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Sporadic Familial Goitrous Hypothyroidism
DONALD E. PICKERING, M.D.;
GLENN E. SHELINE, M.D.;
JACKSON T. CRANE, M.D.
AMA J Dis Child. 1957;93(5):510-518.
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Introduction
A survey of sporadic cretinism in the United States conducted by Sir William Osler in 1897 revealed that 7 among 60 cases studied were goitrous; moreover, 3 of those 7 cases were from one family of five children.1 Observations with respect to sporadic goitrous hypothyroidism were not significantly enlarged upon until the introduction of I131 to clinical studies of human thyroid metabolism by Hamilton and Soley in 1939.2 While sporadic goitrous hypothyroidism has subsequently undergone rather extensive investigation, attempts to clarify its pathophysiology are of recent origin.3-16
Eight children with this disorder are presented in this report in conjunction with data from laboratory investigations of their altered iodine metabolism. Pathologic data from these and other cases are described in a subsequent publication.17
Report of Cases
CASE 1.–A boy was born uneventfully on March 27, 1948, after a normal full-term pregnancy. The family had never
. . . [Full Text PDF of this Article]
Author Affiliations
San Francisco
Dr. Pickering is formerly of the Department of Pediatrics, University of California School of Medicine.; From the Departments of Pediatrics (Dr. Pickering), Radiology (Dr. Sheline), and Pathology (Dr. Crane) of the University of California School of Medicine, and the Department of Pediatrics (Dr. Pickering), University of Oregon Medical School, Portland, Ore.
Footnotes
Submitted for publication Dec. 11, 1956; revised Jan. 18, 1957; accepted Feb. 13.
This study has received support from the Cox Fund (171), the Research Committee, and. the Mary H. K. Welsh Endowment Fund to the University of California Medical Center, and from the United States Public Health Service (Dr. Pickering).
Cases 1 through 7 were seen at the University of California Hospital. Case 8 was seen at the University of Oregon Hospital.
Serum BEI: Serum butanol-extractable iodine was determined by the method of Man. Values in normal children range from 4.5 to 8 per 100 cc.18
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