it is a potential uncoupler of oxidative phosphorylation that may exacerbate the hypermetabolic state Treatment of the hyperthyroidism in thyroid storm is accomplished by the use of iodide and an inhibitor of iodine oxidation in the thyroid gland such as methimazole 0.5 to 0.7 mg/kg/day divided into three oral doses, which blocks iodine’s ability to combine with tyrosine to form thyroxine and triiodothyronine (T3 ); of note, neither medication inactivates circulating T4 and T3 Propylthiouracil was a first-line agent, but, due to its association with pediatric liver failure, it is now contraindicated in children Iodide rapidly terminates thyroid hormone release; however, this effect is overcome after to days of iodide therapy Iodide also decreases the vascularity of the thyroidal arterial supply and can be particularly useful as a preoperative agent Lugol iodide (or SSKI) to drops once every hours orally or sodium iodide 125 to 250 mg/day intravenously over 24 hours is the usual mode of iodide therapy While iodide can reduce thyroid hormone secretion within 24 hours, methimazole’s effects are minimally useful in acute management because the reduction in thyroid levels may take several days Adequate hydration is essential for effective treatment of thyroid storm The estimate of fluid replacement should include a consideration of the significant increase in fluid requirements caused by fever and an accelerated metabolic rate Glucocorticoids are useful in the acute presentation because they appear to inhibit thyroid hormone release from the thyroid and decrease the peripheral conversion of T4 to T3 Dexamethasone (0.2 mg/kg) or hydrocortisone (5 mg/kg) can be given parenterally during the acute phase Intercurrent infection may be the precipitating factor, thus it should be searched for and treated appropriately Broad-spectrum antibiotics should be considered while awaiting the results of cultures, as there is a known association between thyrotoxicosis and pneumococcal bacteremia Improvement should be seen within a few hours after the initiation of treatment with propranolol, especially in terms of cardiovascular status Clinical Indications for Discharge or Admission All patients in thyroid storm should be admitted Full recovery and adequate control of the underlying thyroid disease take several days to achieve For the patient presenting with thyroid storm, serious consideration should be given to permanent treatment of the hyperthyroidism, either by surgery or radioiodide ablation NEONATAL THYROTOXICOSIS Goals of Treatment The goals of treatment are to control metabolic rate and reduce cardiac workload CLINICAL PEARLS AND PITFALLS Neonatal hyperthyroidism is a life-threatening condition found in 1% to 5% of infants born to mothers with history of hyperthyroidism Infants of mothers with thyroid disease can have hyperthyroidism even if the mother’s thyroid condition is not active during pregnancy or well controlled Current Evidence Neonatal thyrotoxicosis may not be correctly diagnosed in the newborn nursery and may be discovered only when the child presents in extremis in the ED It is caused by excessive thyroid hormone produced by the neonatal thyroid that has been stimulated by maternal thyroid-stimulating antibodies (due to Graves disease) present in the immunoglobulin G (IgG) fraction that have crossed the placenta TSH, T4 , and T3 not cross the placenta in significant quantities In most cases, the disease is self-limiting, and hyperthyroidism remits within about weeks Occasionally, the disease may run a protracted course and arise in the absence of maternal thyroid-stimulating antibodies Clinical Considerations Clinical Recognition The diagnosis should be considered in neonates with maternal history of hyperthyroidism, especially if they are presenting with tachycardia, failure to thrive, or congestive heart failure Triage Assess for signs/symptoms of congestive heart failure Initial Assessment/H&P The infant usually presents with a history of failure to gain weight despite a ravenous appetite The child may also be irritable and have tachycardia, as well as signs of congestive heart failure The physical examination is usually remarkable for a goiter and exophthalmos, acknowledging that a goiter may be difficult to appreciate in a small infant with a short neck Management/Diagnostic Testing Laboratory investigations should include estimations of serum total and free T4 and T3 , and serum TSH Increased concentration of T4 in the presence of suppressed TSH levels is consistent with the diagnosis If the mother is taking antithyroid medication, thyroid function tests on the infant may be unreliable in the first days of life because of suppression of the fetal thyroid by transplacental passage of maternal antithyroid medication When tested, the bone age may be advanced In most cases, treatment must be initiated on the basis of historical and clinical findings For an infant who has an elevated level of T4 but who has few, if any, symptoms or signs, consultation with a pediatric endocrinologist is strongly recommended Total T4 levels in all infants tend to be higher than those in older children because of increased thyroid-binding globulin (TBG) induced by maternal estrogen that crosses the placenta Also, an elevated total thyroxine may be seen with defects that alter the binding of T4 to TBG or the end-organ sensitivity to T4 Treatment is identical to that outlined for thyroid storm in older children The duration of treatment is uncertain and should be based on serial thyroid function tests, especially TSH It is anticipated that treatment needs to be continued only for to weeks in most cases because the causative agent is a subclass of IgG molecules with a serum half-life of about weeks Clinical Indications for Discharge or Admission Consider admission for symptomatic children CONGENITAL HYPOTHYROIDISM Goal of Treatment The major goal of treatment is to address acute clinical manifestations of hypothyroidism, confirm the diagnosis, and plan replacement therapy CLINICAL PEARLS AND PITFALLS Congenital hypothyroidism should be considered in infants with significant constipation, prolonged jaundice, hypotonia, or hypothermia Current Evidence The incidence of congenital hypothyroidism is in 3,500 live births Emergency providers should be knowledgeable about congenital hypothyroidism so they can appropriately educate parents and initiate therapy Acquired hypothyroidism rarely results in urgent clinical problems that lead to ED visits The causes of congenital hypothyroidism are numerous; most cases (90%) are permanent About 20% of patients have ectopic glands, and another 50% have hypoplastic or aplastic thyroid glands Other causes are less common and include dyshormonogenesis, maternal ingestion of antithyroid medication, hypothalamic–pituitary disorders, and defects in thyroglobulin metabolism The dyshormonogenetic disorders are inherited as autosomalrecessive conditions Congenital thyroid deficiency may result in impaired neurologic development if not treated before month of age However, a 2012 study did not show differences in neurologic outcomes in children of asymptomatic, untreated mothers found to have hypothyroidism during early pregnancy compared to those who were asymptomatic but treated Clinical Considerations Clinical Recognition Clinical symptoms and signs of congenital hypothyroidism may be subtle and nonspecific, especially during the first month of life All U.S states now screen newborns for congenital hypothyroidism, but the sensitivity of the screen is variable, especially depending on when the sample is drawn, so infants may present with clinical symptoms, generally after the first month Triage Patients are generally well-appearing and at minimal risk for decompensating Initial Assessment/H&P Severely affected infants may be relatively large at birth, have a large posterior fontanel, manifest hypothermia and hypoactivity, feed poorly, tend to become constipated, and have prolonged jaundice An enlarged tongue, coarse facies, and a hoarse cry may also be noted but are unusual in the first weeks of life An umbilical hernia may be present If treatment is not started, the physical characteristics become more prominent as the child grows older Management/Diagnostic Testing Thyroid function tests beyond the first days of life are most useful diagnostically The TSH level is elevated in primary hypothyroidism, and the total and free T4 levels are low or normal for age A thyroid ultrasound or scan (123I) may be helpful in identifying the particular type of primary hypothyroidism, but treatment should never be delayed to obtain this study A low total T4 level in the absence of elevated TSH level may result from a deficiency of TBG, a pituitary deficiency of TSH, or prematurity In term infants, treatment with l-thyroxine, 10 to 15 mCg/kg/day should be instituted as soon as the relevant diagnostic tests are performed In premature infants, mCg/kg/day can be administered This dosage can be adjusted to maintain a TSH value that is normal for age; on appropriate replacement, the TSH will normalize within weeks Total T4 and free T4 concentrations should be maintained in the upper half of the normal range for age Both undertreatment and overtreatment must be avoided Clinical Indications for Discharge or Admission Generally, treatment is as an outpatient Careful follow-up on a monthly basis during the first several months, preferably by a physician who is accustomed to dealing with congenital hypothyroidism, is strongly recommended Suggested Readings and Key References Diabetic Ketoacidosis DeCourcey DD, Steil G, Wypij D, et al Increasing use of hypertonic saline over mannitol in the treatment of symptomatic cerebral edema in pediatric diabetic ketoacidosis: an 11-year retrospective analysis of mortality Pediatr Crit Care Med 2013;14:694–700 Glaser N, Barnett P, McCaslin I, et al Risk factors for cerebral edema in children with diabetic ketoacidosis The Pediatric Emergency Medicine Collaborative Research Committee of the American Academy of Pediatrics N Engl J Med 2001;344:264–269 Grimberg A, Cerri RW, Satin-Smith M, et al The “two bag system” for variable intravenous dextrose and fluid administration: benefits in diabetic ketoacidosis management J Pediatrics 1999;134(3):376–378 Kuppermann N, Ghetti S, Schunk JE, et al PECARN DKA FLUID Study Group Clinical trial of fluid infusion rates for pediatric diabetic ketoacidosis N Engl J Med 2018;378(24):2275– 2287 ... cerebral edema in children with diabetic ketoacidosis The Pediatric Emergency Medicine Collaborative Research Committee of the American Academy of Pediatrics N Engl J Med 2001;344:264–269 Grimberg A,... ketoacidosis management J Pediatrics 1999;134(3):376–378 Kuppermann N, Ghetti S, Schunk JE, et al PECARN DKA FLUID Study Group Clinical trial of fluid infusion rates for pediatric diabetic ketoacidosis... Increasing use of hypertonic saline over mannitol in the treatment of symptomatic cerebral edema in pediatric diabetic ketoacidosis: an 11-year retrospective analysis of mortality Pediatr Crit Care