Spatial clustering of childhood acute lymphoblastic leukaemia in hungary.

The aetiology of childhood acute lymphoblastic leukaemia has been linked with spatially heterogeneous environmental exposures. The presence of spatial clustering would be consistent with geographically localized environmental exposures over long periods of time. The present study is the first to examine spatial clustering amongst children aged 0-4 years using population-based data from Hungary. The data set consisted of 134 children diagnosed with acute lymphoblastic leukaemia who were resident in part of Hungary during the period 1981-2000. Two levels of spatial aggregation were examined: counties and settlements. The Potthoff-Whittinghill and Moran I autocorrelation methods were used to test for spatial clustering. Additionally, an evaluation of the environmental changes during the study period was considered. Specifically analyses were carried out on sub-periods to investigate a possible effect of the Chernobyl catastrophe. There was statistically significant spatial clustering both at the county (estimate of extra-Poisson variation [Formula: see text], P = 0.04) and settlement levels (estimate of extra-Poisson variation [Formula: see text], P = 0.0003). At county level, the finding was attributable to clustering amongst female cases, but at settlement level, the finding was limited to male cases. There was significant spatial autocorrelation in the sub-periods immediately following the accident (1986-1990 & 1991-1995), but not before 1986, nor after 1995. A significant autocorrelation was observed during the 5 year period immediately following the accident (1986-1990, global Moran I = 0.1334, p = 0.005). The centre of significant excesses of ALL cases was located in the county of Baranya. Our study is consistent with an environmental aetiology for acute lymphoblastic leukaemia in children associated with constant exposure to an, as yet unknown, environmental factor in small geographical areas. Although a possible effect of the Chernobyl accident was found in the autocorrelation analysis, the role of chance cannot be excluded.

Hungarian experience with Langerhans cell histiocytosis in childhood.

The Langerhans cell histiocytosis (LCH) in children is relatively rare and the long-term analysis of therapy results has not been done yet in Hungary. The aim of this study was to investigate the incidence, clinical features, prognostic risk factors, and treatment results of children's LCH in Hungary in a 20-year period. Children less than 18 years of age with newly diagnosed LCH in Hungary were entered in this study. Clinical data of all children with LCH were reported to the National Childhood Cancer Registry in Hungary from 1981 to 2000. The clinical files were collected and abstracted for information regarding age at diagnosis, gender, disease characteristics, treatment, and outcome of treatment. Median follow-up duration of surviving patients is 10.98 years. Between January 1981 and December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The annual incidence of LCH in children younger than 18 years of age was 2.24/million children. The male-female ratio was 1.36:1; the mean age was 4 years 11 months. Thirty-eight children had localized disease and in 73 cases systemic dissemination was found already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation, and 5 children got only local irradiation. In 2 cases remission was achieved with local steroid administration. Seventy-five patients received chemotherapy. In the 20 years of the study 14 children died, 9 due to the progression of the disease. Sixteen patients had relapse with a mean of 2.16 +/- 1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n = 111) was 88.3 +/- 3.1% at 5 years and 87.3 +/- 3.2% at 10 and 20 years. Childhood LCH is a well-treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

Adenocarcinoma of the colon developing on the basis of Crohn's disease in childhood.

Colorectal carcinoma rarely affects children and has a dismal prognosis with 5-year survival rates as low as 2.5%-7% despite apparently radical surgery. Here we report the case of an adenocarcinoma of the sigmoid colon in a 15-year-old girl preceded by uncertain abdominal complaints of 5 years' duration. Pathological work-up revealed a tumour with lymph node metastases (pT3NI). Immunohistochemical evidence of p53 overexpression by the tumour cells raised the suspicion of an underlying Li-Fraumeni syndrome. In addition, there were aphthoid ulceration, fissuration of the non-tumorous mucosa, along with a mixed transmural infiltrate composed of macrophages, eosinophils, and non-typical giant cells, which were compatible with simultaneous Crohn's disease. Anamnestic data concerning the occurrence of idiopathic inflammatory bowel disease or colorectal carcinoma in the patient's relatives were non-contributory. The present results suggest a possible relationship between Crohn's disease and colon cancer due to the defective p53 gene product.

[Hypoxic myoglobinemia in severe neonatal disorders of adaptation to extrauterine life]. / Hypoxiás myoglobinaemia vizsgálata az újszülöttek extrauterin élethez való súlyos adaptációs zavaraiban.

In two series of newborns needing intensive care the presence, the degree and the prognostic value of myoglobinaemia was examined. In series I. of hypoxic newborns the myoglobinaemia was present even in infants requiring less than 60% O2 therapy. The serum myoglobin value was significantly higher in cases needing oxygen therapy over 60% oxygen. This was most pronounced in the critically severe and progressive cases. In series II. of 34 consecutive cases of hypoxic newborns exceeding 7 nM/l proved to have a prognostic value indicating critically severe course or fatal outcome of the disease. The myoglobinaemia observed in the present study may explain the effectivity of the peritoneal dialysis therapy introduced previously by us in severe hypoxic newborns. This possibility was supported by further observations on the transperitoneal passage of myoglobin in 4 renal hilus ligated and peritoneally dialyzed newborn piglets. In conclusion, early detection of the elevated myoglobinaemia in severely hypoxic newborns has a definite prognostic value and its degree can be used in the indication of peritoneal dialysis.

Active immunization of children exposed to varicella infection in a hospital ward using live attenuated varicella vaccine given subcutaneously or intracutaneously.

Active immunization using Takahashi OKA live attenuated varicella vaccine was carried out fire 5 times to prevent the spread of "imported" varicella in a hospital ward. Susceptibility was previously tested by serological examinations: 14 children were vaccinated subcutaneously, the other 19 received the vaccine intracutaneously. Vaccination within a few days following exposure provided complete immunity in the great majority of cases. Intracutaneous administration was nearly as protective as the subcutaneous one.