Endocrinology

Multiple Endocrine Neoplasia Type 1

Wednesday, 16 March 2011 01:28
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Diagnosis Of Multiple Endocrine Neoplasia Type 1 In A Patient With Back Pain; Case Report And Review Of Literature

 

Abstract

Multiple endocrine neoplasia syndrome 1 (MEN1) is a paracrine genetic autosomal dominant disease consisting of tumors in parathyroid, pancreas and pituitary glands. We report a case of a 44 year old male with MEN1 presenting insulinomas, parathyroid adenomas and a pituitary prolactin producing microadenoma.A 44 year old male presented to the emergency department with complaints of back pain. Initial imaging of the abdomen to rule out nephrolithiasis showed an 11cm mass at the head of the pancreas. On further questioning, the patient reported to have frequent symptoms suggestive of hypoglycemia for more than 20 years.Laboratory data on admission showed low glucose (46 mg/dl) and high calcium (12 mg/dl). A 72 hour fasting test confirmed the diagnosis of insulinoma. Hypercalcemia work-up revealed an elevated PTH (Calcium 12.6 mg/dl and PTH 200 pg/ml); a parathyroid scan showed multiple adenomas. A pituitary MRI did not reveal any tumors. However, labs were remarkable for decreased total testosterone (144 ng/dl), and elevated prolactin (104 ug/L). CT-guided biopsy of the mass at the head of the pancreas was consistent with a neuroendocrine tumor. The patient subsequently underwent resection of the pancreatic mass and parathyroidectomy. His hypercalcemia resolved after surgery and his hyperprolactinemia improved with dopamine agonist therapy. Unfortunately, his hypoglycemia did not resolve.We report the case of a 44 year old male patient with a new presentation of tumors of the parathyroid, pancreas and pituitary glands. Excellent history taking, further testing, and clinical suspicion lead to the diagnosis of MEN1 syndrome.

Last Updated on Saturday, 30 April 2011 10:53
 

Conn’s Syndrome: A Diagnostic Dilemma

Tuesday, 01 March 2011 02:17
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Conn’s Syndrome: A Diagnostic Dilemma. Case Report

Abstract

Conn’s syndrome or primary hyperaldosteronism is characterized by hypertension, hypokalemia and increased ratio of plasma aldosterone concentration (ng/dl) to plasma renin activity (ng/ml per hour). Primary aldosteronism occurs most commonly due to aldosterone producing adenoma (Conn’s syndrome) or bilateral adrenal hyperplasia. The clinical manisfestation may be varied. We report an atypical neurological presentation of primary aldosteronism due to an aldosterone-producing adenoma.

 

 

Introduction

Primary aldosteronism is characterized by hypertension, hypokalemia, suppressed renin activity and increased aldosterone excretion and was first described by J. W. Conn in 19551. Primary aldosteronism used to be considered as a rare form of hypertension but it is now recognized to be the most common form of secondary hypertension with prevalence estimates of 5-13% of all patients with hypertension 2,3. Primary aldosteronism occurs most commonly due to aldosterone-producing adenoma (Conn’s syndrome) or bilateral idiopathic hyperplasia and less commonly due to primary (unilateral) adrenal hyperplasia, aldosterone-producing adrenocortical carcinoma or familial hyperaldosteronism4.

Last Updated on Saturday, 30 April 2011 10:53