Clinical features and causes of endogenous hyperinsulinemic hypoglycemia in Korea.
BACKGROUND
Endogenous hyperinsulinemic hypoglycemia (EHH) is characterized by an inappropriately high plasma insulin level, despite a low plasma glucose level. Most of the EHH cases are caused by insulinoma, whereas nesidioblastosis and insulin autoimmune syndrome (IAS) are relatively rare.


METHODS
To evaluate the relative frequencies of various causes of EHH in Korea, we retrospectively analyzed 84 patients who were diagnosed with EHH from 1998 to 2012 in a university hospital.


RESULTS
Among the 84 EHH patients, 74 patients (88%), five (6%), and five (6%) were diagnosed with insulinoma, nesidioblastosis or IAS, respectively. The most common clinical manifestation of EHH was neuroglycopenic symptoms. Symptom duration before diagnosis was 14.5 months (range, 1 to 120 months) for insulinoma, 1.0 months (range, 6 days to 7 months) for nesidioblastosis, and 2.0 months (range, 1 to 12 months) for IAS. One patient, who was diagnosed with nesidioblastosis in 2006, underwent distal pancreatectomy but was later determined to be positive for insulin autoantibodies. Except for one patient who was diagnosed in 2007, the remaining three patients with nesidioblastosis demonstrated severe hyperinsulinemia (157 to 2,719 µIU/mL), which suggests that these patients might have had IAS, rather than nesidioblastosis.


CONCLUSIONS
The results of this study suggest that the prevalence of IAS may be higher in Korea than previously thought. Therefore, measurement of insulin autoantibody levels is warranted for EHH patients, especially in patients with very high plasma insulin levels.
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Chang-Yun
CY
Woo
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Ji Yun
JY
Jeong
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Jung Eun
JE
Jang
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Jaechan
J
Leem
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Chang Hee
CH
Jung
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Eun Hee
EH
Koh
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Woo Je
WJ
Lee
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Min-Seon
MS
Kim
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Joong-Yeol
JY
Park
Department of Statistics, University of Ulsan College of Medicine, Seoul, Korea.
Jung Bok
JB
Lee
Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea.
Ki-Up
KU
Lee
Autoimmune diseases; Hyperinsulinism; Hypoglycemia; Insulin antibodies; Insulinoma; Nesidioblastosis