[Acute leukemia in children with Down syndrome: analysis of cases]. / Ostra bialaczka u dzieci z zespolem Downa--analiza przypadków wlasnych.

An analysis of clinical and laboratory parameters and the results of treatment of 14 children with Down Syndrome and acute leukaemia was performed. The children were treated between 1986-1997. Their age ranged from 1 day to 13 years (average 5.5). There were 9 girls and 5 boys. Four of them had congenital heart disease. ALL was observed in 10, AML in 3 and TAM (Transient Abnormal Myelopoesis) in 1. Half of the children with ALL was classified as L1 according to FAB with the majority of common phenotypes and M6 in ANLL group. Remission was achieved in all ALL patients, six of them are still free of symptoms, the remaining four died of brain haemorrhage as a consequence of myelosuppression. Only 1 of 3 children with ANLL achieved remission. The child died of cardiac arrest after induction phase of BFM 95 programme (ADE). The 2 remaining children with ANLL also died of circulation failure before initiation of chemotherapy. The children had complicated cyanotic heart disease. The neonate with TAM is in clinical and hematological remission. In conclusion all children with ALL achieved hematological remission but tolerance of treatment was a problem. The majority of patients had diminished bone marrow reserve. Mortality was frequently related to circulatory failure in children with associated heart defects. It seems necessary to discuss the modification of accepted programmes for leukemia for the treatment of children with Down Syndrome.

[Acute pancreatitis in children with acute lymphoblastic leukemia treated with L-asparaginase]. / Ostre zapalenie trzustki u dzieci z ostra bialaczka limfoblastycana leczonych L-asparaginaza.

The treatment of the acute lymphoblastic leukemia in childhood includes frequent administration of L-asparaginase by intravenous route. L-asparaginase is an enzyme produced by E. coli and Erwinia chrysanthemi strains. Adverse reactions produced by L-asparaginase are numerous, and pancreatitis is being the most severe. Children with the acute lymphoblastic leukemia were followed up for 2 years. Hyperglycaemia and glycosuria were noted in 10% of them resulting in L-asparaginase cessation or replacement by less toxic agents. The acute pancreatitis was produced in 8% of the patients, and was treated typically.

[Histiocytosis X in children--course of the disease and treatment]. / Histiocytosis X u dzieci--przebieg choroby i leczenie.

A clinical analysis was carried out of the course of the disseminated form of the disease in 20 children aged from one month to 8 years (mean age 11 months). Unfavourable prognostic significance was confirmed of such factors as: child's age at the time of falling ill lower than two years, number of the involved organs and their dysfunction, and lacking reaction to treatment during the first three months of the disease.

[Preliminary experience with achieving immune tolerance in patients with hemophilia A and circulating factor VIII inhibitor]. / Wstepne doswiadczenia w uzyskiwaniu tolerancji immunologicznej u chorych na hemofilie A z krazacym antykoagulantem.

The appearance of antibodies to factor VIII is a serious complication in the treatment of haemophilia A. A protocol is presented aiming at suppression of the anticoagulant using continued treatment with factor VIII (25 u/kg b.w. twice weekly). Ten patients with previous maximal factor VIII antibody titre from 7 to 2900 Bethesda units were treated. So far the inhibitor was eradicated in 3 cases treated continuously for from 5 to 20 months. In two of these three cases successful surgical procedures were performed without anamnestic response.