Abstract:
BACKGROUND/PURPOSE:Pulmonary hypertension plays a significant role in the pathophysiology of congenital diaphragmatic hernia (CDH). Although there has been an intensive research effort directed at mediators that may cause pulmonary vasoconstriction, no single agent has been identified. The authors hypothesize that there may be an alteration in the cGMP-nitric oxide (NO) pathway of vasodilatation contributing to the pulmonary hypertension observed in CDH. The purpose of these studies is to begin to elucidate vasoactive properties of pulmonary vessels with particular attention to the cGMP-NO pathway of vasodilatation in fetal lambs with CDH. METHODS:Fourth-generation pulmonary arteries and pulmonary veins were dissected from both right and left lungs of eight, 139-day gestational fetuses with surgically created CDH. Vessels were studied with standard isolated tissue bath techniques. Experiments examined basal release of NO in endothelium-intact PVs and PAs of both right and left lungs by measuring the contractile force of vessels constricted with norepinephrine (NE) in the presence and absence of the nitric oxide synthase (NOS) inhibitor N(omega)-nitro-L-arginine (L-NA). Concentration-response curves to the vasodilating agents zaprinast and A23187 were also obtained in vessels contracted by NE. RESULTS:Left and right pulmonary artery responses to NE are enhanced over those of historic controls. Pretreatment of left pulmonary arteries with L-NA enhances the vasoconstrictor response to NE, whereas right PAs show no increased response. Relaxation responses to A23187 and zaprinast, in both left and right pulmonary arteries were not different from control lambs. Relaxation responses of both left and right pulmonary veins to A23187 and zaprinast are blunted compared with controls. This blunting is significantly more in left pulmonary veins than right. Further, right but not left pulmonary veins display enhanced vasoconstrictive response to NE after L-NA pretreatment. CONCLUSIONS:The NO-cGMP pathway of vasodilatation is abnormal in the near term, fetal lamb with CDH. These abnormalities were most apparent in pulmonary veins and may reflect abnormal NOS activity or content between left and right lungs of the fetal lamb with CDH. Pulmonary arteries from CDH lambs have basal and stimulated NO release equal to that of historic controls but appear to be hypersensitive to exogenous vasoconstrictors.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Irish MS,Glick PL,Russell J,Kapur P,Bambini DA,Holm BA,Steinhorn RHdoi
10.1016/s0022-3468(98)90675-3subject
Has Abstractpub_date
1998-06-01 00:00:00pages
921-8issue
6eissn
0022-3468issn
1531-5037pii
S0022-3468(98)90675-3journal_volume
33pub_type
杂志文章abstract::Vascular anomalies are an uncommon cause of gastrointestinal bleeding in childhood. Confusing nomenclature has made objective comparisons of published cases difficult and has interfered with an established consensus regarding diagnosis and therapeutic modalities. The purpose of this study was to clarify the situation ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(97)90640-0
更新日期:1997-06-01 00:00:00
abstract::Unilateral adrenal calcification was encountered incidentally in a 9-year-old boy during evaluation of enuresis. He was a large baby at birth, and perinatal history was suggestive of difficult labor. The case has been discussed through the possible causes of adrenal calcification and the management of these children. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(99)90505-5
更新日期:1999-03-01 00:00:00
abstract::Most appendiceal carcinoids (ACs) in children present without lymph node metastasis. Lymph node metastasis is rarely present when primary tumor diameter exceeds 1 cm. We present the extraordinary case of an AC with a primary tumor diameter of 0.7 cm and infiltration of the mesentery, as well as 1 positive lymph node o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.07.025
更新日期:2010-11-01 00:00:00
abstract::Lipoblastoma of the greater omentum is an extremely rare benign tumor with less than 10 cases reported in the available literature. We report an unusual case of a 10-month-old girl with a giant omental lipoblastoma that composed 20% of her total body weight (1480 g, 17 x 12 x 16 cm). Complete excision of the tumor was...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2007.02.031
更新日期:2007-06-01 00:00:00
abstract:BACKGROUND/PURPOSE:The genetic, embryological, and pathogenetic aspects of hindgut development remain poorly understood. Recently, the morphogenetic pathway involving the Sonic hedgehog (Shh) gene has been shown essential to the normal development of many midaxial organs, including the foregut. This study reports genet...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(00)90014-9
更新日期:2000-02-01 00:00:00
abstract::This is the report of two newborn boys with imperforate anus associated with a long rectocutaneous fistula running deep into the scrotum and communicating, in its midportion, with the bulbar urethra. The findings are compared with those of a somewhat similar case in the literature, with some speculations as to the pos...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(94)90535-5
更新日期:1994-01-01 00:00:00
abstract:BACKGROUND/PURPOSE:Biliary decompression for congenital or acquired obstruction (eg, biliary atresia) does not uniformly lead to liver repair, restore function, or prevent cholangitis. The authors hypothesize that failed repair is caused by altered macrophage (Mo) phenotypes central to an ongoing inflammatory and fibro...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2001.20059
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND/PURPOSE:The aim of this study was to determine early prognostic indicators for the prediction of long-term survival rate and timing of the liver transplantation after the Kasai operation. METHODS:A retrospective chart study was performed on 142 patients with biliary atresia (BA). The patients were divided i...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2000.7765
更新日期:2000-07-01 00:00:00
abstract:BACKGROUND/PURPOSE:CDH occurs in approximately 1 in 2,450 live births. Bilateral CDH, previously identified through a limited number of case reports, is extremely rare. The care of CDH patients is a challenge for neonatologists and surgeons. This report details the management and outcome of patients with bilateral CDH....
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/jpsu.2003.50092
更新日期:2003-03-01 00:00:00
abstract:BACKGROUND:The purpose of this study was to identify patient and treatment characteristics associated with early (in hospital) hernia recurrence after congenital diaphragmatic hernia (CDH) repair. METHODS:Data from the Congenital Diaphragmatic Hernia Study Group registry were queried from 2007 to 2015. Recurrence of t...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.03.011
更新日期:2017-06-01 00:00:00
abstract::Patients with tracheobronchial disease frequently require mechanical ventilation during therapy and experience iatrogenic complications such as barotrauma and volutrauma. The purpose of this study was to determine whether perfluorocarbon-associated gas exchange (PAGE) results in lower ventilatory pressures and more ef...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(97)90177-9
更新日期:1997-02-01 00:00:00
abstract:BACKGROUND/PURPOSE:Necrotizing enterocolitis (NEC) is a severe intestinal disease that primarily affects premature babies, leading to high mortality and morbidity. Probiotics represent an important alternative prophylaxis for NEC but its mechanism of action is poorly understood. Moreover, intestinal and liver-type fatt...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2014.07.007
更新日期:2015-04-01 00:00:00
abstract:PURPOSE:There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%-20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor out...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2020.06.003
更新日期:2020-06-11 00:00:00
abstract::The purpose of this study was to analyze the preliminary results obtained with endoscopic treatment of children with chronic idiopathic pancreatitis. The disease appears to be caused by a deficit of pancreatic stabilizing proteins that leads to precipitation of solutes contained in the pancreatic secretions; these pre...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(96)90837-4
更新日期:1996-10-01 00:00:00
abstract:BACKGROUND/PURPOSE:The management of patients with a giant omphalocele remains a difficult problem. In this study, the authors described a new surgical approach for delayed closure of ventral hernia--the Lazaro da Silva's technique--in conservatively treated patients. METHODS:Ventral hernias of 11 conservatively treat...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2004.03.064
更新日期:2004-07-01 00:00:00
abstract:AIMS:Systematic reviews (SRs) and meta-analyses (MAs) studies may influence and direct surgical practice. Against this background we have analyzed the quality of systematic reviews and meta-analyses published in the specialty field of pediatric surgery using the Assessment of Multiple Systematic Reviews 11-item tool (A...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.07.022
更新日期:2017-11-01 00:00:00
abstract::Mesenteric leiomyosarcoma occurs most commonly in middle-aged individuals. Primary tumors of the mesentery are uncommon in pediatric and adolescent patients, and primary mesenteric leiomyosarcoma is particularly rare. Accurate preoperative diagnosis of mesenteric soft tumor is generally difficult. We describe a case o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.05.015
更新日期:2010-09-01 00:00:00
abstract::Percutaneous aspiration and tetracycline sclerosis is a safe but temporary therapy of large splenic cysts in children. Between 1985 and 1987, three girls with splenic cysts were seen. Their ages ranged from 5 to 14 years, and the cysts were from 8 to 16 cm in diameter. Despite their large size, all were asymptomatic a...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(89)80711-0
更新日期:1989-07-01 00:00:00
abstract::Testicular feminization (TF) is a syndrome due to androgen insensitivity. It occurs in a complete (CTF) and an incomplete (ITF) form. We have treated 21 patients with TF over the last 24 years. Eight patients presented because of ambiguous genitalia, seven presented as "females" with inguinal hernia and testes were fo...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(05)80110-1
更新日期:1992-06-01 00:00:00
abstract::From 1980 to 1994, the authors treated 65 cases of esophageal atresia (with or without tracheoesophageal fistula). Among these patients, 15 (23%) underwent pure proximal circular myotomies and five (8%) underwent both proximal and distal circular myotomies. Of the latter five cases, three were Gross type C and two wer...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(96)90165-7
更新日期:1996-11-01 00:00:00
abstract::Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the ri...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s0022-3468(99)90644-9
更新日期:1999-11-01 00:00:00
abstract:BACKGROUND/PURPOSE:Current treatment for lethal pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) may be hampered by uncertainty over its origin. Herniation of abdominal organs into the chest was thought to produce lung hypoplasia by compression. Experimental CDH models suggest that disturbed lung growth pr...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(00)80029-9
更新日期:2000-01-01 00:00:00
abstract::Neonates with congenital diaphragmatic hernias (CDH) often die because of pulmonary hypoplasia and high pulmonary vascular resistance (PVR). Pulmonary hypertension and right-to-left shunting precedes progressive hypoxia and death. PVR is increased by acidosis and by high airway pressures. High-frequency oscillation (H...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(83)80030-x
更新日期:1983-12-01 00:00:00
abstract::Duodenum inversum is a rare congenital malformation defined by a duodenum which travels superiorly and then posteriorly prior to crossing the midline above the pancreas. It is often confused with other, more common, anomalies of intestinal rotation. We present a case of duodenum inversum diagnosed incidentally in a on...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2012.10.066
更新日期:2013-01-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to evaluate the characteristics of inguinal hernia (IH) and patent processus vaginalis (PPV) in term and preterm infants less than the age of 6months. METHOD:Between January 2004 and December 2012, 246 term and 165 preterm infants underwent laparoscopic herniorrhaphy within the f...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2014.03.013
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND/PURPOSE:Vascular anomalies are a diverse set of lesions with distinct clinical behaviors, whose biomolecular characteristics are largely undefined. Common hemangiomas proliferate during the first year of life, then involute at a variable pace over several years. Other vascular tumors may involute much more q...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2003.11.043
更新日期:2004-03-01 00:00:00
abstract:BACKGROUND:Perceptions toward surgical innovations are critical to the social processes that drive technology adoption. This study aims to capture attitudes of early adopter pediatric surgeons toward robotic technologies in order to clarify 1) specific features that are driving appeal, 2) limiting factors that are acti...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2014.05.017
更新日期:2014-10-01 00:00:00
abstract::The fifteenth case of neonatal adrenal abscess is reported. It is the first case successfully diagnosed with needle aspiration and treated with percutaneous drainage. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(88)80026-5
更新日期:1988-11-01 00:00:00
abstract::This is a case report in which the diagnosis of conjoined twins was made in a woman by ultrasonography in her eigth month of pregnancy. With this diagnosis, the mother-to-be was transported to a hospital with sophisticated obstetrical, neonatal, and pediatric surgical facilities. The twins were delivered by elective c...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(80)80149-7
更新日期:1980-06-01 00:00:00
abstract::This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children's Oncology Group is provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2018.09.005
更新日期:2019-03-01 00:00:00