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136 results on '"Tondeur, Marianne"'

101. Cerebral palsy: Initial experience with Tc-99m HMPAO SPECT of the brain

Author

Denays, Roger, Tondeur, Marianne, Toppet Hoegars, Véronique, Ham, Hamphrey, Piepsz, Amnon, Spehl, Marianne, Rubinstein, Michael, Noël, Philippe, Denays, Roger, Tondeur, Marianne, Toppet Hoegars, Véronique, Ham, Hamphrey, Piepsz, Amnon, Spehl, Marianne, Rubinstein, Michael, and Noël, Philippe

Abstract

SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1990

111. Malabsorption of liposoluble vitamins in a child with bile acid deficiency

Author

Vanderpas, Jean-Baptiste, Koopman, Berend B.J., Cadranel, Samy, Vandenbergen, Claudine, Rickaert, Fabienne, Quenon, Marianne, Wolthers, Bert B.G., Brauherz, Georges, Vertongen, Françoise, Tondeur, Marianne, Vanderpas, Jean-Baptiste, Koopman, Berend B.J., Cadranel, Samy, Vandenbergen, Claudine, Rickaert, Fabienne, Quenon, Marianne, Wolthers, Bert B.G., Brauherz, Georges, Vertongen, Françoise, and Tondeur, Marianne

Abstract

A male born to first cousins presented at 12 months with hypocalcemic convulsions, rickets, epistaxis due to vitamin K deficiency, and extremely low serum levels of beta-carotene and vitamin A. Liver function was altered moderately (glutamic-oxaloacetic transaminase, 55 U/L; glutamic-pyruvic transaminase, 37 U/L; lactate dehydrogenase, 255 U/L; alkaline phosphatase, 437 U/L). To correct the deficiencies, 8000 IU vitamin D/day, 10,000 IU vitamin A/day, and intramuscular administration of vitamin K1 were required. At 9 years, he presented signs of neuromuscular affection, and the serum vitamin E level (measured for the first time) was extremely low. Classic lipid malabsorption syndromes (abetalipoproteinemia, chronic cholestasis, mucoviscidosis, coeliac disease, Whipple's disease) were excluded by appropriate examinations. Composition of duodenal bile acids was characterized by undetectable levels of cholic acid metabolites, and only chenodeoxycholic acid metabolites were present. Serum total bile acid concentration was normal, with an atypical low cholic acid/chenodeoxycholic acid ratio and abnormal presence of 3β-OH-Δ5-cholenic acid and 6-OH-bile acids. Urinary bile acid composition was also characterized by elevated 6-OH-bile acids. Known enzymopathies of the bile acid synthetic pathway were excluded (cerebrotendinous xanthomatosis, cerebro-hepato-renal syndrome of Zellweger, coprostanic acidemia). Bile acid pool sizes were determined by using stable isotopes: cholic acid pool size [2.90 (N, 32 ± 16) μM/kg] and chenodeoxycholic acid pool size [10.8 (N, 32.6 ± 9.9) μM/kg] were extremely low; fractional turnover rates of both bile acids were in a normal range. Treatment with chenodeoxycholic acid markedly improved the absorption of fat soluble substances and normalized hepatic function. A partial enzyme defect in the metabolism of the steroid nucleus is proposed., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1987

112. Mucocutaneous lymph node syndrome with necrotic pharyngitis

Author

Brion, Luc, Bachelart, D., Szliwowski, Henri, Dickstein, M, Tondeur, Marianne, Heenen, Michel, Courtoy, Michel, Brion, Luc, Bachelart, D., Szliwowski, Henri, Dickstein, M, Tondeur, Marianne, Heenen, Michel, and Courtoy, Michel

Abstract

We report a case of Kawasaki disease in which some unusual symptoms, including petechiae, an urticaria-like eruption, and necrotic pharyngitis, made the diagnosis difficult. No complications were detected during a follow-up period of 15 months. © 1980 Springer-Verlag., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1980

113. Prenatal diagnosis of a pulmonary cyst by ultrasonography

Author

Lebrun, Didier, Goolaerts, J.P. J.P., Tondeur, Marianne, Avni, Efraim, Rocmans, Pierre Arthur, Ardichvili, Daniel, Lebrun, Didier, Goolaerts, J.P. J.P., Tondeur, Marianne, Avni, Efraim, Rocmans, Pierre Arthur, and Ardichvili, Daniel

Abstract

We report a patient in whom a prenatal diagnosis of an intrapulmonary cyst was made by ultrasonography. To our knowledge, no such case has been reported before and prenatal diagnosis permitted prompt management of an asymptomatic neonate. © 1985 Springer-Verlag., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1985

114. Infantile form of sialic acid storage disorder: Clinical, ultrastructural, and biochemical studies in two siblings

Author

Tondeur, Marianne, Libert, Jacques, Vamos, Eszter, Van Hoof, François, Thomas, George G.H., Strecker, Gérard, Tondeur, Marianne, Libert, Jacques, Vamos, Eszter, Van Hoof, François, Thomas, George G.H., and Strecker, Gérard

Abstract

We describe two sibs with coarse facies, hepatosplenomegaly, prominent psychomotor retardation and unexpectedly fair complexion. Ultrastructural studies of conjunctival, skin, bone marrow and liver biopsies from these individuals showed generalized lysosomal storage of polysaccharidelike material, i.e. membrane bound inclusions containing sparse, fibrillo-granular material. Biochemical analyses of urine and cultured fibroblasts from these patients revealed increased levels of free (unbound) sialic acid. The ultrastructural and biochemical findings in these sibs are similar to those previously found in Salla disease, however, the clinical course is much more severe. It is concluded that these children represent a new pathogenetic entity whose basic defect is still to be defined. © 1982 Springer-Verlag., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1982

115. Reproducibility of quantitative 99Tcm-MAG 3 measurements in rats

Author

Tondeur, Marianne, Piepsz, Amnon, Jeghers, Orner, Froideville, J.-L., Ham, Hamphrey, Tondeur, Marianne, Piepsz, Amnon, Jeghers, Orner, Froideville, J.-L., and Ham, Hamphrey

Abstract

Repetitive quantitative renal extraction studies of 99Tcm-TAG 3, performed in nine rats, demonstrated excellent reproducibility of successive measurements. These results are better than with 99Tcm-DTPA, due to the higher renal extraction of the 99Tcm-TAG 3. The % of renal uptake 10 min after the injection was not influenced by the elapsed time between the end of the 99Tcm-MAG 3 preparation and its i.v. administration., SCOPUS: NotDefined.j, info:eu-repo/semantics/published

Published
1989

116. Hypertension intracrânienne bénigne due à une malformation des sinus veineux intracrâniens chez un enfant présentant un syndrome de Marfan

Author

Poster présenté à la 10e réunion de la Société de Neurologie infantile (10: novembre 1987), Schurmans, Thierry, Cordonnier, Monique, Detemmerman, Dominique, Tondeur, Marianne, Monseu, Guy, Balériaux, Danielle, Szliwowski, Henri, Poster présenté à la 10e réunion de la Société de Neurologie infantile (10: novembre 1987), Schurmans, Thierry, Cordonnier, Monique, Detemmerman, Dominique, Tondeur, Marianne, Monseu, Guy, Balériaux, Danielle, and Szliwowski, Henri

Abstract

info:eu-repo/semantics/nonPublished

Published
1987

121. A case of false-positive bone imaging

Author

Tondeur, Marianne, Ham, Hamphrey, Tondeur, Marianne, and Ham, Hamphrey

Abstract

SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1989

122. Unusual ventilation-perfusion patterns in primary lung tuberculosis

Author

Chanoine, Jean-Pierre, Toppet, Michèle, Dab, Isi, Toppet Hoegars, Véronique, Tondeur, Marianne, Ham, Hamphrey, Piepsz, Amnon, Chanoine, Jean-Pierre, Toppet, Michèle, Dab, Isi, Toppet Hoegars, Véronique, Tondeur, Marianne, Ham, Hamphrey, and Piepsz, Amnon

Abstract

We report two cases of primary lung tuberculosis in children with unusual perfusion ventilation scintigraphic patterns. In the first case, a mismatch in the right upper lobe suggests an elective compression of the bronchi by the mediastinal lymph nodes; in the second case, the total absence of ventilation and perfusion of the left lobe at scintigraphy illustrates the discrepancy sometimes encountered between chest x-ray and lung scintigraphy., SCOPUS: ar.j, FLWNA, info:eu-repo/semantics/published

Published
1988

123. La maladie de Farber. Etude anatomo-clinique et ultrastructurale

Author

Dustin, Pierre, Tondeur, Marianne, Jonniaux, G., Vamos-Hurwitz, E., and Pelc, Sabine

Subjects
Généralités
Abstract

The autopsy of a child aged 21 mth who died of Farber's disease is described. Death was the consequence of extensive pulmonary involvement. This is the 14th description of this disease, and the 11th autopsy case. The ultrastructure of a biopsied s.c. nodule and of fragments of thymus, lung and periarticular nodules fixed shortly after death is described. The typical changes of the disease are present, with a particularly severe lung involvement, infiltration of nearly all the thymus with epithelioid cells, and epithelioid nodules in lymphoid tissue of the intestine and appendix. In the spleen, abnormal cells were mainly present in the subcapsular region. The liver was apparently normal. The typical swollen cells contained inclusions, probably of lysosomal origin, with lipidic lamellae, often paired and frequently showing a marked curvature. These lipids which were surrounded by a strongly stained matrix (Thiery's method) were probably of a mucopolysaccharide nature. Some cells, apparently at an advanced stage, contained very large empty vacuoles, the content of which was dissolved. Biochemical analysis comfirmed the presence of a high level of free ceramides in the s.c. nodules. Farber's disease is thus a lysosomal storage disease, characterized by the nodular aspect of most lesions, their location near the articulations and in the respiratory tract. In this case, the thymus and the lymphoid tissue of the intestine were severely infiltrated. The ultrastructure differs from that of most other lipid storage disease, with some resemblance to Gaucher's disease (glucosyl ceramidosis) and Krabbe's disease lactosyl ceramidosis)., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1973

124. MUCOPOLYSACCHARIDOSIS IN A THREE MONTHS OLD INFANT Clinical and Ultrastructural Studies

Author

Tondeur, Marianne, Vamos, Eszter, Cremer, N., Loeb, Helmut, Tondeur, Marianne, Vamos, Eszter, Cremer, N., and Loeb, Helmut

Abstract

A mucopolysaccharidosis is diagnosed in an infant aged 3 months. Clinical and X‐ray data are not typical, while biological studies reveal an increased mucopolysacchariduria affecting both heparan and dermatan sulfate. Study of lysosomal hydrolases in the liver discloses a normal activity of β‐galactosidase. Hepatic ultrastructure shows, in both parenchymal and Kupffer cells, the presence of clear inclusions analoguous to those found in typical mucopolysaccharidoses, although more numerous and generally smaller. Copyright © 1971, Wiley Blackwell. All rights reserved, SCOPUS: ar.j, FLWNA, info:eu-repo/semantics/published

Published
1971

125. Etude ultrastructurelle du foie dans la maladie de morquio

Author

Tondeur, Marianne, Loeb, Helmut, Tondeur, Marianne, and Loeb, Helmut

Abstract

Extract: Liver biopsies from two children with Morquio’s disease were studied by electron microscopy. The diagnosis was based upon typical clinical findings and x-ray examination. Urinary excretion of keratosulfate was abnormally high in both cases. The Kupffer cells contained large relatively electron-lucid inclusions (0.3 to 4.3 μ in diameter) bounded by a single membrane and containing a finely arranged protein-like precipitate. These inclusions occasionally appeared as compact bodies (figs. 2 and 3). Membranous 'myelin-like' round bodies were infrequently seen in the vacuoles. Hepatocytes were usually normal, but some contained a few electron-lucid inclusions that resembled those seen in the Kupffer cells, although they were smaller in size (0.4 to 1.9 μ) (fig. 5). Peribiliary dense bodies were numerous and rarely contained myelin-like figures (fig. 4). In some atypical groups of cells, discontinuous limiting membranes were observed. These cells also contained electron-lucid vacuoles bounded by single membranes of varying sizes (0.3 to 4.0 μ) (fig. 6). The inclusions were very similar to those previously described in different forms of gargoylism, although the site was different. The rupture of some Kupffer and/or parenchymal cell membranes, secondary to technical manipulations, apparently caused the peculiar aspect of the atypical groups of cells. Speculation: This study supplies new evidence linking Morquio’s disease and gargoylism. Some pathogenic mechanisms appear to be similar in these diseases, both of which affect mucopolysaccharides of the liver in a similar way. © International Pediatrics Research Foundation, Inc. 1969. All Rights Reserved., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1969

127. Biochemical and ultrastructural studies in Hurler's syndrome

Author

Loeb, Helmut, Jonniaux, G., Grégoire, Anne, Cremer, N., Dodion, Josée, Tondeur, Marianne, Grégoire, Philippe, Richard, Jacques, Cieters, P., Voet, W., Loeb, Helmut, Jonniaux, G., Grégoire, Anne, Cremer, N., Dodion, Josée, Tondeur, Marianne, Grégoire, Philippe, Richard, Jacques, Cieters, P., and Voet, W.

Abstract

SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1968

128. Gangliosidosis with total hexosaminidase deficiency: Clinical, biochemical and ultrastructural studies and comparison with conventional cases of Tay-Sachs disease

Author

Fontaine, Guy, Grégoire, Anne, Tondeur, Marianne, Jonniaux, G., Farriaux, Jean Pierre, Voet, W., Maillard, E., Loeb, Helmut, Fontaine, Guy, Grégoire, Anne, Tondeur, Marianne, Jonniaux, G., Farriaux, Jean Pierre, Voet, W., Maillard, E., and Loeb, Helmut

Abstract

SCOPUS: ar.j, info:eu-repo/semantics/published

Published
1973

131. CLINICAL, BIOCHEMICAL AND ULTRASTRUCTURAL STUDIES OF AN ATYPICAL FORM OF MUCOPOLYSACCHARIDOSIS

Author

Loeb, Helmut, Tondeur, Marianne, Toppet, Michèle, Cremer, N., Loeb, Helmut, Tondeur, Marianne, Toppet, Michèle, and Cremer, N.

Abstract

Two children, brother and sister, presenting a clinically peculiar form of mucopolysaccharidosis are reported. There exists a discrepancy between clinical and radiological data and biochemical findings. Enzyme study of the liver discloses a striking hyperactivity of the acid /J‐galactosidase. Hepatic ultrastructure differs from that of other previous descriptions of mucopolysaccharidoses, demonstrating a complex storage of lipids and mucopolysaccharides within swollen lysosomes. Copyright © 1969, Wiley Blackwell. All rights reserved, SCOPUS: ar.j, FLWNA, info:eu-repo/semantics/published

Published
1969

132. Muscular injury in a child diagnosed by 99mTc-MDP bone scan

Author

Tondeur, Marianne, Haentjens, Myriam, Piepsz, Amnon, and Ham, Hamphrey R.

Abstract

An 11-year-old boy had bone scanning to rule out an osscous lesion of the right arm. He presented progressive pain and hard swelling of the right arm. His past medical history and general physical examination were unremarkable. He trained for karate. The scan demonstrated considerable muscular uptake in both arms. CPK and CPK MB levels were both abnormally high, suggesting muscle injury. After a 10-day rest period the bone scan returned to normal.

Published
1989
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134. Hemangioblastoma: An Unsuspected Pitfall in PSMA PET/CT.

Author

Gohimont N, Jissendi Tchofo P, Racu ML, Entezari K, Salmon I, Tondeur M, and Ene D

Subjects
Aged, Cerebellar Neoplasms metabolism, Cerebellar Neoplasms pathology, Gene Expression Regulation, Neoplastic, Hemangioblastoma metabolism, Hemangioblastoma pathology, Humans, Male, Antigens, Surface metabolism, Cerebellar Neoplasms diagnostic imaging, Glutamate Carboxypeptidase II metabolism, Hemangioblastoma diagnostic imaging, Positron Emission Tomography Computed Tomography
Abstract

Abstract: Hemangioblastoma (HB) is the most common primary intra-axial posterior fossa tumor in adults and is a benign vascular neoplasm. We report the case of a 73-year-old man suffering from biochemical recurrence of prostate cancer where intense overexpression of prostate-specific membrane antigen (PSMA) was observed in HB in a PSMA PET/CT. Overexpression of PSMA in tumor-associated vascular structures has been proposed as an explanation of PSMA ligand uptake in several nonprostatic tumors. Given the pathological nature of HB, this mechanism may explain the intense overexpression of PSMA observed in present case., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Published
2021
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135. Escaping the correction for body surface area when calculating glomerular filtration rate in children.

Author

Piepsz A, Tondeur M, and Ham H

Subjects
Adolescent, Calibration, Child, Child, Preschool, Humans, Infant, Infant, Newborn, Sensitivity and Specificity, Body Surface Area, Glomerular Filtration Rate
Abstract

Purpose: (51)Cr ethylene diamine tetraacetic acid ((51)Cr EDTA) clearance is nowadays considered as an accurate and reproducible method for measuring glomerular filtration rate (GFR) in children. Normal values in function of age, corrected for body surface area, have been recently updated. However, much criticism has been expressed about the validity of body surface area correction. The aim of the present paper was to present the normal GFR values, not corrected for body surface area, with the associated percentile curves., Methods: For that purpose, the same patients as in the previous paper were selected, namely those with no recent urinary tract infection, having a normal left to right (99m)Tc MAG3 uptake ratio and a normal kidney morphology on the early parenchymal images. A single blood sample method was used for (51)Cr EDTA clearance measurement., Results: Clearance values, not corrected for body surface area, increased progressively up to the adolescence., Conclusion: The percentile curves were determined and allow, for a single patient, to estimate accurately the level of non-corrected clearance and the evolution with time, whatever the age.

Published
2008
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136. Inter-observer reproducibility in reporting on renal drainage in children with hydronephrosis: a large collaborative study.

Author

Tondeur M, De Palma D, Roca I, Piepsz A, and Ham H

Subjects
Child, Europe epidemiology, Female, Humans, Male, Observer Variation, Radiopharmaceuticals, Reproducibility of Results, Sensitivity and Specificity, Hydronephrosis diagnostic imaging, Hydronephrosis epidemiology, Radioisotope Renography statistics & numerical data, Technetium Tc 99m Mertiatide
Abstract

Purpose: The goal of this study was to evaluate the inter-observer reproducibility in reporting on renal drainage obtained during (99m)Tc MAG3 renography in children, when already processed data are offered to the observers., Methods: Because web site facilities were used for communication, 57 observers from five continents participated in the study. Twenty-three renograms, including furosemide stimulation and posterect postmicturition views, covering various patterns of drainage, were submitted to the observers. Images, curves and quantitative parameters were provided. Good or almost good drainage, partial drainage and poor or no drainage were the three possible responses for each kidney., Results: An important bias was observed among the observers, some of them more systematically reporting the drainage as being good, while others had a general tendency to consider the drainage as poor. This resulted in rather poor inter-observer reproducibility, as for more than half of the kidneys, less than 80% of the observers agreed on one of the three responses. Analysis of the individual cases identified some obvious causes of discrepancy: the absence of a clear limit between partial and good or almost good drainage, the fact of including or neglecting the effect of micturition and change of patient's position, the underestimation of drainage in the case of a flat renographic curve, and the difficulties of interpretation in the case of a small, not well functioning kidney., Conclusion: There is an urgent need for better standardisation in estimating the quality of drainage.

Published
2008
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