Abstract:
BACKGROUND:A 12-year-old girl presenting with intermittent epigastric pains and diarrhea was referred to our clinic. Diagnostic workup revealed nonfunctional bilateral adrenal pheochromocytomas as well as a neuroendocrine tumor of the pancreatic head. This is the first report on the combination of a neuroendocrine pancreatic tumor with adrenal pheochromocytoma in a pediatric patient with von Hippel-Lindau (VHL) disease. METHODS:von Hippel-Lindau disease was confirmed by molecular genetic analysis of peripheral blood lymphocytes, which revealed the mutation VHL c. 695 G > A. The family history showed also VHL disease in the mother who carried the same mutation. RESULTS AND CONCLUSION:Open laparotomy, organ-sparing enucleation of pheochromocytoma, and pylorus-preserving resection of the pancreatic head tumor were successfully performed. After an uneventful postoperative course, the child fully recovered. She was free of further manifestations of VHL disease 30 months after surgery.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Langrehr JM,Bahra M,Kristiansen G,Neumann HP,Neumann LM,Plöckinger U,Lopez-Hänninen Edoi
10.1016/j.jpedsurg.2007.02.029subject
Has Abstractpub_date
2007-07-01 00:00:00pages
1291-4issue
7eissn
0022-3468issn
1531-5037pii
S0022-3468(07)00139-Xjournal_volume
42pub_type
杂志文章abstract:PURPOSE:We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. METHODS:BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or olde...
journal_title:Journal of pediatric surgery
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更新日期:2018-02-01 00:00:00
abstract::The hormonal responses to surgical stress in adults are well characterized. We hypothesized that children have age-related differences in the "stress responses" to surgery. To test this hypothesis we prospectively studied 98 children (aged 2 to 20 years) undergoing elective surgical procedures under general anesthesia...
journal_title:Journal of pediatric surgery
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abstract::It is necessary to consider catheter-related events when a previously stable patient has an acute change in cardiopulmonary status, regardless of the duration of catheter use; and to recognize that hydrothorax from vein perforation may be bilateral, necessitating bilateral drainage procedures. ...
journal_title:Journal of pediatric surgery
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abstract::The capacity of fetal and newborn hepatic macrophages to produce cachectin/tumor necrosis factor (TNF) has not been previously examined. The role that TNF plays in newborn infection is also unknown. In the present report, fetal and newborn rat hepatic macrophages have been maintained in short-term tissue culture, and ...
journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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更新日期:1991-07-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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journal_title:Journal of pediatric surgery
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更新日期:2010-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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更新日期:2013-07-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:2007-03-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:2010-11-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(89)80109-5
更新日期:1989-11-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
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更新日期:2000-05-01 00:00:00
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更新日期:2014-11-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1989-01-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:2002-12-01 00:00:00
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更新日期:2005-06-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1991-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1984-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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abstract::Fibrocartilaginous mesenchymoma is a rare bone tumor arising from long bones in children and adolescents. With only 21 cases reported in the literature, it is often not included in the differential diagnosis in patients with a bone tumor. Previously, the youngest case reported was in a 19-month-old boy, and a congenit...
journal_title:Journal of pediatric surgery
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abstract:PURPOSE:Necrotizing enterocolitis (NEC) in very low birth weight infants is a risk factor for developmental delay. To our knowledge, there are no studies published investigating the neurodevelopmental outcome of patients with NEC comparing surgically treated and conservatively treated patients versus match paired contr...
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更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:This investigation aims to assess the variability in practice patterns regarding management of children with cryptorchidism (UDT) among pediatric urologists (U) and pediatric surgeons (S) practicing in Canada. METHODS:All active members of Pediatric Urologists of Canada (PUC) and Canadian Association of Ped...
journal_title:Journal of pediatric surgery
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更新日期:2019-09-01 00:00:00
abstract:PURPOSE:SIX2 and CITED1 are transcriptional regulators that specify self-renewing nephronic progenitor cells of the embryonic kidney. We hypothesized that SIX2, which promotes and maintains this stem cell population, and CITED1 remain active in Wilms' tumor (WT). METHODS:To evaluate expression domains and the pathogen...
journal_title:Journal of pediatric surgery
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更新日期:2012-06-01 00:00:00