Low morbidity and mortality in children with diabetic ketoacidosis treated with isotonic fluids.

OBJECTIVE: To assess current rates of complications of diabetic ketoacidosis (DKA), particularly cerebral edema, in a large tertiary-care pediatric hospital with a consistent management protocol. STUDY DESIGN: We report our single-center retrospective experience with 3712 admissions with DKA in 1999-2011. Our DKA protocol features a "3-bag" system using 2 bags of rehydration fluids, identical except for the presence in 1 bag of 10% dextrose, to allow rapid adjustment of glucose infusion rate. The third bag contains insulin. Fluids are administered at a total rate of 2-2.5 times "maintenance" fluid requirements. Total electrolyte concentration is kept approximately isotonic. Billing and medical records databases at Children's Medical Center Dallas were examined for cases of DKA, cerebral edema, other morbidities, and death. RESULTS: We ascertained 20 cases of cerebral edema (0.5%). Most presented early (median duration of treatment 2 hours). Only 10 of 20 computed tomography scans were graded as moderate edema or worse. Only 10 patients received treatment other than routine DKA management. There was 1 death in a patient with sickle cell trait who developed intravascular sickling. Two patients had neurologic sequelae at hospital discharge but both recovered fully. CONCLUSIONS: Compared with data in recent consensus statements, the Dallas protocol is associated with extremely low rates of death and disability (0.08% vs 0.3%) from DKA.

Hypothyroidism in siblings due to a homozygous mutation of the TSH-beta subunit gene.

We report two American sisters of Scottish-Irish ancestry with isolated thyrotropin (TSH) deficiency. The diagnosis of central congenital hypothyroidism was based on low levels of TSH and free thyroxine. Sequencing of the TSH-beta subunit gene revealed a homozygous single nucleotide deletion in codon 105, producing a frame shift and resulting in inactive TSH. This mutation has previously been reported in a Brazilian family, two German families, and a Belgian family. Our case, along with a review of the other reports, supports the theory that this mutation may be a common cause of isolated TSH deficiency.