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Biomedical subjects

L de Parscau

Publications and source records attributed to L de Parscau.

At least 55 records · Page 3Linked to original sources

[Rehospitalization and early discharge in neonatology: retrospective evaluation].

This retrospective study compared discharge summary data in neonates discharged from the Brest Teaching Hospital Neonatology Unit between May 1, 1980 and April 30, 1981 (Period I) or between May 1, 1990 and April 30, 1991 (Period II). Birth weight, gestational age, duration of hospitalization, corrected age at discharge and rehospitalization rate were compared. Among infants with intrauterine growth retardation (IUGR) with or without prematurity, weight at discharge was 2,500 g or less in none of Period I patients (n = 144) versus 67.8% of Period II patients (n = 87). Four Period II infants weighted 2,000 g or less at discharge (1,850, 1,930, 1,960, and 2,000 g). Among premature infants without growth retardation, weight at discharge was 2,500 g or less in 2.2% of Period I infants versus 52.5% of Period II infants (p < 0.0001). Period II infants were not rehospitalized more often or earlier than Period I infants. Early discharge reduces the duration of separation of the child from his or her parents without increasing the rehospitalization rate.

Birth Weight↗

[Etiologic diagnosis of hypoglycemia in children].

When the etiology of an hypoglycemia is not easily recognizable, the diagnosis can be guided by 2 signs: hepatomegaly and ketosis. If an hepatomegaly is present, an abnormality of glycogen metabolism or neoglucogenesis or an hereditary fructose intolerance may be suspected. A hypoketotic hypoglycemia suggests a hyperinsulinism or an impaired fatty acid oxydation. If the liver is normal and ketosis is normal or increased, an abnormality of branched amino acid metabolism or a ketotic hypoglycemia may be evoked.

Child↗

[Hypothalamic dysfunction. 2 cases: the contribution of nuclear magnetic resonance, therapeutic trial of naltrexone].

Two cases of idiopathic hypothalamic dysfunction (one boy and one girl) are reported. Symptoms of hypothalamic dysfunction were noted by the age of 2 years: initial polyphagia and obesity with subsequent anorexia and emaciation were observed in one patient. Thermoregulation and thirst disorders, recurrent accesses of hypernatremia, acrocyanosis and profuse sweating were present. Impaired growth and delayed puberty in one case, and in the other hypogonadism, absence of growth hormone and gonadotrophins release in response to provocative stimuli were observed as well as abnormal thyroid stimulating hormone response to thyrotropin releasing hormone with hyperprolactinemia. Magnetic resonance imaging showed structural lesion in the lateral part of the lentiform nucleus in one case. Treatment with naltrexone, an opiate antagonist, had little if any effect.

Body Height↗

[Biotidinase deficiency: a disease with neurologic and cutaneous expression susceptible to biotin].

The authors report 2 familial cases of biotin deficiency. The first neurological signs appeared at the age of 2 years in a boy. The diagnosis was established in his sister in the neonatal period. A review of 41 published cases summarizes the neurologic signs (seizures, ataxia, hypotonia and later, developmental delay and deafness) and the cutaneous signs (rash, alopecia). An early treatment with biotin cures or prevents the clinical signs of the disease in most cases.

Amidohydrolases↗

[Neurologic manifestations of arterial hypertension in children].

Neurological complications of arterial hypertension are analyzed in 31 children (mean age = 9 years). All patients presented a renal or renovascular disease (acute nephritis + hypoplastic dysplasia , transplantation = 58%) for which malignant hypertension was the first symptom in 16%. The mean +/- SD initial blood pressure was 189 +/- 33/113 +/- 25 mm Hg and was preceded by previous symptoms in 1 patient out of 6. Neurological abnormalities consisted in seizures (48%), acute intracranial hypertension (39%), cranial palsy (23%), coma (19%), hemiplegia/paresia (16%), retinal changes (6%) or aphasia (6%). The EEG was abnormal in 50% of the patients, sometimes showing permanent paroxysmal activity. Neuroradiologic investigations revealed hemorrhagic and/or ischemic lesions in 1/5 patients. On follow-up, hypertension disappeared in 41% of the children; a decrease in renal function was noted in 56% of the patients at the last examination; neurological sequellae were present in 40% (EEG anomalies +/- epilepsy, motor deficit, retinal changes, psychomotor delay, cranial palsy) and 1 patient died. The morbidity of malignant hypertension stresses the importance of early diagnosis and treatment (calcium channel blockers) when its prevention is not possible.

Adolescent↗

[Glycogenoses type 1b and 1c].

In type 1 glycogen storage diseases, glucose-6-phosphatase may be present but associated with impaired transport of glucose-6-phosphate (type 1b) or inorganic phosphate (type 1c) through microsomal membranes. The type 1c is very rare (2 published cases). The more frequent type 1b presents all the clinical manifestations of type 1a and specific signs: recurrent stomatitis, frequent infections, chronic inflammatory bowel disease secondary to neutropenia and neutrophil dysfunction. Glucose-6-phosphatase activity is low when measured on fresh liver tissue, but is restored after detergent treatment. A good metabolic control does not influence neutropenia and its consequences.

Glycogen Storage Disease Type I↗

[Long-term course of hepatic glycogenosis. A retrospective study of 76 cases].

In order to study the long term outcome of hepatic glycogen storage diseases, a national retrospective inquiry gathered 76 patients older than 12 years. In adolescents and adults, hypoglycemia, failure to thrive, pubertal delay, hepatomegaly and metabolic disturbances are major in type I, intermediary in type III and mild in type "VI+IX". Spontaneous improvement of these symptoms is noted in older patients. Beside these classical signs, anemia, high blood pressure, renal failure and persistent hypercholesterolemia were reported in some type I glycogen storage disease and bad school and professional results in type III. The knowledge of these complications should lead to a better management of these patients.

Adolescent↗

[Protein intolerance with lysinuria. Value of orotic aciduria in adjusting treatment with citrulline].

A new case of lysinuric protein intolerance is described in a 14 year-old Maghrebian child who presented with growth failure, vertebral osteoporosis, aversion to proteins and digital hippocratism, rarely described in this disease. Orotic aciduria was studied after a protein load with and without citrulline supplement and during the course of a 11 month-treatment. There was a clear relationship between orotic aciduria, protein intake and citrulline supplementation. Orotic aciduria appears to be very useful to adjust the treatment.

Adolescent↗

High density lipoprotein alterations induced by bezafibrate in healthy male volunteers.

The alterations of HDL structure and metabolism induced by bezafibrate administration were studied in healthy male volunteers. As usually observed in hyperlipaemic patients, bezafibrate induced a decrease of the plasma concentrations of apo B and LDL-cholesterol and an increase of that of HDL-cholesterol. Analysis of HDL by gradient polyacrylamide gel electrophoresis revealed that bezafibrate administration resulted in a change of the particle size distribution likely suggesting a drop of the HDL2/HDL3 ratio. This was accompanied by a 30% enhancement of the plasma concentration of apoprotein A-II, while that of apoprotein A-I remained unchanged. These data suggest an increase of the HDL concentration, preferentially in the HDL3 subfraction. In spite of these HDL alterations, there was no evidence of change in the three stages of the reverse pathway of cholesterol, since bezafibrate did not induce any significant alteration in the in vitro properties of plasma with respect to (a) cholesterol transport from cultured cells, (b) cholesterol esterification, and (c) transfer of cholesteryl esters from HDL to VLDL-LDL.

Adult↗

[Statural growth of Moghrabin children living in France].

In order to compare the statural growth of French and Maghrebian children living in France, the authors measured the height of 1,243 children aged between one month and 16 years. The study showed no significant difference at any age between the two ethnic groups. It is therefore possible, in daily medical practice, to use the same age charts for French and maghrebian children living in France.

Adolescent↗

Abnormal plasma cholesterol metabolism in cigarette smokers.

Plasma cholesterol metabolism was studied in young, nonobese, normolipidemic men with a moderate level of cigarette smoking (24 +/- 5d-1) and in a comparable nonsmoking normal control group. The smokers showed a decreased cholesterol net transport from cell membranes into plasma (P less than 0.001) and a decreased ratio of cholesteryl ester transfer to low and very low density lipoprotein, relative to lecithin:cholesterol acyltransferase (P less than 0.05). Apoprotein E was increased in smokers' plasma (P less than 0.05) whereas apoprotein A-I, the major apoprotein of HDL, was decreased (P less than 0.05). This pattern of abnormalities has been previously observed in several other groups of subjects at increased risk for atherosclerotic vascular disease (diabetics, dysbetalipoproteinemics, and hyperbetalipoproteinemics). These data suggest a deleterious effect of smoking on plasma lipoprotein metabolism significant even in young smokers, which could partly explain the later incidence of atherosclerotic vascular disease in this group.

Adult↗

[Portacaval anastomosis in 2 cases of homozygote familial hypercholesterolemia].

We report the cases of two siblings, both suffering from familial hypercholesterolemia in homozygotic form, followed up before and after portacaval shunt. A total parenteral nutrition failed to decrease plasma cholesterol levels. However, on the basis of clinical data, a portacaval shunt was performed at the age of seven and five and a half. The two patients showed great effectiveness of the shunt with dramatic regression of clinical signs, reduction of plasma cholesterol, without serious complications over a five to six years period after the shunt.

Child, Preschool↗

[Retroperitoneal actinobacteriosis caused by Haemophilus actinomycetemcomitans].

A 15 years old boy was admitted to the hospital for high fever, and a four month history of abdominal pain and weight loss. Clinical examination showed painful swelling of the left lumbar region. A retro peritoneal mass was revealed by tomodensitometry. There was a marked biological inflammatory syndrome without bacteriological evidence of infectious disease. Final diagnosis was performed by surgery showing a big abscess. Bacteriological culture of pus was positive for a Haemophilus actinomycetemcomitans.

Abscess↗

[Postmeningococcal pericarditis in children. A case favoring an immunoallergic process].

A case of pericarditis following meningococcal meningitis in a 19 month old boy is presented. First clinical signs were noted after 5 days of antibiotherapy (Cefotaxime); at that time the meningitis was cured and the bacteriologic studies yielded negative results. Complete clinical recovery was obtained within 24 hours of treatment with prednisone (2 mg/kg), antibiotherapy being stopped 4 days earlier. The pathogenesis of post meningococcal pericarditis is discussed based on the data from the literature. This observation adds support to an immuno-allergic origin of this pericarditis.

Humans↗

[Byler's disease. Ultrastructural study. Apropos of a case in an infant].

A case of familial cholestasis with cirrhosis is described in a 8 months old boy, presenting with hepatosplenomegaly, portal hypertension, dramatic pruritus, and fluctuating icterus of early post-natal onset. Biological data include positive hepatocyte retention test, with mild hepatocyte cytolysis, without patent hepatocyte insufficiency. The discrepancy between the clinical symptoms and a slight elevation of bilirubin partially conjugated, the absence of elevated blood cholesterol, the absence of evidence of antigen or antibody of virus A or B, the marked elevation of blood biliary acid lead to the suspicion of Byler disease. A liver biopsy with ultrastructural study shows a thickening of the ectoplasm, and the presence of microfilament material in the lumen of partially broken villi. Comparisons are made with the 4 other cases of Byler disease with E.M. study documented in the literature.

Bile Acids and Salts↗