Faculty Bibliography

This study was designed to assess the impact of chronic illness on the sexuality and life-style of a group of veterans with end stage renal disease. Thirty-two married home dialysis patients and 14 of their wives were interviewed by a sex therapist. An Index of Sexual Function (ISF) was developed to quantify 5 aspects of male genital function. The Index was correlated with the following: other major areas of sexuality including masculine self-concept, sex drive, sexual activity, sexual relationship, and sexual satisfaction; quality of life factors such as work, marital adjustment, and sociability; several demographic factors; and 15 medical variables. There were significant correlations between genital function and sexual satisfaction, the patient's attitude toward the importance of sex, sex drive, and positive feelings about the self. Better sexual function occurred in the patients with the least neuropathy; there was no correlation with other medical factors. Of the patients studies, 9 (28%) had full sexual function, 7 (22%) had moderate dysfunction, and 16 (50%) had severe dysfunction. Despite the genital dysfunction, marital and social adjustment of our patients compared favorably with studies of couples in the general population. After this study a sex counseling program was developed.

Eight cases of acquired cystic disease of the kidney (ACDK) associated with chronic renal failure and hemodialysis are described. No patient had a family history or clinical evidence of congenital adult polycystic kidney disease (CAPKD). Glomerulonephritis was the cause of renal failure in 6, and pyelonephritis in 2. Massive renal and perirenal hemorrhage necessitated 3 nephrectomies in 2 patients. Single kidney weights did not exceed 280 Gm., a major feature in the distinction of ACDK from CAPKD. Morphologically, in addition to the usual stigmata of end-stage kidneys, 40 to 80 per cent of the renal parenchyma was replaced by small cysts. Continuity of cysts with tubules was established by nephron dissection

PCP vaccine was administered to 21 adult hemodialysis patients and 25 control patients with no renal nor hepatic dysfunction. Antibody concentrations measured by the RIA technique were lower in the hemodialysis than in the control patients in both prevaccination sera and specimens obtained 3 to 4 weeks following vaccination. However, 6 months after vaccination, RIA GMACs in hemodialysis and control subjects were more nearly alike. OTs against Streptococcus pneumoniae, types 1 and 3, were measured in paired sera from vaccine recipients. Opsonic activity against S. pneumoniae, type I, was detected as frequently in the 3 to 4 week postvaccine sera of the dialysis as in those of control patients. However, there was a poor correlation between OTs and RIA-ACs against this organism. There was good correlation between OT and RIA-AC against type 3 pneumococci in 3 to 4 week postvaccine sera, and significantly more control patients had opsonic activity detectable in these specimens. No clinical S. pneumoniae infections have been observed in vaccine recipients. However, continued follow-up will be necessary to document the degree to which hemodialysis patients are protected by pneumococcal vaccines and the relationship between protection and antibody concentration or OTs

A nodular glomerulopathy characterized by mesangial deposits of monoclonal kappa light chains was detected by immunofluorescence in a renal biopsy from a patient with proteinuria and hypertension. These nodules lacked the tinctorial and morphologic features of amyloid. Ultrastructurally, the nodules contained electron-dense granular deposits as well as fibrils in parallel arrangement. The fibrils measured 110-140 A in diameter. They were consistent in size with amyloid fibrils. However, they differed in lacking the randomly oriented network of typical amyloid fibrils and more closely resembled fibrils intrinsic to mesangial matrix. The patient had no bone marrow or X-ray evidence of myeloma and no evidence of free monoclonal light chains in serum or concentrated urines. Biosynthetic studies of the patient's bone marrow cells demonstrated unbalanced immunoglobulin synthesis with excess production of monoclonal kappa light chains. These observations suggest that the observed glomerulopathy results from direct deposition of monoclonal light chains. Deposits with kappa light chain determinants have been found in 7 other patients with similar nodular glomerulopathies, 4 of whom had diagnosed clinical myeloma. The lesion of nonamyloidotic nodular glomerulopathy previously described in 19 patients, nor examined by immunopathologic techniques or not shown to contain light chain determinants, may have a similar pathogenesis