HBeAg-negative hepatitis B in a previously thalassemic patient during immunosuppressive therapy for chronic GVHD.

We report the case of a 15-year-old previously thalassemic girl who, 15 months after allogeneic BMT, developed HBeAg-negative hepatitis B (variant with mu-1896). In the absence of another route of transmission, HBV reactivation is postulated. The time of emergence of the HBV variant (with mu-1896) is probably related to the development of anti-HBe immunity. This mutant strain is associated with fulminant hepatitis. The patient achieved complete remission and HBV eradication despite having moderate GVHD and receiving immunosuppressive therapy.

Effective detection of active HCV infection: HCV RNA carrier state in a context free of hepatitis symptoms.

HCV immunological assays have limited specificity due to considerable variability of genomic coding sequences. Accordingly, PCR RNA detection also shows variable incidence of HCV in a non-A, non-B (NANB) hepatitis context. We used in-house designed nested PCR applying primers from the 5' untranslated region in 150 thalassemic patients classified in four groups according to anti-HCV screening and glutamic-pyruvate transaminase (GPT) levels. Group A: anti-HCV+/high GPT levels; group B: anti-HCV+/normal GPT levels; group C: anti-HCV(-)+high GPT levels; group D: anti-HCV(-)+normal GPT levels. Viral incidence and concentration, both high in group A, decreased towards group D. Group C RNA incidence was unexpectedly high and, moreover, one control case proved HCV-RNA+. Compared with the Amplicor kit or primers were considerably more sensitive.

Tissue immunodetection of c100 hepatitis C virus antigen in major thalassemic patients.

BACKGROUND/AIMS: Hepatitis C Virus (HCV) detection in the livers of chronically infected patients remains a debatable issue. To determine the significance of hepatic expression of hepatitis C viral antigen c100, an immunohistochemical assay was performed in 113 young thalassemics with chronic HCV infection. METHODOLOGY: One hundred and thirteen patients were seropositive for antibody to HCV by second-generation testing. The monoclonal antibody TORDJI-22 was used in an alkaline phosphatase 3-step staining method, and any possible association between the results of HCV immunodetection and various clinicopathologic variables was investigated by univariate and multivariate statistical analysis. In 36 cases, post-therapy liver biopsy specimens were also studied. RESULTS: HCV c100 antigen was detected in 62% of all pretherapy samples, exclusively in the cytoplasm of rather few hepatocytes. Its expression was positively associated with male gender (p = 0.02) as well as with rather advanced age (p = 0.03) and was frequently accompanied by low necroinflammatory scores (according to the modified HAI grading). At the end of interferon-alpha (IFN-alpha) therapy, the immunoreactive prevalence of c100 antigen decreased significantly (pF = 0.002). CONCLUSIONS: We conclude that hepatic expression of c100 antigen is detected in a considerable percentage of thalassemics but it is not likely to provide information concerning the viral load in the infected liver. IFN therapy appears to reduce the hepatic expression of this viral antigen in thalassemic patients.

Skin biopsy for the diagnosis of Alport syndrome.

Alport syndrome (AS) is the most common hereditary nephritis often associated with extrarenal manifestations. It was first described by Alport on 1927. There is a primary disorder in collagen type IV which is the main component of the basement membranes. Alport syndrome is more frequently inherited as an X-linked and less commonly as an autosomal dominant or autosomal recessive trait. We describe the case of a 3-year-old boy with the X-linked variant of AS. The diagnosis was at first speculated from the child's detailed family history and was finally confirmed by a skin biopsy. Skin biopsy is an efficient and less invasive method for the X-linked variant of the AS diagnosis.

Glucose-6-phosphate dehydrogenase deficiency and Gilbert syndrome: a gene interaction underlies severe jaundice without severe hemolysis.

The authors describe the paradoxical clinical phenotype of an undetected severe hemolysis in parallel with the development of severe jaundice in a 13-year-old male suffering from a confirmed interaction of glucose-6-phosphate dehydrogenase deficiency (Mediterranean variant, 563 C/T) and Gilbert syndrome [variant (TA)7/(TA)7]. The child had 2 acute hemolytic episodes at the age of 10 and 13 years following infections of unknown origin. Both episodes were characterized by considerably high bilirubin levels (1st episode: 10.8 mg/dL, 2nd episode: 17.8 mg/dL) associated with unexpectably mild hemolysis indices (1st episode hemoglobin levels, 11.1 g/dL; reticulocyte counts, 2.5%; 2nd episode hemoglobin values, 12.7 g/dL; reticulocyte counts, 2.5%). During the steady-state condition of the child, hemoglobin values were within the normal ranges for his age (14.2 g/dL) and bilirubin levels were slightly elevated (1.70 mg/dL, indirect 1.5 mg/dL). The interaction of the two genetic abnormalities in the causation of this odd clinical phenotype is discussed.

Long-term follow-up of children with vesicoureteral reflux with and without antibiotic prophylaxis.

The aim of the present study was to obtain data on the outcome of children with persistent vesicoureteral reflux (VUR) after cessation of antibiotic prophylaxis. Children with VUR who had been on antibiotic prophylaxis for at least 2 y and were free of urinary tract infections (UTI), had normal voiding patterns, and no hydronephrosis or new kidney scarring, had antibiotic prophylaxis discontinued, were followed up prospectively with urine cultures, voiding cystourethrography, and technecium-99m dimercaptosuccinate renal scintigraphy. The findings were compared with those of the same patients while they were on antibiotic prophylaxis. In 54 children (39 girls and 15 boys), antibiotic prophylaxis was discontinued. The mean follow-up time on and off antibiotic prophylaxis was 4.4+/-2.1 and 4.4+/-2.2 y, respectively. Nine UTI episodes occurred during the on- and 8 during the off-prophylaxis period. In 80 of 96 and in 68 of 74 ureters the reflux resolved or downgraded during the on- and off-prophylaxis periods, respectively. No new scar lesions were detected in any of the children. In conclusion, in children with persistent VUR and certain characteristics, antibiotic prophylaxis can be safely discontinued.

Immune response of immunocompromised children with malignancies to a recombinant hepatitis B vaccine.

The aim of this study was to interpret the antibody response to hepatitis B vaccination following an intensified four-dose schedule in 140 cancer patients who presented at our clinic between January 1, 1993 and December 31, 1994. According to therapy status, the patients were divided into two groups: group A consisted of 76 patients undergoing chemotherapy and group B of 64 patients in complete remission and off treatment. The eligibility requirements were negative hepatitis B virus (HBV), HCV, and human immunodeficiency virus serologic markers. A total of four dose (20 micrograms per dose) of recombinant HB vaccine was administered intramuscularly in the deltoid region at 0, 1, 2, and 6 months. Blood from the vaccinated subjects was obtained at months 1, 2, 3, and 7 in order to measure anti-HBs titer levels. Protective anti-HBs titers were considered to be those > or = 10 mIU/mL. The overall seroconversion rate 1 month after the fourth dose was 57% (80/140 patients), and the seroconversion rates for groups A and B were 31.5% (24/76 patients) and 87.5% (56/64 patients), respectively. Our results indicated that immunocompromised children undergoing chemotherapy (although less responsive than children in complete remission and off treatment) still preserved their potential to produce protective titers of anti-HBs. On this basis we recommend (1) HB vaccination after diagnosis of malignancy in pediatric patients whenever a high prevalence of HB infection exists and (2) vaccination of patients of therapy and in complete remission.

Childhood acute rheumatic fever in Greece: experience of the past 18 years.

UNLABELLED: This study reports on the authors' experience with acute rheumatic fever (ARF) during the years 1980-1997. The objectives were to estimate the incidence of the disease an area of Greece to characterize its epidemiology, to determine the frequency of the antecedent symptoms and to describe its clinical presentation. The medical records of 66 confirmed cases admitted to the First Department of Pediatrics, "Aghia Sophia" Children's Hospital, were reviewed. Two outbreaks occurred during this period. In contrast to the 3-4 cases seen every year, 14 cases were diagnosed during the 6 mo period from October 1989 to March 1990. An additional 10 cases were diagnosed in 1993. Most of the children (76%) were between 8 and 14 y old. The children were predominantly from middle-class families with ready access to medical care. Carditis, evident by auscultation, and arthritis were the dominant major manifestations in 70% and 68% of the cases, respectively. Mild carditis was present in 54% of children with valvular disease. CONCLUSION: ARF exists in the paediatric Greek population with exacerbations and remissions, but the cardiac manifestations appear mild or moderate.

Car transportation conditions of preschool children: use of children's car safety seats.

The objective of this study was to evaluate children's car safety seat usage in a non-random study population of the Capital and surrounding area of Attica and a provincial region in Greece. One or both of the parents of 1556 children (aged 0-4 years) were interviewed. A questionnaire covering car safety seat usage by area of residence, age of parents, educational background, socioeconomic status and the number of siblings was completed. Optimal car safety seat restraint usage in the Attica region was 10.4% for infants 0-6 months old, 37.6% for infants 7-12 months old, 40.9% for children aged 1-2 years and 12% for children aged 3-4 years; the use of restraint systems in the provincial area was even lower at 4.9%, 15.2%, 24% and 6.6%, respectively. The mother's age, number of children and the socioeconomic status of the family seemed to influence the purchase and usage of the car safety seat. Such a high percentage of non-usage of car safety restraint seats has led to the Paediatric Society's initiative to create informative general public educational programmes and to urge governmental legislation regarding mandatory usage of children's car safety seats.

Quantification of liver iron overload by T2 quantitative magnetic resonance imaging in thalassemia: impact of chronic hepatitis C on measurements.

PURPOSE: Measurement of liver T2 values seems to be an accurate and sensitive magnetic resonance imaging (MRI) method for the quantification of liver hemosiderosis in multiple transfused patients with thalassemia. Because many of these patients have coexistent chronic hepatitis C virus (HCV) infection, the effect of inflammatory changes on liver T2 values was assessed. MATERIALS AND METHODS: Liver MRI studies of 35 HCV+ and 17 HCV- patients with beta-thalassemia, 9 HCV+ patients without thalassemia, and 10 healthy controls of the same age range (13 to 32 years) were reviewed. Iron status was assessed by serum ferritin in all patients, and determination of liver iron concentration (LIC) was available in 16 HCV+ patients with thalassemia. Histologic activity index (HAI) and grades of siderosis were evaluated in all HCV+ patients with thalassemia. RESULTS: Patients with thalassemia had significantly lower T2 values (P < 0.0001) than subjects without thalassemia, whereas no difference existed between HCV+ patients without thalassemia and healthy controls. In HCV+ patients, LIC correlated more nearly with T2 values (r = 0.93) than with serum ferritin (r = 0.73). T2 values were not influenced by HAI score or fibrosis. CONCLUSION: Liver T2 values were found to be more accurate than serum ferritin in predicting liver iron overload and were not influenced by the presence of chronic hepatitis C. Therefore, MRI could serve as a noninvasive alternative to liver biopsy for the quantification of hemosiderosis in HCV+ patients with thalassemia.