Riboflavin in cyclic vomiting syndrome: efficacy in three children

Cyclic vomiting syndrome is an episodic disorder considered to be a migraine variant. Riboflavin is efficient in the prophylactic treatment of migraines in adults. We describe the effectiveness and tolerance of riboflavin treatment in three children with cyclic vomiting syndrome. All of them fulfilled the diagnosis criteria for cyclic vomiting syndrome. They received prophylactic monotherapy with riboflavin for at least 12 months. Excellent response and tolerability was observed

Protective antibody responses to influenza A/H1N1/09 vaccination in children with celiac disease

Patients with celiac disease have an increased risk for severe influenza infection and they show less of a response to certain vaccine types. During the influenza A/H1N1/09 pandemic, we prospectively investigated pandemic vaccine responses in 14 pediatric patients with celiac disease and age-/sex-matched controls. All of the children with celiac disease reached protective antibody titers (≥40) and showed a geometric mean titer comparable with the control group (530 vs 573)

Diagnostic accuracy of a new point-of-care screening assay for celiac disease

To determine the diagnostic accuracy of a new point-of-care assay detecting anti-deamidated gliadin peptides in celiac disease (CD) patients

Bacillus calmette-guerin infection in NADPH oxidase deficiency: defective mycobacterial sequestration and granuloma formation

Patients with chronic granulomatous disease (CGD) lack generation of reactive oxygen species (ROS) through the phagocyte NADPH oxidase NOX2. CGD is an immune deficiency that leads to frequent infections with certain pathogens; this is well documented for S. aureus and A. fumigatus, but less clear for mycobacteria. We therefore performed an extensive literature search which yielded 297 cases of CGD patients with mycobacterial infections; M. bovis BCG was most commonly described (74%). The relationship between NOX2 deficiency and BCG infection however has never been studied in a mouse model. We therefore investigated BCG infection in three different mouse models of CGD: Ncf1 mutants in two different genetic backgrounds and Cybb knock-out mice. In addition, we investigated a macrophage-specific rescue (transgenic expression of Ncf1 under the control of the CD68 promoter). Wild-type mice did not develop severe disease upon BCG injection. In contrast, all three types of CGD mice were highly susceptible to BCG, as witnessed by a severe weight loss, development of hemorrhagic pneumonia, and a high mortality (∼50%). Rescue of NOX2 activity in macrophages restored BCG resistance, similar as seen in wild-type mice. Granulomas from mycobacteria-infected wild-type mice generated ROS, while granulomas from CGD mice did not. Bacterial load in CGD mice was only moderately increased, suggesting that it was not crucial for the observed phenotype. CGD mice responded with massively enhanced cytokine release (TNF-α, IFN-γ, IL-17 and IL-12) early after BCG infection, which might account for severity of the disease. Finally, in wild-type mice, macrophages formed clusters and restricted mycobacteria to granulomas, while macrophages and mycobacteria were diffusely distributed in lung tissue from CGD mice. Our results demonstrate that lack of the NADPH oxidase leads to a markedly increased severity of BCG infection through mechanisms including increased cytokine production and impaired granuloma formation

Is Acute Fibrinous and Organizing Pneumonia the Expression of Immune Dysregulation?

INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a recently described histologic pattern of diffuse pulmonary disease. In children, all cases reported to date have been fatal. In this study, we describe the first nonfatal AFOP in a child and review the literature. DESCRIPTION: A 10-year-old boy developed very severe aplastic anemia (VSAA) after being admitted to our hospital with a fulminant hepatic failure of unknown origin. A chest computed tomography scan revealed multiple lung nodules and a biopsy of a pulmonary lesion showed all the signs of AFOP. Infectious workup remained negative. We started immunosuppressive therapy with antithymocyte globulin and cyclosporine to treat VSAA. Subsequent chest computed tomography scans showed a considerable diminution of the lung lesions but the VSAA did not improve until we performed hematopoietic stem cell transplantation 5 months later. CONCLUSIONS: Aplastic anemia is associated with a variety of autoimmune syndromes. The sequence of events in our patient suggests that the hepatic failure, AFOP, and the VSAA may all have been part of an autoimmune syndrome. AFOP could be the result of immune dysregulation in this pediatric case with favorable outcome after immunosuppressive therapy and hematopoietic stem cell transplantation

Lung parameters in response to BCG infection.

<p>(A) Lung/weight ratio of wild-type (n = 9), <i>Ncf1</i> mutant (n = 5) and <i>Ncf1</i> rescue (n = 8) mice without BCG infection and at 3 days and 4 weeks post infection. (B) Determination of alveolar space score (occupied lung tissue <i>vs.</i> free space) in lung sections at 4 weeks post infection. Data are represented as the mean of alveolar space score ± SD in 4 mice per group with at least 3 lobes analyzed per mouse. (C) Number of viable bacteria was determined at 3 days and 4 weeks following BCG infection. Data are shown as mean log of CFU per organ (±SEM; 3–5 mice per group). (D) iNOS protein expression in lung was detected by western blot 4 weeks after BCG infection. Results are expressed as mean ± SEM of relative units of iNOS/actin (n = 4, per group) after quantification by Image Quant software. (E) Nitrotyrosine quantification by ELISA was done in lungs, 4 weeks after BCG infection. Results are expressed as mean ± SEM of nM per lung (n = 4–5, per group). (***: p<0.001, **: p<0.05, *: p<0.01).</p