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)

Eighteen newborn infants with severe hypoxia during the course of idiopathic respiratory distress syndrome, pneumonia, persistent fetal circulation or right diaphragmatic hernia were treated with chlorpromazine with the aim of improving arterial oxygenation by a postulated vasodilatory action on the pulmonary circulation. Fourteen of the infants improved their PaO2 during the treatment. Nine infants died. The systemic arterial blood-pressure and the urinary output were reduced and some infants were somnolent during the initial period of treatment. No other side effects were noted. Further studies of chlorpromazine as a possible pulmonary vasodilator in newborn infants are justified.
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PMID:The effect of chlorpromazine in severe hypoxia in newborn infants. 713 52

Pulmonary failure is the most frequent cause of mortality in newborns, accounting for 15,000 deaths yearly. It may be the result of the respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS), or persistent fetal circulation (PFC), including infants with congenital diaphragmatic hernia (CDH). Early identification of patients with predictably fatal but potentially reversible respiratory failure refractory to conventional management protocols would permit orderly application of extracorporeal membrane oxygenation (ECMO) as a final resuscitative measure. Eight neonates with severe pulmonary failure manifested by A-a DO2 of greater than 620 torr for greater than 12 hr, persistent cardiovascular instability, and relentless progression of acidosis and hypoxemia were predicted to have a 100% mortality in spite of maximal medical therapy. Four patients presented with MAS and 4 others had PFC, including 2 with CDH. All were supported with ECMO using the internal jugular vein and common carotid artery for access to the right atrium and aortic arch. Following support for 77-313 hr, 6 were successfully weaned from ECMO and then from the ventilator. In these few patients the use of extracorporeal membrane oxygenation after exhaustion of standard therapy was accomplished safely and successfully without untoward short-term sequelae. Extracorporeal ventilatory support may purchase the critical time necessary for resolution of the underlying parenchymal disease, including the pulmonary hypertension associated with CDH.
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PMID:Clinical use of an extracorporeal membrane oxygenator in neonatal pulmonary failure. 717 40

Four neonates (three having undergone repair of a congenital diaphragmatic hernia and developing a persistent fetal circulatory pattern and one having severe infant respiratory distress syndrome) have been supported with prolonged extracorporeal membrane oxygenation (ECMO) at Children's Hospital of Pittsburgh between December of 1979 and April of 1980. Three have survived. This encouraging experience indicates that the pattern of persistent fetal circulation in the newborn infant who has undergone repair of a diaphragmatic hernia can be successfully managed with ECMO even when efforts to lower pulmonary hypertension and improve oxygenation with vasodilators (tolazoline, phenothiazine, acetylcholine, or prostaglandin E1) and have been ineffective. The effectiveness and safety of ECMO is convincing enough to warrant its consideration as therapy for congenital diaphragmatic hernia and persistent fetal circulation prior to the use of vasodilators.
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PMID:Extracorporeal membrane oxygenation. Successful treatment of persistent fetal circulation following repair of congenital diaphragmatic hernia. 720 62

In an effort to find an accurate prediction of survival in congenital diaphragmatic hernia (CDH) patients with severe respiratory distress and persistent pulmonary hypertension, we reviewed the response to vasodilators and te clinical course of 13 patients with this condition. All patients had been operated upon within the first 24 hr of life and had postoperative descending aortic PaO2 values less than or equal to 50 torr on maximum ventilatory support. The only significant change in standard blood gas values after an intravenous test dose of 2 mg/kg of tolazoline was in the PaO2 (p less than 0.05). Patients with good response (PaO2 increase greater than 100 torr) survived. Patients with fair response (PaO2 increase between 20 and 100 torr) had equivocal survival. Patients with poor response (PaO2 increase less than 20 torr) died. There was a postoperative period up to several weeks of marked fluctuations in arterial oxygen tension in survivors before stability occurred. Vasodilator responsiveness appears to be an accurate, therapeutic indicator of the chances for survival in CDH patients with persistent pulmonary hypertension and right to left shunting.
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PMID:Vasodilator response and prediction of survival in congenital diaphragmatic hernia. 724 11

To determine whether vasodilators are useful in persistent pulmonary hypertension associated with congenital diaphragmatic hernia, we reviewed the clinical course, laboratory data, and outcome of 37 patients with respiratory distress and diaphragmatic hernia requiring an operation before 24 hours of life. These patients were divided into two groups, Group I (n = 17) included patients treated prior to the use of tolazoline; Group II (n = 20) included those treated after tolazoline became available. Postoperative severe respiratory distress was observed in ten patients in Group I, and all died. In Group II, 16 patients had severe postoperative respiratory distress and four survived; 12 of these 16 patients received tolazoline, including all four survivors. Treated survivors had significantly higher increase in Pao2 after a test dose of tolazoline than did nonsurvivors. A transient "honeymoon period" of adequate oxygenation correlated with good response to tolazoline, and the presence of both was predictive of survival. No patient survived with the combination of no "honeymoon period" and no response to tolazoline, whereas response to tolazoline without a honeymoon period was sometimes followed by survival. All nonsurvivors had severe lung hypoplasia at autopsy.
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PMID:Tolazoline therapy for persistent pulmonary hypertension after congenital diaphragmatic hernia repair. 744 32

Tracheal instillation of perfluorochemical liquid (PFC) lowers surface tension in the lung and thus might reduce barotrauma commonly associated with conventional gas ventilation (GV) in highly immature and hypoplastic lungs. It could be a promising alternative treatment for congenital diaphragmatic hernia (CDH) when GV alone is proving inefficient. The authors compared data for eight newborn lambs with surgically induced CDH. The animals had GV and were studied (in 2 groups) for up to 3.5 hours. Group 1 (GV, n = 4) had gas ventilation only. In group 2 (PFC, n = 4), after 30 minutes of GV, 10 to 12 mL/kg of warmed, oxygenated PFC liquid (LiquiVent) was instilled into the lung via the trachea under pressure-volume curve monitoring. Arterial pressure, blood chemistry, and pulmonary mechanics were evaluated serially; histological analysis was performed. One preassigned animal in group 1 died after 15 minutes. After 30 minutes of life, the cardiopulmonary profile of survivors was indicative of severe respiratory distress (Pao2 < 72 mm Hg with FIO2 at 1.0, PaCO2 > 90 mm Hg, compliance < 0.10 mL/cm H2O/kg) and not different between groups; the severity of pulmonary hypoplasia was further confirmed postmortem; the ratio of lung weight to body weight was 41% of that observed in control lambs, in both gas-only and combined gas/PFC-ventilated animals, compared with their respective controls. After instillation of PFC, there were dramatic improvements in acid-base status and pulmonary compliance in group 2. Survival at 3.5 hours also was markedly different (4 of 4 PFC animals and 1 of 3 GV animals).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Combined gas ventilation and perfluorochemical tracheal instillation as an alternative treatment for lethal congenital diaphragmatic hernia in lambs. 747 77

The experience with high-risk congenital diaphragmatic hernia (CDH) at an institution that does not offer extracorporeal membrane oxygenation (ECMO) was reviewed. Between January 1, 1983 and December 31, 1993, 38 children presented with Bochdalek-type CDH. Excluded were two infants with lethal cardiac anomalies and four presenting after 4 hours of age. Thus, the authors identified 32 high-risk patients. All had early respiratory distress and were intubated within 5 hours of birth. Sixteen were inborn; 16 were referred to the Izaak Walton Killam Children's Hospital (IWK) within 24 hours of birth. There were 19 males and 13 females. Three died before surgery could be attempted. Twenty-two survived, giving an overall survival rate of 69% (22 of 32). For 28 of the 32, the best preoperative postductal Pao2 (BPDPao2) was recorded. Fifteen of the 28 children had a BPDPao2 of greater than 100 mm Hg. Survival in this group was 14 of 15 (93%). Thirteen of the 28 patients had a BPDPao2 of less than 100 mm Hg. Survival in this group was 5 of 13 (38%). These survival rates are comparable to those of centers offering ECMO. BPDPao2 appears to be a useful discriminating variable.
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PMID:Survival in neonatal congenital hernia without extracorporeal membrane oxygenation support. 747 79

In acute hypoxemic respiratory failure of term and near-term neonates, extra- and intrapulmonary right-to-left shunting contribute to refractory hypoxemia. Inhaled nitric oxide (NO) decreases pulmonary arterial pressure and improves ventilation-perfusion mismatch in a variety of animal models and selected human patients. We report on 10 consecutive term and near-term newborns with severe acute hypoxemic respiratory failure due to diaphragmatic hernia, meconium aspiration syndrome, group B streptococcus sepsis, pneumonia or acute respiratory distress syndrome, who received increasing doses of inhaled NO (up to 80 ppm) to improve the arterial partial pressure of oxygen (PaO2). The response to NO and the optimum NO concentration which improved PaO2 varied considerably between patients. Improvement of PaO2 was absent or poor (less than 10 mm Hg) in the 4 newborns with meconium aspiration syndrome and in 1 patient with congenital diaphragmatic hernia, while in the other 5 patients inhaled NO increased the mean (+/- SE) PaO2 from 41 +/- 6 to 57 +/- 9 mm Hg (P < 0.05). Optimum NO concentrations determined by dose-response measurements performed during the first 8 hr of NO inhalation were 8-16 ppm except for 2 newborns with congenital diaphragmatic hernia who required 32 ppm to effectively increase PaO2. Four of the 5 patients in whom the PaO2 rose by more than 10 mm Hg received inhaled NO for extended periods of time (5 to 23 days) with no signs of tachyphylaxis. The optimum NO concentration dropped to less than 3 ppm after prolonged mechanical ventilation or when intravenous prostacyclin was given concomitantly.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Dose-response to inhaled nitric oxide in acute hypoxemic respiratory failure of newborn infants: a preliminary report. 756 4

Diaphragmatic hernias of the Morgagni type are generally thought to be asymptomatic in adults. This traditional assumption led to a delay in diagnosing a Morgagni hernia as the cause of acute respiratory distress in a chronic schizophrenic man. While Morgagni hernias are usually considered to be long-standing, we present radiological evidence of an acutely expanding hernia. The patient's symptoms were relieved by surgical repair of the hernia. We advise caution before dismissing Morgagni hernias in adults as being long-standing and clinically insignificant.
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PMID:Acute respiratory distress secondary to Morgagni diaphragmatic herniation in an adult. 770 92

Abnormal development of the lung in congenital diaphragmatic hernia (CDH) results in a dysfunctional surfactant system. In premature newborns at risk for respiratory distress syndrome, amniotic fluid lecithin/sphingomyelin (L/S) ratios and phosphatidylglycerol (PG) status have been successfully used to predict the surfactant status in the fetus. The objective of this study was to assess the accuracy of L/S ratios and PG in predicting the surfactant status in CDH. The surgically created lamb CDH model was used. Animals were delivered at 140 days' gestation (term 145) and immediately killed. Before delivery amniotic fluid was collected and L/S ratios and PG status were measured. Bronchoalveolar lavage (BAL) was performed and analyzed for total phospholipid and percent phosphatidylcholine (PC). Analysis of the BAL showed that the CDH lungs had both significantly less total phospholipid (CDH 0.10 +/- 0.03 mg/g versus control (CON) 0.76 +/- 0.28 mg/g) and PC (CDH 38 +/- 7.3% versus CON 70 +/- 3.4%) when compared with controls. In contrast the L/S ratios (CDH 2.44 +/- 0.26 versus CON 2.01 +/- 0.32) and PG status (CDH 8.75 +/- 1.01 nmol versus CON 10.2 +/- 0.9) were the same in CDH and control animals. The BAL from the CDH lamb model has a significant surfactant deficiency. Amniotic fluid L/S ratios and PG status were, however, not different between the control and CDH lambs. These results indicate that amniotic fluid L/S ratios and PG do not accurately predict the surfactant status of a fetus with CDH.
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PMID:Pathophysiology of congenital diaphragmatic hernia. XII: Amniotic fluid lecithin/sphingomyelin ratio and phosphatidylglycerol concentrations do not predict surfactant status in congenital diaphragmatic hernia. 776 Feb 31


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