J Korean Diabetes.  2017 Jun;18(2):125-133. 10.4093/jkd.2017.18.2.125.

A Case of Multiple Endocrine Neoplasia Type 1 in Type 2 Diabetes Mellitus with Poor Glycemic Control

• Lee YH
• Lee YC
• Lee JE
• Lee SJ
• Jeong SJ
• Kim CH

• KMID: 2385575
• DOI: http://doi.org/10.4093/jkd.2017.18.2.125

Abstract

The primary causes of uncontrolled diabetes are poor life-style, infection, ischemic heart disease and inappropriate usage of oral anti-diabetic agents and insulin. Supplementary causes are stroke, acute pancreatitis and endocrine diseases. Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant syndrome characterized by primary hyperparathyroidism, pituitary neoplasia, and foregut lineage neuroendocrine tumors, and is associated with increased glucose levels. We present a case of a 69-year-old woman who had polyuria, polydipsia, weight loss and hyperglycemia over 6 months. She had hypertrophy of the face, hand, and foot, and active bleeding and large folds were observed in the stomach and duodenum upon esophagogastroduodenoscopy. She also had high levels of IGF-1 and gastrin and got the failure of growth hormone suppression after an oral glucose load (75 g). These findings suggested a diagnosis of acromegaly and gastrinoma, which was clinically diagnosed along with MEN 1. The patient improved glycemic control and symptoms after being treated with somatostatin analogues and insulin therapy over a 5-month follow-up period. Here, we report a case of MEN 1 in type 2 diabetes mellitus with a poorly controlled blood glucose level. Clinicians should consider endocrine disease in patients with poor glycemic control in diabetes.

Keyword

Acromegaly; Gastrinoma; Multiple endocrine neoplasia type 1; Type 2 diabetes mellitus

MeSH Terms

Acromegaly
Aged
Blood Glucose
Diabetes Mellitus*
Diabetes Mellitus, Type 2
Diagnosis
Duodenum
Endocrine System Diseases
Endoscopy, Digestive System
Female
Follow-Up Studies
Foot
Gastrinoma
Gastrins
Glucose
Growth Hormone
Hand
Hemorrhage
Humans
Hyperglycemia
Hyperparathyroidism, Primary
Hypertrophy
Insulin
Insulin-Like Growth Factor I
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Myocardial Ischemia
Neuroendocrine Tumors
Pancreatitis
Polydipsia
Polyuria
Somatostatin
Stomach
Stroke
Weight Loss
Blood Glucose
Gastrins
Glucose
Growth Hormone
Insulin
Insulin-Like Growth Factor I
Somatostatin

Figure

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  Fig. 1 A family pedigree of the patient (arrowhead). She refuses genetic test for MEN1 gene mutation.

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  Fig. 2 Sella magnetic resonance imaging (MRI). T2 weighted coronal view of sella MRI shows poorly enhancing mass in right sella, 1.6 × 1.1 × 1 cm.

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  Fig. 3 Endoscopic findings show diffuse mucosal hypertrophy and enlarged gastric folds in the body of the stomach (A, B). There was a peptic ulcer with active bleeding in the 1st portion of the duodenum (C, D).

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  Fig. 4 Insulin-like growth factor-1 (IGF-1) and gastrin level during the treatment with long acting octretide (Sandostatin LAR®; Sandoz Pharma Ltd., Basel, Switzerland) 20 mg for 0, 4th week, 30 mg for 8th, 12th week, 20 mg 16th, 20th week.

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  Fig. 5 Before treatment, an enlarged nose, protruding lips and thicker fingers were observed (A, C). After treatment, the patient's appearance is better (B, D).

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  Fig. 6 At 8 week, Patient wanted to switch insulin treatment to oral anti-diabetes drugs. During insulin treatment with long acting octretide, total insulin dose per day and HbA1c were decreased. IU, international unit.

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