Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The rate of extracardiac malformation (ECM) associated with congenital heart disease (CHD) is high in neonates. 108 cases of 212 neonates with CHD had ECM (50.9%). Main ECMs were digestive system anomalies (36.1%), chromosomal aberrations (26.8%), respiratory system anomalies (21.3%), CNS anomalies (13.0%), and other (2.7%). Single lesion of left to right shunt accounted for 77.2% (61/79) of CHD with ECM from other than chromosomal aberrations. The prognosis of neonates with CHD without ECM was also studied. Total anomalous pulmonary venous connection, pure pulmonary atresia (PPA), pulmonary stenosis (PS), hypoplastic left heart syndrome are not generally associated with ECM, but the prognosis is poor. Coarctation complex which is sometimes associated with ECM has a poor prognosis in neonates. There is an increase of the survival rate in the patients with hypoxemia, such as PPA or severe PS, extreme tetralogy of Fallot, and tricuspid atresia, which can be managed with prostaglandin E1. PDA associated with respiratory distress syndrome is ideally treated with indomethacin. In recent years, mortality from PDA has decreased in neonates. The mortality rate during the neonatal period was 46.8% (51/109): 37.5% (30/80) died before surgical interventions and 72.4% (21/29) died during or after surgery. Half of neonatal deaths from CHD occurred within 3 days of admission. Thus, early detection, early diagnosis, and early treatment of neonates with CHD is most important.
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PMID:Case histories of neonates with congenital heart disease. 723 May

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.
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PMID:The value of cardiac catheterization and cineangiography in infantile lobar emphysema. 745 24

We studied serum prolactin (PRL) in 28 newborn infants with acute encephalopathy. Six patients had electrographically confirmed seizures. Twenty-two patients comprised the nonictal group. In the seizure group, PRL was determined at the first onset of the seizure (baseline) and at 15 and 30 min postictal. In the nonseizure group, PRL was determined at the end of the EEG and 15 min later. EEGs were visually analyzed for the presence of seizures and background abnormality (normal or mildly, moderately, or markedly abnormal). Etiologic diagnoses included congenital heart disease (12), hypoxic-ischemic encephalopathy (4), sepsis (4), respiratory distress syndrome (5) meconium aspiration (1), and metabolic disease (2). Serum PRL was significantly higher (p < 0.05) at baseline and 15 min postictally in the patients with seizures than in the nonictal group. However, PRL levels 15 and 30 min postictally were not statistically different from baseline values. Baseline PRL correlated significantly (p < 0.001) with EEG background abnormality in both groups; therefore, patients with the most abnormal EEG backgrounds had higher levels of PRL than those with a relatively normal EEG background. We conclude that newborns with EEG-confirmed seizures, particularly if seizures are not associated with clinical signs, have high baseline serum PRL levels that do not increase significantly in the immediate postictal period. Serum PRL levels correlate with the severity of the brain insult as evaluated by EEG background. Further studies are needed to enhance our understanding of the dynamics of PRL secretion in newborns with seizures and acute encephalopathy.
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PMID:Serum prolactin in neonates with seizures. 755 85

We reviewed 11 pediatric cases of diaphragmatic paralysis related to nonspinal-cord injury which were managed in our Intensive Care Unit over the past 10 years. Three cases were secondary to birth trauma, 7 followed surgical procedures for congenital heart disease, and 1 occurred in association with injuries sustained in a motor vehicle accident. The paralysis was bilateral in 8 children. The diagnosis was initially suspected on clinical grounds because of respiratory distress, impossibility of weaning from the ventilator, and paradoxical abdominal respiratory movements. Confirmatory investigations included chest radiography, which revealed elevation of the affected hemidiaphragm, fluoroscopy and ultrasound, both of which demonstrated diminished diaphragmatic movement. Electromyography exhibited a failure of diaphragmatic response to phrenic nerve stimulation in 8 patients. All patients were mechanically ventilated; tracheostomy was required in 5 patients. Physiotherapy was considered a beneficial adjuvant measure. Diaphragmatic plication was attempted without success in 3 children. Seven children recovered without sequelae: Partial respiratory autonomy was achieved after an average of 2.6 months, complete autonomy after an average of 5.4 months. Two patients developed chronic lung disease; one of them remains unresponsive, and one child died following accidental extubation. We conclude that the diagnosis of diaphragmatic paralysis is predominantly clinical, and that the outcome of patients treated by adequate endotracheal mechanical ventilation is usually favorable.
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PMID:Diaphragmatic paralysis in children: a review of 11 cases. 780 Apr 36

We studied the effects of pulmonary congestion on the development of atopic asthma in 31 infants with congenital heart disease. Respiratory distress did not resolve after surgery in seven patients, six of whom had a family history of allergy. Pulmonary congestion in infancy may increase the risk of atopic asthma in genetically predisposed children.
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PMID:Development of atopic asthma in infants with pulmonary congestion caused by congenital heart disease. 815 76

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.
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PMID:Pediatric lung transplantation. Indications, techniques, and early results. 815 51

The purpose of the present study is to compare risk factors of necrotizing enterocolitis (NEC) between two group: group A, newborns with the disease and group B, newborns with other diseases different from NEC, in order to know if these risk factors are more frequent or not in the first group. We assessed the clinical records of all the patients hospitalized in the Neonatal Intensive Care Unit and Neonatology Service of the La Raza General Hospital between 1987 and 1991 with the diagnosis of NEC. They were compared with 65 clinical records chosen at random of patients hospitalized in the same Unit with other diagnosis at the same time, and who were discharged by improvement or deceased. In all of them were look for known risk factors for NEC generally accepted such as: prematurity, neonatal asphyxia, poliglobulia, cyanotic congenital heart disease, patent ductus arteriosus, respiratory distress syndrome, catheterization of umbilical vessels, early feeding of elevated formula increases, exchange exchange transfusion, hypoxic ischemic encephalopathy, infection, etc. Just 25 records of the possible 50 with the diagnosis of NEC full filled inclusion criteria. There were no statistically significant difference in weight, sex, mortality and known risk factors of NEC between both groups. Were concluded that NEC is a disease of unknown etiology that should be studied more thoroughly. The known risk factors must be avoided because the patient susceptibility probably play an important role.
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PMID:[Risk factors of necrotizing enterocolitis]. 837 46

Candida is an increasing problem as a causal agent of nosocomial infection in neonates and infants. We report 15 cases of infective endocarditis caused by Candida spp treated at the Hospital Infantil de Mexico between 1980 and 1991. The diagnosis of endocarditis was established by the identification of Candida in at least two blood cultures and echocardiographic assessment. From 110 cases of systemic candidiasis during the eleven years period of study, fifteen patients presented endocarditis (13.6%), all had a central venous catheter. Three had antecedent of congenital heart disease. Candida isolation was obtained an average of 28 days after admission. The major clinical findings were: fever in 13 patients, respiratory distress and cardiac murmurs observed in nine respectively. Thrombocytopenia was present in eight children. The echocardiographic evaluation showed vegetations located in the superior vena cava in six, right atrium in five, tricuspid valve in two, inferior vena cava and right ventricle in one respectively. Three cases were subjected to surgical treatment. Nine patients died for a case fatality rate of 60%. The echocardiographic evaluation practiced in all patients with suspicion of systemic candidiasis is critical for the prognosis. The identification of endocardiac involvement coupled with the opportune administration of antifungal therapy and surgical treatment may improved the survival.
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PMID:[Candida endocarditis in the first year of life]. 844 79

Post-mortem hypoxanthine concentrations in the vitreous humor of human infants were investigated. Hypoxanthine is formed from hypoxic degradation of adenosine monophosphate. The concentrations in the vitreous humor can give information about antemortem hypoxia. The post-mortem levels were corrected for the time elapsing between death and the autopsy. Four groups of infants were compared: 17 babies who died of respiratory distress syndrome (RDS), 72 infants who died of sudden infant death syndrome (SIDS), 23 children dying of congenital heart disease (both cyanotic and acyanotic), and 15 children dying acutely in accidents without any known significant time of hypoxia before death. The corrected, median hypoxanthine levels in victims of SIDS (200 mumol/L) was significantly higher (p < 0.01) than in the accident group (0 mumol/L), but no clear difference was found between the SIDS group and the RDS group (101 mumol/L), or the heart group (54 mumol/L). A number of children with "normal" hypoxanthine levels (0 to 38 mumol/L) were found in all four groups, but the numbers were significantly lower (p < 0.005) in the RDS, SIDS and heart groups than in the accident group. It is concluded that SIDS is probably not a sudden event, but may be preceded by relatively long, or repeated intermittent periods of hypoxia (of unknown etiology).
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PMID:Post-mortem concentrations of hypoxanthine in the vitreous humor--a comparison between babies with severe respiratory failure, congenital abnormalities of the heart, and victims of sudden infant death syndrome. 851 58

Underlying diseases, complications, clinical findings, and laboratory findings were evaluated in 158 cases of septicaemia admitted to Jikei University Hospital from 1975 to 1994, in order to conjectured factors that prescribe for the prognosis. 50% of the patients had underlying diseases. Malignancy including leukaemia (31 cases, 39.2%) was the most common underlying disease, followed by low birth weight infant (17 cases, 21.5%), aplastic anemia (9 case, 11.4%), and congenital heart disease (7 cases, 8.9%). The death rate for patients with underlying disease (27.8%) was significantly greater than the mortality for normal patients with septicaemia (8.9%) (p < 0.05). Meningitis (24.7%) was the most common complication, followed by DIC (19.6%), shock (15.2%), and pneumonia (10.8%). The mortality rate of septicaemia complicated by shock was 66.7% (p < 0.01), and that complicated by DIC was 45.2% (p < 0.01). The mortality rate for patients with the clinical findings of respiratory distress, cough, abdominal distention, cyanosis, splenomegaly, or peripheral coldness was more than 40% and significantly greater (p < 0.01). Mortality rate in patients with granulocyte counts of < 4.000/mm3, platelet counts of < 5 x 10(4)/ mm3, total protein of < 5.0 g/dl, or ESR of < 20 mm/hr were significantly greater (p < 0.01) than those in patients with normal laboratory findings. Coincidence rate of blood and stool cultures was 57.9% for E. coli, and 28.6% for Klebsiella sp., and that of blood and throat cultures was more than 30% for Pseudomonas sp., Haemophilus influenzae, and Staphylococcus aureus. In the study of antimicrobial susceptibility for microorganisms isolated, the number of drug resistant S. aureus had increased in the last 10 years.
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PMID:[Study on septicaemia in infants and children in the past 20 years. Part 2. An analysis of factors that prescribe for the prognosis]. 889 May 45


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