Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Despite improvements in prenatal diagnosis and neonatal intensive care, the Congenital Diaphragmatic Hernia (CDH) Registry still records a 64% survival rate. Many reports demonstrate, however, that approximately 80% of CDH patients with no other malformations may survive if managed with permissive hypercapnia, gentle ventilation, high-frequency oscillatory ventilation (HFOV), surfactant, inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO), and delayed surgical repair. We wished to define the evolving outcome of CDH newborns using a protocol approach to management, which includes surgery in the neonatal intensive care unit (NICU) or operating room (OR). From January 1996, data were collected prospectively on 42 consecutive live-born infants with CDH. Newborns symptomatic at birth were sedated and paralyzed in the delivery room, and treated with elective HFOV, iNO, surfactant, and ECMO as necessary, delaying surgical repair until their clinical conditions were stable. Once the CDH newborn was stabilized, a trial on conventional ventilation was started at least 24 hours before surgery; however, if the patient was unstable, therapy was switched back to HFOV and surgery was performed in the NICU. Demographic and clinical parameters were compared between CDH newborns who underwent surgery in the NICU and in the OR. The two groups were comparable in terms of clinical characteristics and baseline ventilatory and blood gas values. Mean age at surgery was 3 +/- 2 days. After surgery, the NICU group had more infectious complications. However, the survival rate of uncomplicated CDH was 78% and a low rate of chronic lung disease was reported. A prolonged phase of presurgery stabilization is proposed and strict control of infection is recommended for the CDH newborns who might benefit from an exclusive HFOV and NICU surgery.
 ...
PMID:Congenital diaphragmatic hernia: intensive care unit or operating room? 1590 12

The mortality rate associated with congenital diaphragmatic hernia (CDH) varies widely between centers and remains relatively high despite widespread use of new therapeutic modalities. Many of these have been implemented without properly controlled studies. Over the past 10 to 15 years, only 9 randomized trials enrolling a total of approximately 250 infants with CDH have been published. The limited evidence available suggests that better outcomes are observed by delivering infants with CDH at experienced centers, by delaying surgical repair until hemodynamic and respiratory stability is achieved, and by the judicious utilization of nonaggressive mechanical ventilation and permissive hypercapnea. Other therapeutic modalities, such as high frequency oscillatory ventilation, inhaled nitric oxide, and ECMO, may provide additional advantages for selected infants. There is a dire need to establish networks of centers that manage enough infants with CDH, to conduct appropriately sized randomized trials that can answer some of the critical questions about the management and long-term outcome of these infants.
 ...
PMID:Evidence-based management of infants with congenital diaphragmatic hernia. 1605 May 29

The molecular basis of the pathogenesis of pulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) is poorly understood. Variation in responses to therapeutic strategies such as nitric oxide (NO) inhalation and extracorporeal membrane oxygenation (ECMO) in patients with CDH remains a major problem in pediatric critical care. We investigated the expression pattern of NO-generating enzyme nitric-oxide synthase (NOS) (both endothelial [eNOS] and inducible [iNOS] isoforms) in the lungs of CDH patients with PH and evaluated the influence of ECMO on the expression levels of these genes in an attempt to understand the underlying molecular mechanisms. Lung autopsy specimens from 23 cases of CDH not treated by ECMO and 10 ECMO-treated CDH cases were studied and compared with 11 age-matched controls. Expression of iNOS and eNOS was assessed by immunohistochemistry and video-image analysis. Expression of iNOS in the endothelium of small pulmonary arteries (external diameter < or =200 Mum) was significantly lower in CDH cases that had not received ECMO treatment (p = 0.04). ECMO-treated CDH cases did not differ from controls in iNOS expression. Alveolar macrophages (CD68+ cells), of which the number also was increased, showed significantly enhanced staining for iNOS in CDH cases (p = 0.03) compared with controls. The observed decrease in pulmonary expression of iNOS in patients with CDH suggests a potential role in the pathogenesis of pulmonary hypertension in newborns with CDH. ECMO treatment was correlated with induction of this enzyme, which may result in NO-mediated vasodilatation and thereby transiently reduce the pulmonary hypertension in CDH.
 ...
PMID:Pulmonary hypertension in human newborns with congenital diaphragmatic hernia is associated with decreased vascular expression of nitric-oxide synthase. 1645 43

Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50%. Ipsilateral pulmonary hypoplasia, increased pulmonary vascular resistance, and potential cardiac failure complicate early postnatal life. Surgical correction can either be performed on the first day of life or be deferred to a time after stabilization of the infant. Our patient presented with a left-sided Bochdalek's hernia containing large and small bowel. She required intubation and resuscitation on day 1 of life, and surgical repair had to be postponed. Further respiratory deterioration required commencement of inhaled nitric oxide and high-frequency ventilation. Pulmonary artery pressure rose to suprasystemic level. Closure of the ductus arteriosus on day 8 resulted in imminent right-sided heart failure. Commencement of alprostadil (prostaglandin E1) reopened the ductus and stabilized the patient. Surgical repair was successful 3 days later. Alprostadil should be considered as an important component of therapy in severe cases of congenital diaphragmatic hernia, where deterioration of right-sided heart function occurs.
 ...
PMID:Prevention of heart failure in the management of congenital diaphragmatic hernia by maintaining ductal patency. A case report. 1656 66

A case of right diaphragmatic hernia is presented associated with a complex congenital heart disease (double-outlet right ventricle, transposition of the great arteries and left isomerism) diagnosed prenatally. Despite high-frequency oscillatory ventilation plus nitric oxide and uneventful repair of the hernia, the infant died after 6 days of uncontrolled pulmonary hypertension and severe aortic coarctation that developed postnatally.
 ...
PMID:Right congenital diaphragmatic hernia associated with a complex heart disease. 1685 46

Extracorporeal membrane oxygenation (ECMO), a technique for providing life support to patients with cardiac and/or respiratory dysfunction, allows the heart and lungs to "rest." The neonatal respiratory population has been a major benefactor of ECMO since 1982. Its use for neonatal respiratory disease increased dramatically until the past few years, when the number of neonatal respiratory ECMO cases began a downward trend. Fewer patients with persistent pulmonary hypertension of the newborn (PPHN), meconium aspiration syndrome, respiratory distress syndrome, or sepsis are requiring ECMO support as frequently as in the past. Many attribute this decline to the newer respiratory therapies-mainly, surfactant, high-frequency oscillatory ventilation, and inhaled nitric oxide. Neonates who continue to require ECMO today are sicker than the historic norm and have more complicated and lengthy ECMO runs. Patients with congenital diaphragmatic hernia, PPHN, and sepsis remain the most consistent in their representation among ECMO recipients within this author's institution, suggesting that the newer respiratory therapies have not had the same impact on these patients' needs for ECMO support. Better guidelines for determining which patients would benefit from earlier inititation of ECMO are needed.
 ...
PMID:Neonatal ECMO: Current controversies and trends. 1691 34

Congenital diaphragmatic hernia (CDH) affects 1 in every 2,000 to 4,000 live births. Many infants with this condition are diagnosed antenatally through routine ultrasound screening. Nearly 90 percent present at delivery with severe respiratory distress requiring intubation. Many of these infants develop persistent pulmonary hypertension of the newborn due to hypoplasia of the affected lung. The survival of infants with CDH is limited by the degree of pulmonary hypoplasia and requires sophisticated medical technology such as high-frequency ventilation and inhaled nitric oxide. Some infants also require treatment with extracoporeal membrane oxygenation. This article gives details of two cases of CDH in which the presentation was atypical. The more subtle presentation is discussed, as well as the embryology and pathophysiology of CDH and the possibility of associated anomalies. Clinical management and impact on the family are outlined.
 ...
PMID:Congenital diaphragmatic hernia: two case studies with atypical presentations. 1691 35

A review of 100 consecutive cases of congenital diaphragmatic hernia (CDH) treated at our institute focusing on the efficacy of protocolized management (PM) was conducted. Of the 100 cases, 14 who became symptomatic more than 24 h after birth, and seven with fatal anomalies (four cardiac and three chromosomal) were excluded, leaving 79 subjects for this study. Of these, 41 were diagnosed prenatally (PD). Subjects were divided into four groups. Group I: No PD, no PM (n = 34), Group II: No PD, PM (n = 4), Group III: PD, no PM (n = 21), and Group IV: PD, PM (n = 20). PM includes criteria for planned delivery, use of high frequency oxygenation, nitric oxide, echocardiography (EC), and a medication schedule. Overall survival rates for Groups I, II, III, and IV were 73.5% (25/34), 75% (3/4), 38.1% (8/21), and 70.0% (14/20), respectively. Survival rates were higher when PM was used: 70.8% (Groups II, IV) versus 60.0% (Groups I, III). Survival rates were significantly lower if diagnosed prenatally (PD+): 53.7% (Groups III, IV) versus 73.7% (Groups I, II) (P < 0.01). However, in PD+ groups, survival was significantly higher if PM was used (P < 0.05). PM significantly reduced length of hospital stay (35.5 vs. 52.0 days: P < 0.05). EC was found to be a predictor for survival while post-ductal AaDO(2) was not. In 17 cases with cardiac anomalies, PM did not affect survival. Our study suggests that use of PM for prenatally diagnosed CDH cases is associated with improved outcome, although the components of PM need to be tested in prospective trials to determine their true value.
 ...
PMID:Efficacy of protocolized management for congenital diaphragmatic hernia. a review of 100 cases. 1696 80

Recent improvements in perinatal management have improved the prognosis in patients with severe congenital diaphragmatic hernia (CDH). However, in surviving patients with severe CDH, hearing loss has sometimes been reported to occur during the follow-up period. Although some of the risk factors for developing sensorineural hearing loss (SNHL) have been reported in CDH, no definitive risk factors have yet been reported. We, therefore, investigated the risk factors regarding postnatal management in patients with severe CDH. In 16 surviving patients with severe CDH, which had all been detected antenatally, and whose lung-to-thoracic ratio was less than 0.2, four patients demonstrated late onset SNHL, which occurred between 1.5 and 5 years of age. The risk factors for SNHL regarding the postnatal treatment for CDH were analyzed between the four patients with SNHL and the remaining 12 patients without SNHL, regarding such factors as the use of ototoxic drugs, neuromuscular blocking agents, high-frequency oscillation (HFO), and inhaled nitric oxide, the duration of hypocapnia, hypoxia, severe acidosis, severe alkalosis, and mechanical ventilation. In addition, the types of neuromuscular blocking agents were also analyzed, including the administration of pancuronium bromide (PB) and vecuronium bromide (VB). The patients with SNHL were found to have a significantly higher risk than the patients without SNHL regarding the duration of loop diuretics usage and the duration of usage of both mechanical ventilation and HFO. Furthermore, all four patients with SNHL used PB. In contrast, none of the five patients using VB developed SNHL The duration and cumulative dose of PB used in the patients with severe CDH showed a significant correlation to the occurrence of SNHL. Although this study was retrospective, based on our data, the prolonged use of PB, in addition to the duration of treatment by loop diuretics, mechanical ventilation, and HFO usage, might, thus, be suggested to be a possible risk factor for late onset SNHL in patients with severe CDH.
 ...
PMID:Risk factors for sensorineural hearing loss in survivors with severe congenital diaphragmatic hernia. 1704 41

Most infants with congenital diaphragmatic hernia (CDH) require respiratory support. The goal of this report is to present an overview of mechanical ventilation strategies in the management of infants with CDH. The anatomic and physiologic limitations in the lungs of infants with diaphragmatic hernia make decisions on the best strategy and use of mechanical ventilation challenging. We will briefly review lung development in infants with CDH, identifying factors that provide a basis for lung protection strategies. Background on the use of specific mechanical ventilation modes and the rationale for each are provided. Finally, we review mechanical ventilation practices described in published case series of successful CDH management, with a brief review of additional treatments, including inhaled nitric oxide and extracorporeal membrane oxygenation. Although details of a single specific best strategy for mechanical ventilation for CDH infants cannot be identified from current literature, a lung protection ventilation approach, regardless of the device used, appears to reduce mortality risk.
 ...
PMID:Mechanical ventilation strategies in the management of congenital diaphragmatic hernia. 1746 64


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>