WDHA syndrome

Overview

WDHA syndrome (medical condition): A syndrome characterized by watery diarrhea, hypokalemia and achlorhydria.

Symptoms

Watery diarrhoea Abdominal pain Flushing Weight loss Watery diarrhea Reduced blood potassium level Lack of hydrochloric acid in digestive juices High level of potassium in faeces

Causes

WDHA syndrome occurs in 6% of patients with MEN-1 syndrome. Significant advances have been made in elucidating the molecular pathogenesis of WDHA syndrome and other pancreatic endocrine tumors. Studies provide evidence for the importance of several genes, such as the (1) MEN1 gene; (2) p16/MTS1 tumor suppressor gene; (3) DPC4/Smad 4 gene, a tumor suppressor gene located on chromosome arm 18q24; (4) amplification of the HER2/neu proto-oncogene; (5) deletions in chromosome 1; and (6) a possible tumor suppressor gene on chromosome arm 3p.

Diagnosis

The diagnosis can be pursued by ordering a CBC, urinalysis, 24-hr urine potassium, serial electrolytes, a chemistry panel, plasma renin, 24-hr urine aldosterone, d- xylose absorption test, and plasma cortisol, and obtaining an endocrinology or nephrology consult.