Mitochondrial Disease Clinical Pathway — Emergency Department and Inpatient

Initial IV Recommendations

General Fluid Recommendations

Initial Fluid Bolus	20 mL/kg NS If concern for Heart Failure, give 10 mL/kg NS Reassess VS, PE Cardiology Consult as clinically indicated
Maintenance Fluids	Prior to ordering MIVF, consider checking POC glucose For patients with normal blood glucose: Order D5NS +/- potassium chloride (depending on renal function) For patients with: Hypoglycemia (Blood glucose < 70), correct with D10 as indicated and recheck POC glucose in 30 minutes Increased catabolic state (Fever > 102, motor seizures, tachycardia, tachypnea) Order D10NS +/- potassium chloride (depending on renal function). NOTE High dextrose can cause increased elevation in lactate. However, this is primarily a problem if significant acidosis is present. Treat accordingly with bicarbonate.
Lactated Ringers	If Lactate < 5, LR is not contraindicated LR competes for renal excretion (may not know initial lactate status prior to bolus) If LR is deemed the most appropriate resuscitation fluid, benefit may outweigh risk for individual patients
Ketogenic Diet Considerations	Utilize saline fluids as appropriate to meet total fluid needs Check blood glucose every 4 hours while NPO Contact inpatient ketogenic diet team as appropriate Send inpatient neurology ketogenic team Epic staff message Ketogenic Diet and NPO Recommendations

Correcting Severe Metabolic Deficit with IV Sodium Bicarbonate

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

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