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)

We have seen a modest improvement in the survival of a homogeneous group of critically ill newborns with congenital diaphragmatic hernia since 1979. Twenty-seven "critical" infants have been treated who developed respiratory distress shortly after birth, required urgent resuscitation, and could not be stabilized before operation. Two died with other anomalies that appeared incompatible with prolonged survival. Ten of the 27 lived. This survival contrasts with that of only two of 17 similarly affected babies treated from 1962 to 1978. In addition, there has been no operative mortality outside of this "critical" group since 1979; whereas six noncritical babies died between 1967 to 1978. Our current therapeutic plan includes the early establishment of a respiratory alkalosis and vasodilator therapy before or during transport. Postoperatively we have attempted to maintain the baby's arterial pH greater than 7.5, Pco2 less than 25 to 30 and the PO2 approximately 150 torr. The most effective ventilatory parameters have been a rate of 130, PEEP of 5 and an inspiratory:expiratory ratio of 1:1. Peak airway pressures are kept as low as possible. Pharmacologic and ventilator therapy are weaned slowly, and intensive support has been required for at least 48 hours in each baby. Retained secretions and atelectasis of the hypoplastic lung persisted for two to several weeks postoperatively. Two babies that are one year or older still appear to have severely hypoplastic lungs on chest x-ray. M-mode echocardiography has been used to measure ventricular ejection periods. The right ventricular systolic time interval correlates with the degree of pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The "critical" neonate with diaphragmatic hernia: a 21-year perspective. 648 79

Infants with congenital diaphragmatic hernia (CDH) show a wide range of anatomic and physiological abnormalities, making it difficult to compare the efficacy of management protocols between institutions. The purpose of this study was twofold: (1) to analyze the results of treatment of CDH in a large tertiary care pediatric center using conventional mechanical ventilation (CMV) with extracorporeal membrane oxygenation (ECMO) as rescue therapy, and (2) to compare these results with those of a parallel study by a similar large urban center that used high-frequency oscillating ventilation (HFOV) as rescue therapy without ECMO. All patients who had CDH diagnosed within the first 12 hours of life and were referred for treatment before repair (between 1981 and 1994) were included in the analysis (n = 196). CMV was used initially in all patients, with conversion to ECMO for refractory hypoxemia or hypercapnea. Between 1981 and 1984, ECMO was not available. Between 1984 and 1987, ECMO was offered postoperatively. Between 1987 and 1991, ECMO was offered preoperatively. In all three groups, aggressive hyperventilation and alkalosis was the norm. Since 1991, permissive hypercapnia has been used. HFOV was used in three patients as stand-alone therapy with one survivor. Twenty patients died without repair: Ten had other lethal anomalies, eight died before ECMO could be instituted, and two died of ECMO-related complications. Overall, 104 patients (53%) survived and 92 (47%) died. Ninety-eight patients (50%) received ECMO, and 43 (44%) survived. Survivors had significantly higher 1- and 5-minute Apgar scores and higher postductal Po2s than did nonsurvivors. Associated anomalies were present in 39%, who had a significantly lower survival than those with isolated CDH. Antenatal diagnosis and side of the defect had no impact on outcome. Survival was not improved with the institution of ECMO or delayed repair but rose significantly to 69% (84% with isolated CDH, P = .007) with the introduction of permissive hypercapnea. Autopsy results from nonsurvivors showed other lethal anomalies and significant barotrauma as the primary causes of death. Comparisons between the Boston and Toronto series showed similar patient demographics and no significant differences in survival in any time period. The two series differed in the number of associated anomalies, their impact on survival, and in the prognosis of right-sided CDH. From the individual and combined analyses the authors concluded: (1) CMV with ECMO as rescue produced an overall survival in CDH patients equivalent to CMV with HFOV in a parallel series, (2) neither HFOV nor ECMO has significantly improved outcome in CDH patients, (3) institution of permissive hypercapnia has resulted in a significant increase in survival, and (4) the leading causes of death in CDH patients appear to be associated anomalies and pulmonary hypoplasia, which are currently untreatable. Barotrauma, which may contribute in up to 25% of deaths in CDH patients is avoidable.
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PMID:Congenital diaphragmatic hernia--a tale of two cities: the Boston experience. 909 2

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.
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PMID:Risk factors for sensorineural hearing loss in survivors with severe congenital diaphragmatic hernia. 1704 41