Very severe hypertriglyceridemia complicating pediatric acute lymphoblastic leukemia treatment: a call for management guidelines.

OBJECTIVES: Severe and very severe hypertriglyceridemia although rare within the pediatric population occur more often among oncology patients, secondary to chemotherapeutic agents. Currently there exists minimal literature to guide management of severe hypertriglyceridemia among pediatric patients. Very-low-fat dietary restriction should be considered over nil per os (NPO) for initial management of severe hypertriglyceridemia in stable pediatric patients. Pediatricians caring for oncology patients must consider chylomicronemia as a potential etiology for presenting symptoms. Pediatric severe hypertriglyceridemia management guidelines are needed as pediatricians must currently rely on anecdotal experiences for management decisions. CASE PRESENTATION: Three children receiving treatment for acute lymphoblastic leukemia required hospitalization for very severe hypertriglyceridemia. Management varied among the cases but included: NPO or very-low-fat diet, insulin, intravenous fluids, fibrates, and omega-3 fatty acids. CONCLUSIONS: These cases suggest that pediatric severe hypertriglyceridemia management, in the absence of pancreatitis should allow a very-low-fat diet initially rather than NPO followed by pharmacologic therapies.

Thiamine-Responsive Megaloblastic Anemia-Related Diabetes: Long-Term Clinical Outcomes in 23 Pediatric Patients From the DPV and SWEET Registries.

OBJECTIVES: To describe clinical presentation and long-term outcomes in a large cohort of children diagnosed with thiamine-responsive megaloblastic anemia (TRMA)-related diabetes. METHODS: Data from the Diabetes Patienten Verlaufsdokumentation (DPV) and Better control in Pediatric and Adolescent diabeteS: Working to crEate CEnTers of Reference (SWEET) registries were used to identify cases. Complementary information was collected through a chart review of each case. Descriptive analyses with medians and interquartile ranges and numbers (proportions) were tabulated. RESULTS: We identified 23 cases (52% male) in the 2 registries. Eighteen (78%) had genetic confirmation of TRMA. Median age at diabetes onset was 1.4 (quartiles 0.8 to 3.6) years and median age at initiation of thiamine treatment was 5.9 (2.4 to 12.4) years. At their most recent visit, patients' median age was 14.3 (8.1 to 17.5) years, glycated hemoglobin level was 6.9% (6.1% to 7.9%), insulin dose was 0.9 (0.4 to 1.2) units/kg per day and thiamine dose was 200 (100 to 300) mg/day. Three patients were not treated with insulin or antidiabetic drugs. There was no difference in diabetes outcomes in patients with initiation of thiamine ≤1 year after diabetes onset compared to patients with initiation of thiamine >1 year after diabetes onset. CONCLUSIONS: This is the longest case series of pediatric TRMA-related diabetes reported to date. Diabetes onset often occurs several years before initiation of thiamine supplementation. Early initiation of thiamine (within 1 year of diabetes onset) was not linked to improved diabetes outcome. However, the role of thiamine in pancreatic function needs further assessment. Patients with TRMA-related diabetes maintained good glycemic control even after 9 years (median) of follow up.

Complementary and alternative medicine use in children and adolescents with type 1 diabetes.

BACKGROUND: The use of complementary and alternative medicine (CAM) in paediatric patients varies between 11% and 68%. There are limited reports of its use in children with type 1 diabetes mellitus (T1DM). OBJECTIVE: To describe the use of CAM in children with T1DM, and the perceptions of both users and nonusers regarding the effect of CAM on diabetes management. DESIGN/METHODS: A cross-sectional, anonymous questionnaire survey was mailed to a randomly selected subgroup of patients with T1DM. Each patient's main caregiver was asked to complete the questionnaire. RESULTS: Of 403 questionnaires mailed, 195 (48%) were completed. The mean (± SD) age of the children was 12.2±4.0 years (56% boys). Use of CAM was reported in 110 children (56%) (vitamins/minerals [n=99], herbal medicine [n=22], dietary supplement [n=13]). When excluding the use of vitamins/minerals, the CAM number dropped to 47 children (24%). Only the current age of the child was significantly different between users and nonusers of CAM. In users, reasons cited for using CAM were to minimize symptoms, improve control, prevent complications and add benefits to insulin. Only 30% of CAM users stated that CAM improved diabetes control. Nonusers cited satisfaction with current diabetes treatment and lack of knowledge as reasons for not using CAM. CONCLUSIONS: CAM use in children with T1DM was frequent, and appeared to be an attempt to improve control or prevent diabetes complications. However, improved control was not reported as a benefit. Diabetes care teams should assess the use of CAM in children with T1DM, and monitor for any potential positive or negative effects.

Vitamin D deficiency in children and its management: review of current knowledge and recommendations.

Given the recent spate of reports of vitamin D deficiency, there is a need to reexamine our understanding of natural and other sources of vitamin D, as well as mechanisms whereby vitamin D synthesis and intake can be optimized. This state-of-the-art report from the Drug and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society was aimed to perform this task and also reviews recommendations for sun exposure and vitamin D intake and possible caveats associated with these recommendations.