[Electrolyte changes in acute leukemia in childhood]. / Elektrolytveränderungen bei der akuten Leukämie im Kindesalter.

Electrolyte imbalance in leukemia can be due to either organ infiltration and cell death or to a side effect of cytostatic drugs. From the wide variety of these disturbances seen in acute leukemias in childhood, the excess of potassium is most dangerous. Further electrolyte changes, which are however less evident, are hyperphosphataemia, hyperphosphaturia, and hypocalcaemia. The destruction of a large amount of cells during aggressive induction therapy can boost the electrolyte imbalance and therefore lead to renal failure. Such situations are demonstrated in two cases. Following Vincristine and Cyclophosphamide administration, electrolyte changes such as acute or prolonged decrease of sodium in the serum and urinary loss of sodium are seen frequently. Based on the data from 20 patients with acute lymphoblastic leukemias we describe the dynamics of this process. These changes are probably caused by the syndrome of inadequate ADH-secretion. The clinical importance of these findings are discussed and procedures for improving therapy are set out.

Acute respiratory failure after intrathecal methotrexate administration.

This is a report of a case of acute respiratory failure following the administration of intrathecal methotrexate (MTX) for prophylaxis of central nervous system leukemia (CNS) in a 3-year-old girl with acute lymphoblastic leukemia. This could be attributed to acute metabolic or direct toxic effects of methotrexate to the central nervous system tissue. Although the specific agent responsible for this complication following intrathecal injection of MTX is unclear, it is worthwhile to describe this event.

[The Netherton syndrome: clinical characteristics, differential diagnosis and new ways of therapy]. / Das Netherton-Syndrom: Klinische Charakteristik, differential-diagnostische Abgrenzung und neue Wege der Therapie.

The Netherton-syndrome is a rare disease which is probably inherited through an autosomal recessive trait. It is defined by a triad of symptoms: congenital ichthyosiform erythrodermia , trichorrhexis invaginata et nodosa ("bamboo hair") and atopy. Additional disorders affect the immune system, the metabolism of amino acids and the physical development. On the basis of a new case, the cellular immune defect and the genetic background of the disease are more clearly defined. A new form of treatment--a combination of photochemotherapy (PUVA) and systematic application of aromatic retinoid--has so far proved to be successful. In order to establish an accurate diagnosis--a prerequisite for this promising therapeutic approach--diseases with similar symptoms are discussed for comparison.

[Veno-occlusive disease of the liver as a treatment complication in children with Wilm's tumor]. / Veno-occlusive disease der Leber als Behandlungskomplikation bei Kindern mit Wilmstumor.

We report on three children aged 1 1/2, 2 and 9 1/2 years with Wilms' tumor, who developed a tender hepatomegaly and ascites associated with elevated liver enzymes, anemia and thrombocytopenia during chemotherapy. This clinical picture and liver sonography abnormality are best explained by veno-occlusive disease (VOD) of the liver, while other causes of liver disease could not be identified. Actinomycin D dosage was 0.045 mg/kg as bolus injection in two patients and 0.075 mg/kg split over five days in a third patient. Presumable, this drug was the causative agent. VOD was observed after preoperative and postoperative chemotherapy. No child had received abdominal irradiation. The authors comment on the influence of Actinomycin D as the cause for this unusual liver toxicity.

Engraftment failure following bone marrow transplantation in children with thalassemia major using busulfan and cyclophosphamide conditioning.

Thirteen children older than 3 years of age with beta-thalassemia major underwent allogeneic bone marrow transplantation (BMT) from a full human leukocyte antigen (HLA) matched sibling donor in a single institution. These patients received busulfan (Bu). 16 mg/kg followed by cyclophosphamide (Cy) 200 mg/kg for conditioning. Eight of the 13 patients (Group 1) engrafted and have a median age of 13 years (range 5-15 years). The five patients (Group 2) who failed to engraft have a median age of 6 years (range 3-8 years). The association with the following factors was found to be statistically significant: age (older in Group 1), duration of nadir of white blood count (WBC) of < or = .1 x 10(9)/L (longer in Group 1), and the dose of Bu administered to each patient calculated on the basis of body surface area (higher dose in Group 1). The high rate of engraftment failure (5 out of 13) may be related to the suboptimal systemic exposure of Bu in younger children leading to inadequate bone marrow ablation when the standard dose of 16 mg/kg is used.

Bone marrow transplantation in patients with Fanconi anemia: experience with cyclophosphamide and total body irradiation conditioning regimen.

Eleven patients with Fanconi anemia (FA) underwent bone marrow transplantation (BMT) between March 1985 and May 1990 in a single institution. Ten patients received bone marrow from healthy full human leukocyte antigen (HLA) matched siblings and one patient from her father (one antigen mismatch). Ten patients were conditioned with cyclophosphamide (Cy) at a dose of 5 mg/kg per day for 4 days followed by total body irradiation (TBI) for a total of 600 cGy over 3 days. Six of the 11 patients are alive and have normal reconstitution of their bone marrow. Median follow-up was 72 months (range 42-84). Three of the 10 patients who received Cy and TBI (two HLA compatible, one antigen mismatch) had graft failure. Five patients developed at least grade III acute graft-versus-host disease (GVHD). The rates of graft failure and GVHD are, however, still significantly high. Modification of the conditioning regimen and GVHD prophylaxis is needed to improve the outcome.