eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Persistent Hyperinsulinemic Hypoglycemia of Infancy: Multimedia

Author: Robert S Gillespie, MD, MPH, Consulting Staff, Department of Nephrology, Cook Children's Medical Center
Coauthor(s): Stephen Ponder, MD, CDE, Director, Division of Pediatric Endocrinology, Department of Pediatrics, Driscoll Children's Hospital; Professor, Texas A&M College of Medicine
Contributor Information and Disclosures

Updated: Nov 7, 2008

Multimedia

Pancreatic specimen showing diffuse persistent hy...Media file 1: Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at low power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.
Pancreatic specimen showing diffuse persistent hy...

Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at low power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.

Pancreatic specimen showing diffuse persistent hy...Media file 2: Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at medium power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.
Pancreatic specimen showing diffuse persistent hy...

Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at medium power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.

Pancreatic specimen showing diffuse persistent hy...Media file 3: Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at high power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.
Pancreatic specimen showing diffuse persistent hy...

Pancreatic specimen showing diffuse persistent hyperinsulinemic hypoglycemia of infancy (PHHI) viewed at high power. The paler-staining cells are the neuroendocrine (islet) cells, which should be arranged in discrete islands within the acinar lobules. Acinar cells are the exocrine cells that have denser-staining, dark eosinophilic cytoplasm. These acinar cells are arranged in acini-small glands. In PHHI, more of the neuroendocrine cells are present, and they are arranged more diffusely throughout the lobules. Image courtesy of Phil Collins, MD.

Normal pancreas. There are fewer of the paler-sta...Media file 4: Normal pancreas. There are fewer of the paler-staining neuroendocrine (islet) cells, and they are arranged in more discrete islands. Image courtesy of Tom Milligan, MD, Driscoll Children's Hospital, Corpus Christi, Tex.
Normal pancreas. There are fewer of the paler-sta...

Normal pancreas. There are fewer of the paler-staining neuroendocrine (islet) cells, and they are arranged in more discrete islands. Image courtesy of Tom Milligan, MD, Driscoll Children's Hospital, Corpus Christi, Tex.

Combined positron emission tomography (PET)/CT sc...Media file 5: Combined positron emission tomography (PET)/CT scan image of a focal lesion in the head of the pancreas of an infant with congenital hyperinsulinism. Uptake of [18-F]-L-DOPA glows brightly in the head of the pancreas (center), pinpointing abnormal cells in focal hyperinsulinism. The large glowing areas lower in the image are the kidneys, where [18-F]-L-DOPA is excreted. Courtesy of Charles Stanley, MD, Children's Hospital of Philadelphia.
Combined positron emission tomography (PET)/CT sc...

Combined positron emission tomography (PET)/CT scan image of a focal lesion in the head of the pancreas of an infant with congenital hyperinsulinism. Uptake of [18-F]-L-DOPA glows brightly in the head of the pancreas (center), pinpointing abnormal cells in focal hyperinsulinism. The large glowing areas lower in the image are the kidneys, where [18-F]-L-DOPA is excreted. Courtesy of Charles Stanley, MD, Children's Hospital of Philadelphia.

More on Persistent Hyperinsulinemic Hypoglycemia of Infancy

Overview: Persistent Hyperinsulinemic Hypoglycemia of Infancy
Differential Diagnoses & Workup: Persistent Hyperinsulinemic Hypoglycemia of Infancy
Treatment & Medication: Persistent Hyperinsulinemic Hypoglycemia of Infancy
Follow-up: Persistent Hyperinsulinemic Hypoglycemia of Infancy
Multimedia: Persistent Hyperinsulinemic Hypoglycemia of Infancy
References

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Further Reading

Keywords

persistent hyperinsulinemic hypoglycemia of infancy, PHHI, nesidioblastosis, congenital hyperinsulinism, CHI, islet cell dysmaturation syndrome, islet cell adenomatosis, nesidioblastoma, familial hyperinsulinism with pancreatic nesidioblastosis, focal adenomatous hyperplasia, diffuse discrete beta cell abnormality, beta cell, beta-cell, B cell, B-cell, focal adenomatous hyperplasia, seizures, developmental delay, focal neurologic deficits, hepatomegaly, glycogen-storage disorder, galactosemia, fructosemia

Contributor Information and Disclosures

Author

Robert S Gillespie, MD, MPH, Consulting Staff, Department of Nephrology, Cook Children's Medical Center
Robert S Gillespie, MD, MPH is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, and Texas Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Stephen Ponder, MD, CDE, Director, Division of Pediatric Endocrinology, Department of Pediatrics, Driscoll Children's Hospital; Professor, Texas A&M College of Medicine
Stephen Ponder, MD, CDE is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Thomas A Wilson, MD, Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook
Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London), Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece
George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfiser, Inc. Honoraria Consulting

 
 
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