Time course of antibody response to recombinant Aspergillus fumigatus antigens in cystic fibrosis with and without ABPA

We determined follow-up levels of specific serum IgE to the recombinant Aspergillus fumigatus (A. fumigatus) allergens rAsp f 1, 3, 4 and 6 in patients suffering from cystic fibrosis (CF) with and without allergic bronchopulmonary aspergillosis (ABPA). Over a 32-month period follow-up data of 74 patients were collected. According to serology, 11 CF patients were not sensitized (CF controls), 40 were sensitized to A. fumigatus (Asp. f-sens.) and 23 patients fulfilled the serologic criteria for ABPA. Of these 23 ABPA patients 11 expressed the full clinical ABPA picture (classicABPA) and 12 failed to show sufficient relevant clinical signs (seroABPA), despite positive serology. The 23 ABPA patients had 16-18 times higher serum levels of specific IgE to rAsp f 4 and/or rAsp f 6 than those of Asp. f-sens. patients (rAsp f 4: 31.3 +/- 45 EU/ml vs. 1.9 +/- 2.2 EU/ml and rAsp f 6: 39.0 +/- 44.3 EU/ml vs 2.1 +/- 1.7 EU/ml). The combination of increased total serum IgE (>1000 IU/l) and increased specific IgE to rAsp f 4 and/or rAsp f 6 allowed to diagnose classicABPA with 100% specificity and 64% sensitivity and with a high predicted positive (100%) and a high predicted negative (94%) value. During a combined treatment (seven patients) with oral corticosteroid and itraconazole, itraconazole alone (two patients) or neither oral corticosteroid nor itraconazole therapy (two patients) total serum IgE and specific IgE to rAsp f 4 and/or rAsp f 6 did decrease but did not normalize. Over the observation period, lung function remained unchanged, independent of whether oral steroids and/or concomitant itraconazole were either given or not given. In the follow-up of CF patients with ABPA under therapy the determination of total or specific IgE serum levels were of limited value to guide therapy.     

<Emphasis Type="Italic">NPHS2</Emphasis> mutations

Objective  To uncover the frequency and the spectrum of NPHS2 mutations in Egyptian children with non familial steroid-resistant nephrotic syndrome (SRNS). Methods  Sixteen patients were screened by PCR-single-strand conformation polymorphism analysis of NPHS2 gene followed by direct sequencing. Results   NPHS2 mutations were evident in four patients (25%) who were bearing four novel mutations including two frame shift mutations (R238fs and P45fs) and two missense mutations (I136L and F216Y). There were no phenotypic or histological characteristics of patients bearing NPHS2 mutations, apart from the earlier onset of the disease, compared to those who were not bearing mutations. Conclusion   NPHS2 mutations are prevalent in Egyptian children with non-familial SRNS and this may in part explain the less favorable prognosis reported in these patients.     

Vertical transmission of <Emphasis Type="Italic">Salmonella paratyphi A</Emphasis>

Neonatal enteric fever is a rare but life-threatening illness. Patients may present with varying severity, Salmonella enterica serotype Typhi causing more severe illness than Salmonella enterica serotype Paratyphi A. Salmonella enterica serotype Paratyphi A is considered to cause milder infection with fewer complications. We report a rare case of vertical transmission of Salmonella enterica serotype Paratyphi A with severe complications and high mortality. Eventhough there are case reports of vertical transmission of Salmonella enterica serotype Typhi, to our knowledge, this is the first case report of vertical transmission of Salmonella enterica serotype ParatyphiA. The role of blood culture in accurate diagnosis and treatment is also discussed.     

<Emphasis Type="Italic">Saccharomyces boulardii</Emphasis> in childhood

Introduction  Probiotics are live microorganisms which confer a health benefit on the host. Saccharomyces boulardii, a yeast, has been found to be an effective probiotic in double-blind placebo-controlled randomized clinical studies. Materials and methods  We reviewed the established mechanisms of actions and clinical efficacy in children of S. boulardii. Conclusions  The mechanisms of action of S. boulardii depend mainly on the inhibition of some bacterial toxins, anti-inflammatory effects, and on stimulating effects on the intestinal mucosa such as trophic effects on the brush border enzymes and immunostimulatory effects. At present, in pediatric populations, there is evidence that S. boulardii is beneficial for the treatment of acute gastroenteritis and the prevention of antibiotic-associated diarrhea. More data are needed in other indications such as traveller’s diarrhea, Helicobacter pylori eradication, and inflammatory bowel disease. S. boulardii is a yeast strain that has been extensively studied in vitro and in vivo. Recent data have opened the door for new therapeutic indications. An erratum to this article can be found at     

Comparison of community-acquired methicillin-resistant <Emphasis Type="Italic">Staphylococcus</Emphasis><Emphasis Type="Italic">aureus</Emphasis> bacteremia to other staphylococcal species in a neonatal intensive care unit

Hospital acquired infections including staphylococcal species are common in neonatal intensive care units. Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) was recently observed in our unit. The clinical and laboratory characteristics of all neonates with Staphylococcus aureus bacteremia during an 11-year period were retrospectively reviewed. Three groups of patients were compared: 1. Patients with CA-MRSA defined as MRSA-resistant only to beta-lactams, but sensitive to all other antibiotic groups and carried SCCmec IV. 2. Patients with multi-drug-resistant (MDR)-MRSA and 3. Patients with MSSA (methicillin-sensitive S. aureus). Forty-three neonates with documented S. aureus bacteremia were included. Of these 41 were preterm babies. Eleven infants had CA-MRSA, 20 had MDR-MRSA and 12 had MSSA bacteremia, the Panton-Valentine-Leukocidine gene (pvl-gene) was not present in any of these strains. Risk factors, clinical manifestations and laboratory tests were similar in all three groups studied. Although neonates infected with CA-MRSA were more premature and had more related diseases, the mortality rate was similar in all groups (9.1% in the CA-MRSA group). Skin infections, osteomyelitis or pneumatocele were not observed more frequently in the CA-MRSA group. We did not find significant differences in risk factors or outcomes in neonates in the three groups. One possible explanation for this observation is that the CA-MRSA outbreak strain did not contain the pvl-gene, which has been suggested to be a significant virulence factor.     

Role of <Emphasis Type="Italic">CFTR</Emphasis> mutation analysis in the diagnostic algorithm for cystic fibrosis

Background:The cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation identification is being used with increased frequency to aid in the diagnosis of cystic fibrosis (CF) in those suspected with CF.Aim of this study was to identify diagnostic outcomes when CFTR mutational analysis was used in CF diagnosis.CFTR mutational analysis results were also compared with sweat chloride results.Methods:This study was done on all patients at our institution who had CFTR mutation analysis over a sevenyear period since August 2006.Results:A total of 315 patients underwent CFTR mutational analysis.Fifty-one (16.2％) patients had two mutations identified.Among them 32 had positive sweat chloride levels (≥60 mmol/L),while seven had borderline sweat chloride levels (40-59 mmol/L).An additional 70 patients (22.3％) had only one mutation identified.Among them eight had positive sweat chloride levels,and 17 had borderline sweat chloride levels.Fifty-five patients (17.5％) without CFTR mutations had either borderline (n=45) or positive (n=10) sweat chloride results.Three patients with a CF phenotype had negative CFTR analysis but elevated sweat chloride levels.In eighty-three patients (26.4％) CFTR mutational analysis was done without corresponding sweat chloride testing.Conclusions:Although CFTR mutation analysis has improved the diagnostic capability for CF,its use either as the first step or the only test to diagnose CFTR dysfunction should be discouraged and CF diagnostic guidelines need to be followed.     

<Emphasis Type="Italic">Chlamydophila pneumoniae</Emphasis> Myopericarditis in a Child

An 11-year-old boy with serologically confirmed Chlamydophila pneumoniae infection presented with clinical, laboratory, and echocardiographic changes consistent with myopericarditis. No reports on C. pneumoniae myopericarditis in children are found in the medical literature. The boy, previously healthy, presented with fever, rash, constitutional symptoms, elevated acute phase reactants, elevated cardiac enzymes, and high brain natriuretic peptide levels. Hemodynamic instabilities, including hypotension and mild hypoxia, were noted. Two-dimensional echocardiographic findings showed mildly depressed left ventricular systolic function and small pericardial effusion. Requiring inotropic support, the boy was treated with azithromycin 10 mg/kg once daily for 7 days and a single dose of intravenous immunoglobulin 2 g/kg. He recovered fully with improved left ventricular systolic function before hospital discharge. An early definitive diagnosis is essential to knowing the etiology of pediatric myocarditis. Specific therapy may play role in the management and prognosis of this disorder.     

<Emphasis Type="Italic">Enterobius vermicularis</Emphasis> and perianal sepsis in children

Specific aetiological factors are responsible for a significant proportion of cases of perianal sepsis in children. A rarely implicated pathogen is Enterobius vermicularis, an obligate parasite with a ubiquitous presence in children. The authors describe two unequivocal instances of threadworm involvement in perianal sepsis, suggesting a pathophysiological basis.     

Multimodality imaging of <Emphasis Type="Italic">Candida tropicalis</Emphasis> myositis

Fungal myositis is a rare entity that has been described in immunocompromised patients. We present a boy with biopsy proven fungal myositis who was examined with multiple imaging modalities. MR imaging proved to be very effective for diagnostic purposes, while US imaging was able to provide guidance for biopsy.     

Early onset neonatal infection with <Emphasis Type="Italic"> Neisseria meningitidis </Emphasis>serogroup C: case report and literature review

Although Neisseria meningitidis is one of the major causes of meningitis in children and adolescents, meningococcal disease should also be considered as part of the differential diagnoses in the newborn period.     

Tissue damage in rat ovaries subjected to torsion and detorsion: effects of <Emphasis Type="SmallCaps">l</Emphasis>-carnitine and <Emphasis Type="Italic">N</Emphasis>-acetyl cysteine

We aimed to evaluate histopathological changes, to detect HIF-1α staining intensities and to determine MDA levels in rat ovaries, which were subjected to torsion and detorsion and treated with l-carnitine or N-acetyl cysteine (NAC). Forty-eight prepubertal female Sprague–Dawley rats were divided into five groups (n = 8): 1, control; 2, ischemia; 3, reperfusion; 4, l-carnitine; and 5, NAC groups. In groups 3, 4 and 5, an ischemic period of 3 h was followed by reperfusion for 24 h. In groups 4 and 5, ischemia was performed and either l-carnitine or NAC was infused intraperitoneally 30 min before reperfusion. Ovarian tissues were examined histopathologically; tissue MDA levels and serum IL-6 levels were determined biochemically. HIF-1α was applied to all ovaries immunohistochemically. Total tissue damage scores, tissue MDA levels and HIF-1α scores, were significantly higher in group 2 (all P < 0.001) than group 4, and group 3 than group 4 (P < 0.001, P = 0.05 and P < 0.001, respectively). They were also significantly higher in group 2 (all P < 0.001) than group 5. When group 3 is compared to group 5, total tissue damage scores and tissue MDA levels were significantly higher in the former (P < 0.01 and P < 0.001, respectively). Serum IL-6 levels were significantly higher in group 2 when compared to groups 1, 4 and 5 (all P < 0.01). The degree of tissue damage of the torsioned ovaries decreased after a reperfusion period of 24 h in the torsioned ovaries. However, ovaries of both l-carnitine and NAC groups showed better recovery than the reperfusion group. This study was accepted for poster presentation in the 21st European Congress of Pathology, held in Istanbul, Turkey, on 8–13 September 2007.     

<Emphasis Type="Italic">Staphylococcus aureus</Emphasis> meningitis secondary to occult spinal extradural abscess

We report a rare case of spinal extradural abscess presenting as Staphylococcus aureus meningitis in a previously well child. After initial improvement with intravenous antibiotics the patient clinically deteriorated in a non-specific manner. Magnetic resonance imaging (MRI) scan of the spine revealed an occult spinal extradural abscess and urgent neurosurgical laminectomy led to full recovery. No evidence of an underlying spinal abnormality or immunodeficiency was demonstrated on investigation. A high index of suspicion is required in these cases due to the frequent absence in children of classic symptoms of spinal extradural abscess. Conclusion: we advise an urgent MRI of the head and spine in such unexplained cases of Staphylococcus aureus meningitis, to allow early diagnosis and management of occult underlying pathology. Such an approach will minimise the risk of potentially catastrophic neurological sequelae.     

Colostrum from healthy Brazilian women inhibits adhesion and contains IgA antibodies reactive with Shiga toxin-producing <Emphasis Type="Italic"> Escherichia coli</Emphasis>

Although Shiga toxin-producing Escherichia coli (STEC) has been isolated in Brazil, severe manifestations of the infection, such as haemorrhagic colitis and haemolytic-uraemic syndrome, are extremely rare in our population. Enteropathogenic Escherichia coli (EPEC) is the main aetiological agent of acute infantile diarrhoea in Brazil. There are many similarities between STEC and EPEC, such as the ability to produce attaching and effacing (A/E) lesions and some virulence-associated factors. Our aim was to investigate the presence of anti-STEC antibodies in healthy people living in an EPEC endemic area. Colostrum samples collected from 51 women living in low socio-economic conditions were analysed. Two STEC strains: O111:H- (Stx1) and O157:H7 (Stx2), and one EPEC strain (O111:H-) were used in the bacterial adhesion assays to HEp-2 cells, in the Stx1 and Stx2 cytotoxicity assays on Vero cells, in immunoblotting and in ELISA assays. All the samples strongly inhibited the adhesion of the three strains and contained SIgA antibodies reactive with antigens of EPEC O111:H-, STEC O111:H- and STEC O157:H7, mainly STEC and EPEC 94 kDa adhesin intimin. High titres of anti-LPS O111 antibodies were found in many samples. Nevertheless, the cytotoxic effect of both Stx1 and Stx2 on Vero cells was not neutralised by any sample. Conclusion: Our results suggest that Brazilian people may be exposed to Shiga toxin-producing Escherichia coli more frequently than previously thought or alternatively there may be a cross reactive immunity between enteropathogenic Escherichia coli and Shiga toxin-producing Escherichia coli.     

Antibiotic resistant <Emphasis Type="Italic"> Streptococcus pneumoniae </Emphasis>and hemolytic uremic syndrome

The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in young children and most often follows an episode of gastroenteritis caused by an enterohemorrhagic strain of Escherichia coli (O157:H7). HUS induced by Streptococcus pneumoniae (SP) is rare. We report an 18-month-old patient who presented with HUS associated with SP resistant to penicillin and cephalosporins. Conclusion:Despite a protracted course including renal failure requiring 15 days of peritoneal dialysis, her kidney function completely recovered.     

A palpable right lower abdominal mass due to<Emphasis Type="Italic"> Yersinia</Emphasis> mesenteric lymphadenitis

Infection by Yersinia pseudotuberculosis has become of increasing pathological importance. This report describes the case of a 12-year-old female with mesenteric lymphadenitis due to Yersinia pseudotuberculosis. The patient presented with fever, abdominal pain, and a palpable right abdominal mass. Abdominal ultrasonic imaging and computerized axial tomography (CT) revealed a mass. An exploratory laparotomy was performed, followed by appendectomy and mesenteric lymph node biopsy. The diagnosis of Yersinia infection was confirmed by serology and bacterial culture of the biopsy material. This condition should be considered in patients with a right lower abdominal mass and symptoms similar to those of appendicitis.     

Omenn Syndrome with Mutation in <Emphasis Type="Italic">RAG1</Emphasis> Gene

Omenn syndrome is a form of severe combined immunodeficiency associated with erythrodermia, hepatosplenomegaly, lymphadenopathy, and alopecia. Inherited hypomorphic mutations in the recombination activating genes 1 and 2 (RAG1 and RAG2) and in ARTEMIS genes and more recently defects in IL7RA, and RMRP genes have been described to be responsible of this peculiar immunodeficiency. The authors report here a Moroccan patient of four-months-old with classical features of Omenn Syndrome, carrying a deletion at the N terminal part of RAG1. Early recognition of this condition is important for genetic counseling and early treatment.