Nesidioblastosis, myelodysplastic syndrome and nodular diabetic glomerulosclerosis in an elderlynondiabetic woman: an autopsy report

Abstract

Nesidioblastosis as the cause of hyperinsulinaemic hypoglycaemia in an adult is rare. We report here an additional case of nesidioblastosis, which resulted in fatal hyperinsulinaemic hypoglycaemia in a 72-year-old woman with an underlying myelodysplastic syndrome. The diagnosis of nesidioblastosis was established only after post-mortem examination with a careful exclusion of minute insulinoma. To our surprise, the renal pathology disclosed typical diabetic nodular glomerulosclerosis in the same patient who had no previous history of diabetes mellitus (DM). Nesidioblastosis has been reported to cause 'reversal' of Type 1 DM and insulinoma causing 'reversal' of Type 2 disease. We therefore hypothesize that our patient might have had an undiagnosed DM in the past, which resulted in the typical diabetic nodular glomerulosclerosis. The nesidioblastosis caused a 'reversal' of DM and even the ultimate development of hyperinsulinaemic hypoglycaemia.

Nesidioblastosis - a Rare Cause of Hypoglycaemia in Adults

“…The initial increase in glycaemia is believed to be implication of relative hyperinsulinism and insulin resistance in the early period of the disease. Management with sulphonylurea derivatives increases the intensity or reveals the incidents of hypoglycaemia in that group of patients (Kon, 2000;Yeh, 1999). Although these reasons for the initial hyperglycaemia are the most likely in view of the previously described cases, while no ªglucohomeostaticº hormones were measured in our patient in this disease phase, we cannot surely exclude the influence of possibly increased glucagon or somatostatin blood levels.…”

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia

“…When the literature was found only as a reference in another original paper, we describe this in the “literature” section of Supplementary Table S1 . The references for Supplementary Table S1 can be found in the reference section [ 3 , 5 , 7 , 11 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , …”

“…Clinical symptoms were, however, not reported. Yeh et al presented the case of an autopsied 72-year-old woman with myelodysplastic syndrome and nodular glomerulosclerosis, who was, however, never diagnosed with diabetes [ 49 ]. She had a history of symptomatic hyperinsulinemic hypoglycemia, and the pancreas showed signs of nesidioblastosis/islet cell hyperplasia.…”

“…However, according to the available literature, the disease is rare. When excluding post-bariatric surgery patients and patients with diagnosed syndromes (such as MEN1), we could identify approximately 535 cases (approximately 560 when counting the “adolescent” cases reported by Castillo-López et al [ 247 ]), although some of these cases, especially early ones and where the full text is not available/is written in another language than English/German, or demographic data are missing, are equivocal [ 3 , 5 , 7 , 11 , 14 , 15 , 16 , 17 , 18 , 19 , 22 , 23 , 25 , 26 , 27 , 29 , 30 , 31 , 33 , 34 , 35 , 39 , 40 , 41 , 43 , 45 , 46 , 47 , 48 , 49 , 50 , 52 , 54 , 55 , 56 , 57 , 58 , 59 , 61 , 62 , 63 , 66 , 67 , 68 , 69 , 70 , 71 ...…”