Lyn M. Boylan

Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections.

We studied 29 men with Youngs syndrome, a combination of obstructive azoospermia and chronic sinopulmonary infections. Men with this syndrome have only mildly impaired respiratory function and normal spermatogenesis; the azoospermia is due to obstruction of the epididymis by inspissated secretions. The diagnosis is based on the occurrence of chronic sinopulmonary infections, persistent azoospermia, normal spermatogenesis, and characteristic epididymal findings, as well as exclusion of cystic fibrosis and the immotile-cilia syndrome. The sperm themselves appear to be normal in Youngs syndrome. Pregnancies had occurred in five couples; in three paternity was documented by genotyping. Thus, improved microsurgical and medical therapy might restore fertility. We suggest that Youngs syndrome has a prevalence comparable to that of Klinefelters syndrome and is a common cause of both chronic sinopulmonary infection and azoospermia.

Testicular function and glycemic control in diabetic men. A controlled study

Summary:  We have investigated testicular function in 28 insulin‐dependent diabetic men under the age of 50 years and 119 age‐matched controls. Diabetics had reduced testicular volume, semen volume, total and total motile sperm output while plasma LH and FSH levels were elevated. Reduction in semen volume and impotence were more common in long‐standing complicated diabetes. Glycosylated hemoglobin (GHb) levels were positively correlated with plasma LH levels (r = 0.46, p < 0.02) but there was no direct correlation of glycemic control and spermatogenesis. The differences in testicular function were due to decreased spermatogenesis and could not be explained by other forms of testicular pathology or the presence of diabetic neurovascular complications. We conclude that the function of the hypothalamic pituitary testicular axis is impaired in diabetic men, that this impairment is at least partly related to the degree of preceeding glycemic control and that multiple levels of the axis may be dysfunctional.

Psychological and attitudinal profiles in donors for artificial insemination

Few objective data are available concerning the psychological profiles and attitudes of sperm donors in artificial insemination programs. We studied 30 consecutive new volunteers and 45 established sperm donors using the Cattell 16PF personality profile and an attitudinal survey concerning motives for donation, attitudes toward uses of sperm, desire for knowledge of outcomes, and attitudes toward disclosures of identifying and nonidentifying information between the parties to artificial insemination. Donors differed from the population norms in 7 of 16 first-order and 1 of 4 second-order personality factors, and this difference appeared to reflect their self-selection as donors. The predominant motive for donation was altruism, but secondary motives were also common. Financial motivation was very low in this population with few students. Donors approved of all current and most hypothetical uses of sperm but were not in favor of disclosures of information to other participants in the program. Restriction of disclosures to nonidentifying details was more acceptable. Unmarried donors were more often motivated for nonaltruistic reasons and were more in favor of disclosures. Attitudes of sperm donors were mostly stable over at least 3 years, but an increasing minority with time envisaged circumstances that might lead to their withdrawal from the program.

Testicular Function and Fertility in Men with Homozygous Alpha-1 Antitrypsin Deficiency

Summary:  Fertility and testicular function were studied in eight men with severe homozygous (Pi ZZ variant genotype) alpha‐1 antitrypsin (AAT) deficiency. Age‐ and marital duration standardized fertility, clinical androgenic features, mean testicular volume, plasma luteinizing hormone (LH) and follicle‐stimulating hormone (FSH) and semen analysis were all normal apart from a reduction in semen volume. Mean plasma total and free testosterone were elevated and the percentage free testosterone reduced compared with age‐matched, healthy fertile controls indicative of increased sex‐hormone binding globulin (SHBG) levels representing an early marker for subclinical hepatic dysfunction associated with AAT‐deficiency. In view of the preservation of normal fertility and testicular function despite chronic respiratory disease and premature death with deleterious AAT gene variants, it is proposed that the high prevalence of genetic polymorphism in the AAT protein may be maintained by the chronological assynchrony of the periods of maximal male reproductive activity (40 years) and the late onset (> 40 years) of symptoms in severe AAT deficiency rather than by any balance between reduced reproductive fitness of homozygotes and heterozygote advantage.

Pulsatile intravenous gonadotropin-releasing hormone for ovulation-induction in infertile women. I. Safety and effectiveness with outpatient therapy**Presented in part at the Seventh International Congress of Endocrinology, Abstract 897, Quebec City, Quebec, Canada, July 1 to 7, 1984.

Pulsatile intravenous gonadotropin-releasing hormone (IV-GnRH) was used in 36 infertile patients with primary amenorrhea (n = 5), secondary amenorrhea due to hypothalamic chronic anovulation (HCA) (n = 22), hyperprolactinemia (n = 1) or polycystic ovary syndrome (PCOS) (n = 5), and oligomenorrhea (n = 3). Treatment was commonly initiated in the hospital but was then continued outside, with patients and local physicians accepting responsibility for maintaining IV-GnRH delivery systems. Twenty-eight of 113 treatment cycles (24.8%) resulted in pregnancy, with four spontaneous abortions (14.3%) and four twin pregnancies (16.7%) among 24 births. Probability of pregnancy per treatment cycle was significantly higher for primary amenorrhea (0.30) and for HCA (0.33) than for PCOS (0.07; P less than 0.05) and for oligomenorrhea (no conceptions; P = 0.01). Ovulatory cycles were not achieved in five patients (primary amenorrhea, n = 1; PCOS, n = 3; oligomenorrhea, n = 1). There were no serious complications; six patients recorded eight febrile episodes, which responded quickly to antibiotic therapy and cannula change. The authors conclude that outpatient IV-GnRH is safe, practical, and effective for follicular stimulation and ovulation induction in women presumed to have GnRH deficiency and in whom clomiphene therapy fails, and that less intensive monitoring is needed compared with gonadotropin ovulation induction therapy.

Pulsatile intravenous gonadotropin-releasing hormone for ovulation-induction in infertile women. II. Analysis of follicular and luteal phase responses**Presented in part at the Seventh International Congress of Endocrinology, Abstract 897, Quebec City, Quebec, Canada, July 1 to 7, 1984.

Pulsatile intravenous gonadotropin releasing hormone (IV-GnRH) was used in 36 infertile patients with primary amenorrhea (n = 5), secondary amenorrhea due to hypothalamic chronic anovulation (HCA) (n = 22), hyperprolactinemia (n = 1) or polycystic ovary syndrome (PCOS) (n = 5), and oligomenorrhea (n = 3), using several dosage and timing regimens. Early follicular phase responses showed four patterns: type 1 consisted of a delayed follicle-stimulating hormone (FSH) peak and was seen with severe hypothalamic suppression (n = 4); type 2 consisted of a brisk and dominant FSH peak on the first day of treatment, and occurred with mild to moderate hypothalamic suppression (n = 19); type 3, which consisted of an FSH peak accompanied by an immediate and exaggerated luteinizing hormone (LH) rise, occurred with mild PCOS and some cases of HCA (n = 5); and type 4, in which LH levels were high to begin with and neither FSH nor LH levels rose with GnRH, occurred with severe PCOS (n = 2). Exaggerated estradiol responses within 24 hours of therapy were seen in eight cycles: in four cases no ovarian abnormality was apparent; in three cases a dominant follicle was already present; and in one case ovarian hyperstimulation was diagnosed ultrasonographically. With standard human chorionic gonadotropin luteal phase support, luteal phase defects were rare with HCA but common with PCOS.