Childhood-onset Chronic Disease Research Articles

Evaluation of Adult Patients with Childhood-onset Chronic Disease Admitted to the Intensive Care Unit

Evaluation of Adult Patients with Childhood-onset Chronic Disease Admitted to the Intensive Care Unit

Fact-finding survey of doctors at the departments of pediatrics and pediatric surgery on the transition of patients with childhood-onset chronic disease from pediatric to adult healthcare.

The number of adult patients with childhood-onset chronic diseases is increasing. However, the process of transitioning these patients from child- to adult-centered medical services faces many difficulties. Despite the key role that doctors in the pediatric field are considered to play in transition, few fact-finding surveys about transition have been conducted among these doctors. The aim of this study was to demonstrate the current status and challenges in the transition of patients with childhood-onset chronic diseases by a fact-finding survey of pediatricians and pediatric surgeons at a university hospital. A cross-sectional survey was performed using an anonymous self-administered questionnaire. Seventy-six doctors of pediatrics and pediatric surgery (excluding junior residents) in a university hospital were asked to answer an anonymous self-report questionnaire. A multidisciplinary research team selected items related to the transitional process. Sixty (79%) doctors participated, of whom 52 (87%) showed awareness of transition. No doctor answered that "Transition is conducted smoothly." Doctors with shorter pediatric department experience had lower awareness and poorer experience with transition. In contrast to pediatric surgeons, pediatricians explained "job-seeking activities" and "contraceptive methods" to the patient, and reported a higher patient age at which to initiate explanation of transition to the patient and his/her family. Among factors inhibiting transition, 39 (65%) respondents selected "The patient's family members do not desire transition" and 34 (57%) selected "Although a relevant adult healthcare department is available, it will not accept the patient." The medical providers most frequently considered to have responsibility for playing a central role in the transition process were "pediatrician/pediatric surgeon," "medical social worker," and "regional medical liaison office." To promote transition, pediatric and adult healthcare departments should share concerns about and cooperate in the establishment of more effective methods of transition, and provide multidisciplinary collaboration to support patients and their families.

Do Patterns of Early Disease Severity Predict Grade 12 Academic Achievement in Youths With Childhood-Onset Chronic Rheumatic Diseases?

To test the association of early disease severity with grade 12 standards test performance in individuals with childhood-onset chronic rheumatic diseases (ChildCRDs), including juvenile arthritis and systemic autoimmune rheumatic diseases. We used linked provincial administrative data to identify patients with ChildCRDs born between 1979 and 1998 in Manitoba, Canada. Primary outcomes were Language and Arts Achievement Index (LAI) scores and Math Achievement Index (MAI) scores from grade 12 standards test results as well as enrollment data. The secondary outcome was enrollment in grade 12 by 17 years of age. Latent class trajectory analysis identified disease severity groups using physician visits following diagnosis. Multivariable linear regression tested the association of disease severity groups with LAI and MAI scores, and logistic regression tested the association of disease severity with age-appropriate enrollment, after adjusting for sociodemographic factors and psychiatric morbidities. The study cohort included 541 patients, 70.1% of whom were female. A 3-class trajectory model provided the best fit; it classified 9.7% of patients as having severe disease, 54.5% as having moderate disease, and 35.8% as having mild disease. After covariate adjustment, severe disease was associated with poorer LAI and MAI scores but not with age-appropriate enrollment. Among patients with ChildCRDs, those with severe disease performed more poorly on grade 12 standards tests, independent of sociodemographic and psychiatric risk factors. Clinicians should work with educators and policy makers to advocate for supports to improve educational outcomes of patients with ChildCRDs.

Social independence evaluation index for Japanese patients with childhood-onset chronic diseases.

This study established an independent evaluation index for patients with childhood-onset chronic diseases in Japan. From November to December 2020, three Delphi rounds were conducted. Thirty-nine participants completed at least one survey. We asked them about targets of social independence for 10 types of activities (education/labor/finance/acquisition of necessities/housing/transportation/leisure/social relationship/intimate relationships/sexuality). The Delphi technique was to be repeated until a consensus of over 80% of participants was reached. The targets chosen for measuring independence in patients with childhood-onset chronic diseases were as follows: "Graduation from high school," "Labor for livelihood (including temporary turnover)," "Financially independent (including temporary turnover, excluding students)," "Buy or rent a house and buy the daily necessities and get the public services you need to live," "Do housework alone," "Plan alone and use transportation to get around," "Participate in play/recreation/leisure activities on own initiative," "Engage in relationships with other people outside of a limited environment (home, school, office, hospital, etc.)," "Create and maintain intimate or romantic relationships between individuals (couples, lovers, sexual partners)," and "Use or know how to use contraceptives and how to prevent sexually transmitted diseases." We established an independent evaluation index for patients with childhood-onset chronic diseases in Japan through a three-round Delphi process. The assessment of social independence using our independent evaluation index may help plan for and provide appropriate support and assistance to these patients.

Prevalence and barriers to health care transition for adolescent patients with childhood-onset chronic diseases across Japan: A nation-wide cross-sectional survey.

Since the Japan Pediatric Society published its “Recommendations on Transitional Care for Patients with Childhood-Onset Chronic Diseases” in 2014, there has been an increased interest in the health care transition of adolescents with childhood-onset chronic diseases in Japan. However, the actual status of healthcare transition was not studied yet. The purpose of this study was to explore the prevalence of transitional support for adolescent patients with childhood-onset chronic disease and the factors hindering their transition. We conducted an anonymous questionnaire survey in August 2020, targeting physicians and nurses involved in health care transition at 494 pediatric facilities in Japan. Survey items included demographic data, health care systems related to transition to adult departments, health care transition programs based on Six Core Elements (establishing transition policy, tracking and monitoring transition progress, assessing patient readiness for transition, developing the transition plan with a medical summary, transferring the patient, completing the transfer/following up with the patient and family), barriers to transition (34-item, 4-point Likert scale), and expectations in supporting transition (multiple-choice responses), which consisted of five items (78 questions); all questions were structured. Descriptive statistics were used for analysis. Of the 225 responses collected (45.5% response rate), 88.0% were from pediatricians. More than 80% of respondents transferred patients of 20 years or older, but only about 15% had took a structured transition process of four or more based on the Six Core Elements. The top transition barriers were “intellectual disability/rare disease” and “dependence on pediatrics” as patient/family factors, and “lack of collaboration with adult healthcare (relationship, manpower/system, knowledge/understanding)” as medical/infrastructure factors. The study provides future considerations, including the promotion of structured health care transition programs, development of transitional support tailored to the characteristics of rare diseases and disorders, and establishment of a support system with adult departments.

Toward Improving the Transition of Patients With Congenital Adrenal Hyperplasia From Pediatrics to Adult Healthcare in Japan

The transition of patients with childhood-onset chronic diseases from pediatric to adult healthcare systems has recently received significant attention. Since 2013, the Japan Pediatric Society developed working groups to formulate guidelines for transition of patients with childhood-onset chronic diseases from pediatric to their disease specialty. Herein, we report on the activities of the Japan Society of Pediatric Endocrinology (JSPE) and the current status of transition medicine for 21-hydroxylase deficiency (21-OHD) in Japan. The JSPE proposed roadmaps and checklists for transition and prepared surveys on the current status of healthcare transition for childhood-onset endocrine diseases. In Japan, newborn screening for 21-OHD started in January 1989; however, there is no nationwide registry-based longitudinal cohort study on 21-OHD from birth to adult. The current status and the whole picture of healthcare and health problems in adult patients with 21-OHD remain unclear. Thus, we conducted a questionnaire survey on JSPE members to clarify the current status of healthcare transition of 21-OHD and discuss future perspectives for the healthcare transition of patients with 21-OHD in Japan.

Basic Policy Surrounding the Promotion of Health Care Transition for Patients with Childhood-Onset Chronic Diseases of the Ministry of Health, Labour and Welfare in Japan

With the development of medical technology and improvements in medicine, many patients with childhood-onset chronic diseases transition into adulthood. As our society is facing new problems, how we provide necessary and seamless medical care for adult patients with childhood-onset chronic diseases, and how we promote their health care transition become important. The Ministry of Health, Labour and Welfare in Japan has demonstrated basic policies on health care transition and is building support systems for patients with childhood-onset chronic diseases.

Efficacy of a Transitional Support Program Among Adolescent Patients With Childhood-Onset Chronic Diseases: A Randomized Controlled Trial.

It is recommended that patients with childhood-onset chronic diseases (CCD) be transferred from pediatric to adult healthcare systems when they reach adulthood. Transitional support helps adolescents with CCD transition smoothly. Transition readiness is one of the key concepts to assess the efficacy of transitional support programs. This study aims to investigate the effect of a transitional support program on transition readiness, self-esteem, and independent consciousness among Japanese adolescents with various CCD using a randomized controlled trial. Adolescents with CCD aged 12–18 years participated in a randomized controlled trial evaluating the efficacy of a transitional support program. The patients in the intervention group visited transitional support outpatient clinics twice. They answered questionnaires regarding their disease and future perspectives to healthcare professionals and independently made a short summary of their disease. All the participants answered the questionnaires four times. Eighty patients participated in this study. Among those in the intervention group, transition readiness within one, three, and 6 months after interventions, and self-esteem within 1 month after interventions were higher than that of the control group. The scores on the “dependence on parents” subscale at 6 months after interventions were lower for the intervention group as compared to the control group. This program is expected to help patients transition smoothly from pediatric to adult healthcare systems.

Mind-body correlation related to childhood experience of youth with childhood-onset chronic disease

Mind-body correlation related to childhood experience of youth with childhood-onset chronic disease

A Population-based Study of Grade 12 Academic Performance in Adolescents With Childhood-onset Chronic Rheumatic Diseases.

The aims of this study were (1) to compare grade 12 standardized test results of patients diagnosed with childhood-onset chronic rheumatic diseases (ChildCRD) and unaffected peers; and (2) to identify factors associated with test results of patients with ChildCRD and unaffected peers. This was a population-based retrospective cohort study. All patients with ChildCRD (juvenile arthritis and systemic autoimmune rheumatic diseases) from the only pediatric rheumatology center in Manitoba for birth cohorts January 1979 to December 1998 were linked to the provincial administrative databases containing records of healthcare use and education outcomes. Patients were matched by age, sex, and postal codes to their peers who did not have ChildCRD. The primary outcomes were the grade 12 Language Arts Achievement Index (LAI) and the Math Achievement Index (MAI) scores. ChildCRD, sociodemographic, and mental health factors were tested for their associations with LAI and MAI scores using multivariable linear regression. Five hundred and forty-one patients with ChildCRD were matched to 2713 unaffected peers. Patients with ChildCRD had lower LAI and MAI scores compared to their peers. More patients with ChildCRD failed or did not take the language arts (51% vs 41%, P < 0.001) and math (61% vs 55%, P = 0.02) tests. On multivariable analysis, ChildCRD, lower socioeconomic status, younger maternal age at first childbirth, family income assistance, involvement with child welfare services, and mental health morbidities (between ChildCRD diagnosis and standardized testing), were associated with worse LAI and MAI results. This population-based study showed that patients with ChildCRD performed less well than their peers on grade 12 standardized testing, independent of sociodemographic and mental health comorbidities.

Transitioning from paediatric to adult rheumatological healthcare: English summary of the Japanese Transition Support Guide.

Issues related to transitioning from paediatric to adult healthcare are currently receiving international attention. In Japan, 1000 patients with childhood-onset chronic rheumatological diseases reach adulthood every year and require transition from care by paediatric to care by adult rheumatologists. Here, we propose a guide for the latter, wherein the adult caregiver poses the clinical questions about transitional support that they need to have answered, and the paediatric caregiver mainly compiles the plans for the transition. To formulate the guide, we sought comments from both the Japan College of Rheumatology and the Pediatric Rheumatology Association of Japan and obtained their approval. Here, we present the outcome of this consultation in the form of a Guide for Supporting Transitional Care, aiming to provide essential knowledge to physicians in the fields of adult internal medicine and orthopaedics who may be involved in treating patients with rheumatic disease during the transition from paediatric to adult care. The features of transitional support that are common for patients with various different rheumatic diseases are presented in this guide, with the aim of informing policy and strategies to deliver optimal outcomes in transitional care by non-paediatric rheumatologists.

Development and validation of a Japanese version of the TRANSITION-Q.

The evaluation of transition readiness is indispensable for long-term follow-ups of adolescent patients with childhood-onset chronic diseases (CCD). We developed a Japanese version of the TRANSITION-Q (TRANSITION-Q-J) and used it to assess Japanese patients with CCD. The TRANSITION-Q-J was developed through forward and backward translations followed by cognitive interviews with five adolescent patients. The field test was conducted with 125 adolescent patients, and a retest was conducted with 113 adolescent patients. Confirmatory factor analysis supported the two-factor analysis model including F1 (communication and self-management) and F2 (examination behavior). Sufficient internal consistency and test-retest reliability were demonstrated among the total 14 items, F1, and F2 (Cronbach's α>0.80, intraclass correlation coefficient>0.85). Convergent and discriminant validity for the 14 items and F1 were acceptable; however, F2 did not correlate significantly with the Rosenberg Self-Esteem Scale and Independent Consciousness Scale. Regarding known-groups validity, the older group had a significantly higher mean TRANSITION-Q-J score (50.05) than the younger group (43.28; P=0.04). The same results were found for both F1 and F2. The TRANSITION-Q-J for adolescent patients with CCD was developed and its reliability and validity were verified. This scale is easy to administer. In addition to being a tool for transition period support, it could be used to verify effective factors and in program outcome evaluation, including intervention studies.

Association of Childhood Family Connection With Flourishing in Young Adulthood Among Those With Type 1 Diabetes

Higher levels of childhood family connection have been associated with measures of adult flourishing or eudaimonic well-being, such as purpose, self-acceptance, positive relationships, and growth. However, this association has not been examined among those with childhood-onset chronic disease. To investigate whether higher levels of childhood family connection were associated with greater flourishing in young adulthood among those with type 1 diabetes and, secondarily, whether this association was present across levels of adverse childhood experiences and childhood social position. In 2017, the cross-sectional Type 1 Flourish survey was administered to all 743 young adults, aged 18 to 29 years, with type 1 diabetes who had received outpatient care in 2016 at a diabetes specialty clinic in New York, New York. Eligible participants completed the survey online or during clinic visits. Data analyses were conducted in September and October 2019. The main exposure was childhood family connection (sample-defined tertiles), based on scores from a 7-item scale assessing parental attention, affection, and communication during childhood. Adverse childhood experiences, childhood social position, and other sociodemographic characteristics were also reported. Recent hemoglobin A1c levels were abstracted from medical records. Flourishing score calculated from the 42-item Psychological Well-being Scale developed by Ryff. The survey was completed by 423 of 743 patients (56.9%), and the analysis included 415 participants (98.1%) with complete data on family connection and flourishing. The mean (SD) age of the sample was 25.0 (3.2) years, with 246 (59.3%) female respondents and 288 (69.6%) non-Hispanic white respondents. The mean (SD) flourishing score was 221.8 (37.7). After adjusting for age, sex, race/ethnicity, education, income, age at type 1 diabetes diagnosis, and hemoglobin A1c level, mean flourishing scores increased from the lowest (201.0; 95% CI, 195.0-207.0) to medium (225.2; 95% CI, 219.4-231.0) to highest (240.4; 95% CI, 234.4-246.4) tertiles of family connection; compared with those in the lowest tertile of family connection, the flourishing scores were 1.04 (95% CI, 0.81-1.27) SD units higher among those in the highest tertile and 0.64 (95% CI, 0.42-0.86) SD units higher among those in the middle tertile. This association was also present across levels of childhood adversity. In the subgroup of respondents with 2 or more adverse childhood experiences, those in the highest tertile of family connection had adjusted flourishing scores 0.76 (95% CI, 0.14-1.38) SD units higher than those in the lowest tertile. In the subgroup with low childhood social position, those in the highest tertile of family connection had flourishing scores 1.08 (95% CI, 0.63-1.52) SD units higher than those in the lowest tertile. In this cross-sectional study of young adults with type 1 diabetes, higher levels of childhood family connection were associated with greater flourishing in young adulthood across levels of childhood adversity. Beyond disease management, clinician support of family connection may help children with type 1 diabetes flourish in adulthood.

Nurses' perceptions regarding transitional care for adolescents and young adults with childhood-onset chronic diseases.

Nurses are expected to have a role in the transition of care from pediatric to adult medical practices for adolescents and young adults with childhood-onset chronic diseases. This study compares the experience, knowledge, and perceptions regarding the ideal care among adult unit and pediatric nurses regarding the transition to adult care for those with childhood-onset chronic diseases. A cross-sectional study using self-report questionnaires was conducted with nurses in a tertiary hospital in Tokyo. Questions were generated based on a literature review and expert discussion. Data from 1,064 participants were analyzed (adult unit nurses: n = 959, 90.1%; pediatric nurses: n = 105, 9.9%). Among 623 adult unit nurses who had care experience for adult patients with a childhood-onset chronic disease, 458 nurses (73.6%) were unaware of the concept of transitional care. As the obstructive factors for transition, pediatric nurses recognized problems in healthcare providers' attitudes and lack of transitional care coordinators, while the adult unit nurses emphasized the patients' wishes to continue to receive pediatric healthcare. Most adult unit nurses expected pediatric nurses to function as transitional care coordinators. Adult unit and pediatric nurses had different perceptions of the barriers in transitioning children with chronic diseases to adult care. It is important to have educational programs focusing on transitional care for all nurses, both to enable pediatric nurses to improve transition readiness of children with chronic diseases and to offer adult patients with a childhood-onset chronic disease continuing support through adult unit nurses.

Chronic inflammation and the growth hormone/insulin-like growth factor-1 axis.

Interactions between growth hormone (GH), insulin-like growth factor-1 (IGF-1), and the immune system are complex, bidirectional, but not fully explained. Current reviews based on numerous studies have indicated that chronic inflammation could suppress the GH/IGF-1 axis via several mechanisms such as relative GH and/or IGF-1 insufficiency, peripheral resistance to GH and/or IGF-1 resulting from down-regulation of GH and IGF-1 receptors, disruption in the GH/IGF-1 signalling pathways, dysregulation of IGF binding proteins (IGFBPs), reduced IGF bioavailability, and modified gene regulation due to changes in the microRNA system. It is well-known that relationships between the immune system and the GH/IGF-1 axis are mutual and GH as well as IGF-1 could modulate inflammatory response and the activity of systemic inflammation. Available data indicate that the GH/IGF-1 axis exerts both pro-inflammatory and anti-inflammatory effects. Pro-inflammatory cytokines such as interleukin-6 (IL-6), tumour necrosis factor-a (TNF-α), and interleukin-1b (IL-b) are some of the most significant factors, besides malnutrition, chronic stress, and prolonged use of glucocorticoids, which impair the activity of the GH/IGF-1 axis, and consequently lead to growth retardation in children suffering from childhood-onset chronic inflammatory diseases. In this review, we discuss the mechanisms underlying the impact of chronic inflammation on the GH/IGF-1 axis and growth processes during childhood and adolescence, based on a number of experimental and human studies.

Current Status and Issues Regarding Transitional Health Care for Adults and Young Adults with Special Health Care Needs in Japan.

With the progress of medical care in recent years, the prognosis of intractable diseases of childhood onset has markedly improved. Young adults with special health care needs require continuous medical support throughout their lifetimes. To provide them with optimal health care services, a smooth transition from the pediatric medical system to the adult one is essential. However, in Japan many adult health providers are not sufficiently prepared to care for these patients, due both to limited opportunities to gain up-to-date medical knowledge on transitional health care and a lack of familiarity with the medical treatment of childhood-onset chronic diseases. In this review, we discuss current issues in transitional health care in Japan from an internist's viewpoint.

成人した小児期発症慢性疾患患者の成人科移行 : 日本における小児科医と小児専門看護師の意識集約調査(英文誌から)

成人した小児期発症慢性疾患患者の成人科移行 : 日本における小児科医と小児専門看護師の意識集約調査(英文誌から)

Promotion of the Transition of Adult Patients with Childhood-Onset Chronic Diseases among Pediatricians in Japan.

The transition of adult patients with childhood-onset chronic diseases (APCCD) from pediatric to adult health-care systems has recently received worldwide attention. However, Japan is lagging behind European countries and North America as this concept of health-care transition was introduced only 10 years ago. In Japan, before the introduction of this concept, APCCD were referred to as “carryover patients,” who were often considered a burden in pediatric practice. In the late 1990s, groups composed of pediatric nephrologists, developmental and behavioral pediatricians, pediatric nurses, and special education teachers researching the quality of life of adult patients with chronic kidney disease began to discuss the physical and psychosocial problems of APCCD. In 2006, a group of pediatricians first introduced the term “transition” in a Japanese journal. By 2010, a group of adolescent nurses had begun a specialized training program aimed at supporting patients during the transitional period. In 2013, the Ministry of Health, Labour and Welfare in Japan convened a research committee, focusing on issues related to social, educational, and medical support for APCCD, and the Japan Pediatric Society established a committee for the health-care transition of APCCD and summarized their statements. Moreover, in 2013, the Tokyo Metropolitan Children’s Medical Center initiated ambulatory services for APCCD managed by specialized nurses. The concept of health-care transition has rapidly spread over these past 10 years. The purpose of this article is to describe how this concept of health-care transition has advanced in Japan, such that APCCD now experience a positive pediatric to adult health-care transition.

Interventions to improve treatment, retention and survival outcomes for adolescents with perinatal HIV-1 transitioning to adult care: moving on up.

There is an increasing number of deaths among adult survivors of perinatal HIV. Multiple and complex factors drive this mortality, including problems with retention in care and adherence during adolescence, coupled with the critical period of transition from paediatric to adult care, increasing their risk of treatment failure and severe immunosuppression. We reviewed studies that evaluated the impact of service delivery interventions to improve the health of perinatally infected adolescents living with HIV (P-ALHIV) to gain insight into what might help them survive the vulnerable period of adolescence. Youth-focused health services and individual-level interventions may improve P-ALHIV adherence and retention in care. However, there have been few studies, many with small sample sizes and with short durations of follow-up that end before the transition period. Studies from other childhood-onset chronic diseases are similarly limited. Further studies are urgently needed to identify optimal intervention strategies to reduce mortality and poor outcomes as the adolescent population expands and ages into adult care. Until we have a more robust evidence base, programmes can develop transition plans based on best practice recommendations to optimize the health and longevity of ALHIV in adulthood.

Transition of adolescent and young adult patients with childhood-onset chronic kidney disease from pediatric to adult renal services: a nationwide survey in Japan.

Transition of adolescent and young adult (AYA) patients with childhood-onset chronic kidney diseases (C-CKD) from pediatric to adult renal services has received increasing attention. However, information on transition of Japanese patients with C-CKD is limited. The Transition Medicine Working Group, in collaboration with the Japanese Society for Nephrology, the Japanese Society for Pediatric Nephrology and the Japanese Society of Pediatric Urology, conducted a retrospective cross-sectional study in 2014 on issues concerning the transition of Japanese patients with C-CKD. Few institutions in Japan had transition programs and/or transition coordinators for patients with C-CKD. Refusal to transfer by patients or their families, lack of concern about transition and inability to decide on transfer were common reasons for non-transfer of patients still followed by pediatric renal services. Around 25% of patients who had ended or interrupted follow-up by pediatric renal services presented to adult renal services because of symptoms associated with C-CKD. Patients with various types of childhood-onset nephrourological diseases were transferred from pediatric to adult renal services. IgA nephropathy, minimal change nephrotic syndrome and congenital anomalies of the kidney and urinary tract were the most frequent primary kidney diseases in adult patients with C-CKD. These survey results indicate the need for introduction of transitional care for Japanese AYA patients with C-CKD. Consensus guidelines for the optimal clinical management of AYA patients with C-CKD are required to ensure the continuity of care from child to adult renal services.