Jean-Paul Buts

Characteristics of Epstein-Barr virus primary infection in pediatric liver transplant recipients.

BACKGROUND/AIM: Pediatric liver transplant recipients are at high risk of Epstein-Barr virus infection. However the incidence of clinical symptoms and the graft function at the time of acute infection remains poorly documented. The aim of this study was to monitor the clinical and biochemical events associated with primary Epstein-Barr virus infection. METHODS: Clinical and biological patterns associated with Epstein-Barr virus infection were prospectively searched in 38 liver transplanted children. Polymerase chain reaction and anti-Epstein-Barr virus IgM antibodies were used at regular intervals to detect the timing of primary infection. RESULTS: Five children (13%) had pretransplant immunity, 26 (68.5%) developed primary Epstein-Barr virus infection 15 to 90 days after transplantation and seven (18.5%) remained Epstein-Barr virus negative. The four patients with clinical symptoms at the time of infection subsequently developed post-transplant lymphoproliferative disease. A single post-transplant lymphoproliferative disease occurred in non-symptomatic patients (overall incidence 13%). No mortality was associated with post-transplant lymphoproliferative disease. Two asymptomatic patients had abnormal liver function tests possibly related to primary Epstein-Barr virus infection. CONCLUSION: Epstein-Barr virus primary infection occurs in 80% of seronegative patients within 3 months after OLT. Clinical symptoms are rare and closely associated with post-transplant lymphoproliferative disease. Outside post-transplant lymphoproliferative disease, the consequences of infection are marginal.

Role of hepatitis C virus in chronic liver disease occurring after orthotopic liver transplantation.

Paediatric orthotopic liver transplant recipients may develop chronic hepatitis after surgery. To investigate the role of hepatitis C virus in this pathology a cohort of 249 paediatric orthotopic liver transplant recipients was studied. Sixteen children (6.4%) were found to have chronic hepatitis C virus hepatitis after orthotopic liver transplantation. All but one of them had serum transaminase values which were persistently raised two to eight times the upper limit of normal. Thirteen were positive for both serology and serum hepatitis C virus RNA. Serum hepatitis C virus RNA detection occurred five to 33 months before hepatitis C virus antibodies. Liver tissue hepatitis C virus RNA and hepatitis C virus core antigen were detected in five. In one patient, tissue hepatitis C virus core antigen was detected when other tests for hepatitis C were negative. Two patients had positive human cytomegalovirus serum antibodies and RNA before transplantation. Although serum hepatitis C virus RNA was not detected after transplantation, serum enzyme immunosorbent assay and tissue core antigen were still detectable in both patients. In another child, serum hepatitis C virus RNA was positive and hepatitis C virus core antigen was found on a liver biopsy specimen but antihepatitis C virus antibodies were negative as well as liver hepatitis C virus RNA. No patient developed severe liver disease or cirrhosis during a follow up of up to 72 months. It is concluded that hepatitis C virus is a significant cause of morbidity after paediatric orthotopic liver transplantation. Diagnosis cannot rely on serological testing only. The patients remained stable on follow up, but longer prospective histological studies remain necessary to establish prognosis.

Caustic burns of the upper digestive and respiratory tracts.

In a series of 51 children presenting with an accidental caustic burn, symptoms were analysed for their predictive value of significant i.e. necrotizing oesophageal lesions (grade II or III). For the whole group, the incidence of significant oesophageal lesions was 37%. Vomiting and/or respiratory distress were associated with high incidence of significant oesophageal burn (84% and 75% respectively). The particular location of each caustic burn was analysed for its association with caustic burns at other anatomical sites. Of the 18 patients with a laryngeal burn, 72% also had a grade II or III oesophageal burn. The 19 patients with a grade II or III oesophageal burn and the 18 patients with a laryngeal burn, all had lesions at other sites. In the group of 19 patients with a caustic lesion limited to one site, only 1 patient showed mild oesophagitis (grade I) without late sequelae.

Postcricoid hemangioma: an overlooked cause of dysphagia in infants?-a case report.

Feeding and swallowing disorders in children remain a major challenge owing to a wide differential diagnosis. Hemangioma of the upper aerodigestive tract represents one of the numerous non-neoplastic causes of dysphagia. We report two cases of postcricoid hemangioma causing inhalation and recurrent respiratory infections, treated successfully with systemic corticotherapy alone. To our knowledge, these are the second and third cases described in the literature. After a short review of the literature, the diagnostic procedures are discussed and a management strategy is proposed for this clinical entity, by far underestimated.

La gastrite varioliforme diffuse: à propos de deux observations chez l’enfant

RésuméLes auteurs rapportent deux observations de gastrite varioliforme diffuse chez l’enfant. A cette occasion, les principales caractéristiques cliniques et l’évolution de cette affection rarissime chez l’enfant sont commentées à la lumière des données récentes de la littérature.SummaryTwo cases of diffuse varioliform gastritis, diagnosed in childhood are reported. In the light of the recent data published, the authors comment on the clinical presentation and course of this exceedingly rare entity in childhood.

Le pronostic fonctionnel et le traitement des séquelles des malformations anorectales chez l’adolescent et l’adulte

RésuméL’incidence des malformations anorectales est de 1/5000 naissances. Il s’agit d’un spectre de malformations, classées en formes basses ou hautes en fonction de leurs caractéristiques anatomiques et du pronostic de continence fécale chez le patient atteint. La prise en charge des malformations anorectales à la naissance inclut un bilan complet à la recherche des anomalies associées (entité VACTERL). La stratégie thérapeutique consiste en une chirurgie en un, deux ou trois temps, en fonction du type anatomique et de la nécessité d’une colostomie de protection. Les séquelles fonctionnelles à long terme et leur prise en charge dépendent du type malformatif: 1) les formes basses ont un bon pronostic en terme de continence fécale, mais peuvent être associées à une constipation sévère et un mégarectum responsable de pertes fécales (pseudo-incontinence par regorgement); 2) le pronostic des formes hautes est moins bon, avec un risque de développement d’une incontinence fécale qu’il est important d’identifier avant l’âge de 5–6 ans de façon à proposer un traitement adéquat visant à assurer une propreté fécale pendant les périodes scolaires (gestion intestinale par lavements évacuateurs rétrogrades). La réalisation de lavements coliques antérogrades par une appendicostomie continente ombilicale permettra ultérieurement d’accroître l’autonomie du patient à l’adolescence.SummaryAnorectal malformations occur with a frequency of 1/5,000 newborns and constitute a pathological spectrum, categorized in low and high defects according to their anatomical characteristics and their specific potential for the affected patients to reach fecal continence. The neonatal management should include a complete work-up for associated anomalies (VACTERL entity). The surgical strategy consists in a one/two/three-step approach, according to the anatomical type of anorectal defect and the need for a protective colostomy. The long-term functional sequelae and their management will depend on the type of anorectal malformation: 1) low defects have a good prognosis in term of fecal continence, but may be associated with severe constipation and megarectum responsible of fecal soiling (overflow pseudoincontinence); 2) the functional prognosis of high defects is worse, with possibility of developing fecal incontinence which should be identified in early childhood (3–5 years) in order to offer adequate management (conservative measures and/or bowel management) allowing school attendance with fecal cleanliness. The autonomy of the adolescent can be improved subsequently by means of self-administered antegrade colonic enemas through an umbilical continent appendicostomy.