RESUMO
La cetoacidosis diabética constituye la manifestación extrema del déficit de insulina caracterizada por deshidratación, hiperglicemia, acidosis y cetosis. El tratamiento incluye soporte vital básico, hidratación parenteral para reposición de líquidos y electrolitos, insulinoterapia, con el objetivo de revertir la acidosis, la cetosis y obtener glucemia cercanas a lo normal. Exige un monitoreo estricto clínico y laboratorial siguiendo guías de manejo para evitar complicaciones y obtener una evolución favorable. Se presentan las modificaciones en las directrices publicadas en el Primer Consenso de diagnóstico y tratamiento de la Cetoacidosis diabética, en base a las actualizaciones sugeridas por la ISPAD (International Society of Pediatric and Adolescent Diabetes) y el Programa de Diabetes del Ministerio de Salud Pública y Bienestar Social (MSPBS).
Diabetic ketoacidosis is caused by extreme insulin deficiency and is characterized by dehydration, hyperglycemia, acidosis, and ketosis. Treatment includes basic life support, parenteral hydration to replace fluids and electrolytes and insulin therapy to reverse acidosis, ketosis, and normalize blood glucose levels. Management requires following guidelines for strict clinical and laboratory monitoring to avoid complications and obtain a favorable clinical outcome. The guidelines published in our previous Consensus on Diabetic Ketoacidosis diagnosis and treatment are presented, modified according to the latest changes in ISPAD (International Society of Pediatric and Adolescent Diabetes) and the Ministry of Public Health and Social Welfare Diabetes Program guidelines.
RESUMO
OBJECTIVE: To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement. STUDY DESIGN: This retrospective nationwide multicenter study involved 589 patients with pediatric-onset LCH, 148 of whom had endocrine dysfunction. Median follow-up was 11.6 years. RESULTS: Pituitary dysfunction was present in 145 patients, and 141 had diabetes insipidus (DI). The estimated 10-year risks of pituitary involvement were 24.2% +/- 1.8%. GH deficiency occurred in 61 patients. Median age at onset was 2.8 years for LCH, 3.9 years for DI, and 7.7 years for GH deficiency. The risk of cranial involvement; ear, nose, and throat involvement; pneumothorax; and cholangitis was significantly higher in patients with endocrinopathy. The chronology of episodes did not support a causal link between pituitary involvement and involvement of other organs. Systemic treatment of LCH did not prevent pituitary involvement. The most severe complication was a neurodegenerative syndrome, which affected 4.3% and 10.8% of patients, respectively, 5 and 15 years after initial diagnosis, and appeared to be linked to pituitary involvement. CONCLUSION: Patients who develop endocrine LCH disorders are at a high risk of neurodegenerative LCH and require long-term follow-up.