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

Kanamori, Yutaka; Watanabe, Toshihiko; Yorifuji, Tohru; Masue, Michiya; Sasaki, Hideyuki; Nio, Masaki

doi:10.1007/s00383-018-4315-4

Cited by 4 publications

(3 citation statements)

References 19 publications

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“…With regard to the post‐treatment diabetes mellitus, there was a significant decline in its incidence over the past 10 years. In Japan, the pre‐emptive identification of the focal form of adenosine triphosphate‐sensitive potassium channel CHI by fluorine‐18‐l‐dihydroxyphenylalanine positron emission tomography followed by local surgical resection was first reported in 2009, and has gradually become the standard management. With the progress in the diagnosis of focal CHI, more aggressive medical treatments of diazoxide‐unresponsive CHI using continuous octreotide infusion and/or enteral nutrition using gastrostomy or nasogastric feeding have also become the standard care (management flowchart available in Yorifuji et al …”

Section: Discussionmentioning

confidence: 99%

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017–2018): Congenital hyperinsulinism, insulinoma, non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)

Yamada

Kitayama

Oyachi

et al. 2019

J of Diabetes Invest

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Aims/Introduction: We aimed to investigate the nationwide incidence, treatment details and outcomes of patients with endogenous hyperinsulinemic hypoglycemia (EHH), including those with transient/persistent congenital hyperinsulinism (CHI), insulinoma, noninsulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease) in Japan. Materials and Methods: A nationwide, questionnaire-based survey was carried out to determine the number of patients with EHH who were treated for hypoglycemia or hypoglycemia-related complications in 2017-2018. The questionnaires were sent to all hospitals in Japan with >300 beds, and with pediatric and/or adult clinics likely managing EHH patients. The secondary questionnaires were sent to obtain the patients' date of birth, sex, age at onset, treatment details and post-treatment outcomes. Results: A total of 447 patients with CHI (197 transient CHI, 225 persistent CHI and 25, unknown subtype), 205 with insulinoma (118 benign, 18 malignant and 69 unknown subtype), 111 with non-insulinoma pancreatogenous hypoglycemia syndrome (33 post-gastric surgery HH, 57 postprandial HH, 10 nesidioblastosis and 11 unknown subtype) and 22 with insulin autoimmune syndrome were identified. Novel findings included: (i) marked improvement in the prognosis of persistent CHI over the past 10 years; (ii) male dominance in the incidence of transient CHI; (iii) non-insulinoma pancreatogenous hypoglycemia syndrome emerging as the second most common form of EHH in adults; (iv) frequent association of diabetes mellitus with insulin autoimmune syndrome; and (v) frequent post-treatment residual hypoglycemia and impaired quality of life. Conclusions: The first nationwide, all age group survey of EHH showed the current status of each type of EHH disorder and the unmet needs of the patients.

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Section: Discussionmentioning

confidence: 99%

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017–2018): Congenital hyperinsulinism, insulinoma, non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)

Yamada

Kitayama

Oyachi

et al. 2019

J of Diabetes Invest

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“…Although surgical treatment is recommended for patients suspected of having a resectable focal lesion ( 1 , 5 ), approximately 70% of patients with focal CHI in Japan have a lesion in the head of the pancreas ( 13 ). In some cases, removal of lesions in the pancreatic head may require a Roux-en-Y pancreatojejunostomy with a higher risk of injury to the common bile duct ( 31 ). This may be one way to maintain medical treatment with subcutaneous OCT or LRT until remission in patients with CHI with a large focal lesion or a lesion in the pancreatic head adjacent to the common bile duct.…”

Section: Discussionmentioning

confidence: 99%

Clinical management of diazoxide-unresponsive congenital hyperinsulinism: A single-center experience

Takasawa,

Iemura,

Orimoto

et al. 2024

Clin Pediatr Endocrinol

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“…When genetic tests and radiologic imaging studies suggest the likelihood of a focal lesion, surgical resection is the approach of choice since infants can be cured and avoid either ongoing hypoglycemia or the development of diabetes mellitus. This is especially true if the location of the lesion allows complete resection and if the surgical team has the necessary expertise, including a protocol for intraoperative examination of frozen biopsies to guide the operative strategy [152][153][154]. For example, in some cases, removal of lesions in the pancreatic head may require a Roux-en-Y pancreatojejunostomy that preserves pancreatic duct connections from the body and tail of the pancreas [87].…”

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confidence: 99%

International Guidelines for the Diagnosis and Management of Hyperinsulinism

León¹,

Arnoux²,

Banerjee³

et al. 2023

Horm Res Paediatr

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Hyperinsulinism (HI) due to dysregulation of pancreatic beta-cell insulin secretion is the most common and most severe cause of persistent hypoglycemia in infants and children. In the 65 years since HI in children was first described, there has been a dramatic advancement in the diagnostic tools available, including new genetic techniques and novel radiologic imaging for focal HI, however; there have been almost no new therapeutic modalities since the development of diazoxide. Recent advances in neonatal research and genetics have improved our understanding of the pathophysiology of both transient and persistent forms of neonatal hyperinsulinism. Rapid turnaround of genetic test results combined with advanced radiologic imaging can permit identification and localization of surgically-curable focal lesions in a large proportion of children with congenital forms of HI, but are only available in certain centers in ‘developed’ countries. Diazoxide, the only drug currently approved for treating HI, was recently designated as an “essential medicine” by the World Health Organization but has been approved in only 16% of Latin American countries and remains unavailable in many under-developed areas of the world. Novel treatments for HI are emerging, but they await completion of safety and efficacy trials before being considered for clinical use. This international consensus statement on diagnosis and management of HI was developed in order to assist specialists, general pediatricians, and neonatologists in early recognition and treatment of HI with the ultimate aim of reducing the prevalence of brain injury caused by hypoglycemia. A previous statement on diagnosis and management of HI in Japan was published in 2017. The current document provides an updated guideline for management of infants and children with HI and includes potential accommodations for less-developed regions of the world where resources may be limited.

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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

Abstract: Surgical resection of a focal hyperplastic lesion in the pancreas was a safe and effective treatment if the hyperplastic lesion was a focal lesion. However, it is necessary to check the exact distribution of the lesion by intraoperative pathologic examination of frozen sections.

Cited by 4 publications

References 19 publications

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017–2018): Congenital hyperinsulinism, insulinoma, non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017–2018): Congenital hyperinsulinism, insulinoma, non‐insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata’s disease)

Clinical management of diazoxide-unresponsive congenital hyperinsulinism: A single-center experience

International Guidelines for the Diagnosis and Management of Hyperinsulinism

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