Long-Term Results of Bilateral Lung Transplantation in Patients With End-Stage Pulmonary Lymphangioleiomyomatosis

2019 ◽  
Vol 29 (2) ◽  
pp. 115-121 ◽  
Author(s):  
Jawad Salman ◽  
Fabio Ius ◽  
Wiebke Sommer ◽  
Thierry Siemeni ◽  
Felix Fleissner ◽  
...  

Introduction: Lymphangioleiomyomatosis (LAM) is a rare disease in women, leading to progressive deterioration of lung function and respiratory failure. We describe the outcome of patients with end-stage LAM who underwent lung transplantation at our center. Materials and Methods: The records of patients with LAM transplanted at our institution between February 1997 and May 2015 were reviewed retrospectively. Morbidity and mortality were analyzed, and actuarial survival was calculated using Kaplan-Meier methods. The cumulative survival of transplant patients with LAM at our center was compared with survival after transplantation due to different diseases at our center and the results of the International Society for Heart and Lung Transplantation. Quality of life was assessed by a patient self-report at the end of the first postoperative year. Results: During the study period, 25 patients underwent lung transplantation for LAM. All patients were women with a mean age of 50 (9) years. Thirteen patients (52%) had undergone previous thoracotomy. All patients (100%) received bilateral lung transplantation. One (4%) case of in-hospital mortality occurred and 9 (36%) late deaths. Two (8%) cases of late death were due to chronic lung allograft dysfunction. The 1-, 3-, and 5-year survival rates were 92%, 84%, and 76%, respectively. Quality-of-life ratings were above the normal in all eight 36-Item Short Form Health Survey subscales 1 year after transplantation. Conclusions: Lung transplantation offers a valuable therapy for patients with end-stage pulmonary LAM.

CHEST Journal ◽  
2005 ◽  
Vol 128 (3) ◽  
pp. 1371-1378 ◽  
Author(s):  
Margaret W. Gerbase ◽  
Anastase Spiliopoulos ◽  
Thierry Rochat ◽  
Marc Archinard ◽  
Laurent P. Nicod

CHEST Journal ◽  
1994 ◽  
Vol 105 (3) ◽  
pp. 911-917 ◽  
Author(s):  
Jan J.V. Busschbach ◽  
Frank T. de Charro ◽  
Paul E. Horikx ◽  
Jules M.M. van den Bosch ◽  
Aart Brutel de la Rivière

2021 ◽  
Vol 30 (3) ◽  
pp. 178-183
Author(s):  
Kate McEwen ◽  
Lyndell Brodie

Lung transplantation is a well-established treatment for a variety of end-stage pulmonary diseases. However, the journey of a lung transplant recipient is complex and multifaceted. Silicosis is a rare indication for lung transplantation, but no other treatment is yet available for this disease in its end stages. This Australian case study presents a 52-year-old man with silicosis who received bilateral lung transplantation. The patient was frequently noncompliant with noninvasive ventilation therapy and experienced the complication of type 2 respiratory failure. Patient education and support provided, particularly around medication management following transplantation surgery, are discussed here. The patient's social situation and its implications for both him and his family are also considered.


Author(s):  
A. R. Propp ◽  
E. N. Degovtsov ◽  
S. A. Nikulina

Aim. Compare the short-term and long-term results of the traditional and original methods of terminoterminal pancreaticojejunostomy with subtotal resection of the pancreatic head.Materials and methods. The results of subtotal resection of the pancreatic head in 20 patients with chronic pancreatitis with pancreatic duct obstruction at the level of the head are analyzed. In 10 cases at the final stage of the operation, the traditional terminoterminal pancreaticojejunostomy was performed, in 10 – in the original way.Results. Biliary tract decompression was performed in 65% of patients. The average duration of operation from original terminoterminal pancreaticojejunostomy operation was 170 min. [165; 180], intraoperative blood loss – 210 ml [200; 240], the average duration of postoperative hospital stay – 16 days [14; 17]; there were no mortality. After 5 years, in all patients who underwent the original terminoterminal pancreaticojejunostomy, the expressiveness of pain according to the developed ten-point scale was <4 points. Frequency for the first time diagnosed diabetes was 50–60%. The need for enzyme drugs was noted in 37.5–40% of cases. The frequency of complications requiring repeated surgical interventions is 12%, persistent disability – 50%. The quality of life indicators for Medical Outcome Study Short Form-36 and European Organisation for Research and Treatment of Cancer quality of life questionnaire – C30 exceeded those of the traditional method of completing the surgery by 3.9–8.4% and 9.3–16.7%.Conclusion. The original way of a single-row terminoterminal pancreaticojejunostomy with subtotal resection of the pancreatic head allows to reduce averages operative time and postoperative hospital stay. The risk of hemorrhagic complications from the resected pancreatic head does not decrease (10%). After 5 years, an insignificant advantage of the original method of terminoterminal pancreaticojejunostomy compared with the traditional one was noted. The incidence of permanent disability was 15% less, the quality of life indicators for the Medical Outcome Study Short Form-36 and European Organisation for Research and Treatment of Cancer quality of life questionnaire – C30 were better.


Author(s):  
Veronica Rossi ◽  
Serena Tammaro ◽  
Martina Santambrogio ◽  
Mariangela Retucci ◽  
Francesca Gallo ◽  
...  

This study describes the case of an 18-years-old male affected by severe COVID-19, who was receiving bilateral lung transplantation (LT), after 71 days of mechanical ventilation and 55 days of veno-venous extracorporeal membrane oxygenation. From post-operative day 2, early mobilization and physiotherapy treatments were performed. Weaning from mechanical ventilation, the use of non-invasive ventilation and tracheostomy management were included in the treatment. Forty-five days after LT the patient was discharged at home, showing improvements in terms of functional and respiratory parameters, quality of life and mood. While evidences about physiotherapy treatments in lung transplantation post severe COVID-19 remain limited, early approach and a multidisciplinary team may be considered key elements for functional recovery of these subjects.


2019 ◽  
Author(s):  
Petra ◽  
John S. Ogrodniczuk ◽  
Joanna Cheek ◽  
David Kealy ◽  
Ingrid Sochting

Background: Despite much attention in the clinical literature, research on alexithymia in the treatment setting has only recently gained traction. While several reports indicate limited benefit from therapy amongst patients with high alexithymia, this seems to be less so in the context of group therapy. This study considers a specific aspect of the group therapy process - family re-enactment - infacilitating improvement in overall quality of life for patients with high levels of alexithymia.Subjects and methods: Family re-enactment was examined as a potential mediator of the relationship between alexithymia and change in quality of life among 50 patients who completed treatment in an intensive, integrative group therapy programme. Patients completed three self-report measures: Toronto Alexithymia Scale-20 (baseline), Quality of Life Inventory (baseline, post-therapy), and Therapeutic Factors Inventory-Short Form (week 8). Regression with mediation analysis was employed using the change scorefor the QOLI as the dependent variable, alexithymia scores as the independent variable, and the family re-enactment score as the mediator; baseline quality of life was included in the model as a control variable.Results: Family re-enactment emerged as a significant mediator of the relationship between alexithymia and treatment outcome, implicating it as a contributing mechanism of change for alexithymic patients who participate in group therapy.Conclusion: Patients with higher levels of alexithymia (in particular, difficulty identifying and describing feelings) were more likely to positively endorse aspects of family re-enactment during group therapy, which in turn were significantly associated with greater improvement in patients’ overall quality of life.


2019 ◽  
Vol 88 (3-4) ◽  
pp. 115-124
Author(s):  
Matej Keršič ◽  
Maruša Keršič ◽  
Tina Kunič ◽  
Matija Barbič ◽  
Ivan Verdenik ◽  
...  

Background: The aim of our study was to report the extended long-term results of the use of tension-free vaginal tape (TVT) and trans-obturator tape (TOT) for the treatment of female urinary stress incontinence (SUI) at the Division of Gynaecology and Obstetrics / UMC Ljubljana. There are few data on this topic in the literature. Our aim was to find out whether and how the procedure improved the patients’ quality of life and for how long, whether the patients had complications after the procedure, and how this type of procedure affected the long-term results.Methods: A retrospective clinical trial comparing the use of TVT and TOT was carried out from January to August 2017 and included all the patients operated on at the Division of Gynaecology and Obstetrics / UMC Ljubljana with TVT or TOT procedure for stress or mixed urinary incontinence (UI) associated with urethral hyper mobility (the stress component was clinically predominant). The exclusion criteria were more than 10 years from procedure, age more than 80 years in 2016, previous anti-incontinence surgery and/or pelvic organ prolapse more than stage I on POP–q in any vaginal compartment. After inclusion and exclusion criteria, 1104 patients were sent quality-of-life questionnaires (PGI-S, PGI-I, SANDVIK SEVERITY SCALE, UDI-6, IIQ-7, ICIQ-UI Short Form (Slovenian)) with questions about the diagnosis, procedures, complications, reoperations, post-operative results, and satisfaction with the procedure. Till August 2017 (6 months after sending) we received 466 questionnaires (42.2 % response rate). After 225 questionnaires were excluded due to incomplete data, 241 questionnaires were analysed.Results: In the analysed group of patients (N = 241), 189 (78 %) had TOT and 52 (22 %) had TVT. Our retrospective study has confirmed that the efficacy and safety of TOT and TVT in the surgical treatment of SUI are comparable. The TOT and TVT groups did not differ significantly from each other in PGI-S, PGI-I, SANDVIK SEVERITY SCALE, UDI-6, IIQ-7, and ICIQ-UI Short Form or in postoperative complication rate. Repeat surgery was needed in 25/189 (13.2 %) TOT patients and 12/52 (23.1 %) TVT patients; p = 0.082. Urinary retention appeared in 18/189 (9.5 %) TOT patients and 7/52 (13.5 %) TVT patients; p = 0.411. Mesh erosion/inflammation appeared in 12/189 (6.3 %) TOT patients and 2/52 (38 %) TVT patients; p = 0495.Conclusion: We can conclude that the efficacy and safety of TOT and TVT in the surgical treatment of SUI are comparable. The choice of the technique should be based on the relative pros and cons of techniques and the surgeon’s experience.


2019 ◽  
Vol 1 (2) ◽  
Author(s):  
FATHUL RISKY

Introduction. Chronic kidney disease (CKD) as a result of structural and functional renal failure has a high progressivity leading to an end stage renal disease (ESRD), thus a therapy is needed to replace the renal function such as hemodialysis. Long term hemodialysis therapy affects many aspects of life and degrading the quality of life of the patient. Method. This cross-sectional study was conducted at Chasan Boesoirie Hospital, Ternate. Primary data, such as socio-demography were taken through history taking and medical records. Quality of life was assessed using Kidney Disease Quality of Life - Short Form (KDQOL-SF)-36 TM which has been used to measure quality of life of patients undergoing hemodialysis. Results. From this study a sample of 77 patients was obtained, of which 41 were male (53.25%) and 36 were female (46.75%). Of the three quality of life components assessed, KDCS components were 78.65 ± 13.39; the MCS component 54.67 ± 7.53; and PCS components 42.47 ± 7.76. Conclusions. From the results of the study it was found that the highest KDQOL-36TM score was obtained for the kidney disease component (KDCS), then the mental health component (MCS), and the lowest score for the physical health component (PCS). Of all the variables hypothesized to affect the quality of life of kidney failure patients undergoing hemodialis, not a single variable was found to be significantly affected (P value> 0.05).Keywords: end-stage renal disease, hemodialysis, quality of life.


SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A281-A282
Author(s):  
N Foldvary-Schaefer ◽  
M J Thorpy ◽  
Y Dauvilliers ◽  
A Roy ◽  
L Tang ◽  
...  

Abstract Introduction Narcolepsy negatively impacts health-related quality of life (HRQoL). Sodium oxybate is a standard of care for the treatment of cataplexy and excessive daytime sleepiness in narcolepsy. JZP-258 is an oxybate product candidate with 92% less sodium. Efficacy and safety of JZP-258 were established in a double-blind randomized withdrawal study in adults with narcolepsy with cataplexy. Methods Participants 18-70 years of age began JZP-258 treatment during a 12-week, open-label, optimized treatment and titration period, followed by a 2-week stable-dose period (SDP). Participants were then randomized to receive placebo or continue JZP-258 treatment during a 2-week, double-blind, randomized withdrawal period (DBRWP). HRQoL assessments included the 36-Item Short Form Health Survey Version 2 (SF-36) and 5-level EuroQoL 5-Dimensions Self-Report Questionnaire (EQ-5D-5L). Results 201 participants enrolled; 134 were randomized and received at least 1 dose of double-blind study medication (efficacy population; placebo, n=65; JZP-258, n=69). Decreased scores (worsening) were observed in participants randomized to placebo compared with participants randomized to continue JZP-258 treatment for the SF-36 physical component summary (median [Q1, Q3], −1.92 [−3.46, 1.73] for placebo and −0.03 [−2.07, 2.41] for JZP-258; nominal P=0.02), SF-36 mental component summary (−1.92 [−6.28, 1.34] for placebo and 1.55 [−1.88, 3.78] for JZP-258; nominal P=0.03), and EQ-5D-5L visual analog scale (−5.00 [−10.0, 5.00] for placebo and 0 [0, 5.00] for JZP-258; nominal P=0.01). No change was observed in the EQ-5D-5L crosswalk index (0 [−0.05, 0.03] for placebo and 0 [−0.01, 0.03] for JZP-258; nominal P=0.39). The overall safety profile of JZP-258 was similar to sodium oxybate. Conclusion HRQoL worsened in those randomized to placebo during DBRWP but remained stable in participants who continued JZP-258 treatment. Support Jazz Pharmaceuticals


Rheumatology ◽  
2019 ◽  
Vol 59 (2) ◽  
pp. 398-406 ◽  
Author(s):  
Boyang Zheng ◽  
Tatiana Nevskaya ◽  
Carl A Baxter ◽  
Dena R Ramey ◽  
Janet E Pope ◽  
...  

Abstract Objective To determine whether skin score changes are associated with changes in overall disease severity, function and quality of life in early dcSSc patients. Methods A total of 154 and 128 dcSSc patients from the Canadian Scleroderma Research Group database with 1 and 2 year follow-up and a disease duration ⩽5 years without end-stage organ damage and/or significant comorbidity at the initial visit were included. Skin was assessed using the modified Rodnan skin score (mRSS) and disease severity by the summed Medsger disease severity score (DSS) (without skin domain), physician and patient global assessments, function [HAQ disability index (HAQ-DI)] and quality of life [36-item Short Form Health Survey (SF-36) physical component summary (PCS)]. Analyses were repeated in patients with a disease duration ⩽3 years. Results At 2 years, 64 (50%) patients had improved skin (mRSS decrease of ⩾5 points and/or ⩾25%). Skin improvers had improved summed DSS (P = 0.002); better physician global assessments of disease activity, severity and damage (all P ⩽ 0.003); better HAQ-DI (P = 0.001) and SF-36 PCS (P = 0.005). Changes in the mRSS were positively correlated with changes in summed DSS (P = 0.006) and other disease outcomes. In the 26 (20.3%) patients with worsened skin (mRSS increase of ⩾5 points and/or ⩾25%), the summed DSS and physician global assessments were worse (P = 0.01 and P ⩽ 0.009, respectively). In the subgroup with a disease duration ⩽3 years, similar associations were found. Conclusion At 1 and 2 years, overall disease improvement parallels skin improvement in early dcSSc. This is important for prognosis and reflects the value of mRSS as an outcome measure in trials with these patients.


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