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

Seven out of 829 pediatric cardiac patients (0.84%) were found to have sinus node dysfunction (SND) over the past seven years. Of the seven patients, three had structurally normal hearts. One of these three patients had long QT syndrome. In four patients, structural heart disease was noted. In three of these four patients the sinus node dysfunction was attributed to cardiac surgery. The age of onset of SND ranged from four months to eight years. Presenting symptoms and signs included syncope, near-syncope, seizure and congestive heart failure. Two patients were asymptomatic. Five patients had episodic sinus pause. Sinus or junctional bradycardia was noted in four patients. Three had tachy-bradycardia. High grade atrioventricular block was noted in one patient. Treadmill exercise test revealed a nonsustained ventricular tachycardia in two patients. All seven patients were found to have prolonged maximal corrected sinus node recovery time. Prolonged intra-atrial conduction time was found in three, prolonged AV nodal conduction time in one, and prolonged His-Purkinje conduction time in one patient during the electrophysiologic study. All seven patients showed abnormal results in intrinsic heart rate study. Anti-arrhythmic drugs were prescribed. During the follow-up study, no patient died, but two patients received a pacemaker implantation. Because of the extent of their conduction system diseases, it is recommended that patients with SND should be thoroughly investigated.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Sinus node dysfunction in children. 151 8

A case of trisomy 22 liveborn female baby with multiple congenital anomalies is described. Physical manifestations included failure to thrive, hypotonia, pre-auricular sinus, low set ears, hypertelorism, posterior low hair line, micrognathia, cleft palate, congenital heart disease, imperforated anus with anovulvar fistula, contracted pelvis and bilateral rocker-bottom feet. The infant died at two months of age. Cases of trisomy 22 usually present with many severe malformations, and they rarely survive to term. A review of the literature is presented to delineate this chromosome disorder.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Liveborn trisomy 22: report of one case. 151 17

Nineteen children with clinical diagnoses of renal tubular acidosis were followed for periods ranging from 3 months to 20 years. Twelve patients had Type 1 renal tubular acidosis, five had Type 2, and two had Type 4. No sex predilection was found for any one of the types. Most patients had been diagnosed before 18 months of age, with failure to thrive the most common presentation. Tachypnea, polydipsia, polyuria, and vomiting were frequent symptoms. Some of these children had associated renal hypoplasia, vesicoureteral reflux, unilateral renal agenesis, glomerulocystic disease, adult polycystic kidney disease, and cyanotic congenital heart disease. Urinary anion gap may be useful for differential diagnosis of altered distal urinary acidification from other hyperchloremic metabolic acidosis. Furosemide test may need further investigation. Inability to raise urine to blood pCO2 gradient is helpful for diagnosis of Type 1 renal tubular acidosis. Hypokalemia, hypocalcemia, hypophosphatemia, decreased tubular reabsorption of phosphate, and hypercalciuria occurred in some patients. Complications included rickets in two, nephrocalcinosis in one, and episodic hematuria in one. There was relative bicarbonate wasting in children with Type 1 renal tubular acidosis, with a mean therapeutic bicarbonate requirement of 4.4 +/- 2.6 meq/kg/day. The mean bicarbonate dose for patients with Type 2 renal tubular acidosis was 8.3 +/- 2.6 meq/kg/day. Most children had good response to treatment with complete catch-up linear growth in 13, improved growth in 4, and continuing poor growth in 2. Two patients died during follow-up. Two other patients maintained normal growth without medication.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Renal tubular acidosis in childhood. 226 80

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31

A child with congestive heart failure was found to have a paroxysmal ventricular tachycardia (VT) without structural heart disease. This wide QRS tachycardia had an atrioventricular dissociation and a QRS pattern of right bundle branch block and left axis deviation. The tachycardia did not respond to lidocaine or procainamide but instantly responded to verapamil therapy. This VT could be initiated or terminated by either atrial or ventricular pacing during electrophysiologic study. The endocardial biopsy showed that there was myocardial hypertrophy with mild degree of interstitial fibrosis. This entity of ventricular tachycardia often occurs in young people with no overt heart disease but with subclinical endomyocardial changes.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:An electrophysiologic study of a child with idiopathic sustained left ventricular tachycardia. 228 43

In order to investigate the morbidity and mortality in individuals with Down syndrome(DS), we reviewed 237 cases of DS visiting our hospital in a ten-year period. There were 150 males and 87 females with age range from newborn to 25 years. The major illnesses included: 1) congenital heart disease (CHD), 42.6%; with endocardial cushion defect, ventricular septal defect, atrial septal defect and patent ductus arteriosus as the common types; 2) frequent or major respiratory tract infections, 34.2%; 3) summer fever, 5.5%; 4) major infective episodes other than pneumonia, 5.0%. Other diseases such as seizure disorder, gastrointestinal tract anomalies and thyroid dysfunction were also noted. There were 28 deaths of them and the causes of mortality were 1) CHD with congestive heart failure (CHD with CHF) and pneumonia (46.9%); 2) CHD with CHF and sepsis (10.7%); 3) CHD with CHF (14.3%) and 4) acute leukemia with infection (10.7%) respectively. Most of the mortality occurred during the first year of life, especially in patients with CHD. In our series, the one year survival rate was 93.6%; the result indicates that life expectancy in DS is much better than generally believed.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Mortality and survival in Down syndrome in Taiwan. 253 73

Activation of the complement system by iodinated contrast medium (ICM) infusion was studied in 25 children with congenital heart disease who received 76% Urographin during angiocardiographic studies. Total hemolytic activity (CH50), C1q, C3, C4 and C3 activator were determined before and 2, 5, 10, 15 and 20 minutes after Urographin infusion. Results showed that CH50 decreased in 16 patients (64%), C3 in 12 patients (48%), C3 activator in 12 patients (48%), C1q in 11 patients (44%) and C4 in 11 patients (44%). No side effect was encountered in all patients. It is therefore concluded that both the classic and alternative complement pathways could be activated by ICM and no causal relationship could be found between the presence of complement activation and the occurrence of adverse reactions to ICM infusion.
Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi 1980 Jun
PMID:Complement activation by iodinated contrast media. 744 28

Forty-six patients with two or more features of the VATER association admitted to the Mackay Memorial Hospital from May, 1983 to Mar, 1992 were retrospectively enrolled in this study. We compared the incidence of major features with that reported in the literature. Imperforate anus, congenital heart disease, and renal anomalies were the three most common major features in our study. Thirteen patients died. Heart failure was the major cause of mortality. We noted many patients associated minor features, and among them there was a relatively high ratio of cleft lip, cleft palate, and hypospadius. The overall outcome and development were good among the survivals. We suggest that children who have any congenital anomaly included in the VATER association should get a careful examination and evaluation of their heart, genitourinary tract, limbs and vertebrae. Early correction if possible is indicated in such patients.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:The VATER association: analysis of forty six cases without karyotyping. 777 43

To evaluate the relationship between concentrations of ANP in plasma of the right-sided central circulation and hemodynamic parameters in congenital heart disease with left-to-right shunt. We enrolled 20 children aged from 1 month to 4.8 years with ventricular septal defect (VSD) or patent ductus arteriosus (PDA). The concentrations of ANP in plasma were extracted through a cartridge (Sep-Pak C18) before being measured by radioimmunoassay. Significant increased concentrations of ANP in plasma from inferior vena cava (117.6 +/- 18.1 pg/mL), right atrium (160.6 +/- 21.6 pg/mL) to pulmonary artery (PA) (253.4 +/- 38.8 pg/mL) were recognized. In VSD (n = 10) and PDA (n = 10), the concentrations of ANP in plasma from the inferior vena cava correlated significantly with the ratio of pulmonary to systemic blood flow (Qp/Qs) (r = 0.69, p < 0.05; r = 0.94, p < 0.01 respectively), the systolic pulmonary artery pressure (r = 0.90, p < 0.01; r = 0.93, p < 0.01 respectively), the diastolic pulmonary artery pressure (r = 0.76, p < 0.02; r = 0.68, p < 0.05 respectively), and the mean pulmonary artery pressure (r = 0.88, p < 0.01; r = 0.87, p < 0.01 respectively). The concentrations of ANP in plasma from the pulmonary artery also correlated significantly with the Qp/Qs (r = 0.81, p < 0.01; r = 0.87, p < 0.01 respectively). The results indicated that left atrial volume loading may have an important influence on secretion of ANP in some congenital heart disease with left to right shunt.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Relationship between hemodynamics and plasma atrial natriuretic peptide in children with ventricular septal defect or patent ductus arteriosus. 779 85

The Kabuki make-up syndrome, reported in 1981 by Niikawa et al. and Kuroki et al. independently, is also known as "Niikawa-Kuroki syndrome". Here we report two cases of this syndrome, both of them were boys, showing facial dysmorphism, hand abnormalities and congenital heart disease. The first case had developmental retardation noticed since 3 months old and frequent upper respiratory tract infections in the following years, and the cardiac echo revealed mitral regurgitation and tricuspid regurgitation. The second case had patent ductus arteriosus and ventricular septal defect diagnosed at 17 days old. He underwent corrective surgery for his heart problems, he also couldn't complete a sentence at two and half years old. It was estimated that the incidence of this syndrome was 1/32,000 in Japanese Children, and Chinese are similar to Japanese as yellow-skinned oriental, there should be more cases in our country, and a detailed cardiac study may be necessary for them.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Kabuki make-up syndrome associated with congenital heart disease. 817 45


1 2 Next >>