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Katharine C Garvey,1 Jonathan A Finkelstein,2,3 Lori M Laffel,1,4 Victoria Ochoa,1 Joseph I Wolfsdorf,1 Erinn T Rhodes1 1Division of Endocrinology, Boston Children's Hospital, 2Division of General Pediatrics, Boston Children's Hospital, 3Department of Population Medicine, Harvard Pilgrim Health Care Institute, 4Pediatric, Adolescent and Young Adult Section, Joslin Diabetes Center, Boston, MA, USA Background: The purpose of this study was to describe the current status of adult diabetes care in young adults with type 1 diabetes and examine associations between health care transition experiences and care utilization. Methods: We developed a survey to assess transition characteristics and current care in young adults with type 1 diabetes. We mailed the survey to the last known address of young adults who had previously received diabetes care at a tertiary pediatric center. Results: Of 291 surveys sent, 83 (29%) were undeliverable and three (1%) were ineligible. Of 205 surveys delivered, 65 were returned (response rate 32%). Respondents (mean age 26.6 ± 3.0 years, 54% male, 91% Caucasian) transitioned to adult diabetes care at a mean age of 19.2 ± 2.8 years. Although 71% felt mostly/completely prepared for transition, only half received recommendations for a specific adult provider. Twenty-six percent reported gaps exceeding six months between pediatric and adult diabetes care. Respondents who made fewer than three diabetes visits in the year prior to transition (odds ratio [OR] 4.5, 95% confidence interval [CI] 1.2–16.5) or cited moving/relocation as the most important reason for transition (OR 6.3, 95% CI 1.3–31.5) were more likely to report gaps in care exceeding six months. Patients receiving current care from an adult endocrinologist (79%) were more likely to report at least two diabetes visits in the past year (OR 6.0, 95% CI 1.5–24.0) compared with those receiving diabetes care from a general internist/adult primary care doctor (17%). Two-thirds (66%) reported receiving all recommended diabetes screening tests in the previous year, with no difference according to provider type. Conclusion: In this sample, transition preparation was variable and one quarter reported gaps in obtaining adult diabetes care. Nevertheless, the majority endorsed currently receiving regular diabetes care, although visit frequency differed by provider type. Because locating patients after transition was incomplete, our findings suggest the need for standardized methods to track transitioning patients. Keywords: type 1 diabetes mellitus, health care delivery, young adults, transition to adult care

Objective: To examine characteristics of the transition from pediatric to adult care in emerging adults with type 1 diabetes and evaluate associations between transition characteristics and glycemic control.Research Design and Methods: We developed and mailed a survey to evaluate the transition process in emerging adults with type 1 diabetes, aged 22 to 30 years, receiving adult diabetes care at a single center. Current A1C data were obtained from the medical record.Results: The response rate was 53% (258 of 484 eligible). The mean transition age was 19.5 ± 2.9 years, and 34% reported a gap >6 months in establishing adult care. Common reasons for transition included feeling too old (44%), pediatric provider suggestion (41%), and college (33%). Less than half received an adult provider recommendation and <15% reported having a transition preparation visit or receiving written transition materials. The most recent A1C was 8.1 ± 1.3%. Respondents who felt mostly/completely prepared for transition had lower likelihood of a gap >6 months between pediatric and adult care (adjusted odds ratio 0.47 [95% CI 0.25-0.88]). In multivariate analysis, pretransition A1C (β = 0.49, P < 0.0001), current age (β = -0.07, P = 0.03), and education (β = -0.55, P = 0.01) significantly influenced current posttransition A1C. There was no independent association of transition preparation with posttransition A1C (β = -0.17, P = 0.28).Conclusions: Contemporary transition practices may help prevent gaps between pediatric and adult care but do not appear to promote improvements in A1C. More robust preparation strategies and handoffs between pediatric and adult care should be evaluated. [ABSTRACT FROM AUTHOR]

Agus MSD, Alexander JL, Wolfsdorf JI. Utility of immediate hemoglobin A1c in children with type I diabetes mellitus. Objective: Immediate feedback (IFB) of hemoglobin A1c (HbA1c) results to adults with type 1 and 2 diabetes allows more appropriate care decisions at the clinic visit and may improve glycemic control. Our objective is to determine whether IFB of HbA1c results to children with type 1 diabetes will improve patient care and glycemic control. Methods: In this prospective randomized controlled trial, children under 18 years of age were randomly assigned to receive HbA1c results during their diabetes clinic visit by point-of-care fingerstick testing (immediate) or several days after by venipuncture and laboratory assessment (conventional). HbA1c levels, therapy changes, and painfulness of testing were recorded at baseline and every follow-up appointment for a year. Results: The 215 patients studied had similar baseline characteristics including initial HbA1c (7.90 ± 1.24% vs. 7.81 ± 1.13%, p = 0.25). IFB improved HbA1c at 3 months (−0.20 ± 0.66%, p = 0.005) with a return to baseline for the remainder of the study. Subjects receiving conventional feedback had increased HbA1c results at 12 months (+0.27 ± 1.05%, p = 0.048). Less frequent patient-clinician communication between visits was reported with IFB (0.29 ± 0.48 vs. 0.38 ± 0.49 contacts/visit, p = 0.043). Subjects rated fingersticks as less painful than conventional venipuncture (0.30 ± 0.66 vs. 3.9 ± 2.6, p < 0.001). Conclusions: IFB of HbA1c is a more acceptable method of HbA1c determination in children with type 1 diabetes mellitus. Although sustained improvements in glycemic control did not result from this intervention alone, IFB testing resulted in more efficient patient-clinician communication and was less painful. [ABSTRACT FROM AUTHOR]

Objective: Insulin's role in body weight regulation is controversial. We evaluated the effect of parenteral insulin on body weight and physical development in children and adolescents at risk for type 1 diabetes.Research Design and Methods: We performed a secondary analysis of the parenteral arm of the Diabetes Prevention Trial-Type 1 Diabetes (DPT-1), a randomized controlled trial of low-dose parenteral insulin (human ultralente insulin at 0.25 units x kg(-1) x day(-1)) in subjects with a >50% 5-year risk of diabetes. Analysis was limited to 100 subjects (55 intervention, 45 closely monitored) aged <19 years at randomization whose weight was followed for at least 2 years by study end after excluding subjects who were noncompliant within 2 years or developed diabetes within 36 months of randomization.Results: Subjects ranged in age from 4.07 to 18.98 years. There were no significant differences at randomization between subjects in each group with respect to sex, age, weight, height, BMI, Tanner stage, or glucose tolerance. We found no differences over 2 years between the intervention and closely monitored groups in the change in weight (median 6.8 vs. 6.0 kg, P = 0.65), height (median 10.7 vs. 10.1 cm, P = 0.66), BMI (median 0.9 vs. 1.0 kg/m2, P = 0.79), or Tanner stage (median 0 vs. 0, P = 0.35). Multiple regression showed no effect of insulin on change in weight (P = 0.53) or BMI (P = 0.95) over 2 years after adjustment for relevant covariates.Conclusions: Low-dose insulin treatment for 2 years did not affect the weight, BMI, or physical development of nondiabetic children and adolescents. [ABSTRACT FROM AUTHOR]

Sick children often have a decreased appetite and experience vomiting and diarrhea; however, hypoglycemia (plasma glucose concentration ≤50 mg/dL or 2.8 mmol/L) is rare. Ketotic hypoglycemia (KH) is the most common cause of hypoglycemia presenting to an Emergency Department in a previously healthy child between 6 months and 6 years of age. Ketosis and hypoglycemia are now well understood to be normal physiologic responses of young children to prolonged fasting.There is now substantial evidence that the term KH describes a variety of conditions including both the lower end of the normal distribution of fasting tolerance in young children as well as numerous rare disorders that impair fasting adaptation. Recent advances in molecular genetic testing have led to the discovery of these rare disorders. Idiopathic pathological KH is a diagnosis of exclusion that describes rare children who have abnormally limited fasting tolerance, experience recurrent episodes of KH, or develop symptoms of hypoglycemia despite elevated ketone levels, and in whom an explanation cannot be found despite extensive investigation. This review provides an approach to distinguishing between physiological KH and pathological KH and includes recommendations for management., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Context: Hypoglycemia in people with diabetes is common, especially in those taking medications such as insulin and sulfonylureas (SU) that place them at higher risk. Hypoglycemia is associated with distress in those with diabetes and their families, medication nonadherence, and disruption of life and work, and it leads to costly emergency department visits and hospitalizations, morbidity, and mortality., Objective: To review and update the diabetes-specific parts of the 2009 Evaluation and Management of Adult Hypoglycemic Disorders: Endocrine Society Clinical Practice Guideline and to address developing issues surrounding hypoglycemia in both adults and children living with diabetes. The overriding objectives are to reduce and prevent hypoglycemia., Methods: A multidisciplinary panel of clinician experts, together with a patient representative, and methodologists with expertise in evidence synthesis and guideline development, identified and prioritized 10 clinical questions related to hypoglycemia in people living with diabetes. Systematic reviews were conducted to address all the questions. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology was used to assess the certainty of evidence and make recommendations., Results: The panel agreed on 10 questions specific to hypoglycemia risk and prevention in people with diabetes for which 10 recommendations were made. The guideline includes conditional recommendations for use of real-time continuous glucose monitoring (CGM) and algorithm-driven insulin pumps in people with type 1 diabetes (T1D), use of CGM for outpatients with type 2 diabetes at high risk for hypoglycemia, use of long-acting and rapid-acting insulin analogs, and initiation of and continuation of CGM for select inpatient populations at high risk for hypoglycemia. Strong recommendations were made for structured diabetes education programs for those at high risk for hypoglycemia, use of glucagon preparations that do not require reconstitution vs those that do for managing severe outpatient hypoglycemia for adults and children, use of real-time CGM for individuals with T1D receiving multiple daily injections, and the use of inpatient glycemic management programs leveraging electronic health record data to reduce the risk of hypoglycemia., Conclusion: The recommendations are based on the consideration of critical outcomes as well as implementation factors such as feasibility and values and preferences of people with diabetes. These recommendations can be used to inform clinical practice and health care system improvement for this important complication for people living with diabetes., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

The European Society for Paediatric Endocrinology (ESPE) interactive website, https://www.espe-elearning.org, was first published online in 2012. We describe the various applications of the content of the e-learning website that has been greatly expanded over the last 10 years. A large module on pediatric diabetes was added with the support of the International Society for Paediatric and Adolescent Diabetes (ISPAD). A separate multilingual module was created that focuses on frontline health care providers in limited resource settings. This module has been well received, particularly in targeted parts of the world. e-Learning may also be an opportunity to expand or tailor educational activities for learners according to their differing learning needs. The e-learning website provides guidelines for those interested in general pediatrics, neonatology, clinical genetics, and pediatric gynecology. We also describe various new applications such as master classes in the format of interactive video lectures and joint and complementary e-learning/e-consultation webinars. Finally, international certification was recently realized as e-learning courses were recognized by the European Accreditation Council for Continuing Medical Education (EACCME). As a result of the social distancing measures introduced to control the COVID-19 pandemic, digital education, whether individual or in a virtual classroom setting, has become even more important since e-learning can connect and engage individuals across geographic boundaries as well as those who live in remote areas. The future of education delivery may include hybrid learning strategies, which include in-person and e-learning platforms. Combined e-learning and e-consultation webinars illustrate how international academic institutions, learned medical specialty societies and networks are uniquely placed to deliver balanced, disease-oriented, and patient-centered e-learning education and at the same time provide expert consultation. Moreover, they are well equipped to maintain professional standards and to offer appropriate accreditation., (© 2022 The Author(s). Published by S. Karger AG, Basel.)

Purpose of Review: This article reviews recent developments in methods used to monitor paediatric type 1 diabetes (T1D), including an examination of the role of glycated haemoglobin (haemoglobin A1c) and its limitations for long-term assessment of glycaemia in individual patients, self-monitoring of blood glucose, continuous glucose monitoring (CGM) systems and ketone monitoring., Recent Findings: Monitoring of glycemia and ketones, when indicated, is a cornerstone of paediatric T1D management and is essential to optimize glycaemic control. Ongoing technological advancements have led to rapid changes and considerable improvement in the methods used to monitor glucose concentrations in people with T1D. As a result of recent innovations that have enhanced accuracy and usability, CGM is now considered the optimal method for monitoring glucose concentrations and should be introduced soon after diagnosis of T1D., Summary: Patients/families and healthcare providers must receive comprehensive education and proper training in the use of CGM and interpretation of the vast amounts of data. Future challenges include ensuring equal access to and optimizing clinical use of CGM to further improve T1D care and outcomes., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Diabetic ketoacidosis (DKA) is a common emergency department presentation of both new-onset and established diabetes mellitus (DM). β-Hydroxybutyrate (BOHB) provides a direct measure of the pathophysiologic derangement in DKA as compared with the nonspecific measurements of blood pH and bicarbonate. Our objective was to characterize the relationship between BOHB and DKA., Methods: This is a cross-sectional retrospective study of pediatric patients with DM presenting to an urban pediatric emergency department between January 1, 2016, and September 30, 2018. Analyses were performed on each patient's initial, simultaneous BOHB and pH. Diagnostic test characteristics of BOHB were calculated, and logistic regression was performed to investigate the effects of age and other key clinical factors., Results: Among 594 patients with DM, with median age of 12.3 years (interquartile range, 8.7-15.9 years), 176 (29.6%) presented with DKA. The inclusion of age, transfer status, and new-onset in the statistical model did not improve the prediction of DKA beyond BOHB alone. β-Hydroxybutyrate demonstrated strong discrimination for DKA, with an area under the curve of 0.95 (95% confidence interval, 0.93-0.97). A BOHB value of 5.3 mmol/L predicted DKA with optimal accuracy (90.6% of patients were correctly classified). The sensitivity, specificity, and positive and negative predictive values of this cut point were 76.7% (95% confidence interval, 69.8%-82.7%), 96.4% (94.2%-98.0%), 90.0% (84.0%-94.3%), and 90.8% (87.7%-93.3%), respectively., Conclusions: β-Hydroxybutyrate accurately predicts DKA in children and adolescents. More importantly, because plasma BOHB is the ideal biochemical marker of DKA, BOHB may provide a more optimal definition of DKA for management decisions and treatment targets., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Carbohydrate restriction, used since the 1700s to prolong survival in people with diabetes, fell out of favor after the discovery of insulin. Despite costly pharmacological and technological developments in the last few decades, current therapies do not achieve optimal outcomes, and most people with diabetes remain at high risk for micro- and macrovascular complications. Recently, low-carbohydrate diets have regained popularity, with preliminary evidence of benefit for body weight, postprandial hyperglycemia, hyperinsulinemia, and other cardiometabolic risk factors in type 2 diabetes and, with more limited data, in type 1 diabetes. High-quality, long-term trials are needed to assess safety concerns and determine whether this old dietary approach might help people with diabetes attain clinical targets more effectively, and at a lower cost, than conventional treatment.

The International Society for Pediatric and Adolescent Diabetes Clinical Practice Consensus Guideline 2018 for management of diabetic ketoacidosis (DKA) and the hyperglycemic hyperosmolar state provide comprehensive guidance for management of DKA in young people. Intravenous (IV) infusion of insulin remains the treatment of choice for treating DKA; however, the policy of many hospitals around the world requires admission to an intensive care unit (ICU) for IV insulin infusion. During the coronavirus 2019 (COVID-19) pandemic or other settings where intensive care resources are limited, ICU services may need to be prioritized or may not be appropriate due to risk of transmission of infection to young people with type 1 or type 2 diabetes. The aim of this guideline, which should be used in conjunction with the ISPAD 2018 guidelines, is to ensure that young individuals with DKA receive management according to best evidence in the context of limited ICU resources. Specifically, this guideline summarizes evidence for the role of subcutaneous insulin in treatment of uncomplicated mild to moderate DKA in young people and may be implemented if administration of IV insulin is not an option., (© 2020 John Wiley & Sons A/S . Published by John Wiley & Sons Ltd.)

Objective: We explored the impact of TeKnO T1D, an online, case-based, spaced education curriculum about insulin pump and continuous glucose monitor (CGM) use in pediatric type 1 diabetes management., Methods: Pediatric endocrinology fellows (n = 64) were randomized to receive an educational curriculum focused on either insulin pumps or CGMs. Fellows received interactive questions twice weekly via email or mobile app. Median time to completion was 76.5 days. The primary outcome was change in knowledge as measured by performance on multiple-choice questions (MCQ) from the pre-test to the post-test., Results: Forty-eight of 64 (75%) learners completed the curriculum and assessments. The pump group improved from 35.0 ± 15% on the pre-test MCQs to 61.1 ± 17% on the post-test, a 12.2 absolute percentage point greater improvement on pump-specific items than the CGM group (P = .03). The CGM group improved from 30.3 ± 15% on the pre-test MCQs to 61.4 ± 21% on the post-test, a 28.7 absolute percentage point greater improvement on CGM-specific items than the pump group (P < .001). Both groups were more likely to report an appropriate level of understanding of their respective technologies after completing the corresponding curriculum. In thematic analysis of qualitative data, fellows indicated that knowledge gains led to improved patient care. There was universal agreement about enjoyment and effectiveness of the curricula., Conclusions: TeKnO T1D proved to be an engaging, effective way to improve endocrinology fellows' knowledge and confidence about insulin pumps and CGM use in the management of pediatric type 1 diabetes., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Approximately 1 of every 300 children in the United States has type 1 diabetes mellitus (T1D), and these patients may require anesthetics for a variety of procedures. Perioperative coordination is complex, and attention to perioperative fasting, appropriate insulin administration, and management of hypo- and hyperglycemia, as well as other metabolic abnormalities, is required. Management decisions may be impacted by the patient's baseline glycemic control and home insulin regimen, the type of procedure being performed, and expected postoperative recovery. If possible, preoperative planning with input from the patient's endocrinologist is considered best practice. A multi-institutional working group was formed by the Society for Pediatric Anesthesia Quality and Safety Committee to review current guidelines in the endocrinology and anesthesia literature and provide recommendations to anesthesiologists caring for pediatric patients with T1D in the perioperative setting. Recommendations for preoperative evaluation, glucose monitoring, insulin administration, fluid management, and postoperative management are discussed, with particular attention to increasingly prevalent insulin pumps and continuous glucose monitoring (CGM).

Regular self-monitoring of blood glucose levels, and ketones when indicated, is an essential component of type 1 diabetes (T1D) management. Although fingerstick blood glucose monitoring has been the standard of care for decades, ongoing rapid technological developments have resulted in increasingly widespread use of continuous glucose monitoring (CGM). This article reviews recommendations for self-monitoring of glucose and ketones in pediatric T1D with particular emphasis on CGM and factors that impact the accuracy and real-world use of this technology., (Copyright © 2020 Marks and Wolfsdorf.)

Background: Wolfram syndrome is a rare neurodegenerative disorder, characterized by the presence of diabetes insipidus, diabetes mellitus, optic atrophy, and sensorineural deafness. The majority of cases are due to autosomal recessive biallelic variants in the WFS1 gene; however, pathogenic autosomal dominant (AD) mutations have also been described. Glucagon-like peptide (GLP-1) agonists have been studied in both animal models and humans with classic Wolfram syndrome., Case: We present a 15-year-old female with a personal and family history of congenital strabismus, bilateral cataracts, low-frequency sensorineural hearing loss, and diabetes mellitus. Trio whole exome sequencing revealed a previously unknown maternally inherited heterozygous variant in exon 8 of the WFS1 gene c.2605&#95;2616del12 p.Ser869&#95;His872del, leading to the diagnosis of AD WFS1-related disorder. Treatment with a GLP-1 agonist resulted in marked improvement in glycemic control and discontinuation of insulin therapy. This patient's response to a GLP-1 agonist provides suggestive indirect evidence for a role of WFS1 on β-cell endoplasmic reticulum stress and suggests that treatment with a GLP-1 agonist should be considered in patients with dominant forms of WS., (© 2020 S. Karger AG, Basel.)

More than 80% of pediatric solid organ transplant (SOT) recipients now survive into young adulthood and many encounter transplant-related complications. Post-transplantation diabetes mellitus (PTDM), sometimes also referred to as post-transplant diabetes or new onset diabetes after transplant, occurs in 3-20% of pediatric SOT recipients depending upon the organ transplanted, age at transplantation, immunosuppressive regimen, family history, and time elapsed since transplant. To diagnose PTDM, hyperglycemia must persist beyond the initial hospitalization for transplantation when a patient is on stable doses of immunosuppressive medications. Though standard diagnostic criteria used by the American Diabetes Association (ADA) to diagnose diabetes are employed, clinicians need to be aware of the limitations of using these criteria in this unique patient population. Management of PTDM parallels strategies used for type 2 diabetes (T2D), while also carefully considering comorbidities and potential interactions with immunosuppressive medications in these patients. In caring for patients with PTDM, it is important to be familiar with these interactions and comorbidities in order to coordinate care with the transplant team and optimize outcomes for these patients., (© 2021 S. Karger AG, Basel.)

Impaired growth is common in patients with glycogen storage disease (GSD), who also may have "cherubic" facies similar to the "moon" facies of Cushing syndrome (CS). An infant presented with moon facies, growth failure, and obesity. Laboratory evaluation of the hypothalamic-pituitary-adrenal (HPA) axis was consistent with CS. He was subsequently found to have liver disease, hypoglycemia, and a pathogenic variant in PHKA2 , leading to the diagnosis of GSD type IXa. The cushingoid appearance, poor linear growth and hypercortisolemia improved after treatment to prevent recurrent hypoglycemia. We suspect this child's HPA axis activation was "appropriate" and caused by chronic hypoglycemic stress, leading to increased glucocorticoid secretion that may have contributed to his poor growth and excessive weight gain. This is in contrast to typical CS, which is due to excessive adrenocorticotropic hormone (ACTH) or cortisol secretion from neoplastic pituitary or adrenal glands, ectopic secretion of ACTH or corticotropin-releasing hormone (CRH), or exogenous administration of corticosteroid or ACTH. Pseudo-CS is a third cause of excessive glucocorticoid secretion, has no HPA axis pathology, is most often associated with underlying psychiatric disorders or obesity in children and, by itself, is thought to be benign. We speculate that some diseases, including chronic hypoglycemic disorders such as the GSDs, may have biochemical features and pathologic consequences of CS. We propose that excessive glucocorticoid secretion due to chronic stress be termed "stress-induced Cushing (SIC) syndrome" to distinguish it from the other causes of CS and pseudo-CS, and that evaluation of children with chronic hypoglycemia and poor statural growth include evaluation for CS., Competing Interests: No authors disclose any competing interests.

This article covers several aspects of the clinical management of neonatal hypoglycemia that have recently evolved, reviewing the evidence informing these recommended changes in practice. Topics covered include use of buccal dextrose gel, rationale for avoiding the traditional "mini dextrose bolus," and benefits of direct breastfeeding for the treatment of asymptomatic hypoglycemia in at-risk newborns. The reasons for increasing use of more accurate point-of-care devices for measuring neonatal glucose concentrations are discussed, as well as the implications of different published opinions regarding the determination of readiness for discharge and the most important considerations when making this determination., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Recent data demonstrating a lack of improvement in average hemoglobin A1c levels despite the increased use of insulin pumps and continuous glucose monitors (CGMs) suggest that patients are not using these technologies optimally. Suboptimal provider understanding of these devices may be a contributing factor., Methods: We sought to assess fellows' knowledge, attitudes, and practices regarding insulin pumps and CGMs using a mixed-methods survey. We polled 42 pediatric endocrinology fellows and 69 attending physicians in pediatric endocrinology using items on a five-point Likert scale., Results: Perceived fellow knowledge of insulin pumps and CGMs was only 3.6 ± 1.0 and 3.6 ± 0.9, respectively. Despite consensus about the need for pediatric endocrinologists to understand these technologies, only 14.7% of fellows reported the presence of a formal curriculum about these technologies at their institutions. Potential gaps identified in fellows' knowledge include general use and troubleshooting, advanced insulin pump features, infusion sets and dermatological complications, systematic approach to data, interpretation and application of CGM data, and keeping pace with technological advances., Conclusions: Our data suggest suboptimal fellow knowledge and understanding of insulin pumps and CGMs in pediatric type 1 diabetes management, which is likely attributable to inadequate education in fellowship training programs. The development of formal educational curricula targeting areas of weakness identified in this survey may improve clinician knowledge of these technologies and ultimately impact patient education and care.

Aims: Data are sparse concerning use of serum electrolyte parameters as compared to venous blood gas (VBG) measurements to monitor acid-base status during treatment of diabetic ketoacidosis (DKA). We explored the utility of various parameters to define DKA resolution by investigating the relationship of venous pH (vpH), anion gap (AG), serum bicarbonate (HCO 3 ), and glucose concentration during management of DKA in children with new onset diabetes mellitus (NODM)., Methods: We included all patients with NODM presenting with DKA to Boston Children's Hospital from 10/1/07-7/1/13. DKA was defined as serum glucose ≥ 200 mg/dL (11.1 mmol/L) and vpH<7.30; severity as mild <7.30, moderate<7.20, severe<7.10; resolution of DKA as vpH≥7.30 and AG≤18 mmol/L. We used Cox regression to determine time to DKA resolution, and logistic regression to evaluate different serum HCO 3 cut-off values as predictors of DKA resolution., Results: 263 patients (133F, mean age 9.9±4.4 years, 74% White) were included. DKA was mild in 134 (51%), moderate in 75 (28%) and severe in 54 (20%). In mild DKA, AG closed after normalization of vpH; in moderate and severe DKA, AG closed before normalization of vpH. HCO 3 >15mmol/L correlated with vpH≥7.30, and had 76% sensitivity and 85% specificity to predict DKA resolution. Median times to DKA resolution were similar using two different definitions: vpH and AG (8.4h [IQR 6.3-11.9]) vs. HCO 3 >15 mmol/L (7.9 h [IQR 5.0-11.8]), p=.42., Conclusions: During management of pediatric DKA, HCO 3  > 15 mmol/L reliably predicts resolution of DKA. In low-resource settings where VBG is unavailable, electrolyte parameters alone may be used to determine DKA resolution., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: The aim of the present study was to compare characteristics and diabetes management in children and adolescents with and without at least one parent with type 1 diabetes (T1D)., Methods: In all, 12 890 participants aged <18 years at enrollment in the T1D Exchange Registry were included in the present study. Statistical comparisons between those with and without parental T1D were conducted using a univariate generalized linear mixed model., Results: Of the study participants, 1056 (8.2%) registrants had at least one parent with T1D. Those with parental T1D were slightly, albeit significantly, younger (6.3 vs 6.9 years; P < 0.001) and less likely to have diabetic ketoacidosis (DKA) at diagnosis (24% vs 41%; P < 0.001) than those without parental T1D. There were no differences between groups in HbA1c, use of continuous glucose monitoring or insulin pump therapy, or the development of severe hypoglycemia or DKA. In addition, there were no differences found when comparing characteristics or diabetes management in those with a mother versus those with a father with T1D., Conclusions: Children and adolescents with parental T1D tend to be diagnosed earlier. Diabetes management, glycemic control, and acute complications are similar in those with and without parental T1D., (© 2015 Ruijin Hospital, Shanghai Jiaotong University School of Medicine and John Wiley & Sons Australia, Ltd.)

Objective: To determine whether measuring diabetes-associated autoantibodies (DAA) in pediatric new onset diabetes (NODM) can be restricted to patients with equivocal diabetes type., Research Design and Methods: Retrospective analysis of all patients with NODM admitted to Boston Children's Hospital from 1 October 2007 to 1 July 2013 who had measurement of DAA [glutamic acid decarboxylase, insulin, insulinoma-associated antigen 2 (IA-2)]. Data collection included initial diagnosis of diabetes type before DAA results and at follow-up. We used logistic regression to predict type 1 diabetes (T1D) and developed a clinical score to classify diabetes type., Results: Of 1089 patients (45.4% female, 76.7% White, age 10.6 ± 4.5 yr), initial diagnosis was 1021 (93.8%) T1D, 42 (3.9%) type 2 diabetes (T2D), and 26 (2.4%) other. Of 993 patients with clinical T1D, 78 (7.9%) were DAA-, and of 42 patients with clinical T2D, 12 (28.6%) were DAA+. Type of diabetes was reclassified at follow-up in less than 6% of patients. Data from a subset of 736 patients were used to develop a scoring system to predict T1D. Using weight z-score, age, and race, the scoring system had 91.7% sensitivity, 82% specificity, and a positive predictive value of 98.6%, and suggested DAA measurement was unnecessary in 85.3% of patients. Findings were similar in a validation cohort of 234 patients., Conclusions: Application of a simple scoring system may reduce to ∼15% the number of DAA measurements needed to classify diabetes type, resulting in substantial cost savings. Clinical judgment should guide the decision to measure DAA., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Technological innovations have revolutionized the treatment of type 1 diabetes. Although technological advances can potentially improve diabetes outcomes, maintenance of target glycemic control, at the present time, remains largely dependent on patient and family motivation, competence, and adherence to daily diabetes care requirements. Trials of closed loop or "artificial pancreas" technology show great promise to automate insulin delivery and achieve near normal glucose control and reduced hypoglycemia with minimal patient intervention., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: Glycogen storage disease type I (GSD I) is a rare disease of variable clinical severity that primarily affects the liver and kidney. It is caused by deficient activity of the glucose 6-phosphatase enzyme (GSD Ia) or a deficiency in the microsomal transport proteins for glucose 6-phosphate (GSD Ib), resulting in excessive accumulation of glycogen and fat in the liver, kidney, and intestinal mucosa. Patients with GSD I have a wide spectrum of clinical manifestations, including hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia, hyperuricemia, and growth retardation. Individuals with GSD type Ia typically have symptoms related to hypoglycemia in infancy when the interval between feedings is extended to 3–4 hours. Other manifestations of the disease vary in age of onset, rate of disease progression, and severity. In addition, patients with type Ib have neutropenia, impaired neutrophil function, and inflammatory bowel disease. This guideline for the management of GSD I was developed as an educational resource for health-care providers to facilitate prompt, accurate diagnosis and appropriate management of patients., Methods: A national group of experts in various aspects of GSD I met to review the evidence base from the scientific literature and provided their expert opinions. Consensus was developed in each area of diagnosis, treatment, and management., Results: This management guideline specifically addresses evaluation and diagnosis across multiple organ systems (hepatic, kidney, gastrointestinal/nutrition, hematologic, cardiovascular, reproductive) involved in GSD I. Conditions to consider in the differential diagnosis stemming from presenting features and diagnostic algorithms are discussed. Aspects of diagnostic evaluation and nutritional and medical management, including care coordination, genetic counseling, hepatic and renal transplantation, and prenatal diagnosis, are also addressed., Conclusion: A guideline that facilitates accurate diagnosis and optimal management of patients with GSD I was developed. This guideline helps health-care providers recognize patients with all forms of GSD I, expedite diagnosis, and minimize adverse sequelae from delayed diagnosis and inappropriate management. It also helps to identify gaps in scientific knowledge that exist today and suggests future studies.

The current version of the International Society of Pediatric and Adolescent Diabetes (ISPAD) guidelines for management of diabetic ketoacidosis (DKA) is largely based on the Lawson Wilkins Pediatric Endocrine Society/European Society of Pediatric Endocrinology (LWPES/ESPE) consensus statement on DKA in children and adolescents published in 2004. This article critically reviews and presents the most pertinent new data published in the past decade, which have implications for diagnosis and management. Four elements of the guidelines warrant modification: (i) The definition of DKA; (ii) insulin therapy; (iii) water and salt replacement; and (iv) blood ß-hydroxybutyrate measurements for the management of DKA., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: To examine barriers to health care transition reported by young adults with type 1 diabetes and associations between barriers and prolonged gaps between pediatric and adult diabetes care., Methods: We surveyed young adults aged 22 to 30 years with type 1 diabetes about their transition experiences, including barriers to timely establishment of adult diabetes care. We evaluated relationships between barriers and gaps in care using multivariate logistic regression., Results: The response rate was 53% (258 of 484 eligible subjects). Respondents (62% female) were 26.7 ± 2.4 years old and transitioned to adult diabetes care at 19.5 ± 2.9 years. Reported barriers included lack of specific adult provider referral name (47%) or contact information (27%), competing life priorities (43%), difficulty getting an appointment (41%), feeling upset about leaving pediatrics (24%), and insurance problems (10%). In multivariate analysis, barriers most strongly associated with gaps in care >6 months were lack of adult provider name (odds ratio [OR], 6.1; 95% confidence interval [CI], 3.0-12.7) or contact information (OR, 5.3; 95% CI, 2.0-13.9), competing life priorities (OR, 5.2; 95% CI, 2.7-10.3), and insurance problems (OR, 3.5; 95% CI, 1.2-10.3). Overall, respondents reporting ≥1 moderate/major barrier (48%) had 4.7-fold greater adjusted odds of a gap in care >6 months (95% CI, 2.8-8.7)., Conclusion: Significant barriers to transition, such as a lack of specific adult provider referrals, may be addressed with more robust preparation by pediatric providers and care coordination. Further study is needed to evaluate strategies to improve young adult self-care in the setting of competing life priorities.

Objective: Severe hypoglycemia (SH) and diabetic ketoacidosis (DKA) are common serious acute complications of type 1 diabetes (T1D). The aim of this study was to determine the frequency of SH and DKA and identify factors related to their occurrence in the T1D Exchange pediatric and young adult cohort., Research Design and Methods: The analysis included 13 487 participants in the T1D Exchange clinic registry aged 2 to <26 yr with T1D ≥2 yr. Separate logistic regression models were used to evaluate the association of baseline demographic and clinical factors with the occurrence of SH or DKA in the prior 12 months., Results: Non-White race, no private health insurance, and lower household income were associated with higher frequencies of both SH and DKA (p < 0.001). SH frequency was highest in children <6 yr old (p = 0.005), but across the age range, SH was not associated with hemoglobin A1c (HbA1c) levels after controlling for other factors (p = 0.72). DKA frequency was highest in adolescents (p < 0.001) and associated with higher HbA1c (p < 0.001)., Conclusions: Our data show that poor glycemic control increases the risk of DKA but does not protect against SH in youth and young adults with type 1 diabetes. The high frequencies of SH and DKA observed in disadvantaged minorities with T1D highlight the need for targeted interventions and new treatment paradigms for patients in these high risk groups., (© 2013 John Wiley & Sons A/S.)

A female presented in infancy with hypotonia, undetectable serum glucose, lactic acidosis, and triglycerides >5000 mg/dL. The diagnosis of type 1A glycogen storage disease was made via the result of a liver biopsy, which showed increased glycogen and absent glucose-6-phosphatase enzyme activity. The patient was treated with dextrose administered orally, which was replaced by frequent feedings of cornstarch, which resulted in an improvement of her metabolic parameters. At age 18 years of age, she had marked hypertriglyceridemia (3860 mg/dL) and eruptive xanthomas and was treated with fenofibrate, atorvastatin, and fish oil. At age 29 years she was noted to have multiple liver adenomas, severe anemia, and hyperuricemia. Aggressive cornstarch therapy was commenced with a goal of maintaining her blood glucose levels >75 mg/dL and lactate levels <2 mmol/L. After 15 months on this regimen, her lipids levels (measured in mg/dL) off all medications were as follows: total cholesterol 222, triglycerides 179, high-density lipoprotein cholesterol 32, and calculated low-density lipoprotein cholesterol 154. Her weight was stable with a body mass index of 24.8 kg/m(2). Her liver adenomas had decreased in size, and her anemia and hyperuricemia had improved. She was homozygous for the R83C missense mutation in G6PC. Our data indicate that optimized metabolic control to maintain blood glucose levels >75 mg/dL is critical in the management of this disease., (Copyright © 2012. Published by Elsevier Inc.)

The current standard of care for patients with type 1 diabetes (T1D) employs a system of intensive diabetes management aimed at near-normal glycemia, which reduces the risk of micro- and macrovascular complications. Optimal management is an ongoing process based on a patient-centered collaboration with a primary care clinician and a multidisciplinary diabetes team that provides diabetes management, including education and psychosocial support. Intensive diabetes therapy attempts to mimic physiologic insulin replacement. Over the past 15 years, there has been widespread use of multiple-dose insulin regimens using a variety of insulin analogs, administered either by injection or insulin pump therapy, together with medical nutrition therapy, frequent self-monitoring of blood glucose and, more recently, continuous logo glucose monitoring. It is now possible to achieve previously unattainable levels of glycemic control with less risk of severe hypoglycemia, and yet only a minority of patients achieves target hemoglobin A1c values. This review discusses contemporary management of T1D with a focus on health outcomes., (Copyright 2010 Elsevier Inc. All rights reserved.)

This Practice Point commentary discusses the findings of a multicenter clinical trial conducted by the Juvenile Diabetes Research Foundation Continuous Glucose Monitoring Study Group. In the study, 322 individuals aged >/=8 years who were already receiving intensive insulin therapy for type 1 diabetes mellitus were randomly assigned to either a group that performed continuous glucose monitoring (CGM), or to a control group that performed conventional self-monitoring with a blood-glucose meter. At week 26, the between-group difference in the change in HbA(1c) level from baseline varied according to the patient's age. A significantly greater reduction in HbA(1c) level in the CGM group compared with the control group was found in patients aged >or=25 years, but not in those aged 8-14 years or 15-24 years. In patients aged >or=25 years, CGM improved glycemic control without increasing the frequency of hypoglycemia. Motivation, willingness to change diabetes self-care behaviors, and the ability to use CGM effectively are important determinants of whether this technology will improve glycemic control.