[Gilbert's syndrome. 3 pediatric cases]. / El síndrome de Gilbert. Tres casos pediátricos.

The study includes three pediatrics patients with Gilbert's syndrome (GS). The diagnosis was made on the basis of a mild unconjugated hyperbilirubinemia in the presence of repeatedly normal liver function test and absence of over signs of hyperhaemolysis. Two test have been also proposed for the diagnosis of GS, namely the hyperbilirubinaemia induced by a reduction in caloric intake, and hyperbilirubinaemia induced by nicotinic acid. In this study a reduction in caloric intake was associated with a greater absolute increase in serum bilirubin concentration. A single determination of bilirubin in parents and brothers of patients, showed high bilirubin in only one parent. The possibility of arriving at a definite diagnosis of GS is important both to the frequent presentation in adolescence with nonspecific symptoms that abdominal recurrent pain, the high incidence of the benign syndrome, and also to reassure the "patient".

[Cardiopathy in Noonan syndrome. Review of 29 cases]. / Cardiopatía en el síndrome de Noonan. Revisión de 29 casos.

Twenty nine cases, nineteen females and ten males of Noonan's syndrome are reported. The age at diagnosis ranged from five months to 11.5 years (median age: 56 months). Fifteen cases 57% disclose aspects of the Noonan's phenotype in female siblings. Fourteen cases showed an associated cardiopathy. The total included eleven pulmonary valve stenosis, five atrial septal defect. three hypertrophic myocardiopathies, three ventricular septal defect, one patients with pulmonary stenosis manifested an associated situs inversus this concomitance is the first reported in Noonan's syndrome. Eight patients underwent surgery, and two valvuloplasties. We can conclude that Noonan's syndrome includes cardiopathy in 50% of patients the most frequent type is pulmonary valve stenosis. On going follow-up will permit to evaluate the clinical evolution of the hypertrophic cardiomyopathy.

[Significance of IgG serum levels in cystic fibrosis]. / Significado del nivel sérico de IgG en la fibrosis quística.

IgG serum levels were measured in 25 patients with cystic fibrosis aged 1 to 19 years and in 4 additional patients deceased over the last 4 years. Levels were correlated with a number of parameters. Twelve patients (group A) had raised IgG levels, and 17 (group B) normal or low levels. Age between groups did not differ. Patients in group A had lower Shwachman score (p less than 0.001), worse Chrispin and Norman radiologic score (p less than 0.05), lower weight (p less than 0.001) and height (p less than 0.05) and lower FVC (p less than 0.05), and FEV1 (p less than 0.01). Raised levels were associated with increased number of serum precipitins against Pseudomonas aeruginosa but not against Staphylococcus aureus. Raised IgG levels might be secondary to heavier bronchial infection, but there is growing evidence that immunologic response in cystic fibrosis may worsen the course of the disease.