Diabetes in School Health (DiSH): Telementoring Collaboration Between Pediatric Diabetes Specialists and School Nurses to Improve Care of Children With Diabetes.

Rates of diabetes in youth are rising and more than 1 million children have diabetes. School nurses are central to a school-aged child's diabetes care and they must make important moment-to-moment decisions requiring understanding of and comfort with diabetes care and technology. The rapid changes in diabetes care and technology make ongoing education essential, yet access to up-to-date and practical education is limited for many school nurses. Integrating needs data and stakeholders' input, this group developed Diabetes in School Health (DiSH) to address this gap. We adapted a well-established, innovative, and easily-accessible telementoring educational model, Project ECHO, to create a collaborative learning community. In the first year, 9 diabetes experts and >150 school nurses joined live DiSH sessions. DiSH has been well-received by the school community and next steps include expansion of DiSH to other states and study of impact of DiSH on health disparities.

Intractable Seizures in Children With Type 1 Diabetes: Implications of the Ketogenic Diet.

INTRODUCTION: The ketogenic diet is prescribed for seizures in some children with epilepsy. Children with type 1 diabetes are at risk for diabetic ketoacidosis caused by ketosis due to decreased insulin effect. Currently there are no clinical guidelines regarding the safety and efficacy of the ketogenic diet in patients with concurrent epilepsy and type 1 diabetes. OBJECTIVES: This review examines the current literature regarding the association between TID and epilepsy, proposed mechanisms for the observed relationship, risks and benefits of the ketogenic diet, and clinical applications of the ketogenic diet in the context of type 1 diabetes and epilepsy. METHODS: PubMed was used to identify relevant articles. Key search terms included, "type 1 diabetes," "ketogenic diet," "seizure," "epilepsy," and "autoimmunity." RESULTS: There is an observed association between type 1 diabetes and epilepsy, with proposed mechanisms including genetic predisposition, anti-glutamic acid decarboxylase (GAD) antibodies, metabolic derangements and cerebrovascular damages. Case reports describe the use of the ketogenic diet for epilepsy management in children with diabetes with mixed results; however, there are no large, randomized controlled trials to evaluate the broader application of these findings. CONCLUSIONS: In summary, there is inadequate evidence to support the use of the ketogenic diet in patients with coexisting epilepsy and type 1 diabetes in clinical practice. Further research is needed to determine the effectiveness, safety, and monitoring parameters of the ketogenic diet for these patients. The risks and benefits of the ketogenic diet as medical nutrition therapy for patients with both type 1 diabetes and epilepsy should be considered on an individualized basis.

A Practical Screening Tool to Predict Early Childhood Obesity Risk: Examining a Birth Cohort.

Children obese at the age of 5 years are at greater risk of lifelong obesity. Because certain risks of obesity can be identified in early infancy, a tool for obesity risk prediction in early life would be clinically useful. We investigated predictors of obesity risk in a novel, prospectively collected healthy birth cohort recruited for demographic risks to develop iron deficiency at 1 year, a cohort leveraged because risk factors for iron deficiency and obesity overlap. Obesity at the age of 5 years was defined as age- and sex-specific body mass index Z-score (zBMI) >2SD. For each child, obesity risk factors were summed. Of 10 total risk factors, the following 4 key risks were identified: maternal obesity, maternal diabetes, large for gestational age, or breastfeeding <6 months. Childhood obesity was predicted by either ≥3 total number of risks (P < .033), any key risk (P < .002), or summing key risks (P < .0001). In clinical practice, summing early life risk factors may be a useful strategy for preemptive counseling.

Treatment Discontinuation within 3 Years of Levothyroxine Initiation among Children Diagnosed with Congenital Hypothyroidism.

OBJECTIVES: To measure the rates of thyroid gland imaging and levothyroxine (L-T4) discontinuation and to assess whether discontinuation was monitored with thyroid-stimulating hormone testing in subjects with congenital hypothyroidism. STUDY DESIGN: This is a retrospective analysis of claims data from the IBM MarketScan Databases for children born between 2010 and 2016 and continuously enrolled in a noncapitated employer-sponsored private health insurance plan or in Medicaid for ≥36 months from the date of the first filled L-T4 prescription. RESULTS: There were 263 privately insured and 241 Medicaid-enrolled children who met the inclusion criteria. More privately insured than Medicaid-enrolled children had imaging between the first filled prescription and 180 days after the last filled prescription (24.3% vs 12.9%; P = .001). By 36 months, 35.7% discontinued L-T4, with no difference by insurance status (P = .48). Among those who discontinued, 29.1% of privately insured children and 47.7% of Medicaid-enrolled children had no claims for thyroid-stimulating hormone testing within the next 180 days (P = .01). CONCLUSIONS: Nearly one-third of children with suspected congenital hypothyroidism discontinued L-T4 by 3 years and fewer Medicaid-enrolled than privately insured children received timely follow-up thyroid-stimulating hormone testing. Future studies are indicated to understand the quality of care and developmental outcomes for children with congenital hypothyroidism and barriers to guideline adherence in evaluating for transient congenital hypothyroidism.

Hyperkalemia and Acute Kidney Injury in an Adolescent: Thinking Outside the Box.

Addison disease is a rare endocrine disorder, which typically presents with nonspecific symptoms including weight loss, fatigue, and nausea in conjunction with hyponatremia and hyperkalemia. This case demonstrates key diagnostic clues in evaluation of an adolescent who presented with severe hyperkalemia and acute kidney injury that was resistant to insulin, glucose, and sodium polystyrene sulfonate and was found to have primary adrenal insufficiency.

Growth attenuation therapy for children with severe physical and cognitive disability: Practice and perspectives of New Zealand paediatricians.

AIM: There are currently no clinical guidelines concerning the administration of growth attenuation therapy (GAT) for children (regardless of gender) with both severe physical and cognitive disability in New Zealand (NZ). This survey aimed to explore the attitudes of paediatricians towards GAT and the frequency of requests and initiation of GAT in NZ. METHODS: An online survey of paediatricians in NZ was undertaken. Questions covered both clinical experience with GAT and attitudes towards it. RESULTS: Overall, the response rate was 55% (173/317) with 162 complete responses; 25% of respondents (41/166) reported enquiries about GAT. Five had personally prescribed GAT; in total, six NZ children have undergone GAT. A total of 77% of respondents either believed GAT is appropriate or were neutral on the subject. The majority of responders (59%) believed ethical approval should be obtained as part of preparation for GAT. CONCLUSIONS: This is the first study to investigate attitudes and practices of NZ paediatricians regarding GAT for severely disabled children. Results indicate a range of views but suggest that family requests for GAT do occur and that the majority of paediatricians are not opposed to GAT in the appropriate ethical and clinical context. The development of practice guidelines for GAT may lead to a more informed decision-making process about GAT for families and paediatricians.

Pituitary Antibodies in an Adolescent with Secondary Adrenal Insufficiency and Turner Syndrome.

BACKGROUND/AIMS: Autoimmune hypophysitis (AH) is a rare inflammatory disease of the pituitary gland causing varying degrees of hypopituitarism and/or sellar compression. Cranial MRI remains the best noninvasive tool to diagnose AH, although a diagnosis of certainty requires pituitary biopsy. The objective of this study was to assess the utility of detecting pituitary antibodies for the diagnosis of AH. METHODS: A 15-year-old female with Turner syndrome (TS), hypothyroidism, and ovarian failure presented acutely with hypocortisolism. Laboratory studies revealed secondary adrenal insufficiency. MRI showed a hypotrophic pituitary gland and loss of the posterior pituitary bright spot. To establish an autoimmune basis for the adrenal insufficiency, serum was analyzed by double indirect immunofluorescence for the presence of pituitary autoantibodies. RESULTS: The patient's serum contained autoantibodies that recognized 36% of the adrenocorticotropic hormone-secreting cells, suggesting that these adenohypophyseal cells were targeted by autoimmunity. The serum contained antibodies that identified the majority of the gonadotropin-secreting cells (FSH 77%, LH 65%). No recognition of GH-, prolactin-, and TSH-secreting cells was found. Preabsorption experiments showed that antigenic targets of autoantibodies were not anterior pituitary hormones themselves. CONCLUSION: Demonstration of circulating pituitary antibodies expands the diagnostic options for AH. In this adolescent with TS, positive and cell-specific pituitary antibodies suggested that AH was the cause of her secondary adrenal insufficiency.

Curative potential of allogeneic hematopoietic stem cell transplant in type 1 diabetes.

The mainstay of treatment for type 1 diabetes (T1D) is exogenous insulin. Here, we report a case in which exogenous insulin requirements were eliminated after an allogeneic hematopoietic stem cell transplant for aplastic anemia in a pediatric patient recently diagnosed with T1D, and explore the validity of this approach compared with current treatments.

Severe and Persistent Thyroid Dysfunction Associated with Tetracycline-Antibiotic Treatment in Youth.

Thyroid dysfunction in adolescents treated with minocycline for acne has been previously described as transient effect and/or associated with autoimmune thyroiditis. We report nonimmune-mediated thyroid dysfunction associated with minocycline/doxycycline in 3 adolescents whose clinical courses suggest an adverse effect that may be more common, serious, and persistent than realized previously.

Development of filter paper hemoglobin A1c assay applicable to newborn screening.

PURPOSE: Gestational diabetes influences risk for future metabolic disease including type 2 diabetes. Hemoglobin A1c (HbA1c) measurement assesses hemoglobin A glycosylation, and could theoretically be used as a test to estimate gestational glucose exposure. HbA1c assay on dried blood spots (DBS) is needed before potential application to statewide newborn screening (NBS) population studies. The study aimed to establish a reliable method to measure HbA1c on NBS DBS specimens. De-identified blood was used to generate trials to evaluate stability of HbA1c in DBS, optimal elution time, and stability of eluted blood. RESULTS: Analysis of DBS stability HbA1c measurements from 3 to 6days after collection overestimated HbA1c values by a bias factor between 0.83 and 0.87. Sixty minutes of elution time produced maximal reproducibility and minimal bias of results. Within assay standard deviation: 0.058; average bias: -0.02%. Stability of eluted blood did not vary significantly between days 0-2 after DBS elution. CONCLUSIONS: Measurement of HbA1c levels on DBS from human blood is feasible. Results suggest new method using DBS to measure HbA1c level with the following characteristics: optimal time for sample analysis 3-6days after collection, elution time of 60min and eluted blood analysis within 2days of elution. Measurement of neonatal HbA1c could provide insight regarding the infant's in utero exposure to glucose.

Eosinophilic Endomyocarditis: A Rare Case of Neonatal Mortality.

Background Eosinophilic endomyocarditis (EEM) is a rare diagnosis that is extremely uncommon in newborns. This case report aimed to present a case of neonatal mortality from acute cardiac failure due to EEM. Case Our report presents a term male neonate with minor complications in the immediate postnatal course, who was discharged at 48 hours of life, but who developed unexpected respiratory distress, followed by cardiac arrest and death at 3 days of life. One day after discharge, the infant developed respiratory distress and cool skin, and then developed cardiac arrest at the pediatrician's office, undergoing resuscitation with intravenous fluid, cardiopulmonary resuscitation, epinephrine, atropine, and failed intubation. Autopsy revealed EEM, an inflammatory infiltrative process involving the endomyocardium. Pathology Pathogenesis involves three stages: (1) myocarditis with an acute eosinophilic inflammatory infiltrate followed by (2) myocyte necrosis and eventually (3) fibrosis in the final stage of the disease. Discussion The cause of death was acute cardiac failure due to intense eosinophilic infiltration and degranulation with early subendocardial myocyte necrosis but before development of extensive myocyte necrosis. This case appears to be the youngest patient reported with EEM.

Quantification of DNA repair capacity in whole blood of patients with head and neck cancer and healthy donors by comet assay.

Comet assay has been used to estimate cancer risk by quantification of DNA damage and repair in response to mutagen challenge. Our goal was to adopt best practices for the alkaline comet assay to measure DNA repair capacity of white blood cells in whole blood of patients with squamous cell carcinoma of the head and neck (HNSCC). The results show that initial damage by 10 Gy of gamma radiation expressed as percent DNA in comet tail was higher in stimulated lymphocytes (61.1+/-11.8) compared to whole blood (43.0+/-12.1) but subsequent repair was similar with comet tail of approximately 20% at 15 min and 13% at 45 min after exposure. Exposure of whole blood embedded in agarose from 5 to 10 Gy gamma radiation was followed by an approximately 70% repair of the DNA damage within 45 min with a faster repair phase in the first 15 min. Variability of the measurement was lower within repeated measurements of the same person compared to measurement of different healthy individuals. The repair during first 15 min was slower (p=0.01) in ex-/non-smokers (41.0+/-2.1%) compared to smokers (50.3+/-2.7%). This phase of repair was also slower (p=0.02) in HNSCC patients (36.8+/-2.1%) compared to controls matched on age and smoking (46.4+/-3.0%). The results of this pilot study suggest that quantification of repair in whole blood following a gamma radiation challenge is feasible. Additional method optimization would be helpful to improve the assay for a large population screening.