[Supraventricular paroxysmal tachycardia without congenital heart disease: clinical, therapeutic aspects and course in 65 children].

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.

[Study of LDL receptors and response to lovastatin therapy in familial homozygotic hypercholesterolemia].

This study shows the results obtained with lovastatin as a combined therapy with probucol and cholestyramine on the lipid profile of two patients with homozygous familial hypercholesterolemia. Both have been diagnosed according to the clinical and biochemical criteria (tipe IIa hypercholesterolemia) as well as by the cholesterol or low density lipoprotein (LDL-C) receptor analysis. After the initial probucol and cholestyramine treatment we observed a drop of total cholesterol (T-C) of 41.

[Prognosis in tetralogy of Fallot].

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.

[Lumbosacral dermal sinus as entry point of purulent meningitis. Report of 3 cases].

Defects of neural tube closure, although minimal, can provide access for infections of the central nervous system. All skin alterations in rear middle line of the body, however, minimal, must be carefully investigated as they could give access to bacterial meningitis. We present three new cases of dermal lumbosacral sinus which went unnoticed in the neonatal period later becoming the access point for bacterial meningitis.

[Retinoblastoma: multidisciplinary treatment in a group of 32 patients].

Thirty-two children with retinoblastoma (RTB) were treated in our oncology unit between September 1981 and October 1989. There were 18 unilateral RTB and 14 bilateral. The median age at diagnosis was 24 months.

[The treatment of esophageal achalasia in children by endoscopic pneumatic dilatation. A report on 4 cases].

Four patients underwent pneumatic endoscopic dilatation of achalasia at the La Fe Children's Hospital have been studied. Endoscopic pneumatic dilatation has been performed in only one session with four dilatation maneuvers. In the absence of complications, the patients were only hospitalized for twenty-four hours.

Selective effect of mineralocorticoid replacement therapy on renal acid excretion in congenital adrenal hyperplasia.

The renal acid excretion of eight children with salt-losing congenital adrenal hyperplasia, was studied in three different situations: before treatment (period I), under glucocorticoid therapy (period II) and when both glucocorticoid and mineralocorticoid were given as replacement treatment (period III). Although administration of glucocorticoid therapy alone allowed the correction of acidemia, normalization of urinary net acid excretion was achieved only after mineralocorticoid was added to the treatment.

[Natural and unnatural history of the univentricular heart].

We examined the cases of 90 patients found to have univentricular heart at catheterization from January 1971 to January 1988. These patients are the 3.8% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17-year period of time.

[The form of crystallization of perspiration in pancreatic cystic fibrosis].

The present study is based on the typical dendritic forms of the sweat crystallization from patients with cystic fibrosis (CF). The phenomenon was analyzed in sweat collected by pilocarpine iontophoresis in 26 affected subjects (Homozygotes), 42 heterozygotes and 100 healthy-control subjects. The positive crystallization pattern (dendritic form), became useful in identifying the affected patients in 100% of cases and in differentiating them from the healthy-control subjects (100%, negative crystallization).

[Caustic burns of the stomach secondary to ingestion of acid in infants: importance of fibro-endoscopy].

Two cases of corrosive burns of the stomach after the ingestion of acid by children are presented. Both patients were the same sex and of a similar age (19 and 20 months). The chemical nature of the caustic substance ingested (hydrochloric acid) and the lack of initial symptoms were also similar.

[Ten years of renal transplantation in children].

One hundred and fourteen kidney transplants (87 first and 27 second and thirds) were performed in 90 children aged 1 to 15 years during the period 1979-1988; 44% were under 10 years, 14% under 5 and 8% under 3 years of age. The renal grafts were from living related donor (LD) in 30 cases and from cadaveric donor (CD) in 84; 91 were from adult donors and 23 from pediatric ones. The actuarial survival rate of the patients at one and two years was 100% in the LD and 92 and 90% respectively in the CD group.

Hepatosplenic candidiasis in children with acute leukemia.

Three children with acute lymphoblastic leukemia developed disseminated fungal disease predominantly involving the liver and spleen. The three patients were undergoing induction chemotherapy and had neutropenia when they presented prolonged fever not responsive to antibiotics. Once neutropenia was recovered, hepatosplenomegaly leukocytosis, elevated serum alkaline phosphatase, and hypoechoic areas in the spleen and liver ultrasound were observed.

[Natural and modified history of atrial communication of the ostium secundum type].

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.

[Mortality and survival in total anomalous pulmonary venous drainage].

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.

[Acute lymphoblastic leukemia: clinical data and results of intensive chemotherapy].

Twenty-one children, between eight months and twelve years old, diagnosed as having acute nonlymphoblastic leukemia (ANLL), were treated with an intensive chemotherapy based on a modified VAPA protocol. Complete remission rate was 80%, and the 5 year-survival rate is 46%. One chloroma, one central nervous system, and four bone marrow relapses were observed; all except the first having an unfavourable outcome.

[Normal and impaired development of the ostium primum type atrial septal defect].

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.

[Mortality and survival rate in pulmonary atresia with intact intraventricular septum. Determining factors].

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.

[Long-term follow up of children with transposition of great vessels with intact interventricular septum].

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.

Complications of hepatic hydatid cysts: ultrasound, computed tomography, and magnetic resonance diagnosis.

A total of 179 hepatic hydatid cysts (HHCs) were studied by ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI). The diagnosis of HHC complications was established by US and CT, which permitted a distinction between intact cysts and those presenting with contained rupture, as demonstrated by a collapsed endocyst or a globally echogenic appearance. The diagnosis of HHC perforation into the main biliary tree was made by detection of a discontinuity in the cyst wall and/or the presence of hydatid material within the biliary system.