Presentation A father brings his 6-year-old son to the emergency department complaining that “he isn’t acting right.” He has become increasingly confused and less responsive over the last 3 hours. He complained of abdominal pain and nausea earlier today. Dad denies any trauma. The father is unable to provide you with any information about other recent symptoms or changes as he only sees his son every other weekend. The child’s mother is on her way to the hospital.

Physical Exam Vitals: – T 36.1 ⁰C – BP 90/55 – HR 135 – RR 32 – O2 99% on room air General: Ill-appearing, will moan in response to questions, opens eyes spontaneously, withdraws extremities to pain HEENT: PERRL, oral mucosa is dry, no exudates CV: tachycardic, no murmur Resp: CTAB, no crackles or wheezes, respirations are deep and rapid Abd: soft, non-distended, mildly tender to palpation throughout, no rebound tenderness, normal bowel sounds MS: moves all 4 extremities in response to pain, normal tone Neuro: CN II-XII grossly intact

Differential Diagnosis Mnemonic “TIPS from the vowels” for children presenting with AMS – T: trauma, tumor – I: insulin/hypoglycemia, intussusception – P: poisoning, psychogenic – S: shock – A: alcohol – E: epilepsy – I: infection, inborn error of metabolism – O: opiates – U: uremia

The child’s mother has just arrived and is at the bedside as you are finishing your physical exam. What additional information do you want?

Additional Information Mom reports he has seemed tired lately and has been getting up frequently at night to urinate. She also thinks he has lost some weight. She denies fever, rash, recent illness, or any other complaints. He has never had anything like this happen before.

History Past Medical Hx: None Birth Hx: Term infant, SVD, no forceps or vacuum used, mother had standard prenatal care without any complications, infant was discharged with mother at 48 hours Developmental Hx: Normal per Mom Immunizations: UTD Family Hx: Mom and Dad are healthy, paternal grandfather has HTN and prostate cancer. Social Hx: Parents are divorced. Child lives with Mom and visits Dad every other weekend. No siblings. No pets. No smoke exposure. Allergies: None Meds: None

What labs do you want?

Laboratory Tests BMP: < CBC: Accucheck: 675 Serum ketones: moderate reactivity on Acetest Venous blood gas: pH 7.16, HCO 3 11 UA: positive for ketones and glucose Phos: 4.0 Mg: 1.4 Ca: 9.0

What is your diagnosis?

DKA Definition: Hyperglycemia + metabolic acidosis Hyperketosis Dehydration Common presentation for type 1 diabetes in young children

What is your treatment plan?

Treatment IVF: Start initial infusion of NS at rate of ml/kg over one hour. Then continue at maintenance rate. Insulin: Begin at rate of unit/kg/hour. Start this after the initial fluid bolus to decrease the risk of cerebral edema. Monitor glucose, electrolytes, vitals, and neurologic status. When glucose decreases to change IVF to 5% dextrose in isotonic saline to prevent hypoglycemia as you continue to administer insulin. Be sure to keep an eye on potassium levels and replace as needed. Continue treatment until glucose levels stabilize and the metabolic acidosis is corrected.

Type I Diabetes A person who is genetically susceptible develops autoimmunity against the beta cells of the pancreas Beta cells are progressively destroyed, leading to eventual insulin deficiency

Disease Progression Initiation of autoimmunity Preclinical autoimmunity with progressive loss of beta cell function Onset of clinical disease Transient remission Established disease Development of complications

Epidemiology Type I DM accounts for about 10% of all DM, affecting 1.4 million people in the U.S. No apparent correlation with sex or socioeconomic status Incidence rates much higher in whites (32.9 vs 19.2 in African American and 17.6 in Hispanic) 2 peaks of presentation: 5-7 years and puberty

Genetics Concordance rates: – Monozygotic twins 30-65% – Dizygotic twins 6-10% – Other sibilings <6% 85% of newly diagnosed type 1 diabetics do not have a family member with the disease MHC is the genomic region with the greatest contribution to the risk of developing type 1 DM (HLA DR3/4-DQ2/8 genotype is best studied) and the second is the insulin gene locus, IDDM2

Histology Normal pancreasPancreas from a patient with type 1 diabetes showing insulitis, leukocytic infiltrates in the islets. These are primarily T lymphocytes and may be present at initial presentation of the disease, though rarely of diagnostic value. Reduction in the size and number of islets can also be seen.

Longterm Management Start on subcutaneous insulin Refer patient and family for diabetes education Close follow up with PCP Goal HgbA1C < 8.0 for ages 6-11