Abstract:
BACKGROUND:Intestinal failure-associated liver disease (IFALD) remains a serious problem in the treatment of infants with nutritional problems and short bowel syndrome. METHODS:A review of the recent literature from 2010 to 2016, concentrating on articles related to the pathophysiology of IFALD and to outcomes of novel nutritional and pharmacological therapies for neonatal cholestasis in the post-surgical neonate. RESULTS:The pathophysiology of IFALD relates to an increase sensitivity of the neonatal liver to cholestasis in the non-fed state; prolonged cholestasis almost inevitably results in liver damage which will progress from fibrosis to cirrhosis. Clinically discerned risk factors include premature birth, inflammation, sepsis, disruption of the enterohepatic circulation by creation of a proximal stoma, and the duration and type of parenteral nutritional support. Within the hepatocyte, the regulatory enzyme farsanoid receptor X (FXR) appears to play a pivotal role in the development of cholestasis. Recent studies have shown that its activity is suppressed by sepsis, and by plant phytosterols found in soy-based lipid preparations. This paradigm is reflected in the emerging consensus for the care of post-surgical neonates, which is based around a multi-disciplinary team approach. Using an algorithm-driven approach, an appropriate balance between caloric support and prevention of IFALD can be achieved. CONCLUSIONS:Further prospective studies are required to further refine the optimal sequence of use of these therapies and the long-term effects on neurological development and hepatic function. However, with optimal care, the number of IF patients progressing to end-stage liver disease because of IFALD should be very low.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Al-Shahwani NH,Sigalet DLdoi
10.1007/s00383-016-4042-7subject
Has Abstractpub_date
2017-04-01 00:00:00pages
405-411issue
4eissn
0179-0358issn
1437-9813pii
10.1007/s00383-016-4042-7journal_volume
33pub_type
杂志文章,评审abstract:PURPOSE:Haemolytic uraemic syndrome (HUS) is the commonest childhood cause of acute renal failure. Peritoneal dialysis peritonitis (PDP) is a well-recognised complication, with some children requiring surgical intervention (SI). The aim of this study is to determine whether the presence of enteric organisms in cases of...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3833-6
更新日期:2016-03-01 00:00:00
abstract:PURPOSE:Sacrococcygeal teratoma (SCT) is the most common congenital neoplasm in neonates. We wished to assess the long-term functional outcome of children undergoing SCT resection. METHODS:Records of neonates diagnosed with SCT from two surgeons' practices, and operated on between 1970 and 2006, were retrospectively r...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2322-1
更新日期:2009-03-01 00:00:00
abstract::Acute abdomen is caused by a wide variety of etiologies, many of which require surgical intervention. Two boys were hospitalized for acute abdominal pain and low-grade fever. Physical examination revealed epigastric and right upper abdominal fullness, and laboratory studies showed elevated erythrocyte sedimentation ra...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0942-9
更新日期:2003-11-01 00:00:00
abstract::Between 1993-2002 we used both diagnostic and operative laparoscopy in the management of five cases of persistent müllerian duct syndrome (BMDS). Two siblings from two different families accounted for four of the cases. They presented with cryptorchidism and inguinal hernias. The diagnosis was established during diagn...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-003-0984-7
更新日期:2003-09-01 00:00:00
abstract::We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4216-y
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:Pediatric surgeons and patient organisations agree that fewer centers for anorectal malformations with larger patient numbers are essential to reach better treatment. The European Union transacts a political process which aims to realize such centers of expertise for a multitude of rare diseases. All the cen...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3752-6
更新日期:2015-08-01 00:00:00
abstract::Adrenocortical tumors (ACT) are rare in childhood. Determination of malignant potential is difficult. To assess the presentation, clinical behaviour, and histology of these tumors in our center, the records of nine patients with ACTs between 1989 and 2000 were analyzed. The age, sex, clinical presentation, investigati...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0727-1
更新日期:2003-08-01 00:00:00
abstract::There are few publications about urolithiasis of the new born baby and infant (UNI). The UNI represents 20% of the pediatric urolithiasis. The etiologies in this age group are chiefly dominated by the urinary-tract infections and metabolic abnormalities. The purpose of this paper was to investigate the epidemiological...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-007-1889-7
更新日期:2007-04-01 00:00:00
abstract::Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicou...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000414
更新日期:2000-01-01 00:00:00
abstract::Congenital lumbar hernia (CLH) is a rare anomaly with only 45 cases reported in the English-language literature. This paper describes nine patients with CLH treated in our unit. Unusual features included the relatively high incidence of inferior lumbar hernia, presentation at the age of 6 years in one case, and an ass...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050048
更新日期:2000-01-01 00:00:00
abstract::CHARGE association and DiGeorge syndrome (DGS) rarely occur together and only eight cases have been reported in the English literature. Two were associated with esophageal atresia (EA) and severe congenital heart anomalies. We report a third case of EA with tracheoesophageal fistula (EA-TEF) associated with coarctatio...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-008-2222-9
更新日期:2008-10-01 00:00:00
abstract:PURPOSE:For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2417-8
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:To describe a modification of PATIO repair for urethrocutaneous fistula repair and evaluate its outcome. METHODS:We studied 15 boys who underwent modified PATIO repair from Jan 2010 to Sept 2015. Parameters studied included age, type of hypospadias, age at first urethroplasty, hypospadias repair technique, ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3983-1
更新日期:2017-01-01 00:00:00
abstract::Extrarenal malignant rhabdoid tumour is a rare neoplasm of childhood. A congenital example has only been reported once previously, presenting as a facial mass. We report a second congenital case with the tumour over the right shoulder in which postoperative investigations revealed multiple hepatic metastases proving f...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00497837
更新日期:1996-06-01 00:00:00
abstract:PURPOSE:Eosinophilic gastrointestinal disorders are rare in children and present with a broad spectrum of non-specific symptoms. To date, no guidelines for diagnosis, therapy and follow-up are validated. Aim of our study is to focus on eosinophilic colitis (EC), to determine a possible correlation between associated di...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-020-04832-8
更新日期:2021-01-07 00:00:00
abstract:AIM:The Ncx/Hox11L.1 gene is required for adequate development of enteric neurons in mice and Ncx/Hox11L.1 deficient (Ncx-/-) mice are used as a model for human intestinal neuronal dysplasia (IND) because of similar histopathology (hyperganglionosis), however, some 50% of Ncx-/- mice develop megacolon with a caliber ch...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2451-6
更新日期:2009-11-01 00:00:00
abstract::The incidence of postoperative cholangitis has changed very little despite progressive improvement in the treatment of biliary atresia. The role of the bilioenteric conduit in its pathogenesis is still uncertain. A retrospective study of 39 patients undergoing either a conventional Kasai operation (group 1, n = 20) or...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050008
更新日期:2000-01-01 00:00:00
abstract::The successful use of a combination of "patch, drain, and wait" (PDW) and home total parenteral nutrition (TPN) in the management of a case of acute, catastrophic midgut volvulus in a 2-year-11-month-old boy with near-total ischemia/necrosis of his small intestine is reported. The PDW approach to the highly effective ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:
更新日期:1997-03-21 00:00:00
abstract:PURPOSE:Radiologically inserted gastrojejunal tubes (RGJ) and surgical jejunostomy (SJ) are established modes of jejunal feeding. The aim of the study is to review nutritional outcomes, complications and the practical consideration to enable patients and carers to make informed choice. METHODS:Retrospective review of ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-018-4303-8
更新日期:2018-09-01 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:PURPOSE:The Nuss procedure is a minimally invasive procedure for the correction of pectus excavatum. It involves insertion of a substernal metal bar. A feared complication of any implantation procedure is infection, which often requires removal of the implanted device. This report describes the authors' experience with...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3145-z
更新日期:2012-09-01 00:00:00
abstract::Two patients who presented with a history of right inguinal hernia and a third with an impalpable left testis were found to have transverse testicular ectopia. Both testes were fixed in their own scrotum, through a modified Ombredanne operation in two patients and a subdartos pouch in one. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:
更新日期:1997-02-01 00:00:00
abstract::To determine which laser can be used for a vascular lesion, the different working mechanisms of the individual lasers must first be understood. The localization and diameter of the vessels to be treated define whether a certain laser is appropriate for that lesion. For teleangiectasias and port-wine stains the pulsed-...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00497797
更新日期:1996-06-01 00:00:00
abstract:PURPOSE:to report the early experience with the Kelly procedure for the treatment of bladder exstrophy (BE) in males. MATERIALS AND METHODS:Nine boys with BE were treated at our institute. One had an untouched BE, four had epispadias after neonatal bladder closure, and four were secondary phalloplasties. Data on surgi...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2356-4
更新日期:2009-05-01 00:00:00
abstract::A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children's Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomal...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-005-1520-8
更新日期:2005-11-01 00:00:00
abstract:AIM:An experimental study was performed to investigate the efficacy of colchicine in the prevention of fibrosis in the alkaline burn of the esophagus in rats. METHOD:A standard esophageal alkaline burn was produced by the application of 37.5% NaOH for a period of 90 s to the distal esophagus followed by a water rinse....
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1644-5
更新日期:2006-04-01 00:00:00
abstract:PURPOSE:We performed a systematic review and meta-analysis to compare the efficacy and safety between single-incision, transscrotal orchidopexy, and the traditional inguinal orchidopexy in children. METHODS:A systematic search of the electronic databases was conducted to identify studies compared the transscrotal orch...
journal_title:Pediatric surgery international
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00383-016-3956-4
更新日期:2016-10-01 00:00:00
abstract:INTRODUCTION:This review aims to (1) assess the breadth of pediatric orthopaedic research in low- and middle-income countries (LMICs) and (2) determine the impact of academic collaboration (an LMIC and a non-LMIC investigator) in published LMIC research. METHODS:Pediatric orthopaedic clinical studies conducted in LMIC...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-018-4412-4
更新日期:2019-03-01 00:00:00
abstract::Congenital diaphragmatic hernia (CDH) is accompanied by pulmonary hypoplasia and pulmonary hypertension. Fetal lung growth is dependent on the secretion of lung liquid, which normally is absorbed at partus. The ion channel NKCC-1 is involved in this secretory process, but has recently also been reported to be implicat...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2209-6
更新日期:2008-09-01 00:00:00
abstract::Our purpose was to clarify factors that influence the level of depression of Japanese children with anorectal malformations (ARM). The subjects comprised 66 children with ARM, aged 0-16 years, and their mothers. Patients were divided into three groups (Group 1: aged 0-5 years, Group 2: 6-11 years, and Group 3: 12-16 y...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-007-2077-5
更新日期:2008-03-01 00:00:00