Polynesian, in addition to Asian people, are two ethnic groups at particular risk of TPP, and this condition must be considered in the differential diagnosis for patients presenting to the emergency department with severe hypokalaemia and weakness.
A 58-year-old man presented to the Auckland Hospital, Auckland, New Zealand, with a history of multiple stress fractures in both ankles and feet and the right hip over a period of 8 years. He described generalized musculoskeletal pains and had weakness of his proximal muscles. Physical examination confirmed severe proximal muscle weakness with no evidence of neurological disease. There was no relevant family history.When first seen by us, it was noted that he had been consistently hypophosphataemic (<0.4 mmol/L, normal range 0.7-1.5) over at least the previous 3 years. Alkaline phosphatase was elevated at 431 U/L (normal range 40-110 U/L). Serum calcium, creatinine and PTH were normal. Serum 1,25-dihydroxyvitamin D was reduced at 8 ng/L (normal range 18-62 ng/L) and the tubular maximum for phosphate resorption (TmP) was markedly reduced at 0.13 mmol/L (normal 0.8-1.35 mmol/L) confirming severe renal phosphate wasting. A bone biopsy showed osteomalacia. Plain radiographs of the chest and limbs, bone scintigraphy and computed tomography scans of the sinuses, chest and abdomen failed to detect any evidence of an underlying tumour. The patient was commenced on phosphate supplements and calcitriol and experienced significant symptomatic and biochemical improvement.In a further attempt to identify an underlying lesion, a whole-body magnetic resonance imaging scan was undertaken. A lesion was seen in the posterior aspect of the spinal canal at the level of the first and second lumbar vertebrae (Figure 1). He proceeded to surgery, where an extra-dural haemangiopericytoma was found and removed. Postoperatively, phosphate and calcitriol therapy were discontinued, and serum phosphate remained normal. Six months later, serum biochemistry remained normal and the patient experienced total remission of his musculoskeletal symptoms.
EM staff education, together with an automated laboratory message, significantly improved the rate and quality of notification of elevated RVPG levels to GPs. These simple measures have the potential to hasten implementation of preventive and therapeutic measures in patients with IGM.
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