title: Persistent hyperinsulinemic hypoglycemia of infancy: a clinical and pathological study of 19 cases in a single institution.
abstract: OBJECTIVE
  To study the clinical and pathological features of persistent hyperinsulinemic hypoglycemia of infancy.
  
  
  METHODS
  The clinical and pathological data of 19 cases of persistent hyperinsulinemic hypoglycemia of infancy were retrieved and reviewed from the medical records in Children's Hospital of Fudan University.
  
  
  RESULTS
  There were 13 boys and 6 girls. The age interval was from 16 days to 7 months, and the average age was 2.71 ± 2.23 months. The blood glucose concentrations ranged from 0.57 to 3.0 mmol/L (average value 1.60 ± 0.75 mmol/L) and the serum insulin concentrations ranged from 3.1 to 79.4 uIU/ml (average value 27.89 ± 21.81 uIU/ml) at the time of one week before operation. The size of lesion was between 2 cm to 6.5 cm in maximum diameter (average value 4.04 ± 1.18 cm). 19 cases were divided into three types according to the pathological classification criteria: focal type (1 case), diffuse type (17 cases) and atypical type (1 case). The enucleation of the nodule was given for the patient of focal type and subtotal pancreatectomy was administrated for diffuse and atypical type patients. The blood glucose concentrations ranged from 3.0 to 12.4 mmol/L (average value 6.21 ± 2.69 mmol/L) at the time of one month after operation. The difference between the preoperative blood glucose concentrations and the postoperative blood glucose concentrations was statistically significant (1.6037 ± 0.7458 mmol/L vs. 6.2105 ± 2.6882 mmol/L, P<0.05). Insulin was positive for the multiple pancreatic islets, and the ki-67 index was between 5% and 8%. P57(kip2) was negative. 19 patients were followed up for a period of 2 to 38 months. 13 cases recovered well without any complications, and 6 cases had postoperative hyperglycemia and need a medication to control the blood glucose concentrations.
  
  
  CONCLUSIONS
  Persistent hyperinsulinemic hypoglycemia of infancy mainly occurred in infants. Boys had the predominance and the most common pathological type was the diffuse type. The different surgical methods (focal type with lesion enucleation, diffuse and atypical type with subtotal pancreatectomy), were effective for different patients, and most of patients had a good prognosis. Early diagnosis and appropriate treatment to maintain euglycemia remained the mainstay of management to prevent postoperative complications. Therefore, the correct pathological classification could help clinicians to find effective treatment and improve the outcome.
authors.affiliation: Department of Pathology, Kunming Children's Hospital Kunming, Yunnan Province, China.
authors.forename: Jiwei
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Pathology, Fudan University Children's Hospital Shanghai, China.
authors.forename: Yangyang
authors.initials: Y
authors.lastname: Ma
authors.affiliation: Department of Pathology, Kunming Children's Hospital Kunming, Yunnan Province, China.
authors.forename: Mengxing
authors.initials: M
authors.lastname: Lv
authors.affiliation: Department of Pathology, Kunming Children's Hospital Kunming, Yunnan Province, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Zhou
authors.affiliation: Department of Surgery, Fudan University Children's Hospital Shanghai, China.
authors.forename: Baihui
authors.initials: B
authors.lastname: Liu
authors.affiliation: Department of Surgery, Fudan University Children's Hospital Shanghai, China.
authors.forename: Kuiran
authors.initials: K
authors.lastname: Dong
authors.affiliation: Department of Surgery, Fudan University Children's Hospital Shanghai, China.
authors.forename: Xianmin
authors.initials: X
authors.lastname: Xiao
authors.affiliation: Department of Pathology, Fudan University Children's Hospital Shanghai, China.
authors.forename: Lian
authors.initials: L
authors.lastname: Chen
mesh_terms: D015415:Biomarkers / Q000097:blood; D001786:Blood Glucose / Q000187:drug effects / Q000378:metabolism; D002681:China; D044903:Congenital Hyperinsulinism / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D050761:Cyclin-Dependent Kinase Inhibitor p57 / Q000378:metabolism; D005260:Female; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D007515:Islets of Langerhans / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D019394:Ki-67 Antigen / Q000378:metabolism; D008297:Male; D010180:Pancreatectomy; D020127:Recovery of Function; D012737:Sex Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D001786:Blood Glucose; C493964:CDKN1C protein, human; D050761:Cyclin-Dependent Kinase Inhibitor p57; D007004:Hypoglycemic Agents; D007328:Insulin; D019394:Ki-67 Antigen
keywords: Persistent hyperinsulinemic hypoglycemia of infancy; clinical and pathological study
references.citation: Hum Pathol. 2007 Mar;38(3):387-99
references.pmid: 17303499
references.citation: Hum Mol Genet. 1996 Nov;5(11):1809-12
references.pmid: 8923010
references.citation: Eur J Endocrinol. 2003 Jul;149(1):43-51
references.pmid: 12824865
references.citation: Pediatr Radiol. 2012 Nov;42(11):1372-9
references.pmid: 22885604
references.citation: Am J Surg Pathol. 1989 Sep;13(9):766-75
references.pmid: 2669541
references.citation: J Pediatr Surg. 2004 Mar;39(3):267-9
references.pmid: 15017535
references.citation: J Clin Invest. 1997 Aug 15;100(4):802-7
references.pmid: 9259578
references.citation: Bull Acad Natl Med. 2008 Jan;192(1):59-70; discussion 71-2
references.pmid: 18663982
references.citation: Horm Res. 2008;70(2):65-72
references.pmid: 18547951
references.citation: Diabetes. 1977 Jul;26(7):632-42
references.pmid: 194808
references.citation: Diabetes. 2001 Dec;50(12):2763-9
references.pmid: 11723059
references.citation: J Pediatr Endocrinol Metab. 2012;25(7-8):619-22
references.pmid: 23155683
references.citation: N Engl J Med. 1999 Apr 15;340(15):1169-75
references.pmid: 10202168
references.citation: J Pediatr Surg. 2002 Feb;37(2):155-8
references.pmid: 11819190
references.citation: Diabetes. 2000 Aug;49(8):1325-33
references.pmid: 10923633
references.citation: Horm Res. 2008;69(1):2-13
references.pmid: 18059080
references.citation: Arch Dis Child Fetal Neonatal Ed. 2000 Mar;82(2):F79-86
references.pmid: 10685979
references.citation: J Pediatr Nurs. 2005 Apr;20(2):109-18
references.pmid: 15815570
references.citation: Clin Endocrinol (Oxf). 2011 Sep;75(3):342-6
references.pmid: 21521340
references.citation: J Clin Invest. 1998 Oct 1;102(7):1286-91
references.pmid: 9769320
references.citation: Semin Pediatr Surg. 2011 Feb;20(1):45-9
references.pmid: 21186004
references.citation: Diabetes. 2008 Jan;57(1):259-63
references.pmid: 17942822
references.citation: Pediatrics. 2001 Mar;107(3):476-9
references.pmid: 11230585
references.citation: Horm Res. 2006;66(6):289-96
references.pmid: 17003566
references.citation: Science. 1995 Apr 21;268(5209):426-9
references.pmid: 7716548
references.citation: Arch Dis Child Fetal Neonatal Ed. 2004 Jan;89(1):F65-7
references.pmid: 14711860
references.citation: Hum Pathol. 2008 Jan;39(1):3-8
references.pmid: 18070631
references.citation: Diabetes. 2003 Mar;52(3):784-94
references.pmid: 12606521
references.citation: Front Endocrinol (Lausanne). 2013 May 20;4:60
references.pmid: 23730298
references.citation: AMA Am J Dis Child. 1954 Apr;87(4):399-428
references.pmid: 13147532
references.citation: J Clin Endocrinol Metab. 2009 Mar;94(3):709-28
references.pmid: 19088155
references.citation: Eur J Nucl Med Mol Imaging. 2007 Dec;34(12):2120-8
references.pmid: 17661030
references.citation: Mod Pathol. 1998 May;11(5):444-9
references.pmid: 9619597