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A woman with bone pain, fractures, and malabsorption
with bone pain, fractures, and malabsorption A
woman
Aldo Bertoli, Nicola Di Daniele, Antonio Troisi, Renato Lauro A 35-year-old white woman was admitted in late 1993 to Columbus Hospital, Rome (RL) after recent bone fractures. At 17 years of age she was seen at another hospital because of fever and diarrhoea after antibiotic treatment; a malabsorption syndrome was diagnosed. In 1982, she was admitted again to the same hospital and malabsorption was confirmed. Laboratory values from her records are shown in the table. Between 1988 and 1990, she complained of pain in her right leg on walking and low-back pain; investigations on several occasions showed serum calcium values within normal limits. In 1990 alkaline phosphatase (ALP) was raised. In 1992 she fractured her left shoulder and in 1993 her right arm. in our hospital (table) showed blood pH 24-hour urine calcium and phosphate were 7-434, within normal limits. Intact serum parathyroid hormone (PTH) was 126 mmol/L. Oral xylose tolerance showed a peak value below normal (9 mg/dL). IgA anti-gliadin antibodies (AGA) were 40 U and IgG AGA were 42 U (Enzyme fluorometric immunoassay, Eurospital SPA, Trieste, Italy). Radiographs showed generalised demineralisation, severe subperiostal bone resorption with typical brown tumours; no calcification of soft tissues and kidneys was seen. Technetium and thallium scintigraphy of the neck showed a parathyroid tumour. She underwent surgical hemithyroidectomy; the lower parathyroid gland was enlarged (1’5 cm diameter) and the histological appearance was of an adenoma. The upper parathyroid was normal. After surgery, she had severe hungry-bone syndrome requiring intravenous calcium. PTH decreased below normal and then returned to the normal range or above, accordingly to plasma calcium, showing restoration of normal PTH-calcium feedback. Intestinal biopsy was consistent with adult coeliac disease. The patient was discharged on oral calcium and vitamin D and on a
Investigations
was
diet. She was last seen in November, 1995, well with a normal serum calcium.
gluten-free and
was
patient had coeliac disease and hyperparathyroidism with severe bone involvement; osteitis fibrosa cystica, bone pains, and multiple fractures suggest long-standing disease. However, calcium was within normal ranges and there was no nephrolithiasis or softtissue calcification. Normal calcium values argue against long-standing primary disease; however, when the measurements were made, there were confounding Our
factors: in 1982, she had diarrhoea and evidence of malabsorption; total calcium was low, but calculated ionised calcium fraction was in the upper normal range and phosphate was low. Between 1988 and 1993 there was evidence of malabsorption, but vitamin D no deficiency was likely, as a consequence both of the malabsorption and of hyperparathyroidism. The severe osteomalacia observed later is consistent with this hypothesis, while it is not usual in mild hyperparathyroidism;’ non-tropical sprue is reported as a cause of high-grade osteopathy associated with vitamin D and calcium malabsorption. 2,3 We suppose that untreated coeliac disease, vitamin D deficiency, and consequent hypocalcaemia and concomitant hyperparathyroidism resulted in severe osteomalacia. The normal calcium values observed were likely to be a consequence of reduction of total body calcium content and vitamin D deficiency. Hypophosphataemia was present in 1982, suggesting that there was hyperparathyroidism at that time. We thank I De Vitis for the measurement of anti-gliadin antibodies, and S Angelosante and A Serra for bone-mineral content evaluation.
References 1
Cattedra di Medicina Interna, Università di Roma "Tor Vergata" (A Bertoli MD, N Di Daniele MD, A Troisi MD, Prof R Lauro MD); Istituto di Medicina Interna, Università Cattolica SC, Rome, Italy
Correspondence
300
to: Dr A Bertoli
2
3
Heat DA. Primary hyperparathyrodism: clinical presentation and factors influencing clinical management. Endocrinol Metab North Am 1989; 18: 631-46. Kruse HP, Ringe JD, Tomforde-Brunckhorst R. Die einheimische sprue, oft verkannte ursache hochgradiger generalisierter osteopathien. Dtsch Med Wochenschr 1987; 112: 1155-59. Paul R. Skeletal scintigraphy in coeliac disease. J Intern Med 1990; 228: 401-03.
woman
Aldo Bertoli, Nicola Di Daniele, Antonio Troisi, Renato Lauro A 35-year-old white woman was admitted in late 1993 to Columbus Hospital, Rome (RL) after recent bone fractures. At 17 years of age she was seen at another hospital because of fever and diarrhoea after antibiotic treatment; a malabsorption syndrome was diagnosed. In 1982, she was admitted again to the same hospital and malabsorption was confirmed. Laboratory values from her records are shown in the table. Between 1988 and 1990, she complained of pain in her right leg on walking and low-back pain; investigations on several occasions showed serum calcium values within normal limits. In 1990 alkaline phosphatase (ALP) was raised. In 1992 she fractured her left shoulder and in 1993 her right arm. in our hospital (table) showed blood pH 24-hour urine calcium and phosphate were 7-434, within normal limits. Intact serum parathyroid hormone (PTH) was 126 mmol/L. Oral xylose tolerance showed a peak value below normal (9 mg/dL). IgA anti-gliadin antibodies (AGA) were 40 U and IgG AGA were 42 U (Enzyme fluorometric immunoassay, Eurospital SPA, Trieste, Italy). Radiographs showed generalised demineralisation, severe subperiostal bone resorption with typical brown tumours; no calcification of soft tissues and kidneys was seen. Technetium and thallium scintigraphy of the neck showed a parathyroid tumour. She underwent surgical hemithyroidectomy; the lower parathyroid gland was enlarged (1’5 cm diameter) and the histological appearance was of an adenoma. The upper parathyroid was normal. After surgery, she had severe hungry-bone syndrome requiring intravenous calcium. PTH decreased below normal and then returned to the normal range or above, accordingly to plasma calcium, showing restoration of normal PTH-calcium feedback. Intestinal biopsy was consistent with adult coeliac disease. The patient was discharged on oral calcium and vitamin D and on a
Investigations
was
diet. She was last seen in November, 1995, well with a normal serum calcium.
gluten-free and
was
patient had coeliac disease and hyperparathyroidism with severe bone involvement; osteitis fibrosa cystica, bone pains, and multiple fractures suggest long-standing disease. However, calcium was within normal ranges and there was no nephrolithiasis or softtissue calcification. Normal calcium values argue against long-standing primary disease; however, when the measurements were made, there were confounding Our
factors: in 1982, she had diarrhoea and evidence of malabsorption; total calcium was low, but calculated ionised calcium fraction was in the upper normal range and phosphate was low. Between 1988 and 1993 there was evidence of malabsorption, but vitamin D no deficiency was likely, as a consequence both of the malabsorption and of hyperparathyroidism. The severe osteomalacia observed later is consistent with this hypothesis, while it is not usual in mild hyperparathyroidism;’ non-tropical sprue is reported as a cause of high-grade osteopathy associated with vitamin D and calcium malabsorption. 2,3 We suppose that untreated coeliac disease, vitamin D deficiency, and consequent hypocalcaemia and concomitant hyperparathyroidism resulted in severe osteomalacia. The normal calcium values observed were likely to be a consequence of reduction of total body calcium content and vitamin D deficiency. Hypophosphataemia was present in 1982, suggesting that there was hyperparathyroidism at that time. We thank I De Vitis for the measurement of anti-gliadin antibodies, and S Angelosante and A Serra for bone-mineral content evaluation.
References 1
Cattedra di Medicina Interna, Università di Roma "Tor Vergata" (A Bertoli MD, N Di Daniele MD, A Troisi MD, Prof R Lauro MD); Istituto di Medicina Interna, Università Cattolica SC, Rome, Italy
Correspondence
300
to: Dr A Bertoli
2
3
Heat DA. Primary hyperparathyrodism: clinical presentation and factors influencing clinical management. Endocrinol Metab North Am 1989; 18: 631-46. Kruse HP, Ringe JD, Tomforde-Brunckhorst R. Die einheimische sprue, oft verkannte ursache hochgradiger generalisierter osteopathien. Dtsch Med Wochenschr 1987; 112: 1155-59. Paul R. Skeletal scintigraphy in coeliac disease. J Intern Med 1990; 228: 401-03.