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Biomedical subjects

G Teyssier

Publications and source records attributed to G Teyssier.

At least 73 records · Page 4Linked to original sources
PubMed1981

[Mucopolysaccharidosis type VII. Clinical, radiological and biochemical studies in a neonatal case (author's transl)].

Clinical, radiological and biochemical findings are described in a male newborn with type VII mucopolysaccharidosis (betaglucuronidase deficiency). A metabolic storage disease was likely at birth, because of morphological and radiological features and granulated cells in blood and bone marrow. A study of glycosaminoglycans has been performed in urine and various organs post mortem. Enzymatic deficiency was found in serum, leucocytes, skin fibroblasts, liver, spleen and kidneys. Low activities were present in both parents.

Bone and Bones↗
PubMed1979

[Bartter's syndrome: the long term effects of indomethacin on growth (author's transl)].

Six children with Bartter's syndrome aged 6 years 4 months to 13 years 11 months were treated with indomethacin (1.7 to 4.3 mg/kg/day) during 7 to 27 months. A catch up growth was first observed, then growth curve was parallel to the normal. A catch up weight was also observed. The osseous maturation was the faster it was more delayed. These changes were observed despite a partial correction of potassium and plasma renine activity.

Adolescent↗
PubMed1979

[Mucopolysaccharidesis Type VII resulting from beta-glucuronidase deficiency. Report of one family].

The patient is a north african female, fourth born child in a family with consanguinity. Facial dysmorphia, clubfeet, swollen extremities and heel borne ponctuate calcifications are observed soon after birth. beta glucuronidase activity is very low in serum, leukocytes and skin fibroblasts. At 18 months, gorwth and psychomotor development are normal. Flat facies and dorsolumbar cyphosis are striking. There is no clinical sign of storage disease, neither ocular or cytologic (blood, bone-marrow) abnormalities. Squeletal abnormalities are predominant on cervical and lumbar column and pelvis. Urinary excretion of chondroitin 4 sulfate and chondroitin 6 sulfate is increased. A 4 year old sister is affected. Facial dysmorphia, mild squeletal abnormalities are observed. Again, growth is normal and there is no symptom of storage disease. Enzymic expression of parent heterozygotism is marked in serum studies, but less marked in leukocytes and fibroblasts. The last two children are heterozygotes. At the time of a 5th pregnancy, enzymic activity studies of amniotic fluid and amniotic cells have shown that the foetus was an inaffected female. This child was normal at birth.

Abnormalities, Multiple↗
PubMed1978

[The relationship between palmitic acid and stearic acid on the pharyngeal secretions of the newborn (author's transl)].

The authors report on their study of the ratio of palmitic acid to stearic acid (P/S) in the pharyngeal secretions of the newborn before the sixth hour of life. Four groups of infants were studied: 78 infants used as controls who hac no respiratory distress, 36 with hyaline membrane disease, 16 with transitory respiratory distress and 5 with perinatal asphyxia. The aetiological diagnosis of the respiratory distress was based on clinical as well as radiological and biological criteria. The P/S ratio is always less than 3 with hyaline membrane disease and above 3 in asphyxias. In the control group and the group with transitory respiratory distress the ratio ranged between 1.3 and 14 which was evidence of false negative results, probably attributable to treatment with cortisone given to the mother in premature labour in a certain number of cases. Study of the P/S ratio in amniotic fluid and in the pharyngeal secretions of 27 children born by Caesarean operation embodies a contradiction in the results the newborns in whom the ratio in the amniotic fluid was greater than 3 never had respiratory distress although the ratio studied at the same time in the pharyngeal secretions was low and sometimes less than 3.

Amniotic Fluid↗
PubMedDate not supplied

Generation of reactive O2 species in the myocardium of newborn lambs following intrauterine increase in right ventricular pressure.

Fetuses with pulmonary stenosis and constriction of the ductus arteriosus or the recipient twin in the context of a twin-to-twin transfusion syndrome may present with severe right ventricular myocardial dysfunction. Free O2 radicals are known to be increased in hypertrophied adult myocardium secondary to an increase in endocavitary pressure. This study investigates whether products of reactive O2 species generation are abnormally elevated in the myocardium of fetuses with increased right ventricular pressure. Banding of the main pulmonary artery was performed in five fetal lambs at 90 to 100 days of gestation. Three other animals had a sham intervention and were used as controls. Postoperative observation lasted on average 42 days (range 33-49 days). The levels of hydroperoxides were found to be significantly higher in the right ventricle of the stenosed lambs (6.6 +/- 3.5 nmol/mg protein) compared to the left ventricle of the same lambs (0.7 +/- 0.7 nmol/mg protein), and compared to the right (0.12 +/- 0.1 nmol/mg protein) and the left (0.5 +/- 0.8 nmol/mg protein) ventricles of the controls. It is concluded that during fetal life, an increase in right ventricular pressure is associated with a marked accumulation of products of reactive O2 species generation in the right ventricular myocardium.

Animals↗
PubMedDate not supplied

Assessment of fetal pulmonic stenosis by ultrasonography.

This study was designed to determine (1) the value of Doppler echocardiography in depicting the presence of a fetal pulmonary stenosis, (2) its reliability in the assessment of the severity of the lesion, and (3) the usefulness of additional markers from the left side of the heart as criteria of severity. Fourteen pregnant ewes were included in this study (gestational age, 90 to 120 days). Banding of the fetal main pulmonary artery created mild (n = 3), moderate (n = 3), and severe (n = 5) stenosis. Three lambs were sham operated. Intrauterine fetal Doppler echocardiographic data obtained 15 days after surgery were compared with preoperative values. Peak velocities recorded through the band increased linearly from baseline in the groups with mild and moderate stenosis but did not show any further increase in the group with severe stenosis. Compared with the sham-operated group, right ventricular output in the group with stenosis was either similar or reduced significantly. The increase in right ventricular free wall thickness was significantly greater in the groups with stenosis compared with that of the sham-operated group; the correlation with the degree of severity was r = 0.65 and p < 0.05. A A stronger positive correlation was found between the severity of stenosis and aortic valve diameters: r = 0.82 and p < 0.01. The strongest correlation was found for right ventricular/left ventricular outputs (r = 0.92; p < 0.001). Thus Doppler peak velocities through the obstruction can help detect pulmonic stenosis but are not reliable for the assessment of its severity during fetal life. Other ultrasound measurements such as the size of the aortic anulus and especially the ratio of right ventricular/left ventricular output could be used as sensitive markers of the severity of stenosis.

Animals↗
PubMedDate not supplied

Shoshin beriberi: an unusual complication of prolonged parenteral nutrition.

After 4 weeks of total parenteral nutrition (TPN), a 12-yr-old girl exhibited an acute heart failure with high cardiac output, anuria, and severe lactic acidosis. The clinical, hemodynamic, and biological data suggested the diagnosis of shoshin beriberi which was proved by the low erythrocyte transketolase activity with elevated "TPP effect" and by the dramatic improvement of the patient after thiamin administration. Thiamin deficiency and severe neurological disorders have been described during long-term parenteral nutrition. To our knowledge, this is the first report of the cardiovascular complication of this vitamin deprivation in long-term TPN.

Acidosis↗