NLST (finals) - G6PD

Glucose-6 Phosphate Dehydrogenase Deficiency

G6PD stands for?

G6PD

A hereditary enzyme disorder that affects red blood cells, making them VULNERABLE to oxidative damages, which can lead to HEMOLYTIC ANEMIA

X-linked recessive disorder

Etiology in which this deficiency is more common in males?

Jaundice

Yellowing of eyes and skin because of excess bilirubin in blood

Kernicterus

Bilirubin moves from bloodstream into brain tissue

Pentose Phosphate Pathway

Where is NADPH produced?

NADPH

- maintains GLUTATHIONE (WHICH PROTECTS RBCs!!! from oxidative stress)

Reduced NADPH

Result from decreased gluthatione

Hemolysis

Destruction of RBCs

Fava Beans

- MOST WELL-KNOWN CAUSE OF HEMOLYSIS

- it is sometimes called favism

Sulfonamides

A certain antibiotic that can trigger Hemolysis

Infections

One of the most common OTHER FACTOR of the trigger of hemolysis

Napthalene

chemical from MOTHBALLS that can trigger Hemolysis

Hemolytic Episode

Clinical Manifestations during a ___:

- sudden onset of FATIGUE, PALLOR, SHORTNESS OF BREATHE, AND RAPID HEART RATE

Dark/tea-colored urine

Usual color of the urine because of hemoglobin breakdown

Neonatal Jaundice

Found in newborns and CAN BE SEVERE

Results from blood tests

Diagnosis:

- Low hemoglobin

- Elevated bilirubin

Prevention and Supportive

G6PD diet is more focused on ____ nutrition

Certain legumes

Possible food that we must avoid depending on the case of the patient?

Iron

Nutrient that is important if Anemia develops

Folate (folic acid)

Nutrient that supports RBC production, and is RECOMMENDED in CHRONIC HEMOLYSIS

Vit C and E

Antioxidants needed for G6PD patients, it HELPS REDUCE OXIDATIVE STRESS

Hydration

This is important during hemolytic episodes and help prevent kidney complications

Management during hemolytic crisis

- REMOVE TRIGGER IMMEDIATELY

- FLUIDS AND OXYGEN 4 SUPPORT

- BLOOD TRANSFUSION IF SEVERE

Early Feeding

____ to promote bilirubin excretion for newborns

Complications

- Acute Hemolytic Anemia

- SEVERE JAUNDICE IN INFANTS

- Kidney damage (rare and only in severe cases)