Tripling of serum progesterone (P4) and embryonic morphological features in conception and nonconception cycles, after human in vitro fertilization.

A series of 26 in vitro fertilization (IVF) cycles was studied for changes of serum progesterone levels before and after injection of human chorionic gonadotropin (hCG) by a comparison of the number of follicles punctured, number of oocytes recovered, morphological features of oocytes, fertilization rate, cleavage rate, and quality of embryos before their transfer. Subjects were further divided into three groups according to their pregnancy outcome. Five pregnancies were generated and reached the stage of clinical confirmation of live baby delivery or live fetus by ultrasound scanning of the uterine contents. Six patient cycles that had a prolonged luteal phase and beta-hCG levels greater than 40 MIU/ml were described as biochemical pregnancies. Fifteen patient cycles showed no sign of pregnancy. The significant finding of this study is related to the serum progesterone level increase from 20 to 34 hr after hCG injection. The nonpregnancy group yielded a 1.2-fold increase compared with the biochemical pregnancy and pregnancy groups, which had a 3-fold increase. The usage of this multiple effect of progesterone may aid in the prediction of the "high-quality embryo" that might result from a given treatment cycle, indicate the completion of luteinization, the proper timing of hCG to induce final oocyte maturation, and the necessary length of preincubation, and aid in the prediction as to whether or not a pregnancy is likely to result.

The first reported case of Down syndrome in the Southern California Fertility Institute's in vitro fertilization and embryo transfer program.

The first case of a genetic abnormality in a 3-year in vitro fertilization and embryo transfer program was diagnosed after routine amniocentesis of a 38-year-old patient. The G-banded karyotype analysis gave 47, XY, +21, which constitutes an abnormal chromosome pattern consistent with Down syndrome.

Studies of phenylketonurics with dermatitis.

A comprehensive study was undertaken of two patients who had typical phenylketonuria (PKU) and chronic dermatitis. Hematologic, immunologic, and histologic (light and electron microscopy) studies and quantitative intracellular amino acid measurements were made from the skin and cultured fibroblasts of these patients. Findings were: (1) the complement receptor-bearing lymphocytes (EAC rosettes) were decreased; (2) both light and electron microscopic findings revealed nonspecific dermatitis; (3) fibroblastic studies suggested little or no phenylalanine hydroxylase activity in cells of patients with PKU and controls; (4) the intracellular concentration of phenylalanine was significantly higher in the skin of PKU patients than in the controls; and (5) patch tests using 50% phenylalanine in petrolatum were negative in four untreated adult PKU patients and four controls.

Norwegian scabies in Bloom's syndrome.

Norwegian scabies is an unusual Sarcoptes scabiei infestation. It has been reported often in patients who are either severely debilitated or who have congenital or iatrogenic suppression of their immune responses. We report the occurence of Norwegian scabies in a 13-year-old boy with Bloom's syndrome who had impaired humoral and cell-mediated immunity.

The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis.

Herein we report a new familial form of hepatic disease. Each of the four patients had splenomegaly, hypersplenism, a small liver, biochemical evidence of hepatic excretory dysfunction and hepatocellular damage, kidneys without demonstrable cysts, and normal blood pressue. An evaluation of serum immunoproteins, autoantibodies, histocompatibility antigens, and mixed lymphocyte reactivity further defined the immunologic features of this syndrome. Extrahepatic manifestations included a papulosquamous dermatitis with deposition of immunoglobulins and complement in both normal and abnormal skin, a membranoproliferative glomerulonephritis with subendothelial deposits, arthritis, and pericardial, pleural, and synovial effusions.

Corpus luteum function during pregnancies of previously infertile women.

Seventy-nine previously infertile women were studied during the first 9 weeks of their pregnancies. Pregnancy had occurred either without any specific therapy or following treatment with clomiphene, prednisone, or human chorionic gonadotropin, singly or in various combinations. Serum levels of progesterone, 17 alpha-hydroxyprogesterone, and estradiol were measured at 2-week intervals. A profile of early pregnancy in terms of these hormones is presented and discussed with regard to its significance for corpus luteum function. Aspects of the hormonal profile predictive of the pregnancy outcome are noted. Ovarian over-stimulation was directly related to the dosage of clomiphene, and the increased levels of 17 alpha-hydroxyprogesterone thus produced persisted into the seventh week of pregnancy. This hormonal excess probably derives from multiple corpora lutea which may also account for the increased incidence of multiple pregnancy associated with clomiphene therapy.

Hypoplastic enamel, onycholysis, and hypohidrosis inherited as an autosomal dominant trait. A review of ectodermal dysplasia syndromes.

A syndrome consisting of a hypocalcified-hypoplastic enamel, onycholysis with subungual hyperkeratosis, seborrheic dermatitis of the scalp, and hypofunction of the sweat glands with rough dry skin is inherited as an autosomal dominant trait in a kindred of Caucasian ancestry.

Cowden disease.

Cowden disease represents an unusual, but unique syndrome which can be recognized most consistently by the development of characteristic verrucous, keratotic, papular, and nodular lesions about facial orifices, on the oral mucosa, and over the dorsal surfaces of the forearms and hands. The cause of these diverse hyperplastic changes is not known. Recognition of these lesions as signs of more extensive disease should alert the physician to examine the thyroid gland, breasts, female reproductive tract, GI tract, and skeleton for evidence of the associated changes we have enumerated. Because of the tendency for lesions of the thyroid, breast, and intestines to undergo malignant change, these patients require close observation and evaluation.