WDHA Syndrome Clinical Presentation

Updated: Dec 01, 2016
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Presentation

History and Physical Examination

History

Diarrhea is the most common symptom and occurs in at least 89% of patients. Although typically described as painless, the diarrhea may initially be episodic and can be associated with abdominal cramps. Diarrhea eventually becomes voluminous (ie, stool output >3L daily in 80% of patients). It is secretory in nature and persists with fasting. It is often described as having the appearance of weak tea.

Weight loss has been reported in 72% of patients. Abdominal pain is a common symptom, occurring in 50% of patients.

Flushing is observed in 20% of patients and has been attributed to the vasodilatory effects of VIP. In human studies, however, prolonged VIP infusion leads to tachyphylaxis, which may explain why only a minority of patients develop flushing.

Physical

Physical examination may reveal signs of volume depletion, malnutrition, muscle weakness, and chronic history of ill health. The muscle weakness can be associated with elevated creatine phosphokinase (CPK) and as a consequence of hypokalemic rhabdomyolysis. [10] No specific physical findings exist for WDHA syndrome, with even the flushing, which is observed in 20% of patients, occurring in many other conditions. It is believed the flushing is due to the vasodilatory properties of VIP. On occasion, the liver may be palpable and nodular, reflecting hepatic metastatic disease.