La prevención como eje en la extensión / Prevention as the extension shaft

El presente artículo pretende dar a conocer eltrabajo realizado en el marco del programa de extensión ôTrastornos en el desarolo del enguaje y su incidencia en el Aprendizajeö dependiente de la Facultadde Ciencias de la Salud de la UNSL. Dicho programatiene como objetivo general, prevenir, detectar, evaluar yrealizar tratamiento fonoaudiológico en niños de unacomunidad vulnerable. Para alcanzar este objetivo, seplantearon las actividades en cuatro fases. Fase 1, refiere al contacto y acuerdos instiucionales. Fase 2, con laorganización del trabajo y la formación de recursos. Fase3, con la divulgación y presentación a eventos científicos, y por último la fase 4, que se refiere al seguimientode la casuística detectada. La población destinatariadel programa son niños de nivel inicial y primer ciclo dela Escuela Nº 141 ôLos Andesö de la localidad de Baldede la provincia de San Luis. De la totalidad de niñosevaluados, se han detectado 35 niños con difcultadesen diversos aspectos del enguaje y repercusiones en elaprendizaje de grado variable que se coresponde conun 47% de la población total. Indicador que da cuentade la importancia de la deteción temprana para favorecer los procesos de aprendizaje posteriores. Identifcaraquelos casos que son susceptibles de un abordajefonoaudiológico, permite potenciar aquelas áreas enlas que los niños muestran difcultades, favoreciendoasí una adecuada integración escolar, familar y social.(AU)

This article seks to highlight he work done underthe extension program "Developmental disorders oflanguage and its efects on learning proces" under theFaculty of Health Sciences of the UNSL. This program isintended to adres ôPrevent, detect, ases andfonoaudiological treat to children in a vulnerablecommunityö. To achieve this goal, the activites wereraised in four phases. Phase 1 refers to the contact andinstiutional arangements. Phase 2, work organizationand resource training. Phase 3, with the disclosure andpresentation of scientifc events, and finaly phase 4, whichrefers to the monitoring of casuistry detected. The targetpopulations of the program are children of preschol eveland first level of the Schol No. 141 ôLos Andesö Baldetown in San Luis province. Of al children tested, weredetected 35 children with dificulties in various aspects oflanguage and learning implications of varying degreswhich coresponds to 47% of the total sample population.Indicator that realizes the importance of early detection to faciltate subsequent learning proceses. Identify cases that are susceptible to fonoaudiological andpsychological aproach, alows strengthening thoseareas where children show dificulties, which contribute to adequate schol, family and social integration.(AU)

La prevención como eje en la extensión / Prevention as the extension shaft

El presente artículo pretende dar a conocer eltrabajo realizado en el marco del programa de extensión “Trastornos en el desarolo del enguaje y su incidencia en el Aprendizaje” dependiente de la Facultadde Ciencias de la Salud de la UNSL. Dicho programatiene como objetivo general, prevenir, detectar, evaluar yrealizar tratamiento fonoaudiológico en niños de unacomunidad vulnerable. Para alcanzar este objetivo, seplantearon las actividades en cuatro fases. Fase 1, refiere al contacto y acuerdos instiucionales. Fase 2, con laorganización del trabajo y la formación de recursos. Fase3, con la divulgación y presentación a eventos científicos, y por último la fase 4, que se refiere al seguimientode la casuística detectada. La población destinatariadel programa son niños de nivel inicial y primer ciclo dela Escuela Nº 141 “Los Andes” de la localidad de Baldede la provincia de San Luis. De la totalidad de niñosevaluados, se han detectado 35 niños con difcultadesen diversos aspectos del enguaje y repercusiones en elaprendizaje de grado variable que se coresponde conun 47% de la población total. Indicador que da cuentade la importancia de la deteción temprana para favorecer los procesos de aprendizaje posteriores. Identifcaraquelos casos que son susceptibles de un abordajefonoaudiológico, permite potenciar aquelas áreas enlas que los niños muestran difcultades, favoreciendoasí una adecuada integración escolar, familar y social.

This article seks to highlight he work done underthe extension program "Developmental disorders oflanguage and its efects on learning proces" under theFaculty of Health Sciences of the UNSL. This program isintended to adres “Prevent, detect, ases andfonoaudiological treat to children in a vulnerablecommunity”. To achieve this goal, the activites wereraised in four phases. Phase 1 refers to the contact andinstiutional arangements. Phase 2, work organizationand resource training. Phase 3, with the disclosure andpresentation of scientifc events, and finaly phase 4, whichrefers to the monitoring of casuistry detected. The targetpopulations of the program are children of preschol eveland first level of the Schol No. 141 “Los Andes” Baldetown in San Luis province. Of al children tested, weredetected 35 children with dificulties in various aspects oflanguage and learning implications of varying degreswhich coresponds to 47% of the total sample population.Indicator that realizes the importance of early detection to faciltate subsequent learning proceses. Identify cases that are susceptible to fonoaudiological andpsychological aproach, alows strengthening thoseareas where children show dificulties, which contribute to adequate schol, family and social integration.

Interleukin-1 administration before lethal irradiation and allogeneic bone marrow transplantation: early transient increase of peripheral granulocytes and successful engraftment with accelerated leukocyte, erythrocyte, and platelet recovery.

Administration of interleukin-1 beta (IL-1 beta) before a lethal irradiation with or without allogeneic bone marrow transplantation (BMT) protects greater than 90% of the irradiated mice. To approach the mechanisms responsible for the radioprotective effect of IL-1, we examined the effects of IL-1 pretreatment on engraftment and kinetics of peripheral blood, spleen, and marrow cell reconstitution after irradiation and BMT. Although the BMT was not necessary for the survival of the IL-1-pretreated lethally irradiated mice, allogeneic marrow did engraft in these mice as evaluated in the spleen and marrow 2 months after BMT. IL-1 pretreatment significantly accelerated hematopoietic recovery versus transplanted saline-treated controls with a pronounced enhancement of peripheral leukocyte, platelet, and erythrocyte recovery. Leukocyte recovery in IL-1-pretreated mice was unique in that IL-1 first induced an early transient (maximum at day 7) increase of peripheral granulocytes before accelerating leukocyte recovery after day 11. IL-1 pretreatment also significantly enhanced marrow cell recovery after allogeneic BMT with an eightfold increase in marrow cellularity from day 4 to 11 versus control transplanted mice. When lethal irradiation was not followed by allogeneic BMT. IL-1 pretreatment also affected the peripheral reconstitution of leukocytes, platelets, and erythrocytes. Interestingly, in the absence of BMT, IL-1 also induced an early circulation of peripheral granulocytes. Overall, our data demonstrate that a single administration of IL-1 before lethal irradiation and allogeneic BMT can induce an early transient increase of circulating granulocytes, followed by an accelerated multilineage recovery and long-term allogeneic engraftment.

[Choanal atresia and CHARGE syndrome]. / Atrésie des choanes et syndrome CHARGE.

The acronym CHARGE names a syndrome described by Pagon et Graham in 1981, which associates mainly ocular anomalies, i.e. coloboma, and uni or bilateral choanal atresia. We report three observations of children with choanal atresia confirmed by CT scan. One child exhibited all the signs of the CHARGE syndrome including cerebral involvement at autopsy.

[Pseudomonas aeruginosa infection in cystic fibrosis: predominance of a single strain and the influence of antibiotic therapy and the environment]. / Infection à bacille pyocyanique au cours de la mucoviscidose: prédominance d'un clone, influence des antibiotiques et de l'environnement.

We analysed the implantation pattern and persistence of Pseudomonas aeruginosa in the tracheobronchial tracts of patients with cystic fibrosis, and investigated the relation of this bacterium with the environment and antibiotic therapy. We used four different techniques to ensure the precise and detailed identification of isolates. In particular, chromosomal differences were assessed by pulsed field electrophoresis (pulsotype determination). Sputum samples were collected from 8 patients, from 6 to 22 years-old, over a period of 19 to 24 months. Only a single strain was isolated from samples from each five patients taken at different times, and there was a predominant strain in the samples from two others. Patient 8, aged 14, was free of infection throughout the study. None of the infections was eradicated by antibiotic therapy (an association of two antibiotics for 15 days at one or two months interval). The strains isolated from two patients became resistant to imipenem: 3 out of the 4 resistant strains were the result of mutation in the resident, susceptible strain. Swabs were taken from the environments of infected patients and were tested for P. aeruginosa: this bacteria was found in three sites, and two of these contained an isolate with the same pulsotype as the strain responsible for the infection, whereas no P. aeruginosa was detected in the environment of an uninfected patient. The detailed and accurate identification of the isolates (by pulsotyping) enabled us--to show that each infected patient was infected by a single or predominant strain,--to investigate the relationship of these strains with those in the environment and the effects of antibiotic therapy.

[Bourneville tuberous sclerosis manifested by prenatal finding of intracardiac tumors]. / Sclérose tubéreuse de Bourneville à révélation anté-néonatale par tumeurs intracardiaques.

Neonatal cardiac rhabdomyoma is the most frequent cardiac tumour in the newborn, and a classical way to diagnose tuberous sclerosis (Bourneville's disease). The authors report 4 cases, including 2 antenatal diagnosis: 2 of them had arrhythmia, one with asystolic cardiac failure and the other with cyanosis due to a right-left shunt; the tumour was asymptomatic in the 2 others. The 4 babies had clinical and radiological neurologic signs of tuberous sclerosis, initially or during the course of the disease. The authors discuss the polymorphism of the clinical presentation of tuberous sclerosis in the newborn. Although tuberous sclerosis is transmitted through a dominant gene, the expressivity and the penetrance are very variable. The authors discuss the examination and investigation of at risk family members. The genetic counsel is difficult but very important because of the poor neurologic prognosis of the disease.

Transplantation of umbilical cord blood in neuroblastoma.

It has been shown that umbilical cord blood contains concentrations of hematopoietic progenitor cells equal to those of normal adult bone marrow. We successfully performed transplantation of cord blood combined with a hematopoietic growth factor (rGM-CSF) in a seven year old child suffering from neuroblastoma. Cord blood cells were collected from an HLA identical sibling at the time of delivery and stored in liquid nitrogen. The patient was conditioned with busulfan 600 mg/m2 and cyclophosphamide 200 mg/kg before receiving the thawed cells. There were no immediate side effects. Hematological reconstitution occurred promptly (granulocytes greater than 0.5 x 10(9)/L on day 13, platelets greater than 30 x 10(9)/L on day 40), although at day 10 the child experienced acute grade II cortico-sensitive GvHD. Mixed hematopoietic chimerism was observed 2 months later and complete remission lasted for 8 months.

[Congenital myotonic dystrophy. Diagnostic difficulties]. / Dystrophie myotonique congénitale. Difficultés diagnostiques.

Congenital myotonic dystrophy is the neonatal form of Steinert myotonia. It is particularly severe when the mother carries the gene. The diagnosis is made after finding the myotonia in the mother. Prenatal diagnosis can be made by researching the Steinert's disease gene by fetal DNA analysis.

[Counseling for the prevention of HIV infections in pediatric situations]. / Conseils de prévention de l'infection au VIH en situation pédiatrique.

Health care professionals who are not adequately informed may have irrational attitudes when caring for HIV-infected pediatric patients. Prevention of contamination rests on thorough knowledge of high-risk pediatric groups and of potentially contaminating situations. In the delivery room and in intensive care settings, a broad set of measures for preventing contamination by blood is needed, whereas in maternity wards preventive steps are needed only during cord stump care. In pediatric departments, care should be taken to avoid injuries with sharp, blood-soiled instruments and to cover all skin lesions. HIV-infected children should be allowed to participate in group activities if they are healthy enough to do so. If an injury should occur, management includes immediate disinfection, reporting, and serial serologic testing, as well as early AZT therapy.

[Screening for hepatitis B carriers at the maternity unit of Besançon teaching hospital]. / Dépistage du portage de virus de l'hépatite B à la maternité du CHU de Besançon.

There are more than 280 carriers of the hepatitis B virus worldwide. The risk of chronic carriers, and a fatal outcome due to hepatocarcinoma, is estimated to be about 50% in Asian children contaminated by materno-fetal transmission. Serovaccination of the neonatal infant born to a HBV carrying mother provides protection in nearly 100% of cases. Routine screening for HBV carriers at the Besançon teaching hospital maternity unit led to the vaccination of 1.12% of the babies born in 1990, which corresponds to the current national average.

[Chronic intestinal pseudo-obstruction syndrome in infants and associated anomalies]. / Le syndrome de pseudo-obstruction intestinale chronique du nourrisson et les anomalies associées.

The authors report 2 cases of functional intestinal pseudo-obstruction in infancy associated with intestinal and urologic anomalies. In the first case many intestinal obstructions occurred from the age of 3 weeks and the boy was operated on at 14 months of age. A short small bowel and an intestinal malrotation were found at surgery; the alimentary canal was completely aperistaltic, and an ileostomy was performed. Further operations were carried out, for obstruction due to adhesions, and lastly to perform another ileostomy. An antenatal diagnosis of megacystis had been made with ultrasonography. In the second case, the pseudo-obstruction syndrome occurred at the age of 1 month, due to a volvulus of the small bowel with malrotation. A second operation, one month later because of lack of intestinal transit showed an aperistaltic bowel and a colostomy was performed. The intestinal continuity was set up again at the age of 9 months and a fractional feeding was started. A megacystis was found during urologic investigations. Growth is correct for both children at 3 years of age. A review of the literature allowed to list the most frequent digestive or extradigestive anomalies associated with this syndrome.

[Capnocytophaga septicemia during bone marrow transplantation. Apropos of 2 cases]. / Septicémie à Capnocytophaga au cours de greffe de moelle osseuse. A propos de deux observations.

Two cases of septicemia due to Capnocytophaga in pediatric bone marrow recipients are reported. These gram negative rods, which are part of the normal buccal flora, cause periodontitis and localized or systemic infections, usually in immunocompromised hosts. Severe, prolonged neutropenia and mucitis due to chemotherapy and/or radiotherapy are risk factors for this opportunistic infection. Recovery can be achieved with most of the drug combinations including a beta-lactam used in hematology.

[Rubinstein-Taybi syndrome in 4 cases]. / Illustration du syndrome de Rubinstein-Taybi par quatre observations.

The authors report on 4 cases of Rubinstein-Taybi syndrome. The specific features of this clinical entity of unknown aetiology, are emphasized: mental retardation, characteristic faecies, broad thumbs and large toes; short stature and cryptorchidism are often associated.

Marrow transplantation from HLA non-identical family donors for the treatment of leukaemia: a pilot study of 15 patients using additional immunosuppression and T-cell depletion.

Fifteen patients with high-risk leukaemia were given T-cell depleted marrow transplants from HLA non-identical related donors. They were treated with a combination of total body irradiation (TBI), high-dose cytosine arabinoside (Ara-C) and high-dose melphalan in an attempt to prevent a host-versus-graft reaction. Antilymphocyte globulins were given prior to transplantation for additional immunosuppression to 13 patients and in-vivo monoclonal antibody anti-human LFA1 to two. Engraftment and chimaerism assessed by HLA typing were achieved in 14 patients. Seven developed acute graft-versus-host disease (two fatal), one failed to engraft. Six patients died in complete remission from cytomegalovirus (CMV) interstitial pneumonitis and three remain alive in complete remission 2, 3 and 13 months after transplant. We conclude that aggressive immunosuppression allows for sustained engraftment of T-cell depleted HLA non-identical marrow. The incidence and severity of GVHD are acceptable and CMV pneumonitis remains the major problem.

[Infections and bone marrow autograft carried out for leukemias in children. Apropos of 47 cases]. / Infections et autogreffes de moelle osseuse réalisées pour leucémies chez l'enfant. A propos de 47 observations.

This study included 44 children undergoing autologous marrow transplantation for leukemia between August 1979 and June 1987. Three of them received a second transplant. In the phase of neutropenia, 38 children presented with fever. Nineteen septicemia occurred (13 Gram positive cocci, 6 Gram negative bacteria), and 2 interstitial pneumonitis were observed. All children with documented infection or a fever of unknown origin recovered after treatment, except 3, who died from infection. The latest antimicrobial therapy used was a combination of an aminoglycoside and a third generation cephalosporin. When necessary, vancomycin or amphotericin B were added. After engraftment (granulocyte count greater than 0.5 X 10(9)/l) 14 septicemia (which recovered) and 10 herpes zoster infections were observed. Only one patient died of infection (herpes zoster with encephalitis).

[Neurologic effects of acyclovir after an allogenic marrow graft]. / Effets neurologiques de l'aciclovir après greffe de moelle allogénique.

On the 4th day of acyclovir treatment for Herpes simplex pneumonia, a 28 month-old girl who had received allogenic marrow transplant for stage IV neuroblastoma presented with severe neurologic disorders including coma and choreic movements. These symptoms disappeared 9 days after acyclovir was stopped. The disturbance in acyclovir kinetics because of acute renal failure and/or a cerebral cortex atrophy might explain the poor neurologic tolerance of acyclovir. This reversible neurologic involvement on a prone patient should be known as a differential diagnosis of Herpes simplex encephalitis.

Successful graft-versus-host disease prevention without graft failure in 32 HLA-identical allogeneic bone marrow transplantations with marrow depleted of T cells by monoclonal antibodies and complement.

Thirty-two patients with acute leukemia, chronic granulocytic leukemia, or multiple myeloma received a T lymphocyte-depleted HLA-identical marrow. After being treated with pan-T monoclonal antibodies (MoAbs) and one round of baby rabbit complement, the mean percentage of T cell depletion was 94% +/- 4%. The number of residual viable T cell infused to the patient was 0.99 +/- 0.65 X 10(6) per kg body weight. The patients were conditioned with fractionated total body irradiation (TBI) (12 Gy) preceding high doses of cyclophosphamide (120 mg/kg). Methotrexate was used as an additional immunosuppressant in the first ten patients. For the following 22 patients no posttransplant immunoprophylaxis was administered. Eight patients died within three months due to complications related to transplantation. Engraftment was achieved in all evaluable patients, and no patient has a late graft failure. The proof of total chimerism was established in 24 patients. Twenty-four of 27 evaluable patients (88%) did not have an acute graft-v-host disease (GVHD) greater than grade 0 to 1. Two patients had a grade 2 (skin only), and one patient had a grade 4 acute GVHD (the latter had only 80% of T cell depletion). A medullary relapse occurred in 11 patients (nine of them had previously been defined as "high risk leukemia"). Our data suggest that it may not be necessary to deplete nearly all T cells to prevent acute GVHD in recipients of HLA-identical marrow.

[11q trisomy: apropos of 2 cases]. / La trisomie 11q: à propos de deux cas.

The authors report two new cases of 11q trisomy, one due to a "classical" (11q; 22q) translocation, one due to a desequilibrated (8q; 11q) translocation (de novo). The 2 phenotypes are compared with the data of the literature on the 11q trisomy syndrome.

Autologous bone marrow transplantation (ABMT) for acute leukaemia in complete remission: a pilot study of 33 cases.

Thirty-three leukaemic patients in CR were treated by high-dose therapy followed by ABMT: 18 of them had acute non-lymphoblastic leukaemia (ANLL) in first remission (CR1) with a mean age of 23.7 years (3-44). All but one of them were conditioned with a polychemotherapy regimen including 6-thioguanine, Ara-C, CCNU, and cyclophosphamide. The marrow cells were purged by chemical means in 16 cases. Five transplant-related deaths were observed: three cardiac failures, one interstitial pneumonitis and one aspergillus pneumonia. At the time of analysis (October 1984), four patients had relapsed and eight were still in unmaintained CR1 (44+, 46+, 30+, and five between 2.5+ and 8+ months post transplant). Fifteen patients had acute lymphoblastic leukaemia: four were autografted in CR1 and 11 children were grafted in CR2; the conditioning regimen was fractionated total body irradiation followed by cyclophosphamide for all but one patient who was conditioned with BACT (Burkitt leukaemia); the marrow was purged by a chemical agent in 11 patients and by monoclonal antibodies and C' in four: four out of 15 patients relapsed (two grafted in CR1 and two grafted in CR2); 10 patients are still in unmaintained CR: two adults grafted in CR1 (26+; 12+ months) and eight children with a mean follow-up of 13.4 months post graft (2 + -45+ months). The clinical study leads to the following conclusions: in adult patients the marrow should be harvested during CR1 and at the time of minimal residual disease. The quality of previous chemotherapy and conditioning regimen prior to ABMT play a prominent role in the in vivo eradication of the leukaemic cells. The real impact of marrow purging is still unknown and a larger series of homogeneous patients, conditioned with the same protocols and the same transplant timing, is required before any conclusions can be drawn.