## Clinical Context This is a classic presentation of acute hemolytic crisis in G6PD deficiency triggered by oxidative stress from fava bean consumption. The clinical findings (bite cells, Heinz bodies, hemoglobinuria) confirm acute intravascular hemolysis. ## Immediate Management Priority **Key Point:** In acute hemolytic crisis, the immediate focus is **stabilization and prevention of complications**, not diagnostic confirmation. **High-Yield:** The management hierarchy in acute hemolysis: 1. **Immediate:** Supportive care (IV fluids, transfusion if needed) 2. **Concurrent:** Monitor for complications (acute kidney injury, hyperkalemia) 3. **Later:** Enzyme assay confirmation (done during stable phase, not acute crisis) ## Why This Approach? ```mermaid flowchart TD A[Acute Hemolytic Crisis]:::urgent --> B{Hemoglobin < 7 g/dL or<br/>Symptomatic?}:::decision B -->|Yes| C[Blood Transfusion +<br/>IV Fluids]:::action B -->|No| D[Aggressive IV Hydration<br/>Monitor Urine Output]:::action C --> E[Prevent AKI & Hyperkalemia]:::action D --> E E --> F[Once Stable:<br/>G6PD Enzyme Assay]:::action F --> G[Counseling & Avoidance<br/>of Triggers]:::outcome ``` **Clinical Pearl:** G6PD enzyme levels are falsely normal during acute hemolysis (reticulocytosis with high enzyme content). Testing must be deferred 4–6 weeks after crisis resolution for accurate diagnosis. **Key Point:** Dark urine indicates myoglobinuria/hemoglobinuria → risk of acute tubular necrosis. Aggressive hydration with urine alkalinization (sodium bicarbonate) prevents precipitation in renal tubules. ## Supportive Care Details - IV fluids: 200–300 mL/kg/day to maintain urine output > 200 mL/m²/day - Transfusion: if Hb < 7 g/dL or symptomatic (dyspnea, altered mental status) - Avoid NSAIDs, sulfonamides, antimalarials during recovery - Monitor: serum creatinine, potassium, bilirubin, reticulocyte count 
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