4-year-old boy with a chief complaint of multiple caries. The patient had a history of hemolytic anemia was diagnosed with G6PD deficiency at the age of 2 year and 9 months.
Glucose-6-phosphate dehydrogenase (G6PD) help preserve glutathione (GSH), an antioxidant. Deficiency of G6PD may result in destruction of cell walls. Especially Red blood cells (RBCs) do not contain mitochondria, strongly dependent on G6PD activity in RBCs.
Affect 400 million people. Sedative treatment was planned. Avoiding the use of midazolam as a sedative agent and lidocaine as a local anesthetic. The patient was orally administered 1.5 g chloral hydrate (Pocral®, Hanlim, Korea) and 20 mg hydroxyzine (Dudrizine®, Sama, Korea), and the N2O concentration was maintained at 40% during treatment. total 3.6 mL of bupivacaine. To monitor hemolytic attacks, patient visit a pediatrician 3 days after dental treatment.
Periodic follow-up every 3 months. Ectopic eruption of the mandibular right first molar was observed, and a piston elastic appliance was used for molar uprighting. Orthodontic treatment for crossbite of the right posterior teeth is planned to initiate around the time the patient turned 7 years old.
G6PD deficiency is X-linked, more in males. Diagnosis by decrease in G6PD activity in RBC. No overt signs. Newborn severe jaundice, particularly Mediterranean or African ancestry. Severity determined by magnitude of enzyme deficiency.
Most G6PD-deficient are asymptomatic. Manifests as acute hemolysis triggered by drugs, infection, or the ingestion of fava beans.
Infection is probably the most common factor that induces hemolytic anemia. Clinical signs and symptoms of hemolysis typically occur within 24 - 72 hours after exposure to a triggering agent.
Dentists should inform childs parents to be aware of signs and symptoms of a hemolytic crisis include a sudden increase in temperature, nausea, vomiting, cyanosis, headache, fatigue, dyspnea, and dark urine. 1 week follow-up.
In most cases of acute hemolysis, no specific treatment is necessary. Treatment of hemolysis is eliminating the triggering factor, controlling clinical symptoms. Hemoglobin levels recover after 8 - 10 days. Prevent hemolysis by avoiding oxidative stressors.