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Biomedical subjects

E Cutz

Publications and source records attributed to E Cutz.

At least 127 records · Page 7Linked to original sources

Surgical management of persistent neonatal hypoglycemia due to islet cell dysplasia.

We have reviewed ten children who underwent surgical therapy for persistent neonatal hypoglycemia over a 5-year period. All had inappropriately high insulin levels in the face of hypoglycemia, and all failed medical management with intravenous glucose, frequent feeds, diazoxide and glucagon. Two groups of five patients each were analysed retrospectively. Group 1 underwent 95% pancreatectomy, leaving a small amount of pancreatic tissue on the duodenum and common bile duct. The only major complication in this group was in one patient with common duct obstruction requiring choledochoduodenostomy. All these children are developing normally, without diabetes, steatorrhea, or recurrent hypoglycemia. Group 2 underwent 85% pancreatectomy, leaving the uncinate process in situ. Two of these children are well. Two required conversion to 95% resection because of recurrent hypoglycemia; one of these required a subsequent total pancreatectomy, at which time the pancreatic remnant had significantly regenerated. The other Group II patient was normoglycemic but died at age 3 from pneumonia. Pathology in nine cases showed islet cell dysplasia; 5 of these also had microadenomatosis. One case had a histologically normal pancreas. We conclude that 95% pancreatectomy is a safe operation with a lower failure rate than less radical resections, and should be used early in the management of this condition.

Female↗

Congenital ascites as a presenting sign of lysosomal storage disease.

Neonatal ascites is usually attributed to hematologic, genitourinary, gastrointestinal tract, or congenital heart disease. When these lesions have been excluded, metabolic storage disorders should be considered in the differential diagnosis. We report eight cases of neonatal ascites associated with different types of lysosomal storage disease: infantile sialidosis, Salla disease, GM1 gangliosidosis, and Gaucher disease. In each case there was a history of sibling of perinatal death resulting from the disease. In three cases the diagnosis of ascites was made in utero by ultrasound examination. These diseases are characterized by excretion in the fetal urine of abnormal catabolic products or by measurement of decreased activity of specific lysosomal hydrolases in cultured amniocytes. Thin-layer chromatography of the oligosaccharides in amniotic fluid may be indicated when a diagnosis of persistent fetal ascites has been established.

Adult↗

Plasma glucose and insulin responses in growing rats fed a total parenteral nutrition diet either intravenously or intragastrically.

The effect of administering either intravenously (group I) or intragastrically (group II) a glucose-amino acid total parenteral nutrition diet over a 12-day period upon plasma glucose and insulin responses was examined in adolescent rats. Infusion of the 25% glucose - 12.2% amino acid diet at a rate of 300 kCal X kg body weight-1 X 24 h-1 supported normal weight gain over the 12-day study period in both intravenously (group I) and intragastrically (group II) alimented rats. Mean plasma glucose levels rose dramatically in both groups by the end of day 1; group I had significantly higher mean plasma insulin levels. By day 3, the group I mean plasma glucose value decreased significantly while the group II mean glucose value remained virtually unchanged. Mean plasma insulin values more than doubled in both groups with the group I level still remaining significantly above the group II level. At days 6 and 12, group I mean plasma glucose levels were significantly below group II while both groups had similar plasma insulin levels. Data from this 12-day intravenous-intragastric alimentation study reveals quite different metabolic responses compared with acute (120-180 min) studies of the enteroinsular axis.

Animals↗

Autonomic mechanisms in the ductus venosus of the lamb.

Morphological and pharmacological studies were performed in the ductus venosus from near-term fetal and neonatal (1 day of age) lambs. Light and electron microscopic analysis demonstrated a concentration of circularly oriented muscle fibers at the junction of the ductus with the portal sinus (the sphincter region). With the use of histochemical methods, adrenergic and cholinergic fibers were visualized in both the sphincter and extrasphincter regions of the ductus. At either site, nerve fibers were confined to the adventitial layer and never formed a plexus. Norepinephrine and transmural electrical stimulation contracted the ductus sphincter in vitro, and their action was abolished by dibenzyline. In contrast, atropine had no effect on the transmural response. Moreover, acetylcholine contracted the vessel inconsistently. In the fetus, both the norepinephrine-induced and the electrically induced contractions increased on raising the O2 tension of the bathing fluid from 17-31 to 520-705 mmHg. In addition, the transmural response increased with advancing gestation. Norepinephrine and transmural stimulation relaxed dibenzyline-treated preparations in which the tone had been raised with indomethacin. Norepinephrine relaxation was antagonized by propranolol. We conclude that the ductus venosus sphincter contains functional adrenergic nerves, causing contraction and relaxation via, respectively, alpha- and beta-adrenoceptors. The alpha-adrenoceptor-mediated contraction may have a role in postnatal closure of the vessel.

Acetylcholine↗

Ultrastructure of carotid bodies in sudden infant death syndrome.

Recent studies have implicated an abnormality in carotid body structure and function in the pathogenesis of sudden infant death syndrome (SIDS). In the present investigation, the light and electron microscopic findings in carotid bodies from ten victims of SIDS were compared with those in six control infants and five infants dying of congenital heart disease. The cross-sectional area of carotid body chemoreceptor cells and the frequency, distribution, and size of neurosecretory granules were assessed morphometrically. The area of carotid body occupied by chemoreceptor cells (the functional area) was comparable in SIDS victims, control infants, and infants with congenital heart disease. By electron microscopy, the carotid body chief cells from all groups contained numerous electron-dense neurosecretory granules. Distribution, frequency, and size of neurosecretory granules in SIDS victims and control infants did not differ significantly. Morphology of carotid bodies from SIDS victims was found to be normal. The presence of neurosecretory granules in chemoreceptor cells of SIDS victims suggests that the cellular mechanism of neurotransmitter synthesis and storage is not altered.

Carotid Body↗

Biliary disease in metachromatic leukodystrophy.

This paper illustrates the previously unreported sonographic changes in the biliary tract in metachromatic leukodystrophy (MLD). Gallbladder wall thickening due to sulfatide deposition, intraluminal globules of sulfatide or papillomatosis may cause symptoms referrable to the biliary tract in these patients. A series of patients whom we have studied is briefly alluded to in order to bring to the attention of the radiologist MLD as a rare cause of biliary disease in children.

Adolescent↗

Pancreatic changes in cystic fibrosis: CT and sonographic appearances.

The computed tomographic (CT) and sonographic appearances of the late stages of pancreatic damage in three patients with cystic fibrosis are illustrated. All three had severe exocrine pancreatic insufficiency with steatorrhea. In two patients CT revealed complete fatty replacement of the entire pancreas. In the third, increased echogenicity of the pancreas on sonography and the inhomogeneous attenuation on CT were interpreted as being the result of a combination of fibrosis, fatty replacement, calcification, and probable cyst formation.

Adolescent↗

Bombesin-like immunoreactivity in developing human lung.

Bombesin-like immunoreactivity (BLI) was detected by a specific radioimmunoassay in extracts of 15 human lungs from fetuses, neonates, infants and children. A higher concentration of lung BLI was found in the fetal/neonatal group (2053.2 +/- 316.3 ng bombesin/g protein, n = 6) compared to the infant/children group (416.3 +/- 64.3 ng/g, n = 9). The peaking of BLI in lungs during the fetal/neonatal period suggests an important function for this peptide in intrauterine life and neonatal adaptation.

Bombesin↗

Neuroendocrine cells of the lung. An overview of morphologic characteristics and development.

The detailed morphology of pulmonary neuroendocrine (NE) cells has been defined only during the last decade. The purpose of this paper is to review the main morphologic features of the NE cells, to review the methods and techniques used for their identification, and to discuss the development and functional significance of these cells. The main emphasis is on NE cells in human lung, but where appropriate, studies in animal lungs are also included. NE cells are present in the airway epithelium of human and various animal species and occur singly as well as in clusters called neuroepithelial bodies (NEB). The general cytochemical features (common to both single NE cells and NEB) include cytoplasmic argyrophilia, fluorogenic amine content, positive staining with lead-hematoxylin, and masked metachromasia. These staining properties are similar to those found in APUD cells scattered in various tissues. More specific cell markers are immunoreactivity to peptide hormones (bombesin, calcitonin, leu-enkephalin) identified so far in NE cells of human lung, and immunoreactivity to serotonin found in both human and animal lungs. At the ultrastructural level, NE cells are characterized by the presence of cytoplasmic dense core granules (90-150 nm in diameter), which are considered the storage site of amine and peptide hormones. The distinctive feature of NEB, not found with single NE cells, is the presence of nonmyelinated nerve endings in contact with granulated cells, and positive staining for acetylcholinesterase. The single NE cells are scattered throughout the tracheobronchial epithelium, whereas NEB are found only within the intrapulmonary airways. In postnatal lungs, both the single NE cells and NEB appear concentrated in small peripheral airways. In developing human lung, the first NE cells appear at 8 weeks' gestation, when all other epithelial cells are still undifferentiated. The development and cytodifferentiation of NE cells progresses in a centrifugal direction. By the end of the glandular period, single and groups of NE cells are found along the entire length of primitive bronchial epithelium. Based on differences in the size and morphology of cytoplasmic granules, three distinct types of NE cells can be recognized. During terminal stages of development, NE cells appear in small peripheral airways and primitive saccules. The functional considerations include the possible role of NE cells as endocrine, paracrine, or receptosecretory cells involved in neurohormonal regulation of pulmonary vascular or bronchial responses, and possible function of NEB as intrapulmonary hypoxia-sensitive chemoreceptors.

APUD Cells↗

Neuro-epithelial bodies in organ cultures of fetal rabbit lungs. Ultrastructural characteristics and effects of drugs.

Lung explants from fetal rabbit at the late glandular stage of development (20 days' gestation) and near term (31 days' gestation) were maintained in organ culture for up to 22 days. They were studied by light and electron microscopy to determine whether neuro-epithelial bodies (NEB) of the lung retain structural integrity in vitro. Cultured NEB retained argyrophilia and specific amine fluorescence after formaldehyde condensation. Their ultrastructural morphology showed some differences from that of uncultured NEB: the terminal axons had degenerated and the secretory granules (dense-core vesicles, DCV) were slightly larger, more pleomorphic, more electron-dense, and redistributed throughout the cytoplasm rather than being confined chiefly to the basal regions. These changes, together with hypertrophy of Golgi zones, suggest increased synthesis and storage of secretory products in the DCV during culture. In NEB from near-term explants cultured for 7 days and incubated with reserpine, the core of DCV decreased in size and electron-density and became finely granular, a sign of amine release. Ca++ ionophore No. A-23187, also, induced changes in the ultrastructure of DCV, suggesting that the secretory process in lung neuro-endocrine cells, as in other secretory cells, is Ca++-dependent.

Animals↗

Lingeous conjunctivitis with tracheal obstruction. A case report, with light and electron microscopy findings.

A white male infant of 1 year had unilateral membranous conjunctivitis and severe laryngotracheobronchitis which required tracheostomy. Cultures from eye and throat swabs and of fluid suctioned through the tracheostomy grew many organisms, including H. influenzae, adenovirus type 3, and Candida species, but he had no specific immunologic disturbance. Ligneous conjunctivitis was diagnosed. The infant's general condition responded slowly to intensive therapy but the membrane continued to slough off the regrow. The excised membrane contained massive subepithelial deposits of eosinophilic material and a moderately vascular chronic inflammatory-cell infiltrate with numerous mast cells in the perivascular spaces and the hyaline membrane. The conjunctivitis cleared when treated with topical sodium cromoglycate (Intal), a known inhibitor of mediator release from mast-cell granules. The success of Intal therapy in this case supports the theory that mast cells are involved in the pathogenesis of ligneous conjunctivitis.

Anti-Bacterial Agents↗