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Biomedical subjects

E Kahn

Publications and source records attributed to E Kahn.

At least 109 records · Page 6Linked to original sources

Hepatitis in children with acquired immune deficiency syndrome. Histopathologic and immunocytologic features.

Hepatic morphology and immunocytology were evaluated in 4 children with clinical and immunologic characteristics of the acquired immune deficiency syndrome or acquired immune deficiency syndrome related complex. All 4 children had hepatomegaly and increased serum alanine and aspartate aminotransferase activity. Both lobular and portal changes were noted. Lymphocytic infiltration, piecemeal necrosis, hepatocellular and bile duct damage, sinusoidal cell hyperplasia, and endothelialitis were prominent. Vesicular rosettes in sinusoidal lymphocytes and tubuloreticular structures in sinusoidal endothelial cells were demonstrated by electron microscopy. The lymphocytic infiltrate in both the lobular and portal spaces was characterized by a relative increase of cytotoxic/suppressor (T8) cells. Hepatitis may be a common feature of pediatric acquired immune deficiency syndrome and acquired immune deficiency syndrome-related complex. Although the histopathologic changes are consistent with chronic active hepatitis, the specific pathogenesis remains to be determined.

Acquired Immunodeficiency Syndrome↗

Cancer family syndrome: marker studies.

Individuals from kindreds with the cancer family syndrome (CFS) have an increased hereditary risk for the development of adenocarcinoma of the colon in childhood and early adulthood. Previous studies have suggested that this high occurrence of adenocarcinoma may be due to a genetic defect in the control of colonic epithelial proliferation. Others have suggested that these families may have an underlying abnormality in immunologic tumor surveillance. We have investigated these possibilities in 15 cancer-free, at-risk individuals (10 children, ages 3-15 yr, and 5 adults) from two unrelated CFS kindreds. Colonic mucosal proliferative activity was studied by in vitro autoradiography after tritiated thymidine labeling in 7 subjects. The mean labeling index (12.7 +/- 0.9%) was comparable to that in controls, as was the distribution of thymidine labeling. Immunologic evaluation revealed depressed lymphocyte culture responses to stimulation by microbial antigens, but not to that by mitogens. Mixed lymphocyte culture responses were depressed in 4 of 8 subjects, but became normal in 2 of these after filtration through a Sephadex G10 column. Natural killer cell cytotoxicity was significantly depressed in 5 of 13 subjects, and borderline normal in another 3 subjects. These data suggest that many cancer-free members of CFS kindreds have a spectrum of in vitro cell-mediated immunologic defects that might interfere in vivo with the recognition or killing of incipient tumor cells.

Adenocarcinoma↗

Islet cell hyperplasia: an unusual cause of hypoglycemia in an adult.

This is a case presentation of a 32-year-old man with a one year history of symptomatic hypoglycemia and documented elevations of his fasting plasma insulin to glucose ratio, caused by islet cell hyperplasia. Islet cell hyperplasia is a common cause of hypoglycemia in the pediatric population, but is very rare in adults. As in the pediatric group, adults should be treated with subtotal (75-85%) resection of the pancreas and with diazoxide for symptomatic recurrence of hypoglycemia. We suggest that the term islet cell hyperplasia is preferred to designate a diffuse proliferation of endocrine cells that may express itself with different morphologic patterns, varying from case to case. Islet cell hyperplasia, therefore, comprises nesidioblastosis, endocrine cell budding from ductal structures, as well as islet and islet cell hypertrophy, septal islets, islet dysplasia, and adenomatosis. Immunohistochemistry is a valuable method for the demonstration of the polymorphic hormonal content of the proliferated islet cells. We propose that the term nesidioblastosis, previously used to describe some similar cases, should be avoided because of confusion about its definition.

Adult↗

Evaluation of elastosis in breast cancer.

The possibly prognostic value of elastosis in breast carcinoma has usually been visually evaluated, using subjective gradings whose variability has led to contradictory conclusions. We used a Magiscan 2 automated image analyzer to perform objective measurements on elastosis on a set of 52 slides that were also subjectively evaluated. The wide range of parameters measurable by the automated system seemed likely to successfully deal with the lack of homogeneity of elastosis that causes problems in subjective evaluations. The homogeneity parameters (percentage distributions of elastosis in each slide) were processed by factorial analysis. On the plane defined by the first two factors, the principal components analysis produced a clustering of cases that was, on the whole, in concordance with the subjective evaluation.

Breast Neoplasms↗

Evaluation of colonic neoplasia by flow cytometry of endoscopic biopsies.

Colonoscopy is a valuable and frequently used method in the evaluation of colonic neoplasia. Flow cytometry is a technique that can be used to diagnose malignancy. In this study, flow cytometry was used to evaluate colonoscopic biopsies taken from patients with suspected colonic neoplasia. Nineteen colonic biopsies were obtained and evaluated by this technique. Aneuploidy was demonstrated in six patients with carcinoma of the colon. In addition, abnormal DNA histograms were noted in two premalignant conditions (colonic adenoma and inflammatory bowel disease). The results show that flow cytometry can be applied to colonic biopsies and suggest that it may be of use in the diagnosis of malignant and premalignant conditions of the colon.

Adenoma↗

Hemangioendothelioma with intravascular coagulation and ischemic colitis.

An infant who presented with a thigh mass and coagulopathy was found to have a hemangioendothelioma. The tumor rapidly enlarged despite accepted therapeutic modalities, and the child died after a sudden cardiac arrest. Postmortem examination revealed a highly invasive tumor mass that had infiltrated the bowel and involved the inferior mesenteric artery, resulting in ischemic colitis of the left colon. A discussion regarding the pathophysiology with respect to endothelial cell properties and other microscopic features of the tumor is presented.

Abdomen↗

Pseudopolyps of the small intestine in Crohn's disease.

The morphologic features of two types of pseudopolyps of the terminal ileum in a patient with Crohn's disease are described. One form of pseudopolyp is indistinguishable from that observed in the large intestine in Crohn's colitis, while the second, nodular lymphangiectasia, seems to be unique to the small bowel.

Adult↗

Effect of alkylating agents on hematopoiesis in myelofibrosis. 4 case report.

Four patients presenting with myelofibrosis (2 primary myelofibrosis and 2 postpolycythemic myeloid metaplasia) were treated with alkylating agents. For three patients (one treated with busulfan and two treated with chlorambucil) the treatment was a success: the general condition improved, the splenomegaly decreased or disappeared, and the blood picture returned to normal. Moreover, for two cases, a trend towards polycythemia was observed under treatment. For the fourth patient, treated with chlorambucil, there was no improvement: a life-threatening, pancytopenic phase developed at the end of the treatment, but it disappeared after 2 months. For the three successfully treated cases, a redistribution of hematopoiesis from spleen to bone marrow was shown by ferrokinetics, 59Fe scans, and bone marrow biopsies. In addition, in the case treated with busulfan, a decrease in the bone marrow granulopoietic pool at the expense of the erythropoietic one was observed. No redistribution was seen in the patient for whom the treatment was a failure. In this case, the spleen remained the major site of active hematopoiesis. Studies on blood granulomonocytic-colony forming cells (GM-CFC's) helped to discriminate the successfully treated patients from the unsuccessfully treated one. In the successfully treated patients, the GM-CFC concentrations dropped to normal values and increased again within weeks following the treatment interruption; this increase involved mainly high density GM-CFC's (greater than 1.060). In the unsuccessfully treated patient, GM-CFC concentrations decreased only after 5 weeks of intensive treatment. The mean density of the GM-CFC's was 1.064 before treatment, shifted towards 1.060 during the neutropenic phase and returned to 1.064 during the recovery.

Alkylating Agents↗

Perianal disease in children and adolescents with Crohn's disease.

The clinical and pathological features of perianal disease in children and adolescents with Crohn's disease have been delineated. Of the 149 patients (mean age 12.1 yr, range 0.75-21 yr, 68% male), 73 (49%) had perianal disease including 51 with fissures and tags, 10 with fistulas, and 12 with abscesses. Ileocolitis was seen in 68% of all patients and in 75% of patients with perianal disease (p greater than 0.10). Rectal inflammation was present in 94% of patients with fistulas or abscesses, or both, versus 63% of all patients without perianal disease (p less than 0.025). Anorectal granulomata were found in 47% of fistula/abscess patients versus 9% of non-perianal disease patients (p less than 0.05). A variety of medical regimens failed to consistently affect these lesions favorably. Four of 10 patients with fistulas healed after 1-5 yr of intermittent discharge. Nine of 12 patients with abscesses required surgical drainage, and 50% subsequently developed chronic fistulas. Granulomata were equally distributed between those patients who healed and those who developed chronic fistulas. Although an important cause of morbidity in childhood Crohn's disease, perianal disease is not progressively destructive in the majority of patients.

Abscess↗

Physical requirements of a 59Fe bone marrow distribution digital scanning study.

A method is described to provide whole-body scannings of 59Fe bone marrow distribution using a tungsten-alloy collimator. The physical requirements of this method were determined by means of dedicated phantoms. As we were only interested in bounded 59Fe regions (sacral bone, liver and spleen), it was necessary to subtract the 59Fe activity in the blood. An estimate of this activity was obtained by means of 99mTc-labelled erythrocyte whole-body scanning to which a correction factor K was applied. The independence versus depth was performed by computing geometrical means of prone and supine images. The results of these experiments indicate the possible feasibility of a scanning method for a ferrokinetic study in place of the usual external counting.

Bone Marrow↗

Feasibility of a 59Fe ferrokinetic study based on bone-marrow scans.

The purpose of this study was to evaluate the feasibility of a method that would use 59Fe quantitative sequential scintigrams together with a few blood samples in place of conventional ferrokinetic studies. To quantitate the efficiency of erythropoiesis or iron deposition sites, the radio-iron blood activity was subtracted by means of a whole body scanning with 99mTc-labelled erythrocytes. The study was performed on 31 patients (36 examinations) and regions of interest were drawn by three physicians on the sacral bone, the liver, the spleen and the femoral bone. To assess the physical feasibility of the scintigraphic method, correlation coefficients were computed between the number of counts of the 59Fe and 99mTc images and the corresponding 99mTc and 59Fe activities. To assess the clinical feasibility, the consistency of the data resulting from the drawing of the ROIs was verified and those of conventional ferrokinetics were introduced and their consistency verified. These assessments suggest that the scintigraphic method can be used in place of the conventional external counting method.

Bone Marrow↗

Selective fetal malnutrition: the effect of in vivo ethanol exposure upon in vitro placental uptake of amino acids in the non-human primate.

In vitro uptake (45 and 90 minutes) of amino acids, alpha-amino isobutyric (AIB) and valine (VAL), was measured in six placentae from the nonhuman primate, Macacca fascicularis. Three of the pregnant primates were chronically treated with ethanol before and throughout pregnancy (CHR); one during the last trimester only (LT); and two were controls (C). Compared to the C placentae, the LT placenta had significantly decreased uptake only for AIB at 45 min: 33.4 +/- 6.8% reduction (mean +/- S.E.) (P less than 0.05). In contrast, the CHR placentae demonstrated significantly reduced uptake (P less than 0.01) for both amino acids at both time points. Percent reduction at 45 and 90 min: AIB, 35.2 +/- 6.5% and 32.6 +/- 5.6% and VAL, 38.7 +/- 2.9% and 22.1 +/- 4.1%. The results indicate that chronic in vivo ethanol exposure impairs the in vitro placental uptake of two actively transported amino acids, using an animal with a placenta almost identical to the human.

Aminoisobutyric Acids↗

Proliferative defects in ulcerative colitis patients.

The proliferative characteristics of epithelial cells in rectal biopsies from a group of 18 patients (ages 18-72) with ulcerative colitis spanning several months to over 20 years duration were examined, using an in vitro 3HTdR labeling technique. Although the labeling indices for patients with inflammatory bowel disease did not differ significantly from that of control (8.9 +/- 3.6 vs 7.7 +/- 3.9), the number of labeled cells/individual crypt column was higher among some crypts in ulcerative colitis patients. A normal proliferative pattern was demonstrated in crypts of 5/18 patients (27.8%) with DNA synthesis confined to the lower two-thirds of the crypt and the lower third the major zone of proliferation. Thirteen patients (72.2%) showed extension of the proliferative compartment to the surface of the glands, while retaining the lower third of the gland as the predominant proliferative zone (Stage I abnormality). Of these 13, seven had an additional abnormality, namely, a shift of the major zone of proliferation to the middle third of the crypt (Stage II abnormality), a pattern first reported in the colorectal mucosa of patients with a history of colon cancer. Precancerous lesions, including incompletely differentiated epithelium and lateral crypt budding, were recognized in all of these cases, suggesting that the Stage II abnormality is a proliferative parameter forecasting neoplastic transformation.

Adolescent↗

Recurrent free perforation in gastroduodenal Crohn's disease.

Three separate episodes of free perforation of the duodenum and stomach are described in a 47-year-old white man with histological evidence of gastroduodenal Crohn's disease, co-existing with peptic disease. His mother died of Crohn's disease after ostomy surgery and two brothers required ileostomy for inflammatory bowel disease. In 1972, the patient underwent a bypass gastroenterostomy for obstructing Crohn's disease of the descending duodenum. In 1977, 1978, and 1982, three free perforations occurred first in the duodenum and then twice at the gastrojejunostomy site. This occurred while on continuous Cimetidine therapy as well as steroids and after vagotomy. The basal and maximum acid outputs, secretin stimulation test, and serum gastrins were normal throughout his clinical course. This unique situation of acid peptic perforation in the presence of active Crohn's disease, poses an etiological dilemma and signals the vulnerability of the diseased stomach and duodenum in transmural disease.

Crohn Disease↗