Limb Salvage and Amputation in Survivors of Pediatric Lower-Extremity Bone Tumors: What Are the Long-Term Implications?

2002 ◽  
Vol 20 (22) ◽  
pp. 4493-4501 ◽  
Author(s):  
Rajaram Nagarajan ◽  
Joseph P. Neglia ◽  
Denis R. Clohisy ◽  
Leslie L. Robison
Keyword(s):  
Quality Of Life ◽  
Lower Extremity ◽  
Limb Salvage ◽  
Lower Limb ◽  
Bone Tumors ◽  
Pediatric Patients ◽  
Population Based ◽  
Surgical Approaches

ABSTRACT: The past four decades have seen tremendous progress in the treatment of pediatric and adolescent cancers. As a consequence, there are increasing numbers of adult childhood cancer survivors. This has prompted investigation into the long-term consequences of cancer treatments. One group that merits special study is the survivors of lower-extremity bone tumors. Their function and quality of life may depend in part on both the surgery and the age at which it was performed. Comparisons between studies are difficult because small numbers of patients and the use of varying research designs and methods have limited research in this area. The purpose of this article is to review the major surgical approaches to lower-limb bone tumors and their impact on pediatric patients. The results show that survival is equivalent between amputation and limb salvage. Complications occur more frequently in limb salvage. The long-term outcomes of those undergoing amputation and limb salvage have not been found to be substantially different in regard to quality of life. In conclusion, prospective long-term follow-up of pediatric patients with lower-limb tumors is needed to (1) determine in a uniform manner the long-term complications, quality of life, and functionality of this population and describe differences within this patient population based on age at diagnosis and surgical procedure, (2) identify areas of concern that are amenable to intervention, and (3) provide clinicians and future patients a better understanding of the surgical options.

Abstract P162: The Baseline Ankle Brachial Index (ABI), but Not Change in the ABI, is Associated With 11-Year Change in the SF-36: The San Diego Population Study (SDPS)

Circulation ◽  
2015 ◽  
Vol 131 (suppl_1) ◽  
Author(s):  
Christina L Wassel ◽  
Matthew A Allison ◽  
Joachim H Ix ◽  
Julie O Denenberg ◽  
Dena E Rifkin ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lower Extremity ◽  
Physical Functioning ◽  
Ankle Brachial Index ◽  
Population Based ◽  
Component Score ◽  
Lower Baseline ◽  
Sf 36

Background: Lower extremity peripheral artery disease (PAD) affects approximately 9 million people in the US. Less well-recognized is that PAD, even when asymptomatic, is associated with decreased functional status and quality of life. Previous studies have largely examined associations of the ankle brachial index (ABI) and quality of life in participants with PAD. To our knowledge no studies have examined the impact of ABI and change in ABI on change in quality of life in a population-based setting. Methods: The SDPS is a population-based prospective study that evaluated non-Hispanic White, African-American, Hispanic and Asian men and women for lower extremity PAD at two examinations approximately 11 years apart. Participants completed the SF-36 questionnaire, and 802 participants had ABI and SF-36 data available at both examinations. Analyses were restricted to these participants who also had a baseline ABI<1.4. Growth curve models were used to assess the associations of baseline ABI and change in the ABI with change in the SF-36 physical component score (PCS) and mental component score (MCS) over time. Change in the ABI was defined as (follow-up ABI - baseline ABI)/baseline ABI. Results: Participants were on average 57±9 years of age at baseline, and 69±9 at follow-up. At baseline, nearly 2 percent had ABI≤0.90, and the mean±SD ABI was 1.12±0.10. After adjustment for age, sex, race/ethnicity, BMI, ever smoking, physical activity, hypertension, diabetes, and dyslipidemia, each SD lower baseline ABI was significantly associated with an average change of -0.63 points (95% CI (-0.10, -1.17), p=0.02) on the PCS. This association was marginally significant for the MCS (-0.54 points (0.14, 1.22), p=0.12). Lower baseline ABI was also associated with negative change in two SF-36 subscales, physical functioning (p=0.02) and vitality (p=0.01). Change in the ABI over 11 years was not associated with change in the MCS (p=0.97) or PCS (p=0.41). Results were similar when excluding participants with ABI≤0.90. Conclusions: A lower ABI at baseline is associated with a significantly worse physical functioning quality of life approximately 11 years later. However change in the ABI was not significantly associated with change in quality of life over this time period. Additional studies are warranted in larger samples, especially to confirm the lack of findings for change in ABI.

scholarly journals Physical Activity and Long-term Quality of Life among Colorectal Cancer Survivors—A Population-based Prospective Study

2020 ◽  
Vol 13 (7) ◽  
pp. 611-622
Author(s):  
Ruth Elisa Eyl ◽  
Lena Koch-Gallenkamp ◽  
Lina Jansen ◽  
Viola Walter ◽  
Prudence R. Carr ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Physical Activity ◽  
Colorectal Cancer ◽  
Prospective Study ◽  
Cancer Survivors ◽  
Population Based ◽  
Colorectal Cancer Survivors

scholarly journals The Effect of S-Adenosylmethionine Treatment on Neurobehavioral Phenotypes in Lesch-Nyhan Disease: A Case Report

2019 ◽  
Vol 11 (3) ◽  
pp. 256-264
Author(s):  
Ken Momosaki ◽  
Jun Kido ◽  
Shiro Matsumoto ◽  
Atsuo Taniguchi ◽  
Tomoyuki Akiyama ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pediatric Patients ◽  
Inspiratory Stridor ◽  
Involuntary Movements ◽  
Self Injury ◽  
Generalized Dystonia ◽  
To Receive ◽  
Hypoxanthine Guanine Phosphoribosyl Transferase

Lesch-Nyhan disease (LND) is an X-linked recessive disorder caused by a deficiency in hypoxanthine-guanine phosphoribosyl transferase. Patients with LND experience involuntary movements, including dystonia, choreoathetosis, opisthotonos, ballismus, and self-injury. Alleviating these involuntary movements is important to improve the quality of life in patients with LND. Many clinicians have difficulty controlling these involuntary movements in their patients, and there are no established and effective treatments. A 6-month-old boy with LND presented with generalized dystonia and self-injury behavior that was alleviated after receiving S-adenosylmethionine (SAMe). His self-injury behavior completely resolved after he received SAMe and risperidone. Although he had often experienced inspiratory stridor because of laryngeal dystonia and frequently developed aspiration pneumonitis and bronchitis, no inspiratory stridor was noted after SAMe treatment. The patient is continuing to receive SAMe and risperidone. SAMe treatment alleviates dystonic movements and improves quality of life in pediatric patients with LND. Additional research is needed to determine the long-term safety and efficacy of SAMe and its appropriate dosage.

scholarly journals Long-Term Response after 94 Cycles of Trabectedin in a Patient with Metastatic Leiomyosarcoma of the Lower Extremity

2020 ◽  
Vol 13 (1) ◽  
pp. 113-119 ◽  
Author(s):  
Magda Cordeiro ◽  
José Manuel Casanova ◽  
Joana Rodrigues ◽  
João Freitas ◽  
Ruben Fonseca ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Soft Tissue ◽  
Lower Extremity ◽  
Disease Progression ◽  
Stable Disease ◽  
Case Reports ◽  
Soft Tissue Sarcomas ◽  
Term Therapy

Leiomyosarcomas of the lower extremity are extremely rare disorders and account for 10–15% of limb soft tissue sarcomas. These tumours have poor prognosis and even in early stages, patients persist at high risk for local and distant relapse; consequently, the treatment of advanced leiomyosarcoma of the lower extremity embodies a substantial defy. We present the case of a 73-year-old man diagnosed with metastatic lower extremity leiomyosarcoma of the hallux soft tissue, and with bone, lung and lymph node metastasis. After core needle biopsy confirmation of high-grade fusocellular sarcoma, the patient underwent surgery of the primary tumour and received anthracycline-based chemotherapy. However, after a 7-month progression-free survival period, a CT revealed lung disease progression. Sequentially, the patient was treated with trabectedin (Yondelis®) at a dose of 1.5 mg/m2 resulting in complete remission of the lung metastasis and stable disease of the remaining lesions after 26 months of treatment. Afterwards, the patient started on maintenance therapy with trabectedin, resulting in long-lasting stable disease, as he was able to receive 94 cycles with very acceptable quality of life. Finally, in March 2019, the patient died of community-acquired pneumonia without objective progression disease. This clinical case reports the first patient ever treated with 94 cycles of trabectedin. Our results additionally confirm that trabectedin wields relevant oncostatic benefits with a manageable safety profile and without cumulative toxicities. Trabectedin properties enable a maintenance long-term therapy (until disease progression or unbearable toxicity), with a high impact on survival and with a preserved quality of life.

Long-term outcomes and prognostic factors in pediatric patients with severe traumatic brain injury and elevated intracranial pressure

2008 ◽  
Vol 2 (4) ◽  
pp. 240-249 ◽  
Author(s):  
Jay Jagannathan ◽  
David O. Okonkwo ◽  
Hian Kwang Yeoh ◽  
Aaron S. Dumont ◽  
Dwight Saulle ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Intracranial Pressure ◽  
Pediatric Patients ◽  
Elevated Intracranial Pressure ◽  
The Mean

Object The management strategies and outcomes in pediatric patients with elevated intracranial pressure (ICP) following severe traumatic brain injury (TBI) are examined in this study. Methods This study was a retrospective review of a prospectively acquired pediatric trauma database. More than 750 pediatric patients with brain injury were seen over a 10-year period. Records were retrospectively reviewed to determine interventions for correcting ICP, and surviving patients were contacted prospectively to determine functional status and quality of life. Only patients with 2 years of follow-up were included in the study. Results Ninety-six pediatric patients (age range 3–18 years) were identified with a Glasgow Coma Scale score < 8 and elevated ICP > 20 mm Hg on presentation. The mean injury severity score was 65 (range 30–100). All patients were treated using a standardized head injury protocol. The mean time course until peak ICP was 69 hours postinjury (range 2–196 hours). Intracranial pressure control was achieved in 82 patients (85%). Methods employed to achieve ICP control included maximal medical therapy (sedation, hyperosmolar therapy, and paralysis) in 34 patients (35%), ventriculostomy in 23 patients (24%), and surgery in 39 patients (41%). Fourteen patients (15%) had refractory ICP despite all interventions, and all of these patients died. Seventy-two patients (75%) were discharged from the hospital, whereas 24 (25%) died during hospitalization. Univariate and multivariate analysis revealed that the presence of vascular injury, refractory ICP, and cisternal effacement at presentation had the highest correlation with subsequent death (p < 0.05). Mean follow-up was 53 months (range 11–126 months). Three patients died during the follow-up period (2 due to infections and 1 committed suicide). The mean 2-year Glasgow Outcome Scale score was 4 (median 4, range 1–5). The mean patient competency rating at follow-up was 4.13 out of 5 (median 4.5, range 1–4.8). Univariate analysis revealed that the extent of intracranial and systemic injuries had the highest correlation with long-term quality of life (p < 0.05). Conclusions Controlling elevated ICP is an important factor in patient survival following severe pediatric TBI. The modality used for ICP control appears to be less important. Long-term follow-up is essential to determine neurocognitive sequelae associated with TBI.

Clinical outcome after 450 revascularization procedures for moyamoya disease

2009 ◽  
Vol 111 (5) ◽  
pp. 927-935 ◽  
Author(s):  
Raphael Guzman ◽  
Marco Lee ◽  
Achal Achrol ◽  
Teresa Bell-Stephens ◽  
Michael Kelly ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Clinical Characteristics ◽  
Pediatric Patients ◽  
Consecutive Series ◽  
Modified Rankin Scale ◽  
Surgical Data ◽  
Revascularization Surgery

Object Moyamoya disease (MMD) is a rare cerebrovascular disease mainly described in the Asian literature. To address a lack of data on clinical characteristics and long-term outcomes in the treatment of MMD in North America, the authors analyzed their experience at Stanford University Medical Center. They report on a consecutive series of patients treated for MMD and detail their demographics, clinical characteristics, and long-term surgical outcomes. Methods Data obtained in consecutive series of 329 patients with MMD treated microsurgically by the senior author (G.K.S.) between 1991 and 2008 were analyzed. Demographic, clinical, and surgical data were prospectively gathered and neurological outcomes assessed in postoperative follow-up using the modified Rankin Scale. Association of demographic, clinical, and surgical data with postoperative outcome was assessed by chi-square, uni- and multivariate logistic regression, and Kaplan-Meier survival analyses. Results The authors treated a total of 233 adult patients undergoing 389 procedures (mean age 39.5 years) and 96 pediatric patients undergoing 168 procedures (mean age 10.1 years). Direct revascularization technique was used in 95.1% of adults and 76.2% of pediatric patients. In 264 patients undergoing 450 procedures (mean follow-up 4.9 years), the surgical morbidity rate was 3.5% and the mortality rate was 0.7% per treated hemisphere. The cumulative 5-year risk of perioperative or subsequent stroke or death was 5.5%. Of the 171 patients presenting with a transient ischemic attack, 91.8% were free of transient ischemic attacks at 1 year or later. Overall, there was a significant improvement in quality of life in the cohort as measured using the modified Rankin Scale (p < 0.0001). Conclusions Revascularization surgery in patients with MMD carries a low risk, is effective at preventing future ischemic events, and improves quality of life. Patients in whom symptomatic MMD is diagnosed should be offered revascularization surgery.

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