Update of evidence- and consensus-based guidelines for the treatment of juvenile idiopathic arthritis (JIA) by the German Society of Pediatric and Juvenile Rheumatic Diseases (GKJR): New perspectives on interdisciplinary care.

BACKGROUND: New therapeutic strategies for juvenile idiopathic arthritis (JIA) have evolved within the past ten years, and as a result, an update of the 2011 recommendations of the German management guidelines was initiated. METHODS: A systemic literature review was performed, overarching principles were proposed and pre-selected via an online survey followed by two multidisciplinary consensus conferences. Pharmacological and non-pharmacological treatments were discussed, statements were proposed and ultimately agreed upon by nominal group technique (NGT). RESULTS: 12 overarching therapeutic principles, as well as 9 recommendations on pharmacological and 5 on non-pharmacological treatments for JIA were agreed upon. CONCLUSION: This report summarizes the recent update of the interdisciplinary, consensus-based German guidelines on the management of JIA. The multi- and interdisciplinary participation of all caregivers was central for this patient-focused update. With these guidelines, physicians can choose an evidence-based approach, which allows better tailored treatment in this vulnerable cohort of children and adolescents.

Psychological care in children and adolescents with type 1 diabetes in a real-world setting and associations with metabolic control.

BACKGROUND: International guidelines recommend psychosocial care for children and adolescents with type 1 diabetes. OBJECTIVE: To assess psychological care in children and adolescents with type 1 diabetes in a real-world setting and to evaluate associations with metabolic outcome. METHODS: Delivery of psychological care, HbA1c, and rates of severe hypoglycemia and diabetic ketoacidosis (DKA) in children and adolescents with type 1 diabetes from 199 diabetes care centers participating in the German diabetes survey (DPV) were analyzed. RESULTS: Overall, 12 326 out of 31 861 children with type 1 diabetes were supported by short-term or continued psychological care (CPC). Children with psychological care had higher HbA1c (8.0% vs 7.7%, P<.001) and higher rates of DKA (0.032 vs 0.021 per patient-year, P<.001) compared with children without psychological care. In age-, sex-, diabetes duration-, and migratory background-matched children, HbA1c stayed stable in children supported by CPC during follow-up (HbA1c 8.5% one year before psychological care started vs 8.4% after two years, P = 1.0), whereas HbA1c was lower but increased significantly by 0.3% in children without psychological care (HbA1c 7.5% vs 7.8% after two years, P <.001). Additional HbA1c-matching showed that the change in HbA1c during follow-up was not different between the groups, but the percentage of children with severe hypoglycemia decreased from 16.3% to 10.7% in children receiving CPC compared with children without psychological care (5.5% to 5.8%, P =.009). CONCLUSIONS: In this real-world setting, psychological care was provided to children with higher HbA1c levels. CPC was associated with stable glycemic control and less frequent severe hypoglycemia during follow-up.

The association between socio-economic status and diabetes care and outcome in children with diabetes type 1 in Germany: The DIAS study (diabetes and social disparities).

OBJECTIVE: To evaluate the association between socioeconomic status (SES) and diabetes outcomes in German children and adolescents. METHODS: A total of 1829 subjects <18 years old with type 1 diabetes mellitus from 13 German diabetes centers were included from June 2013 until June 2014. Data were collected within the multicenter DPV (Diabetes Prospective Follow-up) registry. SES was measured with a composite index. Multivariable regression models were applied to analyze the association of SES and outcomes adjusted for age, sex, diabetes duration, and migration status. RESULTS: Low SES was significantly associated with worse diabetes outcomes: higher hemoglobin A1C (HbA1c) (64.3 mmol/mol), lower proportion of insulin pump therapy (43.6%), fewer daily self-monitored blood glucose (SMBG) measurements (5.7), more inpatient days per patient-year (5.8) compared to patients with medium/high SES (HbA1c: 61.3 mmol/mol, P < 0.001/59.8 mmol/mol, P < 0.0001; proportion of pump therapy: 54.5%, P < 0.01/ 54.9%, P < 0.01; SMBG: 6.0, P < 0.01/ 6.1, P < 0.01; inpatient days: 4.5, P < 0.0001/3.4, P < 0.0001). The inclusion of migration status in the models resulted in only minor changes in the outcomes. CONCLUSION: Despite free health care, low SES is associated with unfavorable diabetes outcomes in Germany. The poorer diabetes outcomes of children with diabetes have been attributed to their migration status and may be partly explained by low SES. Both factors must become part of targeted diabetes care in children and adolescents with type 1 diabetes.

Oral contraception in adolescents with type 1 diabetes and its association with cardiovascular risk factors. A multicenter DPV study on 24 011 patients from Germany, Austria or Luxembourg.

OBJECTIVE: To investigate differences in cardiovascular risk factors and metabolic control in girls with type 1 diabetes with or without use of oral contraceptives (OC) from the multicenter "diabetes prospective follow-up" (DPV) registry. METHODS: Twenty-four thousand eleven adolescent girls (13 to < 18 years of age) from Germany, Austria or Luxembourg with type 1 diabetes from the DPV registry were included in this cross-sectional study. Multivariable regression models were applied to compare clinical characteristics (hemoglobin A1c [HbA1C ], blood pressure, serum lipids, body mass index) and lifestyle factors (smoking, physical inactivity, alcohol consumption) between girls with or without OC use. Confounders: age, diabetes duration and migration background. STATISTICAL ANALYSIS: SAS 9.4. RESULTS: In girls with type 1 diabetes and OC use, clinical characteristics and lifestyle factors were less favorable compared to non-users. Differences were most pronounced for the prevalence of dyslipidemia (OC-users: 40.0% vs non-users: 29.4; P < .0001) and the number of smokers (OC-users: 25.9% vs non-users: 12.5%; P < .0001). OC use, sociodemographic characteristics and lifestyle factors explained between 1 and 7% of the population variance in serum lipids and blood pressure. The use of OC explained a small additional proportion in all variables considered (<1%). CONCLUSIONS: OC use in adolescent girls with type 1 diabetes was associated with a poorer cardiovascular risk profile. Biological risk factors were partly explained by a clustering of sociodemographic and lifestyle factors with a small additional contribution of OC use. Prescription of OC should therefore be combined with a screening for cardiovascular risk factors and targeted education.

[How are Effects of Patient Education Programs Influenced by Psychosocial Risk Factors in Pediatrics?] / Wie beeinflussen psychosoziale Risiken den Erfolg von Patientenschulungen in der Pädiatrie?

BACKGROUND: Modular patient education programs are effective in children with chronic conditions and their families. Little is known about the influence of socioeconomic status (SES), migration background (MB) and children's mental-health problems on the programs' effects. OBJECTIVES: Do SES, MB or mental-health problems influence the success of education programs (disease-specific knowledge, children's health-related quality of life (HRQoL) and life satisfaction and parents' condition-specific burden)? MATERIALS AND METHODS: Children with different chronic conditions and their parents participated in modular patient education programs. Before and 6 weeks after the participation SES, MB, children's mental-health problems, parents' und children's disease-specific knowledge, children's HRQoL and life satisfaction and parents' condition-specific burden were assessed by standardized questionnaires. The influence on the programs' effects of SoS, MH and mental-health problems were examined with variance and correlation analyses. RESULTS: 398 children (mean age 10.2 yrs) and their parents participated. Irrespective of SoS, MH and mental-health problems the programs were associated with improved disease-specific knowledge, children's HRQoL and life satisfaction and parents' disease-specific burden. At follow-up SoS, MH and mental-health problems were associated with reduced knowledge, reduced children's' HRQoL and life satisfaction and increased parents' disease-specific burden. CONCLUSIONS: Disadvantaged families and children with mental-health problems benefit from education programs, but have an increased need of education due to special challenges.

Comorbidity of type 1 diabetes and juvenile idiopathic arthritis.

OBJECTIVE: To analyze the prevalence of juvenile idiopathic arthritis (JIA) and diabetes end points in pediatric patients with type 1 diabetes. STUDY DESIGN: Patients with type 1 diabetes, recorded from 1995 up to September 2013 in the Diabetes Patienten Verlaufsdokumentation database (n = 54,911, <16 years of age, 47% girls), were analyzed. The patients' height, weight, and body mass index SDS, glycosylated hemoglobin A1c (HbA1c); insulin dose; hypertension and dyslipidemia prevalence; rate of hypoglycemic events; and ketoacidosis were compared between patients with and without JIA. To adjust for age, sex, diabetes duration, and migration background, data were analyzed in hierarchic multivariable regression models. RESULTS: The prevalence of JIA in type 1 diabetes was 106 of 54,911 patients; 66% were girls. Diabetes onset was earlier in children with JIA (7.2 years vs 8.3 years, P = .04). Children with JIA were smaller (SDS: -0.22 vs 0.09, P = .004). Correspondingly, weight SDS was lower in patients with JIA (-0.02 vs 0.22, P = .01). Body mass index SDS did not differ. HbA1c was marginally lower in children with JIA (63 mmol/mol [8.0%] vs 67 mmol/mol [8.3%], P = .06). Insulin requirement was greater in patients with JIA (1.03 vs 0.93 insulin units/weight/day, P = .003). Hypertension and dyslipidemia were comparable in both groups. CONCLUSIONS: The JIA-prevalence in patients with type 1 diabetes (0.19%) was considerably greater than in the general population (0.05%). Growth is influenced negatively by JIA. Surprisingly, HbA1c was somewhat lower in children with JIA, possibly because of a more intensive treatment or a latent hemolysis caused by the inflammation.

Depression, metabolic control, and antidepressant medication in young patients with type 1 diabetes.

OBJECTIVE: Recent literature suggests an association between type 1 diabetes (T1D) and depression. So far, most studies explored this link in adult populations, with few data being available on diabetes and depression from minors and young adults. This study aimed to look for associations between symptoms of depression/antidepressant treatment and metabolic outcomes of T1D. METHODS: We conducted an observational study using the German diabetes database (Diabetes-Patienten-Verlaufsdokumentation--DPV) and searched for patients up to the age of 25 yr, with depressive symptoms and/or receiving antidepressant medication. RESULTS: Of 53 986 T1D patients below the age of 25 yr, antidepressant medication and/or depressive symptoms were reported in 419 (0.78%). After adjustment for age, gender, diabetes duration and center heterogeneity, minors and young adults with depressive symptoms showed worse outcome parameters such as a higher rate of severe hypoglycemia (0.56 vs. 0.20/patient year, p = 0.005) and more episodes of diabetic ketoacidosis (0.20 vs. 0.07/patient year, p < 0.001). Hemoglobin A1c (HbA1c) was higher in the depression group (74.50 vs. 67.58 mmol/mol, p < 0.001) and young patients with T1D and depression showed longer duration of inpatient treatment (7.04 vs. 3.10 hospital days/patient year, p < 0.001) and more frequent admissions to hospital care (0.63 vs. 0.32/patient year, p < 0.001). Antidepressant medication was recorded in 52.3% of the depressed patients, with selective serotonin reuptake inhibitors (SSRIs) being the most widely described class of antidepressants (29.1%). CONCLUSIONS: Our findings demonstrate an adverse treatment outcome for young patients with T1D and comorbid depressive symptoms underlining an urgent need for collaborative mental and somatic health care for patients with T1D and depression.

Family structures and parents' occupational models: its impact on children's diabetes.

AIMS: This study examines how family-related factors influence the management of children and adolescents with type 1 diabetes (T1DM). We investigate the relationship between family patterns, parental work schedules and metabolic control. MATERIALS AND METHODS: We analysed data from a nationwide diabetes survey (DPV) focusing on HbA1c, severe hypoglycaemia, diabetic ketoacidosis, hospital admissions and inpatient treatment duration. We used linear regression and negative binomial regression models. Our study includes 15,340 children under the age of 18 with data on family structure and parental division of labour. RESULTS: Children from two-parent households have better HbA1c outcomes than children from single-parent, blended or no-parent households (p < .0001). Higher HbA1C levels are associated with children living with an unemployed father, as opposed to those with full-time working parents or with a full-time working father and a part-time working mother (p < .001). CONCLUSIONS: These findings emphasise the importance of carefully considering family structure and working time models in the management of paediatric T1DM. Our results highlight risk factors within the family environment and emphasise the need for family-focused counselling of high-risk patients or severe cases in clinical practice.

Mental disorders in children and adolescents with type 1 diabetes before and during the COVID-19 pandemic: results from the DPV registry.

OBJECTIVES: The COVID-19 pandemic affected the mental health of children and adolescents in the general population, yet its impact on those with chronic conditions is relatively unknown. This study aimed to compare the incidences of comorbid mental disorders and substance misuse in children and adolescents with type 1 diabetes before and during the pandemic. METHODS: A total of 42,975 patients aged 6-18 years from the multicentre DPV (Diabetes prospective follow-up) registry were included. Multivariable regression models were applied to compare newly diagnosed comorbid mental disorders, adjusted for demographic and clinical variables, among them the number of medical visits, during the pre-pandemic period (09/2017-02/2020) and the COVID-19 pandemic period (03/2020-08/2022). RESULTS: Analysing both sexes together, there were no differences in the incidence rates of overall mental disorders between the pandemic and the pre-pandemic period. However, girls showed an increased incidence rate (odds ratio 1.2, CI 1.1-1.3) during the pandemic. Adolescent girls also displayed higher incidence rates of depression, eating disorders, and self-harm. Substance misuse declined overall during the pandemic (odds ratio 0.8, CI 0.7-0.9). CONCLUSIONS: During the COVID-19 pandemic, we found higher incidence rates of overall mental disorders in girls, but not in boys and not in the total study population of children and adolescents with type 1 diabetes. Adolescent girls displayed increased incidence rates of depression, eating disorders, and self-harm. Substance misuse declined substantially. Clinicians should be aware of the high-risk group of adolescent girls during times of increased strain.

Metabolic control during the first two years of the COVID-19 pandemic in pediatric patients with type 1 diabetes: results from the German DPV initiative.

AIM: To assess effects of the SARS-CoV2 pandemic on metabolic control in youth with type 1 diabetes (T1D) in Germany in a population-based analysis. METHODS: Data from 33,372 pediatric T1D patients from the Diabetes Prospective Follow-up (DPV) registry, with face-to-face visits or telemedicine contacts in the years 2019-2021, were available. Datasets from eight time periods between March 15, 2020, and December 31, 2021, according to SARS-CoV2 incidence waves, were compared to those from five control time periods. Parameters of metabolic control were assessed with adjustment for sex, age, diabetes duration, and repeated measurements. Laboratory-measured HbA1c values and those estimated from CGM were aggregated into a combined glucose indicator (CGI). RESULTS: There was no clinically relevant difference in metabolic control between pandemic and control time periods with adjusted CGI values ranging from 7.61% [7.60-7.63] (mean [95% confidence interval (CI)]) in the third quarter of 2019 to 7.83% [7.82-7.85] in the time period from January 1 to March 15 2020, in the other control periods, and during the pandemic, CGI values lay between these values. BMI-SDS rose during the pandemic from 0.29 [0.28-0.30] (mean [95% CI]) in the third quarter of 2019 to 0.40 [0.39-0.41] during the fourth wave. Adjusted insulin dose rose during the pandemic. Event rates for hypoglycemic coma and diabetic ketoacidosis remained unchanged. CONCLUSIONS: We found no clinically relevant change of glycemic control or incidence of acute diabetes complications during the pandemic. The observed BMI increase may represent an important health risk for youth with T1D.

A syndrome with congenital neutropenia and mutations in G6PC3.

BACKGROUND: The main features of severe congenital neutropenia are the onset of severe bacterial infections early in life, a paucity of mature neutrophils, and an increased risk of leukemia. In many patients, the genetic causes of severe congenital neutropenia are unknown. METHODS: We performed genomewide genotyping and linkage analysis on two consanguineous pedigrees with a total of five children affected with severe congenital neutropenia. Candidate genes from the linkage interval were sequenced. Functional assays and reconstitution experiments were carried out. RESULTS: All index patients were susceptible to bacterial infections and had very few mature neutrophils in the bone marrow; structural heart defects, urogenital abnormalities, and venous angiectasia on the trunk and extremities were additional features. Linkage analysis of the two index families yielded a combined multipoint lod score of 5.74 on a linkage interval on chromosome 17q21. Sequencing of G6PC3, the candidate gene encoding glucose-6-phosphatase, catalytic subunit 3, revealed a homozygous missense mutation in exon 6 that abolished the enzymatic activity of glucose-6-phosphatase in all affected children in the two families. The patients' neutrophils and fibroblasts had increased susceptibility to apoptosis. The myeloid cells showed evidence of increased endoplasmic reticulum stress and increased activity of glycogen synthase kinase 3beta (GSK-3beta). We identified seven additional, unrelated patients who had severe congenital neutropenia with syndromic features and distinct biallelic mutations in G6PC3. CONCLUSIONS: Defective function of glucose-6-phosphatase, catalytic subunit 3, underlies a severe congenital neutropenia syndrome associated with cardiac and urogenital malformations.

Working Towards a Treat-to-Target Protocol in Juvenile Proliferative Lupus Nephritis - A Survey of Pediatric Rheumatologists and Nephrologists in Germany and Austria.

Background: To describe treatment practices for juvenile proliferative lupus nephritis (LN) class III and IV of pediatric rheumatologists and nephrologists in Germany and Austria in preparation for a treat-to-target treatment protocol in LN. Methods: Survey study by members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Pediatric Nephrology (GPN) on diagnostics and (concomitant) therapy of LN. Results: Fifty-eight physicians completed the survey. Overall, there was a considerable heterogeneity regarding the suggested diagnostics and management of juvenile proliferative LN. Increased urinary protein excretion, either assessed by 24 h urine collection or spot urine (protein-creatinine ratio), and reduced estimated glomerular filtration rate were specified as important parameters for indication of kidney biopsy to diagnose proliferative LN and monitoring of therapy. Corticosteroids were generally proposed for induction and maintenance therapy, most often in conjunction with either mycophenolate mofetil (MMF) or cyclophosphamide (CP) as steroid-sparing immunosuppressants. MMF was clearly preferred over CP for induction therapy of LN class III, whereas CP and MMF were equally proposed for LN class IV. MMF was most often recommended for maintenance therapy in conjunction with oral corticosteroids and continued for at least 3 years and 1 year, respectively, after remission. Hydroxychloroquine was widely accepted as a concomitant measure followed by renin-angiotensin system inhibitors in cases of arterial hypertension and/or proteinuria. Conclusion: The majority of pediatric rheumatologists and nephrologists in Germany and Austria propose the use of corticosteroids, most often in combination with either MMF or CP, for treatment of proliferative LN in children. The considerable heterogeneity of responses supports the need for a treat-to-target protocol for juvenile proliferative LN between pediatric rheumatologists and nephrologists.

[Is it possible to prevent diabetic ketoacidosis at diagnosis of pediatric type 1 diabetes? Lessons from the COVID-19 pandemic]. / Kann die Ketoacidose bei pädiatrischen Patienten mit Manifestation eines Diabetes mellitus Typ 1 vermieden werden? Lehren aus der COVID-19-Pandemie.

BACKGROUND: Diabetic ketoacidosis (DKA) is a life-threatening emergency in children and adolescents with manifestation of type 1 diabetes mellitus (DM1) and often associated with delayed diagnosis or previous diagnostic errors. During the coronavirus disease 2019 (COVID-19) lockdown period in Germany, less patients presented at emergency departments and private practices. OBJECTIVE: The aim of this study was to investigate the DKA risk in children and adolescents with DM1 manifestation during the COVID-19 lockdown and associated risk factors. MATERIAL AND METHODS: The frequency of DKA at DM1 onset in patients <18 years between 13 March and 13 May 2020 in pediatric diabetes centers was analyzed. The centers also documented their assessment, if the presentation was delayed or the diagnosis was not made on the first medical consultation. In order to analyze the influence of the risk factors on the frequency of DKA, the data from 2020 were compared with the same periods in 2018 and 2019 using multivariable linear and logistic regression. RESULTS: The data of 532 patients from 216 diabetes centers showed that the risk for DKA increased by 84.7% and the risk for severe DKA increased by 45.3% compared to the years 2018/2019. Children <6 years had the highest risk with an 141.6% increase for DKA and 97.0% for severe DKA compared to the previous years. Migration background was a risk factor independent of COVID-19. Of the patients 31% had either a delayed presentation or a missed diagnosis. CONCLUSION: During the COVID-19 lockdown the frequency of DKA and severe DKA at DM1 onset was significantly increased for children and adolescents in Germany. Age <6 years, migration background and delayed diagnosis were the main risk factors.

Declining Frequency of Acute Complications Associated with Tubeless Insulin Pump Use: Data from 2,911 Patients in the German/Austrian Diabetes Patienten Verlaufsdokumentation Registry.

Objective: To characterize patients with diabetes treated with a tubeless insulin pump (Omnipod® Insulin Management System; Insulet Corp., Acton, MA), and to evaluate the frequency of acute complications with long-term use of the system. Methods: This retrospective analysis of the German/Austrian Diabetes Patienten Verlaufsdokumentation (DPV) registry included data from 3657 patients with diabetes (n = 3582 type 1, n = 25 type 2, n = 50 latent autoimmune diabetes in adults/other) treated with a tubeless insulin pump. Hemoglobin A1c (HbA1c) levels and frequency of diabetic ketoacidosis (DKA) and severe hypoglycemia (SH) were compared between 1 year pre- and 1 year (n = 2911) or up to 3 years (n = 1311) post-tubeless insulin pump initiation and compared with a contemporary cohort on multiple daily injections (MDI) with 3-year data (n = 1874). Results: Patients using tubeless insulin pump therapy had a median age of 13.7 years [interquartile range 10.8, 17.3], diabetes duration 3.7 years [1.7, 8.0], and HbA1c 7.5% [6.9, 8.2]. In patients with 3 years of follow-up data (n = 1311), the percentage with ≥1 episode of DKA, SH (Level 3, requiring assistance), and SH (coma) event with prior treatment was 6.3%, 5.5%, and 1.7%, respectively. After 3 years of tubeless insulin pump therapy, the frequency of DKA, SH (Level 3), and SH (coma) decreased to 2.2%, 4.1%, and 0.5%, respectively. Both DKA and SH remained significantly lower compared with MDI after adjustment in multiple regression analysis. High treatment retention rates (>90%) were observed. Conclusion: Real-world registry data document that tubeless insulin pump therapy is associated with good glycemic control and a low frequency of DKA and SH in an age group prone to acute complications.

Incidence of COVID-19 and Risk of Diabetic Ketoacidosis in New-Onset Type 1 Diabetes.

OBJECTIVES: With this study, our aim was to quantify the relative risk (RR) of diabetic ketoacidosis at diagnosis of type 1 diabetes during the year 2020 and to assess whether it was associated with the regional incidence of coronavirus disease 2019 (COVID-19) cases and deaths. METHODS: Multicenter cohort study based on data from the German Diabetes Prospective Follow-up Registry. The monthly RR for ketoacidosis in 2020 was estimated from observed and expected rates in 3238 children with new-onset type 1 diabetes. Expected rates were derived from data from 2000 to 2019 by using a multivariable logistic trend regression model. The association between the regional incidence of COVID-19 and the rate of ketoacidosis was investigated by applying a log-binomial mixed-effects model to weekly data with Germany divided into 5 regions. RESULTS: The observed versus expected frequency of diabetic ketoacidosis was significantly higher from April to September and in December (mean adjusted RRs, 1.48-1.96). During the first half of 2020, each increase in the regional weekly incidence of COVID-19 by 50 cases or 1 death per 100 000 population was associated with an increase in the RR of diabetic ketoacidosis of 1.40 (95% confidence interval, 1.10-1.77; P = .006) and 1.23 (1.14-1.32; P < .001), respectively. This association was no longer evident during the second half of 2020. CONCLUSIONS: These findings suggest that the local severity of the pandemic rather than health policy measures appear to be the main reason for the increase in diabetic ketoacidosis and thus the delayed use of health care during the pandemic.

Diabetes care in pediatric refugees from Africa or Middle East: experiences from Germany and Austria based on real-world data from the DPV registry.

OBJECTIVE: With increasing migration to Europe, diabetes diagnosis and treatment of refugees became challenging. To describe the current experience with pediatric refugees in Germany and Austria. DESIGN AND METHODS: 43,137 patients (<21 years) with type 1 diabetes from the diabetes patient follow-up registry (DPV) were studied and divided by refugee status into patients born in Middle East (n = 365) or Africa (n = 175) and native patients (child and parents born in Germany/Austria; G/A: n = 42,597). Groups were compared using multivariable regression adjusted for age, sex and diabetes duration (SAS 9.4). In refugees the first year after arrival was studied, and for native children the most recent year of care. RESULTS: After adjustment, HbA1c was highest in refugees (1. ME and 2. AFR vs 3. G/A: 72.3 ± 1.0 and 75.0 ± 1.4 vs 66.0 ± 0.1 mmol/mol, 1 vs 3: P < 0.001 and 2 vs 3: P < 0.001) and microalbuminuria (9.9 and 13.6 vs 6.5%, 1 vs 3: P = 0.039 and 2 vs 3: P = 0.002) was more prevalent. African children experienced severe hypoglycemia (17.8 ± 4.3 and 25.4 ± 8.7 vs 11.5 ± 0.3 per 100 patient years, 1 vs 3: P > 0.05 and 2 vs 3: P = 0.045) significantly more often, whereas hypoglycemia with coma (5.1 ± 1.1 and 4.1 ± 1.6 vs 2.6 ± 0.1 per 100 patient years, 1 vs 3: P = 0.006 and 2 vs 3: P > 0.05) and retinopathy (2.1 and n/a vs 0.2%, 1 vs 3: P < 0.001) were significantly more common in children from Middle East compared to natives. Insulin pumps were used in a markedly larger proportion of native patients (7.4 and 13.2 vs 43.0%, 1 vs 3: P < 0.001 and 2 vs 3: P < 0.001). CONCLUSIONS: A relevant number of pediatric refugees with type 1 diabetes are treated in German/Austrian diabetes clinics. Refugee children, parents and caregivers are faced with several problems in diabetes therapy and outcome that should be addressed more intensively by pediatric diabetes teams.

The majority of patients with newly diagnosed juvenile idiopathic arthritis achieve a health-related quality of life that is similar to that of healthy peers: results of the German multicenter inception cohort (ICON).

BACKGROUND: Achieving the best possible health-related quality of life (HRQoL) for a patient is an important treatment goal in juvenile idiopathic arthritis (JIA). We investigated the 36-month trajectories of HRQoL in children with JIA compared with healthy peers and identified the predictors of an unfavorable HRQoL. METHODS: Patients with a recent JIA diagnosis were enrolled in the German inception cohort study ICON. As a peer group, friends of patients of the same age and sex were asked to cooperate. Children were prospectively followed and regularly questioned about their HRQoL using the Pediatric Quality of Life Inventory 4.0 (PedsQL). Disease activity was assessed by the clinical Juvenile Arthritis Disease Activity Score (cJADAS-10), and the burden of the child's chronic illness on their family was assessed by the Family Burden Questionnaire (FaBel). Linear mixed models were used to compare the HRQoL of the patients and their peers. Associations between the health status of a patient at enrollment and an unfavorable HRQoL (PedsQL total < 79.3) at their 3-year follow-up (FU) were analyzed by logistic regression. RESULTS: Data from 953 patients (median symptom duration 6 months, mean age 7.9 years) and 491 healthy peers (aged 8.4 years) were analyzed. During 3 years of FU, the disease activity and HRQoL of the patients improved significantly (cJADAS-10 from 9.8 (6.2) to 2.7 (3.6) and PedsQL total score from 71.7 (18.2) to 87.3 (13.9)). While the HRQoL of the patients varied among the several JIA categories at the time of enrollment, no significant differences were found at the 3-year FU. After 36 months, the HRQoL of the patients had largely converged with that of their healthy peers. JIA patients had a psychosocial health status comparable with their healthy peers, whereas a small significant mean difference remained in physical health (5.8, 95% confidence interval (CI) 4.1-7.6). Up to the 36-month FU, three-quarters of JIA patients attained a favorable HRQoL (PedsQL ≥ 79.3) which was achieved by 90% of the peers. A higher family burden, higher pain level, and lower well-being at enrollment were associated with an unfavorable HRQoL. CONCLUSIONS: Under current therapeutic conditions, an HRQoL corresponding with that of healthy children is a realistic treatment goal in JIA.

Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany.

BACKGROUND: Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany. METHODS: We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany. Subsequently, via online surveys and teleconferences among pediatric rheumatologists with a special expertise in the treatment of SJIA, we identified current diagnostic and treatment approaches in Germany. Those were harmonized via the formulation of statements and, supported by findings from a literature search. Finally, an in-person consensus conference using nominal group technique was held to further modify and consent the statements. RESULTS: Up to 50% of patients diagnosed with SJIA in Germany do not fulfill the International League of Associations for Rheumatology (ILAR) classification criteria, mostly due to the absence of chronic arthritis. Our findings suggest that chronic arthritis is not obligatory for the diagnosis and treatment of SJIA, allowing a diagnosis of probable SJIA. Malignant, infectious and hereditary autoinflammatory diseases should be considered before rendering a diagnosis of probable SJIA. There is substantial variability in the initial treatment of SJIA. Based on registry data, most patients initially receive systemic glucocorticoids, however, increasingly substituted or accompanied by biological agents, i.e. interleukin (IL)-1 and IL-6 blockade (up to 27.2% of patients). We identified preferred initial therapies for probable and definitive SJIA, including step-up patterns and treatment targets for the short-term (resolution of fever, decrease in C-reactive protein by 50% within 7 days), the mid-term (improvement in physician global and active joint count by at least 50% or a JADAS-10 score of maximally 5.4 within 4 weeks) and the long-term (glucocorticoid-free clinically inactive disease within 6 to 12 months), and an explicit treat-to-target strategy. CONCLUSIONS: We developed consensus-based strategies regarding the diagnosis and treatment of probable or definitive SJIA in Germany.