Elizabeth K. Smith

Serum protein, lipid, and lipoprotein fractions in normal human pregnancy☆☆☆

Abstract Serial determinations of total serum protein, protein, and lipoprotein distribution measured by paper electrophoresis, and serum cholesterol, cholesterol esters, and lipid phosphorus have been made in a normal primigravida beginning at 5 weeks of gestation, continuing at monthly intervals during pregnancy, and post partum at 9 hours, 4 days, 7 and 15 weeks, and 6 months. Serum albumin decreased during pregnancy, whereas the alpha globulins and beta globulin increased progressively to delivery. Serum cholesterol, lipid phosphorus, and beta lipoprotein, as well as total lipid, also increased progressively to a maximum early in the puerperium. All the changes were reversed following delivery, but most values required 6 months to return completely to normal.

Adrenocorticotropic Hormone Controls Infantile Spasms Independently of Cortisol Stimulation

Summary: Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one‐quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.

Isovaleric acidemia: Identification of isovalerate, isovalerylglycine, and 3-hydroxyisovalerate in urine of a patient previously reported as having butyric and hexanoic acidemia

Urine from one of the original patients with butyric and hexanoic aidemia was examined for the possible excrection of glycine conjugates of butyryl-CoA and hexanoyl-CoA. Thin-layer chromatography of acylglycines revealed a distinct spot with an Rf values imilar to that of isovalerylglycine. Gas liquid chromatography of a distillate of urine showed a peak with the same retention time as the isovaleric acid and a second peak with a retention time longer than that of hexanoic acid. Furthermore, gas liquid chromatography of the trimethylsilyl derivative of an extract of urine showed peaks of isovalerylglycine and β-hydroxyisovaleric acid. The structure of these compounds was established by mass spectrometry. These findings indicate that the case reported actually had isovaleric acidemia.

Congenital adrenal hyperplasia (pseudohermaphroditism) before and after cortisone therapy: I. Adolescent development and menstruation

Abstract The results of continuous treatment of a 16-year-old girl with congenital adrenal hyperplasia with cortisone for a period of one year have been evaluated. Cortisone in amounts of 100 mg. orally or 50 mg. intramuscularly daily was required to suppress adequately the adrenal androgen secretion. At this dosage level considerable feminization occurred with normal breast development, cornification of the vaginal smear, regular menstruation, and a decrease in hirsutism. Although the urinary estrogen excretion and vaginal smear cornification showed cyclic changes coinciding with the menstrual periods, no positive evidence of ovulation was obtained during the period of study.

The fluorescence of Δ 4-3-keto-11-deoxysteroids: I. Optimal conditions for the measurement of 11-deoxycortisol

Abstract The presence of an oxygen function on C11 usually is regarded as necessary for the fluorescence of adrenocorticosteroids in acid-alcohol media. Conditions are described under which 11-deoxycortisol can be made to fluoresce in quantities as low as 0.05 μg, demonstrating that 11-deoxycortisol can be as fluorogenic as cortisol.