Visualization of the focus in congenital hyperinsulinism by intraoperative sonography

Rohden, L von; Mohnike, Klaus; Mau, Hans; Eberhard, Traugott; Mohnike, Wolfgang; Blankenstein, Oliver; Empting, Susann; Koch, Michael; Füchtner, F.; Barthlen, Winfried

doi:10.1053/j.sempedsurg.2010.10.011

Cited by 13 publications

(12 citation statements)

References 12 publications

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“… Von Rohden et al . reported the usefulness of intraoperative ultrasound showing that in 3 out of 5 cases of the focal form CHI, the findings obtained by ultrasound were almost identical to those obtained by preoperative 18 F-DOPA PET scans ( 116 ). In some of the patients, ultrasound detected a lesion which could not be identified by 18 F-DOPA PET scans.…”

Section: Surgical Treatment ( Fig 1 )mentioning

confidence: 85%

“…The authors also highlighted the weakness of ultrasound in detecting a large segmental lesions. Generally, focal lesions are characterized by hypoechogenicity relative to the surrounding normal pancreas ( 116 ). When a focal lesion cannot be identified by intraoperative visual inspection, palpation, or ultrasound, the extent of the resection can only be determined by intraoperative pathological diagnosis of multiple biopsies taken from each part of the pancreas ( 102 ).…”

Section: Surgical Treatment ( Fig 1 )mentioning

confidence: 99%

Section: Surgical Treatment ( Fig 1 )mentioning

confidence: 99%

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Clinical practice guidelines for congenital hyperinsulinism

Yorifuji

Horikawa

Hasegawa

et al. 2017

Clin Pediatr Endocrinol

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Abstract.Congenital hyperinsulinism is a rare condition, and following recent advances in diagnosis and treatment, it was considered necessary to formulate evidence-based clinical practice guidelines reflecting the most recent progress, to guide the practice of neonatologists, pediatric endocrinologists, general pediatricians, and pediatric surgeons. These guidelines cover a range of aspects, including general features of congenital hyperinsulinism, diagnostic criteria and tools for diagnosis, first- and second-line medical treatment, criteria for and details of surgical treatment, and future perspectives. These guidelines were generated as a collaborative effort between The Japanese Society for Pediatric Endocrinology and The Japanese Society of Pediatric Surgeons, and followed the official procedures of guideline generation to identify important clinical questions, perform a systematic literature review (April 2016), assess the evidence level of each paper, formulate the guidelines, and obtain public comments.

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Section: Surgical Treatment ( Fig 1 )mentioning

confidence: 85%

Section: Surgical Treatment ( Fig 1 )mentioning

confidence: 99%

See 1 more Smart Citation

Clinical practice guidelines for congenital hyperinsulinism

Yorifuji

Horikawa

Hasegawa

et al. 2017

Clin Pediatr Endocrinol

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“…It also helps the surgeon to enucleate the entire focus while preserving the healthy pancreatic tissue. [31]…”

Section: Differentiation Between Focal and Diffuse Forms Of Persistenmentioning

confidence: 99%

Persistent hyperinsulinemic hypoglycemia of infancy: An overview of current concepts

Goel

Choudhury

2012

J Indian Assoc Pediatr Surg

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Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is relatively rare but one of the most important causes of severe neonatal hypoglycemia. Recognition of this entity becomes important due to the fact that the hypoglycemia is so severe and frequent that it may lead to severe neurological damage in the infant manifesting as mental or psychomotor retardation or even a life-threatening event if not recognized and treated effectively in time. Near-total pancreatectomy may be required for patients with intractable hypoglycemia despite medical treatment; however, that may result in diabetes mellitus or recurrent postoperative hypoglycemia. This review aims to consolidate the traditional concepts and current information related to the pathogenesis and management of PHHI.

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“…However, the lesion is not always visible or palpable at the site indicated by 18 F-DOPA PET. Although intraoperative sonography can aid in identification75), repeated intraoperative biopsy may be necessary to help surgeons determine the extent of pancreatectomy. Such a treatment is made possible only by a multidisciplinary team composed of surgeons, radiologists, pediatric endocrinologists, and pathologists who are well experienced in the treatment of CHI76,77).…”

Section: Current Treatment Strategiesmentioning

confidence: 99%

Congenital hyperinsulinism: current status and future perspectives

Yorifuji

2014

Ann Pediatr Endocrinol Metab

147

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The diagnosis and treatment of congenital hyperinsulinism (CHI) have made a remarkable progress over the past 20 years and, currently, it is relatively rare to see patients who are left with severe psychomotor delay. The improvement was made possible by the recent developments in the understanding of the molecular and pathological basis of CHI. Known etiologies include inactivating mutations of the KATP channel genes (ABCC8 and KCNJ11) and HNF4A, HNF1A, HADH, and UCP2 or activating mutations of GLUD1, GCK, and SLC16A1. The understanding of the focal form of KATP channel CHI and its detection by 18F-fluoro-L-DOPA positron emission tomography have revolutionized the management of CHI, and many patients can be cured without postoperative diabetes mellitus. The incidence of the focal form appears to be higher in Asian countries; therefore, the establishment of treatment systems is even more important in this population. In addition to diazoxide or long-term subcutaneous infusion of octreotide or glucagon, long-acting octreotide or lanreotide have also been used successfully until spontaneous remission. Because of these medications, near-total pancreatectomy is less often performed even for the diazoxide-unresponsive diffuse form of CHI. Other promising medications include pasireotide, small-molecule correctors such as sulfonylurea or carbamazepine, GLP1 receptor antagonists, or mammalian target of rapamycin inhibitors. Unsolved questions in this field include the identification of the remaining genes responsible for CHI, the mechanisms leading to transient CHI, and the mechanisms responsible for the spontaneous remission of CHI. This article reviews recent developments and hypothesis regarding these questions.

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Visualization of the focus in congenital hyperinsulinism by intraoperative sonography

Cited by 13 publications

References 12 publications

Clinical practice guidelines for congenital hyperinsulinism

Clinical practice guidelines for congenital hyperinsulinism

Persistent hyperinsulinemic hypoglycemia of infancy: An overview of current concepts

Congenital hyperinsulinism: current status and future perspectives

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