ABCD syndrome, an uncommon cause of hypercalcemia in pediatrics. / Síndrome ABCD, una causa infrecuente de hipercalcemia en pediatría.

ABCD syndrome (ABnormal Calcium, Calcinosis, and Creatinine in Down syndrome) is characterized by an association of hypercalcemia, hypercalciuria, nephrocalcinosis, and impaired kidney function in patients with Down syndrome. Only 7 cases have been published worldwide, although it is believed to be underdiagnosed. This report describes 2 new patients with ABCD syndrome and compares them with the cases reported to date. Although it is a rare cause of pediatric hypercalcemia, it should be considered in children with Down syndrome once other more common etiologies have been ruled out. Once this diagnosis is confirmed, the recommended treatment is to reduce dietary calcium intake and work with an interdisciplinary team to maintain an adequate calorie and protein intake.

El síndrome ABCD (por sus siglas en inglés, ABnormal Calcium, Calcinosis and Creatinine in Down syndrome) se caracteriza por la asociación de hipercalcemia, hipercalciuria, nefrocalcinosis y alteración de la función renal en pacientes con síndrome de Down. Existen solo 7 casos previamente publicados en el mundo, aunque se cree que está subdiagnosticado. En este reporte, presentamos 2 nuevos pacientes con este síndrome y realizamos una comparación con los casos informados hasta el momento. Si bien es una causa rara de hipercalcemia pediátrica, debe considerarse en niños con síndrome de Down una vez descartadas otras etiologías más frecuentes. Al confirmarse este diagnóstico, el tratamiento recomendado es la reducción de calcio en la dieta, trabajando de manera interdisciplinaria para mantener un aporte calórico proteico adecuado.

The role of sex on the prevalence of cardiovascular risk factors in children and adolescents with Type 1 diabetes: The SWEET international database.

OBJECTIVE: To assess the prevalence of cardiovascular risk factors (CVRFs) in children and adolescents with type 1 diabetes (T1D) in the International SWEET registry and the possible role of clinical variables in modifying the risk of having single or multiple CVRFs. STUDY DESIGN: The study is a cross-sectional study. Cut-off points for CVRFs were fixed according to International Society for Pediatric and Adolescent Diabetes (ISPAD) guidelines and WHO parameters: LDL cholesterol (LDL-C) > 100 mg/dL; Systolic Blood Pressure (BP-SDS) > 90th percentile for sex, age, and height; BMI-SDS > 2SD for sex and age. Logistic regression models were applied to evaluate variables associated with at least 1 or 2 CVRFs among registry children and adolescents. RESULTS: 29,649 individuals with T1D (6-18 years, T1D ≥ 2 years) participating in the SWEET prospective multicenter diabetes registry were included. In the cohort, 41 % had one or more CVRFs, and 10 % had two or more CVRFs. Thirty-five percent of enrolled individuals had LDL-C > 100 mg/dL, 26 % had BMI-SDS > 2SD, and 17 % had Systolic BP-SDS > 90th percentile. Females had higher frequency than males of having 1 or 2 CVRFs (45.1 % vs 37.4 %, 11.8 % vs 7.8 %; p < 0.001). Multivariable logistic regression models showed that sex (female), HbA1c category (>7.0 %), and age (>10 years) were associated with a higher chance of having at least 1 or 2 CVRFs (p < 0.001). CONCLUSIONS: In children and adolescents with T1D, female sex, in addition to HbA1c above 7 %, and older age (>10 years) was associated with a higher risk of having at least a CVRF (LDL-C, BMI-SDS, BP) according to internationally defined cut-offs.

Diabetic Ketoacidosis in Type 1 Diabetes Onset in Latin American Children.

OBJECTIVE: To describe the patterns of diabetic ketoacidosis (DKA) occurrence in children newly diagnosed with type 1 diabetes (T1DM) across several Latin American pediatric diabetes centers from 2018 to 2022. METHODS: A retrospective chart review included children under 18 with new-onset T1DM from 30 Latin American pediatric diabetes centers (Argentina, Chile, and Peru) between 30 December 2018 and 30 December 2022. Multiple logistic regression models examined the relationships between age, gender, medical insurance, BMI, and DKA at new-onset T1DM. As far as we know, there are no large studies in Latin American countries exploring the patterns of DKA in new-onset T1DM. RESULTS: A total of 2,026 (983 females) children, median age 9.12 (5.8 -11.7) years with new-onset-T1DM were included. Approximately 50% had no medical insurance. Mean glucose values were 467 mg/dL, pH 7.21, bicarbonate 13 mEq/L, HbA1c 11.3%, and BMI 18. The frequency of DKA was 1,229 (60.7%), out of which only 447 (36%) were severe. There was a significant decrease in the frequency of DKA as age increased: 373 (70.2%) in children under 6, 639 (61.6%) in those between 6 and 12, 217 and (47.5%) in those over 12. Children with medical insurance (58.8%) had a significantly lower frequency of DKA than those without (62.7%). The multiple logistic regression models showed that DKA was significantly and inversely associated with age [OR, 0.72 (95% CI 0.60-0.86)], BMI [OR, 0.95 (95% CI 0.92-0.99)], and medical insurance [OR, 0.75 (95% CI 0.60-0.94)] adjusted for sex. CONCLUSION: Latin American children with new-onset T1DM exhibited a substantial occurrence of DKA. Younger ages and the lack of medical insurance were significantly associated with DKA in new-onset T1DM.

Excessive weight gain in exclusively breast-fed infants.

BACKGROUND: Breastfeeding is recommended as the best source of nutrition in the first months of life and observational studies have associated exclusive breastfeeding with decreased weight gain and a protective effect against obesity in childhood. The objective of this study was to describe the characteristics of a cohort of exclusively breastfed obese infants to determine factors that may lead to this unusual weight gain. METHODS: Infants seen between 2003 and 2015 who were exclusively breastfed and showed excessive weight gain in the first year of life were followed with a focus on features of the mother, the child, feeding patterns and the presence of concomitant factors that influence nutritional status. Additionally, in a subset of the sample, macronutrients of the maternal breast milk were analyzed. A descriptive, prospective cross-sectional study was conducted. RESULTS: Of 73 patients, 63% were girls. At 3 months of life, 64% had a weight-for-height standard deviation score (SDS) >2. At 6 and at 12 months, 100% of the patients had a weight-for-height >2 SDS. The mean age at semisolid-food introduction was 7 months. The mean age at weaning was 15.8 months. The babies were fed on demand and no hunger-satiety pattern was observed. In the breast milk samples analyzed, a significantly lower fat content was found. CONCLUSIONS: The results of our study lead to the assumption that inter-individual variations in mother's milk composition may affect the growth patterns of children.

Reporte de altos requerimientos de insulina en pacientes críticos pediátricos con COVID-19. Experiencia con monitoreo remoto continuo de glucosa / Report of high insulin requirements in pediatric critically ill patients with COVID-19. Experience with continuous remote glucose monitoring

Los pacientes en estado crítico con COVID-19 sufren hiperglucemias sostenidas de difícil manejo. A esto se suma el desafío de minimizar la exposición al contagio. En el presente artículo analizamos la evolución metabólica de dos pacientes pediátricos con COVID-19 admitidos en unidad de cuidados intensivos (UCI) para pacientes COVID-19 del Hospital "Prof. Dr. Juan P. Garrahan" de la Ciudad Autónoma de Buenos Aires, Argentina, que requirieron tratamiento con insulina endovenosa y cuya glucemia fue monitoreada de manera remota con la plataforma InsuMate® desarrollada en la Universidad Nacional de La Plata. Los pacientes requirieron tasas de infusión de insulina en dosis marcadamente mayores que las de otros pacientes críticos que impresionaron relacionadas con los valores de marcadores de inflamación. La infusión pudo ajustarse con cuatro monitoreos diarios de glucosa y las métricas obtenidas con el monitor de glucosa. El uso del sistema de monitoreo remoto continuo de glucosa permitió disminuir la frecuencia de monitoreo glucémico durante el tratamiento.

Critically ill patients with COVID-19 suffer from sustained hyperglycemia that is difficult to manage. Added to this is the challenge of minimizing exposure to contagion. In this article we analyze the metabolic evolution of two pediatric patients with COVID-19 admitted to the intensive care unit (ICU) for COVID-19 patients at the Hospital "Prof. Dr. Juan P. Garrahan "from the Autonomous City of Buenos Aires, Argentina, who required treatment with intravenous insulin and whose blood glucose was remotely monitored with the InsuMate® platform developed at the National University of La Plata. The patients required insulin infusion rates in doses markedly higher than those of other critically ill patients, who were impressively related to the values of inflammation markers. The infusion could be adjusted with four daily glucose monitors and the metrics obtained with the glucose monitor. The use of the continuous remote glucose monitoring system made it possible to decrease the frequency of glycemic monitoring during treatment.

Caracterización clínica de lactantes menores de un año, con excesiva ganancia de peso durante la alimentación con leche materna / Clinical features of infants younger than one year with excessive weight gain while being breast fed

Introducción: La lactancia materna es recomendada como fuente de alimentación optima para los primeros meses de vida. En nuestro Servicio hemos recibido un grupo particular de niños alimentados con lactancia materna exclusiva, que muestran aumento excesivo de peso desde el primer semestre de vida. Objetivo: describir las características clínicas de un grupo de lactantes menores de un año, con aumento excesivo de peso y alimentación con lactancia materna. Población y métodos: estudio observacional, prospectivo y longitudinal. Incluimos todos los niños menores de un año derivados al servicio de Nutrición entre 2003 y 2015, con lactancia materna exclusiva durante los primeros 6 meses, persistencia de la misma al momento de la evaluación y peso- edad mayor a 2 desvíos estándar (DS). Describimos características clínicas y de laboratorio de los lactantes y sus madres, así como el patrón alimentario que pudiera explicar esta inusual ganancia de peso. Resultados: incluimos 73 lactantes, 63% niñas. El 64% tenía peso-talla mayor a 2DS a los 3 meses de vida y el 100% a los 6 meses. No se pudo establecer patrón hambresaciedad. El 44% de las madres aumentó más de 18 kilogramos durante el embarazo. Las niñas nacidas de las madres que habían aumentado más de 18 Kg fueron significativamente más grandes al nacer. En el 14% de los lactantes se encontró alguna enfermedad de base. De toda la muestra, el 75% presentó anemia y el 14.5% hipotiroidismo. Conclusiones: describimos un grupo de lactantes con aumento excesivo de peso desde los primeros 3 meses de vida que se mantiene durante toda la etapa de lactancia materna. Diferentes factores como relación madre-hijo, patrón alimentario, composición de la leche humana, predisposición genética; podrían haber contribuido con este patrón de crecimiento.(AU)

Introduction: Maternal breastfeeding is recommended as the best source of nutrition in the first months of life. At our Department we have seen a group of exclusively breastfed children who showed excessive weight since the first semester of life. Objective: To describe the clinical features of a group of infants under one year of age with excessive weight gain while being breast-fed. Population and methods: A prospective, longitudinal, observational study was conducted. We included all infants under one year of age who were referred to the Department of Nutrition between 2003 and 2015, who were exclusively breastfed during the first 6 months of life and were still being breastfed at the moment of the evaluation, and who had a weight-for-age of more than 2 standard deviation (SD). We describe the clinical and laboratory features of the infants and their mothers, as well as feeding patterns that may explain this unusual weight gain. Results: We included 73 infants, 63% girls. Overall, 64% had a weight-for-height greater than 2 SD at 3 months of life and 100% at 6 months of life. No hunger-satiety pattern could be established. Of the mothers, 44% gained more than 18 kg during the pregnancy. The girls born from mothers who had increased more than 18 Kg were significantly larger at birth. In 14% of the infants, an underlying disease was found. Of all the infants in the sample, 75% had anemia and 14.5% hypothyroidism. Conclusions: We describe a group of infants with excessive weight gain in the first 3 months of life which was maintained throughout the maternal breastfeeding period. Different factors, such as the motherchild relationship, feeding pattern, human milk composition, and genetic predisposition may have contributed to this particular growth pattern. (AU)