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Cerebral Gigantism Associated With Wilm’s Tumor
0022-534 7/84/1326-1284$02.00/0 Vol. 132, December
THE JOURNAL OF UROLOGY
Copyright © 1984 by The Williams & Wilkins Co.
Printed in U.S.A.
ABSTRACTS INFECTIONS AND ANTIBIOTICS
of Hematology/Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois
Transferrin in Disease II: Defects in the Regulation of Transferrin Saturation With Iron Contribute to Susceptibility to Infection
R. L. HUNTER, B. BENNETT, M. TOWNS AND W.R. VOGLER, Department of Pathology and Laboratory Medicine, and Division of Hematology and Medical Oncology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia Amer. J. Clin. Path., 81: 748-753 (June) 1984 Data suggest that the high incidence of infections in patients with acute myelogenous leukemia may be related partly to defects in iron metabolism, including 1) a low level of transferrin, 2) a high level of serum iron and 3) an inability to reduce iron levels at the start of infection. The authors assayed the sera of 47 patients with acute myelogenous leukemia for iron, transferrin, percentage saturation and the ability of the serum to inhibit the growth of Pseudomonas aeruginosa in vitro. Of the 4 7 patients 28 had elevations in the percentage saturation of transferrin. Furthermore, these patients had a much lower P. aeruginosa inhibition index compared to normal controls. Patients undergoing bone marrow transplantation who failed to decrease the serum iron level and percentage saturation of transferrin were prone to refractory septic episodes. Normal host defenses effect a decrease in serum iron at the onset of infection by 2 mechanisms: 1) interleukin-I production by activated monocytes reduces serum iron and 2) neutrophilderived lactoferrin aids in iron binding. The authors concluded that patients with neutropenia and sustained hyperferremia are most prone to septic complications. J. M. K. 5 figures, 1 table, 18 references
Amer. J. Dis. Child., 138: 486-488 (May) 1984 Cerebral gigantism or Sotos' syndrome is marked by growth with excessive rapidity and nonprogressive mental retardation. A large head with hypertelorism, high-arched palate, and large hands and feet are the most common physical features. Approximately 132 cases have been reported in the literature. The authors report on 2 children with this syndrome who subsequently had Wilms tumor. Eight other children have been reported with malignant tumors of various types but this case represents the first Wilms tumor reported with this syndrome. It appears that cerebral gigantism is associated with an increased incidence of neoplasia and, in that regard, is similar to the Beckwith-Wiedemann syndrome and its association with malignancies. W. J. C. 3 figures, 1 table, 25 references
Editorial comment. Cerebral gigantism Joms nonfamilial (sporadic) aniridia, hemihypertrophy and the BeckwithWiedemann syndrome as another congenital anomaly in which the affected children are at high risk for Wilms tumor. It is interesting that gigantism of other organs, such as the tongue and liver, and of over-all body size is a hallmark of the Beckwith-Wiedemann complex, along with umbilical hernia or omphalocele and hypoglycemia. L. R. K. Nephrogenic Adenoma and Embryonic Kidney Tubules Share PN A Receptor Sites P. DEVINE, A. A. UCCI, H. KRAIN, V. E. GAVRIS, B. BHAGAVAN, J. A. HEANEY AND J. ALROY, Departments
S. of Pathology and Urology, Tufts University Schools of Medicine and Veterinary Medicine, and New England Medical Center, Boston, Massachusetts, Department of Pathology, Sinai Hospital, Baltimore, Maryland, and Mayo Clinic and Mayo Foundation, Rochester, Minnesota
Viral Infections in Immunosuppressed Patients
S. Z. HIRSCHMAN, Division of Infectious Diseases, The Mount Sinai Medical Center, New York, New York N. Y. State J. Med., 84: 241-244 (May) 1984 The author reviewed the general problems of viral infections in the immunosuppressed patient. The general mechanisms of host defenses against viral infections, such as antibody induction and cell mediated immunity, as well as nonspecific factors, such as interferon, nutrition, temperature and so forth, are presented. The major viruses involved in infections of the immunocompromised patient are discussed and the acquired immune deficiency syndrome is detailed with clarity and insight, considering that the etiological cause of this disease was found after the paper was written. J. A. A. 30 references
ONCOLOGY AND CHEMOTHERAPY Cerebral Gigantism Associated With Wilm's Tumor
S. GAYNON AND A. K. POZNANSKI, Department of Radiology and Division of Hematology, Children's Memorial Hospital, and Department of Radiology and Division
V. MALDONADO, P.
Amer. J. Clin. Path., 81: 728-732 (June) 1984 N ephrogenic adenoma is an unusual lesion of the urothelium believed most often to arise via metaplastic transformation in response to operative injury, presence of foreign bodies and chronic urinary tract infection. However, the striking similarity between nephrogenic adenomas and mesonephric tubules lends credence to an embryonic mechanism of origin. Lectins are carbohydrate-binding proteins located on the cell surface, the analysis of which aids in comparing membrane-associated glycoconjugates of 1 cell type to another. The authors used biotinylated lectin probes and an avidin-biotin-peroxidase visualant to assess previously documented cases of nephrogenic adenomas and compared them to 1) normal transitional epithelium, 2) human embryonic kidney, 3) cystitis cystica, 4) cystitis glandularis and 5) squamous metaplasia. The most notable finding was an impressive binding homology for free receptors of the lectin pentosenucleic acid between nephrogenic adenomas and the tubules of the embryonic kidney. Such an association was not seen in the other groups studied, which suggests some validity to an embryonic etiology for nephrogenic adenomas. J. M. K. 4 figures, 2 tables, 27 references
1284
THE JOURNAL OF UROLOGY
Copyright © 1984 by The Williams & Wilkins Co.
Printed in U.S.A.
ABSTRACTS INFECTIONS AND ANTIBIOTICS
of Hematology/Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois
Transferrin in Disease II: Defects in the Regulation of Transferrin Saturation With Iron Contribute to Susceptibility to Infection
R. L. HUNTER, B. BENNETT, M. TOWNS AND W.R. VOGLER, Department of Pathology and Laboratory Medicine, and Division of Hematology and Medical Oncology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia Amer. J. Clin. Path., 81: 748-753 (June) 1984 Data suggest that the high incidence of infections in patients with acute myelogenous leukemia may be related partly to defects in iron metabolism, including 1) a low level of transferrin, 2) a high level of serum iron and 3) an inability to reduce iron levels at the start of infection. The authors assayed the sera of 47 patients with acute myelogenous leukemia for iron, transferrin, percentage saturation and the ability of the serum to inhibit the growth of Pseudomonas aeruginosa in vitro. Of the 4 7 patients 28 had elevations in the percentage saturation of transferrin. Furthermore, these patients had a much lower P. aeruginosa inhibition index compared to normal controls. Patients undergoing bone marrow transplantation who failed to decrease the serum iron level and percentage saturation of transferrin were prone to refractory septic episodes. Normal host defenses effect a decrease in serum iron at the onset of infection by 2 mechanisms: 1) interleukin-I production by activated monocytes reduces serum iron and 2) neutrophilderived lactoferrin aids in iron binding. The authors concluded that patients with neutropenia and sustained hyperferremia are most prone to septic complications. J. M. K. 5 figures, 1 table, 18 references
Amer. J. Dis. Child., 138: 486-488 (May) 1984 Cerebral gigantism or Sotos' syndrome is marked by growth with excessive rapidity and nonprogressive mental retardation. A large head with hypertelorism, high-arched palate, and large hands and feet are the most common physical features. Approximately 132 cases have been reported in the literature. The authors report on 2 children with this syndrome who subsequently had Wilms tumor. Eight other children have been reported with malignant tumors of various types but this case represents the first Wilms tumor reported with this syndrome. It appears that cerebral gigantism is associated with an increased incidence of neoplasia and, in that regard, is similar to the Beckwith-Wiedemann syndrome and its association with malignancies. W. J. C. 3 figures, 1 table, 25 references
Editorial comment. Cerebral gigantism Joms nonfamilial (sporadic) aniridia, hemihypertrophy and the BeckwithWiedemann syndrome as another congenital anomaly in which the affected children are at high risk for Wilms tumor. It is interesting that gigantism of other organs, such as the tongue and liver, and of over-all body size is a hallmark of the Beckwith-Wiedemann complex, along with umbilical hernia or omphalocele and hypoglycemia. L. R. K. Nephrogenic Adenoma and Embryonic Kidney Tubules Share PN A Receptor Sites P. DEVINE, A. A. UCCI, H. KRAIN, V. E. GAVRIS, B. BHAGAVAN, J. A. HEANEY AND J. ALROY, Departments
S. of Pathology and Urology, Tufts University Schools of Medicine and Veterinary Medicine, and New England Medical Center, Boston, Massachusetts, Department of Pathology, Sinai Hospital, Baltimore, Maryland, and Mayo Clinic and Mayo Foundation, Rochester, Minnesota
Viral Infections in Immunosuppressed Patients
S. Z. HIRSCHMAN, Division of Infectious Diseases, The Mount Sinai Medical Center, New York, New York N. Y. State J. Med., 84: 241-244 (May) 1984 The author reviewed the general problems of viral infections in the immunosuppressed patient. The general mechanisms of host defenses against viral infections, such as antibody induction and cell mediated immunity, as well as nonspecific factors, such as interferon, nutrition, temperature and so forth, are presented. The major viruses involved in infections of the immunocompromised patient are discussed and the acquired immune deficiency syndrome is detailed with clarity and insight, considering that the etiological cause of this disease was found after the paper was written. J. A. A. 30 references
ONCOLOGY AND CHEMOTHERAPY Cerebral Gigantism Associated With Wilm's Tumor
S. GAYNON AND A. K. POZNANSKI, Department of Radiology and Division of Hematology, Children's Memorial Hospital, and Department of Radiology and Division
V. MALDONADO, P.
Amer. J. Clin. Path., 81: 728-732 (June) 1984 N ephrogenic adenoma is an unusual lesion of the urothelium believed most often to arise via metaplastic transformation in response to operative injury, presence of foreign bodies and chronic urinary tract infection. However, the striking similarity between nephrogenic adenomas and mesonephric tubules lends credence to an embryonic mechanism of origin. Lectins are carbohydrate-binding proteins located on the cell surface, the analysis of which aids in comparing membrane-associated glycoconjugates of 1 cell type to another. The authors used biotinylated lectin probes and an avidin-biotin-peroxidase visualant to assess previously documented cases of nephrogenic adenomas and compared them to 1) normal transitional epithelium, 2) human embryonic kidney, 3) cystitis cystica, 4) cystitis glandularis and 5) squamous metaplasia. The most notable finding was an impressive binding homology for free receptors of the lectin pentosenucleic acid between nephrogenic adenomas and the tubules of the embryonic kidney. Such an association was not seen in the other groups studied, which suggests some validity to an embryonic etiology for nephrogenic adenomas. J. M. K. 4 figures, 2 tables, 27 references
1284