BACKGROUND: Multiple endocrine neoplasia type 1 (MEN 1), or Wermer's syndrome, is a rare autosomal dominant genetic syndrome characterized by tumors or hyperplasia involving the pituitary, parathyroid, and pancreatic islet cells. Association between MEN 1 and nephrocalcinosis is well known, though data published in medical literature regarding Wermer's syndrome and chronic renal failure relation are still rare. CASE: A 70-year-old Caucasian female patient had a history of primitive hyperparathyroidism, prolactinoma, glucagonoma, adrenal adenoma and pulmonary neuroendocrine neoplasia. She presented at our clinic first when she was 62 years old because of hypertension, nephrolithiasis (calcium oxalate) and diabetes mellitus treated with oral agents. During the eight-year follow-up she developed chronic renal failure (serum creatinine 1.8 mg/dL, glomerular clearance 35 mL/min) and partial thrombosis of abdominal aortic wall. CONCLUSIONS: Although the association between renal failure and MEN 1 is rarely reported, patients affected by Wermer's syndrome have several risk factors of decreasing renal function such as hypertension, nephrolithiasis and diabetes mellitus. Moreover, hyperparathyroidism in MEN 1 is clinically similar to the kidney failure condition; indeed, diffuse hyper-plasia of more than one gland is common. Vitamin D should not be administered to these patients. Nephrologists should be involved in MEN 1 follow-up, with the aim to prevent kidney failure development by correcting risk factors.
MEN type 1 and chronic renal failure: a rarely reported association
FABBIAN, Fabio;
2007
Abstract
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN 1), or Wermer's syndrome, is a rare autosomal dominant genetic syndrome characterized by tumors or hyperplasia involving the pituitary, parathyroid, and pancreatic islet cells. Association between MEN 1 and nephrocalcinosis is well known, though data published in medical literature regarding Wermer's syndrome and chronic renal failure relation are still rare. CASE: A 70-year-old Caucasian female patient had a history of primitive hyperparathyroidism, prolactinoma, glucagonoma, adrenal adenoma and pulmonary neuroendocrine neoplasia. She presented at our clinic first when she was 62 years old because of hypertension, nephrolithiasis (calcium oxalate) and diabetes mellitus treated with oral agents. During the eight-year follow-up she developed chronic renal failure (serum creatinine 1.8 mg/dL, glomerular clearance 35 mL/min) and partial thrombosis of abdominal aortic wall. CONCLUSIONS: Although the association between renal failure and MEN 1 is rarely reported, patients affected by Wermer's syndrome have several risk factors of decreasing renal function such as hypertension, nephrolithiasis and diabetes mellitus. Moreover, hyperparathyroidism in MEN 1 is clinically similar to the kidney failure condition; indeed, diffuse hyper-plasia of more than one gland is common. Vitamin D should not be administered to these patients. Nephrologists should be involved in MEN 1 follow-up, with the aim to prevent kidney failure development by correcting risk factors.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
