From Kansas, USA:
My niece with very low blood sugar since her birth was discovered to have an abnormal pancreas, and surgeons removed 90% of it in a high risk surgery. The biopsy showed a disease called nesidioblastosis; are you familiar with it? The surgeon said he has only seen 3 cases, and that it is highly unusual. Will she be able to function on that little of a pancreas? So far her sugar levels are up without insulin. Thank you for any information you can give me.
Nesidioblastosis, also called Persistent Hyperinsulinemic Hypoglycemia of Infancy or PHHI, is the name given to a group of rare conditions in which excessively large amounts of insulin are secreted by the beta cells in relation to the prevailing blood sugar level. Sometimes the condition occurs later in life and sometimes it is due to an autosomal recessive genetic defect. In the latter cases there is an abnormality of what is called the 'sulfonylurea receptor' which affects the rate at which potassium and calcium ions cross the cell membrane and thus the rate at which insulin is released.
Treatment initially is both urgent and precarious and usually involves drugs such as diazoxide, somatostatin and glucagon. However the best long term results are still obtained by removing 90% or more of the pancreas. In the future though it is possible that another group of drugs called calcium channel blockers may have an important role to play in controlling the high insulin levels.
The long term outlook is surprisingly good. Sometimes insulin supplements are needed and sometimes there is a mild recurrence of the hyperinsulinism and one stage may follow the other. Prompt detection and treatment of hypoglycemia is very important especially in the early years.
If you have access to a medical library and can get help with the technicalities there is a good review of the condition in the Archives of Disease in Childhood, Volume 74, page 373, 1996.
Original posting 9 Feb 97
Last Updated: Tuesday April 06, 2010 15:08:52
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