eprintid: 25796
rev_number: 13
eprint_status: archive
userid: 1589
dir: disk0/00/02/57/96
datestamp: 2019-02-08 10:47:26
lastmod: 2024-04-10 10:51:14
status_changed: 2019-02-08 10:47:26
type: article
metadata_visibility: show
creators_name: Welters, Alena
creators_name: Meissner, Thomas
creators_name: Grulich-Henn, Jürgen
creators_name: Fröhlich-Reiterer, Elke
creators_name: Warncke, Katharina
creators_name: Mohnike, Klaus
creators_name: Blankenstein, Oliver
creators_name: Menzel, Ulrike
creators_name: Datz, Nicolin
creators_name: Bollow, Esther
creators_name: Holl, Reinhard W.
title: Characterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinism
subjects: 610
divisions: 910500
keywords: Congenital hyperinsulinism, Diabetes, Pancreatectomy, DPV initiative
abstract: Background: Congenital 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.   

Results: The 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).

Conclusions: In 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.
date: 2018
publisher: BioMed Central
id_scheme: DOI
ppn_swb: 1656190974
own_urn: urn:nbn:de:bsz:16-heidok-257965
language: eng
bibsort: WELTERSALECHARACTERI2018
full_text_status: public
publication: Orphanet Journal of Rare Diseases
volume: 13
number: 230
place_of_pub: London
pagerange: 1-10
issn: 1750-1172
citation:   Welters, Alena ; Meissner, Thomas ; Grulich-Henn, Jürgen ; Fröhlich-Reiterer, Elke ; Warncke, Katharina ; Mohnike, Klaus ; Blankenstein, Oliver ; Menzel, Ulrike ; Datz, Nicolin ; Bollow, Esther ; Holl, Reinhard W.  (2018) Characterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinism.  Orphanet Journal of Rare Diseases, 13 (230).  pp. 1-10.  ISSN 1750-1172     
document_url: https://archiv.ub.uni-heidelberg.de/volltextserver/25796/1/13023_2018_Article_970.pdf