[Evaluation of therapeutic strategies in congenital nephrotic syndrome of the Finnish type]. / Evaluación de las estrategias terapéuticas en el síndrome nefrótico congénito tipo finlandés.

INTRODUCTION: Congenital nephrotic syndrome of the Finnish type (CNFS) is caused by mutations in the nephrin gene. This disease manifests as massive proteinuria, gross peripheral edema, and ascites during the first weeks of life. In the last few years the prognosis has improved due to new treatment strategies: antiproteinuria drugs, intensive nutrition, nephrectomy, dialysis, and renal transplantation. The aim of this study was to determine the impact of these therapeutic measures. PATIENTS AND METHOD: We performed a descriptive retrospective epidemiological study of 12 patients diagnosed with CNSF between January 1985 and August 2005. We included patients aged less than 14 years old with massive proteinuria and generalized edema during the neonatal period, a large placenta (> 25 % of birth weight), and normal glomerular filtration rate during the first 6 months of life, in whom other causes of congenital nephrotic syndrome were ruled out. RESULTS: The diagnosis was established after a median period of 17 days (range 6-30). The most commonly used treatments were albumin infusions (91.7 %), angiotensin-converting enzyme inhibitors (66.7 %), and indomethacin (58.3 %). Dialysis was started in 58.3 %, at a median age of 3.76 years (2.81-7.6). The main complication was acute peritonitis (85.7 %). Renal transplantation was performed in 58.3 % of the patients; of these, 71.4 % have normal renal function after a median follow-up of 3.73 years (0.8-6.3). The median plasma albumin level during the pretransplant period was 0.17 g/dL (0.12-0.28). Plasma cholesterol and triglyceride levels decreased significantly after renal transplantation (p = 0.043). Fifty percent of the patients achieved adequate height and weight for their age and gender. Mortality was 33.3 %. CONCLUSION: Antiproteinuria drugs and intensive nutritional therapy improve clinical control and delay the start of dialysis and renal transplantation, increasing the probability of success.

[Epidemiologic study of accidental poisoning cases treated at a pediatric hospital]. / Estudio epidemiológico de las intoxicaciones accidentales atendidas en un hospital pediátrico.

A prospective study, by epidemiological survey, was carried out in the 447 children with acute poisoning attended in our hospital between February 1, 1990 and January 31, 1991. All data was processed to analyze the possible associations that would help to better understand the factors that take part in poisoning. We observed a predominance in male infants, with a greater incidence between two and three years of age, and the accidents occurred almost exclusively at home, mainly in the kitchen or bedroom. The child is often not adequately watched and the toxic elements are usually within easy access. There was neither a seasonal nor daily predominance. We noticed an hourly predominance with most accidents occurring between 12 a.m. and 4 p.m. Drugs are the most common agents, followed by household cleaning products. Morbidity was scarce and there was no mortality.