Table 3 Clinical and laboratory characteristics of Shwachman-Diamond syndrome-associated diabetes in the here reported patient

Key features of diabetes mellitus in the reported patient with SDS

 age at diabetes diagnosis < 2 years

 prodromal phase with spontaneous episodes of hypoglycemia before the diagnosis of diabetes mellitus

 impaired beta-cell responsiveness to oral glucose load with rigid pattern of insulin secretion without clear first and second phases

 slowly progressive dysglycemia with high glucose variability and persisting episodes of spontaneous hypoglycemia without antidiabetic treatment

 slowly increasing levels of GAD-autoantibodies

 no response to treatment with sulfonylurea

 maintenance of adequate glycemic control (TIR > 70%), and significant reduction of glucose variability (< 35%) and of hypoglycemic episodes ≥ 12 months using high-fiber tube feed formula diet only