Literature DB >> 28508606

Surgery in Focal Congenital Hyperinsulinism (CHI) - The "Hyperinsulinism Germany International" Experience in 30 Children.

Winfried Barthlen1, Emine Varol1, Susann Empting2, Ilse Wieland3, Martin Zenker3, Wolfgang Mohnike4, Silke Vogelgesang5, Klaus Mohnike2.   

Abstract

OBJECTIVES: Results of surgery for focal CHI in 30 children PATIENTS AND METHODS: All showed an ABCC8 or KCNJ11 mutation. After PET/CT in 29 children and PET/MRT in 1 case, frozen-section guided resection was performed, in left-sided cases by laparoscopy. Mean age at surgery was 11.7 months (2-49).
RESULTS: In 28/30 children, the PET/CT or MRT correlated with histopathology. In two cases, a focal lesion was undectable; one of these was cured, one not. In total, 24 children showed lesions with sizes of 5-12 mm. All were cured instantly. In four children with huge lesions in the pancreatic head, pathological cells remained at the resection margins. One child was cured instantly, two children after a 2nd surgery, and one child was not cured, even after three surgeries. The overall cure rate was 93%.
CONCLUSIONS: Imaging, surgical findings, histopathology and clinical outcome in surgery for focal CHI match in most, but not all cases. Copyright© of YS Medical Media ltd.

Entities:  

Keywords:  18F-DOPA PET/CT; Focal congenital hyperinsulinism; Genetics; MRI; Surgery

Mesh：

Substances：

Year:  2016        PMID： 28508606     DOI： 10.17458/PER.2016.BVE.Surgeryinfocal

Source DB:  PubMed          Journal:  Pediatr Endocrinol Rev        ISSN： 1565-4753


  9 in total

1.  Variation in Glycemic Outcomes in Focal Forms of Congenital Hyperinsulinism-The UK Perspective.

Authors:  Antonia Dastamani; Daphne Yau; Clare Gilbert; Kate Morgan; Paolo De Coppi; Ross J Craigie; Jamshed Bomanji; Lorenzo Biassoni; Rakesh Sajjan; Sarah E Flanagan; Jayne A L Houghton; Senthil Senniappan; Mohammed Didi; Mark J Dunne; Indraneel Banerjee; Pratik Shah
Journal:  J Endocr Soc       Date:  2022-03-15

2.  Congenital hyperinsulinism treated by surgical resection of the hyperplastic lesion which had been preoperatively diagnosed by 18F-DOPA PET examination in Japan: a nationwide survey.

Authors:  Yutaka Kanamori; Toshihiko Watanabe; Tohru Yorifuji; Michiya Masue; Hideyuki Sasaki; Masaki Nio
Journal:  Pediatr Surg Int       Date:  2018-08-03       Impact factor: 1.827

3.  Intraoperative Ultrasound: A Tool to Support Tissue-Sparing Curative Pancreatic Resection in Focal Congenital Hyperinsulinism.

Authors:  Julie Bendix; Mette G Laursen; Michael B Mortensen; Maria Melikian; Evgenia Globa; Sönke Detlefsen; Lars Rasmussen; Henrik Petersen; Klaus Brusgaard; Henrik T Christesen
Journal:  Front Endocrinol (Lausanne)       Date:  2018-08-22       Impact factor: 5.555

4.  Differential Morphological Diagnosis of Various Forms of Congenital Hyperinsulinism in Children.

Authors:  Lubov Borisovna Mitrofanova; Anastasia Arkadyevna Perminova; Daria Viktorovna Ryzhkova; Anna Andreyevna Sukhotskaya; Vladimir Gireyevich Bairov; Irina Leorovna Nikitina
Journal:  Front Endocrinol (Lausanne)       Date:  2021-08-23       Impact factor: 5.555

5.  Correlation of PET-MRI, Pathology, LOH, and Surgical Success in a Case of CHI With Atypical Large Pancreatic Focus.

Authors:  Hendrik Vossschulte; Konrad Mohnike; Klaus Mohnike; Katharina Warncke; Ayse Akcay; Martin Zenker; Ilse Wieland; Ina Schanze; Julia Hoefele; Christine Förster; Winfried Barthlen; Kim Stahlberg; Susann Empting
Journal:  J Endocr Soc       Date:  2022-04-06

6.  Clinical Diversity in Focal Congenital Hyperinsulinism in Infancy Correlates With Histological Heterogeneity of Islet Cell Lesions.

Authors:  Ross J Craigie; Maria Salomon-Estebanez; Daphne Yau; Bing Han; Walaa Mal; Melanie Newbould; Edmund Cheesman; Stefania Bitetti; Zainab Mohamed; Rakesh Sajjan; Raja Padidela; Mars Skae; Sarah Flanagan; Sian Ellard; Karen E Cosgrove; Indraneel Banerjee; Mark J Dunne
Journal:  Front Endocrinol (Lausanne)       Date:  2018-10-17       Impact factor: 5.555

7.  Possible New Strategies for the Treatment of Congenital Hyperinsulinism.

Authors:  Jelena Sikimic; Theresa Hoffmeister; Anne Gresch; Julia Kaiser; Winfried Barthlen; Carmen Wolke; Ilse Wieland; Uwe Lendeckel; Peter Krippeit-Drews; Martina Düfer; Gisela Drews
Journal:  Front Endocrinol (Lausanne)       Date:  2020-10-27       Impact factor: 5.555

8.  Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism: A Finnish Population-Based Study.

Authors:  Jonna M E Männistö; Jarmo Jääskeläinen; Timo Otonkoski; Hanna Huopio
Journal:  J Clin Endocrinol Metab       Date:  2021-03-25       Impact factor: 5.958

9.  Intraoperative ultrasound imaging in the surgical treatment of congenital hyperinsulinism: prospective, blinded study.

Authors:  A P Bjarnesen; P Dahlin; E Globa; H Petersen; K Brusgaard; L Rasmussen; M Melikian; S Detlefsen; H T Christesen; M B Mortensen
Journal:  BJS Open       Date:  2021-03-05
  9 in total

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