Balancing the benefits and harms of thyroid cancer surveillance in survivors of Childhood, adolescent and young adult cancer: Recommendations from the international Late Effects of Childhood Cancer Guideline Harmonization Group in collaboration with the PanCareSurFup Consortium.

Radiation exposure to the thyroid gland during treatment of childhood, adolescent and young adult cancer (CAYAC) may cause differentiated thyroid cancer (DTC). Surveillance recommendations for DTC vary considerably, causing uncertainty about optimum screening practices. The International Late Effects of Childhood Cancer Guideline Harmonization Group, in collaboration with the PanCareSurFup Consortium, developed consensus recommendations for thyroid cancer surveillance in CAYAC survivors. These recommendations were developed by an international multidisciplinary panel that included 33 experts in relevant medical specialties who used a consistent and transparent process. Recommendations were graded according to the strength of underlying evidence and potential benefit gained by early detection and appropriate management. Of the two available surveillance strategies, thyroid ultrasound and neck palpation, neither was shown to be superior. Consequently, a decision aid was formulated to guide the health care provider in counseling the survivor. The recommendations highlight the need for shared decision making regarding whether to undergo surveillance for DTC and in the choice of surveillance modality.

Late effects in patients with Fanconi anemia following allogeneic hematopoietic stem cell transplantation from alternative donors.

Hematopoietic stem cell transplantation (HSCT) is curative for hematological manifestations of Fanconi anemia (FA). We performed a retrospective analysis of 22 patients with FA and aplastic anemia, myelodysplastic syndrome or acute myelogenous leukemia who underwent a HSCT at Memorial Sloan Kettering Cancer Center and survived at least 1 year post HSCT. Patients underwent either a TBI- (N=18) or busulfan- (N=4) based cytoreduction followed by T-cell-depleted transplants from alternative donors. Twenty patients were alive at time of the study with a 5- and 10-year overall survival of 100 and 84% and no evidence of chronic GvHD. Among the 18 patients receiving a TBI-based regimen, 11 (61%) had persistent hemochromatosis, 4 (22%) developed hypothyroidism, 7 (39%) had insulin resistance and 5 (27%) developed hypertriglyceridemia after transplant. Eleven of 16 evaluable patients (68%), receiving TBI, developed gonadal dysfunction. Two patients who received a TBI-based regimen died of squamous cell carcinoma. One patient developed hemochromatosis, hypothyroidism and gonadal dysfunction after busulfan-based cytoreduction. TBI appears to be a risk factor for malignant and endocrine late effects in the FA host. Multidisciplinary follow-up of patients with FA (including cancer screening) is essential for early detection and management of late complications, and improving long-term outcomes.

Disorders of glucose homeostasis in young adults treated with total body irradiation during childhood: a pilot study.

Impairment of glucose metabolism (in particular insulin resistance and type 2 diabetes mellitus) has been reported in patients who have undergone hematopoietic SCT (HSCT) during childhood, especially those treated with TBI. This pilot study was conducted to determine prevalence of and possible underlying mechanisms for impaired glucose homeostasis in young adults treated with HSCT and TBI and who were not previously known to have diabetes mellitus. A total of 10 subjects (6 males, 4 females) were evaluated. Mean ages were 13.0+/-1.0 years at the time of TBI and 24.0+/-1.1 years at the time of this study. Five subjects had laboratory evidence of insulin resistance using the homeostasis model assessment and the quantitative insulin sensitivity check index indices. Two of these subjects had impaired fasting glucose and four had decreased plasma insulin-like growth factor 1 levels. All 10 subjects had evidence of abdominal obesity. Insulin resistance is frequently observed in adult survivors of HSCT treated with TBI in childhood. Underlying mechanisms may include radiation-induced growth hormone deficiency and changes in body composition.

Advocacy skills training for young adult cancer survivors: the Young Adult Survivors Conference at Camp Mak-a-Dream.

GOALS OF THE WORK: This manuscript reports on the design, implementation, and evaluation of the Young Adult Survivor Conference (YASC)-a 4-day retreat designed to provide an educational and support experience for cancer survivors diagnosed as children, adolescents, and young adults. The goals of the program were to address issues of survivorship, provide cancer education and tools for self-advocacy, and build bridges of support among young adult cancer survivors who may be experiencing similar journeys through life. THE PROGRAM: YASC was designed to offer advocacy skills specific to participants' needs. Workshops focused on understanding late effects, mentoring and communication, tips for starting peer support programs and networks, and opportunities for offering public testimony and involvement in public policy. EVALUATION: Program evaluation indicated that participants achieved personal goals and expectations for having fun, meeting other survivors, understanding more about their cancer and potential late effects, and learning about how to tell their story so that it will help others. CONCLUSIONS: Involvement with peers who have shared a similar experience provided participants an opportunity to address areas of concern such as coping with uncertainty, dependency vs autonomy, social exclusion, body image, intimacy, sexuality and fertility, and career options. Participation in programs such as the YASC offers young survivors opportunities for life experiences that may promote successful achievement of age-appropriate developmental tasks.

Cardiovascular risk factors in young adult survivors of childhood acute lymphoblastic leukemia.

PURPOSE: To assess cardiovascular risk factors (CVRF) in young adult survivors of childhood acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: Twenty-six subjects (median age, 20.9 years; median interval since completion of therapy, 13.3 years) were evaluated. Ten participants had received cranial irradiation (CRT), whereas 16 had received only chemotherapy. Primary outcome measures included body mass index (BMI), blood pressure, fasting lipoprotein, glucose, and insulin levels. Secondary measures included insulin-like growth factor-1 (IGF-1) and IGF binding protein-3 levels, physical activity index, a 7-day dietary recall, tobacco product use, and measurement of the intima-media thickness (IMT) of the common carotid artery. RESULTS: Sixty-two percent (16/26) of participants had at least one CVRF potentially related to their cancer treatment (obesity, dyslipidemia, increased blood pressure, or insulin resistance), with 30% (7/26) having more than two CVRF. Thirty-one percent (8/26) of subjects were obese (BMI > or = 30). Subjects who were treated with CRT (BMI, 30.4 +/- 6.7) had an increased BMI (P = 0.039) in comparison with those who received only chemotherapy (BMI, 25.4 +/- 5.1). Triglyceride and very low-density lipoprotein C levels were significantly higher in those treated with CRT (P = 0.027 and 0.022, respectively). The IGF-1 was inversely correlated with IMT (total group, -0.514, P = 0.009; females only, -0.729, P = 0.003). CONCLUSIONS: Young adult survivors of childhood ALL, especially those treated with CRT, are at risk for obesity and dyslipidemia, insulin resistance, hypertension, and cardiovascular disease. Further investigation of these risks is warranted.

Providing primary care for long-term survivors of childhood acute lymphoblastic leukemia.

Primary care physicians will be providing longitudinal health care for long-term survivors of childhood acute lymphoblastic leukemia (ALL) with increasing frequency. Late effects (sequelae) secondary to treatment with radiation or chemotherapeutic agents are frequent and may be serious. Depending on treatment exposures, this at-risk population may experience life-threatening late effects, such as cirrhosis secondary to hepatitis C or late-onset anthracycline-induced cardiomyopathy, or life-changing late effects, such as cognitive dysfunction. Many survivors of childhood ALL will develop problems such as obesity and osteopenia at a young age, which will significantly affect their risk for serious health outcomes as they grow older. The goal of our review is to assist primary care physicians in providing longitudinal health care for long-term survivors of childhood ALL. We also highlight areas needing further investigation, including the prevalence of different late effects, determination of risk factors associated with a late effect, a better understanding of the potential impact of late effects on the premature development of common adult health problems, and the value and timing of different tests for screening asymptomatic survivors.

Grading of late effects in young adult survivors of childhood cancer followed in an ambulatory adult setting.

BACKGROUND: The objective of the current study was to describe a multidisciplinary transition program for following young adult survivors of childhood cancer in an adult-based ambulatory medical setting and to report the late effects with grades of toxicity diagnosed in all adult survivors followed in the program. METHODS: The study population was comprised of all young adult survivors (n = 96) of childhood cancer who were seen in the After the Cancer Experience (ACE) Young Adult Program prior to January 31, 1999. The median age of the survivors was 22.8 years (range, 17-34 years) and the median interval from the time of cancer diagnosis was 15.2 years (range, 6-25 years). Primary cancer groups included: leukemia, 33%; sarcoma, 24%; Hodgkin disease, 15%; non-Hodgkin lymphoma, 12%; Wilms' tumor, 9%; and other, 7%. Late effects were graded using the Common Toxicity Criteria, Version 2 (CTCv2), developed by the National Cancer Institute. RESULTS: Approximately 69% of the patients (66 of 96) had at least 1 late effect. Thirty-three percent of patients had a single late effect whereas 36% had >/= 2 late effects. Thirty percent of patients had a CTCv2 Grade 3 or 4 late effect. CONCLUSIONS: The current study represents an example of a successful multidisciplinary transition program in an ambulatory, adult setting for young adult survivors of childhood cancer. Late effects of cancer treatment are common in young adult survivors, with approximately 33% being moderate to severe. Further studies are needed to modify CTCv2 with the aim of developing a reliable and valid tool to assess late effects in long term survivors of childhood cancer.

Programs for adult survivors of childhood cancer.

PURPOSE: The potential for late effects of treatment necessitates long-term monitoring of adult survivors of childhood cancer. The purpose of this study was to determine how institutions follow up young adult survivors of pediatric malignancy. Specifically, we were interested in the types of health care providers who follow up these patients, how the follow-up is administered, and what barriers to follow-up have been encountered. METHODS: A 16-item questionnaire was mailed to the 219 members of the Children's Cancer Group (CCG) and the Pediatric Oncology Group (POG). The survey consisted of four categories of questions that asked for information regarding the existence of a program to follow up young adults, the setting of the program, routine activities of the program, and commonly encountered barriers to care. RESULTS: One hundred eighty-two members returned the survey (83% response rate). Fifty-three percent of the respondents have a long-term follow-up clinic at their institution. Whereas 44% have a mechanism for following up adult survivors, only 15% of the programs have established a formal database for adults. Nearly all the programs (93%) use a pediatric oncologist. Although an adult oncologist assists in 13% of the programs, primary care physicians are uncommonly (8%) involved. CONCLUSION: Few programs focus on the long-term health care needs of adult survivors of childhood cancer. The majority of existing programs are in pediatric institutions, without significant input from adult-oriented, generalist health care providers.

Treatment of acute bronchitis in adults. A national survey of family physicians.

BACKGROUND: The purpose of this study was to determine how family physicians in the United States treat acute bronchitis in an otherwise healthy adult. METHODS: A 33-item questionnaire on the diagnosis and treatment of acute bronchitis was mailed to a random sample of 500 physicians who are members of the American Board of Family Practice. RESULTS: Thirty-two of the 500 sampled physicians could not be located by mail; 265 of those who received the questionnaire responded. The response rate was 57% (265/468). Sixty-three percent of responding physicians indicated that antibiotics are their first choice of treatment for the otherwise healthy, nonsmoking adult with acute bronchitis. The decision to use antibiotics as the first choice of treatment did not vary by physician's sex, age, years in practice, practice location, practice type, or percentage of HMO patients. Only 6% of responding physicians reported using beta 2 agonist bronchodilators as their first choice of treatment. Physicians in this study stated that they prescribe an antibiotic 75% of the time in treating nonsmoking patients with acute bronchitis (first choice or otherwise). If the patient is a smoker, physicians reported that they prescribe antibiotics 90% of the time (F = 110.25; df = 1; P > .0001). Physicians reported that for patients who smoke it takes longer for coughs to totally resolve and longer for them to return to a normal activity level than for nonsmokers. CONCLUSIONS: Family physicians report that antibiotics are their most common treatment for acute bronchitis in the otherwise healthy adult. Previous clinical trials have shown only marginal improvement in symptoms when patients with this condition are treated with an antibiotic. With antibiotic resistance emerging as a major global health problem, it is essential that other methods of treatment be evaluated.

Diagnosis of acute bronchitis in adults: a national survey of family physicians.

BACKGROUND: The purpose of this study was to determine how family physicians in the United States diagnose acute bronchitis in otherwise healthy adults. METHODS: A 33-item questionnaire on the diagnosis and treatment of acute bronchitis was mailed to a random sample of 500 physicians who are members of the American Board of Family Practice. RESULTS: Two hundred sixty-five physicians responded. Of those who did not respond, 32 could not be located. Of those who did respond, 10 were either retired or were practicing in another specialty. The net response rate was 56% (255/458). Responding physicians stated that character of cough and sputum production are the most important items used in diagnosing acute bronchitis. Fifty-eight percent indicated that the cough should be productive, and 60% stated that the sputum should be purulent. Seventy-two percent of respondents did not feel that wheezing or rhonchi need to be present. Younger physicians and those who selected antibiotics as their first treatment choice were more likely to define acute bronchitis as the presence of a productive cough with purulent sputum (P < .05). Physicians from an academic setting were more likely to define acute bronchitis as a productive cough (P < .05). Thirty-six percent of physicians from practices serving populations with > or = 60% managed care patients included wheezing or rhonchi in the definition of acute bronchitis, compared with 26% of all others (P < .05). CONCLUSIONS: Variations in the diagnosis of acute bronchitis in otherwise healthy adults can be attributed to physician age, treatment choice, and practice setting. A significant number of family physicians did not require a productive cough as part of the diagnostic criteria for acute bronchitis. This finding needs to be considered in studies evaluating treatment. Additional qualitative studies are necessary to identify other factors involved in diagnosing acute bronchitis.

Support and rewards for scholarly activity in family medicine: a national survey.

BACKGROUND AND OBJECTIVES: This survey examined how time is allotted for family medicine faculty to pursue scholarly activities and how these activities are rewarded. METHODS: A survey was sent to all directors of family practice residency programs (n = 373) and chairs of family medicine departments (n = 112). Four primary questions were asked: 1) How is faculty time allotted for scholarly activities? 2) Does the residency or department use an explicit reward system? 3) What activities are rewarded? and 4) What rewards are used? RESULTS: A total of 363 surveys were returned, for a response rate of 75%. Forty-nine percent of respondents have regular, protected faculty time for scholarly activities. Faculty at university-based residencies and departments were more likely to have protected time (68/93, 73%) than faculty at community-based residencies (93/238, 39%). Thirty-eight percent of respondents have an explicit reward system. Activities rewarded and rewards used are department and program-type specific. CONCLUSIONS: Only 39% of community-based residencies and 73% of university programs allot regular protected time for faculty. The majority of programs and departments do not have an explicit reward system. Further studies are needed to determine if the use of protected time and reward systems enhance scholarly productivity.

Antiphospholipid syndrome.

The antiphospholipid syndrome is an illness characterized by recurrent venous or arterial thrombosis, recurrent fetal loss, and thrombocytopenia. It is diagnosed by the presence of antiphospholipid antibodies, such as anticardiolipin antibody or lupus anticoagulant. Although the antiphospholipid syndrome affects a significant number of patients, these patients may be unrecognized because the syndrome has not been well reported in the primary care literature.

Scurvy: more than historical relevance.

The clinical description of scurvy is well recorded in the history of medicine. Yet, because scurvy is relatively uncommon in industrially developed countries, it is often underdiagnosed, and unnecessary tests are performed. The result of a prolonged deficiency of ascorbic acid, scurvy is classically manifested by perifollicular hemorrhages, corkscrew hairs, large ecchymoses and hemorrhagic gingivitis. Although the disease is considered uncommon in the United States, two populations at greatest risk--the institutionalized elderly and alcoholics--are increasing.

The effect of patient education on pediatric immunization rates.

BACKGROUND: Over the last decade, the immunization rate among preschool children has decreased, especially in the lower socioeconomic population. During this period, reports of outbreaks of immunizable diseases, especially pertussis and measles, have correspondingly increased. This study was designed to evaluate the effect of a brief patient education encounter with new mothers on pediatric immunization rates. METHODS: Two hundred thirty-eight mothers and infants were assigned to an intervention or control group. On the first day postpartum, the mothers in the intervention group participated in a 10- to 15-minute discussion on the importance of immunizations and were given a patient education handout. A reminder letter was mailed to the intervention group at 2 months postpartum. The control group received no special intervention. Infants were followed for their 2- and 4-month immunizations for diphtheria, pertussis, and tetanus and oral polio vaccine (DPT/OPV). At 1 year of age, the infants' immunization records were assessed for the completion of their first three DPT/OPV immunizations. RESULTS: There was no statistically significant difference, by chi-square analysis, in the immunization rates of the control and intervention groups at 2, 4, or 12 months of age. At 1 year of age, 29 of 122 (24%) of the control group had received all three DPT/OPV immunizations, compared with 33 (28%) of 116 infants in the intervention group. CONCLUSIONS: Concordant with similar studies, the immunization rate among infants of parents of lower socioeconomic status (26%) is low. An educational intervention presented to mothers in the postpartum period did not improve the rate of immunization by the age of 12 months. There are undoubtedly several reasons for this failure. Other means to improve immunization rates of infants should be developed and tested.

Developing on outpatient care syllabus for a third-year clerkship in family medicine.

Selecting and evaluating student reading resources are important steps in preparing for a clerkship, but a literature review yields little information about these processes. The following brief report outlines the method used to produce the outpatient care syllabus for the third-year clerkship at the University of Texas Southwestern Medical School. The discussion includes: 1) the purposes of an outpatient care reading resource; 2) selection of materials; 3) correlation of materials with a student experience patient log form; and 4) plans for evaluation and periodic revision of the syllabus.