[Atrioventricular and ventriculoarterial discordance (corrected transposition of the great vessels). Diagnosis with bidimensional echocardiography].

We present 15 patients with atrioventricular and ventriculoarterial discordance diagnosed with two-dimensional echocardiography from January 1973 to June 1991. The 15 had situs solitus and six dextrocardia. A different level of insertion of the atrioventricular valves worth from 0.

[Application of bidimensional echocardiography in the diagnosis of ventricular septal defect].

Between 1985-1990 we studied by means of two-dimensional echocardiography 286 patients with isolated ventricular septal defect (VSD). A hundred and sixty three being male and 123 female, as a mean age of 5 +/- 3 months. Sixty two cases (22.

[Systemic-pulmonary shunt with autologous internal jugular vein].

We report a variant of systemic-pulmonary shunt for newborns or low-weight neonates affected with hypoplasia or atresia of pulmonary artery. An autologous internal jugular vein was used. Six months after surgery all patients referred no symptoms and a normal systemic-pulmonary shunt with an arterial oxygenation of 82-90% was present.

[Celiac disease and short stature in children].

Celiac disease (CD) is a known cause of short stature and sometimes, this is its only symptom. The aim of this work was to determine the incidence of CD in children with short stature. We studied 178 infants with short stature and with no gastrointestinal symptoms.

[Cardiovascular risk in the pediatric population. Plasma fatty acid levels].

Thirty-seven offspring of patients who had acute myocardial infarctions were investigated. Serum fatty acids of the whole plasma lipids were evaluated within the frame of a full biochemical assessment. Baseline values were obtained and the children were subsequently put on appropriate diets and advised to cut-out smoking and alcohol.

[Diverticulum of the right ventricle with dilated myocardiopathy].

We report on a newborn infant with right ventricular diverticulum associated to dilated myocardiopathy, who was diagnosed by two-dimensional echocardiography within 24 hours after vaginal delivery. We believe this case represents the youngest one ever diagnosed with this disease.

[Angioplasty of a stenosed Blalock-Taussig shunt. Its long-term assessment].

A Blalock-Taussig shunt was performed in a child of 14 months diagnosed with tetralogy of Fallot associated with a complete atrioventricular defect. Seven months later, a balloon dilatation was performed because the shunt was stenosed in the site of the distal anastomosis. Repeat angiography we proved the patency of the shunt without restenosis 4 year later.

Normokalaemic pseudohypoaldosteronism is present in children with acute pyelonephritis.

The present study demonstrates that renal tubular unresponsiveness to aldosterone, without associated hyperkalaemia, is present in children with acute pyelonephritis. We studied 32 children with a diagnosis of acute pyelonephritis established by high fever, flank pain/tenderness, increased blood levels of C-reactive protein and significant Escherichia coli growth in the urine culture. Renal tubular function tests and determinations of plasma renin activity and aldosterone concentration were performed at diagnosis (study 1), after three days of iv gentamycin (study 2) and after 21 days of antibiotic therapy (study 3).

[The double-chambered right ventricle: the surgical results in 28 cases].

Between May 1974 and June 1990, 28 double chambered right ventricle underwent surgical repair. Twenty-one (75%) had high obstructive anomalous muscle bundles and in seven (25%) the anomalous muscle bundles were in low position. A ventricular septal defect (VSD) was present in all cases and a subaortic stenosis in six.

[Supravalvular aortic stenosis. Apropos of 10 cases].

We present 10 patients diagnosed of supravalvular aortic stenosis (5 dumb bell shaped, 4 membranous and 1 undifferentiated), 7 in association with Williams-Beuren syndrome, 5 were male and 5 were female. None showed alterations of calcium metabolism and in 8 cases we found other associated cardiac anomalies. In 9, the diagnosis was confirmed with Eco-Doppler.

[Heart malformations in trisomy 13 and trisomy 18].

Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations.

[Fixed subaortic stenosis in childhood. The surgical correction of 28 cases].

Between January 1976 and December 1989, 28 patients with fixed subaortic stenosis underwent surgical correction. Twenty-one were male and seven female. The mean age when surgery was performed was 7.

[Aortic coarctation in the first 3 months of life. Surgical results].

Between January 1973 and September 1989, 51 patients younger than 3 months with coarctation of the aorta underwent surgery. All of them had atrio-ventricular and ventriculo-arterial concordance with well developed ventricular cavities. Thirty-four were male and 17 female.

[Aortic valva atresia with normal left ventricle and interventricular communication].

We present three patients with aortic atresia, normal left ventricle and ventricular septal defect by bidimensional echocardiography. In two patients the Norwood's procedure was performed, but they died in the immediate postoperative time. The different procedures for the correction of this kind of aortic atresia and the necessity of prompt surgical treatment are discussed here.

Neonatal citrullinaemia with satisfactory mental development.

In an infant with neonatal citrullinaemia therapy was instituted on day 1 of life with a low-protein diet and oral supplements of arginine, alpha-keto-acids, essential amino acids and carnitine. The latter may have contributed to the excellent clinical outcome, as evidenced by normal growth and satisfactory psychomotor development at 3 years of age.

Renal potassium excretion is reduced in children with idiopathic hypercalciuria.

Increased luminal calcium decreases potassium secretion in microperfused rat distal tubule. To determine if such an effect is also present in vivo, we evaluated renal potassium excretion in 49 children with idiopathic hypercalciuria (urinary excretion of Ca = 5.5 +/- 1.

[Congenital absence of the pulmonary valve. Analysis of 7 cases].

We present seven cases of congenital absence of pulmonary valve observed during a 15 years period. There are five females and two males, aged from one day to six years. Three patients with a birth weight under 1.

Renal tubular acidosis.

The term renal tubular acidosis (RTA) is applied to a group of transport defects in the reabsorption of bicarbonate (HCO3-), the excretion of hydrogen ions, or both. On clinical and pathophysiological grounds, RTA can be separated into three main types: distal RTA (type 1), proximal RTA (type 2) and hyperkalaemic RTA (type 4). Some patients present combined types of proximal and distal RTA or of hyperkalaemic and distal RTA.