Familial nesidioblastosis: Severe neonatal hypoglycemia in two families
Severe neonatal hypoglycemia with pathologic findings of diffuse nesidioblastosis of the pancreas is described in five children of both sexes from two families with unaffected parents. This appears to represent an autosomal recessive disorder of pancreatic development. Despite extensive testing, the...
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| Published in | The Journal of pediatrics Vol. 95; no. 1; pp. 44 - 53 |
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| Main Authors | , , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
United States
Mosby, Inc
01.07.1979
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| Subjects | |
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| Abstract | Severe neonatal hypoglycemia with pathologic findings of diffuse nesidioblastosis of the pancreas is described in five children of both sexes from two families with unaffected parents. This appears to represent an autosomal recessive disorder of pancreatic development. Despite extensive testing, the diagnosis of hyperinsulinism was difficult in the index case of each family and delayed definitive treatment. Medical therapy with steroids and diazoxide was unsuccessful; pancreatectomy was required to treat persistent hypoglycemia. An abnormality of circulating glucagon found in one child with this disorder suggested that hyperinsulinism may not be the sole hormonal imbalance present, but rather that this disease is one of generalized disturbance of islet cell function. The history of severe, persistent neonatal hypoglycemia in an older sibling should lead the physician to investigate subsequent children for the presence of asymptomatic hypoglycemia. |
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| AbstractList | Severe neonatal hypoglycemia with pathologic findings of diffuse nesidoblastosis of the pancreas is described in five children of both sexes from two families with unaffected parents. This appears to represent an autosomal recessive disorder of pancreatic development. Despite extensive testing, the diagnosis of hyperinsulinism was difficult in the index case of each family and delayed definitive treatment. Medical therapy with steroids and diazoxide was unsuccessful; pancreatectomy was required to treat persistent hypoglycemia. An abnormality of circulating glucagon found in one child with this disorder suggested that hyperinsulinism may not be the sole hormonal imbalance present, but rather that this disease is one of generalized disturbance of islet cell function. The history of severe, persistent neonatal hypoglycemia in an older sibling should lead the physician to investigate subsequent children for the presence of asymptomatic hypoglycemia. Severe neonatal hypoglycemia with pathologic findings of diffuse nesidioblastosis of the pancreas is described in five children of both sexes from two families with unaffected parents. This appears to represent an autosomal recessive disorder of pancreatic development. Despite extensive testing, the diagnosis of hyperinsulinism was difficult in the index case of each family and delayed definitive treatment. Medical therapy with steroids and diazoxide was unsuccessful; pancreatectomy was required to treat persistent hypoglycemia. An abnormality of circulating glucagon found in one child with this disorder suggested that hyperinsulinism may not be the sole hormonal imbalance present, but rather that this disease is one of generalized disturbance of islet cell function. The history of severe, persistent neonatal hypoglycemia in an older sibling should lead the physician to investigate subsequent children for the presence of asymptomatic hypoglycemia. |
| Author | Schwartz, Stanley S. Straus, Francis H. Rosenfield, Robert L. Gonen, Boas Wolfsdorf, Joseph Rubenstein, Arthur H. Rich, Barry H. Lucky, Anne W. Madden, John D. Thorp, Frank W. Burrington, John D. |
| Author_xml | – sequence: 1 givenname: Stanley S. surname: Schwartz fullname: Schwartz, Stanley S. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 2 givenname: Barry H. surname: Rich fullname: Rich, Barry H. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 3 givenname: Anne W. surname: Lucky fullname: Lucky, Anne W. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 4 givenname: Francis H. surname: Straus fullname: Straus, Francis H. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 5 givenname: Boas surname: Gonen fullname: Gonen, Boas organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 6 givenname: Joseph surname: Wolfsdorf fullname: Wolfsdorf, Joseph organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 7 givenname: Frank W. surname: Thorp fullname: Thorp, Frank W. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 8 givenname: John D. surname: Burrington fullname: Burrington, John D. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 9 givenname: John D. surname: Madden fullname: Madden, John D. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 10 givenname: Arthur H. surname: Rubenstein fullname: Rubenstein, Arthur H. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA – sequence: 11 givenname: Robert L. surname: Rosenfield fullname: Rosenfield, Robert L. organization: Department of Pediatrics,The University of Chicago Hospitals and Clinics, Pritzker School of Medicine, Chicago, Ill. USA |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/383929$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1542/peds.57.5.702 10.1136/adc.53.5.422 10.1001/jama.1974.03240110030015 10.1007/BF00445763 10.1210/jcem-42-1-173 10.1016/S0022-3476(73)80128-3 10.2337/diabetes.26.7.632 10.1542/peds.39.1.49 10.1016/S0022-3476(71)80105-1 10.1016/0002-9343(72)90071-X 10.1097/00000658-197705000-00002 10.1093/ajcp/20.7_ts.665 10.1172/JCI108521 10.2337/diab.12.2.115 10.1093/ajcp/65.4.462 10.1002/aja.1000380203 10.1016/S0022-3476(73)80581-5 10.1016/S0065-3101(22)00724-1 10.1016/S0140-6736(76)91814-6 10.1097/00000658-196210000-00005 10.1097/00005072-196501000-00012 10.1055/s-0028-1093820 10.1056/NEJM197706092962305 10.1016/S0140-6736(78)90569-X 10.1016/S0022-3476(75)80192-2 10.1016/0022-3468(71)90469-6 10.1136/adc.51.7.528 10.1210/jcem-15-9-1089 |
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infancy contributor: fullname: Cornblath |
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| Snippet | Severe neonatal hypoglycemia with pathologic findings of diffuse nesidioblastosis of the pancreas is described in five children of both sexes from two families... Severe neonatal hypoglycemia with pathologic findings of diffuse nesidoblastosis of the pancreas is described in five children of both sexes from two families... |
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| SubjectTerms | Chromosome Aberrations - genetics Chromosome Disorders Female Glucagon - immunology Humans Hypoglycemia - etiology Hypoglycemia - genetics Infant, Newborn Infant, Newborn, Diseases - genetics Islets of Langerhans - abnormalities Islets of Langerhans - pathology Male Pancreatic Diseases - complications Pancreatic Diseases - diagnosis Pancreatic Diseases - genetics |
| Title | Familial nesidioblastosis: Severe neonatal hypoglycemia in two families |
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