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Prodefen® is a dietary food supplement formulated as a synbiotic that has shown additional benefit to the standard supportive therapy in the management of acute viral diarrhoea in children. There is scarce evidence of this synbiotic in adults. The objective of this randomised double blind placebo-controlled clinical trial was to evaluate the efficacy and safety of Prodefen Plus® in the prevention of antibiotic-associated diarrhoea (AAD) in an adult population requiring either antibiotic treatment for an oral infection or antibiotic prophylaxis for a dental surgical procedure in a dental consultation. 151 subjects were randomised to the active (synbiotic) or control arm (placebo) for 14 days. There was a significantly higher reduction in the AAD incidence, and an improvement in the stool consistency in the active group. A higher reduction in both the frequency and duration of the diarrhoea episodes in the active group was also observed, as it was an improved perception of the diarrhoea severity. Overall, the study medication was well tolerated. In conclusion, results from this study confirm the beneficial effect of the synbiotic administered as adjuvant therapy in preventing the antibiotic-associated diarrhoea.

Introduction Childhood acute leukemias (AL) and lymphomas achieve good survival rates. However, second neoplasms (SN) are a devastating event. Methods From August 1987 to December 2016, 34 of 3321 (1%) patients with diagnosis of AL or lymphoma developed SN. SN were AL (n=16), CNS tumors (n=5), endocrinal tumors (n=3), lymphomas (n=2), schwannoma (n=2) assorted sarcomas (n=4), retinal melanoma (n=1), and Vanek tumor (n=1). Median latency was 51 (range, 10 to 110) months for hematological malignancies and 119 (range, 25 to 236) months for solid tumors (P=0.001). Results A total of 33 patients with SN were treated taking into account cumulative doses of anthracyclines and radiotherapy. Twenty-three (67.6%) patients achieved complete remission (CR), 5 died early during therapy and 5 were refractory or partial responders. Six patients presented relapses of the SN and 1 died in CR. Seventeen patients remain alive in CR, with a median follow-up of 110 (range, 4 to 276) months. Conclusions (1) The latency period was significantly longer for patients developing solid tumors than for those developing AL. (2) AL was the most frequent SN. (3) Our results strongly encourage giving standard therapy to SN, considering cumulative doses of previous treatment, since similar probabilities of surviving as "de novo" counterparts can be achieved.

The association between mature-B phenotype and MLL abnormalities in acute lymphoblastic leukemia (ALL) is a very unusual finding; only 14 pediatric cases have been reported so far. We describe the clinical and biological characteristics and outcome of five pediatric cases of newly diagnosed B lineage ALL with MLL abnormalities and mature immunophenotype based on light chain restriction and surface Ig expression. Blasts showed variable expression of CD10/CD34/TdT. MLL abnormalities with no MYC involvement were detected in all patients by G-banding, FISH, and/or RT-PCR. Three patients were treated according to Interfant protocol, one to ALLIC-09, and one received B-NHL-BFM-2004. All patients achieved complete remission and three of them relapsed. Despite the small cohort size, it could be postulated that B lineage ALL with MLL abnormalities and mature phenotype is a distinct entity that differs both from the typical Pro B ALL observed in infants and mature B-ALL with high MYC expression.

Childhood acute myeloid leukemia (AML) achieves event-free-survival (EFS) rates of ∼50%. Double induction phase has been introduced for improving these results. Four consecutive protocols for AML treatment were evaluated to assess the impact of the addition of a second induction course in our setting. From January 1990 to January 2014, 307 evaluable AML patients were accrued. They were classified into low-risk (LR) and high-risk (HR) according to cytogenetic/molecular findings and response on day 15. The first two studies administered one induction cycle while the latter two protocols administered double induction. Relapse was the most frequent event and early-deaths were reduced by 50% in the last protocol. Statistically significant differences were observed when comparing EFS in LR and HR groups. Patients from both risk-groups who received double induction achieved significantly better outcome. EFS improved in protocols with double induction and early-deaths rate was decreased. Cytogenetic/molecular features and early-response were confirmed as prognostic factors.

Congenital gliobastoma multiforme (GBM) is rare and little is known about the molecular defects underlying the initiation and progression of this tumor type. We present a case of congenital GBM analyzed by conventional cytogenetics, fluorescence in situ hybridization, array comparative genomic hybridization and next generation sequencing. On cytogenetic analysis we detected a reciprocal translocation t(6;12)(q21;q24.3). By sequencing, the translocation was shown to form a fusion between the 5' region of ZCCHC8 and the 3' region of ROS1. RT-PCR analyses confirmed the existence of an in-frame fusion transcript with ZCCHC8 exons 1-3 joined to ROS1 exons 36-43. In addition to the ZCCHC8-ROS1 fusion, we detected a deletion in the short arm of chromosome 9, including homozygous loss of the CDKN2A/2B locus in 9p21.3 and heterozygous deletion of the HAUS6 gene in 9p22.1. The latter encodes a protein involved in faithful chromosome segregation by regulating microtubule nucleation and its deletion might be associated with the marked subclonal changes of ploidy observed in the tumor. This report adds the ZCCHC8-ROS1 fusion as oncogenic driver in GBM and supports the role of ROS1 activation in the pathogenesis of a subset of GBM. © 2016 Wiley Periodicals, Inc.

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive and uncommon neoplasm of the central nervous system that usually occurs in children less than 2 years of age. It is characterized by deletions and/or mutations of the INI1 tumor suppressor gene located in chromosome band 22q11.2. We performed cytogenetic and molecular studies of an AT/RT on a 15-month-old boy. The tumor showed a complex karyotype with one cell line showing monosomy 22 and another near-tetraploid one with additional chromosomal abnormalities, involving chromosomes 2, 3, 5, 6, and Y, which had not been previously described. Sequence analysis of the tumor did not identify mutations of the INI1 gene. The karyotypic evolution observed in this tumor suggests that INI1 has an epigenetic role in the maintenance of genome integrity by affecting genes, which produces mitotic defects and polyploidy. Finally, this case is the first to support the theory that loss of INI1 could induce the chromosomal instability that might be responsible for the genesis of this tumor.

We present a case of acute myeloblastic leukemia (AML-M2) with a complex t(8;21) translocation and additional acquired chromosomes yielding a hyperdiploid karyotype. AML1/ETO transcript was observed by reverse transcription-polymerase chain reaction. Fluorescence in situ hybridization (FISH), spectral karyotyping (SKY), and comparative genomic hybridization (CGH) were performed to further identify the chromosomes observed by G banding. The patient was treated according to our current protocol for AML. He remains in complete remission +11 months from diagnosis. Further follow-up of this patient and the analysis of a larger number of children are needed to define whether the gains of the specific extra chromosomes modify the good prognosis that t(8;21) confers to this subgroup of AML. Pediatr Blood Cancer 2008;50:651–654. © 2007 Wiley-Liss, Inc.

Patients with Down's Syndrome have a higher risk of developing acute megakaryoblastic leukemia (AML). Ten per cent of newborn infants with this syndrome have transient abnormal myelopoiesis (TAM), indistinguishable from AML, which generally remits spontaneously. A high incidence of GATA-1 gene mutations was described in both groups of patients. Fourteen bone marrow DNA samples (10 ATM/4 AML) were analyzed by PCR and sequencing; these samples were obtained from 13 patients with Down's Syndrome to describe the rate and mutation characteristics of the GATA-1 gene in the studied population and its consequences at a protein level. Mutations were detected in 10 out of 10 TAM and in 3 out of 4 AML, which at a protein level would result in an early termination codon (n= 5), alterations in the splicing site (n= 6) or sequence change (n= 3). The high rate of GATA-1 gene mutations was confirmed in newborn infants with Down's Syndrome and MAT or AML.

We describe an 8 year old boy who had received chemotherapy for an acute promyelocytic leukemia and developed a secondary leukemia 27 months after the diagnosis of this first malignancy. Blasts cells were positive for cytoplasmic markers CD22, CD3 and myeloperox-idase. Cell surface T and myeioid-associated markers were also detected. Cytogenetic study disclosed monosomy 7. The patient achieved complete remission, but relapsed 15 months later with identical immunophenotypic and cytogenetic findings. Three-lineage commitment is proved by the expression of specific criteria for myeloid, and lymphoid T and B typing. A multipotent immature progenitor must be the target of leukemogenic agents. The prognosis is obviously ominous.

We report a boy with partial distal 5p15.1-->pter trisomy and normal development. We compared the clinical findings in our patient with those previously reported of the same 5p duplicated region. Several cases of autosomal duplications and normal development have been described. The present case is another example of a chromosomal anomaly with little, if any, phenotypic effect without mental retardation.

The present study was performed to establish the prevalence of the recurrent fusion transcripts in Argentinean pediatric patients with acute lymphoblastic leukemia (ALL). A total of 380 newly diagnosed children (including 50 infants and 44 T-ALL) were screened by RT-PCR; the incidence of recurrent rearrangements was: ETV6-RUNX1, 12.9%; TCF3-PBX1, 5.0%; BCR-ABL1, 1.6%; and MLL rearrangements, 10.5%. STIL-TAL1 was detected in 22.7% of T-ALL cases. In B-ALL cases, the pEFS was significantly influenced by the presence of genetic alterations. RT-PCR studies improved patients' stratification and also the overall outcome of children treated in a pediatric hospital from a developing country.

Our aim was to compare two different schedules of maintenance in pediatric acute lymphoblastic leukemia (ALL) treated with a BFM-based therapy, in a randomized study: an Arm with 6-MP + MTX (with or without vincristine and dexamethasone pulses) versus a more intensive continuation phase.From January 1996 to November 2002, 429 eligible children with ALL were enrolled in a protocol with BFM-based back-bone, followed by a randomized continuation phase in standard (SRG) and intermediate (IRG) risk groups. Patients were randomized between Arms A and B for SRG and B or C for IRG. Arms A and C consisted of 6-MP and MTX and in Arm C, six pulses of VCR and dexamethasone were added. Arm B combined four pairs of drugs rotated weekly. All risk-groups received maintenance until completing 2 years of therapy from diagnosis.With a median follow-up of 138 (range: 96-178) months, the overall pEFS (SE) was 72 (6)% for all patients and the different risk groups showed: SRG: 85 (3)%, IRG: 71 (1)%, and HRG: 42 (7)% (P-value ≤ 0.0001). The pDFS (SE) according to the assigned arm of maintenance was, for Arm A: 89 (3)% and for Arm B: 85 (4)% in SRG, and, for Arm B: 77 (4)% and for Arm C: 75 (4)% in IRG, at 10 years follow-up. There were no statistically significant differences in outcome between arms of maintenance for both risk groups.In protocols with initial BFM-based strategy, a more intensive continuation phase did not benefit any risk group of patients.

Pediatric obesity has increased dramatically all over the world and nonalcoholic fatty liver disease (NAFLD) is one of the most frequent complications associated with excess adiposity. NAFLD causes serum transaminase elevation and liver disease, which could end up in fibrosis, cirrhosis and eventually hepatocellular carcinoma. NAFLD seems to be associated with the metabolic complications of obesity, mainly insulin resistance. The aim of the present article is to review the role of serum liver enzyme assessment as a suitable non invasive predictor of NAFLD in children. Although serum liver enzyme elevation does not accurately measure liver damage, it may be a valuable and non invasive test to screen NAFLD in children and adolescents and a marker to control NAFLD evolution. To detect NAFLD in obese children and adolescents, transaminases serum concentrations should be routinely determined in these patients. In this sense, it seems necessary to obtain transaminase reference standards for children and adolescents.

By High Resolution Consultation (HRC) we mean an ambulatory process of assistance fulfilled in a single day, by which treatment and diagnosis are established after all complementary tests have been carried out. Once diagnosed, the patient is discharged and resent to the doctor who had previously remitted him/ her. In the Cáceres sanitary area, with distances longer than 100- 120 km. and precarious local road communications, the introduction of hypertension (HTA) HRC has brought along important savings of sanitary and economical resources and it is perceived by the user as highly satisfactory. We have carried out an observational one- year study of our HCR HTA, in which 90 patients have been evaluated, out of which 74.4% came from primary assistance and 25.6% from specialized assistance. Once diagnosed, 61 patients were discharged and sent to receive primary assistance and 29 were kept in our outpatient nephrology consultation, justified by severe and/ or rebellious HTA in 11 cases and by renal failure in 16 cases; two of these patients are still being tested. Taking into account that in a traditional consultation a patient would need two or three visits and one or two days for complementary tests, our HTA HRC (by which patients are tested and diagnosed in a single day), brings along savings of one or two consultations and two to four relocations for new consultations and diagnostic explorations. In one year of HTA- HRC with 90 patients, we have saved from 212 to 302 consultations and from 302 to 604 relocations in comparison to the traditional organization and we have thus generated from 100 to 150 places for first appointments.

The aim of this paper is to analyze the ways of appearance, clinical characteristics, diagnosis and treatment related to patients suffering from primary hyperaldosteronism (HA1 masculine) in external nephrology consultation since their opening.a retrospective study was carried out, checking out all HA1 masculine diagnosed patients of clinical records from 1981-2005.35 patients were diagnosed, with an average age of 50 and a predominance of men (82%). The main reason for starting the HA1 masculine study was persistent hypertension; other reasons were hypertension and hypopotassemia (34%). Sixteen of the cases were adenomas (7 classic adenomas and 9 renin-dependents) and fourteen of them were hyperplasia (10 bilateral hyperplasias and 4 primary adrenals hyperplasia). Five cases were excluded because they were waiting for complementary tests. For location diagnosis, gammagrafía I131cholesterol was the test showing more agreement with final diagnosis, and then RMN and TAC. In eight of the cases, an adrenal vein sampling was made. Ten of sixteen adenomas suffered a surgery performance. The result showed standardization of tensional levels, without any treatment in 60% of the cases. The rest of them are currently treated with spironolactone under an appropriate tensional control. Gynecomastia was the most usual adverse effect found.Contrary to other published papers, we found out a male predominance in our database. A similar incidence of adenomas e hyperplasias was obtained. The most usual way of appearance was persistent hypertension to treatment. Adenomas surgery does not imply healing results, though it achieves a better tensional levels control, using less drugs and diminishing aldosterone levels. It implies a descent in myocardic and vascular toxicity.

The objective of this study is the determination of the normal kidney size in children analysing the variables that have a good correlation with it. With these correlations the normal kidney size can be established and a diagnose of possible diseases set.The data was obtained through ecographies taken in 474 children between 0 and 18 years old that were under control for several reasons differently from the suspicion of a kidney disease. The information related to age, sex, weight, height and corporal surface was also taken.We studied 265 girls and 209 boys. No difference was obtained between the size of the girls and boys kidneys. The regression analysis of the left kidney shows correlations of 0.911 for the height, 0.896 for the corporal surface, 0.863 for the weight and 0.857 for the age; while the one of the right kidney shows correlations of 0.921 for the height, 0.902 for the corporal surface, 0.872 for the age and 0.871 for the weight. The determination coefficients were 0.83 for the left kidney and 0.85 for the right one. The multivariant analysis shows that for both kidneys the height and the age are variables statistically significant as well as the corporal surface for the left kidney.The size parameters measured in the kidneys show a statistically significance correlation with certain parameters of the growing. The maximum correlation appears for the height of the patient, which allows establishing regression equations with the kidney size and the height and therefore to detect any deviations from these regression curve.

CD4+ CD56+ malignancies have only recently been related to dendritic cell (DC) lineage. The few cases described, mostly adults and elderly, typically present with cutaneous lesions, followed by disseminated tumor localizations within a few months, with a generally very aggressive course and fatal outcome, despite the different therapeutic approaches employing chemotherapy and/or radiotherapy. Considering that leukemias in childhood and in adults are different diseases, we describe three pediatric cases to help compare the biological characteristics, immunophenotype, clinical features, treatment response and incidence of this disease in both age groups. From a total 1363 new patients with acute leukemia (AL), we report three cases with blasts of French - American - British L2 morphology, an absence of the most specific markers for myeloid, T or B lineage and lacking CD34, which led us to evaluate the blasts with an extensive panel of antibodies, including those related to the other putative pathways of lymphoid differentation: natural killer and DC. The cells expressed CD4, CD56, HLA-DR, BDCA-2 and BDCA-4. None of our cases presented with skin involvement. All three children showed good response to acute lymphoblastic leukemia (ALL) protocols, achieving complete remission even when one of the patients relapsed and received an allogeneic transplant. These findings, in spite of the small number of patients, suggest that the clinical course in children might be less aggressive, and that regular ALL protocols would be effective. We emphasize the importance of including antibodies for DC lineage in cases of CD34(-) unclassifiable AL to further characterize these rare cases (0.22%), considering that the tumor cell affiliation to DC lineage relies exclusively on immunophenotypic criteria.

We describe a child with ATR-16 [alpha-thalassemia (thal)/mental retardation], who was referred for genetic evaluation because of minor anomalies and developmental delay. Cytogenetic analysis demonstrated a de novo complex rearrangement of chromosome 16. Fluorescence in situ hybridization (FISH) analysis, using chromosome 16 subtelomeric probes, showed that this patient had a deletion of the distal short arm of chromosome 16 that contains the alpha-globin genes and a duplication of 16q. Analysis of the alpha-globin locus by Southern blot showed a half normal dose of the alpha-globin gene. Microsatellite marker studies revealed that the duplicated 16q region was maternal in origin. Hematological studies revealed anemia, hypochromia and occasional cells with Hb H inclusion bodies. A hematological screening for alpha-thal should be considered in patients with mild developmental delay and a suggestive phenotype of ATR-16 with microcytic hypochromic anemia and normal iron status. The stellate pattern of the iris, a new finding in our patient, may contribute to a better clinical delineation of both syndromes, ATR-16 and/or duplication of 16qter.

To compare the accuracy of the breath test using the isotope ratio mass spectrometer (IRMS) versus the nondispersive isotope-selective infrared spectrometer (NDIRS) in the diagnosis of Helicobacter pylori infection.Multicenter study in 4 Spanish hospitals. One group of dyspeptic patients who had not undergone prior eradication therapy and another group of patients with gastric ulcer or gastrointestinal bleeding due to gastroduodenal ulcer receiving H. pylori eradication therapy were included in the study. A reference standard based on histology and the rapid urease test was used. The breast test (TAU-KIT, Isomed S.L., Madrid, Spain) was performed with citric acid and 100 mg of 13C-urea. Samples of expired air were collected in tubes and bags for reading with the IRMS (ABCA, PDZ, Crewe, Manchester, England) and the NDIRS (UBiT-IR200, Otsuka Electronics, Co, Osaka, Japan), respectively. The endoscopist, pathologist and person responsible for reading the urease test and both breath tests were blinded to the results of the other diagnostic methods.Forty-one patients were included. The prevalence of H. pylori was 26%. No differences were found on comparing the mean values obtained with the IRMS and the NDIRS: 13 (standard deviation) (24) and 14 (25) delta units, respectively. The area under the ROC curve for the IRMS and the NDIRS was 0.96. The diagnostic accuracy for the best cut-off point with the IRMS and the NDIRS was, respectively: sensitivity (90 and 100%), specificity (96 and 89%), positive predictive value (90 and 77%), negative predictive value (96 and 100%), + likelihaod ratio (25 and 9.3) and (0.1 and 0). A close correlation was found between the values of the IRMS and those of the NDIRS (lineal regression equation, Y = 1.1 + 1.004. X; r = 0.97).Both the spectrometers used to evaluate the breath test, the IRMS and the NDIRS, offer a high degree of accuracy in the diagnosis of H. pylori infection.

Cochlear implant have been proven to be safe and cost-effective in deafened adults. Generally, a single cochlear implants is proposed providing monaural audition. Some teams have published interesting results in patients wearing two cochlear implants. The cost of the surgical procedure is greatly increased for bilateral implantation. In an attempt to provide patients with binaural hearing and a better chance to improve their capacity for frequency discrimination without an excessive increase in cost, we developed an new concept and design for binaural cochlear implants. The aim of the present study was to establish the surgical technique for binaural cochlear implantation with a single internal receptor-stimulator. We report descriptive data on surgical procedures performed in two cadavers and in two deafened adults. The special implant is composed of a single receptor-stimulator and two electrode arrays: a short one implanted into the ipsilateral ear and a long one in the contralateral ear. A specially designed tool for endoscopic facial lifting was used to create subcutaneous tunnels from the ipsilateral to the contralateral ear. Insertion into cochleas was unremarkable. Additional time for surgery (compared with mean operative time for routine adult surgery in our unit), and surgical details are discussed. Binaural cochlear implantation is feasible, easy and not very time consuming. Studies are in progress to determine patient benefit. Obtaining auditory brainstem evoked potentials electrically in patients with binaural cochlear implants is possible and provides an interesting mode for in vivo analysis of the long-term effect of profound/total deafness on neural transmission. In our patients, results have demonstrated that neural response properties in auditory pathways are negatively related to duration of deafness.

In the present study, we evaluated the seroprevalence of HTLV-I/II infection among the blood donors in Santa Fe and Santiago del Estero provinces. A total of 1327 serum samples from blood donors from Rafaela blood bank of Santa Fe province and 3382 serum samples from blood donors from Dr Edgar Bouzon blood bank of Santiago del Estero province were studied. The antibody screening was done by particle agglutination assay (PA) (SERODIA, Fujirebio Inc., Tokyo, Japan) or by enzyme immuno assay (EIA) (Abbott HTLV-I/HTLV-II EIA, Abbott, Germany). The "in house" indirect immunofluorescence assay (IFA) and Western blot (Bioblot HTLV Biokit, Barcelona, Spain) were used as confirmatory assays. All the samples resulted negative for specific antibodies against HTLV-I/II. These results suggest that HTLV-I/II are not circulating in low risk populations in these provinces or that the prevalences of infection would be lower than that reported by blood banks in other provinces of Argentina.

Few cytogenetic data are available concerning the chromosomal constitution of post-transplant lymphomas. We report two paediatric cases of trisomy 3, as a primary anomaly, in post-transplant lymphoproliferative disease (PTLD) associated with B immunophenotype. Using cytogenetic analysis and fluorescence in situ hybridization on chromosome preparations, we found trisomy 3 in both patients and an extra X chromosome in one. Clinical, histological and immunophenotypical data are presented. Trisomy 3 has been observed in different types of non-Hodgkin's lymphomas but it is relatively rare in B-cell lymphomas, with the exception of marginal zone lymphoma and mantle cell lymphoma. To our knowledge, trisomy 3 is an uncommon cytogenetic finding in PTLD. Further cytogenetic studies of these lymphoproliferative disorders might contribute to evaluate the role of these chromosomal anomalies in the pathogenesis of this disease.

In this study we have determined the seroprevalence of infections by HTLV-I/II in the blood donor population from the city of Córdoba. A total of 5476 blood donor sera were screened for HTLV-I/II antibodies by particle agglutination assay (PA) (SERODIA HTLV-I, Fujirebio INC, Tokyo, Japan). The reactive sera samples were confirmed by an "in house" indirect immunofluorescence assay (IFA). 14 out of 5476 blood donors studied were PA reactive and were confirmed positive by IFA, showing a prevalence of 0.26% (95% confidence interval: 0.126%-0.394%). All the positive samples, except one, met the criteria for HTLV-I. Although one HTLV-I infected donor was an intravenous drug abuser and two donors were born in highly endemic areas for HTLV-I, no specific risk factors were identified among the others. The demonstration that HTLV-I circulates in blood donor population of Córdoba, points out that the systematic screening of blood for HTLV-I/II antibodies must be implemented in the blood banks, in an attempt to prevent the spread of infections with this oncogenic virus in Argentina.

We present case of focal fatty liver lesions. Echography images revealed multiple hyperechogenic lesions similar to those observed in metastatic disease. Aspiration was negative and diagnosis was based on the results of liver biopsy alone, carried out during laparoscopy. The difficulty of determining the etiology of this type of lesión by echography is discussed.

The association between t(8;21) and granulocytic sarcoma (GS) is well known, but to the authors' knowledge the prognostic significance of GS in these patients has not been defined clearly.Between January 1990 and July 1999 174 children with acute myeloid leukemia were admitted to the study institution. Translocation (8;21) was identified in 20 patients (11.5%). Eighteen patients were evaluable for the current study and 8 presented with GS at the time of diagnosis (GS+).The authors defined two groups of patients: those who were GS+ and those who were GS-. One patient in the GS+ group and two patients in the GS- group died during the induction phase of the study. Complete remission was achieved in seven patients in the GS+ group and eight patients in the GS- group. Two patients developed a recurrence in the GS+ group as did one patient in the GS- group. The event free-survival probability (the standard error) was 58% (18%) in the GS+ group and 70% (14%) in the GS- group. Localization of GS was in only one site in seven patients and at multiple sites in one patient. Patients with an epidural mass received local radiotherapy (one patient) or surgery (two patients). Two of these patients developed paraplegia as sequelae: one patient after surgery and one patient after radiotherapy. One patient with orbital GS received local radiotherapy because of progressive proptosis. The remaining four patients had a complete resolution of the GS with chemotherapy only.In the current study of patients with t(8;21)(q22;q22), the presence of granulocytic sarcoma was not found to be an adverse prognostic factor. However, careful attention should be paid, especially to patients with an epidural site, to avoid sequelae. Chemotherapy appears to be the optimum treatment for these children.

We report results achieved in our institution with an acute lymphoblastic leukaemia risk-oriented treatment trial opened in January 1990 and closed on December 1995. The study was similar to the German ALL-BFM’90, except for using Protocol III for the standard-risk group, 2 g/m 2 of methotrexate in Protocol M, and preventive cranial irradiation for the high-risk group only. The high-risk group included mostly patients with prednisone poor initial response and/or adverse cytogenetic features. This analysis included 374 patients, whose mean age was 6 years (range: 1 month–17 years). The overall complete remission rate was 94.4% (353/374) and the 5-year event-free survival (standard error) probability is 64(5)%. The 5-year event-free survival estimates for each risk group were: (1) high-risk group 37(5)%; (2) intermediate-risk group 66(1)%; and (3) standard-risk group 74(4)% ( P =0.0001). There are significantly higher-rates of isolated bone marrow and testicular relapses in the high-risk subset of patients. Our dismal results and the published experience, lead us to conclude that the optimal treatment for these high-risk acute lymphoblastic leukaemia patients is not currently known.

Between July 1990 and December 1995, 111 new consecutive pediatric patients with acute myelogenous leukemia (AML) have been treated in our institution. Eleven of them (9.9%) had Down's syndrome (DS), 6 boys and 5 girls. The median age was 22.5 (range 10–40) months. FAB subtypes were the following: M7: 6, M4: 3, and M0: 2. Five of them had previously had myelodysplasia and in 3, all FAB M7, myelofibrosis was detected. This population was treated with two consecutive protocols. Nine patients were included in the AML-HPG-90 protocol and 2 patients in the AML-HPG-95 study, respectively. However, all DS patients in this series received the same treatment. Eight patients achieved complete remission: two patients received two cycles of intensification with high dose (HD) ara-C, and 1 patient, only one cycle; the other 5 were prevented from receiving such therapy because of unacceptable toxicity or death. At 45 months, event-free survival and overall survival estimates were 0.30, S.E. 0.16. Mortality was remarkably high. All deaths (7) were associated with sepsis (5) or pulmonary infection (2). Three deaths occurred before achieving complete remission, 3 patients died during the consolidation phase and 1 died whilst off treatment. No one presented leukemic relapse. We conclude that this AML-BFM treatment strategy is highly toxic to children with DS and AML in our setting. Efforts will be made to improve clinical support and to administer less intensive therapy to this particular pediatric AML subgroup, which, in fact, has a better prognosis than the same non-trisomic population.

We report results achieved in our institution with a study opened in July 1990 (similar to the German AML-BFM-87 in which daunorubicin was replaced by idarubicin in the induction phase and cranial preventive radiotherapy was omitted) and closed in December 1994, for the treatment of newly diagnosed acute myeloblastic leukemia (AML), without prior malignancies except for myelodysplasia. This evaluation included 68 patients, whose mean age was 6 years (range: 1 month-16 years). Thirty-nine were boys and 29 were girls. Complete remission rate was 80.9% (55/68), death on induction rate was 14.7% and induction failure rate was 4.4%. At median follow up of 38 months (range: 12-66 months), the 4-year event-free survival (EFS) estimate was 0.428 (S.E.: 0.062), event-free interval (EFI) estimate was 0.529 (S.E.: 0.07) and overall survival (OS) estimate was 0.44 (S.E.: 0.071). We conclude that idarubicin in combination with cytarabine and etoposide is a highly effective regimen for induction in children with AML. Although preventive cranial irradiation was not delivered, we have observed only one combined CNS relapse. Finally, we corroborate that in this setting two definite risk groups may be identified in children with AML.

We compare the radiologic manifestations of chest TB in three groups of patients: HIV patients (52 cases), HIV negative patients (85 cases and 100 cases). Initial radiologic findings were similar in the two seronegative groups, even though there is an eight-year interval between both series. However, seropositive patients have a higher risk of acquiring the infection (x 2.25), of extrathoracic disease (x 4.22), of coincidental infections (p0.0002) and of progression of the disease p0.0003) than the normal population. Lymphadenopathy and miliary TB are much more common in the HIV positive patients (x 6.23 and x 44 respectively). Cavitation, pulmonary scarring, volume loss and calcification are more frequent among the seronegative patients (p0.03), as well as pleural disease (p0.0001).

A 4-year-old boy with chronic myeloid leukemia (CML) was shown to have a variant Ph t(Y;22)(p11;q11). To our knowledge, this is the first report of a variant Ph translocation involving Y. Molecular analysis showed that the breakpoint on chromosome 22 is in the breakpoint cluster region (bcr), typical of CML with the classic t(9;22), suggesting that it might be a complex Ph translocation with the involvement of 9q34.

The present study considers the analytic importance of the excitation pulse features delivered by a Nucleus cochlear implant using the F0F1F2 strategy. Four cochlear implant patients and 10 speakers uttering two 48-item lists constructed from four basic French vowels participated in this study. Patterns were presented to the patients and played at the input of an acquisition system in order to record the pulse features. Confusion matrices obtained with the patients and with automatic recognition procedures were then compared in order to find out the best-matching models simulating the patients' performances, out of 255 possibilities. The automatic recognition was carried out according to fuzzy logic based on the elementary features of the pulses coding the vowels. Results show that the essential features strongly depended on the subject.

We identified 40 patients with malignant lymphoproliferative diseases (MLD) and HIV infection (seropositive) at a single Spanish university hospital. Thirty-two patients had non-Hodgkin's lymphoma (NHL), 6 primary central nervous system lymphoma (PCL) and 8 patients Hodgkin's disease (HD). Median age at presentation was 32 years. Four histopathological groups had distinct presenting clinical features: in 93% of the Burkitt-type lymphomas, the lymphoma itself was the AIDS defining criterion, while high and intermediate grade NHL other than Burkitt-like tended to have a more advanced HIV infection, demonstrated by antecedent AIDS criteria in 58% of these patients and a median CD4 positive cell count of 291 mm3; HD occurred in some patients without previous opportunistic infections (7/8 patients) but with median CD4 cells of 105 mm3; PCL occurred in a terminal stage of HIV infection, in patients with a low performance status, and frequent antecedent AIDS criteria. Objective response to chemotherapy could be seen in 62% of NHL patients and 100% of HD. Survival was adversely related to an antecedent diagnosis of AIDS, low performance status, and a primary localization in the central nervous system. Overall median survival was 5 months, but patients without the mentioned three adverse prognostic factors had a median survival of 10 months.

Cyclophosphamide is used both in the treatment of malignant and non-malignant diseases. Urinary neoplasms secondary to its use have been described. We discuss the case of a patient with panarteritis nodosa treated with cyclophosphamide during 63 months, with a total dose of 210 grams, and that showed a bladder neoplasm 8 years after beginning of the treatment. In patients receiving a total dose of cyclophosphamide over 85 grams, a follow-up of ten years minimum should be performed aimed to the early detection of secondary neoplasms.

The biological activity of aqueous extracts of Solanum malacoxylon (SM) and of extracts preincubated with ruminal fluid (SMRF) on calcium and phosphate metabolism in vitamin D depleted rats was compared. The responses in intestinal 32P and 45Ca absorption, measured in an isolated duodenal loop in situ after a single oral dose, were qualitatively similar to that of 1,25-OH2-vit.D3. However, the effects elicited by SMRF were significantly higher than those caused by SM. Both extracts were equally effective in promoting bone resorption when administered orally or subcutaneously. These results provide a basis to explain the greater effectiveness of SMRF to produce calcinosis in rats. The hypothesis is advanced that biologically active steroidal metabolites with predominant action on intestine, are formed during incubation with rumen.

From january 1982 to january 1986 we treated 23 pediatric patients who had non Hodgkin's lymphoma, with a new therapeutic protocol. This protocol is based on the LSA2 L2, modified during the induction phase with high-dose methotrexate and increasing of intrathecal therapy. Nineteen patients (83%) were in stages III and IV and the 47.4% were in stage IV. The distribution according to histology was: 10 Burkitt's undifferentiated lymphomas; 2 non-Burkitt's undifferentiated lymphomas; 5 lymphoblastic lymphomas convoluted-cells; 5 lymphoblastic lymphoma non-convoluted cells and 1 indeterminate lymphoma. The follow-up oscillated between 6 months and 4 years. The total survival rate is 82.6% with a 100% survival in stages I, II and III and 63.5% in stage IV. The survival according histology is 91.7% for undifferentiated lymphomas and 70% for lymphoblastic lymphomas; the actuarial survival rates in the two groups at 30 months are 87% and 55% respectively (p greater than 0.10). We concluded that adding high-dose methotrexate to the LSA2 L2 protocol improve the survival rate in undifferentiated lymphomas and in a less extent in lymphoblastic lymphomas, without significant toxicity. This new protocol is mainly indicated in undifferentiated and indeterminate non-Hodgkin's lymphomas.

Fifty five patients with Schöelein-Henoch nephropathy were reviewed. These patients were divided into five groups according to the clinical picture initially presented: 1. Hematuria with proteinuria. 2. Hematuria with nephrotic syndrome. 3. Isolated hematuria. 4. Hematuria, nephrotic syndrome and renal failure. 5. Proteinuria. From a histopathological point of view, they were classified into six groups according to ISKDC criteria. A strong correlation was found between clinical presentation and histological lesion. The worst prognosis corresponded to patients with nephrotic syndrome and renal failure, whose biopsy revealed more than 75% of glomeruli with epithelial crescents.

Background. A Phase II trial with a new regimen of methotrexate, carboplatin, and vinblastine (M-CAVI) was conducted for patients with bladder cancer who could not receive cisplatin-based chemotherapy. Methods. Treatment consisted of methotrexate (30 mg/m2) on days 1,15, and 22; carboplatin (300 mg/m2) on day 2; and vinblastine (3 mg/m2) on days 2, 15, and 22, scheduled at 28-day intervals. The dosage of carboplatin was adjusted for creatinine clearance. Twenty-five patients with metastatic (n = 9) or locally advanced or locoregional bladder cancer with a poor prognosis In (= 16) were treated with M-CAW. Fifteen patients had abnormal serum creatinine levels, 4 had a low performance status, 3 had cardiac disease, and 3 were older than 70 years old. Results. Among 23 patients assessable for clinical response, the response rate was 48%. The median duration of response for metastatic disease was 7 months. Among nine patients assessable for pathologic response, there were two complete responses and three partial responses. The toxic effects have been moderate. Conclusions. M-CAVI is an active and well-tolerated regimen that can be used in patients with bladder cancer who are ineligible to receive cisplatin-based chemotherapy. Cancer 1992; 70:1974–1979.

Early nephrological referral and planned start of dialysis are associated with better prognosis after the beginning of renal replacement therapy (RRT). The aim of our study was to analyse patient clinical and analytic characteristics at the time of initiating dialysis and to evaluate if morbimortality was affected by planned start.We performed a retrospective study of all patients commencing RRT in a Spanish Hospital of The National Health System over two years (2003-2004). A total of 117 patients (47 female and 70 male) were included. We carried out a retrospective analysis of the demographic characteristics, patients' clinical and analytic conditions at the time of starting dialysis and hospitalization days and mortality in six months after starting dialysis. Patients were classified as planned (P) or unplanned (NP), depending on whether the first dialysis was planned or an emergency.Sixty five patients (56.4%) started dialysis in a planned group while 52 (43.6%) were unplanned. In the former group, 83.1% of the patients had a vascular or peritoneal access available when starting RRT, whereas in the later group only the 3.8% had it. Planned dialysis initiation was associated with a high level of serum haemoglobin, haematocrit, calcium and albumin (p0.001), and a low level of serum urea, creatinine (p0.001) and phosphate (p0.05). More patients of the unplanned group were admitted at hospital at the initiation of dialysis (90.4% vs. 6.1%) and during the first 6 months (48% vs. 15.3%). The period of hospitalization was longer for the unplanned group (23.6 days vs 3 days) (p0.001). The 6-month-mortality was lower in the planned group (4.6% vs. 11.5%), whitout statistical difference.Planned dialysis initiation is associated with better clinical and metabolical conditions, greater probability of a vascular or peritoneal access availability and lower rate of hospitalization and mortality within 6 months after starting RRT

An unusual case of histologically proven ileal adenocarcinoma mimicking regional enteritis in a 22-year-old man is reported. Although in 2 previous reports the patient's age was stressed as an important factor for the differential diagnosis, this was not of diagnostic help in our case. This report emphasizes the need to include other diagnostic possibilities when regional enteritis has an anomalous evolution and responds poorly to treatment.