Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0019270 (hernia)
15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective review we analysed alveolar-arterial oxygen difference (AaDO2) as an entry criterion for extracorporeal membrane oxygenation (ECMO) in neonates with several forms of acute respiratory insufficiency. Although for meconium aspiration syndrome, respiratory distress syndrome, sepsis, and idiopathic pulmonary hypertension of the newborn we found values in accordance with the literature, patients with congenital diaphragmatic hernia (CDH) met 80% mortality criteria with significant lower AaDO2 values. Several patients died before ever reaching usual entry criteria for ECMO, because serious lung deterioration makes AaDO2 values unreliable. Awaiting classical ECMO entry criteria for patients with CDH may at least partially explain the lower survival rate for ECMO in CDH.
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PMID:Do we use the right entry criteria for extracorporeal membrane oxygenation in congenital diaphragmatic hernia? 822 83

The aim of this study was to assess the impact of surfactant deficiency on the pathophysiology of congenital diaphragmatic hernia (CDH). Pregnant ewes were operated on at 80 days of gestation for creation of a diaphragmatic hernia in the lambs. Twenty-one lambs survived to be delivered by cesarean section and were studied. Compliance was improved when surface tension effects were removed by saline solution in lungs of both control animals and lambs with CDH; however, the lungs of the lambs with CDH still had significantly impaired compliance. In a second series of experiments, two groups were studied: a surfactant-treated and a control, nontreated group. Surfactant was given prophylactically into the liquid-filled lungs before the first breath. All lambs were paralyzed and sedated and their lungs mechanically ventilated with 100% oxygen for 30 minutes; gas exchange was then assessed, pressure-volume data were obtained, and compliance was calculated. Surfactant significantly improved gas exchange; arterial oxygen pressure increased from 39 +/- 11.4 to 316 +/- 53.6 mm Hg, arterial carbon dioxide pressure decreased from 148 to 63 mm Hg, and pH increased from 6.87 to 7.16 (p < 0.001). Lung volume at 25 cm H2O, functional residual capacity, and compliance were all increased (p < 0.02). Thus, in the CDH lamb model, pulmonary mechanics are impaired by both parenchymal and surfactant abnormalities. Both lung mechanics and gas exchange are markedly improved by exogenous surfactant therapy.
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PMID:Pathophysiology of congenital diaphragmatic hernia. V. Effect of exogenous surfactant therapy on gas exchange and lung mechanics in the lamb congenital diaphragmatic hernia model. 830 41

To determine the role of inhaled nitric oxide (NO) in a population of critically ill hypoxic near-term infants and to determine the dose response to inhaled NO, we examined a consecutive group of 23 infants referred for neonatal extracorporeal membrane oxygenation (ECMO) who had an oxygen index of 20 or greater after treatment with bovine surfactant. Inhaled NO was administered in concentrations from 5 to 80 ppm in random order to 23 infants. Overall, 13 infants had a significant response (an improvement in arterial oxygen pressure > 10 mm Hg or arterial oxygen saturation > 10%) to the first administration of inhaled NO, and one infant had a late response. There was no significant difference in the response to inhaled NO as measured by changes in arterial oxygen pressure or in the alveolar-arterial difference in partial pressure of oxygen, for any of the doses from 5 to 80 ppm. Thirteen infants had echocardiographic evidence of persisted pulmonary hypertension; 11 of these infants responded, compared with 3 responders among the 10 infants without persistent pulmonary hypertension of the newborn (p < 0.01). Overall, 11 infants required ECMO; there were two deaths in this group. Seven infants had congenital diaphragmatic hernia; five of those had a response to NO inhalation and four required ECMO. Our study demonstrates that there is no significant difference in response between low and high doses of inhaled NO and that this treatment may prevent the need for ECMO in some infants referred for this therapy, especially in infants with pulmonary hypertension. Prospective, controlled, randomized, and blinded trials of low doses of inhaled NO are needed to determine the clinical role of this potentially useful therapy.
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PMID:Inhaled nitric oxide in infants referred for extracorporeal membrane oxygenation: dose response. 830 43

We studied the efficacy of low-dose nitric oxide inhalation in nine consecutive patients with severe persistent pulmonary hypertension of the newborn (PPHN) who were candidates for extracorporeal membrane oxygenation (ECMO). All patients had marked hypoxemia despite aggressive ventilator management and echocardiographic evidence of pulmonary hypertension. Associated diagnoses included meconium aspiration syndrome (3 patients), sepsis (3 patients), and congenital diaphragmatic hernia (2 patients). Infants were initially treated with inhaled nitric oxide at 20 ppm for 4 hours and then at 6 ppm for 20 hours. In all infants, oxygenation promptly improved (arterial/alveolar oxygen ratio, 0.077 +/- 0.016 at baseline vs 0.193 +/- 0.030 at 4 hours; p < 0.001) without a decrease in systemic blood pressure. Sustained improvement in oxygenation was achieved in eight patients treated with inhaled nitric oxide for 24 hours at 6 ppm (arterial/alveolar oxygen ratio, 0.270 +/- 0.053 at 24 hours; p < 0.001 vs baseline). One patient with overwhelming sepsis had an initial improvement of oxygenation with nitric oxide but required ECMO for multiorgan and cardiac dysfunction. We conclude that low doses of nitric oxide cause sustained clinical improvement in severe PPHN and may reduce the need for ECMO. However, immediate availability of ECMO is important in selected cases of PPHN complicated by severe systemic hemodynamic collapse.
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PMID:Clinical responses to prolonged treatment of persistent pulmonary hypertension of the newborn with low doses of inhaled nitric oxide. 832 Jun 29

Congenital diaphragmatic hernia is a congenital malformation associated with pulmonary hypoplasia. It often leads to respiratory failure, requiring artificial ventilation with high inflation pressures and high percentages of oxygen. We evaluated radiographic evidence of bronchopulmonary dysplasia (BPD) in survivors, who presented with respiratory distress within 6 hours after birth, by a radiographic scoring system measuring the severity of BPD by the Toce score and the degree of pulmonary hypoplasia by the Touloukian score. Fifteen of 45 survivors (33 percent) had clinical and radiological lung disease resembling BPD. As a group they had significantly higher Touloukian and Toce scores than survivors without BPD. Morbidity expressed as the duration of artificial ventilation, supplemental oxygen, and hospital stay was much higher in the BPD group. The hypoplastic lung in infants with congenital diaphragmatic hernia appears to be as susceptible to barotrauma and pulmonary oxygen toxicity as the lungs of prematurely born infants. To what extent BPD occurring in congenital diaphragmatic hernia survivors might influence the future development of lung function is not yet known.
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PMID:Radiographic evidence of bronchopulmonary dysplasia in high-risk congenital diaphragmatic hernia survivors. 846 76

As more infants with congenital diaphragmatic hernia (CDH) survive with extracorporeal membrane oxygenation (ECMO), it seems prudent to detail the longterm outcome in these medically complex infants. Eighteen children with CDH-treated with postoperative ECMO were recruited for participation in this study. The mean duration of ECMO was 193 hours (range 82 to 493 hours), mean time to extubation after ECMO was 142 hours (range 34 to 312 hours), and median duration of hospitalization was 46 days (range 30 to 181 days). Of the 18 infants, 4 (22%) were discharged home requiring oxygen therapy. At follow-up the notable findings were a high incidence of gastroesophageal reflux and failure to thrive. At both 1 and 2 years of age, 50% of infants were at less than the 5th percentile for weight. At 1 and 2 years of age, 39% and 21%, respectively, were at less than the 5th percentile for weight/length ratio. A total of 16 children (89%) had clinical evidence of reflux, and 8 (44%) were discharged home on a regimen of nasogastric feedings. Reherniation occurred in 4 children (22%) and was more frequent when a patch was used. An electrocardiogram showed right ventricular hypertrophy in 6 (43%); oxygen saturation by pulse oximetry was > 95% in all children, and pulmonary artery pressure was estimated by Doppler echocardiography to be normal in 12 of 14 children examined. The neurodevelopmental outcome (Bayley Scales or Stanford-Binet scale) at 1 to 4 years of age was not dissimilar from that of other ECMO-treated children. Given the severity of illness in the neonatal period, the general health and development of children with CDH surviving after ECMO are good. Surprisingly few children have long-term respiratory complications related to pulmonary hypoplasia. Follow-up in the first few years should be aimed at aggressive nutritional intervention to prevent the growth failure that appears to be prevalent in these children.
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PMID:Congenital diaphragmatic hernia: long-term outcome in neonates treated with extracorporeal membrane oxygenation. 850 65

Most cases of acute lung injury in pregnancy are attributed to hydrostatic pulmonary edema. In this report, however, we describe a 20-year-old pregnant woman who developed a unique case of increased permeability pulmonary edema following surgery for the repair of a fetal congenital diaphragmatic hernia. Two days after surgery, the patient developed acute respiratory failure and diffuse alveolar edema, requiring intubation and positive pressure ventilation for 5 days. The diagnosis of increased permeability pulmonary edema was confirmed by the ratio of pulmonary edema fluid to plasma protein (ratio=0.99). The patient received IV nitroglycerine for tocolysis. As a nitric oxide donor, the nitroglycerine may have combined with exogenous oxygen to form peroxynitrite, a known impediment to alveolar epithelial cell function. Many cases of pulmonary edema in pregnancy are diagnosed as hydrostatic based on clinical parameters, such as positive maternal fluid balance. In this case, these parameters would have been misleading. Measurement of the protein concentration in the pulmonary edema fluid allowed us to accurately determine that the patient had increased permeability pulmonary edema as the cause of her acute respiratory failure. Sampling of pulmonary fluid can differentiate the type of edema formation and in some cases help to identify mechanisms of acute lung injury.
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PMID:Pulmonary edema in a woman following fetal surgery. 863 43

The widespread use of newly developed techniques including extracorporeal membrane oxygenation (ECMO) has led to the survival of a number of patients with congenital diaphragmatic hernia (CDH) and associated hypoplastic lung. However, it is not fully recognized whether the hypoplastic and small lung of the affected side has the ability to develop its function after repair of CDH. The authors studied the lung function of 32 patients with CDH in whom these new methods were used. Two parameters, lung volume and pulmonary perfusion amount, were used to evaluate lung function. The former (checked by computed tomography scan) was used to evaluate the size of lung; the latter (checked by perfusion scintigram) was used to assess vascular density. The patients were divided into two groups, based on values of alveolar-arterial difference in oxygen content (AaDo2) at the time of admission. In group A (AaDo2 < 500 mm Hg; 12 cases), whose respiratory distress was mild and could be managed with ventilator care alone, the mean lung volume value for the affected side was 86% of the contralateral lung value from the initial study, and reached 93% at the time of follow-up study. The perfusion amount also exceeded 80% of the contralateral lung value from the initial study. Thus, it is likely that group A's affected-side lung is not small and has developed at a rate similar to that of the contralateral lung. However, in group B patients (AaDo2 > 500 mm Hg; 20 cases), who had severe respiratory distress at the admission and were managed with new techniques including ECMO, both lung volume and perfusion amount of the affected side initially were low in all cases (ie, mean values were 61% and 53% of contralateral-lung values, respectively). At the time of follow-up, the lung volume had increased in most cases (mean value, 88% of the contralateral lung value), but the perfusion amount of the affected side had not increased in most cases. It remained low, or decreased to below the initial value; the mean was 53% of the contralateral lung value. The initial mean perfusion: volume ratio (87%) had decreased significantly (to 62%) by the time of follow-up. This tendency was exaggerated in the 11 ECMO cases. These data might indicate that in most group B cases, the lung of the affected side has little ability to develop arterial branches, or certainly will be delayed in comparison to the contralateral lung, and that enlargement of lung volume may depend on overexpansion or emphysematous change rather than cellular growth. The present data also suggest that, in group B cases, total lung function will depend on the contralateral lung for a relatively long time.
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PMID:Development in lung function of the affected side after repair of congenital diaphragmatic hernia. 870 2

Congenital diaphragmatic hernia is associated with significant mortality and morbidity. The aim of this study was to compare a series of tests with respect to prediction of outcome. Tidal volume and compliance of the respiratory system (CRS) were measured preoperatively and on the first and second postoperative days. The maximum and modified ventilation indexes and the maximum Paco2 were noted for the first 6 hours of life and the first 6 hours postoperatively. In addition, it was recorded whether the stomach was within the ipsilateral hemithorax preoperatively. Twenty infants were studied (median gestational age, 38 weeks; range, 31 to 40), six of whom had a poor outcome, ie, they died or remained oxygen-dependent after 28 days. A CRS of less than 0.18 mL/cm H2O/kg was the most accurate predictor of poor outcome, with 66% sensitivity and 100% specificity. The authors conclude that lung function measurement are useful in the assessment of infants with congenital diaphragmatic hernia.
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PMID:Prognostic indicators in congenital diaphragmatic hernia. 874 26

Despite inguinal hernia being both common and problematic in a significant proportion of preterm infants with bronchopulmonary dysplasia (BPD), there has been a reluctance to intervene surgically for fear of exacerbating the underlying lung disease. We report our experience of early operation in 12 consecutive infants with varying degrees of oxygen-dependent BPD and investigate the effect of general anaesthesia and herniotomy on pulmonary function by measuring oxygen requirements prior to and following operation. Two infants who required oxygen in a concentration in excess of 95% failed to improve and died from the pulmonary disease 6 and 8 weeks following their operation. The remaining infants all showed a reduction in mean oxygen requirements in the weeks following operation. We conclude that, in the short term, hernia repair performed under general anaesthesia in infants with BPD of varying severity had no adverse effects on respiratory function, as determined by oxygen requirements. We suggest that in certain infants early repair may have been beneficial--potential mechanisms are explored.
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PMID:Early repair of inguinal hernia in preterm infants with oxygen-dependent bronchopulmonary dysplasia. 883 87


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