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BACKGROUND: Deterioration of pulmonary function after hematopoietic stem cell transplantation (SCT) is a well-known late effect of this treatment, but the course of pulmonary function over time is less clear. The aim of our study was to establish both the prevalence and course of pulmonary function abnormalities in children following SCT. METHODS: Thirty-nine of 106 patients, who visited a post-SCT late effects clinic and who underwent a pulmonary function test (PFT) both before and at least twice after SCT were included in this study. Forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and total lung diffusion capacity (TLCO) were determined and recorded as percentage predicted for age, sex, and length matched controls. Values of less than 80% of predicted were considered abnormal. Change in PFT parameters over time was determined by comparing the mean PFT parameter in our group at three different time points: pre-SCT, < or =1 year post-SCT (SCTpost1) and >1 year post-SCT (SCTpost2). RESULTS: After SCT restrictive and/or diffusion abnormalities are most prevalent (45% and 76% at SCTpost1, respectively). A significant decrease of TLC (-9.7%) and TLCO (-20.3%) was observed during the first year after SCT, with improvement over time, but no normalization. Obstructive lung disease was less common (6% at SCTpost1). Clinical signs of lung function impairment were rare. CONCLUSIONS: Restrictive and diffusion lung function disorders are common after SCT. They improve over time but do not normalize. As only a few patients with pulmonary function abnormalities had clinical signs of lung function impairment, the clinical relevance of performing long-term follow-up of PFT is questionable.  相似文献   

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BACKGROUND: We sought to determine the prevalence of abnormal pulmonary function tests (PFTs) in a cohort of children who had received whole lung irradiation (WLI) for treatment of metastatic disease. PROCEDURE: This was a retrospective (1988-2003) chart review that included all children treated at our institution with WLI who had undergone PFT. Data abstracted included oncologic diagnosis, radiation dose and fractionation, spirometry (FVC, FEV1, FEV1/FVC, FEF25%-75%), plethysmography (TLC, FRC, RV, RV/TLC), diffusing capacity (DLCO), and respiratory muscle strength (MIP, MEP). PFTs were normalized according to standard deviation (Z) scores. RESULTS: Thirty patients were identified who had one or more PFT. The incidence of mild, moderate, or severe reductions in FEV1 was 30%, 10%, and 10%, respectively, with 50% having normal FEV1. Seventeen percent of patients had mild reduction in total lung capacity (TLC), while 13% and 30% had moderate or severe reductions. Thirty-eight percent had mild reductions in diffusing capacity, while 29% and 14% had moderate or severe reductions. CONCLUSIONS: Pulmonary function abnormalities were common in this cohort of children treated with WLI, and may be progressive in nature. Further studies are warranted to identify patients at highest risk.  相似文献   

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Pulmonary complications are among the most frequently encountered sequelae of pediatric hematopoietic stem cell transplantation (HSCT). Non-infectious complications are becoming increasingly more common in this unique population. This review addresses the diagnosis and management of non-infectious manifestations of lung disease in pediatric HSCT patients and briefly discusses the long-term pulmonary function of childhood HSCT survivors.  相似文献   

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PURPOSE: To assess health and musculoskeletal function in survivors of pediatric sarcomas. PATIENTS AND METHODS: Thirty-two individuals treated for Ewing sarcoma family of tumors (ESFT), rhabdomyosarcoma (RMS), or non-rhabdomyosarcoma soft tissue sarcomas (NR-STS) with multi-modality therapy were enrolled on this cross-sectional study. Median age at the time of therapy was 15.4 years (range 7.1-34.2), median age at the time of analysis was 37.4 years (17.5-55.4), and median duration of time elapsed from completion of therapy was 17.3 years (2.9-32.6). Participants underwent assessments of musculoskeletal functioning, cardiac function, metabolic and lipid analyses, renal and gonadal function, and psychological evaluation. RESULTS: This cohort of sarcoma survivors shows expected locoregional limitations in function of the area affected by sarcoma, and impaired global musculoskeletal functioning as evidenced by limited endurance and limited overall activity levels. The cohort also demonstrated substantial rates of cardiac dysfunction, elevated body fat index, hyperlipidemia, chronic psychological distress, and infertility in men (76%) and premature menopause (49%) in women. CONCLUSION: Sarcoma survivors demonstrate diminished locoregional and global musculoskeletal functioning which likely limit occupational opportunities and socioeconomic health. In addition, the combination of diminished cardiac reserve, limited activity levels, and lipid dysregulation in sarcoma survivors suggests that this population is at increased risk for cardiovascular disease, even many years following completion of sarcoma therapy. Sarcoma survivors may benefit from life long follow-up for cardiovascular disease and from occupational counseling upon completion of therapy.  相似文献   

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HSCT is associated with a high risk of late morbidity. The aim of this study was to evaluate the frequency, time frame, risk factors, and possible etiology of pulmonary dysfunction following allogeneic HSCT in childhood. We evaluated the pulmonary function of 51 HSCT patients (>6 yr), by including FVC and FEV1 values prior to (baseline) and annually up to five yr after HSCT. A Cox proportional hazards model was used to analyze the risk factors for a pulmonary event. Over half (59%) of the patients developed pulmonary dysfunction, mainly consisting of restrictive abnormalities. Acute GvHD (HR 4.31, 95% CI 1.47–12.63), chronic GvHD (HR 10.20, 95% CI 2.42–43.03), and an abnormal baseline pulmonary function (HR 4.82, 95% CI 1.02–22.84) were associated with post‐transplant dysfunction. FEV1 (p < 0.001) and FVC (p < 0.001) declined significantly by 12 months after HSCT and both remained below the pre‐HSCT level at up to four yr post‐transplantation. HSCT in childhood is associated with early and persistent restrictive impairment of pulmonary function. Patients with extensive chronic GvHD are particularly vulnerable to severe pulmonary dysfunction. Scheduled pulmonary function testing is warranted as part of the follow‐up of survivors of HSCT in childhood.  相似文献   

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To identify risk factors associated with the development of diabetes mellitus and to describe the prevalence of diabetes in pediatric hematopoietic cell transplant (HCT) survivors. The follow-up records of 748 patients who survived for at least 2 years after pediatric HCT were retrospectively reviewed for diagnosis of diabetes. Risk factors for type 2 diabetes were analyzed using multivariate statistics. Among 748 patients with a median of 11 years of follow-up, 38 developed diabetes after HCT. Four patients (three leukemia and one neuroblastoma) developed type 1 diabetes 8 to 14 years after HCT, at between 10 and 19 years of age. Thirty-four patients (32 leukemia and 2 aplastic anemia) developed type 2 diabetes 1 to 24 years after HCT, at between 11 and 41 years of age. Of the 34 patients with type 2 diabetes, 23 were non-Hispanic white, 3 had experienced asparaginase toxicity (hyperglycemia and/or pancreatitis), and 26 had a family history of diabetes. Risk factors associated with type 2 diabetes were diagnosis of acute or chronic leukemia, race/ethnicity other than non-Hispanic white, family history of diabetes, and asparaginase toxicity. The prevalence of type 1 diabetes among all surviving patients was 0.52%, or three times higher than the general U.S. population. The prevalence of type 2 diabetes was 9% among leukemia survivors and 2% among aplastic anemia survivors, both higher than expected. Pediatric HCT survivors are more likely to develop diabetes than the general population.  相似文献   

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The data of allogeneic HSCT in nine children with acquired AA between June 1998 and July 2006 were analyzed retrospectively. The median duration of time to neutrophil and platelet engraftment was 18 and 25 days, respectively. None of the patients had primary graft failure. Two (22.2%) patients developed acute GVHD and of these, one (11.1%) was Grade 1, and the other (11.1%) was Grade 3. Although the study group was composed of higher risk patients, including six of nine resistant to previous immunosuppressive treatment, eight had multiple not irradiated or filtered transfusion histories and one of the cases was only 5/6 HLA-compatible with his donor, the five-yr overall and EFS was 100%, and all recipients are alive without any graft failure. This may be attributed to the dose adjusted use of ATG according to individual transfusion history and gradual tapering of CsA and cessation at least nine months after allogeneic HSCT.  相似文献   

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As the number of pediatric cancer survivors increases, the cost of cure (i.e., late effects) needs to be measured through a consistent mechanism. Through the use of the Children's Oncology Group's (COG) Long-Term Follow-Up (LTFU) guidelines, individualized risk profiles can be discerned for each off-therapy patient. These guidelines were used to compare and contrast risk profiles for identical twin females treated for acute lymphoblastic leukemia (ALL). The first twin was treated for high-risk leukemia and the second for standard-risk leukemia, each with different ALL protocols. Timely use of the LTFU guidelines has aided in identifying and treating their adverse late effects.  相似文献   

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We report on six patients who developed diabetes mellitus after hematopoietic cell transplantation (HCT). The prevalence in our cohort of long-term survivors after HCT performed below 18 yr of age was 3%. The median age at onset of diabetes was 22.4 yr (range 11.3-34.4). The median period between HCT and diabetes was 10.1 yr (range 5.6-22.1). Five out of the six patients received total irradiation therapy and five had other endocrinological abnormalities. The onset of diabetes in all patients was insidious and none had diabetic ketoacidosis. Body mass indexes at diabetes onset were within normal levels. The clinical and laboratory features that characterized our patients with diabetes after HCT make it difficult to classify them as having type-1 or type-2 diabetes. The relatively high prevalence of diabetes and its insidious onset in this group of patients, advocate clinicians to evaluate carefully even slight variations in fasting blood glucose, usually included in the routine biochemistry follow-up. These data also suggest that HbA1c and oral glucose-tolerance test should be added to the follow-up program of late complications if fasting blood glucose levels are slightly increased.  相似文献   

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BACKGROUND: Few studies have assessed late effects in neuroblastoma (NB) survivors, particularly those with advanced stage disease. METHODS: Retrospective analysis of a cohort of advanced stage NB survivors followed in a late effect clinic at a single institution. Screening tests to detect late effects were tailored depending on the individual's treatment exposures. RESULTS: The study included 63 survivors (31 males). The median age at diagnosis was 3.0 years. The median follow-up from diagnosis was 7.06 years. All patients had surgery and received chemotherapy, 89% received radiation therapy (RT), 62% immunotherapy, and 56% autologous stem cell transplant. Late complications were detected in 95% of survivors and included: hearing loss (62%), primary hypothyroidism (24%), ovarian failure (41% of females), musculoskeletal (19%), and pulmonary (19%) abnormalities. The majority of complications were moderate, with only 4% being life-threatening. Survivors who received cisplatin were at greater risk to develop hearing loss compared to those not so treated (OR 9.74; 95% CI: 0.9-101.6). A total dose of cyclophosphamide greater than 7.4 g was associated with ovarian failure (P = 0.02). CONCLUSIONS: Late complications occur frequently in survivors of advanced stage NB. The majority of these problems are of mild-moderate severity. Long-term follow-up (LFTU) and screening of this population is essential.  相似文献   

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BACKGROUND: The aim of our study was to evaluate the long-term effects of chemotherapy and/or radiotherapy on lung function in 75 childhood Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL) survivors several years after treatment. PATIENTS AND METHODS: We studied 37 HD and 38 NHL survivors. These patients were divided into two groups according to the treatment protocols applied. Group I consisted of 23 patients who were treated with both chemotherapy and thoracic irradiation and Group II consisted of 52 patients who were treated with chemotherapy and no thoracic irradiation. A detailed history of smoking habits, respiratory symptoms, and diseases was recorded. Complete physical examinations and pulmonary function tests [PFT, including spirometry, lung volume, and diffusion capacity for carbon monoxide (DLCO)] were performed on all subjects. RESULTS: No patients reported acute or chronic respiratory symptoms or diseases. Pulmonary function abnormality (reduced lung volume and diffusion capacity) was found in 13% of patients at a median 5 years after diagnosis. The percentage of predicted normal value of forced expiratory volume in the 1st sec (FEV(1)), residual volume (RV), and DLCO were significantly lower in Group I than these values for Group II. There were no significant differences in PFT parameters between patients with HD and NHL (P > 0.05). It appears that the risk of reduced lung function was greater the younger the patient in therapy. CONCLUSION: Chemotherapy or chemo-radiotherapy-induced pulmonary sequalae in childhood may remain asymptomatic for many years.  相似文献   

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