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Related Experiment Videos

Multiple endocrine neoplasia type-1

D Kaura1, K Singh, R Murlidharan

  • 1Department of Internal Medicine, Post Graduate Institute of Medical Education & Research, Chandigarh.

Indian Journal of Gastroenterology : Official Journal of the Indian Society of Gastroenterology
|October 1, 1994
PubMed
Summary
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Multiple Endocrine Neoplasia type 1 (Wermer's syndrome) can cause refractory duodenal ulcers and pancreatic gastrinomas. This case highlights the complex endocrine and metabolic challenges in MEN1 management.

Area of Science:

  • Endocrinology
  • Gastroenterology
  • Oncology

Background:

  • Multiple Endocrine Neoplasia type 1 (MEN1), also known as Wermer's syndrome, is a rare genetic disorder.
  • MEN1 is characterized by tumors in multiple endocrine glands, including the pancreas, parathyroid glands, and pituitary gland.

Observation:

  • A 38-year-old male patient presented with refractory duodenal ulcers, elevated basal acid output, and significantly high fasting serum gastrin levels.
  • Abdominal CT revealed pancreatic gastrinomas, indicative of Zollinger-Ellison syndrome.
  • The patient also exhibited hypercalcemia, hypophosphatemia, ureteric calculi, and enlarged parathyroid glands, consistent with primary hyperparathyroidism.

Findings:

  • The patient's condition was attributed to MEN1, manifesting as pancreatic gastrinomas and parathyroid hyperplasia.

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  • Diagnostic workup confirmed elevated parathormone levels and increased urinary calcium and phosphorus.
  • Pituitary gland evaluation via CT scan showed normal findings.

    • Implications:

    • This case underscores the importance of recognizing the diverse clinical manifestations of MEN1.
    • Comprehensive endocrine and metabolic evaluation is crucial for accurate diagnosis and management of MEN1-associated complications.
    • Multidisciplinary management, including pharmacotherapy and surgical intervention (parathyroidectomy, tumor enucleation), is essential for optimal patient outcomes.