Hana Hrstková

Late anthracycline cardiotoxicity protection by dexrazoxane (ICRF-187) in pediatric patients: echocardiographic follow-up.

BackgroundThe authors conducted a retrospective study to determine whether dexrazoxane (ICRF-187) would reduce late anthracycline-induced cardiotoxicity in patients treated in childhood for hematological malignancy.Patients and methodsThe authors examined 108 patients (63 male, 45 female) 5–29 years old, (median 15 years). All patients were in long-term remission of their malignancy. The cardioprotection was given to 68 patients (39 male, 29 female), and standard treatment was used in 40 patients (24 male, 16 female). Dexrazoxane (cardioxane, Chiron Company, The Netherlands) was given in 20:1 ratio to anthracycline. The follow-up time was 2–20 years (mean 7 years). The control group consisted of 41 volunteers (22 males, 19 females) 4–31 years old (median 18 years). The cardiotoxicity has been defined as the presence of heart failure or the decline of shortening fraction below 30% or ejection fraction (EF) below 55%. The end-systolic wall stress (ESS), myocardial performance index (MPI; Tei index), and parameters of left ventricular diastolic filling were also assessed.ResultsThe anthracycline cardiomyopathy with the presence of heart failure was diagnosed in only one patient treated with a standard regimen. The pathological decline of fractional shortening was present in three (5%) and six (15%) patients with and without cardioprotection given, respectively. Similarly, none of the patients with cardioprotection revealed a pathological value of EF, while four (10%) patients without cardioprotection showed an EF decrease. Finally, ESS and isovolumic relaxation time were pathologically increased in the group without cardioprotection in comparison to the controls and to the group with cardioprotection. However, the MPI was significantly increased in both groups of patients.ConclusionsDexrazoxane reduces the risk of late clinical and subclinical cardiotoxicity and does not affect the response rates to chemotherapy and overall survival during the median follow-up period of 7 years (follow-up period 2–20 years).

Long-term results of treatment of childhood acute lymphoblastic leukemia in the Czech Republic

Long-term results of treatment of childhood acute lymphoblastic leukemia in the Czech Republic

HUMAN HERPESVIRUS-6 INFECTION IN CHILDREN WITH CANCER

Pediatric cancer patients treated with multimodal therapy are at a great risk of opportunistic infections or reactivation of latent infections. Human herpesvirus-6 (HHV-6) can serve as an example of such infection, with high seroprevalence in population. In 66 children with cancer and in 45 healthy controls, age matched, the presence of DNA HHV-6 was examined in peripheral blood by the polymerase chain reaction method. HHV-6 serology was also performed. No difference has been found between patients at the time of cancer diagnosis and the group of healthy children in the presence of DNA HHV-6 in blood, 17.4 and 15.6%, respectively. During cytotoxic chemotherapy the presence of HHV-6 in peripheral blood raised to 37.1% in patients with fever. Other parameters and symptoms such as febrile neutropenia, lymphopenia, exanthem, hepatopathy, lymphadenopathy, enteritis, bone marrow aplasia, pneumonitis, and encephalitis were examined in both the HHV-6 positive and HHV-6 negative groups of pediatric cancer patients. Statistically significant differences (p < .05) were found in case of lymphopenia, exanthem, and hepatopathy. In 4 out of 66 patients (6.1%) severe HHV-6 infection has been found: in 3 patients during cytotoxic chemotherapy and in 1 at the time of cancer diagnosis. Reactivation of HHV-6 infection in pediatric cancer patients under treatment with cytotoxic chemotherapy is frequent and can lead to severe complications as described in patients after bone marrow or organ transplantation.

GASTRIC CARCINOMA IN A 9-YEAR-OLD BOY

Gastric carcinoma is an extremely rare cancer in children. A case is presented of a 9-year-old boy admitted to The University Hospital Brno with a 4-month history of abdominal pain, anorexia, weight loss, nausea, and vomiting. Several of his family members died from or have been treated for cancer. Barium meal examination performed 2 months prior to admission was nondiagnostic. When gastroendoscopy, laparoscopy, and abdominal computer to mography scan were performed, the diagnosis of adenocarcinoma of the stomach was established. The patient died 10 days after admission because of rapid cancer spread. Miliary metastases of the peritoneum, mesenterium, omentum, liver, bowels, lungs, heart, bone marrow, and skin werefound. No penetration through the hematoencephalic barrier was noticed.

Recombinant human interferon α-2a therapy in children with chronic immune thrombocytopenic purpura

Background In a prospective study, 11 children with chronic immune thrombocytopenic purpura between ages 3 and 18 years were treated with recombinant human interferon &agr;2a (rhIFN&agr;-2a). Patients and Methods A dose of 3 × 106 U/m2 three times weekly for 4 to 5 weeks (one cycle) was administered. Patients were treated with one to four cycles of rhIFN&agr;-2a, and the outcomes were measured initially and 18 to 30 months after the last cycle. Results Good therapeutic responses (defined as platelet count >100 × 109/L) lasting for 18 to 30 months from the last interferon cycle were achieved in 6 of the 11 (55%) patients, including one with a probable spontaneous remission. Fair responses (platelet count 31–60 × 109/L) for 18 months were achieved in 3 of the 11 (27%) patients. Only two patients, each treated only with one interferon cycle, exhibited no response. Side effects of treatment included fever and a flulike syndrome, which were usually present during the first 14 days of therapy only. Conclusions Interferon-&agr; appears to be an effective therapeutic approach to children with chronic immune thrombocytopenic purpura, with the potential of sustained long-term remission. A randomized, placebo-controlled study is needed to confirm its role in this population.

Acute lymphoblastic leukemia in infants: A decade of experience in the Czech Republic

Acute lymphoblastic leukemia (ALL) in infants is a rare disease. Particularly babies under 6 months of age and those with a t(4,11) have a poor prognosis early treatment failure being the rule. Here we report on the outcome of 28 infants with ALL treated in 1986-1995 by members of the Czech Pediatric Hematology Working Group.

TWENTY-FOUR-HOUR BLOOD PRESSURE PROFILE AND BAROREFLEX SENSITIVITY IN CHILDREN AND ADOLESCENTS WITH ESSENTIAL HYPERTENSION: P1.124

Citlivoszt baroreflexu vyjadřena v Hz na mmHg je signifikantně nižsi u děti s vysokou hodnotou systolickeho krevniho tlaku zjistěnou při ambulantnim 24 hodinovem monitorovani.

The development of LF/HF ratio and its dependence on the mean heart rate in children and adolescents

We have shown previously how the dependency of the baroreflex sensitivity on the inter-beat intervals (IBI) can cover up the developmental changes. Therefore we decided to analyse LF/HF ratio. We have calculated mean IBI and heart rate (HR) and their power spectra in 424 subjects (11 - 20 years) from the 5-min continuous blood pressure monitoring at rest. LF/HF ratios of IBI and HR spectra were determined. The statistic was done for a whole group (WG), and for subgroups of older children (C: 11 -1 5 years), adolescents (A:16-20 years), andfor 10 particular age-subgroups. In the WG and the A, we shown significant relationship between age and mean IBI or HR, but LF/HFIBI or LF/HFHR were age-independent. LF/HFIBI or LF/HFHR was also significantly age-dependent. Negative correlation of IBI vs. LF/HFIBIand a positive correlation of HR vs. LF/HFHR in the WG and in C or A was found. Relationship IBI vs. LF/HFIBI and HR vs. LF/HFHR were in particular age-subgroups from 11 to 14 years, but it was insignificant in subgroups from 15 to 20 years. Our analysis showed the increase of LF/HF ratio up to the age of 15 years when the development of sympathovagal balance seems to be finished. Strong relationship between IBI or HR and LF/HF ratios could cover the development changes.

CHANGES IN SYSTOLIC BLOOD PRESSURE AND PULSE INTERVAL VARIABILITY IN CHILDREN WITH TYPE 1 DIABETES MELLITUS: PP.27.363

Introduction: Polyneuropathy quite often accompanies diabetes mellitus and probably affects all organs. The aim of the study was to show differences in systolic blood pressure and heart rate variability in children with type 1 diabetes mellitus (T1DM). Methods: We examined 382 healthy (Co) children and adolescents (14.8 +- 2.6 years) and 34 patients with T1DM (15.0+- 3.2 years). We recorded systolic blood pressure (SBP) and pulse intervals (PI) beat-to-beat by non-invasive methods (Finapres) in all subjects for 5 min. The breathing was controlled by a metronome at a frequency of 0.33 Hz. The SBP and PI variability were determined as spectral power in the low frequency (LF) range (nSBPlf, nPIlf) and of respiratory (HF) range (nSBPhf, nPIhf) in normalised units, and in absolute values as well (vSBPlf, vPIlf, vSBPhf, vPIhf). Results: We did not find significant differences between controls and T1DM in SBP and PI variability LF range (nPIlf: Co=0.057 +- 0.031, T1DM=0.063 +- 0.027; nSBPlf: Co=0.046 +- 0.026, T1DM=0.046 +- 0.021; vPIlf: Co=15132 +- 14167, T1DM=16672 +- 16583 ms2/Hz; vSBPlf: Co=147 +- 119, T1DM=224 +- 242 mmHg2/Hz). However, there were significant differences in the HF range (nPIhf: Co=0.104 +- 0.064, T1DM=0.081 +- 0.056, p<0.05; nSBPhf: Co=0.058 +- 0.048, T1DM=0.035+-0.038, p<0.001; vPIhf: Co=30570+-39385, T1DM=17078+-16653 ms2/Hz, p<0.01; vSBPhf: Co=152+-145, T1DM=98+-63 mmHg2/Hz, p<0.05). Discussion: Decreased values of SBP and PI variability in children with T1DM in the high frequency region indicate early impairment of parasympathetic activity.

Wilson's Disease: Monocentric Experiences Over a Period of 16 Years

BACKGROUND Wilsons disease (WD) is an autosomal recessive disorder of copper metabolism. The objective of this study is to present diagnostic pitfalls and long time follow-up data in Wilson disease. PATIENTS/METHODS We studied 21 WD patients and 14 heterozygote carriers aged 2-43 years, retrospectively. 18 WD patients presented liver disease, three had mixed neurological and hepatic involvement and 9 patients underwent orthotopic liver transplantation (OLT). RESULTS The median age at diagnosis of WD children without OLT was 10.16+/-3.8 (range, 5-16). All of females and younger age categories of patients prevailed in acute liver failure group. Serum ceruloplasmine levels were below 0.2 g/l in about (1/3) of WD carriers (X =0.27+/-0.09 g/l) and nearly (2/3) of children with WD (X = 0.21+/-0.13 g/l). A statistically significant difference (p<0.05) in the 24-h excretion of copper in urine was noticed between healthy controls, children with WD and WD heterozygote carriers. As diagnostic important proved the copper content of more than 250 microg/g hepatic dry weight. The Kayser-Fleischer?s ring was not observed in children. Ceruloplasmine, haemoglobin, ALT, ALP and plasma albumin were significantly different between fulminant and non-fulminant WD and could be used as indirect markers in evaluation of urgent OLT. CONCLUSION Detection of WD in children remains very difficult. The most important investigation is liver biopsy with the assessment of liver copper. Genetic analysis may help in doubtful cases.