Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia.

Children with Down syndrome (DS) and acute lymphoblastic leukemia (ALL) have an inferior prognosis compared with non-DS ALL patients. We reviewed methotrexate (MTX)/mercaptopurine (6MP) maintenance therapy data for children with DS treated according to the Nordic Society of Pediatric Hematology and Oncology (NOPHO) ALL92 or the NOPHO ALL2000 protocols between 1992 and 2007. The 5-year event-free survival probability (pEFS(5 yr)) for the 66 DS patients was inferior to the 2602 non-DS patients (0.50 ± 0.07 vs 0.77 ± 0.01 (P<0.001)). The 48 DS patients in first remission at the beginning of maintenance therapy had pEFS(10 yr) below that of the 522 non-DS control patients (pEFS(10 yr): 0.58 (95% confidence interval (CI) 0.43-0.77) vs 0.83 (95% CI 0.80-0.86), respectively (P<0.0001)). The DS patients received lower median doses of MTX (median: 11.8 vs 15.4 (P<0.0001)) and 6MP (median: 43.6 vs 59.4 (P<0.0001)). In Cox regression analysis, male gender, presence of DS and high median maintenance therapy white blood cell levels (mWBC) were associated with increased risk for relapse. DS-ALL patients with mWBC above or below 3.5 × 10(9)/l (protocol target) had pEFS(10 yr) of 0.31 and 0.72 (P=0.02), and the mWBC hazard ratio for DS-ALL patients was 2.0 (P<0.0005). We conclude that insufficient treatment intensity during maintenance therapy of DS-ALL patients may contribute to their poor prognosis.

Vaccination associated thrombocytopenic purpura in children.

Patients who presented with purpura and blood platelets <30x10(9)/l within 1 month after vaccination were collected from a population based material of 506 consecutive pediatric patients with newly diagnosed ITP. Of the 35 such patients, 24 had thrombocytopenia after MMR vaccination giving an estimated ITP risk of approximately 1 in 30,000 MMR inoculations. Symptoms of the 35 patients were nearly always acute. Thrombocytopenia disappeared within a month in 74% of the study patients and lasted longer than 6 months in only 10%. Bleeding episodes were uncommon during the follow-up period. We conclude that the incidence of symptomatic thrombocytopenia after vaccinations is much lower than that after respective natural infections and that the outcome in most cases is excellent.

Long-term results in children with AML: NOPHO-AML Study Group--report of three consecutive trials.

In all, 447 children with acute myeloid leukaemia (AML) have been treated on three consecutive NOPHO studies from July 1984 to December 2001. NOPHO-AML 84 was of moderate intensity with an induction of three courses of cytarabine, 6-thioguanine and doxorubicin followed by four consolidation courses with high-dose cytarabine. The 5-year event-free survival (EFS), disease free survival (DFS) and overall survival (OS) were 29, 37 and 38%. NOPHO-AML 88 was of high intensity with the addition of etoposide and mitoxantrone in selected courses during induction and consolidation. The interval between the induction courses should be as short as possible, that is, time intensity was introduced. The 5-year EFS, DFS and OS were 41, 48 and 46%. In NOPHO-AML 93, the treatment was stratified according to response to first induction course. The protocol utilised the same induction blocks as NOPHO-AML 88, but after the first block, children with a hypoplastic, nonleukaemic bone marrow were allowed to recover before the second block. Consolidation was identical with NOPHO-AML 88. The 5-year EFS, DFS and OS in NOPHO-AML 93 were 48, 52 and 65%. The new NOPHO-AML protocol has been based on experiences from previous protocols with stratification of patients with regard to in vivo response and specific cytogenetic aberrations.

Influenza A among patients with human immunodeficiency virus: an outbreak of infection at a residential facility in New York City.

Although annual influenza vaccination is recommended for persons who are infected with human immunodeficiency virus (HIV), data are limited regarding the epidemiology of influenza or the effectiveness of influenza vaccination in this population. We investigated a 1996 outbreak of infection with influenza A at a residential facility for persons with AIDS. We interviewed 118 residents and employees, reviewed 65 resident medical records, and collected serum samples for measurement of influenza antibody titers. After controlling for history of smoking, influenza vaccination, and resident or employee status, in a multivariate model, HIV infection was not statistically associated with influenza-like illness (ILI). Symptoms and duration of ILI were similar for most HIV-infected and HIV-uninfected persons. However, 8 (21.1%) of 38 HIV-infected persons with ILI (vs. none of 15 HIV-uninfected persons) were either hospitalized, evaluated in an emergency room, or had ILI lasting > or = 14 days (P=.06). Vaccination effectiveness (VE) was similar for HIV-infected and HIV-uninfected persons. Vaccination was most effective among HIV-infected persons with CD4 cell counts of >100 cells/microL (VE, 65%; 95% CI, 36%--81%) or HIV type 1 virus load of <30,000 copies/mL (VE, 52%; 95% CI, 11%--75%). Providers should continue to offer influenza vaccination to HIV-infected persons.

Fate of nitrogen released from 15N-labeled litter in European beech forests.

The decomposition and fate of 15N-labeled beech litter was monitored in three European beech (Fagus sylvatica L.) forests (Aubure, France; Ebrach, Germany; and Collelongo, Italy) for 3 years. Circular plots around single beech trees were isolated from roots of neighboring trees by soil trenching, and annual litterfall was replaced by 15N-labeled litter. Nitrogen was continuously released from the decomposing litter. However, over a 2-year period, this release was balanced by the incorporation of exogenous N. Released N accumulated mainly at the soil surface and in the topsoil. Microbial biomass remained almost constant during the experiment at all sites except for considerably lower values at Ebrach. The 15N enrichment of the microbial biomass increased strongly during the first year and then remained stable. The 15N released from the decomposing litter was rapidly detected in roots and leaves of the beech trees, increasing regularly and linearly over the course of the experiment. The uptake of litter-released 15N by the trees was reduced under conditions that reduced tree growth. Under these conditions, leaves and fine roots were the dominant N sinks, and little N was allocated to other plant parts. By contrast, N uptake and N allocation from leaves to stem and bark tissues increased when tree growth was enhanced. Budgets for 15N showed that 2 to 4% of litter-released N was incorporated into the trees, about 35% remained in the litter and about 50% reached the topsoil.

[Acute pyelonephritis in children with minimal or normal urine findings]. / Akutt pyelonefritt ved sparsomme eller normale urinfunn hos barn.

BACKGROUND: Over the past years we have seen several children with serious bacterial infections in whom the focus of the infection was found in the kidneys despite of normal or minimal urine findings. MATERIAL AND METHODS: We review the cases of all children hospitalised at our paediatric department from 1995-2000 with normal or minimal urine findings, and in whom acute pyelonephritis was diagnosed by means of computerized tomographic imaging of the kidneys. RESULTS: All 10 children (aged 9 months to 9 years) had focal areas of decreased enhancement in the kidney parenchyma. All received intravenous antibiotic treatment. Vesicouretheral reflux was detected in four patients and required surgical intervention in one. One patient had considerable renal scarring and decreased renal function on dimercaptosuccinic acid (DMSA) scintigraphy. INTERPRETATION: In children with suspected serious bacterial infection, it is important to have the possibility of renal infection in mind in spite of normal urine findings. The importance of establishing the right diagnosis lies in the fact that patients with pyelonephritis need further investigation and follow-up.

Immune thrombocytopenic purpura in childhood in Norway: a prospective, population-based registration.

A prospective, population-based registration of children with immune thrombocytopenic purpura (ITP) was performed in Norway in 1996 and 1997. Ninety-two cases were identified, indicating an incidence of 5.3 per 100,000 children under 15 years. The sex ratio (female/male) was 1.2/1. Fifty-six percent presented with cutaneous signs only. The lowest platelet count was < 20 x 10(9)/L in 91%. In spite of mild bleeding symptoms, medical treatment was given in 68%, in most cases (57/63) with intravenous immunoglobulin. A total of 41/44 patients with platelet counts of < or = 5 x 10(9)/L were treated, regardless of whether they had mucous bleedings or not. Eighteen percent had platelet counts < 150 x 10(9)/L at 6 months, and 9% at 12 months following diagnosis. One patient with therapy-resistant chronic ITP died 16 months after diagnosis from an anesthesia complication related to profound epistaxis. This study shows a relatively high incidence. As in other studies, there was a tendency to treat platelet counts rather than bleeding symptoms.

[Kawasaki disease]. / Kawasakis syndrom.

Kawasaki syndrome was first described in 1967. The diagnosis is based on clinical criteria including fever for more than five days, conjunctivitis, lymph node enlargement and changes in the oral mucosa as well as in the palms and soles. Coronary complications including aneurysmal dilatation have been reported in 20-25% of untreated patients. Kawasaki syndrome is now the most common cause of acquired heart disease in children. Six patients were seen in our department during the period 1980-99. Five were treated with intravenous immunoglobulin. All of them showed immediate improvement of their general condition and were afebrile after 24-48 hours. One patient had a coronary aneurysm of moderate size, and four patients had signs of coronary vasculitis. All coronary changes were normalized after a maximum of 17 months. An update on Kawasaki disease is presented.

Decomposition of 15N-labelled beech litter and fate of nitrogen derived from litter in a beech forest.

The decomposition and the fate of 15N- labelled beech litter was monitored in a beech forest (Vosges mountains, France) over 3 years. Circular plots around beech trees were isolated from neighbouring tree roots by soil trenching. After removal of the litter layer, 15N-labelled litter was distributed on the soil. Samples [labelled litter, soil (0-15 cm depths], fine roots, mycorrhizal root tips, leaves) were collected during the subsequent vegetation periods and analysed for total N and 15N concentration. Mass loss of the 15N-labelled litter was estimated using mass loss data from a litterbag experiment set up at the field site. An initial and rapid release of soluble N from the decomposing litter was balanced by the incorporation of exogenous N into the litter. Fungal N accounted for approximately 35% of the N incorporation. Over 2 years, litter N was continuously released and rates of N and mass loss were equivalent, while litter N was preferentially lost during the 3rd year. Released 15N accumulated essentially at the soil surface. 15N from the decomposing litter was rapidly (i.e. in 6 months) detected in roots and beech leaves and its level increased regularly and linearly over the course of the labelling experiment. After 3 years, about 2% of the original litter N had accumulated in the trees. 15N budgets indicated that soluble N was the main source for soil microbial biomass. Nitrogen accumulated in storage compounds was the main source of leaf N, while soil organic N was the main source of mycorrhizal N. Use of 15N-labelled beech litter as decomposing substrate allowed assessment of the fate of litter N in the soil and tree N pools in a beech forest on different time scales.