[Hepatosplenomegaly and motor delay in a 6-month old infant].
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Biomedical subjects
Publications and source records attributed to P Frontera Izquierdo.
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OBJECTIVE: To determine the relative frequency and describe the predisposing causes of recurrent pneumonia in infants and children aged between 1 month and 14 years. METHODS: We retrospectively reviewed the medical records of a tertiary care pediatric hospital covering a 10-year period, from January 1994 through December 2003. Children with cystic fibrosis were not included in the analysis. Recurrent pneumonia was defined as at least two pneumonia episodes in a 1 year period or at least three episodes over a lifetime. RESULTS: Of 1644 children hospitalized with pneumonia, 106 (6.4 %) met the criteria for recurrent pneumonia. An underlying cause was identified in 92 patients (86.7 %). Of these, the underlying cause was diagnosed prior to pneumonia in 67 (72.8 %), during the first episode in 12 (13 %) and during recurrence in 13 (14.1 %). Underlying causes included asthma in 28 patients (30.4 %), congenital cardiac defects in 27 patients (29.3 %), aspiration syndrome in 25 patients (27.1 %), immune disorder in nine patients (9.7 %), pulmonary anomalies in two patients (2.1 %), and anhidrotic ectodermal dysplasia in one patient (1 %). CONCLUSIONS: Recurrent pneumonia occurred in 6.4 % of all children hospitalized for pneumonia. The underlying cause was identified in 86.7 % of the children. The most common causes were asthma, congenital cardiac defects, and aspiration syndrome.
We review here our results in the management of 314 patients with tetralogy of Fallot. These cases represent 13.5% of the congenital heart defects diagnosed by angiohemodynamic methods in the period 1971-1988. Of the 314 children, 234 were subjected to intracardiac repair. The overall mortality was 25.8%. The surgical mortality rate for the intracardiac repair was above 30% in the period 1972-1979, and decreased to nearly 10% afterwards. The 7-year survival was 70% for the entire cohort, 76.3% for the children subjected to complete correction, and 90% for children operated after 1983. Residual hemodynamic abnormalities and dysrhythmias were uncommon in this series. Our results favour primary repair of the defect in practically all patients.
We have reviewed the records of 65 children with paroxysmal supraventricular tachycardia (PST) without congenital heart disease followed a mean of 4 years, with a total of 121 episodes. PST appeared before 6 months of age in 42 (64.6%) children. Thirteen patients (20%) had a present factor which might predispose to PST in 66.2% of the patients who were younger than 6 months of age, and in only 4.3% of those over 6 months. Wolff-Parkinson-White syndrome was present on surface ECG during sinus rhythm in 26.1% of children younger than 6 months, and in 39.1% of those over 6 months. Digoxin was the initial treatment in 84.3% of the episodes with a success rate of 75% when were employed alone and of 84.2% when were employed in combination of quinidine. PST recurred at least once in 35 children (53.8%), the 90% within three months of the first episode. All patients were alive and 63 (96.9%) doing well. One patient developed cerebral anoxia and now has hemiparesia and another patient has incessant PST. We conclude that children with PST without congenital heart disease and without delay in diagnosis had a good outcome.
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Between january 1971 and january 1988, the number of infants of less than 12 months diagnosed in our Hospital of symptomatic coarctation of aorta by cardiac catheterization and angiography was 101, 50 between 1971-1982, before the introduction of infusion of prostaglandin E, and 51 between 1983-1988. In the first period only 25 of the infants (50%) were treated surgically, the overall mortality was 74% and the surgical mortality was 60%. Between 1983 and 1988 the number of children who had surgical repair was 48 (94%), the overall mortality was 35.2% and the surgical mortality was 33.3%. In the two periods all the deaths took place in the first year. The high risk of mortality in this group, mark again the importance of the surgical correction in optimal conditions and as early as possible. The actuarial survival data support that improvements in the medical treatment before surgery are the main factors in the improvement of the prognosis after 1983. Four children survived after a second operation for repair of other cardiac associated anomalies. At 9.5 years mean follow-up the frequency of recurrence of the coarctation was low (5.1%), the repair was good in the majority of the survivors and the less satisfactory functional results was done in the cases with associated defects. None of the survivors has systemic hypertension. The results shows the important modification of the natural history of this malformation in the last decade.
The results in the management of 36 cases of isolated total anomalous pulmonary venous connection are presented. This patients are the 1.55% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in our hospital at the 1971-1988 period. Nineteen had a type I, five a type II, eight a type III and four a mixed type lesion. The total mortality rate was 63.8%. Twenty six infants underwent surgical correction at a mean age of 2.5 months with an operative mortality rate of the 57.7%. Eleven infants survived operation. There have been no late deaths but one children developed stenosis at the anastomosis. The final actuarial survival rate was the 34.4% in the total group and the 42.3% in the operated group. The strongest determinants of survival were the type of lesion and pulmonary venous obstruction. No improving in the total and the surgical results has been observed in the last 10 years.
From January 1971 to January 1988, 140 patients were diagnosed of complete transposition of the great arteries with an intact ventricular septum. These patients are the 6% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17 year period. The overall mortality was 63.5%. Sixty children died at a mean age of 55 days before cardiac surgery was performed, mostly before 1982. Forty nine patients underwent a Senning procedure with a surgical mortality of 30.6%. Between 1983 and 1988, with the use of infusion of prostaglandin E in the neonatal period, 28 children underwent a Senning procedure at a mean age of 12 months with a surgical mortality of 17.8%, and an actuarial survival rate of 80.9%. At late examination the 55.8% of the survivors were asymptomatic and the 8.8% showed caval or pulmonary venous obstruction.
Sixty nine children with pulmonary atresia and intact ventricular septum diagnosed by catheterization from 1971 to 1988 are reviewed. These patients are the 2.9% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17 year period. The overall mortality was 72.4%. Twenty children died at a mean age of 37 days before cardiac surgery was performed, mostly before 1982, before the use of prostaglandin infusion. Forty seven children received surgical treatment, with a surgical mortality rate of 74% at the period 1971-1982 and 54% at the period 1983-1988. The right ventricular hypoplasia was the anatomic factor of dismal outlook. The actuarial survival in the patients without hypoplastic right ventricle and surgical treatment was 52% in the period 1971-1988 and near 80% in the first 3 years of life in the period 1983-1988. The use of preoperative prostaglandin infusion and improved operative techniques has changed the natural history of this malformation.
We evaluate 48 patients who had catheterization-proved atrial septal defect ostium primum type. These patients are the 2% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in our hospital at the 1971-1988 period. Of 48 children, 36 underwent corrective surgical repair at mean age of 6 years, with a surgical mortality rate of 11.1%. Our mortality rate total was 12.5% and the average follow-up was 9 years. The most recent evaluation in the 32 survivors of complete repair shows that 2 children had complete atrioventricular block and pacemaker implanted, and 26 children had residual mild to moderate mitral regurgitation. Two children are in the functional New York Heart Association class III and 24 children in the class I or II. The estimated actuarial survival rate at the 10-15 years is 86.3% in the total and 88.2% in the operated group.
We examined the cases of 151 patients found to have atrial septal ostium secundum type, isolated or with association of partial anomalous pulmonary venous drainage, at catheterization from 1971 to 1988. This patients are the 6.5% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in our hospital in this period of time. The mean follow-up are 10 years. Only 5 children, the 3.3%, are symptomatic in early childhood and failed to respond adequately to conservative treatment and required operation in the first two years of life. Four of this 5 children are pulmonary hypertension. A total of 139 children underwent surgical correction at a mean age of 6.5 years. The final actuarial survival rate are the 97.29% in the total group and the 97.8% in the operated group. No cases of pulmonary vascular obstructive disease has been observed.
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Clinical data of nine cases of chondrodysplasia punctata, eight males and one female are reported. Two males presented with rhizomelic form, characterized by severe symmetrical shortness of humeri and femora, marked metaphyseal changes, severe psychomotor retardation and cataracts. Consanguinity of parents in one of these cases was consistent with homozygote for an autosomal retarded physical development starting early in life. Cataracts were absent, and psychomotor development was normal except in two cases who were severely retarded. These two cases suffered also from congenital cardiomyopathy (one case of aortic coarctation and the other of severe pulmonary hypertension without shunts). These seven cases were diagnosed of Conradi-Hunermann type which is a mild form of disease.
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Growth status was followed in 60 children with Fallot's tetralogy for a mean of 3,3 years after total correction. In 32 of the patients a palliative shunt had been performed earlier with no effect on their growth. Complete correction improved growth status in 50 children. Best results were seen when the correction was performed between 2 and 4 years of age. Ten children (16.6%) did not improve their growth status; 5 of them underwent surgical correction before 2 years of age, although only 11 of the 50 children with significant improvement were operated before this age. Pathophysiological and therapeutic implications of these findings are discussed.