Mutational activation of ras genes is absent in pediatric osteosarcoma.

Activation of ras oncogenes is found in human cancers; overall it is observed in 15% of all neoplasms. The purpose of this study was to assess the extent of involvement of ras oncogenes in osteosarcoma. Tumor samples from a series of 49 pediatric patients diagnosed with osteosarcoma and treated at our institution were evaluated. Paraffin-embedded tumor samples from diagnostic biopsies, from tumor en bloc resection tissue after neoadjuvant chemotherapy, and samples from metastases were examined in search of point mutations in H, K, and N-ras genes at codons 12 and 61 by means of polymerase chain reaction (PCR), slot-blotting, and radioactive labeled specific DNA probes. A total of 92 archival samples were studied. No point mutations activating these genes were found. These findings suggest that the activation by point mutations at codons 12 and 61 of the H, K, and N-ras genes does not play a role in the pathogenesis of human osteosarcoma. Since no point mutations in codons 12 and 61 were detected, it was not possible to establish any correlation between the ras genes and clinical or histologic findings.

[The pediatric heart transplant. Its evolution and early postoperative complications]. / Trasplante cardíaco pediátrico. Evolución y complicaciones postoperatorias tempranas.

BACKGROUND: Cardiac transplantation is an acceptable therapeutic alternative for cardiac diseases refractory to other forms of management in adults as well as in infants and children. METHODS: Between 1987-1992 7 children (4 girls and 3 boys) underwent cardiac transplantation: four with dilated cardiomyopathy, one with cardiac fibroma and two with hypertrophic cardiomyopathy. Age at transplantation ranged from 2 months to 13 years and 5 months, with a follow-up ranging from 15 months to 5 years and 9 months. Prophylaxis of acute rejection consisted of cyclosporine, azathioprine and glucocorticoids. RESULTS: Two patients presented acute rejection three weeks after cardiac transplantation, with a good response to high dose glucocorticoids. Two patients developed severe infection (sepsis by Staphylococcus aureus) with successful outcome after antibiotic treatment. One patient died in the early postoperative period and other after 4 years 11 months postransplantation because myelodysplastic syndrome. At present only one case is receiving glucocorticoids in immunoprophylaxis. The status is asymptomatic in the other 5 patients with a normal height-weight development. CONCLUSIONS: Heart transplantation provides durable therapy for congenital and myopathic heart disease in infants and children with an excellent quality of life.

Respiratory distress syndrome in copper deficiency: an experimental model developed in rats.

An experimental model has been developed to investigate the effect of copper deficiency on lung maturity in the newborn rat. Three groups of female Sprague-Dawley rats were used: the copper-deficient group was fed with a copper-free diet; the control group received a copper-adequate diet, and the pair-fed group was fed with a limited copper diet. After gestation and delivery, 35% of the newborn copper-deficient group showed respiratory distress syndrome (RDS). The neonatal lungs were isolated and processed for ultrastructural and biochemical study: A greater thickness of the air-blood barrier was observed in the lungs of the copper-deficient group, however, no other differences were observed in the rest of the pulmonary structures. Quantitative differences in pulmonary surfactant were not found in the three groups. The thickness of the air-blood barrier might explain the RDS observed in the copper-deficient newborns.

Study of HLA antigens in patients with osteosarcoma.

HLA typing was carried out in a group of twenty-four Spanish unrelated patients diagnosed with conventional high-grade osteosarcoma. All of them were typed for HLA-A and HLA-B antigens. A significant increase in the HLA-A11 (33.3 vs. 12%, chi 2 = 9.752, corrected p value = 0.016) and HLA-B7 (33.3 vs. 11.3%, chi 2 = 10.919, corrected p value = 0.011) was found in osteosarcoma patients compared with the control group. A trend towards an increased frequency in HLA-A24 and HLA-A28 antigens was also found.

[Importance of copper in respiratory distress syndrome in newborn rats]. / Importancia del cobre en el síndrome de dificultad respiratoria en neonatos de rata.

To investigate the effect of copper-deficiency on Respiratory Distress Syndrome in newborn rats, 30 female Sprague-Dawley rats were used. The animals were divided at random into 3 groups of 10: The copper-deficient group was fed a copper-free diet from 35 days before gestation to delivery; the control group, fed a copper adequate diet and the pair-fed group was fed a limited copper adequate diet. After birth, an important percentage of the copper-deficient newborn showed symptoms of respiratory distress syndrome, such as apnoea, tachypnoea, and subcostal retraction; however, the pulmonary surfactant in the three groups did not present quantitative significant differences.

A sporadic case of Wiskott-Aldrich syndrome.

We present a sporadic case of Wiskott-Aldrich syndrome, with no possibility of complete correction through bone marrow transplant, because no histocompatible donor could be found. The findings obtained in the immunopathological study performed are presented, in the attempt of correlating them with some clinical manifestations and with the aim of starting symptomatic treatment of the same.

Relation between maternal plasmatic zinc levels and uterine contractility.

336 pregnant women participated in a study to determine the possible relation between maternal plasmatic zinc levels ( PZL ) and uterine contractibility . PZL during gestation and at the moment of delivery were evaluated in relation to the mode of delivery, the duration of the active period of delivery, and the presence of hemorrhage during the first 30 min postpartum. A significant difference (p less than 0.001) between PZL at different periods of gestation was found. There were significant differences (p less than 0.05) between PZL and duration of the active period of delivery and PZL and final mode of delivery. In 10 cases, a cesarean section had to be performed due to uterine atony. In these patients, the PZL were significantly lower than in the rest. The role of zinc in uterine muscle contractibility is discussed. The possibility of supplementation in those women with low zinc levels (less than 60 micrograms/dl; 9 mumol/l) is recommended.

[Experience with the use of a subcutaneous venous access system in 57 pediatric patients]. / Experiencia con el uso de un sistema de acceso venoso subcutáneo en 57 pacientes pediátricos.

Since May of 1983 to December of 1988, 62 subcutaneous venous reservoires were implanted in 57 pediatric patients; 29 were boys and the remaining 28 were girls between the ages of 2 and 18 years. Fifty-one patients had malignant illnesses and seven other pathologies. The reservoir was used for the endovenous administration of cytotoxic chemotherapy and antibiotics, hidration, nutrition, the transfusion of blood product and the extraction of samples for laboratory tests. In our experience the most important complication was the infection of the system, on the average 0.06 episodes of infections occurred for every 100 days per reservoir. Other complications observed were less frequent. In our opinion the subcutaneous reservoir represents an useful tool for the care of the pediatric patients who require a central venous catheter for long periods of time.

Use of recombinant human granulocyte-macrophage colony stimulating factor in an infant with reticular dysgenesis.

We present the case of a 2-month-old infant with reticular dysgenesis who was treated with recombinant granulocyte-macrophage colony stimulating factor with the aim of stimulating granulopoiesis while awaiting bone marrow transplant.

[Ultramicroscopic aspects of the lungs in copper-deficient newborn rats]. / Aspectos ultramicroscópicos en pulmón de neonatos de rata cuprodeficiente.

In order to investigate the possible morphological alterations in the lungs of copper-deficient neonatal rats, three groups of 10 female Sprague-Dawley rats were used. The copper-deficient group was fed with a copper-free diet beginning at 35 days before pregnancy and throughout the gestation period. The control group received a diet adequate in copper and the pair-fed group received a diet with adequate copper content, but in limited amounts. After birth, the ultrastructural study of the neonatal lungs showed a thickening of the air-blood barrier in the copper-deficient group. No other differences were observed in the rest of the pulmonary structures.

Zinc concentration of amniotic fluid in the course of pregnancy and its relationship to fetal weight and length.

The interest of the zinc content of amniotic fluid and its relationship to different maternal and fetal parameters is recent. It was reported that the zinc content of amniotic fluid increases in the course of pregnancy probably as an indirect indicator of an increased rate of protein synthesis. These considerations and the known important metabolic role of zinc led us to study the zinc content of amniotic fluid and its importance in the growth and development of the fetus. Amniotic fluid samples of 24 women with normal pregnancies and normal term deliveries were studied. The samples were obtained between weeks 29 and 42 of gestation by transabdominal amniocentesis and the zinc content was determined by means of atomic absorption spectrophotometry. The zinc content of amniotic fluid increases significantly during the third trimester. A positive linear correlation was found between the zinc concentration of amniotic fluid and fetal weight and length. Fetal growth and development would depend on the zinc available due to its biological functions and thus the increase of zinc concentration of amniotic fluid in the last weeks of gestation and its correlation with fetal weight and length. These results suggest that the determination of zinc content of amniotic fluid can be useful in the assessment of fetal development and well-being, and of course of pregnancy.

Partial splenic embolisation and improvement of hypersplenism before liver transplantation.

A case of partial splenic embolisation (PSE) that was performed in a girl with severe leucothrombocytopenia due to hypersplenism is described. Treatment with PSE is a safe and effective alternative to splenectomy that prevents clinical complications due to haematological disorders and improves hypersplenism before liver transplantation.

[Neuronal ceroid-lipofuscinosis. Diagnosis by skin biopsy. Physiopathology (author's transl)]. / Ceroidolipofuscinosis neuronal. Diagnóstico por biopsia cutánea. Fisiopatología.

Authors report a case of Batten's disease (neuronal ceroid-lipofuscinosis), in its late infantile form (Jansky-Bielschowsky), so as the ability to identify the process by ultrastructural study of the skin.