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.
Highlights
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in infancy that leads to unfavourable neurological outcome if not treated adequately
Congenital hyperinsulinism (CHI) is a heterogeneous metabolic disorder that is characterized by the unregulated release of insulin from pancreatic beta cells leading to recurrent episodes of hypoglycaemia [1]
Even though all CHI patients treated by near-total pancreatectomy eventually develop insulin-dependent diabetes mellitus, very little is known about the characteristics of this specific diabetes type, the intensity of diabetes management, the associated risk of hypoglycaemia and long-term glycaemic control
Summary
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in infancy that leads to unfavourable neurological outcome if not treated adequately. Near-total pancreatectomy is associated with high rates of insulin-dependent diabetes mellitus and exocrine pancreatic insufficiency. In those patients with severe medically-unresponsive DCHI near-total pancreatectomy, i.e. resection of approximately 95–98% of pancreatic tissue may be required [17, 18]. High rates of persisting hypoglycaemia (up to 60%), hyperglycaemia (almost 100% at 11 years post-surgery) and exocrine pancreatic insufficiency (almost 50%) have been reported in patients with DCHI following pancreatectomy [19,20,21,22,23]. Even though all CHI patients treated by near-total pancreatectomy eventually develop insulin-dependent diabetes mellitus, very little is known about the characteristics of this specific diabetes type, the intensity of diabetes management, the associated risk of hypoglycaemia and long-term glycaemic control
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