[Eosinophilic gastroenteritis in a patient with Albright's hereditary osteodystrophy]. / Gastroenteritis eosinofílica en un paciente afectado de osteodistrofia hereditaria de Albright.

Eosinophilic gastroenteritis is an uncommon chronic disease, of unknown cause, characterized by eosinophilic infiltration of the gastrointestinal tract, which is usually associated with peripheral blood eosinophilia. The symptoms of this complex disorder are variable, and frequently include abdominal pain, nausea, diarrhea, protein losing enteropathy and malabsorption. In general, patients can be successfully treated with corticosteroids, but relapses are common. We present the first case of a 6-year-old boy with Albright's hereditary osteodystrophy (Pseudohypoparathyroidism Ia) associated with eosinophilic gastroenteritis. Alternatives to traditional treatment with corticosteroids are discussed.

Gastroenteritis eosinofílica en un paciente afectado de osteodistrofia hereditaria de Albright / Eosinophilic gastroenteritis in a patient with albright's hereditary osteodystrophy

La gastroenteritis eosinofílica es una rara enfermedad crónica, de causa desconocida, caracterizada por la infiltración del tracto gastrointestinal por leucocitos eosinófilos, que habitualmente se acompaña de eosinofilia en sangre periférica. Esta compleja enfermedad desarrolla síntomas variables, con frecuencia dolor abdominal, náuseas, diarrea, enteropatía con pérdida proteínica y malabsorción. En general, los pacientes pueden ser tratados satisfactoriamente con corticoides, pero son posibles las recidivas. Se presenta el primer caso de un paciente de 6 años afectado de osteodistrofia hereditaria de Albright (seudohipoparatiroidismo Ia) asociado a gastroenteritis eosinofílica, y se discuten las alternativas al tratamiento tradicional con corticoides (AU)

[Interventricular communication in the neonatal period]. / Comunicación interventricular en la edad neonatal.

OBJECTIVE: The aim of this study was to evaluate the prevalence and the follow-up in the long run of ventricular septal defect in the neonatal period. MATERIAL AND METHODS: 222 patients with ventricular septal defect from a total amount of 30,840 newborns admitted to our Hospital were evaluated between January 1991 and December 1996 (The incidence accounts for 7.19 per 1,000 newborns). 123 of these patients were males (55.4%) and 99 females (44.6%). The mean age at the time of examination was 3.09 +/- 1.67 days (range from 1 to 11 days). Diagnosis was suspected because of a harsh systolic murmur in 207 cases (93.2%), a malformative syndrome in 10 (4.5%) or polypnea in 5 (2.3%). The study was performed with a Sonos 1000 Echocardiograph device. The mean follow-up period ranged from 1 to 72 months. 10 patients underwent cardiac catheterization (4.5%). RESULTS: A muscular defect was the common finding in 177 patients (79.7%) whereas 41 patients (18.5%) exhibited a perimembranous defect. The mean size defect was 2.9 +/- 1.2 mm (range 1-10 mm), being smaller in the muscular defects (2.56 +/- 0.68) than in the perimembranous defects (4.33 +/- 1.56) p < 0.001. Spontaneous closure occurred in 176 ventricular septal defects (79.3%), 155 of them corresponding to muscular defects (87.6%) and 18 to perimembranous defects (43.9%). The mean age at the time of spontaneous closure was 9.5 +/- 8.9 months (9.4 +/- 9 months in the muscular defects and 10.3 +/- 4 months in the perimembranous defects). Spontaneous closure occurred by age 6 months in 52% of the patients with ventricular septal defect (54.5% muscular defects, 33.3% perimembranous defects), 12 months in 72.7% (71.2% muscular defects and 83.3% perimembranous defects) and 24 months in 96% (96.1% muscular defects and 94.4% perimembranous defects). 10 patients underwent surgical repair by age 11 +/- 8 months. All of them exhibited perimembranous defects with a mean size 6 +/- 1.5 mm which were tantamount to 24.4% of this group. 3 patients died (1.4%), 2 suffering from trisomy 18 and 1 after cardiac surgical repair. CONCLUSION: The most common ventricular septal defects in the neonatal period occur in the region of the muscular septum. The prognosis is favourable. Most of the patients experience spontaneous closure and show a very low mortality rate which is basically related to extracardiac anomalies.