Prevention of infection in children with acute leukaemia. No major difference between total and selective bowel decontamination.

To evaluate the effect of total bowel decontamination (TD) and selective bowel decontamination (SD) in a non-protective environment clinical and laboratory data of children treated for acute leukaemia between 1983 and 1991 were analysed retrospectively. From 1983 until 1989 34 patients [18 acute non-lymphoblastic leukaemia (ANLL) patients, 16 acute lymphoblastic leukaemia (ALL) patients] received TD and 31 patients (8 ANLL patients, 23 ALL patients) received SD from 1987 until 1991. TD consisted of colistin sulphate, neomycin, cephaloridine and amphotericin B orally as well as Orabase and sterilized food, while the patients were nursed in a single room. SD consisted of oral colistin sulphate, neomycin and amphotericin B. Those patients with ANLL were nursed in a single room; patients with ALL were nursed in a single room during remission induction therapy only. All patients except those with ANLL receiving TD received Pneumocystis carinii pneumonia prophylaxis with cotrimoxazole. Because the two groups were heterogeneous for diagnosis and chemotherapy the occurrence of fever (central body temperature at least 38.5 degrees C) and major infections (septicaemia of infections of the deep tissues or organs) were registered during periods of neutropenia (neutrophilic granulocytes < or = 500/mm3 for at least 8 days). Patients on TD had 55 periods of neutropenia, patients on SD 80. Patients on TD had 89.1 periods of fever/100 periods of neutropenia whereas patients on SD had 56.3. Also patients on TD had 27.3 major infections/100 periods of neutropenia whereas patients on SD had 11.3. Major infections predominantly consisted of septicaemia caused by gram-positive bacteria. We conclude that, in this study, TD in a non-protective environment does not offer better protection against major infections that SD in patients with ALL or ANLL.

Cytogenetics of three cases of ANLL M2 and M4. Involvement of chromosomal region 8q22 in all three but 21q22 in only one.

Cytogenetic investigation of the bone marrow of two patients with acute nonlymphocytic leukemia (ANLL), French-American-British Cooperative group (FAB) classification M2, revealed a translocation (8;22)(q22.1;q13.3), without involving chromosome 21, and a variant translocation (8;21)(q22;q22). These findings, together with a del(8)(q22) found in a patient with refractory anemia, erythroblastic (RAEB)-t with progression to acute myelogenous leukemia (AML)-M4, are discussed in relation to the possible role of abnormalities of chromosomes 8 and 21 in the oncogenesis of ANLL M2 and M4.

Dedifferentiated parosteal osteosarcoma of the femur with aneuploidy and lung metastases.

In this report, the pathologic findings and the results of cellular DNA measurements of a tumor that on first presentation seemed to be a classical parosteal osteosarcoma are described. After resection 8 months later, part of the tumor appeared to display highly malignant features. DNA flow cytometry of this part of the tumor showed an aneuploid cell population. The aggressive nature of the tumor was confirmed by the development of lung metastases approximately 1 year after resection of the primary tumor.

The Aase syndrome. Case report and review of the literature.

A 5-month-old boy with congenital hypoplastic anaemia and triphalangeal thumbs, known as the Aase syndrome, is described. In addition he had unilateral cleft lip and palate and abnormal dermatoglyphics. Only ten cases have been reported previously; these are reviewed. This case is the third patient reported to have the Aase syndrome who also has a cleft lip. Bone marrow cultures failed to stimulate production of erythropoietic precursors.

[A newborn infant with the xxxxy-syndrome]. / Een pasgeborene met het XXXXY-syndroom.

In a three weeks old infant with dysmorphic features a 49, XXXXY karyotype was demonstrated from chromosome preparations of lymphocytes. In the literature only a few newborn infants have been described with this syndrome. The most frequent symptoms of the syndrome in older patients are mental retardation, dysmorphic signs, hypogonadism and skeletal malformations. In our patient we found a low birth weight, a peculiar facies, in addition to a patent ductus arteriosus, a scoliosis and normal external genitals. The most typical skeletal malformations may develop at a more advanced age.