VLCAD deficiency
Overview
A rare condition that is characterised by cardiomyopathy, fatty liver, skeletal myopathy, pericardial effusions, ventricular arrhythmias , and sudden death
Symptoms
* Shortness of breath * Dyspnoea * Fatigue * Syncope * Tachypnea * Cyanosis * Diaphoresis * Hepatomegaly * Liver pain * Splenomegaly * Ascites * Oedema * Malaise * Fever * Chest pain * Hypotension * Palpitations * Lightheadedness * Weakness * Prominent neck veins
Diagnosis
The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of VLCAD deficiency. This medical information about signs and symptoms for VLCAD deficiency has been gathered from various sources, may not be fully accurate, and may not be the full list of VLCAD deficiency signs or VLCAD deficiency symptoms. Furthermore, signs and symptoms of VLCAD deficiency may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed VLCAD deficiency symptoms.
Treatment
Fasting and long term exercise must be avoided. In particular, fasting and exercise that normally requires catabolism of fat should be avoided. Fasting that should be avoided includes even short, overnight, fasts especially in the young -- children may need to be awakened for a bottle or fed a complex carbohydrate at bedtime so they can make it through the night. Frequent, small, high-carbohydrate and high-protein meals are required. Short-term exercise supportable by muscle and liver glycogen should not cause problems. Diet must be modified, with intake of long chain fatty acids minimized, and medium chain fatty acids supplemented - usually as a medium chain triglyceride oil. This will likely mean a diet high in carbohydrates and isolated protein.