Abstract:
BACKGROUND/PURPOSE:Progressive familial intrahepatic cholestasis (Byler's disease) is often characterized by pruritus-induced self-mutilation with minimal response to medical therapy. The causative cholestasis is likely to progress to cirrhosis necessitating transplantation. Partial external biliary diversion has been used with promising results for the jaundice and debilitating pruritus but all the potential complications and aesthetic concerns of long-term stomas attend this approach. METHODS:The authors describe a terminal ileal exclusion that was first developed for patients who had previously undergone cholecystectomy. Over a 3-year period, we identified for study seven children with liver histology characteristic of Byler's disease accompanying a clinical picture of chronic cholestasis without a defined metabolic or anatomic abnormality. The first two patients underwent a cholecystojejunal cutaneous stoma, until now, the recommended treatment for this condition. The third had previously undergone cholecystectomy so an ileocolonic anastomosis was performed excluding the distal 15% of the small bowel. This child had complete relief of pruritus without evidence of diarrhea. Two more terminal ileal exclusions were performed with similar results before standardizing this approach. The authors approximated small intestinal length using Siebert's graph relating crown-heel length to small intestinal length. The midpoint between the mean and one standard deviation below the mean was determined. Fifteen percent of the estimated small bowel length was measured back from the ileocecal valve and then divided using a linear stapling device. A stapled anastomosis was created between the proximal ileum and the cecum, bypassing the terminal ileum. RESULTS:Four of five children have had relief from their pruritus and self-mutilation with no evidence of diarrhea. Terminal ileal bypass offers a stoma-free, completely reversible "biliary diversion." CONCLUSION:Early results on a few patients are promising, but long-term evaluation of growth, development, and liver function and histology is needed before advocating this as the primary therapy for Byler's disease.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Hollands CM,Rivera-Pedrogo FJ,Gonzalez-Vallina R,Loret-de-Mola O,Nahmad M,Burnweit CAdoi
10.1016/s0022-3468(98)90435-3subject
Has Abstractpub_date
1998-02-01 00:00:00pages
220-4issue
2eissn
0022-3468issn
1531-5037pii
S0022-3468(98)90435-3journal_volume
33pub_type
杂志文章abstract::Prevention and treatment of allograft rejection remain the major issues in clinical small bowel transplantation. New strategies for manipulating immune responses using more powerful immunosuppressive agents continue to be evaluated. The fetal small bowel from BALB/c (H-2d) or C3H/He (H-2k) mice was transplanted into t...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(95)90349-6
更新日期:1995-07-01 00:00:00
abstract:BACKGROUND:This study provides important updates to the epidemiology of pediatric trauma in the United States. METHODS:Age-specific epidemiologic analysis of the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample, representing 2.4 million pediatric traumatic injury discharges in the US from 2000 to ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.04.014
更新日期:2018-04-01 00:00:00
abstract::A model of the isolated bowel segment (IBS, Iowa Model II) was successfully created in experimental animals using a new surgical technique we developed. The IBS is completely free of its mesenteric attachment, yet its viability is preserved. The technique consists of two staged procedures: (1) initial enteropexy betwe...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(91)91036-x
更新日期:1991-12-01 00:00:00
abstract:BACKGROUND/PURPOSE:The optimal time for delivery of neonates with a prenatal diagnosis of gastroschisis (GS) is controversial. We compared the outcomes for GS at three different gestational ages (GAs), 33-34 weeks, 35-36 weeks, and ≥ 37 weeks. METHODS:We analyze hospital discharge data of neonates with GS using the 20...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.07.015
更新日期:2018-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:We aim to justify the need for surgical intervention in our patients with childhood small bowel intussusceptions (SBIs) and review the current concepts in childhood SBI. MATERIALS AND METHODS:We retrospectively reviewed the clinical charts of all patients with surgically confirmed SBI between July 1...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2005.12.028
更新日期:2006-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:Inguinal hernia repair and orchidopexy are among the most common operations in boys. The impact on future fertility has not been conclusively defined. This study evaluates sperm quality after previous inguinal surgery. METHODS:Spermiograms of men with a desire to conceive children were analyzed. His...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.10.012
更新日期:2019-01-01 00:00:00
abstract::The natural history of a rare parotid tumor, the sialoblastoma (embryoma) is reported. It is a blastematous neoplasm said to recapitulate the epithelial differentiation of a gland at various stages of development. The tumor grew in a period of 5 years (from shortly after birth until it was excised) to a firm asymptoma...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(99)90338-x
更新日期:1999-12-01 00:00:00
abstract:BACKGROUND:Minimally invasive bar repair for pectus patients produces substantial pain which dictates the post-operative hospital course. We have data from 2 randomized trials comparing epidural catheter placement to patient controlled analgesia. The purpose of this study was to compare the outcomes of patients who wer...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jpedsurg.2015.05.009
更新日期:2015-11-01 00:00:00
abstract:OBJECTIVE:The aims of the study were to evaluate duration of symptoms, clinical manifestation, radiological findings, fibrobronchoscopic findings, and the complications of airway foreign body (FB). METHODS:A retrospective review of 304 children who had airway FB removed via fibrobronchoscopy from January 1997 to June ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2005.10.064
更新日期:2006-01-01 00:00:00
abstract::The distribution and localization of neurofilament (NF) and microtubule-associated tau protein (Tau) in the colon from Hirschsprung's disease were examined by immunohistochemistry. Specimens of the normoganglionic, oligoganglionic, and aganglionic segments of colons from nine patients with Hirschsprung's disease were ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(93)90688-h
更新日期:1993-07-01 00:00:00
abstract::Numerous factors have been advocated as being paramount to the development of necrotizing enterocolitis (NEC) including hypoxia, abnormal bacterial flora, and by products of enteral feedings. In an effort to better understand mechanisms involved at the level of the intestinal mucosal barrier the authors have chosen th...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(97)90598-4
更新日期:1997-03-01 00:00:00
abstract:BACKGROUND/PURPOSE:Spontaneous pneumomediastinum is an infrequent condition in pediatric patients. Presenting symptoms include chest pain, dysphagia, or vomiting, without initiating event. Patients may undergo esophagram because of concern for esophageal perforation as the source for pneumomediastinum, however, abnorma...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2016.05.024
更新日期:2016-11-01 00:00:00
abstract::Hepatocellular carcinoma is rare in infancy. This report describes the first documented case of congenital hepatocellular carcinoma associated with macronodular cirrhosis. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(86)80079-3
更新日期:1986-02-01 00:00:00
abstract::The authors report a case of acute intestinal bleeding caused by ulcerated segmental ileal dilatation occurring in a 5-month-old infant. The profuse emission of dark red blood per rectum caused acute anemia (Hb 5.3 g/dL). Because of absence of any other sign and symptom and normal plain x-ray, a bleeding Meckel's dive...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2002.35434
更新日期:2002-10-01 00:00:00
abstract:PURPOSE:We sought to determine optimal timing for CPAM resection within the first year of life. METHODS:We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.02.032
更新日期:2018-05-01 00:00:00
abstract::Although infestation with Ascaris lumbricoides causes various intestinal complications, massive gastrointestinal bleeding is rare. The authors report on 2 infants who presented with massive hematemesis. In the first patient, a gastroduodenoscopy was performed. Roundworms were found adhering to an oozing duodenal ulcer...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(03)00584-0
更新日期:2003-11-01 00:00:00
abstract::Necrotizing fasciitis is a rapidly progressive soft tissue infection involving the skin, subcutaneous tissue, and superficial fascia. It is a rare entity in the pediatric population. The specific etiologic factors that contribute to the establishment of necrotizing fasciitis in children are not evident. It may occur a...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2005.01.024
更新日期:2005-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:The authors have shown previously that surgical specimens from infants with acute necrotizing enterocolitis (NEC) show upregulation of inducible nitric oxide (NO) synthase (iNOS) and interferon-gamma mRNA. However, the contribution of other inflammatory cytokines such as interleukin-8 (IL-8), IL-11, ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2001.25726
更新日期:2001-08-01 00:00:00
abstract:BACKGROUND/PURPOSE:Pulse oximetry has been proposed as an appropriate and feasible technique in the assessment of intestinal ischemia in recent years. In this study the authors aimed to assess the reliability of anastomoses in the dog small intestine in which there is graded irreversible ischemia as measured by pulse o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(97)90517-0
更新日期:1997-12-01 00:00:00
abstract::An instance of fibrous hamartoma of infancy (FHI) in the perianal region of an 8-mo-old infant, a hitherto unreported location, is described. The majority of the reported cases occur in the torso and upper extremities. The differential diagnostic features which aid differentiating FHI from other conditions is discusse...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(79)80582-5
更新日期:1979-02-01 00:00:00
abstract::The ideal surgical closure for large omphaloceles remains controversial. Most techniques described advocate removal of the amniotic sac prior to repair of the abdominal wall defect. Herein, the authors describe a surgical technique preserving the amniotic sac, which has distinct advantages in achieving safe and rapid ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(86)90013-8
更新日期:1986-12-01 00:00:00
abstract::Data from 31 infants with gastrointestinal perforation were analyzed to determine the microorganisms usually present in the peritoneum and blood, and the antibiotic susceptibility of those organisms. Multimicrobial contamination of the peritoneal cavity was present in over one-half of the patients and 23% were found t...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(80)80775-5
更新日期:1980-08-01 00:00:00
abstract:PURPOSE:To determine whether treatment of lichen sclerosus et atrophicus (LS), with topical steroids reduces the rate of circumcision. METHODS:Two independent reviewers performed a literature search of studies reporting treatment of LS with topical steroids using EMBASE and MEDLINE database(s). INCLUSION CRITERIA:boy...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.12.021
更新日期:2018-11-01 00:00:00
abstract::Extralobar pulmonary sequestrations have been occasionally described in the abdomen but rarely in the diaphragm. We present the case of a 10 month old girl with an intradiaphragmatic pulmonary sequestration. The minimally invasive operative technique is outlined in detail, including the combine use of laparoscopy and ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2012.09.036
更新日期:2012-11-01 00:00:00
abstract:BACKGROUND/PURPOSE:Recent advances including prenatal diagnosis, high-frequency oscillatory ventilation, and nitric oxide inhalation therapy have gradually improved the survival of high-risk congenital diaphragmatic hernia. However, the factors affecting the long-term outcome of these patients have not been well establ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2005.08.022
更新日期:2005-12-01 00:00:00
abstract::Tracheal agenesis is a rare and usually lethal congenital malformation of the forgut. Although some infants can be resuscitated with an intra-esophageal intubation temporarily, long-term airway management is difficult because of the collapsing airway. We report a long-term survivor with tracheal agenesis in whom a Gor...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2008.06.013
更新日期:2008-10-01 00:00:00
abstract:BACKGROUND:Single visit surgery (SVS) consists of same-day pre-operative assessment and operation with telephone post-operative follow-up. This reduces family time commitment to 1 hospital trip rather than 2-3. We began SVS for ambulatory patients with clear surgical indications in 2013. We sought to determine family s...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.10.021
更新日期:2017-10-10 00:00:00
abstract:OBJECTIVES:It is now well established that hydrocephalus is associated with impaired bladder function. The aim of this study was to determine the effects of hydrocephalus on bladder smooth muscle (BSM) reactivity in the rat model. MATERIALS AND METHODS:Hydrocephalus was induced in 7-day-old rats by injection of kaolin...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2007.12.064
更新日期:2008-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:Herniorrhaphy is the most common general surgical procedure performed on children, and hernia sac material is one of the most common tissue specimens microscopically examined in the authors' surgical pathology laboratory. The risk of accidental vas deferens ligation has prompted the recommendation th...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(98)90537-1
更新日期:1998-07-01 00:00:00
abstract:BACKGROUND:Ovarian immature teratomas (ITs) are relatively rare among all pediatric ovarian tumors. The histological grading for ovarian ITs, which ranges from 1 to 3, is based on the proportion of immature neuroepithelial component. Higher-grade ITs in adults are treated as malignant neoplasms and require adjuvant che...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.04.037
更新日期:2020-04-01 00:00:00