1606
PEDIATRIC UROLOGY
Pulmonary Embolectomy: Its Place in the Management of Pulmonary Embolism H. H. GRAY, G. A.H. MILLER AND M. PANETH, Department of Cardiology and Cardiothoracic Surgery, Brampton Hospital, London, England Lancet, 1: 1441-1445 (June 25) 1988 The authors present a literature review on pulmonary embolism and conclude that pulmonary embolectomy is indicated in patients in whom thrombolytic therapy is contraindicated, or in those who are so compromised that a trial of medical management is not warranted and in those deteriorating on thrombolytic therapy. They state that patients with acute massive pulmonary emboli should be transferred to a center with full cardiopulmonary bypass capabilities. 60 references James H. Nelson, III, M.D. Columbus, Ohio
PEDIATRIC UROLOGY Worldwide Experience in Newborn Screening for Classical Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency S. PANG, M. A. WALLACE, L. HOFMAN, H. C. THULINE, C. DORCHE, I. C. T. LYON, R. H. DOBBINS, S. KLING, K. FUJIEDA AND S. Suw A, Department of Pediatrics, University of Illinois, College of Medicine, Chicago, Illinois; Royal Infirmary and Stobill General Hospital, Glasgow, Scotland; Department of Human Services, State Laboratory of Public Health, Seattle, Washington; Centre de Depistage Neonatal, Hopital Debrousse, Lyon, France; National Testing Center, Auckland, New Zealand; Illinois Department of Public Health, Chicago, Illinois; Hokkaido University School of Medicine, Sapporo, Japan and Department of Pediatrics, Kanagawa Children's Medical Center, Yokohama, Japan Pediatrics,, 81: 866-874 (June) 1988 The authors report the results of a worldwide 8-year program to screen newborns for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. France, Italy, Japan, New Zealand, Scotland and the United States participated in the study. High incidences of congenital adrenal hyperplasia occur in the Yupik Eskimos of Alaska (1:282) and in people of La Reunion, France (1:2,141). Excluding these 2 areas 1,093,310 newborns were screened by a heel stick capillary sample at the same time as newborn screening for phenylketonuria and congenital hypothyroidism. Samples were submitted for 17-hydroxyprogesterone analysis. A total of 77 cases of classical congenital adrenal hyperplasia was discovered for a worldwide incidence of 1:14,199 for homozygous patterns and 1:60 for heterozygous patients. The salt wasting form of congenital adrenal hyperplasia occurred in 58 cases (1:18,850) compared to 19 (1:57,543) with the simple virilizing form. This ratio of 3:1 was significantly higher than previous reports when comparing the salt losing and simple virilizing forms of congenital adrenal hyperplasia. This finding suggests improved case detection by newborn screening. The authors note that the benefit of screening was the prevention of adrenal crisis, death, incorrect sex assignment (3 cases in the group) and early diagnosis. The average cost was
$1.16 per newborn, which the authors believed was cost-effective compared to a lifetime of productive individuals. The authors noted false positive levels in sick, premature and low birth weight infants. 7 tables, 44 references Robert Kay, M.D. Cleveland, Ohio
Demonstration of Both Primary and Secondary Reninism in Renal Tumors in Children K. YOKOMORI, T. HORI, T.. TAKEMURA AND Y. TSUCHIDA, Department of Pediatric Surgery, The Japanese Red Cross Medical Center, Tokyo, Japan
J. Ped. Surg., 23: 403-409 (May) 1988 Hypertension is a well recognized complication in children with renal tumors. Although recent reports have related this finding to elevated renin activity, it remains unclear as to whether this represents renin secretion by the tumor cells themselves or renin secretion by glomeruli rendered ischemic by compression by the tumor. In this report histochemical studies were made in 2 patients with this association. A 9month-old girl had bilateral Wilms tumor associated with the Beckwith-Wiedemann syndrome. Blood pressure averaged 140/ 90 before treatment. Histochemical stains of the excised tumor revealed staining for renin in the well developed glomeruloid bodies comprising the tumors. A female newborn had a congen ital mesoblastic nephroma with elevated plasma renin activity and tissue renin activity in the tumor 5 times that of normal control renal tissue. Blood pressure reached 140/90 by the time of the operation. Histochemical studies of the tumor revealed no renin staining. The tumor consisted primarily of leiomyomatous stroma but it did show staining in the juxtaglomerular apparatus of normal glomeruli entrapped within the tumor. Thus, it appears that the increased renin activity associated with these tumors may arise as a consequence of either mechanism, that of a primary secretion by cells within the glomeruloid bodies of Wilms tumor and that of compression of normal glomeruli by invading tumor issue. 7 figures, 5 tables, 31 references Terry D. Allen, M.D. Dallas, Texas
Extrarenal Wilms' Tumor-A Case Report H.-S. LAI, W.-T. HUNG AND S.-W. How, Departments of Surgery and Pathology, National Taiwan University Hospital, National Taiwan University, Taipei, Taiwan, Republic of China J. Ped. Surg., 23: 454-456 (May) 1988
Extrarenal Wilms tumor is a rare but well recognized phenomenon. A 5-year-old girl presented with a mass in the right groin that proved to be a Wilms tumor incarcerated in a hernia sac. The tumor was removed completely, and sonography and excretory urography failed to reveal evidence of any additional disease. Chemotherapy was recommended but refused by the family. She returned with a recurrent mass and repeat evaluation, including a computerized tomography scan, again failed