Shewanella putrefaciens bacteraemia associated with terminal ileitis.

Shewanella putrefaciens has a wide geographical distribution, including sea- and fresh water, fish and soil, but it is an unusual pathogen in humans. A previously healthy boy without known immunodeficiency presented with fever, abdominal pain and diarrhoea, and a mass in the right lower quadrant. Terminal ileitis was confirmed by MRI and Shewanella putrefaciens was isolated from two separate blood cultures. This is the first report of terminal ileitis associated with Shewanella putrefaciens septicaemia.

HSV-2 meningitis disseminated from a herpetic whitlow.

HSV-2 meningitis is uncommon in childhood and is mainly associated with genital lesions or a history of sexual abuse. A 7-year-old boy with recurrent herpetic whitlow developed HSV-2 meningitis. HSV-2 was identified in the CSF by PCR. In children with herpetic whitlow, the risk of HSV-2, although rare, should be considered.

Severe jaundice in two children with Kawasaki disease: a possible association with Gilbert syndrome.

Kawasaki disease is a systemic vasculitis, mainly encountered in children. It may affect any organ. Acute cholestasis and severe obstructive jaundice is an atypical manifestation of the disease. We herein present two children with Kawasaki disease and severe direct hypebilibirunemia who also were homozygous and heterozygous respectively for the (TA)(7) promoter polymorphism of Gilbert syndrome. Intravenous immunoglobulin was administered to both patients at the acute phase of the disease and the fever remitted within 24 hr following the immunoglobulin administration. Furthermore oral aspirin at a dose of 80-100 mg/kg/24 hr was also given. The first child did not develop any coronary ectasia or aneurysm, whereas dilation of the right coronary artery was identified in the second child, one month after the disease onset. We discuss the possible contribution of Gilbert syndrome to the development of jaundice in our patients.

Miller-Fisher syndrome in association with enterovirus infection.

Miller-Fisher is a rare syndrome of childhood that presents with external ophthalmoplegia, ataxia, and areflexia. It has been mainly associated with a preceding Campylobacter infection and less commonly with other bacterial or viral infections. This report describes, for the first time, a child with Miller-Fisher syndrome and documented Enterovirus infection, as it was proven by the isolation of Enterovirus from cerebrospinal fluid by polymerase chain reaction testing.

Gilbert syndrome.

UNLABELLED: Gilbert syndrome is a common autosomal dominant hereditary condition with incomplete penetrance and characterized by intermittent unconjugated hyperbilirubinemia in the absence of hepatocellular disease or hemolysis. In patients with Gilbert syndrome, uridine diphosphate-glucuronyl transferase activity is reduced to 30% of the normal, resulting in indirect hyperbilirubinemia. In its typical form, hyperbilirubinemia is first noticed as intermittent mild jaundice in adolescence. However, Gilbert syndrome in combination with other prevailing conditions such as breast feeding, G-6-PD deficiency, thalassemia, spherocytosis, or cystic fibrosis may potentiate severe hyperbilirubinemia and/or cholelithiasis. It may also reduce plasma oxidation, and it may also affect drug metabolism. Although in general the diagnosis of the syndrome is one of exclusion, molecular genetic tests can now be performed when there is a diagnostic problem. The most common genotype of Gilbert syndrome is the homozygous polymorphism A(TA)7TAA in the promoter of the gene for UDP-glucuronosyltransferase 1A1 (UGT1A1), which is a TA insertion into the promoter designated UGT1A1*28. No specific management is necessary as Gilbert syndrome is a benign condition. CONCLUSION: Gilbert genotype should be kept in the clinician's mind, at least as a contributor factor, in cases with unexplained indirect hyperbilirubinemia.

Recurrent meningitis attributable to herpes simplex virus-2 in a child.

A boy manifested episodes of recurrent meningitis that were attributed to herpes simplex virus-2 infection. He presented no concurrent or previous history of involvement of the genitourinary system. He exhibited headaches, dizziness, photophobia, loss of balance, and vomiting. He underwent three episodes of aseptic meningitis. The herpes simplex virus-2 etiology was confirmed by polymerase chain reaction of the cerebrospinal fluid in the last two episodes. After the third occurrence, he was treated with acyclovir. Five years have elapsed since then, without the recurrence of aseptic meningitis.

Acute acalculous cholecystitis in children with Epstein-Barr virus infection: a role for Gilbert's syndrome?

Acute acalculous cholecystitis (AAC) in association with acute Epstein-Barr virus (EBV) infection has rarely been described in childhood. In the literature, there are only four reported pediatric cases of AAC associated with isolated primary EBV infection. We present two cases (one new, one retrospectively reviewed) of children with Gilbert's syndrome (GS) who presented with AAC during the course of primary EBV infection. Antibiotics were not used and AAC subsided gradually as the infection regressed. The co-occurrence of GS might have played a contributory role in the pathogenesis of AAC during acute EBV infection.

Evaluation of non-culture diagnosis of invasive meningococcal disease by polymerase chain reaction (PCR).

Antibiotic treatment prior to transport or admission to hospital has reduced the proportion of cases of invasive meningococcal disease (IMD) from which Neisseria meningitidis can be isolated by standard microbiological techniques. Identification of meningococci by polymerase chain reaction (PCR) was assessed in relation to microbiological diagnosis for cases over a 4-year period between 1998 and 2001. A screening assay for the IS1106 gene was used to detect meningococcal DNA and five additional assays for siaD and orf-2 genes were performed to determine the serogroup. PCR results were compared with results of bacteriological culture, other laboratory test results and clinical data. The sensitivity of the PCR assay for culture-confirmed cases was 98.5%. The specificity of the assay was 96% based on test results for patients from whom other bacteria were isolated, children with viral meningitis and afebrile negative controls. The siaD B/C/W-135 and Y as well as the orf-2 gene for serogroup A PCR assays were able to determine the serogroup for 75.2% of cases that were positive by PCR screening assay. When isolates from patients with IMD were tested by both agglutination and PCR, the results agreed in all cases. PCR is a useful tool for diagnosis of IMD when Gram stain and culture tests are negative due to antibiotic treatment prior to collection of samples for microbiological analyses.

Mesenteric lymphadenopathy as a cause of abdominal pain in children with lobar or segmental pneumonia.

We investigated whether mesenteric lymphadenopathy could be a cause of abdominal pain in children with lobar or segmental pneumonia. The study population consisted of 1)119 consecutive children with lobar pneumonia, older than 4 years of age, and 2) 31 healthy controls. Demographic, clinical, inflammatory, and radiographic data were recorded in all patients. All study subjects underwent abdominal ultrasound, focusing on the identification of mesenteric lymphadenopathy. One month later, a follow-up ultrasound was performed in patients with enlarged mesenteric lymph nodes at the initial examination. Forty patients complaining of abdominal pain were included in group 1, while the remaining 79 were in group 2. The two groups of patients did not significantly differ regarding their demographic, clinical (other than abdominal pain), and radiographic indices. In contrast, enlarged mesenteric lymph nodes with a sagittal diameter of at least 10 mm were identified significantly more commonly in the children of group 1 (P = 0.001). The association of enlarged lymph nodes with the presence of abdominal pain remained significant when the data were analyzed through multiple regression analysis (odds raio, 1.47; 95% confidence interval, 3-44). Enlarged mesenteric lymph nodes were found in a significantly lower ratio of healthy controls (3/31) compared to that observed in group 1 (P = 0.003). In all patients who were followed up, mesenteric lymph nodes had either decreased or were not detectable. Our findings indicate that mesenteric lymphadenopathy might be considered as a mechanism responsible for the development of abdominal pain in a relatively high percentage of children with pneumonia.

Refractory iron-deficiency anaemia due to silent Helicobacter pylori gastritis in children.

UNLABELLED: We describe the cases of three children with chronic active Helicobacter pylori gastritis and iron-deficiency anaemia without evidence of oesophagogastrointestinal bleeding. In all cases, long-standing iron supplementation became effective only after eradication of Helicobacter pylori. CONCLUSION: Iron-deficiency anaemia may be due to clinically inapparent H. pylori gastritis.

The impact of serum lipid levels on circulating soluble adhesion molecules in childhood.

Cell adhesion molecules play a rather important role in the development of atherosclerosis mediating the attachment of monocytes to the endothelium. It has also been well established that hyperlipidemia is a risk factor for atherosclerosis from childhood. The aim of this study was to investigate whether the soluble adhesion molecules correlate with the circulating lipid levels in children. The study population consisted of 107 children (64 boys, 43 girls) aged 6-13 y. Parental history of cardiovascular disease, age, gender, and anthropometric parameters were recorded in all children. Blood samples were obtained from every child following a 12-hour fasting period. Serum triglycerides, total cholesterol, and its fractions as well as plasma levels of P and E selectins and adhesion molecules sVCAM-1 and sICAM-1 were determined. After controlling for age and body mass index, both sVCAM-1 and sP-selectin levels were inversely associated with HDL values (r = -0.33, p = 0.005 and r = -0.39, p = 0.001, respectively). A significant positive correlation was found between sVCAM-1 and triglycerides (r = 0.48, p < 0.001). An increment of 10 mg/dL of HDL corresponds to about 50% reduction of the odds for endothelial dysfunction whereas an increment of 10 mg/dL of triglyceride levels indicates a more than 3-fold excess risk, using either sP-selectin or sVCAM-1 levels as a surrogate for the determination of endothelial dysfunction. We suggest that HDL-C and triglycerides correlate in a biologically plausible way with soluble adhesion molecules, which therefore could be considered as useful indicators of the process of preclinical atherosclerosis even from childhood.