Testicular function of 17 males treated in childhood or adolescence for nephrotic syndrome (NS) with cyclophosphamide (CY) for a mean time of 240 days (mean total dosage of 16.4 g or 641 mg/kg body weight) was evaluated at a mean time of 11.8 years after treatment. Five were azoospermic, 1 oligospermic, and 11 normospermic. There was a significant inverse correlation of sperm density with CY dosage and duration of treatment. All patients had undergone normal pubertal development and had normal sexual characteristics. Both basal and gonadotropin-releasing hormone-stimulated follicle-stimulating hormone (FSH) and luteinizing hormone (LH) concentrations were significantly raised in oligo- and azoospermic patients. Raised basal and peak FSH and LH concentrations in normospermic patients with a sperm count of less than 40 x 10(6)/ml were in keeping with impairment of two testicular components. However, mean basal plasma testosterone levels and mean peak plasma testosterone responses to human chorionic gonadotropin (HCG) did not differ significantly between patients and controls. Although LH responses to gonadotropin-releasing hormone suggested compensated Leydig cell failure in patients with testicular tubular damage, secretory reserve capacity of these cells, estimated by a HCG stimulation test, was preserved. Further follow-up is required to ascertain whether in these patients Leydig cell failure will develop with time.
A boy who developed haemolytic-uraemic syndrome (HUS) at 8 years 6 months of age had four further episodes of the disease during the next 3 years. No renal abnormalities were detected between the attacks nor in the 2.5 years after the last recurrence. Reduced levels of serum complement were found during four of the episodes and in two intervening periods.
The association of a spondyloepiphyseal dysplasia and disproportionate short stature with focal glomerular sclerosis is reported in two girls. Renal disease manifested by proteinuria at the age of 2.5 and 11 years, leading to treatment-resistant nephrotic syndrome over 15 and 45 months, respectively. One patient went into end-stage renal failure shortly after nephrotic syndrome developed, the other died from sepsis. The association of spondyloepiphyseal dysplasia and focal glomerular sclerosis with nephrotic syndrome may represent a distinct disease entity.
Ovarian and pituitary-gonadal function was evaluated in 12 women who were treated with cyclophosphamide for nephrotic syndrome before or during puberty. The mean age at the start of treatment was 8.7 years; the mean total dose of cyclophosphamide was 439 mg/kg body weight; and the mean follow-up time was 12.3 years. The investigations included detailed developmental, menstrual and fertility histories; general and gynaecological examinations; basal levels and follicle-stimulating hormone and luteinizing hormone responses to gonadotropin-releasing hormone, and plasma oestradiol determinations. All patients had normal pubertal development and regular menstrual patterns. Two had borne healthy children. Although hormonal studies did not show obvious ovarian or pituitary-gonadal dysfunction, further follow-up is required to ascertain whether the patients with the most prolonged treatment undergo a premature menopause.
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