High rate of fecal carriage of extended-spectrum-ß-lactamase-producing Escherichia coli in healthy children in Gipuzkoa, northern Spain.

The prevalence of extended-spectrum-ß-lactamase-producing Enterobacteriaceae (ESBLPE) was studied in stool samples from 125 8- to 16-month-old healthy children. Twenty-four percent of them and 10.7% of the 318 fecal samples studied yielded extended-spectrum-ß-lactamase-producing Escherichia coli, with the types being SHV-12, CTX-M-1, CTX-M-14, and TEM-52, the most common types of ß-lactamases. This high prevalence of ESBLPE in healthy people, which is to our knowledge the highest currently reported in Europe, may represent a risk for increased infections by these organisms in the future.

A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins.

We report on ten individuals with a fatal infantile encephalopathy and/or pulmonary hypertension, leading to death before the age of 15 months. Hyperglycinemia and lactic acidosis were common findings. Glycine cleavage system and pyruvate dehydrogenase complex (PDHC) activities were low. Homozygosity mapping revealed a perfectly overlapping homozygous region of 1.24 Mb corresponding to chromosome 2 and led to the identification of a homozygous missense mutation (c.622G > T) in NFU1, which encodes a conserved protein suggested to participate in Fe-S cluster biogenesis. Nine individuals were homozygous for this mutation, whereas one was compound heterozygous for this and a splice-site (c.545 + 5G > A) mutation. The biochemical phenotype suggested an impaired activity of the Fe-S enzyme lipoic acid synthase (LAS). Direct measurement of protein-bound lipoic acid in individual tissues indeed showed marked decreases. Upon depletion of NFU1 by RNA interference in human cell culture, LAS and, in turn, PDHC activities were largely diminished. In addition, the amount of succinate dehydrogenase, but no other Fe-S proteins, was decreased. In contrast, depletion of the general Fe-S scaffold protein ISCU severely affected assembly of all tested Fe-S proteins, suggesting that NFU1 performs a specific function in mitochondrial Fe-S cluster maturation. Similar biochemical effects were observed in Saccharomyces cerevisiae upon deletion of NFU1, resulting in lower lipoylation and SDH activity. Importantly, yeast Nfu1 protein carrying the individuals' missense mutation was functionally impaired. We conclude that NFU1 functions as a late-acting maturation factor for a subset of mitochondrial Fe-S proteins.

Emergency visits for childhood poisoning: a 2-year prospective multicenter survey in Spain.

OBJECTIVES: To describe the characteristics of childhood poisoning leading to consultation to 17 pediatric emergency departments in Spain. METHODS: During a 2-year period (January 2001 to December 2002), accompanying people of 2157 children with acute intoxication who visited consecutively at the emergency room were prospectively surveyed. RESULTS: Childhood poisoning accounted for 0.28% of all emergency visits during the study period. The median (interquartile range, 25th-75th percentile) age was 24 months (22-60 months); 67% of children were younger than 4 years. Drug ingestion was involved in 54.7% of cases (paracetamol was the most frequent drug), domestic products in 28.9%, alcohol in 5.9%, carbon monoxide in 4.5%, and illicit drugs in 1.5%. A total of 61.3% of patients were admitted within 1 hour after exposure to the toxic substance, and 10.3% had been already treated before arrival; 29.1% of patients were referred for clinical manifestations which were mostly neurological symptoms. Laboratory tests and other investigations were performed in 40.7% of cases. Gastrointestinal decontamination was used in 51.7% of patients, with activated charcoal in 32.3%. Treatment varied significantly according to the individual hospitals. A total of 83.3% of patients were treated as outpatients, 15.2% were hospitalized, and 1.5% were admitted to the intensive care unit. One 11-month-old boy with carbon monoxide intoxication died. Six patients had permanent sequelae (esophageal stenosis in 5 and partial blindness in 1). CONCLUSIONS: Young children who accidentally ingested drugs and, less frequently, domestic products accounted for most cases of intoxication who presented at the pediatric emergency department.