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Biomedical subjects

E Cutz

Publications and source records attributed to E Cutz.

At least 91 records · Page 5Linked to original sources
PubMed1993

Localization of cholecystokinin-like and calcitonin-like peptides in infant carotid bodies: a light- and electron-microscopic immunohistochemical study.

Previous immunohistochemical studies have identified several regulatory peptides in the carotid body chief cells in both humans and animals. These peptides, together with amines, may be important in the modulation of the chemoreflex by the carotid body. We report the localization and distribution of calcitonin and cholecystokinin-like (CCK) immunoreactivity in chief cells of human infant carotid body by light- and electron-microscopic immunohistochemical techniques. Consecutive sections immunostained with calcitonin and/or CCK antibodies revealed positively stained chief cells, both alone and in clusters, scattered throughout the carotid body lobule. Generally more chief cells were positive for calcitonin than for CCK. This was confirmed by quantitative analysis showing that the ratio of calcitonin to CCK immunoreactive cells was consistently > 2:1 in all cases studied. There was no apparent correlation between the immunoreactivity for the two peptides and the age, sex, or postmortem interval. Calcitonin-like and CCK-like immunoreactivities were localized electron-microscopically over the dense core granules of the chief cells. Calcitonin and CCK-like peptides in carotid body chief cells may act as neurotransmitters or neuromodulators involved in chemoreception.

Calcitonin↗
PubMed1993

Localization of MOC-1 cell surface antigen in small-cell lung carcinoma cell lines: an immunohistochemical and immunoelectron microscopic study.

Expression of cell surface antigens of the neural cell adhesion molecule (N-CAM) class was recently shown to be shared by both fetal and neoplastic neuroendocrine cells, including those of the lung. We investigated the expression and localization of MOC-1 antigen on small-cell (neuroendocrine) lung carcinoma cell lines with immunohistochemical methods at the light (LM) and electron microscopy (EM) level and by Western blot. At LM level, using monoclonal antibody (MAb) MOC-1 with the ABC method and immunofluorescence, positive staining was observed on surfaces of cells from all tumor lines examined. Strongest immunostaining was found on cell surfaces of pulmonary small-cell carcinoma-derived cell line NCI-H69 with the majority of cells showing positive staining. An adherent variant of NCI-H69 cell line, H69V, exhibited positive staining in about 60% of cells, whereas only occasional cells of NCI-H727 cell line derived from pulmonary carcinoid tumor were positive for MOC-1 antigen. Western blot analysis confirmed these findings, showing a strong MOC-1-specific band in cell extracts of NCI-H69, with weaker band densities for H69V and NCI-H727. Immunoelectron microscopy (IEM) revealed that MOC-1 was not uniformly distributed on the outer surface of plasma membrane; immunogold particles appeared concentrated in areas of thick cell surface "fuzz" coating, surface microvilli, and in areas of cell-cell contact. In some cells, areas of plasma membrane invaginations and a few intracytoplasmic vesicles were also labeled, suggesting endocytosis. Surface labeling for SEM confirmed the finding of more dense labeling over the microvilli, cell membrane folds, and in areas of cell-cell contact. The cell lines derived from pulmonary neuroendocrine cell tumors can provide a useful model to study the role and function of neural adhesion molecules in pulmonary neoplasia and during lung development.

Animals↗
PubMed1993

Hepatic morphology and iron quantitation in perinatal hemochromatosis. Comparison with a large perinatal control population, including cases with chronic liver disease.

We compared hepatic morphology, hepatocellular siderosis, extrahepatic parenchymal siderosis, and (by chemical assay of liver and spleen) the amount of elemental iron and copper in 12 cases of perinatal hemochromatosis (PH) with 119 perinatal controls. Controls were subgrouped according to diagnoses based on clinical and autopsy findings; 37 had chronic liver disease, either hepatic fibrosis (17) or cirrhosis (20). Graded semiquantitatively, hepatocellular siderosis varied widely among controls, and some showed more than PH cases. By chemical assay, total hepatic iron in PH cases was not significantly greater than in any control group except the preterm. Therefore, our findings do not support an etiological role for iron in PH. Its distinctive hepatic morphology seems related to onset of liver disease during fetal life, when periportal hepatocytes normally contain hemosiderin (as in 71 of 82 controls without chronic liver disease). Environmental agents (such as hypoxia, virus, drug) that could damage a fetal liver would usually damage other fetal organs as well. They would be unlikely to recur in a subsequent pregnancy and thereby account for PH occurring in siblings. In initiating PH, therefore, putative environmental agents may need to interact with a factor or factors intrinsic to the developing fetal liver.

Chronic Disease↗
PubMed1992

Tissue-specific expression of a rat intestinal mucin-like peptide.

Expression of the gene for a rat intestinal mucin-like peptide (MLP) was studied by Northern-blot analyses of RNA prepared from a panel of rat tissues. Four probes (A-D) were constructed so as to span a 3.5 kb-long cDNA for rat MLP, and used for hybridization. Positive signals were obtained in intestine and colon, whereas lung, liver, stomach, submandibular gland and spleen were negative. The only transcript detected was approx. 9.5 kb in size. No mRNA splice variants were found. Hybridization in situ using probe B1, which corresponds to a cysteine-rich region near the C-terminus of MLP, confirmed that the gene for MLP is expressed by goblet cells of rat intestine and colon.

Animals↗
PubMed1992

Isolation and culture of neuroendocrine cells from fetal rabbit lung using immunomagnetic techniques.

We describe a novel method for the isolation and subsequent culture of pulmonary neuroendocrine cells (PNEC) from normal fetal rabbit lung using immunomagnetic techniques with a monoclonal antibody, MOC-1. This surface antigen has originally been identified on small cell carcinoma of the lung. Our immunohistochemical studies have shown that MOC-1 cross-reacts with PNEC of human and rabbit fetal lungs on frozen sections, and in fixed cultures of rabbit fetal lung. Using a combination of mechanical and enzymatic disaggregation, a single-cell suspension of fetal rabbit lung was obtained. These cells were incubated with MOC-1 conjugated to magnetic beads. PNEC were selectively removed from the heterogeneous mixture using a magnet, giving up to 2-fold enrichment compared with our previously reported method. These cells were maintained in culture in a functional state for up to 7 days. The ability to prepare PNEC from rabbit fetal lung offers an opportunity to develop in vitro models to investigate the physiologic and biochemical properties of these cells, and ultimately it may lead to a better understanding of their function in health and disease.

Animals↗
PubMed1991

Current status of pancreatectomy for persistent idiopathic neonatal hypoglycemia due to islet cell dysplasia.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histopathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases, 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

Female↗
PubMed1991

Cardiac rhabdomyomata and megacystis-microcolon-intestinal hypoperistalsis syndrome.

Multiple cardiac rhabdomyomata were discovered on necropsy tissue review of a previously well child with megacystis-microcolon-intestinal hypoperistalsis syndrome, who died unexpectedly at home at 40 months of age. Multiple cardiac rhabdomyomata occur rarely and have not previously been reported with this syndrome. They are most frequently associated with tuberous sclerosis. The finding of multiple cardiac rhabdomyomata in this patient suggests the possibility that these two rare conditions may be associated. Putative gene loci for tuberous sclerosis have been assigned to the long arms of chromosomes 9 and 11 and it is possible that the cardiac rhabdomyomata seen in this patient are a serendipitous indicator of the location of the megacystis-microcolon-intestinal hypoperistalsis gene.

Chromosome Mapping↗
PubMed1991

Diffuse pulmonary angiomatosis.

A six year old girl with diffuse pulmonary angiomatosis presented with haemoptysis and diffuse interstitial changes with bilateral pleural effusions on the chest radiograph. The lung lesion as seen on biopsy specimens (and confirmed at necropsy) consisted of bloodless, thin walled, endothelium lined channels, affecting the interstitial septae, pleura, bronchi, and adventitia of large vessels. There was no response to oral corticosteroids or a trial of cyclosphamide. This lesion may be an example of an angiogenic disease.

Angiomatosis↗
PubMed1991

Four unusual cases of sudden and unexpected cardiovascular death in infancy and childhood.

Four unusual cases of sudden and unexpected cardiovascular death in infancy and early childhood that were found during a ten-year autopsy review at our institutions are presented. In Case 1, an asymptomatic 2-year-old male, sudden death resulted from an acute myocardial infarct due to occlusion of coronary arteries that had been previously damaged by unsuspected arteritis. Case 2, a previously well 7-month-old male, died from an acute myocardial infarct due to an unsuspected anomalous origin of the coronary arteries from a single, stenotic coronary ostium. Case 3, a previously well 21-month-old black male, died from a splenic sequestration crisis due to undiagnosed sickle cell disease. Case 4, a 2-month-old female with suspected Marfan syndrome, died from a ruptured dissecting aneurysm of a patent ductus arteriosus. Accuracy of diagnosis was of particular importance in the latter two cases because of the possible genetic implications.

Cardiovascular Diseases↗
PubMed1990

Liver disease in neonatal lupus erythematosus.

We report the cases of neonatal lupus erythematosus associated with significant hepatic involvement in three living infants and in one infant who died 3 hours after delivery. The three living infants had neonatal cholestasis as a major component of their clinical findings. Pathologic changes included giant cell transformation, ductal obstruction, and extramedullary hematopoiesis. Liver involvement has been noted incidentally in children with neonatal lupus erythematosus, but it has generally been attributed to hemodynamic compromise as a result of congenital heart block or systemic toxic reactions. We speculate that neonatal hepatitis proceeding to hepatic fibrosis may occur in neonatal lupus erythematosus, analogous to the occurrence of "idiopathic" congenital heart block. The neonatal hepatitis associated with neonatal lupus erythematosus is a form distinguishable from the "idiopathic" group. Liver involvement may be more common than was previously recognized, and prospective studies to look for maternal autoantibodies in idiopathic neonatal liver disease should be undertaken.

Antibodies, Antinuclear↗
PubMed1990

Xanthogranulomatous cholecystitis and cholecystoduodenal fistula formation associated with total parenteral nutrition in a six year old child.

A unique complication of florid xanthogranulomatous cholecystitis with cholecystoduodenal fistula formation is described in a 6 yr old male. The patient, who had a short gut syndrome, had been maintained on lifelong total parenteral nutrition (TPN) following extensive neonatal ischemic bowel necrosis secondary to gastroschisis. Endoscopic duodenal mucosal biopsy demonstrated a granulomatous inflammatory infiltrate surrounding bile casts suggesting the possibility of a fistula between the biliary tract and duodenum. Additional clinical and radiological evidence of a cholecystoduodenal fistula prompted surgical intervention. At laparotomy the gallbladder was firmly bound to the duodenum by dense fibrous adhesions. Histologic examination showed xanthogranulomatous inflammation in association with fragments of bile that were present both within the gallbladder wall and within a fistulous tract in the adjacent connective tissue.

Biliary Fistula↗
PubMed1990

Pathological features of multiple endocrine neoplasia type IIb in childhood.

The features of two patients with multiple endocrine neoplasia type IIb are described. Patient 1, a 9-year-old boy with marfanoid features, presented with chronic constipation and failure to thrive since infancy. Patient 2, a 12-year-old boy with marfanoid features, presented with a five-year history of persistent cervical lymphadenopathy. In patient 1, the myenteric and submucosal nerve plexuses at all levels of the small and large intestines were comprised of diffusely disorganized, hyperplastic, mature ganglion cells and nonmyelinated nerve fibers. Nerve plexus dissection with morphometric analysis showed marked thickening of the myenteric plexus with a quantitative increase in neural tissue. Patient 2 had a submucosal neuroma of the tongue. Both patients had occult medullary thyroid carcinoma, and patient 2 had cervical lymph node metastases. Both neoplasms showed positive staining for cytokeratin, carcinoembryonic antigen, calcitonin, bombesin, chromogranin, serotonin, and Leu 7. Electron microscopy showed membrane-bound, intermediate-sized, dense-core neurosecretory granules in tumor cells. In patient 2, calcitonin-positive amyloid was present with localization of calcitonin by immunoelectron microscopy to cytoplasmic secretory granules and to extracellular amyloid fibrils. These cases illustrate the potential for missed or delayed diagnosis in multiple endocrine neoplasia syndromes.

Carcinoma↗
PubMed1990

The radiologic and endoscopic investigation and etiologic classification of gastritis in children.

In 11 children the endoscopic criteria for gastritis were correlated with radiologic and pathological findings. An etiologic classification is proposed. The diagnoses included gastric involvement by Crohn's disease (five children), eosinophilic gastroenteritis (one), diffuse varioliform gastritis (two), erosive gastritis associated with Campylobacter pylori infection (two) and idiopathic erosive gastritis (one). Eight of the nine double-contrast barium studies and only one of the three single-contrast examinations correlated with the endoscopic and histologic findings. In 10 children the endoscopic results correlated well with the biopsy findings.

Adolescent↗
PubMed1990

Sudden and unexpected death in infancy and childhood due to pulmonary thromboembolism. An autopsy study.

Because massive pulmonary thromboembolism is a rarely described cause of sudden and unexpected death in the pediatric age group, a 50-year retrospective review of autopsy cases at the Hospital for Sick Children, Toronto, Canada, was conducted to determine the local incidence and clinicopathologic features of such patients. Only 8 cases (0.05%) from a total of approximately 17,500 autopsies were found. The ages ranged from 1 month to 13 years and predisposing factors included recent surgery, congenital heart disease, in-dwelling venous catheters, sepsis, an arteriovenous malformation, occult malignancy, and prolonged immobility. This study confirms that pulmonary thromboembolism is a diagnosis that must be considered in cases of sudden and unexpected death in the pediatric age group. Its incidence, however, appears to be extremely low even in a relatively high-risk population.

Adolescent↗
PubMed1990

Lamb ductus venosus: evidence of a cytochrome P-450 mechanism in its contractile tension.

We have recently shown that a cytochrome P-450-based mechanism is important for the generation of contractile tension by the ductus arteriosus and have now examined whether the same mechanism operates in the ductus venosus. Carbon monoxide (CO/O2 ratio, 0.27) and cytochrome P-450 inhibitors [metyrapone; 4-phenylimidazole; 14-isocyano, 15-(methoxymethyleneoxy)-5Z,8Z,11Z- eicosatrienoic acid; alpha-naphthoflavone] were tested in vitro on the ductus venosus sphincter from mature fetal lambs. Each preparation was precontracted with indomethacin (2.8 x 10(-6) M). Carbon monoxide completely relaxed the ductus, and its action was reversed by illumination with monochromatic light. Peak photocontraction occurred at 450 nm. With the exception of alpha-naphthoflavone, all cytochrome P-450 inhibitors were also relaxant agents. Alpha-naphthoflavone (the sole type I inhibitor tested) produced instead a modest contraction that was often transient. Relaxation brought about by both carbon monoxide and drugs was fully reversed by the thromboxane A2 analog 9,11-epithio-11,12-methano-thromboxane A2 and by excess potassium (55 mM). Carbon monoxide was equally effective in the intact ductus and the ductus denuded of endothelium, whereas cytochrome P-450 inhibitors were marginally less effective in the latter preparation. These findings indicate that the ductus venosus sphincter, like the ductus arteriosus, relies on an intramural cytochrome P-450 mechanism to develop its contractile tone. The actual constrictor remains to be characterized in both vessels.

Animals↗
PubMed1989

Distribution and frequency of neuro-epithelial bodies in post-natal rabbit lung: quantitative study with monoclonal antibody against serotonin.

The distribution, frequency and size of neuroepithelial bodies (NEB) were studied in lungs of rabbits during different stages of development (27-day fetus, newborn, 6, 11, 21, 28 and 56 days postnatally). NEB were visualized by immunostaining with monoclonal antibody against serotonin. Detailed quantitation of NEB was performed by use of camera lucida drawings of immunostained serial sections from the same anatomical region, i.e. the lower lobe of the left lung. The total number of NEB was counted and expressed per epithelial length of airway, surface area and volume. The size of NEB defined as surface area as well as the position of NEB in relation to the airway bifurcations was assessed in airways of different sizes. The overall number and size of NEB were found to increase during the immediate perinatal period followed by a sharp decline at 56 days of age. The number of NEB peaked at 6 days postnatally (mean 175.5 NEB/mm3 of airway epithelium) and declined significantly (3.0 NEB/mm3) at 56 days of postnatal age. The size of NEB reached its maximum at 11 days (mean surface area 659.54 microns 2, with the largest NEB measuring 1839.98 microns 2). By 56 days of age, NEB became significantly smaller (mean surface area 177.29 microns 2) consisting of small clusters of cells situated deep within the airway epithelium. At all ages, about half of all NEB (mean 47.6%) were localized within the small peripheral airways with up to 63.9% located at airway bifurcations. These findings indicate that the "functional activity" of NEB may be confined predominantly to the perinatal period. The postulated functions of NEB include those of intrapulmonary hypoxia-sensitive chemoreceptors and/or endocrine-paracrine activity in the lung. Such functions(s) may be important during adaptation to extrauterine life as well as for growth and development of the lung.

Amines↗
PubMed1989

Adherence of bacteria to the intestine in sporadic cases of enteropathogenic Escherichia coli-associated diarrhea in infants and young children: a prospective study.

Intimate adherence of bacteria to duodenal enterocytes was demonstrated in a 12-mo-old child with sporadic diarrhea that was associated with an enteropathogenic Escherichia coli (EPEC) of the serogroup O111:K58. Therefore, a prospective study was initiated to determine if identification of EPEC in stools from sporadic cases of diarrhea of longer than 10 days in duration in children under 24 mo of age correlated with E. coli colonization of the proximal small intestine and with binding of bacteria to intestinal epithelial cells. Colonization was determined by culture of duodenal aspirates and enteroadherence by light- and electron-microscopic evaluation of both duodenal and rectal mucosa. Each EPEC isolate was examined for several previously proposed laboratory markers of virulence including alpha-hemolysin production, agglutination of erythrocytes, cell surface hydrophobicity properties, adherence to HEp-2 cells, and Verotoxin production. Ten sporadic cases of EPEC-associated diarrhea, severe enough to require hospitalization in each instance, were present among 105 patients in whom EPEC were identified in stools. Of the 10 cases, 9 were evaluated in more detail. In contrast to the first case, in the prospective study E. coli were cultured from duodenal aspirates in only 1 patient and enteroadherent organisms were not present on careful review of small bowel (0/9) and rectal (0/7) mucosa. Hemolysin production (9 of 10 EPEC strains), mannose-sensitive hemagglutination (7/10), hydrophobic cell surface properties (0/10), adherence to HEp-2 cells (7/10), and production of Verotoxin (0/10) did not distinguish the one enteroadherent EPEC from the nine EPEC strains in which in vivo enteroadherence was not documented. In this study of sporadic cases of EPEC-associated diarrhea in young children, bacterial colonization of the small bowel and enteroadherence in vivo could not routinely be demonstrated. In addition, those laboratory assays of bacterial virulence that were evaluated did not distinguish the adherent strain from nonadherent EPEC strains.

Bacterial Adhesion↗