Subcutaneous infusion ports in the pediatric patient with hemophilia.

Central venous access devices have become important tools in the management of pediatric patients with malnutrition, malignancy, and infections requiring long-term antibiotic treatment. Hemophilia presents a lifetime challenge for venous access and at times can be an urgent or life-threatening situation. Since 1986, the authors have implanted 11 subcutaneous infusion ports in nine patients with hemophilia. The systems have remained in place for up to 7 years, without major complications or problems. Two catheters were replaced, after 4 and 6 years, because of skin erosion and blockage, respectively. One catheter was removed after 7 years because of blockage following local trauma and was not replaced. A recent survey through the Canadian Hemophilia Centre Directors Group obtained a further 45 subcutaneous infusion ports in other centers across Canada. The benefits of this system are overwhelming enthusiasm by the parents and children and no major complications. Some of the patients are now HIV-positive and are able to use their system for ongoing drug therapy.

Immunosuppressive measles encephalitis.

A case of immunosuppressive measles (rubeola) encephalitis in a 12-year-old boy in remission from acute lymphoblastic leukemia is described. The patient presented with focal seizures which led to epilepsia partialis continua and then progressive obtundation. Magnetic resonance imaging revealed focal abnormalities, predominantly in the cortex, that on light and electron microscopic examination were demonstrated to be highly localized areas of neuronal loss, gliosis, and secondary Wallerian degeneration with paramyxovirus inclusions in the oligodendrocytes and surviving neurons.

Management of children with acute lymphoblastic leukemia by the Dana-Farber Cancer Institute protocols. An update of the Ontario experience.

The primary purpose of this study was to determine the therapeutic efficacy of a protocol of treatment for acute lymphoblastic leukemia (ALL) in children. A prospective approach was adopted with an inception cohort of patients. Outcome measures were assessed on December 31, 1990. The study was conducted at two tertiary care centres (pediatric oncology programs) in Ontario, Canada. All children with ALL were eligible for study and consecutive recruitment took place between May 1984 and July 1987. They were classified at diagnosis into one of three categories for risk of relapse according to standardized criteria. Thirty-nine children were designated as having standard risk (SR), 31 as having high risk (HR), and 12 as having very high risk (VHR) disease. All patients are included in the analysis. Treatment was administered according to risk category-specific chemotherapy protocols, the details of which have been published. A distinguishing feature of these strategies is the intensive use of intramuscular L-asparaginase. Patients remained on these regimens for 2 years or until relapse or toxic death (events) ensued. Total and event-free survival data were determined by life-table analysis (Kaplan-Meier plots). With a minimum interval from diagnosis of 186 weeks and a median interval exceeding 5 years, the cumulative proportion of the entire cohort (C) surviving is 85% [95% confidence interval (CI), 77-93%]. For the respective risk groups, the corresponding proportions are SR 94% (95% CI, 87-100%), HR 74% (95% CI, 59-89%), and VHR 81% (95% CI, 59-100%).(ABSTRACT TRUNCATED AT 250 WORDS)

Management of Ontario children with acute lymphoblastic leukemia by the Dana-Farber Cancer Institute protocols.

There is ample evidence of the value of intensive therapeutic strategies in the management of acute lymphoblastic leukemia (ALL), the commonest form of malignant disease in children. Such a program, devised at the Dana-Farber Cancer Institute (DFCI), Boston, and incorporating high-dose L-asparaginase, was adopted in 1984 by the Children's Hospital at Chedoke-McMaster, Hamilton, Ont., and the Children's Hospital of Western Ontario, London. We describe the experience of these institutions in the treatment of 82 children with ALL, 19 of whom were switched to the DFCI protocols while in continuing first remission with other treatment programs to complete a minimum of 2 years of maintenance therapy; the remaining 63 children, who had recently diagnosed disease, were consecutively enrolled in the DFCI protocols. Each child was assigned at diagnosis to a category of risk for relapse and treated accordingly. There were no remission induction failures or deaths due to induction therapy among the patients with newly diagnosed disease. There were no differences in total or event-free survival rates between the patients in Hamilton and those in London or between those whose protocols were switched and those who were treated from the beginning with the DFCI protocols. With a median follow-up interval of 144 weeks the total survival rate was 95% and the event-free survival rate 88%. For patients at standard risk of relapse the event-free survival rate was 100%, for those at high risk the rate was 82%, and for those at very high risk the rate was 67%. If infants (all of whom suffered a relapse) are excluded from the last category the rate was 89%. These results were achieved with moderate toxic effects (except for two deaths, one of which was due to a therapeutic misadventure) and suggest that the prospect for cure in children with ALL. may now approximate 80%, a degree of success that demands that consideration be given to reducing total therapy, at least for children with standard-risk disease. Further follow-up will determine whether these high event-free survival rates will stabilize and meet the criteria for cure.

Increased activity on immediate phase of bone imaging does not always indicate increased blood volume.

The immediate Tc-99m MDP image of a neoplastic right wrist mass in a 10-year-old boy demonstrated intense focal accumulation that suggested a very vascular tumor, but delayed images showed only slightly increased activity in the mass. A Tc-99m labeled RBC study was performed to permit whole-body blood pool imaging for other vascular lesions, and it did not demonstrate an increased blood volume in the mass. Biopsy showed the tumor to be a Ewing's sarcoma, probably of soft tissue origin. The sarcoma was not very vascular. The intense immediate accumulation of Tc-99m MDP in this case may be attributed to increased microvascular permeability.