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Biomedical subjects

M Tondeur

Publications and source records attributed to M Tondeur.

At least 55 records · Page 3Linked to original sources
PubMed1989

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

An 11-year-old boy had bone scanning to rule out an osseous 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.

Arm Injuries↗
PubMed1989

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

Repetitive quantitative renal extraction studies of 99Tcm-MAG 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-MAG 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.

Animals↗
PubMed1989

Brain single photon emission computed tomography in neonates.

This study was designed to rate the clinical value of [123I]iodoamphetamine (IMP) or [99mTc] hexamethyl propylene amine oxyme (HM-PAO) brain single photon emission computed tomography (SPECT) in neonates, especially in those likely to develop cerebral palsy. The results showed that SPECT abnormalities were congruent in most cases with structural lesions demonstrated by ultrasonography. However, mild bilateral ventricular dilatation and bilateral subependymal porencephalic cysts diagnosed by ultrasound were not associated with an abnormal SPECT finding. In contrast, some cortical periventricular and sylvian lesions and all the parasagittal lesions well visualized in SPECT studies were not diagnosed by ultrasound scans. In neonates with subependymal and/or intraventricular hemorrhage the existence of a parenchymal abnormality was only diagnosed by SPECT. These results indicate that [123I]IMP or [99mTc]HM-PAO brain SPECT shows a potential clinical value as the neurodevelopmental outcome is clearly related to the site, the extent, and the number of cerebral lesions. Long-term clinical follow-up is, however, mandatory in order to define which SPECT abnormality is associated with neurologic deficit.

Amphetamines↗
PubMed1989

Regional brain blood flow in congenital dysphasia: studies with technetium-99m HM-PAO SPECT.

Congenital dysphasia is a developmental speech disorder not explained by deafness, phonation disorder, mental retardation, neurologic lesion, or psychiatric disease. The existence of brain lesions has often been postulated but conventional investigations fail to demonstrate any cerebral abnormality. By means of [99mTc]hexamethyl-propyleneamine oxime (HM-PAO) brain single photon emission computed tomography (SPECT) we have studied 14 children suffering from congenital dysphasia. The brain computed tomographic scan was normal in all cases. In two patients with expression impairment the SPECT study demonstrated a hypoperfusion in the inferior frontal convolution of the left hemisphere, involving the Broca's area. In nine of 12 patients with global dysphasia (deficits in both comprehension and expression), SPECT study showed two hypoperfused areas: an abnormality in the left temporoparietal region and a hypoactivity in the upper and middle areas of the right frontal lobe. These results suggest that congenital dysphasia could be due, like acquired aphasia, to specific impairment of the language cerebral areas and that brain SPECT studies with [99mTc]HM-PAO could be useful for a better comprehension of the physiopathology of these disorders.

Aphasia↗
PubMed1988

Unusual ventilation-perfusion patterns in primary lung tuberculosis.

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.

Airway Obstruction↗
PubMed1985

Prenatal diagnosis of a pulmonary cyst by ultrasonography.

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.

Adult↗
PubMed1982

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

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 polysaccharide-like 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.

Bone Marrow↗
PubMed1980

Mucocutaneous lymph node syndrome with necrotic pharyngitis.

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.

Biopsy↗
PubMed1978

Farber's disease as a ceramidosis: clinical, radiological and biochemical aspects.

A case of Farber's disease associated with athyreosis is reported in a Belgian infant born from consanguineous parents. A detailed clinical observation made from the early onset of symptoms until death of the patient at age of 22 months, together with radiological, morphological and biochemical data confirmed the diagnosis of Farber's disease and its specific storage process. Cultured fibroblast studies disclosed an abnormal catabolism of ceramides, presumably related to the deficiency in lysosomal ceramidase. Family history confirms that the disease is inherited as an autosomal recessive trait.

Ceramides↗
PubMed1978

[Mucolipidosis type I. Sialidosis due to alpha-2-6-neuraminidase deficiency with neurological symptoms].

A case close to mucolipidosis I was observed and the description of this mucolipidosis has to be revised since there were initially some confusions with mannosidosis. Three types of abnormalities appear to be important in its distinction from the other types of oligosaccharidoses: presence on the fundus of a cherry-red spot; in the bone marrow, reticulo-histiocytic cells, whose central nucleus is surrounded by multiple optically empty droplets; very elective overload of the Kupffer cells. 3a neurological syndrome with cerebellar ataxia appears in the evolution. However, it is not present in our patient nor the electroencephalogram changes. This latter point does not authorize the definite identification of our case with Spranger and Wiedemann description of mucolipidosis I especially as the neuraminidase deficiencies are certainly heterogenous.

Child, Preschool↗
PubMed1977

The cellular lesions of Farber's disease and their experimental reproduction in tissue culture.

In a case of Farber's disease, lysosomal inclusions were shown to contain lamellar, rectilinear, or curvilinear material. In tissue culture, when fibroblasts from the patient were overloaded with ceramides containing nonhydroxylated fatty acids a lysosomal accumulation of small curvilinear structures identical with those observed at autopsy were seen. These inclusions persisted for several weeks after the cells were replaced in a normal culture medium. Normal fibroblasts overloaded in the same experimental conditions showed identical, although less numerous, lysosomal inclusions, which disappeared rapidly in a ceramide-free culture medium. No inclusions were found after overloading normal or pathologic fibroblasts with ceramides containing hydroxylated fatty acids.

Ceramides↗
PubMed1977

Sialidosis (mucolipidosis I).

The term "sialidosis" is suggested for the deficiency of alpha-neuraminidase activity in peripheral leukocytes and cultured fibroblasts which results in a considerable urinary excretion of sialyl-oligosaccharides. This defect was found in two siblings with a mild form of mucolipidosis I. 12 sialyl-acid rich oligosacharides have been isolated from the urine of the patients. The structure of ten of them has been determined. The studies of the patients show a remarkable variability of the clinical expression of this disease. The two siblings exhibited a progressive reduction of visual acuity, red-green blindness, a bilateral cherry red macular spot, punctate opacities of the lens, and minimal neurological symptoms. Morphologically, vacuolized lymphocytes, refringent inclusions in cultured fibroblasts, numerous cytoplasmatic inclusions containing a fine protein-like reticulum and some osmiophilic granules mainly in Kupffer's cells were found.

Adolescent↗
PubMed1976

The use of conjunctival biopsy and enzyme analysis in tears for the diagnosis of homozygotes and heterozygotes with Fabry disease.

In most inborn errors of metabolism, the detection of heterozygotes is either impossible, or is feasible only on a statistical basis, so that some uncertainty always remains in that diagnosis. Heterozygote detection is by far more imperative in sex-linked diseases than in autosomal recessive disorders. Indeed, female carriers are at risk of having affected children whatever is the genome of their husband. The method we describe realizes the secure detection of heterozygotes in Fabry disease. It combines the ultrastructural examination of conjunctival biopsies and the assay of thermolabile alpha-galactosidase in tears. The technique is harmless and relatively simple: Both biopsy and tear collection were performed at home in most of the subjects.

Adult↗