Abstract:
:Progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AS) are conditions caused by either an autosomal recessive or an autosomal dominant genetic defect, and they are both characterized by cholestasis, jaundice, and severe debilitating pruritus refractory to medical management. Before the advent of liver transplantation, most PFIC patients would die from end-stage liver disease in the first decade of life. Although liver transplantation has led to patients' survival, disease recurrence (PFIC-2) and severe extra-hepatic manifestations of the disease (PFIC-1) occurred post transplant. In the late 1980s, Whitington described the use of partial external biliary diversion in PFIC and AS patients as a successful way to improve symptoms and decrease circulating bile acid serum concentrations. Since then, other diversion techniques have been described (ileal exclusion and partial internal biliary diversion). These techniques have the benefit of avoiding a stoma, but equivalent results have not been demonstrated (recurrence of cholestasis after ileal exclusion, limited follow up after internal biliary diversion). Overall, studies have showed that biliary diversions in children with cholestasis are safe procedures with low morbidity and mortality, and that they can reduce inflammation and ongoing liver injury, therefore delaying or avoiding the need for liver transplantation in some patients.
journal_name
Semin Pediatr Surgjournal_title
Seminars in pediatric surgeryauthors
Lemoine C,Superina Rdoi
10.1016/j.sempedsurg.2020.150946subject
Has Abstractpub_date
2020-08-01 00:00:00pages
150946issue
4eissn
1055-8586issn
1532-9453pii
S1055-8586(20)30066-4journal_volume
29pub_type
杂志文章abstract::Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2009.11.019
更新日期:2010-05-01 00:00:00
abstract::Hirschsprung disease affects many children every year around the world. Currently, there is an extensive menu of diagnostic methods, and surgical treatments. This situation compels the physicians to follow the rationale of these interventions. The comprehensive diagnosis and treatment of Hirschsprung disease need sing...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2019.04.013
更新日期:2019-06-01 00:00:00
abstract::Conventional treatment of respiratory failure involves positive pressure ventilation with high concentrations of inspired oxygen. If adequate gas exchange still cannot be achieved extracorporeal membrane oxygenation (ECMO) may be an option. The general indication for ECMO for respiratory insufficiency is a reversible ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2007.10.006
更新日期:2008-02-01 00:00:00
abstract::We present two patients who developed delayed fistulization following esophageal replacement surgery. The first is a 13-year-old child who, at the age of 3 years, underwent a trans-mediastinal colonic esophageal replacement for a refractory corrosive injury followed by a retrosternal reverse gastric tube after an earl...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2009.02.008
更新日期:2009-05-01 00:00:00
abstract::Variation in clinical practice patterns has attracted the attention of specialty organizations, payers, government health agencies, and the public. Such variation raises concerns about efficacy and cost relative to the care provided. Consequently, the establishment of national benchmarks has become an increasing prior...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2008.02.006
更新日期:2008-05-01 00:00:00
abstract::For most children, cancer has become a curable disease. With this increased survival, the late effects of the diagnosis of cancer and its therapy have assumed increased importance for these children. Multiple late effects of essentially all organ systems have been described in selected groups of survivors. These may b...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1993-02-01 00:00:00
abstract::Gastrointestinal bleeding in infants and children can be quite alarming and anxiety-provoking for parents and caregivers alike. In most cases the cause is benign, and the course self-limited. However, in the patient with significant bleeding, an aggressive diagnostic approach is warranted. The differential diagnosis c...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s1055-8586(99)70027-2
更新日期:1999-11-01 00:00:00
abstract::Enterocolitis remains a relatively common complication of Hirschsprung's disease with significant morbidity and mortality. The etiology of Hirschsprung's enterocolitis (HEC) is multifactorial and remains poorly understood. Preventative measures and better treatment modalities will evolve out of a better understanding ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2004.10.014
更新日期:2004-11-01 00:00:00
abstract::The outcome for children with congenital enteropathies or massive surgical resections has improved significantly over the past two decades. Advances in understanding of the pathophysiology of intractable diarrhea and of the mutations causing many of the congenital enteropathies have enabled initiation of preventive me...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2009.11.003
更新日期:2010-02-01 00:00:00
abstract::Understanding the complex multisystem dysfunction in the infant with a congenital hernia of the posterolateral diaphragm is still evolving and has changed radically during the last decade. The reduction in lung mass, in conjunction with surfactant deficiency and diminished compliance, leads to initial deficiencies in ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1996-11-01 00:00:00
abstract::Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2018.08.007
更新日期:2018-10-01 00:00:00
abstract::The field of intestinal transplantation has experienced dramatic growth since the first reported cases 3 decades ago. Improvements in operative technique, donor assessment and immunosuppressive protocols have afforded children who suffer from life-threatening complications of intestinal failure a chance at long-term s...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2017.07.007
更新日期:2017-08-01 00:00:00
abstract::Myelomeningocele (MMC) is a common birth defect that is associated with significant lifelong morbidity. Little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord, preventing infection, and treating hydrocephalus wit...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s1055-8586(03)00029-5
更新日期:2003-08-01 00:00:00
abstract::Obesity affects 50% of adults and 18% of children in the USA. It has wide-ranging comorbidities with clinical, psychosocial, and economic ramifications. Obesity refers to a condition of excess body fat. The basis for weight gain is a fundamental imbalance between caloric intake and output, but individual variation bas...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2009.04.008
更新日期:2009-08-01 00:00:00
abstract::Most children with short bowel syndrome experience spontaneous small bowel adaptation over time. This allows the majority to be weaned from parenteral nutrition. There are, however, some children who cannot be weaned and are potential candidates for techniques to promote intestinal adaptation and intestinal lengthenin...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/spsu.2001.22855
更新日期:2001-05-01 00:00:00
abstract::Blunt cerebrovascular injury in children is an uncommon occurrence that if missed and left untreated can result in devastating long-term neurologic consequences. Diagnosis can be readily obtained by a computed tomographic angiogram of the head and neck. If confirmed, treatment with antithrombotic therapy dramatically ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2017.01.003
更新日期:2017-02-01 00:00:00
abstract::Pediatric heart transplantation has undergone major changes over the past two decades, marked by a substantial improvement in survival, reduction in posttransplant complications, and enhancement in quality of life for transplant recipients. Actuarial survival has improved substantially in the last decade. Indications ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2006.03.011
更新日期:2006-08-01 00:00:00
abstract::Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. Unfortunately, these conditions present as neonatal emergencies and demand early surgical intervention. This article reviews the embryological development of the chest wall, specific sternal def...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2008.03.004
更新日期:2008-08-01 00:00:00
abstract::Splenectomy in childhood usually is required for hematologic disorders such as hereditary spherocytosis, immune thrombocytopenic purpura, or sickle cell disease. The laparoscopic approach for splenectomy in children has been developed and advanced over the past 5 years. New techniques and equipment allow the procedure...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s1055-8586(98)70032-0
更新日期:1998-11-01 00:00:00
abstract::Focal developmental malformations of the lung are a heterogeneous group of lesions that display a variety of features. They often are thought to be quite distinct entities, but there is considerable overlap among these lesions. Both standard nomenclature and common usage do not identify clearly the components of these...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/spsu.2003.00001
更新日期:2003-02-01 00:00:00
abstract::The unfavorable prognosis of congenital hyperinsulinism (CHI) can be avoided if the patients are treated with high-dose glucose infusions and timely surgical intervention. Circumscribed foci used to be identified by selective percutaneous pancreatic vein catheterization and determination of the insulin level. Fluorine...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2010.10.007
更新日期:2011-02-01 00:00:00
abstract::Minimally invasive surgical approaches to various pediatric surgical disease processes are becoming the standard of care. Laparoscopic Nissen fundoplication is transitioning toward the preferred method for the surgical correction of gastroesophageal reflux (GER) disease in infants and children that do not respond to m...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/spsu.2002.35349
更新日期:2002-11-01 00:00:00
abstract::Adult and pediatric urologists, gynecologists, and surgeons are often faced with primary reconstruction of complex congenital defects of the vagina as seen in cloacal anomalies, disorders of sexual development (DSD), and vaginal agenesis as well as with the secondary repair of post-surgical or radiation-induced vagina...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.sempedsurg.2019.150843
更新日期:2019-10-01 00:00:00
abstract::The increase in multiple gestation pregnancies has resulted in significant health care implications for both mother and child. Our ability to diagnose and intervene on an at-risk multi-gestation pregnancy has dramatically improved. It is important for the pediatric surgeon to be equipped with a basic fund of knowledge...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2019.07.008
更新日期:2019-08-01 00:00:00
abstract::Improvements in parenteral nutrition and supportive therapy have led to a growing population of patients who survive for prolonged periods with short bowel syndrome. Definitive treatment for these patients requires innovative therapy based on a sound knowledge of small intestinal physiology and adaptation. Current und...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1995-02-01 00:00:00
abstract::Caring for children with colorectal and pelvic reconstructive needs is complex and requires a lifelong commitment from clinicians devoted to the field. There has been a myriad of advances that have improved care and it has become clear that an integrated, multi-disciplinary approach maximizes the goal of improving the...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.sempedsurg.2020.150992
更新日期:2020-12-01 00:00:00
abstract::After a diagnosis of omphalocele during pregnancy, questions regarding long-term prognosis are of primary importance for parents. It is imperative that their questions are answered with substantiated data to promote confident decisions for their children. They frequently express concerns regarding long-term survival, ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2019.04.004
更新日期:2019-04-01 00:00:00
abstract::Management strategies for infants with very low birth weight (VLBW) who have abdominal wall defects essentially are the same as for those in larger infants. The authors favor primary closure in infants with gastroschisis, and have achieved this goal in 91% of infants since 1985. Treatment of infants with omphalocele i...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/s1055-8586(00)70022-9
更新日期:2000-05-01 00:00:00
abstract::Inflammatory bowel disease in childhood refers to ulcerative colitis, Crohn's disease, and colitis of an indeterminate type. Their gross and microscopic features are discussed along with the differential diagnosis from other childhood conditions associated with bloody diarrhea. ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2007.04.005
更新日期:2007-08-01 00:00:00
abstract::Management of overgrowth syndromes with complex vascular anomalies is challenging. Careful analysis of the various clinical features by an interdisciplinary team of physicians experienced in this field is paramount to proper diagnostic and therapeutic approaches. In this article, we focus on the spectrum of the clinic...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2014.06.013
更新日期:2014-08-01 00:00:00