A B S T R A C T Highly specific antisera to triiodothyronine (T3) were prepared by immunization of rabbits with T3-bovine serum albumin conjugates. Antisera with T3 binding capacity of up to 600 ng/ml were obtained. The ability of various thyronine derivatives to inhibit the binding of T3-MI to anti-T3 serum was found to vary considerably. L-Ts, D-T3 and several triiodoanalogues were potent inhibitors of the reaction. Little inhibition of T3-1"I binding was produced by L-thyroxine (T4) or other tetraiodo-analogues, thyronine or iodotyrosines. Chromatography of several T4 preparations indicated that most of their very slight activity could be ascribed to contamination with T3.Successful assay of Ts in serum was accomplished by the addition of diphenylhydantoin to the assay system. Under these circumstances, recovery of Ta added to serum was excellent, and addition of T4 was without significant effect. Serum Ts concentrations in normal subjects averaged 145 ±25 ng/100 ml (SD). Increased concentrations (429 ±146 ng/100 ml) were observed in hyperthyroid patients whereas those with hypothyroidism had serum Ts levels of 99 ±24 ng/100 ml. Elevated Ts concentrations were found also in hypothyroid patients receiving 25 gg or more of T3 daily and in those receiving 300 ,g of T4 daily. Serial measurements of T3 concentrations in subjects after oral T3 administration revealed peak Ta concentrations 2-4 hr after T3 administration. Intramuscular administration of thyrotropin (TSH) resulted in earlier and more pronounced increases in serum T3 than in serum T4 concentrations.Triiodothyronine (T3)1 was recognized to be a biologically active secretory product of the thyroid gland over a decade ago (1). Recent studies have indicated that it is formed extrathyroidally as well (2, 3). Nevertheless, relatively little information concerning the role of Ts secretion in different thyroid disorders has

Among 18 NIH probands with anosmia and hypogonadotropic hypogonadism (AHH), seven had affected relatives and three had consanguineous parents. Both sexes were equally affected and parents were phenotypically normal. Parental age was not increased. Cleft lip and palate occurred in both eugonadal and hypogonadal persons, a previously reported association that may represent variable expression of AHH. Diabetes mellitus, usually insulin-dependent, was frequent in probands and their families. Other common traits included obesity, cryptorchidism, and hearing loss. All probands were chromosomally normal. The frequency of some dermatoglyphic traits of probands differed from normal, but no trait was unique to AHH. Segregation analysis of our proband sibships was consistent with a hypothesis of autosomal-recessive inheritance with variable expression. However, genetic heterogeneity was apparent when previous reports of familial AHH were surveyed. An X-linked or male sex-limited autosomal-dominant form with unilateral renal agenesis, mental retardation, and hypotelorism has been observed. The infrequent reports of direct male-to-male transmission limit characterization of an autosomal-dominant form of AHH. Our phenotypic analysis suggests that the traits of mental retardation, renal anomalies, hypotelorism, diabetes, and hearing loss may help to distinguish various forms of AHH, whereas cryptorchidism, clefts, and obesity appear in several types of families. At present, genetic counseling is dependent upon establishing inheritance pattern after examination for the known associated anomalies.

No abstract

Two men are described who fulfill the criteria for both the Kallmann and the fertile eunuch syndrome, and we report the erythrocyte and HLA phenotypes of these men and their children. There were no paternal exclusions noted in red blood cell phenotypes encompassing seven separate red cell systems. The HLA phenotypes indicate that the probability that these men were the fathers of the children was greater than 99.99%.