Therapeutic approaches to factor X deficiency with emphasis on the use of a new clotting-factor concentrate (Konyne).
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Biomedical subjects
Publications and source records attributed to F B Ruymann.
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Juvenile polyps occur in adults and infants; where multiple, they may be complicated by progressive cachexia with hypoalbuminemia and electrolyte depletion. We report a fatal case of multiple juvenile polyposis with cachexia, alopecia, and megalocephaly presenting in a 9-month-old infant, and review 2 additional cases in infancy. A similar syndrome in adults had been described by Cronkhite and Canada, with intestinal polyposis, nail dystrophy, hyperpigmentation, and alopecia. Histological examination of a polyp from one case of Cronkhite-Canada syndrome suggests a juvenile rather than adenomatous pathology. Multiple juvenile polyps can cause devastating enteropathy and cachexia.
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Two boys with neurofibromatosis-Noonan syndrome in whom acute lymphoblastic leukemia (ALL) developed are described. Both patients demonstrated B-lineage leukemias with normal cytogenetics. They were treated with combination chemotherapy and remain in remission off therapy. Patients with neurofibromatosis-Noonan syndrome may be at increased risk for ALL.
A 15-month-old boy with rapidly progressive proptosis had an unusual malignant germ-cell tumor of the orbit and nasopharynx identified as an endodermal sinus tumor (yolk-sac carcinoma). Endodermal sinus tumors arising in extra gonadal locations are highly malignant and generally lethal. The boy was treated with radiation and three-drug chemotherapy and has survived more than eight years.