Bone demineralization, hypocalcaemia, excess parathormone in a 63-year-oldman in poor general condition: late manifestations of pseudohypohyperparathyrodism

History and clinical findings: A 63-year-old man was hospitalized because o f his worsening general condition and weight loss. physical examination rev ealed marked thoracic kyphosis with impaired mobility of his back and restr icted walking because of pain in the right hip. He also had other bone pain s. Investigations: Serum calcium was reduced (1.60 mmol/l) and there was gener alized bone demineralization. Subsequently an increased parathormone (PTH) level was noted (499.0 ng/l) as well as markedly increased activity of enzy mes involved in bone metabolism, decreased renal excretion of phosphates (4 .76 mmol/24 h) and multiple pathological fractures. Treatment and course: The listed abnormalities indicated the diagnosis of p seudohypoparathyroidism (PHP), type 1 b. After treatment had been started w ith vitamin D (calcitriol 2 x 0.25 mu g/d) and calcium (calcium gluconate, 3 x 500 mg/d), the levels of calcium, PTH and enzymes in bone metabolism gr adually became normal. A cataract operation had to be performed because of calcification of the lens. Conclusion: The level of PTH should be determined in patients with extensiv e bone demineralization and hypocalcaemia. If PTH is raised, PHP should be included in the differential diagnosis. Normalization of serum calcium by c alcium substitution and vitamin D administration will normalize PTH and imp rove mineralization of the skeleton. In this way the debilitating effects o f osteodystrophea cystica generalisata (OCG) (Engel von Recklinghausen synd rome) can be prevented. Also, the consequences of extraosseous calcificatio n, such as extrapyramidal symptoms of calcification of the brain-stem gangl ia can be avoided if treated in time.