scholarly journals Combined Sodium Dimercaptopropanesulfonate and Zinc versus D-penicillamine as First-line Therapy for Neurological Wilson’s disease

2020 ◽  
Author(s):  
Jing Zhang ◽  
Lulu Xiao ◽  
Wenming Yang
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Neurological Symptoms ◽  
Neurological Deterioration ◽  
First Line ◽  
First Line Therapy ◽  
Cell Counts ◽  
Line Therapy ◽  
Group 2 ◽  
Group 1

Abstract Background: Even though recent research has achieved significant advancement in the development of therapeutic approaches for Wilson’s diseases (WD), the current treatment options available for the neurological symptoms of WD are significantly limited and yet to be standardized. Objective: The aim of this study was to compare the course of treatment for WD patients with neurological symptoms receiving either combined sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) and zinc treatment or D-penicillamine (DPA) monotherapy as first-line therapy. Methods: The case records of 158 patients diagnosed with neurological WD were retrospectively analyzed. These patients were administrated with either intravenous DMPS + Zinc (group 1) or DPA (group 2) for 8 weeks. During the period of treatment, neurological symptoms were assessed using Global Assessment Scale (GAS), the key hematological and biochemical parameters (such as aminotransferase, serum ceruloplasmin, 24-h urine copper excretion), as well as adverse effects were recorded and analyzed. Results: 93 patients in Group 1, displayed decreased GAS scores consistently in comparison to the baseline (P < 0.01). Among them, 64 patients (68.1%) displayed a significant improvement in their neurological status after 8 weeks, while 10 patients (10.8%) experienced neurological deterioration. Among the 65 patients in Group 2, 30 patients (46.2%) displayed neurological improvements, while 21 patients (32.3%) displayed neurological deterioration. 6 patients discontinued their treatment midway due to their exacerbating neurological symptoms. A comprehensive comparison of the effectiveness of the two courses of treatment revealed that patients in Group 1 demonstrated a higher improvement ratio (P < 0.01) and lower worsening ratio of the neurological symptoms of the patients (P < 0.01) in comparison to the patients in group 2. Meanwhile, renal function, liver enzyme and the blood cell counts remained stabilized in group1. Conclusions: This study suggests that the combined therapeutic approach of treatment with DPMS and zinc should be the preferred first-line therapy in treating the neurological symptoms of WD, in comparison to the treatment with DPA as the experimental data demonstrates that it is significantly more efficient. Keywords: Wilson’s disease (WD), Sodium 2, 3-dimercapto-1-propane sulfonate (DMPS), D-penicillamine (DPA), Zinc, Global Assess

scholarly journals “Combined Sodium Dimercaptopropanesulfonate and Zinc versus D-penicillamine as First-line Therapy for Neurological Wilson’s disease”

2020 ◽  
Author(s):  
Jing Zhang ◽  
Lulu Xiao ◽  
Wenming Yang
Keyword(s):  
Therapy Group ◽  
Neurological Symptoms ◽  
Neurological Deterioration ◽  
First Line ◽  
Global Assessment Scale ◽  
First Line Therapy ◽  
Cell Counts ◽  
Line Therapy ◽  
Group 2 ◽  
Group 1

Abstract Background: Even though recent research has achieved significant advancement in the development of therapeutic approaches for Wilson’s diseases (WD), the current treatment options available for WD are still limited, expecially for WD patients with neurological symptoms. Objective: The aim of this study was to compare the course of treatment for WD patients with neurological symptoms receiving either combined sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) and zinc treatment or D-penicillamine (DPA) monotherapy as first-line therapy. Methods: The case records of 158 patients diagnosed with neurological WD were retrospectively analyzed. These patients were treated with intravenous DMPS+Zinc and in combination with oral Zinc as a maintenance therapy (Group 1) or DPA alone (Group 2) for 1 year. During the period of treatment, the neurological symptoms of the patients were assessed using the Global Assessment Scale (GAS). The key hematological and biochemical parameters of the patients (such as the levels of aminotransferase, serum ceruloplasmin, 24-h urine copper excretion), as well as adverse effects were recorded and analyzed. Results: 93 patients in Group 1, displayed decreased GAS scores consistently in comparison to the baseline (P < 0.01). Among them, 82(88.2%) patients displayed a significant improvement in their neurological status after 1 year, while 8 patients (8.6%) experienced neurological deterioration. Among the 65 patients in Group 2, 37 patients (58.5%) displayed neurological improvements, while 17 patients (26.2%) experienced neurological deterioration at 1-year follow up. 6 patients discontinued their treatment midway due to their exacerbating neurological symptoms. A comprehensive comparison of the effectiveness of the two courses of treatment revealed that patients in group 1 demonstrated a higher improvement ratio ( P < 0.01) and lower worsening ratio of the neurological symptoms of the patients ( P < 0.01) in comparison to the patients in group 2. Meanwhile, renal function, liver enzyme and the blood cell counts remained stabilized in group1. Conclusions: This study suggests that the combined therapeutic approach of treatment with DPMS and zinc should be the preferred first-line therapy in treating the neurological symptoms of WD, in comparison to the treatment with DPA, as the experimental data demonstrates that it is significantly more efficient.

scholarly journals Combined Sodium Dimercaptopropanesulfonate and Zinc versus D-penicillamine as First-line Therapy for Neurological Wilson’s disease

2019 ◽  
Author(s):  
Jing Zhang ◽  
Lulu Xiao ◽  
Wenming Yang
Keyword(s):  
Global Assessment ◽  
Neurological Symptoms ◽  
Neurological Deterioration ◽  
Assessment Scale ◽  
First Line ◽  
Global Assessment Scale ◽  
First Line Therapy ◽  
Line Therapy ◽  
Group 2 ◽  
Group 1

Abstract Background: Even though recent research has achieved significant advancement in the development of therapeutic approaches for Wilson’s diseases (WD), the current treatment options available for the neurological symptoms of WD are significantly limited and yet to be standardized. Objective: The aim of this study was to compare the course of treatment for WD patients with neurological symptoms receiving either combined sodium 2, 3-dimercapto-1-propane sulfonate (DMPS) and zinc treatment or D-penicillamine (DPA) monotherapy as first-line therapy. Methods: The case records of 158 patients diagnosed with neurological WD were retrospectively analyzed. These patients were administrated with either intravenous DMPS + Zinc (group 1) or DPA (group 2) for 8 weeks. During the period of treatment, neurological symptoms were assessed using Global Assessment Scale (GAS), the key hematological and biochemical parameters (such as aminotransferase, serum ceruloplasmin, 24-h urine copper excretion), as well as adverse effects were recorded and analyzed. Results: 93 patients in Group 1, displayed decreased GAS scores consistently in comparison to the baseline (P < 0.01). Among them, 64 patients (68.1%) displayed a significant improvement in their neurological status after 8 weeks, while 10 patients (10.8%) experienced neurological deterioration. Among the 65 patients in Group 2, 30 patients (46.2%) displayed neurological improvements, while 21 patients (32.3%) displayed neurological deterioration. 6 patients discontinued their treatment midway due to their exacerbating neurological symptoms. A comprehensive comparison of the effectiveness of the two courses of treatment revealed that patients in Group 1 demonstrated a higher improvement ratio (P < 0.01) and lower worsening ratio of the neurological symptoms of the patients (P < 0.01) in comparison to the patients in group 2. Meanwhile, renal function, liver enzyme and the blood cell counts remained stabilized in group1. Conclusions: This study suggests that the combined therapeutic approach of treatment with DPMS and zinc should be the preferred first-line therapy in treating the neurological symptoms of WD, in comparison to the treatment with DPA as the experimental data demonstrates that it is significantly more efficient. Keywords: Wilson’s disease (WD), Sodium 2, 3-dimercapto-1-propane sulfonate (DMPS), D-penicillamine (DPA), Zinc, Global Assessment Scale (GAS).

D-penicillamine versus zinc sulfate as first-line therapy for Wilson's disease

2014 ◽  
Vol 21 (4) ◽  
pp. 599-606 ◽  
Author(s):  
A. Członkowska ◽  
T. Litwin ◽  
M. Karliński ◽  
K. Dziezyc ◽  
G. Chabik ◽  
...  
Keyword(s):  
Zinc Sulfate ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy

Bevacizumab and its impact on survival for patients receiving subsequent anti-EGFR therapy: Updated results from the SA metastatic CRC registry.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 3569-3569
Author(s):  
Timothy Jay Price ◽  
Christos Stelios Karapetis ◽  
Robert Padbury ◽  
Matthew E. Burge ◽  
Amitesh Chandra Roy ◽  
...  
Keyword(s):  
Single Agent ◽  
Acquired Resistance ◽  
Patient Characteristics ◽  
First Line ◽  
Test Analysis ◽  
First Line Therapy ◽  
Line Therapy ◽  
Impact On Survival ◽  
Group 2 ◽  
Group 1

3569 Background: Debate exists as to whether first line bevacizumab effects subsequent sensitivity to anti-EGFR therapy. Authors hypothesize that initial anti-VEGF therapy may induce biological changes that then increase the risk of acquired resistance to subsequent EGFR inhibitors. Methods: A retrospective cohort study was performed to compare the characteristics and survival of patients who were treated with an anti-EGFR therapy 2nd line and beyond by two groups defined by the first line therapy; 1. chemotherapy (chemo) plus bevacizumab (bev) and 2. chemo alone. Survival for this analysis is from the time of commencing first line chemotherapy and secondly from anti-EGFR therapy. Pearson chi test analysis was performed to determine whether receiving first line bev was associated with worse overall survival (OS). Results: 348 mCRC patients who received chemo with or without bev and then an anti-EGFR therapy were studied. Patient characteristics are summarised in the table below. The significant differences between group 1. Vs. 2. were as follows; median age 63.8 years v 67.9 years (p = 0.005), lower use of single agent FU 6.4% v 19.2%, KRAS status not tested (reflecting the practice changes over time) 19.3% v 39.2%, KRAS MT 2% v 4%, and where BRAF MT status was known (11%); BRAF MT rate 23% v 0. Median OS for the 2 groups was 34.2 months, and 28.2 months respectively (p = 0.12) from first line therapy. Median OS for patients who underwent single agent anti-EGFR as subsequent therapy was also not significantly different, 31.1 months group 1 (n = 60) versus 27.7 months group 2 (n = 85), p = 0.52. Results based on commencement of anti-EGFR therapy are under way. Conclusions: Survival was not significantly different between the two groups, and the trend was towards higher OS with chemo plus bev suggesting that in our registry population, bev administration in first line therapy with chemo did not lead to a worse outcome overall for those patients subsequently receiving anti-EGFR therapy, either with chemotherapy or as a single agent. Updated results from commencement of anti-EGFR therapy will give further insights and will be presented at the meeting.

scholarly journals The Comparison of Efficacy Between Generic and Branded Imatinib in Achievement of Overall Survival and Cytogenetic Responses in CML Patients in Bosnia and Herzegovina

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5451-5451 ◽  
Author(s):  
Amina Kurtovic Kozaric ◽  
Erna Islamagic ◽  
Jerald P. Radich ◽  
Emina Suljovic Hadzimesic ◽  
Azra Hasic ◽  
...  
Keyword(s):  
Overall Survival ◽  
Side Effects ◽  
Bosnia And Herzegovina ◽  
Therapy Group ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Solidarity Fund ◽  
Group 2 ◽  
Group 1

Abstract Introduction Imatinib mesylate (Glivec, Novartis) is the first tyrosine kinase inhibitor (TKI) targeting the BCR-ABL1 fusion protein responsible for the pathogenesis of chronic myeloid leukemia (CML). Low cost generic alternatives to imatinib are an integral part of cost effective healthcare strategies for developing countries. However, the use of generics has been associated with different clinical outcomes. In this study, we compared outcomes of two groups of patients who received Glivec as first-line therapy (Group 1) to patients who received generic imatinib as first-line therapy (Group 2) in Bosnia and Herzegovina. Material and methods This was a multicenter retrospective cohort study of BCR-ABL1 positive CML patients (n = 53) in the Federation of Bosnia and Herzegovina between 1 June 2005 and 31 March 2016. Glivec was used from 01 June 2005 until 30 September 2013, when all patients had to switch to generics, which was mandated by the Federal Solidarity Fund that allocates targeted cancer therapies. The following generic imatinib was available: Anzovip (Zdravlje, Actavis) from 09/2013 to 09/2014, Meaxin (Krka) from 09/2014 to 12/2015, and Plivatinib (Pliva) from 12/2015. Patient data was collected from the database of the Federal Solidarity Fund, a subsidiary of the Federal Health Insurance Agency. Branded and generic imatinib was administered orally at dosage of 400 mg/day. Patients who were switched to nilotinib received orally 400 mg/day. Patients on Glivec included in this study started therapy from 0-6 months from time of diagnosis, while patients who started with generics did not wait for therapy. Patient variables that were collected included age, gender, town, date of diagnosis, date of start of therapy, monthly TKI dosage, adverse side effects, progression, lethal outcome, prognostic factors and diagnostic parameters, including cytogenetics and molecular testing. In September 2013, Glivec stopped being available in Bosnia and all CML patients were switched to generic therapy Anzovip. Median duration of each therapy is given in Table 1. Results We compared patients on Glivec as first-line therapy (Group 1, n=26) to patients on first-line generic imatinib (Group 2, n=27) with the follow-up period of at least three years for each group. When we compare Groups 1 and 2 using intention to treat analysis, Kaplan-Meier estimated rate of overall survival at 24 months of therapy was 88% vs. 68%, respectively (p=0.14), while 69% vs. 70% achieved CCyR (p=0.12), respectively. In Group 1, 27% (7/26) patients switched to nilotinib (treatment failure in 2 patients and side effects in 5 patients), 54% (14/26) patients switched to generics because Glivec was no longer available, and 19% (5/26) patients stopped therapy (2 patients stopped therapy and 3 patients died). Of the 7 patients who switched to nilotinib, 71% (5/7) achieved CCyR, 29% (2/7) achieved MMR and none died. Of 19 patients who stayed on imatinib, 68% (13/19) achieved CCyR, 63% (12/19) achieved MMR and 3/19 (16%) died. Of the 54% (14/26) patients who were switched from branded imatinib to generic imatinib, one patient (7%) lost complete cytogenetic response. Regarding Group 2, 52% (14/27) of patients switched to nilotinib due to treatment failure (n=8) and side effects (n=6), while 48% (13/27) of patients stayed on generics. Of patients who switched to nilotinib, 43% (6/14) achieved CCyR and 15% (2/14) achieved MMR. Of the patients who stayed on generic imatinib, 100% (13/13) achieved CCyR and 85% (11/13) achieved MMR. Conclusion Our results suggest that there was no obvious difference in the treatment efficacy between generic and branded imatinib. At 3 years, there was no significant difference in the overall suvival and achievement of CCyR between first-line Glivec and first-line generic imatinib (p=0.14, and p=0.12, respectively). * Median duration of therapy on generic imatinib Table Table. Disclosures Radich: Bristol-MyersSquibb: Consultancy; Pfizer: Consultancy; ARIAD: Consultancy; TwinStrand: Consultancy; Novartis: Consultancy, Other: laboratory contract.

d-Penicillamine versus zinc sulfate as first-line therapy for Wilson's disease

2013 ◽  
Vol 333 ◽  
pp. e140
Author(s):  
A. Czlonkowska ◽  
T. Litwin ◽  
M. Karlinski ◽  
M. Czerska
Keyword(s):  
Zinc Sulfate ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy

The Fate of Chronic Lymphocytic Leukemia Patients After Failure of Fludarabine, Cyclophosphamide, and Rituximab Regimen

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4603-4603
Author(s):  
Anna Panovska ◽  
Daniel Lysák ◽  
Lukas Smolej ◽  
Yvona Brychtova ◽  
Martin Simkovic ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Treatment Option ◽  
Conflicts Of Interest ◽  
Lymphocytic Leukemia ◽  
High Dose ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Group 2 ◽  
Group 1

Abstract Abstract 4603 Background: The clinical course of patients with chronic lymphocytic leukemia (CLL) is remarkably heterogeneous. All CLL patients who require therapy are destined to relapse. The prognosis and management of these relapsed patients differs based on the nature of the first-line therapy and the quality and duration of remission to that therapy, as well as biological prognostic factors. Currently, the first treatment option for younger and fit CLL patients is FCR (fludarabine, cyclophosphamide, rituximab) chemoimmunotherapy. Although it regards a very potent treatment option, patients inevitably relapse after the treatment and even approximately 7% of the patients, during initial FCR regimen treatment would fit the standard definition of refractory CLL. So far, little is known about the efficacy of subsequent therapy of patients who relapsed after FCR or were refractory to FCR. Therefore, the goal of this study was to analyze the fate of these patients in the context of routine hematological practice. Methods: We retrospectively analyzed the data of 119 patients (85 males; 34 females) consecutively entered into the databases of three large tertiary Czech hematological centers. All patients were indicated to receive FCR regimen in standard doses (F: 25mg/m2i.v. day 1–3; C: 250mg/m2i.v. day 1–3; R: 375mg/m2i.v.day 0 cycle 1, and 500mg/m2day 1 of all subsequent cycles). Results: The median age was 57 years (range, 31–75) at the time of the CLL diagnosis, and 60 years (range, 32–78) at the start of FCR therapy. The median follow-up was 42 months (range, 3–114). Our cohort of patients consisted of two groups of patients which received the FCR regimen as first-line therapy (Group 1; n=63) and patients receiving FCR in second or subsequent line of treatment (Group 2; n=56). With respect to the basic parameters (age, clinical stage, FISH cytogenetics, molecular genetics), the statistics of these groups did not differ significantly. In Group 1, the overall response rate (ORR) was 84%, in Group 2, 78% (p=ns) after FCR. Complete remission (CR) rate was 53% in Group 1, and 38% in Group 2 (p=0.04). The median progression-free survival (PFS) was 18.6 months for Group 1 and 14.7 months for Group 2 (p=ns). With subsequent therapy (repeated FCR, alemtuzumab, R-CHOP, rituximab plus high-dose corticosteroids) for relapsed disease after FCR, ORR was 59% in Group 1 (33% CR) and 44% in Group 2 (21% CR) (p=ns). PFS after subsequent therapy after FCR was 13.3 months (Group 1) and 5.9 months (Group 2) (p=0.01). The median OS was insignificantly shorter in Group 2 (44.5 months in Group 2 vs. not reached in Group 1; p=ns). After FCR therapy, there was no statistically significant change in cytogenetic findings, however, new occurrence of 17p deletion was observed in 5 patients. Prior to FCR therapy and during relapse after FCR, the p53 function was analyzed in 37 patients. After FCR, the new mutation of p53 was newly found in 6 patients (16%). These patients received a higher cumulative dose of FCR than the patients who did not develop the mutation (F 591 mg vs. 334 mg; C 5072 mg vs. 3375mg; R 3700mg vs. 2000 mg) (p=ns). Conclusion: Subsequent treatment for patients who relapse after FCR or are FCR refractory is very heterogeneous. Regardless of the type of therapy selected, the prognosis of these patients is poor. In addition, new p53 mutations/deletions occur after FCR. Disclosures: No relevant conflicts of interest to declare.

Nelarabine Alone or in Combination in High Risk Childhood / Adolescent and Young Adults (AYA) T- Cell Acute Lymphoblastic Leukemia

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3723-3723
Author(s):  
Benoit Brethon ◽  
Laetitia Morel ◽  
Arnaud Petit ◽  
Etienne Lengliné ◽  
Aurelie Cuinet ◽  
...  
Keyword(s):  
High Risk ◽  
Board Of Directors ◽  
Lymphoblastic Leukemia ◽  
First Line ◽  
Advisory Committees ◽  
Allogeneic Hsct ◽  
First Line Therapy ◽  
Line Therapy ◽  
First Relapse ◽  
Group 1

Abstract Introduction: T-cell acute lymphoblastic leukemia (T-ALL) represent 15-20% of childhood/adolescent young adults (AYA) ALL. An intensive chemotherapy is generally needed to obtain the same results than in B-lineage ALL. Day 8 Poor Prednisone Response (PPR) and early resistant disease (refractoriness after induction course or MRD level >10-3 at time point 1 (TP1) and/or TP2) remain particularly challenging as relapses are very difficult to treat especially if they occur early. Nelarabine is a water-soluble prodrug of araG (9-B-arabinofuranosylguanine) which is cytotoxic to T lymphoblasts due to the accumulation of araG nucleotides, especially araGTP, which result in inhibition of ribonucleotide reductase and inhibition of DNA synthesis. Nelarabine was shown to be effective and safe in phase II-III adult and pediatric ALL trials. We describe here a 7 consecutive years experience of nelarabine in “real life” in 3 pediatric / AYA centers. Methods: All children and AYA who received nelarabine in first line therapy or after relapse between 2006 and 2013 were reviewed retrospectively. Classical initial prognostic factors were collected: age, leucocytosis, CNS status, day 8 prednisone response, complete remission (CR) or not, minimum residual disease (MRD) level at TP1 and TP2. Eighty two % of the patients (pts) followed the French FRALLE 2000-T recommendations. Nelarabine, alone or in combination, was used in two groups of pts: group 1: pts in whom nelarabine is given in first line therapy because of high MRD level >10-3 at TP1 and/or TP2 (whatever the level at time of nelarabine infusion) and pts refractory to induction course, and group 2: pts in relapse. Group 1 and 2 are compared for MRD level after nelarabine, number of patients able to go to allogeneic HSCT and overall survival. Finally the safety profile was assessed. Results: 33 T-ALL patients received nelarabine alone (n= 22) or in combination (n= 11, most often with cyclophosphamide and etoposide) from 2006 to 2013. At initial diagnosis, median age was 11.6 y old [3-24], sex ratio 4.5 (M/F 27/6) and median leukocytosis 184.7.109/L [0.1-914]. These patients shared poor risk factors: CNS3 (n=8, 24.1%), D8 PPR (n=23, 69.7%), day 21 M3 bone marrow (n=13, 36.4%), no CR after one induction course (n=6, 18.2%) and MRD level > 10-3 at CR1 (n=15, 42.4%). Regarding group 1 (high MRD level at TP1 and/or TP2 n= 11, refractoriness to induction course n= 5), the status just before nelarabine was: 6 in CR1 with finally MRD <10-3, 5 in CR1 but MRD >10-3 and 5 refractory. Nelarabine was given alone in 12 patients and in combination in 4 patients. MRD level after nelarabine was <10-3 in 12/16 patients. Overall, 11 pts received an allogeneic HSCT and 13/16 (81%) are alive in CR1 at the time of the analysis with a median FU from first nelarabine infusion of 13.7 months [0.8-58.3]. Overall survival is 79.8%+/-10.5 at 5 years. Regarding group 2 (relapsed patients, n= 17), nelarabine was infused at the time of first relapse in 4 patients and in refractory first relapse or more than first relapse in 13 patients. Among these heavily pretreated patients, only 6 obtained a MRD level <10-3 leading to allogeneic HSCT but none of 6 survived. Only one patient survived in CR3 after a success of nelarabine alone and received other chemotherapy without allogeneic HSCT. Regarding toxicities, the only WHO grade III-IV observed side effects are cytopenias (n= 25, 75.8%). Others reported side effects are limited (grade I-II): fever of undetermined origin or infections (n= 7, 21.2%), neurological (n= 6 pts, 18.2%; some of them with more than one side effect: sensory neuropathy in 4, motor neuropathy in 2, headaches in 2, motor-facial neuropathy in 1, ataxia in 1) or muscular (n= 4, 12.1%; 2 myalgia, 1 myositis, 1 amyotrophia), liver toxicities (n= 4, 12.1%; 3 transaminase increases, 1 hyperbilirubinemia). Conclusions: In a non selected population of childhood / AYA high-risk T-ALL, nelarabine was very useful for poor risk patients in first line therapy. The majority of patients received nelarabine as a monotherapy. By contrast nelarabine mostly failed to improve the survival in heavily pretreated relapsed patients. Overall, this study conforts the use of nelarabine in first line T-ALL and high-risk features with acceptable tolerance. The evaluation of nelarabine in selected high-risk patients in a first line setting should be evaluated prospectively to confirm these results. Disclosures Baruchel: JAZZ: Membership on an entity's Board of Directors or advisory committees; ARIAD: Membership on an entity's Board of Directors or advisory committees; Seattle Genetics: Membership on an entity's Board of Directors or advisory committees; Onyx: Membership on an entity's Board of Directors or advisory committees.

scholarly journals Combined dimercaptosuccinic acid and zinc treatment in neurological Wilson’s disease patients with penicillamine-induced allergy or early neurological deterioration

2020 ◽  
Vol 40 (8) ◽  
Author(s):  
Xiao-Qun Zhu ◽  
Liang-Yong Li ◽  
Wen-Ming Yang ◽  
Yu Wang
Keyword(s):  
Wilson’S Disease ◽  
Combined Treatment ◽  
Wilson's Disease ◽  
Dimercaptosuccinic Acid ◽  
Neurological Symptoms ◽  
Neurological Deterioration ◽  
Zinc Treatment ◽  
Safety And Efficacy ◽  
Long Term Treatment ◽  
Early Neurological Deterioration

Abstract The clinical data of safety and efficacy of a combined treatment with dimercaptosuccinic acid (DMSA) and Zinc with 2 years’ follow-up in 60 neurological Wilson’s disease (WD) patients was retrospectively analyzed. All the patients included in the present study were newly diagnosed and initialized with D-penicillamine (DPA) treatment but were found to have either neurological deterioration or allergy, and their treatment was switched to a combined treatment of DMSA and Zinc. Fifty-one patients (85%) had the neurological symptoms improved 1 and 2 years after treatment, 7 (11.67%) experienced a stable neurological condition, and 2 (3.33%) suffered deterioration of neurological symptoms. No early neurological deterioration was observed in all patients. Twenty-five percent patients experienced mild adverse reactions which did not require a discontinuation of the DMSA and Zinc treatment. Our study confirmed the safety and efficacy of the combined DMSA and Zinc therapy as an initial and probably long-term treatment in neurological WD patients.

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