Biomedical subjects
Publications and source records attributed to D Kidron.
A large, nonspecific, chronic ulcer was found in the sigmoid colon of a 13-year-old child with neglected, undiagnosed Hirschsprung's disease (HD). There is no known association between HD and colonic ulcers, suggesting that the ulcer was a true stercoraceous ulcer of the colon and not an intrinsic defect of the aganglionotic bowel.
A congenital right diaphragmatic hernia was diagnosed in a full-term newborn who presented with respiratory distress at the age of 10 hours. The patient required respiratory support, and was operated on at age 6 days. During surgery, a central defect of the diaphragm was found. The herniated liver was only partially reducible because of intrathoracic adhesions. The patient died at 10 days of age as a result of persistent fetal circulation. Postmortem pathological examination demonstrated a central diaphragmatic defect and a nonseparable fibrous fusion between the liver and the lung.
Delayed memory impairments and medial temporal-lobe atrophy are considered to be cardinal features of Alzheimer's disease (AD). The goal of the present magnetic resonance (MR) volumetry study was to investigate the relationship between both features. We determined MR-derived estimates of hippocampal and parahippocampal volume in a sample of 27 AD patients and in a group of 26 healthy control subjects (NCs) of comparable age and education. We examined the performance of the two groups on immediate and delayed recall trials of an auditory-verbal list-learning task (CVLT), a visual non-verbal memory task (Visual Reproduction of the WMS-R), and a screening procedure that provides an estimate of overall cognitive functioning (DRS). Volumes of the hippocampus and the parahippocampal gyrus were significantly smaller in AD patients than in NCs. AD patients were impaired in their overall level of cognitive functioning and showed memory deficits under immediate and delayed recall conditions. The association between medial temporal-lobe atrophy and cognitive impairments in AD was found to be highly specific: Hippocampal volume correlated positively with delayed but not immediate recall of the verbal auditory list learning task. In contrast, parahippocampal gyrus volume, specifically in the right hemisphere, was positively related to delayed but not immediate recall of the non-verbal visual memory task. In NCs, there was a trend towards a negative association between hippocampal volumes and delayed verbal recall. Our results suggest that hippocampal and parahippocampal gyrus atrophy in AD are related to distinct aspects of the patients' memory impairments. Our findings have implications for current discussions regarding contributions of the hippocampus and the parahippocampal gyrus to memory in the intact human brain.
The human AML1 gene encodes a heterodimeric transcription factor which plays an important role in mammalian hematopoiesis. Several alternatively spliced AML1 mRNA species were identified, some of which encode short protein products that lack the transactivation domain. When transfected into cells these short isoforms dominantly suppress transactivation mediated by the full length AML1 protein. However, their biological function remains obscure. To investigate the role of these short species in cell proliferation and differentiation we generated embryonic stem (ES) cells overexpressing one of the short isoforms, AML1-d, as well as cells expressing the full length isoforms AML1-b and AML2. The in vitro growth rate and differentiation of the transfected ES cells were unchanged. However, overexpression of AML1-d significantly affected the ES cells' ability to form teratocarcinomas in vivo in syngeneic mice, while a similar overexpression of AML1-b and AML2 had no effect on tumor formation. Histological analysis revealed that the AML1-d derived tumors were poorly differentiated and contained numerous apoptotic cells. These data highlight the pleiotropic effects of AML1 gene products and demonstrate for the first time an in vivo growth regulation function for the short isoform AML1-d.
OBJECTIVE: While clock-drawing tests are commonly used to screen for cognitive impairment in the elderly, little is known about the performance of elderly depressives. METHODS: We compared thirty-three patients with major depression to forty-two Alzheimer's disease and thirty age-matched controls on clock-drawing, copying, and reading. RESULTS: Patients with Alzheimer's disease had significantly lower scores on clock-drawing, copying, and reading than patients with depression or the controls (p < 0.05). Patients with depression did not differ significantly from controls on quantitative scores or qualitative errors. CONCLUSIONS: Clock tests may be useful for identifying depressed patients with underlying dementia.
A 26-year-old woman with twin fetuses of 28 weeks' gestational age had symptoms of preeclampsia and was admitted to the hospital for observation. Nine days later, after reporting a severe headache, the patient experienced loss of vision in both eyes. An emergency computed tomographic brain scan was performed to rule out intracranial hemorrhage, and cesarean delivery was performed. Twelve hours after the operation, the patient's vision improved gradually and returned to normal after 24 hours. The placenta was submitted to pathologic examination, and magnetic resonance imaging was performed 4 days after birth. Recent thrombosis observed in the histologic section of the placenta, ischemic changes in the brain seen in the computed tomographic and magnetic resonance scans, and severe proteinuria manifested clinically suggest vascular endothelial damage as the underlying mechanism in this case of preeclampsia-related transient cortical blindness.
We determined topographic selectivity and diagnostic utility of brain atrophy in probable Alzheimer's disease (AD) and correlations with demographic factors such as age, sex, and education. Computerized imaging analysis techniques were applied to MR images in 32 patients with probable AD and 20 age- and sex-matched normal control subjects using tissue segmentation and three-dimensional surface rendering to obtain individualized lobar volumes, corrected for head size by a residualization technique. Group differences emerged in gray and white matter compartments particularly in parietal and temporal lobes. Logistic regression demonstrated that larger parietal and temporal ventricular CSF compartments and smaller temporal gray matter predicted AD group membership with an area under the receiver operating characteristic curve of 0.92. On multiple regression analysis using age, sex, education, duration, and severity of cognitive decline to predict regional atrophy in the AD subjects, sex consistently entered the model for the frontal, temporal, and parietal ventricular compartments. In the parietal region, for example, sex accounted for 27% of the variance in the parietal CSF compartment and years of education accounted for an additional 15%, with women showing less ventricular enlargement and individuals with more years of education showing more ventricular enlargement in this region. Topographic selectivity of atrophic changes can be detected using quantitative volumetry and can differentiate AD from normal aging. Differential effects of sex and years of education can also be detected by these methods. Quantification of tissue volumes in vulnerable regions offers the potential for monitoring longitudinal change in response to treatment.
A case of severe fetal congestive heart failure due to occlusion of the ductus arteriosus in a mother treated with indomethacin for polyhydramnios and premature contractions is described. Closure of the fetal foramen ovale that escaped detection by prenatal echocardiography was later demonstrated at neonatal autopsy. This case suggests that indomethacin treatment in a ductus-dependent fetus may be hazardous. Therefore, careful surveillance of the fetus exposed to indomethacin in utero is warranted.
Cytogenetic and fluorescent in situ hybridization (FISH) studies were performed on several formalin-fixed tissues obtained from four fetuses diagnosed at amniocentesis as 45,XO-Turner syndrome. Three of the four were phenotypically normal and one had malformations. The three phenotypically normal cases were found to have an additional normal cell line, which may explain their ability to survive, at least to the time of pregnancy termination well into the second trimester. The abnormal 45,XO fetus was not found to be mosaic in all of the tissues examined. In 45,XO cases in which no malformation is detected, the possibility of mosaicism should be raised and thus the counselling should be modified accordingly.
We evaluated the feasibility of retrospective genetic testing for numerical chromosomal aberrations by applying the FISH technique to formalin-fixed fetal tissue. Fetal tissue from 10 old cases with known aneuploidy and from 13 cases with known fetal malformations, were tested with specific DNA probes for pericentromeric repeat regions of chromosomes 13/21, 18, X and Y. FISH diagnosis concurred with karyotype in all nine cases with sufficient cells. Numerical aberration was diagnosed in six out of 13 cases with fetal malformations.
Blast injuries are rare, and although blast-induced perforations of the bowel have been described in the past, the entity of a delayed perforation caused by an evolving injury has not been reported. We report three men injured by the explosion of a terrorist bombing in open air. They suffered primary blast injuries, which resulted in isolated perforations of the terminal ileum. They were operated at different times after the blast event. The resected specimens were examined under light microscopy. One patient was operated immediately, and had three perforations in the terminal ileum. In the other two patients, abdominal complaints appeared only 24 and 48 hours later. These two patients were found to have hematomas in the wall of the terminal ileum, and small perforations therein, with almost no contamination of the peritoneal cavity. On histological examination, there were small perforations with disruption of all intestinal layers. In the vicinity of the perforations, the mucosa was necrotic and disorganized. The submucosa showed edema and vascular thrombi, and at several points mucus was shown dissecting through the muscularis propria, thus creating minute microperforations. Because of the findings in these patients, we suggest a mechanism of evolving damage to the bowel wall and delayed perforation rather than delayed diagnosis, after blast injuries. We suggest that patients exposed to a significant blast should be watched carefully for at least 48 hours.
Prenatal brain hemorrhages are associated with considerable morbidity and mortality in neonates. They appear predominantly as bleeding into periventricular germinal matrix with subsequent hemorrhages into lateral ventricles and subarachnoid space. Other patterns of brain hemorrhage are not widely documented in second trimester fetuses. We report a case of hemorrhage presenting as extensive hemosiderin deposits in leptomeninges and adjacent brain parenchyma, as observed in superficial hemosiderosis. The lesion was associated with substantial tissue damage. Clinically, it was diagnosed during the second half of second trimester by successive ultrasound examinations. If differs from germinal matrix hemorrhage and other forms of hemorrhage in origin, pathogenesis, morphology, and probably in clinical manifestations.
Explore the source record for details and available documents.
The spectrum of the primary antiphospholipid syndrome has expanded in recent years. It has been associated with a number of non-thrombotic syndromes such as pulmonary hypertension, adrenal insufficiency, chorea and avascular necrosis of bone. Yet, it has not been described in association with inflammatory pulmonary disease. We describe a young male with definite primary antiphospholipid syndrome who developed insidious diffuse pulmonary infiltrates. The histopathologic examination of the involved lung demonstrated alveolitis and fibrosis. We suggest that this pulmonary involvement may represent another manifestation of the primary antiphospholipid syndrome.
The aim of this study was to investigate the use of a prosthetic biologic material-lyophilized dura mater (Lyodura) in patching esophageal defects in dogs, having in view its potential use in bridging long gap congenital esophageal atresia and patching acquired esophageal defects. To follow the incorporation process, 20 mongrel dogs had full-thickness, 6 cm2 cervical esophageal defects, patched with Lyodura. The dogs were sacrificed at different postoperative periods and the patched esophagus removed for gross and microscopic examination. In a second group of 10 dogs, a segment of the esophagus was excised and replaced by 3 cm in length and 2 cm in diameter, Lyodura tubes. In this group, prolonged follow-up was undertaken including radiologic, endoscopic, and histological assessment. Special attention was given to the swallowing function and to growth and development. This study shows that: (1) the area of prosthetic replacement was characterized by a narrowing of the esophageal wall and the histopathologic study showed that the epithelialization process was complete in about 2 months; and (2) muscle reconstruction did not take place at the patched areas. We conclude that, pending further studies and improvement, lyophilized dura mater can be considered as a successful alternative for bridging esophageal defects.
Massive hemorrhage from the gastrointestinal tract in a 12-yr-old boy caused by a congenital atypically located colonic arteriovenous malformation is described. Guided and "clean" resection of the involved colon was possible due to preoperative selective angiography, which proved to be the most efficient diagnostic tool. Histologic documentation of this rare pathology in childhood is presented, and the classification and features of the disease are briefly reviewed.
Explore the source record for details and available documents.
The inflammatory response during thermal injury increases the adhesiveness of white blood cells. A direct slide test was used to compare the state of leucocyte adhesiveness/aggregation (LAA) in the peripheral blood of mice subjected to a thermal injury with the findings in control animals. The state of LAA in the peripheral blood increased from baseline values of 1.1 +/- 1.1 per cent to 6.5 +/- 1.3 per cent within 1 h and to 11.0 +/- 1.2 per cent and 14.8 +/- 4 per cent after 3 and 6 h respectively following thermal injury. The respective leucocyte counts were 3075 +/- 277/mm3 (baseline), 3871 +/- 359, 3840 +/- 687 and 6395 +/- 1152 cells/mm3. The LAA values had subsided by 5 days following burning and correlated with the degree of pulmonary leukostasis. Our study suggests that the LAA is an early and sensitive marker of inflammation and that it can be used as a marker for the presence of pulmonary leukostasis during thermal injury.