Relapses in Wilms tumour

During an 18-year period, 54 children were treated for Wilms tumour (WT). Thirteen of them, 6 boys and 7 girls, had a relapse. Mean age at diagnosis was 50 months, range 5-233 months. The investigations revealed stage I in 5 cases, stage II in 2, stage III in 3, stage IV in 1 and stage V in 2.The histology was favourable in 6 cases, intermediate in 4 and unfavourable in 3. Clonal chromosome aberrations were detected in 8 cases. The mean time to first relapse was 17 months, range 1-76 months. The location was the local region in 3 cases, other kidney in 3, lungs in 7 and skeletal in 2. A second relapse occurred in 6 patients and a third relapse in 2 patients. Seven patients died after a mean follow-up of 84 months, range 42-180 months, from primary treatment. The 6 surviving children had no evidence of disease after a mean follow-up of 70 months, range 42-120 months, after treatment ended. High stage at diagnosis, unfavourable histologic subtype and occurrence of nephroblastomatosis were found not only in the children with relapse. No significant correlations were found when comparing the karyotypic features with clinical outcome. All 3 tumours with deletions of chromosome arm 16q metastasized.     

Operative management of duodenal atresia

The management of duodenal atresia (DA) in two Scandinavian pediatric centers is reported. A total of 67 infants with DA were retrospectively analyzed. A high incidence of associated anomalies was present, including Down's syndrome in 40% and cardiac anomalies in 20% of the infants. No immediate postoperative mortality was noted. The operative procedures were duodenoduodenostomy and duodenojejunostomy. Follow-up did not disclose any frequent gastrointestinal disturbances or differences in postoperative complications when the two procedures were compared. The prognosis for these patients is thus determined exclusively by the presence of associated anomalies, especially Down's syndrome and cardiac malformations.     

Evaluation of renal parenchyma in children by DMSA scintigraphy, X-ray computed tomography and intravenous urography

The demonstration of diminished or scarred renal parenchyma in children is often the decisive factor in determining the future management of children with urinary tract malformations. Renal scintigraphy using technetium 99m-labelled dimercaptosuccinic acid (DMSA), computed tomography (CT) and intravenous urography (IU) were used to evaluate the renal parenchyma prior to ureter re-implantation in a series of 13 children. Their ages ranged from 5 months to 3 years 8 months. The indication for operation was ureteric reflux in 8 children and distal ureteric stenosis in 5. CT was performed on a Toshiba TCT-80 scanner. Renal scintigraphy was performed 3 hours after intravenous injection of DMSA. Compared with IU, DMSA imaging gave more information about the renal parenchyma in 6 children, gave equal information in 6 and less in 1. Compared with CT, DMSA imaging gave more information in 2 children, was equally informative in 8 and less so in 3. Accordingly, DMSA imaging was judged to be more sensitive than IU and as sensitive as CT. DMSA imaging can be used both for the initial evaluation and for follow-up assessment of renal parenchymal damage.     

Evaluation of a new identification system, Crystal Enteric/Non-Fermenter, for gram-negative bacilli.

A total of 505 fermentative and 201 nonfermentative gram-negative bacilli, identified by conventional methods, were tested by the Crystal Enteric/Non-Fermenter ID kit and by the API 20E or API 20NE identification system. The overall correct results for fermenters were 92.9% by the Crystal kit and 89.1% by the API 20E system. The false identifications (genus and species incorrect) accounted for 3.1 and 7.1% for the Crystal and API systems, respectively. For nonfermenters, figures for correct identifications by the two systems were comparable (Crystal, 75.9%; API 20NE, 75.3%) while the API 20NE system gave twice as many incorrect results (13.8%) as Crystal (6.3%); however, Crystal failed to precisely identify several species included in a "miscellaneous" group. The Crystal Enteric/Non-Fermenter system is an easy-to-use kit which compares favorably with other commercial systems.     

Use of a multiplex real-time PCR to study the incidence of human metapneumovirus and human respiratory syncytial virus infections during two winter seasons in a Belgian paediatric hospital

Viruses are an important cause of acute respiratory tract infection (ARTI) in children. This study aimed to develop and evaluate a rapid molecular diagnostic test (duplex real-time PCR) for human respiratory syncytial virus (hRSV) and human metapneumovirus (hMPV), and to determine the frequency of these two viruses as causative agents of ARTI in Belgium. Nasopharyngeal aspirates were collected over two winter and spring seasons (November 2003 to May 2004 and November 2004 to May 2005) from children aged <5 years with ARTI (n = 778). The duplex real-time PCR showed a linear range of 10(4)-10(10) copies/mL for both hMPV and hRSV. Analysis of the stability of the hRSV and hMPV genomes revealed that nasopharyngeal aspirates could be stored at room temperature for up to 1 month without significant loss of detection. hRSV was detected by antigen testing and by real-time PCR; hMPV was detected by real-time PCR only. The hRSV antigen test was less sensitive than PCR, and failed to detect one-third of the hRSV infections. Overall, 54 (6.9%) and 306 (39.3%) of the 778 samples were positive for hMPV and hRSV, respectively. Both viruses infected young infants, but the mean age of infants infected by hRSV was lower than that of infants infected by hMPV (12 months vs. 17 months, respectively).     

Granulocytic sarcomas in body cavities in childhood acute myeloid leukemias with 11q23/MLL rearrangements

Three childhood acute monoblastic leukemias (AML M5) with granulocytic sarcomas (GSs) are described. All displayed 11q23/MLL abnormalities, t(9;11)(p22;q23) in two cases and t(11;17)(q23;q21) in one case, constituting around 20% of all 11q23-positive AML cytogenetically investigated in our department. Two of the patients had GS in multiple locations, and all three had abdominal GS. In two of them, t(9;11)-positive GS was diagnosed prior to the diagnosis of AML. Fourteen (1.9%) of 752 published AML cases with 11q23 aberrations have had GS, either as a presenting feature or during disease progression. The incidence of GS has varied significantly (P < 0.05) between children (3.8%) and adults (0.8%). The most common AML subtype has been AML M5 ( approximately 75%) and the most frequent GS sites have been the skin, abdomen, orbit, and thorax. Considering the possibility of underreporting of GS in published cases and the relatively high frequency in our own series, we believe that 11q23/MLL rearrangements may predispose to GS development. Although extramedullary infiltrates in the skin are known to be frequent in cases of AML M5, which is often associated with 11q23 aberrations, the present findings indicate that GS in the abdomen, orbit, and thorax may also be common, especially in pediatric AML. Thus, the possibility of 11q23/MLL-positive GS should be suspected when tumors of uncertain derivation occur in these sites. Finally, the identification of 11q23/MLL abnormalities in GSs in two patients without overt AML underscores the importance of using cytogenetic and molecular genetic investigations as a diagnostic approach in the evaluation of tumorous lesions of unknown origin. Genes Chromosomes Cancer 27:136-142, 2000.     

Diagnosis and treatment of ovarian cysts in children

Seventeen girls were treated following the diagnosis of ovarian cysts. Four patients were operated on within the first 6 months of age and the other 13 patients were 10-15 years old at the time of diagnosis. Antenatal diagnosis was made in 3 cases. The presenting symptoms in the infants were distended abdomen in 3 cases, abdominal pain in 1 and vomiting in 1. In the older children the presenting symptoms were abdominal pain in 12, vomiting in 5 and elevated temperature in 6. Preoperative ultrasound was performed in 5 patients, 3 neonates and 2 older children. Sixteen of the 17 girls were operated on. The indication for surgery was an ovarian cyst with complication in the infants and in the older children the suspicion of acute appendicitis. The operative procedure was cyst uncapping in 7 cases, salpingo-oophorectomy in 4, ovarian resection in 2, ovarian fixation only in 1 and no ovarian intervention in 2. Asymptomatic infants with an ovarian cyst less than 4 cm in diameter can be managed conservatively. Surgery can be recommended after documented change of the cyst on ultrasound, large cysts giving rise to symptoms or presentation of an acute abdomen. Salvage of variable ovarian tissue is desirable.