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Incidence of IgA-related nephritides in American Indians in New Mexico.

The racial distribution of the findings in 664 renal biopsies was studied for the state of New Mexico. The incidence of IgA-related nephritides (Bergers disease and Henoch-Schönlein purpura) was significantly greater in American Indians than in Hispanics and Anglos; IgA-related nephritides were found in 38 per cent of renal biopsies in American Indians. The clinicopathologic presentations of IgA-related nephritides were similar in the three ethnic groups.

Experimental allergic orchitis in mice

Inbred strains of mice were studied for their susceptibility to the induction of experimental allergic orchitis after sensitization with mouse testicular homogenate in complete Freunds adjuvant accompanied by injections of extract from Bordetella pertussis. Susceptibility to autoimmune orchitis was found to be linked to the major histocompatibility complex in BALB/c and C57BL/10 mice and mapped to genes encoded within the H-2Ddregion. In five of six groups of bidirectional (susceptible × resistant) F1 hybrids, H-2Dd-linked susceptibility was inherited as a dominant autosomal trait. However, in (BALB/cByJ × DBA/2J)F1 and (DBA/2J × BALB/cByJ)F1 hybrids, dominant autosomal resistance to the induction of autoimmune orchitis was observed. Backcross analysis between the resistant F1 hybrid and the susceptible BALB/cByJ parent suggests that a single independently segregating DBA/2J locus is capable of negating H-2Dd-linked susceptibility, and controls resistance to the induction of autoimmune orchitis.

Mesangial Proliferative Glomerulonephritis in Southwestern American Indians

A series of 166 American Indian renal biopsy specimens from 1971 to 1989 showed a very high proportion with mesangial proliferative glomerulonephritis with mesangial immunoglobulin deposition (Ig-pos mesGN). This disease comprised 68.7% of all the biopsies and 83.8% of all primary GN, proportions much greater than those (23.5% and 37.7%, respectively) of a local contemporaneous biopsy series from non-Indians ( P

Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation.

POEMS (polyneuropathy, organomegaly [hepatosplenomegaly or lymphadenopathy], endocrinopathy, M protein, and skin changes) syndrome is an uncommon plasma cell dyscrasia with diverse manifestations, including lymphadenopathy as well as those that comprise the acronym. Dermatologic changes may include hyperpigmentation, thickened skin, hypertrichosis, and papular angiomas. These changes are believed to result from humoral products of the plasma cell clone and frequently resolve with treatment of the neoplasm. We describe a man with typical POEMS syndrome with a unique skin lesion: a large, sharply circumscribed, pigmented plaque on the anterior chest wall centered over a plasmacytoma of the sternum. Results of histologic examination showed a proliferation of capillaries of varying size, enlarged fibroblasts, and increased amounts of collagen and proteoglycan from the dermis to the periosteum. Because the outer cortex of the sternum was eroded, peptide mediators of vascular and fibroblast proliferation may have diffused directly from the plasma cell lesion into the tissues of the chest wall.

Experimental allergic orchitis in mice: III. Differential susceptibility and resistance among BALB/c sublines

Significant differences in susceptibility to the induction of experimental allergic orchitis (EAO) were found to exist among the various substrains of BALB/c mice. Substrains which were susceptible to disease induction include BALB/cKa, BALB/cByJ, BALB/cWtJ, BALB/cCmcCr, BALB/cCrgl, BALB/cHeA, BALB/cAnNCr, BALB/cPt, BALB/cWehiUnm, BALB/cOrnl and BALB/cArg whereas BALB/cJ and BALB/cSki BoyPt were resistant to EAO. Aspermatogenesis and autoimmune vasitis, two additional lesions characteristic of murine EAO, were found to correlate with susceptibility to autoimmune orchitis. Susceptibility and/or disease resistance did not segregate within any of the evolutionary branches of the BALB/c genealogic tree nor did it correlate with any of the previously described allelic differences distinguishing BALB/c substrains. Therefore, differential disease susceptibility may represent the manifestation of mutational and/or retroviral induced genotypic differences in the regulatory genes controlling testicular autoimmunity.

Nephrotic syndrome and rapid renal failure in autosomal dominant polycystic Kidney disease

A 44-year-old man, with autosomal dominant polycystic kidney disease and hypertension under satisfactory control, developed nephrotic syndrome with negative serology. Open renal biopsy revealed focal glomerular sclerosis. Prior to the appearance of heavy proteinuria, serum creatinine was 1.7 mg/dl. After the nephrotic syndrome had been established, renal function deteriorated rapidly and hemodialysis was started within 2.6 years. In patients with autosomal dominant polycystic kidney disease, the appearance of nephrotic range proteinuria along with a rapid decline in renal function indicates the presence of a glomerular lesion, which needs to be investigated by renal biopsy.

Lupus nephritis in a pediatric renal transplant recipient

A case of aggressive lupus nephritis in a pediatric renal transplant patient is described. She initially presented with end-stage glomerulonephritis for which an underlying etiology could not be determined. Ten months after cadaveric renal transplantation, systemic lupus erythematosus was diagnosed, when she developed diffuse proliferative glomerulonephritis in association with antinuclear antibody, anti-double-stranded DNA antibody and extrarenal manifestations of lupus. It is plausible that she developed recurrent rather thande novo lupus nephritis following transplantation. Reactivation of lupus nephritis in a renal transplant is unusual in adults, and is previously unreported in children.

IgA Nephropathy in Renal Allografts: Increased Frequency in Native American Patients

We investigated the frequency of IgA nephropathy in transplanted kidneys in 2 ethnic groups in New Mexico (USA). A total of 80 renal graft biopsies were obtained from 66 patients when clinically indicated for the differential diagnosis of graft dysfunction. Glomerulonephritis was present in 16 patients, in biopsies obtained after the first posttransplantation month. The frequency of IgAN in allografts was not the same in Native Americans and in Caucasians: Nondonor IgAN was observed in 4/18 biopsies from Native American patients (22.2%) but only in 4/48 biopsies from Caucasians (8.3%) (p < 0.01). This study demonstrates that in New Mexico the frequency of IgAN in transplanted kidneys in Native American patients is 2.7 times higher than in Caucasian graft recipients.

Alcohol abuse-related mesangial glomerulonephritis: immunoelectron microscopy.

Immunoelectron microscopy, using post-embedding immunohistochemistry with colloidal gold, was performed on renal samples from forensic autopsies. We confirmed that electron-dense deposits seen in alcohol abuse-related mesangial nephritis correspond to immunoglobulins, as has been shown previously by others in idiopathic cases. We investigated seven control samples and 13 specimens from individuals with evidence of alcohol abuse, six of whom had mesangial nephritis with IgA deposition. We found concentration of the gold particles over large electron-dense deposits in four of six cases of mesangial nephritis, confirming that they correspond to the IgA shown by immunofluorescence. Furthermore, a similar concentration of gold particles was not observed in control cases or in alcoholics without mesangial glomerulonephritis (4/6 vs 0/14; p = 0.005). IgM, seen as small aggregates, was confirmed in only two of six of the same cases. This is the first time that immunoelectron microscopy is performed on tissues obtained post mortem.

Dermal vascular IgA deposits in IgA nephropathy secondary to alcohol abuse

Skin, kidney and liver samples were investigated from 103 people with evidence of alcohol abuse at forensic autopsy. The diagnosis of alcohol abuse was based on clinical history, post mortem blood alcohol level and liver pathology. The findings confirmed the frequent widespread vascular IgA deposition in individuals with evidence of alcohol abuse. The frequency of IgA deposition in superficial small vessels of the dermis was not significantly different between individuals with IgA nephritis (16/85) and without such pathology (7/38). Based on these results, it can be inferred that had a pre‐mortem skin biopsy been performed, it would not have had clinical usefulness in predicting the renal disease.