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In a suspected case of CPT II deficiency the following are considered: (1) dextrose infusion to prevent catabolism till special milk feeds can be arranged, (2) a high carbohydrate and low-fat diet to provide substrate for glycolysis, (3) avoidance of known triggers, e.g., fasting, illness etc., (4) urgent IEM screen/genetic testing/echocardiography/and ultrasound scan of the brain and kidneys, (5) MCT supplementation, (6) intensive care monitoring, and (7) a multidisciplinary team including metabolic specialists and palliative care doctors
This process gradually lightens the skin tone
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Otherwise we are building on a weak foundation
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