Teaching young patients--and their families--about home peritoneal dialysis.
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Biomedical subjects
Publications and source records attributed to S Gross.
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In order to evaluate the utility of the neutrophil specific antigens NA1, NA2, Vaz (NC1), NB1 and 9a in the documentation of bone marrow chimeras in recipients of allogeneic bone marrow grafts, neutrophil antigen typing was performed by EDTA microagglutination on the families of 17 patients with hematopoietic disorders under evaluation for bone marrow transplantation. Mendelian segregation independent of HLA and mutually independent was noted for the NA, NB and 9a systems. Vaz (NC1) segregated with and was included in NA2. Serological complexity was noted for NA1 and NA2. Typing for neutrophil antigens was achieved for 13 of 17 patients. Eight of 10 patients with HLA identical siblings had neutrophil antigen markers differing between donor and recipient. Conversion to donor neutrophil phenotype was documented for four recipients of bone marrow grafts. The neutrophil antigens, particularly of the NA system, appear to be useful additional markers for allogeneic bone marrow engraftment.
A 13-year-old boy with Hodgkin's disease developed acute polyneuropathy and autoimmune hemolytic anemia. Sural nerve biopsy and postmortem examination demonstrated no metastatic involvement and a lack of cellular inflammatory infiltration in the nervous system. There was significant axonal degeneration in peripheral nerves and the dorsal funiculus. These findings suggest a non-metastatic polyneuropathy associated with Hodgkin's disease, and similar cases, predominantly in adult patients, have been reported. This case emphasizes the occurrence of a paraneoplastic syndrome in children, though such syndromes appear to be rare compared with adults.
Eighty-one children undergoing tonsillectomies and adenoidectomies (T and A) were compared with a control group of 88 hospital patients. A significantly higher incidence of prior T and A was found to have been performed on the siblings and parents of the T and A group than the controls. Environmental, genetic or attitudinal factors could be responsible for this familial clustering.
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We prepared human conjugated bilirubin by isolation from fresh gall bladder bile and by biosynthesis using liver homogenates. The isolation protocol was modified after Lucassen (doctoral thesis, Univ. Utrecht, 1961), and the in vitro synthesis was done with fresh liver homogenates in the presence of D-glucaro-1,4-lactone (a glucuronidase inhibitor). From direct analyses of these preparations with 270-MHz proton nuclear magnetic resonance and field-desorption mass spectrometry, we deduced that a major conjugated bilirubin species in bile is a diglucuronide, whereas in liver biosynthesis it is a diconjugate containing glucuronic acid and possibly glucuronolactone co-esterified to the bilirubin backbone. If the glucuronolactone is indeed present in the native biosynthetic material, we do not know whether it derives from glucuronic acid and, if so, whether lactonization occurs before or after the acid has been esterified to form the conjugate.
Normal newborn infants have a prolonged partial thromboplastin time compared to that of older infants or adults. This finding has been related to combined deficiencies of multiple clotting factors, with the exception of proaccelerin (factor V) and antihemophilic factor (factor VIII). The present study confirms the presence of decreased titers of Hageman factor (HF, factor XII), plasma prekallikrein, and high molecular weight kininogen during the neonatal period, as demonstrated in clotting assays; the degree of these relative deficiencies is usually such that only the low titer of HF appears to contribute significantly to the abnormally long PTT. Additionally, procoagulant titers of HF and plasma prekallikrein were relatively lower than the concentration of these factors determined immunologically. The mechanisms underlying this phenomenon are not known.
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Leukocytosis with predominance of poly morphonuclear leukocytes is not unusual in a variety of primary or metastatic hepatic tumors. However, to date no apparent explanation for this event is available. This paper describes extreme leukocytosis in a 17-year-old black male with lymphoepithelioma after development of metastases to the liver. In order to identify the source of leukocytosis, studies of granulocytic colony-stimulating activity (CSA) of the patient's plasma and leukocyte-conditioned media (LCM) were carried out and compared with normal controls. The assay system consisted of nonadherent marrow cells of hematologically normal individuals and normal mice cultured in semi-solid culture media. In this system, the number of granulocytic colony-forming units (CFU-C) is proportional to the amount of stimulating activity present, and therefore allows for quantitative comparison. The number of colonies produced under the influence of patient's plasma was significantly higher (p less than 0.001) than those obtained from the patient's LCM or from control plasma or LCM. This pattern is the reverse of that seen in normal individuals in whom the major sources of CSA are macrophages and monocytes. It is conceivable that malignant cells are the source of additional CSA.
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Glutathione peroxidase, a selenium-dependent enzyme, and tocopherol interact and may, indeed, compensate for deficiencies of one or the other. Iron-supplemented, "tocopherol-sufficient," low-birth-weight premature infants fed (selenium-poor) formulas rich in polyunsaturated fatty acid (greater than 35% of fat as PUFA) develop severe hemolysis secondary to erythrocyte phosphatidyl ethanolamine loss. By further increasing tocopherol supplementation one can prevent the clinical expression of a relative tocopherol and an absolute selenium--glutathione peroxidase deficiency. Selenium-poor cow's milk preparations containing 12--15% of fat as polyunsaturated fatty acids, trace amounts of iron, and 12--15 mg/liter of tocopherol are sufficient to maintain proper nutrition without risk of increased peroxidation.
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