Abstract：
:Multiple endocrine neoplasia (MEN) 2B is a hereditary syndrome including medullary thyroid carcinoma (MTC), pheochromocytoma, gastrointestinal (GI) disorders, marfanoid facies, and multiple ganglioneuromas. MTC is the major cause of mortality, and often appears during the 1st decade of life. RET proto-oncogene mutations are responsible for MEN 2B. Other RET mutations cause MEN 2A syndrome, familial MTC, or Hirschsprung's disease. We studied three MEN 2B patients with the aim of delineating the best diagnostic and therapeutic protocol. The gold standards for diagnosis are histochemical study of the rectal mucosa and molecular analysis of RET, which in familial cases detects MEN 2B at a preclinical stage so that early total prophylactic thyroidectomy can be performed. In non-familial cases, the diagnosis can be suggested by the presence of GI symptoms, ganglioneuromas, and/or the typical facies. The intestinal innervation pattern, analyzed with the acetylcholinesterase technique, is pathognomonic for MEN 2B. In our protocol a rectal biopsy is, therefore, the first measure. The surgical treatment of MEN 2B is total thyroidectomy with cervical lymphadenectomy of the central compartment of the neck. When possible, this intervention should be performed prophylactically before 1 year of age.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Torre M,Martucciello G,Ceccherini I,Lerone M,Aicardi M,Gambini C,Jasonni Vdoi
10.1007/s00383-002-0824-1subject
Has Abstractpub_date
2002-09-01 00:00:00pages
378-83issue
5-6eissn
0179-0358issn
1437-9813journal_volume
18pub_type
杂志文章abstract：:A new technique for primary reconstruction of a congenital anterior urethral diverticulum is described. This operation eliminates the obstruction caused by the distal lip of the diverticulum and at the same time strengthens the unsupported urethral wall using tissue from the walls of the diverticulum. It has been used...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050582
更新日期：1999-01-01 00:00:00
abstract：UNLABELLED:Vascular trauma is uncommon in children but may be complex. Arterial injury in children presents a challenge to the surgeon and may have long-term implications. Children have major advantage of a rich-collateral circulation but are still growing and developing. Decisions about when to operate remain an issue...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-011-3033-y
更新日期：2012-04-01 00:00:00
abstract：:Malignant rhabdoid tumor (MRT) of the kidney is a rare pediatric tumor characterized by its aggressive nature and chemoresistance. Our patient had MRT of the right kidney with tumor thrombus in the renal vein, inferior vena cava, and right atrium. He developed transfusion-resistant hematuria. This was successfully con...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-013-3260-5
更新日期：2013-05-01 00:00:00
abstract：:An infant developed massive lower gastrointestinal haemorrhage secondary to necrotising enterocolitis after cardiac surgery. The haemorrhage was only controlled following a colectomy. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050613
更新日期：1999-07-01 00:00:00
abstract：:Renal and perinephric abscess in children are uncommon. Three basic pathophysiologic mechanisms are involved, namely, hematogenous spread, ascending infection and contamination by proximity to an infected area. Six pediatric patients diagnosed with renal abscess were treated at our institution from 1990-2000. Five pat...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-002-0888-y
更新日期：2003-04-01 00:00:00
abstract：:Colostomy shift en masse is a novel technique in which the colostomy is shifted along with a rim of skin and abdominal wall tissue. This provides additional length of distal bowel if needed during pull-through anastomosis. We have treated three cases (two Hirschsprung's disease and one anorectal malformation) with col...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-003-1043-0
更新日期：2003-11-01 00:00:00
abstract：:Congenital pyloric atresia (CPA) is a very rare condition that was first described by Calder in 1749. Commonly, CPA occurs as an isolated lesion, which has an excellent prognosis, but it can also be seen in association with other malformations, which can have a negative impact on the final outcome. The medical records...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-007-1903-0
更新日期：2007-06-01 00:00:00
abstract：BACKGROUND:For a definitive diagnosis of congenital solitary kidney, renal scintigraphy is suggested as being the gold standard of ruling out ectopic functioning renal tissue, possibly missed by ultrasound. The aim of our study was to test ultrasonography precision in comparison with renal scintigraphy on a larger coho...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-019-04478-1
更新日期：2019-06-01 00:00:00
abstract：PURPOSE:The Mac-2-binding protein glycosylation-modified isomer (M2BPGi) is a new marker for progression of hepatic fibrosis. We examined the relationship between serum M2BPGi levels and liver histological findings in patients with biliary atresia (BA) who were not transplant candidates. METHODS:Patients with BA who w...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-018-4317-2
更新日期：2018-10-01 00:00:00
abstract：:Polyorchidism is defined as the presence of more than two testes. We report the case of a 3-year-old boy and review the embryology and surgical management of the condition. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050247
更新日期：1998-01-01 00:00:00
abstract：PURPOSE:This study aimed to characterize the surgical complications, especially gastroesophageal reflux disease (GERD), intestinal adhesion obstruction (IAO), and diaphragmatic hernia recurrence, in patients with congenital diaphragmatic hernia (CDH). METHODS:Between January 1995 and December 2013, we determined the i...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-014-3575-x
更新日期：2014-09-01 00:00:00
abstract：:Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However,...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：
更新日期：1997-02-01 00:00:00
abstract：:Congenital/infantile fibrosarcoma (IFS) is a relatively rare form of fibrosarcoma diagnosed at birth or during early years of life and that differs from its adult counterpart because of a more favorable behavior. IFS is also known as cellular congenital mesoblastic nephroma, when it affects the kidney and is often but...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-015-3670-7
更新日期：2015-04-01 00:00:00
abstract：PURPOSE:To perform a medical evaluation of penile necrosis causes and treatment approaches by examining patients who had penile necrosis after circumcision surgery. METHODS:A total of 24 patients with penile necrosis after circumcision surgery, who presented at various hospitals in Turkey between September 2003 and Ap...
journal_title：Pediatric surgery international
pub_type： 杂志文章,多中心研究
doi：10.1007/s00383-020-04630-2
更新日期：2020-04-01 00:00:00
abstract：:A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identifi...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050281
更新日期：1998-03-01 00:00:00
abstract：BACKGROUND:Preoperative factors have been correlated with pre-incision hypotension (PIH) in children undergoing surgery, suggesting that PIH can be predicted through preoperative screening. We studied blood pressure (BP) changes in the 12 min following the induction of anesthesia to study the incidence of post-inductio...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-018-4359-5
更新日期：2018-12-01 00:00:00
abstract：PURPOSE:Partial hepatectomy (PH) can be an inevitable surgical therapy in some conditions, such as hepatic malignancies, trauma or partial liver transplantation. Its capacity for regeneration distinguishes the liver from other essential organs. Regeneration is a complex process involving growth factors, cytokines, tran...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-009-2529-1
更新日期：2010-02-01 00:00:00
abstract：:Gastroschisis closure is performed either primarily or after staged reduction of the prolapsed bowel. A technique for surgical closure of the abdominal wall defect is described which allows for an almost scarless appearance of the abdominal surface with preservation of a midline umbilicus. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-008-2215-8
更新日期：2008-09-01 00:00:00
abstract：:Migration of particulate matter from plastic tubing and solid plastic implants has been documented in a number of studies, including some with the use of cardiac bypass, haemodialysis, and pump-assisted intravenous infusions. In order to ascertain whether silicone embolisation occurs when children have an Ivac 560 pum...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/BF01194802
更新日期：1997-01-01 00:00:00
abstract：BACKGROUND:There is a shortage of surgeons in Africa, and this shortage is particularly acute in paediatric surgery with most paediatric patients being cared for by general surgeons. The use of information technology to augment teaching in paediatric surgery in Africa is appealing but often unsuccessful due to the cost...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-010-2806-z
更新日期：2011-04-01 00:00:00
abstract：INTRODUCTION:Hepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-015-3799-4
更新日期：2015-12-01 00:00:00
abstract：:This article describes five cases of congenital epulis, a rare and benign swelling in the mouth of a newborn, which is not widely known. We present five cases: four cases presented as single pedunculated nodules of the gingiva and in one case two nodules were present. Of all, 50% were located at the maxilla. Excision ...
journal_title：Pediatric surgery international
pub_type： 杂志文章,评审
doi：10.1007/s00383-008-2304-8
更新日期：2009-02-01 00:00:00
abstract：:Cystic teratoma of the pancreas is an extremely rare entity: only 11 cases have been described so far in the world literature. We report the 12th case, in a 4-month-old girl. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/BF00183759
更新日期：1996-03-01 00:00:00
abstract：:Eight children with Morgagni hernia were operated between January 2000 and May 2005. Medical records of the patients were evaluated retrospectively. Ages of the patients were between 3.5 months and 9 years. The diaphragmatic defect was on the right in all patients except one. One patient had bilateral diapragmatic her...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-006-1750-4
更新日期：2006-10-01 00:00:00
abstract：BACKGROUND:Subclinical varicocele is a relative rare condition. The aim of this study is to identify the role of bioflavonoids in the management of subclinical varicocele. MATERIALS AND METHODS:We analyzed the medical charts of patients treated for subclinical varicocele with bioflavonoids between 1999 and 2004; inclu...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-010-2574-9
更新日期：2010-05-01 00:00:00
abstract：:Anorectal malformations (ARM) remain a significant birth defect with geographic variation in incidence, individual phenotypes and regional geographic subtypes. Although early studies indicated a low incidence in Black patients, there is a great paucity of knowledge as to the types, frequency and incidence of ARMs enco...
journal_title：Pediatric surgery international
pub_type： 杂志文章,多中心研究
doi：10.1007/s00383-008-2131-y
更新日期：2008-06-01 00:00:00
abstract：BACKGROUND:Inguinal hernia repair is the most frequently performed surgical procedure in infants and children. Especially in premature infants, prevalence reaches up to 30% in coincidence with high rates of incarceration during the first year of life. These infants carry an increased risk of complications due to genera...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-014-3590-y
更新日期：2014-10-01 00:00:00
abstract：:Hypospadias surgery is one of the most difficult areas in pediatric urology and has been characterized by constant evolution. Some of the surgical techniques proposed in the past are now considered inadequate because of an unacceptable complication rate or poor functional and aesthetic results. The key for assessing a...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-004-1147-1
更新日期：2004-03-01 00:00:00
abstract：:The authors report two patients with extrahepatic portal hypertension and repeated massive bleeding from esophageal varices who underwent urgent distal splenorenal shunting (DSRS) after having proved refractory to medical treatment, endoscopic sclerotherapy, and ligation of esophageal varices. Their ages and weights w...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830100014
更新日期：2001-11-01 00:00:00
abstract：AIM:To determine if follow-up by consultant pediatric surgeons (CPS) affects morbidity due to incarceration (INC) in children with indirect inguinal hernia (IH). METHODS:We educate parents so they can identify possible INC and advise them to attend our emergency department anytime for immediate review by on-call CPS. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章,多中心研究
doi：10.1007/s00383-011-3005-2
更新日期：2012-01-01 00:00:00
