Characterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinism

Abstract

BackgroundCongenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in infancy that leads to unfavourable neurological outcome if not treated adequately. In patients with severe diffuse CHI it remains under discussion whether pancreatic surgery should be performed or intensive medical treatment with the acceptance of recurrent episodes of mild hypoglycaemia is justified. Near-total pancreatectomy is associated with high rates of insulin-dependent diabetes mellitus and exocrine pancreatic insufficiency. Little is known about the management and long-term glycaemic control of CHI patients with diabetes after pancreatic surgery. We searched the German/Austrian DPV database and compared the course of 42 CHI patients with diabetes to that of patients with type 1 diabetes mellitus (T1DM). Study groups were compared at diabetes onset and after a follow-up period of 6.1 [3.3–9.7] (median [interquartile range]) years.ResultsThe majority of CHI patients with diabetes were treated with insulin (85.2% [70.9–99.5] at diabetes onset, and 90.5% [81.2–99.7] at follow-up). However, compared to patients with T1DM, significantly more patients in the CHI group with diabetes were treated with conventional insulin therapy (47.8% vs. 24.4%, p = 0.03 at diabetes onset, and 21.1% vs. 6.4% at follow-up, p = 0.003), and only a small number of CHI patients were treated with insulin pumps. Daily insulin dose was significantly lower in CHI patients with diabetes than in patients with T1DM, both at diabetes onset (0.3 [0.2–0.5] vs. 0.6 IE/kg/d [0.4–0.8], p = 0.003) and follow-up (0.8 [0.4–1.0] vs. 0.9 [0.7–1.0] IE/kg/d, p = 0.02), while daily carbohydrate intake was comparable in both groups. Within the first treatment year, HbA1c levels were significantly lower in CHI patients with diabetes (6.2% [5.5–7.9] vs. 7.2% [6.5–8.2], p = 0.003), but increased to a level comparable to that of T1DM patients at follow-up. Interestingly, in CHI patients, the risk of severe hypoglycaemia tends to be higher only at diabetes onset (14.8% vs. 5.8%, p = 0.1).ConclusionsIn surgically treated CHI patients insulin treatment needs to be intensified in order to achieve good glycaemic control. Our data furthermore emphasize the need for improved medical treatment options for patients with diazoxide- and/or octreotide-unresponsive CHI.

Global Registries in Congenital Hyperinsulinism

“…The early results of the insights generated by the only patient-reported data source are consistent with the literature and other studies completed, including the genetics ( 1 , 2 ), the reported side-effects of current medications ( 3 , 20 , 21 ), the timing and development of post-pancreatectomy diabetes ( 22 , 23 ), and the neurological implications associated with prolonged hypoglycemia ( 2 – 4 ).…”

Comparing clinical characteristics of pediatric patients with pancreatic diabetes to patients with type 1 diabetes: A matched case‐control study

“…Other pathogenic subgroups, which play a minor role in pediatric patients, are hereditary or chronic pancreatitis, pancreatectomy for trauma or congenital hyperinsulinism, iron overload because of thalassemia and hemochromatosis . Specific subgroups of pancreatic diabetes, such as congenital hyperinsulinism and hereditary chronic pancreatitis, were investigated in a small number of studies, which have been limited by small sample sizes …”

Case of Insulinoma in a 2-Months-Old Infant

“…The age at onset of hypoglycemia reflects the severity of CHI. Welters et al [8] reported that in surgically treated CHI patient's insulin treatment needs to be intensified to achieve good glycaemic control. Our data furthermore emphasize the need for improved medical treatment options for patients with diazoxide and/or octreotide unresponsive CHI.…”