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Introduced predatory fishes have had consistently severe consequences for native fishes in stream environments around the world, although the drivers of these effects are often unclear. In the Swartkops River headwaters in South Africa, native Eastern Cape redfin Pseudobarbus afer were always absent from sites occupied by non-native black basses Micropterus salmoides and Micropterus dolomieu, but generally co-occurred with the native predators Anguilla marmorata and Anguilla mossambica. A natural experiment provided by flood-mediated recolonization of black-bass occupied sites by P. afer demonstrated depletion in black-bass invaded sites. Field behavioural observations of P. afer indicated that they foraged among benthic cover during the day, but suspended in open water at night. As the nocturnal A. marmorata and A. mossambica foraged actively within structural cover at night and M. dolomieu and M. salmoides are diurnal or crepuscular predators, P .afer is thus optimized to avoid predation by native anguillid predators and not the functionally unique predatory black basses. The integration of distributional, temporal population dynamics and behavioural data suggests that the severe effects of Micropterus spp. are probably a consequence of prey naïveté and behaviour evolved to evade native predators., (© 2018 The Fisheries Society of the British Isles.)

Background: Cadaveric kidneys experiencing longer cold ischemia time (CIT) are associated with higher levels of delayed graft function, acute rejection, and early graft loss. One mechanism to explain these results is that ischemia/reperfusion (I/R) injury makes the allograft more immunogenic by upregulating molecules involved in the immune response (e.g., HLA Class I/II)., Methods: We evaluated the influence of CIT on the production of HLA Class I antibody level, measured by an antihuman globulin panel reactive antibody (AHG PRA) level, in 90 unsensitized recipients of primary cadaveric renal transplants (from a total of 1442 between 1985 and 1997) who rejected their kidneys., Results: By multivariate analysis, a CIT of 15 hr or more (vs. < 15 hr) independently increased the risk of the AHG Class I PRA level being > or = 20% after unsensitized patients rejected their first kidneys (relative risk=3.57; 95% confidence interval=1.26 to 10.14; P=0.01), despite the same degree of Class I/II mismatch between the two CIT groups. The overall mean peak PRA level after primary kidney rejection was significantly lower for the CIT < 15 hr group (25.9%+/-33.9; n=24) compared with the CIT > or = 15 hr group (46.3%+/-36.5; n=66) (P<0.001)., Conclusion: Longer CIT induces a humorally more immunogenic kidney.

A positive crossmatch that is rendered negative by treating the serum with the IgM-reducing agent dithiothreitol (DTT) is generally reported not to influence short-term renal graft outcome. Its effect on long-term (> or = 3 years) cadaveric and live-donor transplant function, however, is less clear. We evaluated the effect of IgM antibodies in a DTT-ameliorated positive crossmatch (DTT-APXM) on long-term renal graft outcome in 1,290 consecutive cadaveric renal transplants (8-year survival) and 384 live-donor renal transplants (7-year survival) from patients transplanted between 1990 and 1999. The data show that 1- and 8-year graft survival for cadaveric renal transplants in patients with IgM antibodies (n=72) (DWFG censored = 91% and 65%; DWFG not censored = 90% and 60%) was not significantly different from the group without IgM antibodies (n = 1,218) (DWFG censored = 92% and 71%; DWFG not censored = 87% and 55%) (log-rank = 0.25 for DWFG censored, log-rank = 0.92 for DWFG not censored). The one- and seven-year graft survival for live-donor renal transplants in patients with IgM antibodies seen in a DTT-APXM (n = 22) (DWFG censored = 95% and 83%; DWFG not censored = 95% and 66%) was not significantly different from the group without IgM antibodies (n = 362) (DWFG censored = 94% and 81%; DWFG not censored = 92% and 73%) (log-rank = 0.61 for DWFG censored, log-rank = 0.89 for DWFG not censored). DR phenotype was found to be associated with the strong (>40% cell death) IgM reactivity in both black and white patients. In white patients, DR2 was more frequently seen with a strong IgM crossmatch (48.2%) than in molecularly typed controls (28.5%) (P < 0.03) and concomitant with that DR increase, DR4 was decreased in white patients (6.8%) compared with controls (25.5%) (P < 0.02). In black patients with strong IgM reactivity, DR6 was increased in patients (46.1%) compared with controls (20.5%) (P = 0.07) and concomitant with that DR6 increase, DR5 was decreased in frequency in black patients (7.6%) compared with controls (41%) (P < 0.03). These data show that long-term graft survival in renal transplantation is not negatively influenced by the presence of donor-reactive lymphocytotoxic antibodies in the crossmatch ameliorated by serum DTT treatment. They also suggest that the strength of the IgM antibody response is regulated in part by certain gene (s) of the DR region.

Background: Single wedge biopsy of cadaveric kidneys from donors older than 55 is currently the standard method of evaluating their viability for transplantation. The degree of glomerulosclerosis presently determines whether a kidney can be transplanted, but most biopsies sample only the subcapsular region and may not accurately represent the true renal architecture. Our study evaluated the accuracy of transplant suitability determinations based upon the single wedge biopsy of cadaveric kidneys., Methods: We took kidneys that were refused by UNOS centers on the basis of biopsy results, examined their histology in detail, and reviewed donor medical histories. Sections were taken from the upper, lower, and mid-portion of each kidney and stained with the periodic acid Schiff stain. Percentage and location of glomerulosclerosis and other relevant pathology were then determined in each section. We compared our findings with the results of the original wedge biopsies obtained at the time of procurement., Results: Nine kidneys were obtained and examined. The wedge biopsies at the time of procurement showed glomerulosclerosis ranging from 8 to 36% (median 17%). The multiple kidney sections we analyzed showed fewer sclerosed glomeruli, ranging from 3 to 15% (median 7%, P<0.001), with most of the sclerosed glomeruli identified located in the immediate subcapsular region (P<0.001)., Conclusions: Wedge biopsies of donor kidneys can overestimate the total amount of glomerulosclerosis, apparently because of a predominance of sclerosis in the kidney's subcapsular region, the area predominantly sampled by the usual wedge biopsy. These inappropriately high estimates of glomerulosclerosis can result in refusal of kidneys that might be suitable for transplantation.

The purpose of our investigation was to evaluate long-term graft survival and the role of histocompatibility in patients who were highly sensitized to human leukocyte antigen (HLA) Class I antigens and received a cadaveric renal transplant. Our multi-institutional study evaluated 7-yr graft outcomes and the histocompatibility requirements of 61 (6.1%) highly sensitized (anti-human globulin panel reactive antibody [AHG PRA], > or = 80%) cadaveric renal transplantation patients, transplanted between 1988 and 1997, among 999 consecutive cadaveric renal transplants. One- and 7-yr graft survival in the high PRA group (n = 61) was 76 and 59%, and was not significantly different from that in the low PRA group (n = 938), 86 and 59% (Wilcoxon = 0.11; log-rank = 0.45) (died with a functioning graft [DWFG] not censored). When those data were divided into primary and regrafts, 1- and 7-yr graft outcomes for high and low PRA groups were not significantly different [(primary, 1- and 7-yr survival: high PRA = 83 and 74%, n = 30, and low PRA = 87 and 61%, n = 825; log-rank = 0.37 for DWFG not censored) (regrafts, 1- and 7-yr survival: high PRA = 70 and 42%, n = 31, and low PRA = 80 and 43%, n = 113; log-rank = 0.36 for DWFG not censored)]. We did observe a subgroup of the high PRA patient group that had inferior graft outcomes. Graft outcome at 1 and 6 yr in the high PRA group for patients who had one to two DR mismatches (65 and 50%, n = 41) was significantly worse than for high PRA patients who had zero DR mismatches with their donors (100 and 78%, n = 20) (log-rank = 0.01 for DWFG not censored). Furthermore, the mean number of HLA-A and -B mismatches was significantly greater in the high PRA/DR-mismatched group (1.7 +/- 1.2, n = 41) compared with the high PRA/zero DR-mismatched group (0.5 +/- 1.1, n = 19) (p < 0.001). Overall, these data suggest that the patient who is highly sensitized to HLA Class I antigens has a long-term graft outcome that is equivalent to less sensitized patients, but that HLA-DR mismatching and a higher degree of Class I mismatching may be poor prognostic indicators in such patients.

Background: We have increased the transplantation rate for blood group B cadaveric waiting list candidates by transplanting them with A2 and A2B kidneys., Methods: Since 1991, five of the seven renal transplant programs in our organ procurement organization service area have preferentially transplanted blood group A2 and A2B cadaveric kidneys to B blood group waiting list candidates with histories of low anti-A isoagglutinin titers., Results: Between 1991 and 1997, these five centers performed transplantations on 71 patients from the B cadaveric waiting list. Of those 71 patients, 29% (21 of 71) underwent transplantation with either A2 (n=18) or A2B (n=3) cadaveric kidneys. In 1997 alone, 48% (11 of 23) of the B patient transplant recipients received A2 or A2B kidneys., Conclusions: Transplantation of A2 and A2B kidneys into B waiting list patients has successfully increased access of B patients to kidneys. Such an allocation algorithm implemented nationally may similarly increase the transplantation rate of B waiting list candidates.

Background: Cadaveric renal retransplantation is associated with a higher risk of early graft failure than primary grafts. A large proportion of those graft losses is likely attributable to donor-directed HLA class I antibodies, detectable by flow cytometry cross-matching but not by conventional crossmatching techniques., Methods: Long-term graft survival in a group of 106 recipients of consecutive cadaveric renal regrafts between 1990 and 1997, in whom a negative flow T-cell IgG crossmatch was required for transplantation, was compared with two other groups of cadaveric transplant recipients. The first group consisted of 174 cadaveric regrafts transplanted between 1985 and 1995 using only a negative anti-human globulin (AHG) T-cell IgG crossmatch. The second group was primary cadaveric transplants done concurrently with the flow group (1990 to 1997) using only the AHG T-cell IgG crossmatch., Results: The long-term (7 year) graft survival rate of flow crossmatch-selected regraft recipients (68%; n= 106) was significantly improved over that of regraft recipients who were selected for transplantation by only the AHG crossmatch technique (45%; n=174; log-rank=0.001; censored for patients dying with a functioning graft). Graft outcome for the flow cross-matched regraft recipients was not significantly different from that of primary cadaveric patients (72%; n=889; log-rank=0.2; censored for patients dying with a functioning graft). Finally, a positive B-cell IgG flow cytometric crossmatch had no influence on long-term regraft outcome., Conclusions: The use of the flow T-cell IgG cross-match as the exclusion criterion for cadaveric renal retransplantation yields an improved long-term graft outcome over that obtained when only the AHG cross-match is used and has improved survival of regraft recipients to the level of our primary cadaveric renal transplant population.

Background: The Rh (D) blood group system has not traditionally been considered to be a clinically relevant histocompatibility barrier in transplantation since conflicting results of its clinical importance have been reported., Methods: We analyzed 786 consecutive primary cadaveric renal transplants performed by transplant centers in our Organ Procurement Organization (OPO) between 1990 and 1997. We also analyzed United Network for Organ Sharing (UNOS) data on 26,469 kidney transplants done from April 1994 to June 1996., Results: Multivariate analysis revealed that Rh identity between the recipient and donor was significantly related to better graft outcome (risk ratio, 0.43; 95% confidence interval, 0.30 to 0.61; P=0.0001). Multivariate analysis of the UNOS data revealed that the Rh -/- group may have a positive influence on graft survival with a risk ratio of 0.43 (P=0.14)., Conclusion: Multivariate analysis of primary cadaveric renal allografts performed within the Midwest Organ Bank OPO indicates that Rh (D) is a clinically relevant histocompatibility barrier that influences 7-year graft survival.

Background: This article summarizes our 10-year multicenter experience with transplantation of 50 blood group A2 and A2B kidneys into B and O patients., Methods: Since 1986, we have transplanted kidneys from 46 cadaver donors and 4 living donors who were blood group A2 (47 donors) or A2B (3 donors) into 19 B and 31 O patients. In 1991, we began allocating these kidneys preferentially to B and O recipients who were selected based on a history of low (< or =4) anti-A IgG isoagglutinin titers. Immunosuppression was no different from that used in ABO-compatible grafts., Results: The 1-month function rate before thus selecting the patients was 68% (19/28), but is now 94% (17/18). Two-year cadaver-donor graft survival with this selection method is 94%, compared with 88% for 640 concurrent and consecutive ABO-compatible transplants (log-rank, 0.15). All four living-related transplants are still functioning, with a mean follow-up of 71 months. Since we began allocating A2 kidneys preferentially to B and O recipients, the percentage of the B patients who received A2 or A2B kidneys has increased from 29% (8/28) to 55% (10/18)., Conclusions: Transplantation of A2 or A2B kidneys into B and O patients is clinically equivalent to that of ABO-compatible transplantation when recipients are selected by low pretransplant anti-A titer histories. This approach increases access of blood group B recipients to kidneys.

We describe a case of de novo membranous glomerulopathy in the renal allograft of a diabetic patient, treated with the newer immunosuppressive agent FK 506. Twenty-two months later this patient developed nephrotic range proteinuria.

Because of the inherent difficulties in allele assignment with HLA-DR serological typing, in 1993 our organ procurement organization-based HLA laboratory replaced serology with the molecular method of polymerase chain reaction using sequence-specific primer mixes (PCR-SSP) to type for DR and DQ at a resolution level equivalent to that of serologically defined antigens. In this study, we compared the incidence of DR blanks, where allocative homozygosity occurred, and graft outcome during our serology epoch (1987-1993) with that of our molecular epoch (1993-1996). The incidence of DR blanks by PCR-SSP (17.0%; 138/1101) was significantly lower (P<0.005) than in the serology epoch (21.5%; 569/2647). Although DQ is not a component of the allocation algorithm, the incidence of blanks in the molecular era (21.9%; 196/895) was 46% lower (P<0.001) than in the serology epoch (40.8%; 931/2277). Graft survival in 163 cadaveric renal transplant recipients for whom molecular DR allocation occurred (patient and donor were molecularly typed) showed that PCR-SSP typing had no significant effect on 2.5-year graft survival for patients mismatched for 0 (97%), 1 (90%), or 2 (94%) HLA-DR antigens (P=0.4; log-rank). In conclusion, molecular typing lowered the rate of DR and DQ blanks, but molecular matching for HLA DR and DQ did not influence graft outcome at 2.5 years.

Objective: To evaluate the role of flow cytometry cross-matching on graft survival in patients undergoing cadaveric renal retransplantation compared with our conventional antihuman globulin cytotoxic crossmatch., Design: In 1990, 6 of 7 transplantation centers in 1 organ procurement organization service area began performing cadaveric renal retransplantation only if the flow T-cell IgG crossmatch was negative. During that period, 1 center continued to use only the antihuman globulin T-cell IgG crossmatch. Prior to 1990, all centers used only the antihuman globulin T-cell IgG crossmatch as their crossmatch selection criterion for retransplantation. Regraft survival was compared between those centers by crossmatch selection criteria., Patients: Patient selection and immunosuppression decisions were made at the transplantation center., Setting: All flow cytometry crossmatches for all 7 centers participating in the evaluation were performed at the Histocompatibility Laboratory of the Midwest Organ Bank Inc, Westwood, Kan., Results: Graft survival is significantly better (P = .03 [logrank test]) in regrafts when the flow crossmatch is used to select patients for transplantation., Conclusion: Flow crossmatching improves graft survival in cadaveric renal retransplantation by identifying a subset of patients with donor-directed HLA class I antibodies that are not detectable by our conventional antihuman globulin crossmatch.

A 39-week-old phenotypically female infant was born with hypoplastic left heart syndrome and expired on the third day of life. An autopsy revealed the patient to also have male pseudohermaphroditism and uterus bicornis bicollis. The association of hypoplastic left heart syndrome and male pseudohermaphroditism has been reported in only two previous patients.

We describe two cases of de novo membranous glomerulopathy in the renal allograft, with unusual histologic findings. In one patient the allograft nephrectomy specimen showed numerous foam cells in the intima of hyperplastic arteries along with prominent features of chronic rejection. In the other patient, prominent IgM deposits were seen in the glomerular mesangium without chronic rejection.

Renal involvement is a major cause of morbidity in patients with systemic lupus erythematosus (SLE). Histologic examination of renal tissue using light microscopy, immunofluorescent staining, and electron microscopy permit identification of glomerular immune complex deposits in virtually all patients with SLE. We report a patient who fulfilled four American College of Rheumatology criteria for the classification of SLE whose clinical course was consistent with SLE, yet whose renal failure resulted from focal glomerulosclerosis that was not mediated by immune complexes. The characteristics of this case of focal glomerulosclerosis that differentiate it from healed focal proliferative glomerulonephritis are discussed.

We studied a large North American Caucasian population of patients with biopsy proven IgA nephropathy for polymorphisms of HLA-A,B,C, HLA-DR, HLA-DQ and HLA-DP using a combination of serologic phenotyping and polymerase chain reaction sequence specific oligonucleotide probe (PCR-SSOP) hybridization genotyping. We also examined patients for polymorphisms of immunoglobulin by determining Gm and Km allotypes. When compared to healthy local controls there was an apparent decrease in HLA-DR5 (DRw11, DRw12) suggesting that this allele had a protective effect on disease susceptibility. None of the previously reported HLA-DQ associations found among Japanese or british Caucasian patients were found among this large North American Caucasian population. The HLA-DPB1*0601 genotype was increased among patients, but this was not significant when corrected for multiple comparisons. There were no differences in the distribution of Gm or Km allotypes among patients versus controls, regardless of whether they were stratified into those with progressive or non-progressive renal disease. Taken together, these findings suggest that there is substantial genetics heterogeneity in susceptibility to IgA nephropathy among different ethnic and/or geographically distinct populations.

Renal dysplasia and agenesis may be a familial disorder. We report the familial occurrence of unilateral renal agenesis and proteinuria that, at least in one case, was related to focal glomerulosclerosis. Whether these abnormalities are related to an intrinsic abnormality in the remaining kidney, hyperfiltration injury, systemic hypertension, or some other poorly defined factor is unclear at present. However, this report, along with previous case reports of familial renal agenesis, suggests that ultrasonographic screening of first-degree relatives of patients with renal agenesis is appropriate. Whether treatments such as dietary protein restriction, use of angiotensin-converting enzyme inhibitors, or other therapeutic interventions will have a beneficial effect in asymptomatic individuals with unilateral renal agenesis remains to be determined.

The effects of calcium antagonism on the development and progression of renal disease are controversial. To address this problem, studies were performed on young, uninephrectomized spontaneously hypertensive rats (SHRs) with the dihydropyridine calcium antagonist, manidipine, to assess its effect on the early pathogenesis of focal glomerulosclerosis. Male SHRs underwent uninephrectomy at age 10 to 11 weeks and were subsequently assigned to no treatment (control), a predetermined subvasodepressor (low) dose of manidipine (2.5 mg/kg body weight), or a predetermined antihypertensive (high) dose of manidipine (20 mg/kg body weight). All animals received a diet containing 0.4% sodium and 23% protein. Serial determinations of body weight, systolic tail cuff pressure, and 24-hour urinary excretion of creatinine, sodium, and protein (UprotV) were made at 1- to 6-week intervals, for a total treatment period of 12 weeks. In final experiments plasma was obtained for creatinine, angiotensin I, and angiotensin II determinations, and renal tissue was harvested for histologic and morphometric analysis. Compared with the untreated control, low-dose manidipine therapy had no effect on body weight, systolic blood pressure, creatinine clearance, UprotV, renal histologic findings, glomerular volume, or plasma angiotensin I or II concentrations. In contrast, high-dose manidipine therapy decreased systolic blood pressure from 194 +/- 3 to 160 +/- 4 mm Hg (p < 0.01). Creatinine clearance and UprotV were unchanged. Although body weight was not different, kidney weight was higher. However, mean glomerular volume was lower. More importantly, the prevalence of mesangial expansion with proliferation was lower: 6.7% (control) versus 2.8% (high-dose manidipine) (p < 0.01). Finally, plasma angiotensin I and angiotensin II concentrations did not differ.(ABSTRACT TRUNCATED AT 250 WORDS)

Serum samples from patients with connective tissue disease (CTD) were characterized using a recently developed enzyme-linked immunosorbent assay for reactivity with individual specific polypeptides of U1 small nuclear ribonucleoproteins, including the U1-70-kd protein. The distribution of HLA antigens was compared in CTD patients with and in those without anti-U1-70-kd autoantibodies and in normal controls. The frequencies of HLA-DR4 and HLA-DRw53 were increased among the anti-U1-70-kd autoantibody positive CTD group compared with the frequencies in anti-U1-70-kd autoantibody negative systemic lupus erythematosus patients and compared with normal controls. We conclude that the presence of autoantibodies reactive with the anti-U1-70-kd protein antigen is associated with HLA-DR4 and HLA-DRw53.

Two cases of ciliated endometrial adenocarcinoma which came to attention in less than one year are described. This entity deserves emphasis, as its ciliation may distract from its histological malignant features. Association of cilia with atypical nuclear features in the same cell is the diagnostic characteristic which distinguishes it from non-ciliated adenocarcinoma overrunning non-malignant ciliated endometrium. The ultrastructural detail of the cilia of malignant cells does not differ from that of non-malignant endometrial glandular epithelium. Our cases raise the question of a possible association of estrogenic effect with ciliated endometrial adenocarcinoma.

The ultrastructure, differential diagnosis, and biologic behavior of the peripheral pulmonary spindled carcinoid tumor are reviewed. Electron microscopy is useful in distinguishing the spindled carcinoid from a variety of neoplasms with similar histologic features. The spindled morphology is a rare expression of the carcinoid tumor that is almost exclusively confined to the lung periphery. It appears that the spindled carcinoid without atypical features is fully capable of regional lymph node metastases in approximately 20 per cent of the cases.

The authors report a case of leiomyosarcoma in the mediastinum presenting with superior vena cava obstruction, and also demonstrate the aggressive nature of this tumor in spite of radiotherapy. Where technically feasible, surgery appears to be the treatment of choice. The pathologic and electron microscopic features of this tumor are also described.

High levels of allosensitization (greater than 50%), which often occur in dialysis patients awaiting renal transplant, make donor selection difficult. Such patients may be included in elaborate protocols in which they are crossmatched with all available ABO compatible donors, or crossmatching may be deferred until a very-well-matched donor becomes available. The former approach of random crossmatching is costly and inefficient, while the latter approach may overlook crossmatch-compatible donors. We believe that the identification of antibodies present in highly reactive sera and the use of this information in donor selection would increase the frequency of crossmatch-negative donors for these patients. In this study eleven sera, reactive with 70% to 100% of a random cell panel, were obtained from multiply transfused dialysis patients. Sera were analyzed by standard (CDC) and antiglobulin augmented (AHG-CDC) lymphocytotoxicity, and by differential absorption with HLA-typed platelets. All sera contained only one or two antibodies directed against the high frequency public HLA epitopes, accounting for 85% to 100% of each serum's total reactivity. These characterized sera were crossmatched with 114 random normal donors. The frequency of negative crossmatches was 20.5%. However, if the serum antibody data had been used to preselect donors for crossmatch--that is, to exclude donors that were likely to be positive--the negative crossmatch frequency would have increased to 86.4%. The use of the serum analysis data in donor selection would have reduced the total number of required crossmatches by 78%. Serum analysis correctly predicted the outcome of 95.6% of crossmatches performed with an average of 3% false positives and 1.3% false negatives. This approach to donor selection reduces unnecessary crossmatching and increases the likelihood of finding crossmatch-compatible donors for highly reactive patients.

Following renal allograft transplantation, renal scans are frequently performed to evaluate anatomical and functional causes for allograft dysfunction. In our retrospective study of 20 patients, renal scans were found to be more expensive compared to renal biopsies $68,688 vs $7,421, and, in only one patient was aggressive anti-rejection therapy instituted based solely on the renal scan results. The 95% confidence interval for the proportion of correct diagnosis by renal scan was 0.16 to 0.62.

Sixteen years earlier a 42-year-old woman with an IgA kappa plasma cell neoplasm presented with bleeding disorder. Her prolonged course was complicated by subsequent development of rheumatoid arthritis, vascular purpura, and an acute membranoproliferative glomerulonephritis (MPGN). The paraprotein and its (Fab')2 fragment showed affinity for a test myeloma IgG2 (lambda ) paraprotein. The patient's serum and the IgA-IgG complex separated by gel filtration did not exhibit cryoprecipitation. The complex also did not dissociate by ultracentrifugation. Electron microscopic and immunofluorescent studies of a renal biopsy sample taken during the episode of nephritis showed subendothelial deposits and a lacy fluorescent pattern strongly positive for IgA and IgG. The same immunoglobulins were eluted from the kidney at postmortem. A low concentration of monoclonal IgA kappa (antibody) and excess unbound polyclonal IgG (antigen) were demonstrated in the patient's serum at the time of MPGN, apparently analogous to the conditions necessary for the induction of experimental immune complex nephritis.

Acute poststreptococcal glomerulonephritis (PSGN) is uncommonly seen in the elderly population and its diagnosis is not without some difficulty because clinical manifestations may mimic other diseases. Renal biopsy for diagnosis and early intervention, if indicated, is very valuable in such a situation. We present here a case of an elderly patient with PSGN and a review of the literature. In addition to the typical clinical manifestations of the disease, dyspnea and pulmonary congestion are commonly present in elderly patients, probably a result of excessive salt and water retention in the face of compromised cardiovascular function. Elderly patients with PSGN appear to have a remarkably poor prognosis, with significant incidences of acute mortality and chronic renal disease. Our patient had the unusual finding of a large number of glomerular crescents with near complete clinical recovery after short-term follow-up.

Presented is a unique case of accelerated hypertension in a 26-year-old black man. Clinically, the patient was found to have a left renal artery lesion and lateralizing renal vein renins. At surgery, a stenosing lesion was found in the artery supplying the upper pole of the left kidney. This was caused by a densely fibrotic mass involving the main renal artery. Microscopy revealed a necrotizing arteritis with no other arteries involved. This case of primary necrotizing arteritis of the main renal artery appears to represent a new entity because of its pathological and clinical differences with the known forms of large vessel arteritis. It is possible that this may represent a particularly acute and necrotizing stage of Takayasu's aortitis, or an extremely rare case of classical periarteritis nodosa confined solely to the main renal artery. The renin tissue assays document the hyperreninemia produced by arterial stenosis. The differential diagnosis of the lesion is considered, and the pathophysiology of hypertension due to large vessel arteritis is discussed.

Cowden disease (CD) is a familial syndrome characterized by tumors of the skin, oral mucosa, breast, thyroid, and intestinal epithelium. Since the syndrome is inherited as an autosomal dominant, we examined a battery of gene markers in a family with CD to detect linkage between the CD gene and known marker genes. There was no positive evidence for linkage of a CD locus with any of the markers; other investigators can add to our data to confirm and extend these findings. Additionally, we measured epidermal growth factor (EGF) in body fluids from CD patients and controls to determine if elevated EGF levels might be responsible for the widespread epithelial proliferation in CD. EGF levels in saliva, serum, plasma, and urine were similar in CD patients and control subjects. Although alterations in growth factors or their receptors may play a role in CD, excess circulating EGF is not responsible for the manifestations of the syndrome.

A report of the occurrence of membranous nephropathy and peripheral neuropathy in a patient with systemic mastocytosis is presented. Previous reviews of patients with systemic mastocytosis have not noted this association. During cyclical therapy with prednisone and chlorambucil in this case, nephrotic-range proteinuria remitted. Peripheral neuropathy resolved 10 months after discontinuation of therapy. Pathophysiological mechanisms resulting in this clinical presentation may be immunologically mediated.

To examine the role of the renal vascular receptor in the control of renin secretion in the rat, a denervated, nonfiltering kidney model (DNFK) was developed. The left kidney was subjected to a 2-hr period of total renal ischemia followed by ureteral ligation and section Denervation was accomplished by stripping all visible nerves and painting the renal vessels with 5% phenol. Forty-eight hours later lissamine green dye was injected iv and failed to appear in either the cortical or medullary tubules, indicating that glomerular filtration had ceased. Histological study of these kidneys revealed diffuse tubular necrosis with extensive intratubular cast formation. Norepinephrine content of the DNFK was reduced 91% compared to the contralateral normal kidney (P less than 0.001). In another group of anesthetized rats with a single DNFK, 15 min of suprarenal aortic constriction (SAC) increased plasma renin activity (PRA) from 3.4 +/- 0.6 to 11.5 +/- 1.6 ng AI/ml/hr; in a time control series, PRA was unchanged. To exclude the influence of adrenal catecholamines in this response, bilateral adrenalectomy was performed in a separate group of animals with a DNFK. In this series, SAC also markedly increased PRA. The present data indicate that in the rat the macula densa, the renal nerves, and adrenal catecholamines were not essential for the hyperreninemia induced by a reduction in renal perfusion pressure.

Two children with the clinical and histopathological features of disseminated histiocytosis X became refractory to chemotherapy and a diffuse erythrophagocytic process developed. No cause of the erythrophagocytosis could be found. Pancytopenia and marked erythrophagocytosis persisted until death ensued. The relationship of this disorder to familial erythrophagocytosis is discussed.

A patient with erosive peripheral arthritis in whom vasculitis and monoclonal IgG kappa paraprotein were associated with sacroiliitis and widespread destruction of intervertebral discs is reported. Crystals resembling apatite were identified in intervertebral disc material, and we postulate that the discitis was accelerated by apatite deposition. Our case illustrates a unique example of axial involvement in rapidly progressive joint disease.