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The hemolytic uremic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) is one of the most frequent causes of pediatric acute renal failure. The aim of this study was to report the clinic and microbiologic features associated with 13 post-diarrheal HUS cases identified in pediatric intensive care units in the city of São Paulo, Brazil, from January 2001 to August 2005. Epidemiologic, clinic, and laboratorial information, along with fecal and serum samples, were collected for identifying the genetic sequences of Stx and for studying antibodies directed against LPS O26, O111 and O157. STEC was isolated from three patients, and serotypes O26:H11, O157:H7 and O165:H- were identified. In nine patients, high levels of IgM against LPS O111 (n=2) and O157 (n=7) were detected. Dialysis was required in 76.9% of the patients; arterial hypertension was present in 61.5%, neurological complications were observed in 30.7%, and only one patient died. During a 5–year follow-up period, one patient developed chronic kidney disease. The combined use of microbiologic and serologic techniques provided evidence of STEC infection in 92.3% of the HUS cases studied, and the importance of O157 STEC as agents of HUS in São Paulo has not been previously highlighted.

IPEX is a rare X‐linked syndrome, with immune dysfunction, polyendocrinopathy and enteropathy. We describe an infant who died at the age of 11 months after developing eczema, severe diarrhoea, diabetes, hypothyroidism, thrombocytopenia and four episodes of septicaemia. Immunophenotyping of peripheral blood at 8 months revealed normal CD3+ T, CD4+ T and CD8+ T cell numbers, with low NK and B cells. CD4+ and CD8+ T lymphocytes showed remarkably low numbers and percentages of naïve cells and high numbers of memory CD4 and CD8 cells. At autopsy, an intense depletion of immune cells in thymus, spleen and lymph nodes was observed. No Hassall’s corpuscles were found in thymus. Lymphocytic pancreatitis and intense villous atrophy with mucosal lymphocytic infiltration in small bowel were also seen. FOXP3 gene studies revealed a: C→G substitution 3 bp upstream of exon 10, which prevents splicing between exons 9 and 10, likely resulting in a functionally altered or deficient protein. Florid clinical findings are usually observed in association of forkhead DNA‐binding domain mutations. The intense depletion of naïve T cells we report suggest that depletion of immune cells might take place due to uncontrolled activation due to the absence of regulatory T cells.

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Objective: To report the case of a one-year-old patient with a bloodstream infection associated with probiotics, and to discuss the indications and precautions concerning the therapeutic use of probiotics. Case description: A one-year-old male patient with Down syndrome in a late postoperative period of congenital cardiac disease correction. The patient was severely malnourished and had been hospitalized since he was two months old in the Pediatric Intensive Care Unit. While in the hospital, the patient presented multiple infections related to mechanical ventilation and invasive devices, and received recurrent treatment with broadspectrum antibiotics for long periods. The patient developed chronic diarrhea and feeding intolerance, which lead to the use of probiotics (Saccharomyces boulardii) for four days. Two days after the end of the treatment, the patient developed septic shock, and the Saccharomyces cerevisiae was isolated in the central and peripheral blood cultures. After antifungal treatment (Amphotericin B), the blood cultures were negative. The patient had no further clinical complications after this event. Comments: Despite the well-documented benefits of probiotics in some clinical situations, we should be cautious about the indication of their use, preparation, and administration, in addition to the safe handling of invasive devices.