
...  

Background and objectives: Bipolar disorder (BD) is one of the most burdensome psychiatric illnesses, being associated with a negative long-term outcome and the highest suicide rate. Although affective temperaments can impact on BD long-term outcome, their role remains poorly investigated. The aims of the present study are to describe the clinical characteristics of patients with BD more frequently associated with the different affective temperaments and to assess the relation between affective temperaments and severity of clinical picture in a sample of patients with BD. Materials and Methods: A total of 199 patients have been recruited in the outpatients units of two university sites. Patients’ psychiatric symptoms, affective temperaments, and quality of life were investigated through validated assessment instruments. Results: Predominant cyclothymic and irritable temperaments are associated to higher number of relapses, poorer quality of life, higher rates of aggressive behaviors, and suicide attempts. Conversely, the predominant hyperthymic disposition was a protective factor for several outcome measures, including relapse rate, severity of anxiety, depressive and manic symptoms, suicidality, and earlier age at onset. One limitationo of the present study is that the recruitment took place in two university sites; therefore, our findings cannot be fully generalized to the whole community of BD patients. Other limitations are the lack of a control group and the cross-sectional design of the study. Conclusions: The early identification of affective temperaments can help clinicians to identify those BD patients who are more likely to show a poor long-term outcome. An early screening of affective temperaments can be useful to develop targeted integrated pharmacological and psychosocial interventions.


2016 ◽  
Vol 31 (6) ◽  
pp. 2596-2601 ◽  
Author(s):  
Helle Ø. Kristensen ◽  
Jakob Kirkegård ◽  
Daniel Willy Kjær ◽  
Frank Viborg Mortensen ◽  
Rastislav Kunda ◽  
...  

2019 ◽  
Vol 25 (1) ◽  
pp. 33-44 ◽  
Author(s):  
Susana Andrés-Pepiñá ◽  
Maria Teresa Plana ◽  
Itziar Flamarique ◽  
Sonia Romero ◽  
Roger Borràs ◽  
...  

Objective: To assess the outcome of adolescents with anorexia nervosa (AN) about 20 years after first treatment. Methods: Sixty-two women diagnosed with AN during adolescence were invited to participate. Of these 62 patients, 38 agreed to participate and were assessed with a battery of questionnaires and interviews. A control group of 30 women of similar age was also assessed. Results: Of the patients who completed the full assessment, 13 (34%) presented some degree of eating disorder (ED) at follow-up (10 (26%) met full Diagnostic and Statistical Manual of Mental Disorders (5th ed.; DSM-5) criteria for an ED and 3 (8%) showed partial remission of an ED). The remaining 25 (66%) patients had fully recovered from AN. The duration of untreated illness before admission was significantly associated with an increased risk of a current ED (odds ratio (OR) = 3.334 (1.3–8.7); p = .014). Of the patients who had recovered totally from their ED, 24% showed another psychiatric disorder. This percentage rose to 70% in patients with a current ED. Conclusion: Sixty-six percent of adolescents who completed the assessment achieved remission of their AN. Comorbidity was more common in the current ED group. The variable that best predicted complete remission was the number of years without treatment, showing the importance of detection and early intervention.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 903-903
Author(s):  
Marco Ladetto ◽  
Sonia Vallet ◽  
Paolo Corradini ◽  
Fabio Benedetti ◽  
Umberto Vitolo ◽  
...  

Abstract Introduction. i-HDS is a promising though experimental treatment for FL patients at diagnosis. We have published that i-HDS is feasible also in the context of a multicenter trial with limited toxicity and promising results (Ladetto et al, Blood, 2002). This analysis is an update at 42 months from the previous closing date. Particular attention has been devoted to late toxicities and outcome according to molecular remission (MR). Patients and methods. 92 untreated patients aged 18–60 years with stage III-IV FL requiring treatment were enrolled by 20 Italian centers between 1997 and 1999 and evaluated on an intention-to-treat basis. Inclusion criteria have been previosly described. Clinical features at diagnosis were: Ann Arbor stage IV: 84%; BM involvement: 80%; extranodal and extra-BM disease: 55%; bulky mass: 51%; elevated serum LDH: 37%; "B" symptoms: 30%; leukemic disease: 12%; aaIPI ≥ 2: 37%. The i-HDS schedule included 2APO, 2DHAP, Etoposide 2g/sqm, Methotrexate 8g/sqm and Cyclophosphamide 7g/sqm with PBPC collection. The myeloablative regimen was Mitoxantrone 60mg/sqm + Melphalan 180mg/sqm followed by 5–8x106 CD34+ cells/kg. Minimal residual disease analysis with the bcl-2/IgH rearrangement was available in 47% of patients. Results: 87% of patients completed the planned treatment. CR rate was 88%. Currently the median follow-up is 62 months. Late toxic effects included five myelodysplastic syndromes and /or secondary leukemias (MDS/2AL) all occurring within two years from the end of treatment. In 4 of 5 cases MDS/2AML occurred in subjects already re-treated for disease relapse. Two fatal solid tumors (one gastric cancer and one lung cancer) were also recorded. Long-term hematopoietic fuction was satisfactory in all patients not developing MDS/2AML. Heart failure occurred in three patients, always manageable with oral medical treatment. The projected overall survival and progression-free survival (PFS) at 7,5 years are 74% and 56%. Notably, following i-HDS, patients with aaIPI≥2 had an outcome comparable to those with aaIPI ≤ 1 (p=NS). Attainement of MR in the first year following i-HDS was highly predictive even for the long term outcome. Among patients achieving MR the relapse rate (RR) was 13% while in those failing to achieve MR was 81% (p<0001). Conclusions. This long-term analysis indicates the following: a) i-HDS allows long-term PFS in 56% of advanced FL patients regardless of the aaIPI. This result compares favorably with those achieved with Rituximab-free non-intensified therapy at least for high-risk patients; b) MR achievement is a powerful indicator for prolonged PFS; c) although severe side effects occurred in this series, relapse remains the major cause of treatment failure. Thus, treatment implementation is required. Rituximab inclusion is probably one of the most feasible approaches, suitable for multicenter trials. The new GITMO trial, comparing CHOP vs i-HDS (both supplemented with Rituximab), currently ongoing for high-risk (aaIPI≥2) FL patients, will help clarifying wheter dose-intensification has still a role in the monoclonal antibody era.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 7100-7100
Author(s):  
J. S. Moreb ◽  
D. Salmasinia ◽  
C. Cline ◽  
E. Rosenau

7100 Background: Poor peripheral blood stem cell (PBSC) mobilization has been reported as an obstacle to autologous stem cell transplant (ASCT). Plerixafor (AMD3100) has been recently approved as a mobilization agent for PBSC in the setting of ASCT. Methods: We retrospectively analyzed the data on patients who received plerixafor in our institution over two-year period. Four lymphoma patients received the drug in their first cycle of mobilization and 17 patients (hard to mobilize, HTM) as a rescue after failing to achieve cell target using G-CSF alone. These patients include 8 multiple myeloma (MM) and 9 lymphoma patients. A control group of 26 randomly picked MM and lymphoma patients who were good mobilizers and received ASCT during the same period were used for comparison. Results: Sixteen of the 17 HTM patients proceeded to ASCT with median CD34+ cell dose of 3.68 X 106/kg (range, 1.88–5.01 X 106), and two of them had tandem transplants. All MM patients achieved minimum cell dose for two ASCTs. One MM patient died of progressive disease prior to ASCT. In comparison to the control group, plerixafor patients tended to have lower median CD34+ cell dose/kg collected and higher number of apheresis days, however the content of CFU-GM/kg on 1st day of apheresis was either equal or higher in the plerixafor group versus the control. The length of hospital stay, number of serious bacterial infection, time to granulocyte engraftment (AGC > 500) and long-term hematopoietic recovery at ≥ 12 mo post ASCT were not different among the two groups. Time to platelet recovery > 20,000/mm3 was similar for the MM patients, while more delayed for the plerixafor mobilized lymphoma patients (24 versus 12 days in the control group). One lymphoma patient in the control died of transplant-related complications before engraftment and none in the plerixafor group. Disease relapse at 12 months post ASCT was 0 and 10 % for plerixafor and control MM patients, respectively, and 46 and 20 % for lymphoma patients. However, the overall survival for both groups was not significantly different. Conclusions: all poor mobilizers were able to obtain adequate transplant CD34+ cell dose by using plerixafor and G-CSF. In general, patients mobilized with plerixafor had similar post transplant course and long-term outcome. [Table: see text]


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 3603-3603
Author(s):  
Ji Zhu ◽  
Xinchen Sun ◽  
Anwen Liu ◽  
Yaqun Zhu ◽  
Tao Zhang ◽  
...  

3603 Background: Adding UGT1A1-guided irinotecan to capecitabine-based neoadjuvant chemoradiotherapy (CRT) significantly increased the pathological complete response (pCR) rate nearly doubling [J Clin Oncol. 2020 Dec 20;38(36):4231-4239]. Here, results of long-term outcome are reported. Methods: Eligible patients with clinical stage II/III rectal adenocarcinoma, UGT1A1 genotype *1*1 or *1*28 were randomized to the control group: pelvic radiation of 50 Gy/25 fractions with concurrent capecitabine, followed by a cycle of oxaliplatin and capecitabine; or the experimental group: radiation with capecitabine combined with weekly irinotecan 80 mg/m2 for patients with *1*1 or 65 mg/m2 for patients with *1*28, followed by a cycle of irinotecan and capecitabine. Surgery was scheduled for 8 weeks after completion of CRT. Five cycles of adjuvant XELOX chemotherapy were administered regardless of the pathologic result. Patients were stratified by UGT1A1 genotype (*1*1 vs. *1*28) clinical T stage (cT3 vs. cT4) and tumor distance from the anal verge (≤5 cm vs. > 5 cm). The primary end point of pCR was reached. Survival time was calculated from the date of randomization to the date of event or the last follow-up. Secondary endpoints were defined as local failure for local control (LC), tumor recurrence or death from any cause for disease-free survival (DFS), and death from any cause for overall survival (OS). Results: Of the 360 patients initially enrolled, 356 were evaluated as the modified intention-to-treat population (n = 178 in both groups). A total of 311 patients underwent surgery and pCR was achieved in 80 patients, another 10 patients undergo a watch-and-wait approach after achieving cCR. With a median follow-up time of 48 months (Q25-Q75, 41-55 months), 57 deaths (33 and 24), 17 local failures (11 and 6) and 69 distant metastases (37 and 32) were observed, respectively. Overall, the 4y LC rate were 93% and 96% in control and experimental groups, with estimated LC HR of 0.53 (95% confidence interval [CI], 0.20-1.43), the 4y DFS rates were 69% and 74% (HR = 0.74, 95% CI 0.49-1.10), and the 4y OS were 80% and 85%, (HR = 0.70, 95% CI 0.42-1.19), respectively. In the subgroup analysis, irinotecan showed a significant improvement in DFS (HR = 0.77, 95% CI 0.61-0.98) and OS (HR = 0.71, 95% CI 0.51-0.98) in UGT1A1 *1*1 patients. Conclusions: The addition of irinotecan to standard capecitabine-based CRT had a tendency towards improving LC, DFS, and OS, but without reaching statistical significance. UGT1A1 *1*1 patients seem to benefit the most from irinotecan. Molecular studies and subsequent therapies should be considered. Clinical trial information: NCT02605265.


2016 ◽  
Vol 124 (5) ◽  
pp. 1221-1227 ◽  
Author(s):  
Eun-Hee Kim ◽  
Mi-Sun Yum ◽  
Young-Shin Ra ◽  
Jun Bum Park ◽  
Jae Sung Ahn ◽  
...  

OBJECT Moyamoya disease (MMD) is an idiopathic cerebrovascular occlusive disorder prevalent in East Asia. In the pathogenesis of MMD, the important role of genetic factors is being elucidated, and RNF213 has recently been identified as a susceptibility gene for MMD. The aim of this retrospective study was to investigate the RNF213 genotype in patients with MMD and to determine their genotype-phenotype associations. METHODS The study involved 165 Korean MMD patients from 155 unrelated families who were diagnosed with MMD at a single center from 1995 to 2013. Their demographic, radiological, and clinical findings were evaluated. Direct sequencing of the major RNF213 single nucleotide polymorphisms was performed. The association of the common RNF213 variant with MMD risk was evaluated using historical controls for comparison. Correlations between RNF213 genotype and phenotype were statistically analyzed. RESULTS The c.14429G>A (p.R4810K) variant was identified in 125 (75.8%) of 165 MMD patients. Most patients (112) were heterozygous, and 13 patients had 2 copies of the c.14429G>A variant. A novel heterozygous variant, c.12086A>G (p.Q4029R), was found in 1 additional patient. The minor allele frequency of the c.14429G>A variant was significantly higher in the MMD group (138 [41.8%] of 330 patients) than in the control group (8 [1.36%] of 588 subjects; p < 0.001). The c.14429G>A (p.R4810K) variant significantly increased the risk of MMD in Korean patients, with an OR of 52.11 (p < 0.001) compared with controls. Moreover, c.14429G>A (p.R4810K) genotypes occurred more frequently in patients with a family history of MMD. The homozygous variant was highly associated with early-onset MMD (age at onset < 5 years), cerebral infarction at diagnosis, and cognitive impairment in long-term outcome. CONCLUSIONS The findings indicate that the c.14429G>A (p.R4810K) allele of RNF213 is strongly associated with Korean patients with MMD. The homozygous c.14429G>A (p.R4810K) variant is particularly related to early-onset MMD, severe symptomatic manifestations at diagnosis, and poor prognosis. This genotypic variant may be a useful biomarker for early-onset MMD or unstable MMD with cerebral infarction, which requires early diagnosis and revascularization treatment.


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