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Biomedical subjects

A Kessler

Publications and source records attributed to A Kessler.

At least 55 records · Page 3Linked to original sources

Cystic kidneys associated with connective tissue disorders.

Advances in molecular genetics have resulted in the identification of several forms of autosomal dominant polycystic kidney disease (PKD). Cystic kidneys have also been observed in tuberous sclerosis, von Hippel-Lindau syndrome, oro-facial-digital type I syndrome, Hajdu-Cheney syndrome, Ehlers-Danlos syndrome, and an "overlap" connective tissue disorder, and cannot be distinguished by ultrasonography from PKD. We have studied four children with similar cystic kidneys. None had a family history of PKD. One child has osteogenesis imperfecta type IV, two appeared to have a mild Ehlers-Danlos syndrome, and the fourth has inguinal hernias and undescended testes. We speculate that polycystic kidneys may occur in connective tissue dysplasias. We also realize that these may be chance associations with spontaneous mutations for PKD.

Abnormalities, Multiple↗

Corrosive gastritis: sonographic findings in the acute phase and follow-up.

This paper reports our experience with transabdominal ultrasound in both the acute phase and follow-up in a patient with corrosive gastritis. The case presented demonstrates that serial sonography can localise the injury, demonstrate its depth and reveal the presence of peristalsis, thereby reducing the radiation exposure resulting from barium studies.

Acute Disease↗

Overexpression of p53 protein during pancreatitis.

Overexpression of p53 correlates with neoplasia in many cytological specimens. To test the specificity of overexpressed p53 as a tumour marker for the detection of pancreatic cancer, we analysed cytological specimens of pancreatic juice samples from patients with pancreatitis or pancreatic carcinoma (n = 42) for p53 protein overexpression. p53 protein overexpression was found in 59% of patients with pancreatitis and 67% of patients with pancreatic carcinoma. Thus, the assessment of p53 protein overexpression is not useful in the diagnosis of pancreatic cancer. Overexpressed p53 during pancreatitis appears to be wild-type p53. Overexpression of p53 may result from DNA damage occurring during chronic inflammation. It is well established that p53 can induce apoptosis upon DNA damage. Consequently, we found apoptotic cell death in five out of five tested cytological preparations from patients with pancreatitis as well as in one out of one pancreatic carcinoma specimen.

Adenocarcinoma↗

Passive immunity to infection with Yersinia spp. mediated by anti-recombinant V antigen is dependent on polymorphism of V antigen.

The V antigen is a 37-kDa secreted polypeptide encoded on the 70-kb virulence plasmid of pathogenic Yersinia spp. Besides having regulatory functions, it is known to be a virulence factor and a protective antigen. DNA sequencing of the most common serotypes of human pathogenic Yersinia enterocolitica and Y. pseudotuberculosis revealed that two evolutionary distinct types of V antigen exist in Yersinia spp. One type is represented by Y. enterocolitica serotype 08 strains WA, WA-314, and NCTC 10938 (designated LcrV-YenO8); the other type comprises Y. pestis, Y. pseudotuberculosis, and Y. enterocolitica serotypes O3, O9, and O5,27 (LcrV-Yps). A hypervariable region between amino acids 225 and 232 represents the main difference between the two types. By raising monospecific antisera against both types of V antigen (anti-rVO8 and anti-rVO3), we were able to demonstrate that, in general, passive immunization of mice against a challenge with yersiniae was possible with both anti-Y. enterocolitica V antigen sera. However, anti-V antigen serum was protective only if the immunizing V antigen was the same type as the V antigen produced by the infective strain. The failure of the American V antigen type represented by Y. enterocolitica serotype O8 to protect against Yersinia spp. carrying the other V antigen type (LcrV-Yps) could be an explanation for the presence of plague foci in American countries.

Amino Acid Sequence↗

Phosphoinositolglycan-peptides from yeast potently induce metabolic insulin actions in isolated rat adipocytes, cardiomyocytes, and diaphragms.

Polar headgroups of free glycosyl-phosphatidylinositol (GPI) lipids or protein-bound GPI membrane anchors have been shown to exhibit insulin-mimetic activity in different cell types. However, elucidation of the molecular mode of action of these phospho-inositolglycan (PIG) molecules has been hampered by 1) lack of knowledge of their exact structure; 2) variable action profiles; and 3) rather modest effects. In the present study, these problems were circumvented by preparation of PIG-peptides (PIG-P) in sufficient quantity by sequential proteolytic (V8 protease) and lipolytic (phosphatidylinositol-specific phospholipase C) cleavage of the GPI-anchored plasma membrane protein, Gce1p, from the yeast Saccharomyces cerevisiae. The structure of the resulting PIG-P, NH2-Tyr-Cys-Asn-ethanolamine-PO4-6(Man1-2)Man1-2Man1-+ ++6Man1-4GlcNH(2)1-6myo-inositol-1,2-cyclicPO4, was revealed by amino acid analysis and Dionex exchange chromatography of fragments generated enzymatically or chemically from the neutral glycan core and is in accordance with the known consensus structures of yeast GPI anchors. PIG-P stimulated glucose transport and lipogenesis in normal, desensitized and receptor-depleted isolated rat adipocytes, increased glycerol-3-phosphate acyltransferase activity and translocation of the glucose transporter isoform 4, and inhibited isoproterenol-induced lipolysis and protein kinase A activation in adipocytes. Furthermore, PIG-P was found to stimulate glucose transport in isolated rat cardiomyocytes and glycogenesis and glycogen synthase in isolated rat diaphragms. The concentration-dependent effects of the PIG-P reached 70-90% of the maximal insulin activity with EC50-values of 0.5-5 microM. Chemical or enzymic cleavages within the glycan or peptide portion of the PIG-P led to decrease or loss of activity. The data demonstrate that PIG-P exhibits a potent insulin-mimetic activity which covers a broad spectrum of metabolic insulin actions on glucose transport and metabolism.

Adipocytes↗

Effect of oral nifedipine on ocular blood flow in patients with low tension glaucoma.

AIM: To investigate the effect of oral nifedipine on ocular blood flow in patients with low tension glaucoma (LTG). METHODS: In this prospective study we examined the effects of 3 weeks of treatment with oral nifedipine 30 mg/day in 11 patients with LTG, by using colour Doppler ultrasound imaging to measure haemodynamic variables in the central retinal (CRA), short posterior ciliary (SPCA), and ophthalmic (OA) arteries. Intraocular pressure (IOP) and blood pressures were also evaluated. RESULTS: Nifedipine failed to alter IOP nor did it change peak systolic velocity, end diastolic velocity,or the resistance index in any of the three ocular vessels studied (p > 0.05). However systolic and diastolic systemic arterial blood pressure measurements varied significantly after nifedipine treatment compared with baseline (p < 0.05). CONCLUSION: Our study failed to demonstrate a significant effect of nifedipine on retrobulbar circulation of patients with LTG.

Administration, Oral↗

[Primary and metastatic hepatic cancer: the surgical option].

Major hepatic resection is the treatment of choice in patients with primary and secondary liver cancer. During a 22-month period 31 men and 27 women (mean age 63 years, range 14-84) with space-occupying hepatic lesions were admitted. All 15 patients with benign lesions were operated, except for 3 in whom a liver abscess was drained percutaneously. Of the 43 with malignant liver lesions, 30 had liver metastasis secondary to colorectal cancer, 15 of whom underwent major, anatomical and nonanatomical, liver resection and 1 had cryoablation of the tumor. 9 had hepatocellular carcinoma, 1 of whom had a 4-segment non-anatomical resection and 1 tumor cryoablation. 2 with metastasis from a neuroendocrine tumor had anatomical resection of liver lobes. Of 2 with liver metastasis secondary to breast cancer, 1 underwent resection. CT portography, intraoperative ultrasonography and intraarterial injection of Lipiodol were found to be very useful in selecting patients for liver resection.

Adolescent↗

A plasmid-borne O-antigen chain length determinant and its relationship to other chain length determinants.

We identify a function-controlling O antigen chain length for a plasmid-borne gene, cldpHS-2, harboured by Flexneri strains of Escherichia coli known to cause reactive arthritis. The predicted amino acid sequence of the gene product is very similar to those of other cld genes and that of fepE, thought to be part of the enterobactin iron uptake system of E. coli. The predicted proteins are compared with rfb-associated chain length determinants as a family of related genes.

Amino Acid Sequence↗

MRI, MRA, and neurodevelopmental outcome following neonatal ECMO.

Cranial magnetic resonance imaging (MRI) of 31 newborn infants treated with venoarterial cardiopulmonary bypass for severe but reversible respiratory failure, revealed major focal parenchymal lesions in 7 of 31 infants (23%) and demonstrated abnormal enlargement of extra-axial and ventricular cerebrospinal fluid spaces in 16 of 31 (51%). No preferential left versus right lateralization of focal injury was observed in conjunction with right common carotid artery and jugular vein ligation. No statistically significant relationships were found between major brain lesions on MRI scans and the clinical characteristics of the pre-extracorporeal membrane oxygenation (ECMO), ECMO, and post-ECMO course. Major focal brain lesions were significantly associated with an asymmetric cerebrovascular response to carotid ligation of the right versus left middle cerebral arteries as detected by magnetic resonance angiography (P < .05). Enlarged cerebrospinal fluid spaces were not significantly related to the presence of parenchymal MRI lesions, but were associated with lower Bayley neurodevelopmental scores for mental (MDI) and psychomotor evaluations (PDI) at 6 and 12 months (P < .05). It is concluded that asymmetries of cerebral vascular adaptation detected by magnetic resonance angiography after ECMO may be associated with major brain lesions revealed by MRI. Thereafter, the presence of enlarged cerebrospinal fluid spaces on MRI is associated with a poor shortterm developmental outcome.

Brain↗

Type 1 tympanoplasty in children.

OBJECTIVE: To identify factors affecting the surgical success rate and reperforation rate in type 1 tympanoplasty. Controversy continues regarding the advisability of this procedure in young children, largely because of the likelihood of recurrent middle ear disease and eustachian tube dysfunction. DESIGN: Retrospective medical record review of a case series. SETTING: Pediatric hospital that serves both as a primary care and referral center. PATIENTS: All private patients younger than 18 years, undergoing type 1 tympanoplasty from 1985 through 1989, for whom at least 6 months' follow-up was available. Two hundred nine tympanoplasties on 183 patients were included; 22 patients were excluded for insufficient follow-up. MAIN OUTCOME MEASURES: Surgical success was defined by confirmation of an intact tympanic membrane at least 6 months postoperatively. Procedures were deemed long-term successes if the tympanic membrane remained free of perforation to the end of follow-up. RESULTS: The overall short-term surgical success rate was 92%, with 87% of ears remaining free of reperforation to the end of follow-up. If the perforation involved the margin, the surgical success and long-term success rates dropped to 86% and 77%, respectively. Although reperforation was more likely in patients younger than 6 years or in those with contralateral otitis media at surgery, even these groups had long-term success rates of 81% and 74%, respectively. CONCLUSIONS: Tympanoplasty may be considered at any age. Even in young children, there is a high likelihood of return to normal function.

Adolescent↗

Salivary gland neoplasms in children: a 10-year survey at the Children's Hospital of Philadelphia.

Fifteen salivary gland tumors were treated at The Children's Hospital of Philadelphia between 1982 and 1991. Eight of these lesions were malignant and 7 were benign. All of the benign tumors were pleomorphic adenomas. For these, superficial parotidectomy or excision of the submandibular gland was the treatment of choice. One child had recurrence 2 years after her initial surgery. Among the malignant lesions, mucoepidermoid carcinoma was diagnosed in 5 children, and acinic cell carcinoma in 3. Six malignant tumors involved the parotid gland, while 2 originated in the submandibular salivary gland. Superficial or total parotidectomy, or excision of the submandibular gland was performed, according to the nature and the location of the lesion. The facial nerve was sacrificed in one patient because of extensive involvement of the nerve. A 2-year survival rate of 100% was achieved, and all the patients were free of disease at the end of the follow-up period. Successful management of salivary gland lesions in children requires a high index of suspicion of possible malignancy and complete surgical removal as the initial treatment. Radiation therapy is recommended in the management of those patients with microscopic residual tumor and/or nodal involvement.

Adenoma, Pleomorphic↗

Total and partial ossicular replacement prostheses in children.

Although ossicular reconstruction in children may carry a risk of failure because of recurrent middle ear disease, the procedure offers the potential for restoring binaural hearing during the school years when it is so critical. Of a series of 45 reconstructions with total and partial ossicular replacement prostheses, 6 were extruded for a surgical success rate of 87%. Of the successful cases, 74% had air-bone gaps of 30 dB or better, and 74% had speech reception thresholds of 30 dB or better on initial postoperative audiogram. Outcomes for these children were comparable with those reported for adults, supporting the value of early reconstruction.

Child↗

Effect of anesthetic gas on middle ear fluid.

Tympanometry was performed before (preoperative) and after (intraoperative) the administration of inhalation anesthesia including nitrous oxide and halothane on 109 children undergoing myringotomy with pressure equalization tube insertion. A total of 213 preoperative tympanograms were compared with their intraoperative counterparts and the presence or absence of middle ear effusion at myringotomy. When preoperative tympanograms were consistent with pneumatized middle ears, intraoperative findings demonstrated a mean middle ear pressure increase of +147 daPa. When preoperative tympanometry suggested middle ear effusion, less than 1% demonstrated intraoperative tympanometric changes and/or findings at surgery that would support anesthesia clearing middle ear effusion. Preoperative tympanometric data were poor predictors of the presence or absence of effusion at myringotomy. The relationship between inhalation anesthetics (i.e., nitrous oxide and halothane) and middle ear fluids, and the reliability of tympanometry to predict middle ear effusion are discussed.

Acoustic Impedance Tests↗

Immunoluminometric assays for the quantification of apolipoproteins A-I, B, C-II, apolipoprotein(a) and lipoprotein(a).

Immunoluminometric assays were developed for apolipoproteins A-I, B, C-II (as the apolipoprotein C-II: apolipoprotein B complex), apolipoprotein(a) and lipoprotein(a). The assays were evaluated clinically and methodologically. The results for apolipoprotein A-I, apolipoprotein B and lipoprotein(a) were compared with those obtained by turbidimetric assays. No comparison was possible for apolipoprotein C-II. Sample predilution was necessary for apolipoprotein A-I, apolipoprotein B, apolipoprotein(a) and lipoprotein(a). Not all antibody combinations gave rise to assays with acceptable recovery rates. This was especially the case for lipoprotein(a). These assays for apolipoprotein(a) and lipoprotein(a) had relatively low detection limit (< 5 mg/l) and measuring ranges up to 800 mg/l (using a 1:10 sample dilution), and were suitable for routine use, especially for longer series. Assay times were all less than 3 h, each assay using the streptavidin-biotin technique and being based on a coated-ball technology. The median intra-assay coefficients of variation for all assays lay between 2.9 and 5.9% in the range of interest, expressed in terms of precision profiles. Inter-assay (im)precision lay between 6.2 and 12.2%, calculated in the accepted way. Correlations between turbidimetric and immunoluminometric assays for apolipoprotein A-I and apolipoprotein B were statistically acceptable, although the correlation coefficients were mediocre (apolipoprotein A-I - r = 0.65, apolipoprotein B - r = 0.83) and the slope of the regression line differed from unity. This was most probably due to standardisation and differences in assay design (one-site competitive versus two-site immunometric assays).(ABSTRACT TRUNCATED AT 250 WORDS)

Apolipoprotein A-I↗