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Biomedical subjects

C E Alpers

Publications and source records attributed to C E Alpers.

At least 163 records · Page 9Linked to original sources

Single light chain subclass (kappa chain) immunoglobulin deposition in glomerulonephritis.

Renal glomerular disease characterized by the deposition of immunoglobulin light chains or monoclonal immunoglobulins was demonstrated by immunofluorescence microscopy in 11 patients. The most common histopathologic findings were those of mesangiocapillary glomerulonephritis, but considerable variability was observed. Lesions resembling diabetic glomerulosclerosis and amyloidosis were seen in some patients. Immunofluorescence findings in seven patients showed concomitant, equally intense staining for kappa light chain and immunoglobulin heavy chain (IgG or IgA), indicative of monoclonal immunoglobulin deposition. Specimens in the remaining cases stained predominantly for kappa light chain alone. In six cases the histologic and ultrastructural pattern was similar to that of type I mesangiocapillary glomerulonephritis. In three cases linear deposits were present, predominantly in subendothelial and inner glomerular basement membranes and, to a lesser degree, in mesangial locations, as in type II mesangiocapillary glomerulonephritis. In one of the latter cases dense deposits were intermixed with aggregates of amorphous fibrillar material indistinguishable from amyloid. In two cases involving IgA kappa chain deposition the histologic and ultrastructural appearance was that of mesangial glomerulonephritis. Considerable heterogeneity was found in the clinical features of the patient population. Specific clinical or serologic parameters for this disease could not be identified. Only one patient had an associated lymphoplasmacytic disorder. After follow-up periods ranging from six months to 17 years, all of the patients were alive, including four who had progressed to end-stage renal disease and required dialysis. Two of the latter patients underwent successful renal transplantation; one had been alive for five years and the other for three months without evidence of recurrence of the renal disease at the last follow-up examination.

Adult↗

The prevalence of carcinoma in situ in normal and cancer-associated breasts.

Two hundred ninety-two human breasts were examined in toto by a subgross sampling technique with histologic confirmation. The samples consisted of 185 breasts from random autopsies, 63 cancer-containing breasts, and 44 breasts contralateral to cancer-containing breasts. The method permits the identification and enumeration of essentially all of the dysplastic, hyperplastic, and neoplastic lesions present in each breast. Emphasis was on the prevalence within each sample category of ductal carcinoma in situ (DCIS), lobular carcinoma in situ (LCIS), and epithelial proliferative lesions with severe atypia, previously termed ALA 4 and ALB 4, which correspond to the clinicopathologic entities atypical ductal hyperplasia and atypical lobular hyperplasia, respectively. Additional primary foci of DCIS (unrelated to invasive breast carcinoma, if present) were found in 52.5 per cent of cancer-containing breasts, and were seen in 47.7 per cent of contralateral and 5.9 per cent of the breasts from random autopsies. Lobular carcinoma in situ was generally seen only in association with infiltrating carcinoma, usually of the ductal type. No LCIS was seen in the breasts from random autopsies. These trends are the same if the proliferative lesions with severe atypia are included with carcinoma in situ. The numbers of lesions were also markedly greater in affected cancer-associated breasts than in affected breasts obtained from autopsies. These findings suggest that LCIS, although a rare lesion in the general population, may be a significant marker for clinical carcinoma. They support previous studies showing a small percentage of women with undetected DCIS of uncertain clinical and biological potential. The multicentric nature of preinvasive breast carcinoma is further substantiated. Finally, when the prevalence and number of lesions are considered in association with the ages of the patients, the lower prevalence of such lesions in the older patients in each sample suggests that at least some DCIS and LCIS may be dependent on a premenopausal hormonal milieu for their continuing existence.

Adult↗

Enzyme histochemistry in plastic-embedded sections of normal and diseased kidneys.

Interest in the role of mononuclear phagocytes in glomerulonephritis (GN) and in defining markers of renal neoplasms led the authors to study alpha-naphthyl acetate/butyrate esterase (ANAE/ANBE), alkaline phosphatase (AlkP), acid phosphatase (AcP), 5'-nucleotidase (5'N), and ATPase (ATP) activity in paraformaldehyde-fixed, plastic-embedded renal tissue from patients with a variety of pathologic conditions. These conditions included GN, renal tumors, and transplant rejection. Enzymatic staining for ANAE, ANBE, AcP, AlkP, and ATP was generally confined to tubules and collecting ducts in normal kidney. Nine of 10 cases of renal carcinoma had weakly to strongly positive reactions with AlkP, AcP, and ANAE; 9 of 10 cases of Wilms' tumor showed weakly positive reactions with AcP and ANAE, particularly in tubular structures. Severely damaged kidney allografts showed surprising retention of normal histochemical features. In all cases 5'N stained both glomerular capillaries and interstitial vasculature; ATPase and AlkP stained interstitial vessels only. Plastic embedding provides superb preservation of both microscopic anatomy and enzymatic activity, which may allow utilization of enzyme histochemistry for diagnostic and research purposes.

5'-Nucleotidase↗

Malignant neuroendocrine tumor of the jejunum with osteoclast-like giant cells. Enzyme histochemistry distinguishes tumor cells from giant cells.

We report a case of a neuroendocrine tumor of the jejunum metastatic to the liver in a 26-year-old woman. Light and electron microscopy of this tumor revealed a poorly differentiated neoplasm composed of clusters of round to polygonal cells compatible with a diagnosis of neuroendocrine tumor. In the absence of identifiable silver-staining granules or immunocytochemical demonstration of a specific hormone product in tumor cells, this tumor cannot be further classified among the various neuroendocrine tumors that may arise in this location. However, interspersed among tumor cells was a distinct population of multinucleate giant cells having an appearance similar to benign osteoclasts. Enzyme histochemistry for 5'-nucleotidase, acid phosphatase, and nonspecific esterase each showed a dichotomous staining pattern for the small tumor cells and giant cells and suggest that the giant cells are not tumor derived, but represent a second, presumably reactive, cell population.

5'-Nucleotidase↗

Magnetic resonance imaging and spectroscopy of the periarticular inflammatory soft-tissue changes in experimental arthritis of the rat.

Arthritis was induced in rats by intradermal injection of Freund's complete adjuvant. MRI was performed with a resistive imager operating at 0.35 T. A spin echo (SE) technique with TR = 0.5 and 2.0 seconds, TE = 28 and 56 msec was used. Transaxial images of hindpaws and knees were obtained at different times after injection of adjuvant. In vitro proton spectroscopy of normal and arthritic hindpaws was also performed. Histologic confirmation was obtained in each case. Inflammatory soft-tissue lesions were seen as focal areas of high intensity on spin echo images obtained with TR = 2.0 seconds and TE = 56 msec and were characterized by long T1 and T2 relaxation times and high spin density. In comparison with both conventional radiography and physical examination, early soft-tissue changes were detected more frequently by MRI. This study suggests that MRI is likely to be of value for the early diagnosis of arthritis.

Animals↗

Hemangiomatous anomaly of bone in Crouzon's syndrome: case report.

A striking and diffuse hemangiomatous architecture was identified by microscopic examination of bone specimens obtained at cranial synostectomy and from the craniofacial area of a young boy with Kleeblättschadel deformity and Crouzon's syndrome. A retrospective review of the histopathological appearance of bone specimens from other patients with Crouzon's syndrome treated on our service and a review of the literature on craniosynostosis suggest that the hemangiomatous pattern seen in this patient is rare if not unique. The unusual pathological findings provide evidence that the underlying pathophysiology of this disorder may be heterogeneous.

Abnormalities, Multiple↗

Fetus in fetu associated with an undescended testis.

Fetus in fetu is an extremely uncommon cause of an abdominal mass in the neonate; fewer than 30 generally accepted cases are recorded in the literature. We report a case of intraabdominal fetus in fetu, with a unique location within an undescended left testicle. Chromosomal studies of cells from the fetus in fetu and the surviving infant revealed identical 46,XY karyotypes.

Abnormalities, Severe Teratoid↗

Autopsy findings in the acquired immune deficiency syndrome.

The medical records, premortem biopsy specimens, and autopsy materials from 36 patients with acquired immune deficiency syndrome (AIDS) were reviewed. The majority of the patients were homosexual men. Widely metastatic Kaposi's sarcoma was seen in eight patients, while tumor was confined to the skin in an additional ten. Four patients had high-grade lymphomas. Thirty-five of the 36 patients had at least one opportunistic infection at some point in the course of their illness. Many patients had multiple infectious agents. The most consistent and striking autopsy finding was a severe depletion of lymphoid tissues. Death in the majority (83%) of the patients was attributable to opportunistic infections affecting most commonly the respiratory tract (64%) and meninges (11%). Three patients (9%) died with widely metastatic Kaposi's sarcoma and secondary hemorrhage.

Acquired Immunodeficiency Syndrome↗

Malignant fibrous histiocytoma, myelomonocytic leukemia, and Hodgkin's disease arising in an elderly man.

A 74-year-old man developed a locally invasive malignant fibrous histiocytoma of the scalp with multiple facial recurrences and concomitant hematopoietic dysplasia occurring over a 2-year period. One month prior to his death, his hematologic profile evolved into myelomonocytic leukemia. Mixed cellularity Hodgkin's disease involving mediastinal and periportal lymph nodes, which was not suspected antemortem, was discovered at autopsy. In the experience of the authors, this association of neoplasms is unique and raises the possibility in this case of an unidentified stimulus to neoplastic transformation of cells of histiocyte/monocyte origin.

Aged↗

Light-chain glomerulopathy with amyloid-like deposits.

A 40-year-old man with rapidly progressive renal failure was found to have a lobular glomerulonephritis by renal biopsy. Immunofluorescent microscopy showed prominent glomerular deposition of both kappa and lambda light chains but no significant heavy-chain component. Ultrastructurally, electron-dense deposits in the mesangium and capillary basement membranes had a fibrillar appearance indistinguishable from amyloid. This case illustrates a "light-chain glomerulopathy" distinct from previously reported glomerulopathies associated with the deposition of light chains of a single subclass.

Amyloid↗

Subgross pathologic features and incidence of radial scars in the breast.

Eighty-three breasts obtained at random autopsy, which were presumed to be at low risk for development of cancer, and 107 cancer-associated breasts (containing cancer or contralateral to cancer-containing breasts), which were presumed to be at high risk, were studied in their entirety using a subgross slicer method with histologic confirmation. Twelve breasts (14 per cent) from the random autopsy series had from one to 13 radial scars each, with an average of 7.7 radial scars per involved breast; the average number of radial scars per breast for the entire series of 83 breasts from the autopsy series was 1.1. Twenty-eight breasts (26 per cent) of those from cancer-associated series had from one to 31 radial scars each, with an average of 15.5 per involved breast; the average number of radial scars per breast for the entire series of 107 cancer-associated breasts was 4.4. Radial scars are observed in breasts from patients in the same age--37 to about 85 years--in both series. Atypical epithelial hyperplasia ( epitheliosis ) was less frequent and less severe in breasts from the random autopsy series than in the cancer-associated breasts. Invasive carcinoma was not found in step sections of any of the radial scars; however, carcinoma in situ of the ductal type was found in the radiating arms of one. It is concluded that the presence of radial scars is at least a "marker" for enhanced tissue risk for cancer development, in much the same manner that some other mammary dysplastic features are "markers." The data do not support or definitely negate the hypothesis that tubular carcinoma develops in radial scars and subsequently evolves into the more common types of cancer, as proposed by some authors.

Adult↗

Congenital (infantile) hemangiopericytoma of the tongue and sublingual region.

Congenital (infantile) hemangiopericytoma is a rare lesion, previously described only in subcutaneous and central nervous system locations. The authors report here an obstructing tumor of the tongue and sublingual oral cavity, discovered at birth, in an otherwise normal female infant of 35 weeks' gestation. The tumor had a histopathologic and ultrastructural appearance similar to previous descriptions of infantile hemangiopericytoma. Clinical features in this case included rapid local recurrence after initial excision; however, after 30 months of follow-up, there has been no evidence of further recurrence or metastasis. Because this patient was treated with chemotherapy, the authors cannot determine to what extent this benign course reflects the natural history of this process or the influence of the treatment administered.

Cyclophosphamide↗

Enzyme histochemistry of normal and neoplastic transitional epithelium.

The authors utilized the technic of plastic embedding with enzyme histochemistry for the evaluation of enzymatic expression by epithelium of the lower urinary tract in humans. Alpha-naphthyl acetate esterase and acid phosphatase generally were expressed by normal and neoplastic urothelium. Expression of 5'-nucleotidase and ATPase was more restricted. Alkaline phosphatase, prominent in the transitional cells of lower mammalian species, generally was not present in human urothelium. Enzyme histochemistry has not been applied generally to the study of disease of the lower urinary tract, but this study suggests it may be of value in understanding the biology of this tissue and be of potential use in histopathologic diagnosis of diseases of this region.

5'-Nucleotidase↗

Sonographic evaluation of experimental acute renal arterial occlusion in dogs.

Eleven segmental and eight total renal artery occlusions were evaluated by sonography in transplanted kidneys of 19 adult mongrel dogs. The segmental occlusions were serially scanned daily or every other day for up to 35 days. The total occlusions were scanned daily for up to 10 days. Each occlusion was confirmed angiographically, and kidneys were examined pathologically. Acute segmental renal artery occlusion produces a sequence of sonographic changes, beginning with a focal hypoechoic mass at 24 hr, which stays unchanged for 5-7 days. At 7 days, internal echoes appear, and the infarct slowly consolidates to an echogenic, slightly depressed focus at 17 days. Total renal artery occlusion produced no appreciable change in cortical echogenicity and only slight increase in size. Acute segmental renal infarction can be detected early in its course and demonstrates a sequence of changes that may aid in dating the infarct. Total renal infarction may appear normal sonographically, and further studies are needed to confirm that diagnosis.

Animals↗

Pleomorphic carcinoma of the gallbladder: case report and ultrastructural study.

Carcinomas of the gallbladder with pleomorphic spindle cell histology are unusual. We report such an occurrence in a 47-year-old male with a neoplasm showing both a mixed squamous and a pleomorphic spindle cell pattern. Ultrastructural studies supported the likelihood that this lesion represented a pleomorphic carcinoma and revealed unique paracrystalline structures intimately associated with tonofilaments. For this reason we believe that this and some other previously diagnosed sarcomas and carcinosarcomas of the gallbladder are in reality poorly differentiated carcinomas of either adeno, squamous, or mixed type. This illustrates the need for critical ultrastructural assay to determine the nature of poorly defined and differentiated neoplastic growths.

Carcinoma↗

Computerized tomography of cranial sutures. Part 1: Comparison of suture anatomy in children and adults.

Knowledge of normal suture anatomy and development is vital in order to understand abnormal suture development and to be able to distinguish sutures radiographically from normal anatomical structures and possible skull fractures. The anatomy of the sutures and synchondroses of 150 normal pediatric and adult patients was studied using high-resolution computerized tomography scanning. Sutures of both the calvaria and skull base were most accurately identified in axial and coronal high-resolution thin-section scans when bone window algorithms were used. Developmental changes of the sutures and synchodroses , the inner and outer tables, and the diploic space were all well delineated. Vault sutures could be identified routinely in children, but their presence in adults varied considerably. With increasing age, parasutural sclerosis developed and sutures were more closely apposed.

Adolescent↗

Computerized tomography of cranial sutures. Part 2: Abnormalities of sutures and skull deformity in craniosynostosis.

Preoperative computerized tomographic (CT) scans of 24 children who had surgery for either single or multiple craniosynostoses were compared with skull radiographs and operative and pathological findings. In addition to providing accurate imaging of calvarial and skull base deformities secondary to premature suture closure, high-resolution CT with bone definition algorithms supplied valuable detail of anatomical changes at the abnormally developed suture. The CT findings varied with the location of the suture. Thickened bony ridges predominated at the sagittal suture, focal bone thickening and erosions were more likely to be found at the metopic suture, and parasutural sclerosis was the prevalent finding on one side of the lambdoid suture. No evidence of the suture could be detected in the majority of patients with complete coronal craniosynostosis. Radiographs of the skull were shown to be a relatively insensitive means of imaging the zone of limited fusion, especially the lambdoid suture. An excellent correlation was found between the CT scan and the operative and pathological findings. There was histological evidence of progressive suture fusion in virtually all patients. An asymmetrically narrowed lucent zone with parasutural sclerosis or bony ridges seen on CT scans correlated well with fibrous union of the suture found on histological examination. The authors conclude that high-definition CT used in conjunction with bone windows and thin and coronal slices for the evaluation of sagittal sutures is a useful imaging method for the evaluation of craniosynostosis.

Cranial Sutures↗

Late development of systemic lupus erythematosus in patients with glomerular "fingerprint" deposits.

Two patients presenting with nephrotic syndrome but without evidence of collagen vascular disease had organized glomerular immune deposits with a "fingerprint" pattern. This finding has been previously associated with lupus nephritis and, in our institution, has been seen in 6% of the biopsy specimens from patients with lupus nephritis. Clinical signs and symptoms of systemic lupus erythematosus in these two patients did not develop until 2 and 5 years later, respectively. The cases of these patients suggest that glomerular deposits with a fingerprint pattern may be a specific marker for lupus erythematosus even when overt clinical features of this disease are lacking. Patients with this finding on renal biopsy should have an extended follow-up for possible development of lupus erythematosus.

Adult↗