Management

Treatment of Manifestations:
•    Nutritional support via nasogastric or gastrostomy tube feeding as needed.
•    Dietary adjustments to manage malabsorption and lymphangiectasia
•    Hydration and albumin infusions for protein-losing enteropathy.
•    Pain management with NSAIDs, opioids, and possibly gabapentin.
•    Physiotherapy to mitigate joint contractures, although pain may limit effectiveness
•    Dermatologic and surgical interventions for obstructive lesions
•    Specialized anesthesia precautions due to potential airway complications

Surveillance:
•    Regular monitoring of serum albumin and immunoglobulin levels
•    Assessment of nutritional status and gastrointestinal function.
•    Evaluation of joint contractures and pain levels.
•    Screening for oral lesions impacting feeding and dental health.
•    Periodic cardiac evaluations.