Laparoscopic identification and removal of focal lesions in persistent hyperinsulinemic hypoglycemia of infancy

Bax, N. M. A.; Zee, David C. van der; Vroede, Monique De; Jansen, Marlijn S; Nikkels, Peter G. J.

doi:10.1007/s00464-002-4268-4

Cited by 18 publications

(15 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The technical aspects were discussed previously. 16 The combination of magnification (up to 18-fold when the 5-mm scope is at a distance from the object of 2 cm) and the "palpation" of the pancreatic tissue with a suction cannula allows identification of lesions in the entire pancreas. After detachment of the lower border of the pancreas, the posterior part of the pancreas can also be well visualized and searched with a palpating probe.…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy

Vroede¹

2004

Pediatrics

View full text Add to dashboard Cite

ABSTRACT. Persistent hyperinsulinemic hypoglycemia of infancy or congenital hyperinsulinism of the neonate is a rare condition that may cause severe neurologic damage if the disease is unrecognized or inadequately treated. Current treatment aims to restore normal blood glucose levels by providing a carbohydrate-enriched diet and drugs that inhibit insulin secretion. If medical treatment fails, then surgery is required. Because congenital hyperinsulinism may be caused either by diffuse involvement of pancreatic ␤-cells or by a focal cluster of abnormal ␤-cells, the extent of pancreatectomy varies. We report on 2 patients with a focal form of the disease for whom diagnosis was made with laparoscopy. P ersistent hyperinsulinemic hypoglycemia of infancy (PHHI), or congenital hyperinsulinism, is a rare disease characterized by inappropriate insulin secretion in the presence of hypoglycemia. Severe neurodevelopmental morbidity among these patients is reportedly attributable to late diagnosis and/or inability to control the profound hypoglycemia. Most cases are attributable to mutations in the genes coding for the subunits of the adenosine triphosphate-sensitive potassium (K ATP ) channels found in pancreatic ␤-cells, sulfonylurea receptor (SUR)1 (ABCC8) and potassium inward rectifier (Kir)6.2 (KCNJ11). 1 In rarer cases of PHHI, mutations in the glucokinase gene (GCK), glutamate dehydrogenase gene (GLUD1), and short-chain hydroxyacylcoenzyme A dehydrogenase (HADHSC) gene are pathogenic and act mainly by subverting the operation of K ATP channels. 2,3 Treatment is directed toward normoglycemia, through provision of adequate carbohydrate coverage and the use of drugs that interact with the ion channels to lower insulin secretion. 4 Diazoxide, octreotide, and calcium channel blockers are available, but diazoxide is the drug of choice. PHHI is a heterogeneous disease; in a few cases, it may be transient and mild, requiring only short-term medical treatment. In other cases, including those involving mutations in glucokinase, glutamate dehydrogenase, and short-chain hydroxyacylcoenzyme A dehydrogenase genes, diazoxide and appropriate diet are required for a longer time but are very effective in controlling hypoglycemia. 2,3,5,6 However, almost 80% of neonates with congenital hyperinsulinism fail to respond to medical treatment and require near-total pancreatectomy. 5 This procedure leads to important digestive and endocrine morbidities, whereas removal of insufficient amounts of the pancreas increases the duration of hypoglycemia, necessitates reintervention, and enhances the risk of hypoglycemic neuronal injury.The observations in 1975 7 and then in 1989 8 that PHHI may be caused by either diffuse or focal abnormalities of ␤-cells changed the surgical approach and led to the development of radiologic procedures to identify these focal forms. We now report on 2 children with PHHI for whom a focal lesion was diagnosed during laparoscopy and for whom laparoscopic enucleation was curative. CASE REPORTS Patient 1Patient 1 is the...

show abstract

Section: Discussionmentioning

confidence: 99%

Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy

Vroede¹

2004

Pediatrics

View full text Add to dashboard Cite

show abstract

“…Because this represents a significant number of patients that are potentially curable through a limited surgical intervention to remove just the focal lesion, much effort is now being placed on attempts to diagnose Fo-HI to avoid the obvious long-term complications of radical pancreatic surgery (49, 173,192,201,299). Suggested diagnostic procedures include preoperative interventional radiography such as intra-arterial calcium stimulation tests, pancreatic venous sampling or transhepatic portal venous insulin sampling, positron emission tomography, laparoscopy, and intraoperative histological examination of biopsies of the resected pancreas (14,20,25,33,62,82,220,245,246). A greater understanding of the pathophysiology of ␤-cells in HI has also led to the introduction of a noninvasive procedure to monitor acute insulin-response profiles to glucose and tolbutamide in patients to distinguish focal forms of HI from diffuse disease (108, see sect.…”

Section: B Genetic and Histopathophysiological Diversity Of Hi-katp:mentioning

confidence: 99%

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Dunne

Cosgrove²,

Shepherd³

et al. 2004

Physiological Reviews

260

277

View full text Add to dashboard Cite

Dunne, Mark J., Karen E. Cosgrove, Ruth M. Shepherd, Albert Aynsley-Green, and Keith J. Lindley. Hyperinsulinism in Infancy: From Basic Science to Clinical Disease. Physiol Rev 84: 239–275, 2004; 10.1152/physrev.00022.2003.—Ion channelopathies have now been described in many well-characterized cell types including neurons, myocytes, epithelial cells, and endocrine cells. However, in only a few cases has the relationship between altered ion channel function, cell biology, and clinical disease been defined. Hyperinsulinism in infancy (HI) is a rare, potentially lethal condition of the newborn and early childhood. The causes of HI are varied and numerous, but in almost all cases they share a common target protein, the ATP-sensitive K+ channel. From gene defects in ion channel subunits to defects in β-cell metabolism and anaplerosis, this review describes the relationship between pathogenesis and clinical medicine. Until recently, HI was generally considered an orphan disease, but as parallel defects in ion channels, enzymes, and metabolic pathways also give rise to diabetes and impaired insulin release, the HI paradigm has wider implications for more common disorders of the endocrine pancreas and the molecular physiology of ion transport.

show abstract

“…One report contained one patient who underwent laparoscopic pancreatectomy followed by an open near-total pancreatectomy 7 days later to control hypoglycemia [11]. The other report contained 2 patients with PHHI because of focal lesions found laparoscopically [12]. Its laparoscopic enucleation was curative.…”

Section: Discussionmentioning

confidence: 98%

“…Most of these reports are among adult population with mostly distal pancreatectomies. Few case reports have shown the feasibility of the technique in the pediatric population [8][9][10], and only 2 reports have 3 patients with PHHI [11,12]. We report our experience with laparoscopic pancreatectomy for PHHI for the last 4 years.…”

mentioning

confidence: 93%

Laparoscopic pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy

Al-Shanafey¹,

Zayeni²,

AlNassar³

2009

Journal of Pediatric Surgery

View full text Add to dashboard Cite

show abstract

Laparoscopic identification and removal of focal lesions in persistent hyperinsulinemic hypoglycemia of infancy

Abstract: A laparoscopic approach seems to be ideal for patients with PHHI not only because of the magnification but also because of the delicate surgery it allows and the avoidance of major abdominal wall problems.

Cited by 18 publications

References 8 publications

Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy

Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Laparoscopic pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy

Contact Info

Product

Resources

About