Recommendations for surveillance of pulmonary dysfunction among childhood, adolescent, and young adult cancer survivors: a report from the International Late Effects of Childhood Cancer Guideline Harmonization Group.

Childhood, adolescent, and young adult (CAYA) cancer survivors are at risk of pulmonary dysfunction. Current follow-up care guidelines are discordant. Therefore, the International Late Effects of Childhood Cancer Guideline Harmonization Group established and convened a panel of 33 experts to develop evidence-based surveillance guidelines.

Changing high-risk asthma in Memphis through partnership: Results from the CHAMP program.

Background: Children in metro Shelby County, Tennessee, have disproportionally high asthma-related health care resource use (HRU) compared with those in other regions in Tennessee.

Objective: To describe the goals, logistics, and outcomes of the Changing High-Risk Asthma in Memphis through Partnership (CHAMP) program implemented to improve pediatric asthma care in Shelby County.

Methods: CHAMP established a multidisciplinary team with dedicated medical staff and community health workers, implemented a 24/7 call line to improve access to care, established a patient data registry to address fragmented care, assigned community health educators to improve asthma education and social needs, and partnered with services to address environmental triggers and social determinants of health.

Leveraging Therapy-Specific Polygenic Risk Scores to Predict Restrictive Lung Defects in Childhood Cancer Survivors.

Unlabelled: Therapy-related pulmonary complications are among the leading causes of morbidity among long-term survivors of childhood cancer. Restrictive ventilatory defects (RVD) are prevalent, with risks increasing after exposures to chest radiotherapy and radiomimetic chemotherapies. Using whole-genome sequencing data from 1,728 childhood cancer survivors in the St.

F1099L-CFTR (c.3297C>G) has Impaired Channel Function and Associates with Mild Disease Phenotypes in Two Pediatric Patients.

(1) Background: many rare (CFTR) mutations remain poorly characterized with regard to functional consequences of the mutation. We present the clinical features of two pediatric cystic fibrosis (CF) subjects who are heterozygous for F1099L (c.3297C>G), one with G551D (a class III mutation) and one with 3849 + 10kbC->T (a class V mutation).

Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series.

The effect of ivacaftor in patients with cystic fibrosis (CF) with recurrent pancreatitis is unknown. We conducted a multicenter retrospective study of patients with CF taking ivacaftor who had a history of recurrent pancreatitis. During the first 3 months of therapy, only 1 of the 6 patients had an episode of pancreatitis, which was managed on an outpatient basis.

Recovery of Pulmonary Function after Allogeneic Hematopoietic Cell Transplantation in Children is Associated with Improved Survival.

Abnormal pulmonary function is prevalent in survivors of allogeneic hematopoietic cell transplantation (HCT). Post-transplantation recovery of pulmonary function, and its effect on survival, in children are not known. This retrospective cohort study of 308 children followed for 10 years after HCT at a single institution included 2 groups of patients.

Respiratory Care Considerations in the Childhood Cancer Patient.

This article reviews the common pulmonary complications seen in the pediatric oncology population and our approach to diagnosis, management, and therapy considerations in this specialized population, including patients receiving chemotherapy, radiation, and hematopoietic stem cell transplantation. Although infections cause the most significant complications in this population, non-infectious complications, including acute lung injury from chemotherapy or radiation, idiopathic interstitial pneumonia, diffuse alveolar hemorrhage, bronchiolitis obliterans, and cryptogenic organizing pneumonia, also occur commonly. With improvements in survival of childhood cancer, there are now a growing number of adults who are childhood cancer survivors who may be encountered by therapists in adult hospitals.

Pulmonary Function after Treatment for Childhood Cancer. A Report from the St. Jude Lifetime Cohort Study (SJLIFE).

Rationale: The relationship between treatment-related impairment of pulmonary function in adult survivors of childhood cancer and subsequent physical function has not been studied.

Objectives: In this prospective evaluation of 606 adult survivors of childhood cancer, we sought to determine the risk factors for, as well as the functional impact of, clinically ascertained pulmonary function impairment.

Methods: We measured FEV1, FVC, total lung capacity (TLC), and single-breath diffusing capacity of the lung for carbon monoxide corrected for hemoglobin (DlCOcorr), expressing the results as percent predicted and lower limit of normal (LLN) values, and we also assessed functional exercise capacity (6-minute-walk distance).

c.3623G > A mutation encodes a CFTR protein with impaired channel function.

Background: The aims of this study were to characterize clinical features of a pediatric African-American cystic fibrosis (CF) patient heterozygous for F508del and a novel c.3623G > A mutation, and to identify the molecular defect(s) associated with c.3623G > A mutation.

Monitoring Central Venous Catheter Resistance to Predict Imminent Occlusion: A Prospective Pilot Study.

Background: Long-term central venous catheters are essential for the management of chronic medical conditions, including childhood cancer. Catheter occlusion is associated with an increased risk of subsequent complications, including bloodstream infection, venous thrombosis, and catheter fracture. Therefore, predicting and pre-emptively treating occlusions should prevent complications, but no method for predicting such occlusions has been developed.

Pulmonary Function After Treatment for Embryonal Brain Tumors on SJMB03 That Included Craniospinal Irradiation.

Purpose: The treatment of children with embryonal brain tumors (EBT) includes craniospinal irradiation (CSI). There are limited data regarding the effect of CSI on pulmonary function.

Methods: Protocol SJMB03 enrolled patients 3 to 21 years of age with EBT.

Pulmonary function in infants with swallowing dysfunction.

Background: Swallowing dysfunction can lead to recurring aspiration and is frequently associated with chronic symptoms such as cough and wheezing in infants. Our objective was to describe the characteristics of infants with swallowing dysfunction, determine if pulmonary function abnormalities are detectable, and if they improve after therapy.

Methods: We studied 38 infants with a history of coughing and wheezing who had pulmonary function tests performed within two weeks of their diagnosis of swallowing dysfunction.

Pre-hematopoietic stem cell transplant lung function and pulmonary complications in children.

Rationale: Pulmonary complications are a significant cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation.

Objectives: The relationship between pretransplant pulmonary function tests (PFTs) and development of post-transplant pulmonary complications in children was studied.

Methods: This is a retrospective single institution cohort study of 410 patients who underwent pretransplant PFT and were monitored to 10 years posttransplant.

Cigarette smoking and pulmonary function in adult survivors of childhood cancer exposed to pulmonary-toxic therapy: results from the St. Jude lifetime cohort study.

Treatments for childhood cancer can impair pulmonary function. We assessed the potential impact of cigarette smoking on pulmonary function in 433 adult childhood cancer survivors (CCS) who received pulmonary-toxic therapy, using single breath diffusion capacity for carbon monoxide corrected for hemoglobin (DLCOcorr), forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and total lung capacity (TLC). FEV1/FVC median values among current [1.

Long-term pulmonary function after metastasectomy for childhood osteosarcoma: a report from the St Jude lifetime cohort study.

Background: Complete resection of lung metastases improves survival in patients with osteosarcoma. We evaluated the long-term effect of metastasectomy on pulmonary function of patients treated for osteosarcoma during childhood.

Study Design: We reviewed the medical records of patients who had pulmonary function tests (PFTs) after metastasectomy for osteosarcoma.

Pulmonary outcomes in survivors of childhood central nervous system malignancies: a report from the Childhood Cancer Survivor Study.

Background: Adult survivors of childhood central nervous system (CNS) tumors may be at risk for pulmonary dysfunction. This study enumerates the incidence of pulmonary dysfunction and explores associations between craniospinal irradiation (CSI) and pulmonary dysfunction among survivors of childhood CNS tumors.

Methods: Participants included Childhood Cancer Survivor Study (CCSS) cohort members treated for CNS malignancies when View Article and Find Full Text PDF

Pulmonary disease after treatment for Wilms tumor: a report from the national wilms tumor long-term follow-up study.

Purpose: This study was undertaken to evaluate the incidence of pulmonary disease among patients treated with radiation therapy (RT) for pulmonary metastases (PM) from Wilms tumor (WT).

Patients And Methods: We reviewed records of 6,449 patients treated on National Wilms Tumor Studies-1, -2, -3, and -4 whose flow sheets or annual status reports documented one of several pulmonary conditions. Cases were fully evaluable if pulmonary function test (PFT) results were available, pulmonary fibrosis was identified on a chest radiograph or was listed as the primary or a contributing factor to death.

Increased tricuspid regurgitant jet velocity by Doppler echocardiography in adult survivors of childhood cancer: a report from the St Jude Lifetime Cohort Study.

Purpose: To determine the prevalence of pulmonary hypertension, a late effect of cancer therapy not previously identified in aging survivors of childhood cancer, and associations with chest-directed radiation therapy (RT) and measures of current cardiac function, lung function, and exercise capacity.

Patients And Methods: Cross-sectional evaluation of 498 survivors at a median age of 38.0 years (range, 20.

Pulmonary function after whole lung irradiation in pediatric patients with solid malignancies.

Background: Although whole lung irradiation is used to treat pulmonary metastases of pediatric solid malignancies, few studies have addressed its long-term pulmonary consequences.

Methods: The authors conducted a retrospective study of longitudinal changes in 171 pulmonary function tests (PFTs) and their relation with clinical features in 48 survivors of pediatric malignant solid tumors treated with whole lung irradiation.

Results: Although active respiratory symptoms were seen in only 9 patients (18.