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Notes: Abstract  Pulmonary thromboembolism (PTE) in leukemic children undergoing intensive chemotherapy should be promptly recognized so that specific therapy can be started. Our experience with the two cases reported here has led us to propose guidelines for the treatment of initial PTE in a pediatric hematology unit. Two children with leukemia developed PTE, the first during the relapse for acute lymphoblastic leukemia and the second at the onset of acute promyelocytic leukemia. In both cases, the diagnosis of PTE was based on clinical assessment of sudden acute respiratory failure with positive pulmonary perfusional scintigraphy in spite of a negative chest X-ray. The subintensive supervision consisted of instrumental monitoring with the assistance of an intensive care anesthetist. The clinical monitoring was based on the serial registration of respiratory rate, cardiac rate, SaO2 and body temperature. The thrombolytic therapy, together with heparin prophylaxis, was successfully administered in the hematology ward without the need for intensive care support (i.e. mechanical ventilation). The success of the treatment was documented by the criterion of a return to the normal cardiorespiratory parameters a few hours after the start of the thrombolytic treatment. Furthermore, a chest CT scan in case 1 and an arteriography in case 2 confirmed the PTE-related hypoperfusion. On the basis of this experience, the authors point out that in the course of acute respiratory failure in leukemic children, the combination of a negative chest X-ray and a positive pulmonary perfusional scintigraphy (compared whenever possible with ventilatory scintigraphy) in the presence of a negative CT scan could be a reliable diagnostic tool for PTE. This pathology should be treated promptly and with specific therapy to avoid progression to a severe, massive PTE.

Notes: Summary Plasma pharmacokinetics of VP16-213 were investigated after a 30–60 min infusion in 14 adult patients and six children. In adults the elimination half-life (T1/2 β), plasma clearance (Clp) and volume of distribution (Vd) were respectively 7.05±0.67 h, 26.8±2.4 ml/min/m2, and 15.7±1.8 l/m2; in children 3.37±0.5 h, 39.34±6.6 ml/min/m2, and 9.97±3.7 l/m2. After repeated daily doses no accumulation of VP16-213 was found in plasma. The unchanged drug found in the 24 h urine after administration amounted to 20–30% of the dose. In eight choriocarcinoma patients plasma levels of VP16-213 were measured after oral capsules and drinkable ampoules. The bioavailability compared to the i.v. route was variable, mean values being 57% for capsules and 91% for ampoules. In one further patient, with abnormal d-Xylose absorption results, VP16-213 was not detectable in plasma after the oral ampoule dose. Steady state levels investigated in three patients after 72 h continuous VP16-213 infusion (100 mg/m2/24 h) were around 2–5 μg/ml. Levels of VP16-213 were undetectable in CSF after i.v. or oral administration.

Notes: [Auszug] Figure 1 shows the titration of 0-globin sequences in DNA from a normal human, a case of Hb Lepore, a southern Italian patient with newly discovered d thalassaemia and two patients with HPFH, Negro type (all homozygotes). The cDNA3 probe used was a full-size band eluted from an agarose gel (see ...

Notes: Abstract  The aim of this study was to compare the Hickman and Groshong central venous catheters (CVCs) for incidence and severity of catheter-related complications in children. Seventy-three patients with hematological malignancies were observed, 42 with Groshong CVCs and 31 with Hickman CVCs. The number of infective episodes per 100 CVC-days was not significantly different (0.25 in the Hickman group versus 0.13 in the Groshong group;P=0.24). The most frequent type of CVC-related infection in both groups was microbiologically documented sepsis; in most cases Gram-positive bacteria were isolated. Neutropenia (P〈0.001 for both CVCs) and hospital CVC management (P=0.0047 for the Hickman group, P〈0.001 for the Groshong group) emerged as the major risk factors for the outbreak of infections. The rate of mechanical complication episodes per 100 CVC-days was similar in both groups (1.01 in the Hickman group versus 1.1 in the Groshong group;P=0.58). Some complications (fissures, ruptures, total lumen obstruction by clots) occurred only in the Groshong group. Our study did not demonstrate any statistically significant difference in the incidence of mechanical and infective CVC-related complications between these two types of catheter.

Notes: Abstract This study explores the psychosocial problems experienced by families with children aged 6 to 14 years suffering from β-thalassemia major (N=188). The psychosocial problems and the family's adjustment to the effects of the illness were compared across a number of cultures where the disease is prevalent, namely Cyprus, Greece, and Italy. A small number of migrant children.in the United Kingdom was also included in the study. Semi-structured interviews were conducted with parents who also completed the Rutter Parental Questionnaire and the Goldberg General Health Questionnaire. Teachers were asked to complete a Children's Behaviour Questionnaire designed by Rutter. In all countries the disease seemed to have a binding effect on the family, thus mobilizing adaptive mechanisms. Father's low education level and the presence of major medical complications were predictors of poor family adjustment. Differences between and within countries may well reflect differences in health policies, existing level of socio-economic development, and in the cultural patterns in coping with a chronic illness.

Notes: Abstract Background: Childhood Hodgkin's disease (HD) in low-income countrieshas been reported to have distinct presenting features, including a highprevalence of the mixed cellularity subtype, which also seems to be associatedwith poorer prognosis. Further investigations are needed to evaluate theseissues. Another controversial aspect of childhood HD is the use ofradiotherapy (RT) in its treatment and the growing concern about its seriousadverse side effects. In this paper, data on the diagnosis and outcome ofchildren treated without RT in a low-income country (Nicaragua) are reported. Patients and methods: Forty-eight consecutive children aged0–15 years, diagnosed at ‘La Mascota’ Hospital of Managua (Nicaragua)from January 1990 to October 1995, entered this study. Follow-up was updatedin May 1996. Clinical and histopathological staging was performed accordingto Ann Arbor and Rye criteria, respectively. Treatment consisted of COPP (sixcycles) for stages I or IIA, or COPP-ABV (hybrid): eight cycles for stages IIBor III, and 10 cycles for stage IV. Total cumulative doses of adriamycin andbleomycin in this protocol are, respectively, 200 and 80 mg/sqm for stages IIB or III and 250 and 100 mg/sqm for stage IV. Results: The median age of the 48 patients at diagnosis was sevenyears, and the mean age was 7.9 years (range 3–15 years). Clinicalstages were IA in 5, IIA in 9, IIB in 6, IIIA in 5, IIIB in 14, and IVB in 9.Histopathologically, 25 cases presented with mixed cellularity, 15 withnodular sclerosis, 5 with lymphocytic predominance and 3 with lymphocyticdepletion. Four patients did not proceed with treatment and were lost tofollow-up. Two patients (stages IIIB and IVB), who never achieved completeremission (CR) during treatment, presented progressive disease at the end ofthe scheduled chemotherapy. The remaining 42 patients were in completeremission at the end of chemotherapy. Following discontinuation of therapy,one patient (stage IA) was lost to follow-up and two patients with stage IIIB,who were in CR after the second chemotherapy cycle, relapsed 20 and 9 monthsfollowing the diagnosis. EFS at three years is 100% for the 25 patientswith stages I, II, IIIA and 74.9% for the 23 patients with stages IIIBor IV. Conclusion: The presenting features found in these patients aresimilar to those reported from other low-income countries. In our experience,however, the high prevalence of the mixed cellularity subtype was notassociated with poorer prognosis. Satisfactory results have been achieved inpatients with stages I, II or IIIA HD using COPP or COPP-ABV (hybrid) regimenswithout RT. The treatment was also well tolerated and can thus be recommendedfor these patients in low-income countries, where RT facilities may be scarceor unavailable. The use of more aggressive treatment schedules and/or RT oninvolved fields in front-line treatment may, however, be needed for the moreadvanced stages IIIB or IV. Large studies with adequate follow-up are neededto evaluate whether, if RT is omitted, higher cumulative doses of more toxicdrugs are required and thus compare the long-term toxic effects of differenttreatment modalities.