The use of glucagon and isoproteronol in the evaluation of isolated pulmonary stenosis.
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Biomedical subjects
Publications and source records attributed to A Kahn.
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The medical records of 299 Belgian and immigrant children who died between 15 days and one year in the years 1970-1977 have been analysed. Fifty two died of congenital malformations, 119 of major infections and 128 died suddently for no obvious reason; neither the clinical history nor the laboratory data would account for death. In both the Belgian and immigrant population the children who died suddenly and unexpectedly did not differ from the other groups of children who died in respect of social class, mother's age at delivery, her parity and medical history, the baby's gestational age, birth weight and Apgar score, the method of feeding, the physical growth or psychomotor development. Infection is not more frequent in the week before death and there is no seasonal variation. The majority of unexpected deaths occur between 2 and 4 months of age and the child is most often discovered between 6 and 12 a.m. These characteristics are also observed in children who die of sudden infant death syndrome.
Meningococcic shocks may induce fatal acute pulmonary edemas. Ten children, who died in this condition, have been compared to 19 others, who during the treatment of the shock, developed right heart failure, without acute pulmonary edema (8 deaths, 11 survivals). The three groups of children could not be differentiated regarding their clinical status on admission, during the shock phase, or the treatments administered. Composition, volume and speed of administration of fluids were similar in the three groups. An increase in pulmonary capillary permeability could have occurred, and lead to the development of acute pulmonary failure, as presented by the children of the first group.
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Clinically, the splayfoot is characterized by valgus of the great toe with bunion formation in association with a relative varus position of the first metatarsal. On the lateral side of the foot, there is varus of the fifth toe with a relative valgus position of the fifth metatarsal and resultant bunionette formation. Radiographically, splayfoot is characterized by an intermetatarsal angle between the first and second metatarsals of greater than 12 degrees, and an intermetatarsal angle between the fourth and fifth metatarsals of greater than 8 degrees. The Giannestras splayfoot procedure consists of excision of the bunion and bunionette, adductor tenotomy, opening-wedge osteotomy of the first and fifth metatarsal bases, and realignment of the first and fifth toes. One-hundred and sixteen splayfoot operations were performed in 72 patients, with an average follow-up of 5.2 years. Results were considered excellent in 33% (39 feet), good in 45% (52 feet), fair in 14% (16 feet), and poor in 8% (9 feet). The primary cause of a fair or poor result was collapse of the bone graft at the base of the first metatarsal, causing recurrent varus and symptomatic hallux valgus.
Balanced chromosomal abnormalities such as translocations and inversions have been identified in many genetic diseases. Cloning of the breakpoints involved in these abnormalities has led to the identification of the disease-related genes. Recent reports suggest the presence of a mental retardation locus at Xq11-12. We have identified a female patient with a balanced translocation t (X;12) (q11;q15) associated with mild mental retardation. We identified a yeast artificial chromosome spanning the X-chromosome breakpoint by using fluorescent in situ hybridization techniques. A cosmid library of this YAC has been constructed and the search for candidate genes is in progress.
The use of genetically modified tumor cells as vaccines has been successful in numerous animal models of grafted syngenic tumors and has provided the groundwork for many clinical trials of gene therapy in cancer patients. To investigate the real efficacy of ex vivo gene therapy-based vaccines, we used transgenic mice that express the SV40 large T and small t antigens under the control of hepatic antithrombin III (ASV-B)-regulatory sequences. These mice systematically develop hepatocarcinoma. Hepatoma cells, derived from ASV-B transgenic mice, were gene-transduced to express either interleukin-2, interleukin-4, the granulocyte-macrophage colony-stimulating factor, or the T-cell costimulatory molecule B7.1. First, we demonstrated the vaccine potential of engineered hepatoma cells by immunizing nontransgenic mice with these cells, which prevented the growth of subsequent grafted nontransduced hepatoma cells. However, vaccination of pretumoral transgenic animals with various combinations of engineered hepatoma cells failed to inhibit hepatoma onset and progression. Rather, tumor development in ASV-B mice appears to be dependent on the immune system, since neonatal induction of immunotolerance to tumor in ASV-B mice cells was associated with a moderate, but significant, acceleration of tumor development. These results seriously call into question the efficacy of this strategy of active vaccinotherapy against natural tumors.