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Biomedical subjects

E Kahn

Publications and source records attributed to E Kahn.

At least 91 records · Page 5Linked to original sources

Prognostic significance of epithelioid granulomas found in rectosigmoid biopsies at the initial presentation of pediatric Crohn's disease.

The prognostic significance of epithelioid granulomas in Crohn's disease (CD) remains controversial. We have determined the prognostic significance of epithelioid granulomas noted in endoscopic rectosigmoid biopsies obtained from untreated pediatric patients at initial presentation of CD. Data collected from 19 subjects with rectosigmoid granulomas and inflammation (Group 1) were compared to those obtained from another 37 subjects (Group 2) with CD of the rectosigmoid, but in whom no granulomas were present. Both groups had similar ages at disease onset [Group 1: 11.6 +/- 3.6; Group 2: 10.4 +/- 4.0 years (X +/- SD)]. At diagnosis, Group 1 had more extensive CD (small bowel and colon involvement, with Group 1 74% versus Group 2 30%; isolated rectosigmoid involvement, with Group 1 11% versus Group 2 35%; p less than 0.01). Perianal disease was also more common in Group 1 (58% versus 27% Group 2; p less than 0.05). However, CD activity scores and clinical laboratory findings were comparable. Duration of follow-up was identical (5.6 +/- 3.3 years) for both groups. By the end of the period of follow-up, 43% of Group 2 had developed small bowel involvement, but Group 1 continued to have more extensive CD (p less than 0.05). Perianal fistulae and abscesses and anal stenosis were more frequent in Group 1. Throughout this period, the need for oral and parenteral corticosteroids, 6-mercaptopurine, and nutritional support modalities were similar in the two groups. Although the number of subjects requiring hospitalization and the number of hospitalizations per patient were similar in both groups, Group 1 had more surgery (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗

Microcomputer system for ion microscopy digital imaging and processing.

Analytical ion microscopy is a powerful tool for biological tissue analysis as it allows direct chemical distribution imaging, even at low element concentrations. A microcomputer based digital imaging system achieving acquisition at low light level is presented. It includes a high sensitivity video camera connected to a specialized image processor subsystem. Acquired images consist of 512 x 512 pixels with 8 bits accuracy. Real-time image processing software has been implemented so that image processing may be performed on-line. Image processing software allows off-line image manipulation and correlation for biological interpretation of elemental mapping images. System capabilities are illustrated by a study of stable and radio iodine mapping in rat thyroid tissue.

Algorithms↗

Shape description in osteology using automatic Fourier analysis. Example of the human atlas.

An image analysing procedure for the shape characterisation in osteology is described. We used expansion in Fourier series of an equiangular polar representation of the contour. Fourier analysis is an information preserving technique, and it is therefore possible to reconstruct the original contour from the shape descriptors (Fourier coefficients and descriptors). The properties of the truncated expansion of the Fourier series can be used for smoothing effect and noise reduction, for interpolated reconstruction, and for data compression. Fourier analysis also allows a quantitative description of the shape. The first components describe the gross feature, the following ones the fine details.

Cervical Atlas↗

Osteometry by computer-aided image analysis: application to the human atlas.

Computer-assisted image-analysis having almost not been applied to macroscopical anatomy, particularly to osteometry, we used it for the automatic measurement of 8 osteological parameters on a series of 150 human atlases. From these measured parameters, 5 parameters have been directly calculated. The values obtained by image analysis and by measurement with vernier calliper are identical and similar to the data of the literature. The accuracy, the sources of error, and the great advantages of the image analysis method are then discussed.

Calibration↗

Implications of flow cytometry in malignant conditions of the stomach.

UNLABELLED: Aneuploidy, abnormal nuclear DNA content, has been demonstrated in most malignant processes, including gastric malignancies. Utilizing flow cytometry on endoscopic biopsies, we have attempted to characterize the prevalence of aneuploidy and to investigate the prognostic implications of gastric biopsy DNA content with regard to survival. DNA aneuploidy was detected in 71% of specimens revealing malignancy by histologic evaluation. When aneuploidy was demonstrated, 63% of the specimens proved to be positive for malignancy (positive predictive value). However, the absence of aneuploidy had a negative predictive value for malignancy of 93%. Patients with diploid adenocarcinomas had a median survival of 32 months, compared to a median survival of 4 months in the group with DNA aneuploidy. CONCLUSIONS: 1) The prevalence of aneuploidy in endoscopically obtained specimens compares favorably with other previously reported series. 2) The presence of aneuploidy in gastric malignancies appears to correlate with decreased survival and may be helpful in making therapeutic decisions.

Adenocarcinoma↗

Isolated gastric sarcoidosis. Unique remnant of disseminated disease.

Isolated granulomatous disease was identified in a gastrectomy specimen after a gastrointestinal hemorrhage in a 61-year-old white woman. She died postoperatively. No other areas of granulomatous tissue were identified at autopsy. Ten years previously, pulmonary sarcoidosis was proven on lung biopsy. Conceivable, other reported cases of unexplained "idiopathic" granulomatous disease of the stomach may represent residua of prior disseminated sarcoidosis.

Female↗

Renal-hepatic-pancreatic dysplasia: a syndrome reconsidered.

Five infants, three dying neonatally and two later in the first year of life, had renal, hepatic, and pancreatic dysplasia, a combination of abnormalities first described by Ivemark et al [1959]. The renal malformation consisted of cystic dysplasia, with abnormally differentiated ducts, deficient nephron differentiation, and glomerular cysts. The hepatic abnormality consisted of enlarged portal areas containing numerous elongated biliary "profiles," with a tendency to perilobular fibrosis. Serial liver biopsies in one child with cholestasis from birth showed a progression from bile duct paucity at 1 1/2 wk to typical biliary "dysgenesis" at 7 mo. Four of the five children had intrahepatic ductal dilatation, diagnosed ante mortem in the two older children as Caroli disease. The pancreatic abnormality consisted of fibrosis and cysts, with a diminution of parenchymal tissue. The clinical and functional reflection of these abnormalities in the two children surviving the newborn period included renal insufficiency, chronic jaundice, and insulin-dependent diabetes mellitus. Similar renal, hepatic, and pancreatic abnormalities occur in other syndromes, including trisomy 9, Meckel syndrome, Jeune, Saldino-Noonan, and Elejalde types of chondrodysplasia, and glutaric aciduria II. After exclusion of identifiable syndromes, the remaining cases of renal-hepatic-pancreatic dysplasia do not necessarily constitute a homogeneous group.

Abnormalities, Multiple↗

Female external genitalia and müllerian duct derivatives in a 46,XY infant with the smith-lemli-Opitz syndrome.

We report on a 46,XY newborn infant with Smith-Lemli-Opitz (SLO) syndrome with female external genitalia, intraabdominal testes with epididymides and deferent ducts and a normally shaped uterus and vagina. Polydactyly, cleft palate, and several internal organ malformations were also present, and the patient died shortly after birth. Data on six reported male infants with SLO syndrome and female external genitalia suggest a correlation between degree of genital involvement and overall degree of severity. Scoring systems to quantify overall degree of severity (SLO score) and degree of genital involvement in males (genital score) were devised and applied to 122 reported cases from the literature. Statistical analyses showed a unimodal distribution of the SLO severity scores, and positive correlations between the SLO score and the genital score in males, the presence of polydactyly, and the presence of cleft palate. In 19 multiplex families the affected sibs were generally similar in their SLO scores. The above analyses suggest that the wide phenotypic variability in the SLO syndrome is determined by variable expressivity of the same entity as opposed to genetic heterogeneity. The observed phenotypic correlations naturally determine that males with complete feminization are among the more severe patients and tend to have polydactyly and cleft palate.

Abnormalities, Multiple↗

Berry aneurysms, cirrhosis, pulmonary emphysema, and bilateral symmetrical cerebral calcifications: a new syndrome.

Familial idiopathic nonarteriosclerotic cerebral calcification (FINCC) constitutes a rare but pathologically well defined disorder. Thus far, central nervous system symptoms and signs have been the only recorded expression of this disease. Autosomal dominant and autosomal recessive inheritance have both been postulated as cause. We describe three sibs who had symmetrical cerebral calcifications, but three also had cirrhosis and pulmonary emphysema; two had congenital cerebral aneurysms. All were male and of short stature; they also had delayed development and seizures, and two had other neurologic deficits. One sib died at age 3 years of hepatic failure and portal hypertension. Ruptured cerebral aneurysms led to the death of the other two boys at ages 8 and 13 years. The cerebral calcifications symmetrically involved the basal ganglia and thalami, the dentate nucleus, and the cortical and subcortical areas of the cerebrum. The liver was studied by sequential biopsies in two of the children and in all three by autopsy. Fatty degeneration and portal fibrosis preceded a periportal and micronodular cirrhosis. Severe bilateral pulmonary emphysema was present in one sib at age 12 years, whereas all three had bullae and cysts at autopsy. Ruptured left middle cerebral artery aneurysms were demonstrated in two sibs, and one also had aneurysms of the anterior and posterior communicating arteries. We conclude that in this family FINCC is a complex pleiotropic mendelian mutation, either of autosomal or X-linked recessive nature, whose basic pathogenesis remains unknown but may involve a metabolic defect. This form of FINCC may be a previously undescribed syndrome or a form of FINCC in which extraneural manifestations were previously overlooked.

Adolescent↗

High grade dysplasia in Crohn's colitis characterized by flow cytometry.

A patient with Crohn's ileocolitis had high grade dysplasia. Preoperative evaluation of colonoscopic biopsies by flow cytometry demonstrated aneuploidy, a marker of malignancy. In the surgical specimen, however, carcinoma was not demonstrated despite extensive sampling. The detection of abnormal DNA content in this premalignant lesion suggests a useful role for this technique in selecting those patients with inflammatory bowel disease at risk for carcinoma.

Aneuploidy↗

Hemolytic uremic syndrome associated with cisplatin therapy.

An adolescent with a small round cell tumor of the chest wall, who was treated with cisplatin, developed hemolytic uremic syndrome with severe hypertension, which ultimately contributed to her death. Cisplatin's role as a possible causative agent of this syndrome is discussed. Recommendations are made for monitoring abnormalities that may signal the onset of this potential complication.

Adolescent↗

Renal cell carcinoma in patients with tuberous sclerosis.

An adolescent with anemia and weight loss was found to have bilateral renal cell carcinoma (hypernephroma). Further investigation revealed an underlying tuberous sclerosis that had escaped previous clinical detection. Several reports of this association were subsequently found when the world's literature was reviewed. Physicians treating patients with tuberous sclerosis should be aware of the possible development of these renal malignancies in their patients.

Adolescent↗

DNA content in Barrett's esophagus and esophageal malignancy.

Barrett's esophagus is a premalignant condition; endoscopic surveillance is often performed to search for early adenocarcinoma of the esophagus. In an attempt to detect early changes of malignancy, we have added the use of flow cytometry to routine endoscopic surveillance procedures. DNA histograms were generated from biopsy samples by utilizing a specific DNA fluorochrome (4',6-diamidino-2-phenylindole) and flow cytometry. Sixty-three samples from patients with esophagitis, Barrett's esophagus, and esophageal malignancy were analyzed. An abnormal DNA histogram (aneuploidy) was detected in 79% of esophageal malignancies. In addition, aneuploidy was detected in seven patients with Barrett's esophagus, two of whom had dysplasia. DNA quantification with flow cytometry may be a useful adjunct in screening patients with Barrett's esophagus for early malignant change.

Adenocarcinoma↗

The use of an image analyser in human tumour clonogenic assays.

The counting of tumoral colonies growing in semisolid media must be achieved in human tumour clonogenic assays. An image analyser can be advantageously used to overcome the limit of the eye when performing this task. Colonies growing from human cancer cell lines were analysed. Quantitative results on the cytotoxic effect of chemotherapeutic agents were obtained that could not be reached by means of the eye.

Antineoplastic Agents↗