[The pediatrician, atherosclerosis and hypercholesterolemia].
Explore the source record for details and available documents.
Arteriosclerosis↗
Biomedical subjects
M Bost
Publications and source records attributed to M Bost.
[Roles of iron overload and chelating treatment in Yersinia infectious complicated by major thalassemia].
A study of 3 personal and 32 published cases showed that Yersinia septicaemia in subjects under 21 presents as digestive disorders with fever and occurs in one half of the cases in thalassaemia patients. Among the factors which may be blamed for this predisposition the most important are excessive tissue iron levels and treatment with chelating agents. Iron overload is present in 70% of patients with Yersinia septicaemia. The risk of infection in such subjects requires appropriate therapeutic measures.
Child↗
[Atheromatous lesions of the proximal aorta. Severe complications of homozygous type IIa hypercholesterolemia in children].
Coronary lesions with atheromatous deposits occurring in later childhood characterize homozygous type IIa hypercholesterolaemia and condition the somber prognosis of a disease which affects one subject in a million. However, aortic lesions are constantly found, as shown by routine ultrasonographic and angiographic studies in these children. The walls of the proximal aorta are cardboard-like and thick, the origin of the aorta is narrow and the semilunar aortic valves are thickened. The valvular or supravalvular aortic gradient may be considerable; it is often progressive, but is sometimes stabilized or made regressive by medical treatments combined with plasmapheresis or porto-caval shunt. Aortoplasty or aortic valve replacement being difficult to perform in these patients, more aggressive therapeutic procedures, such as liver or heart transplantation, have been suggested. The last generation cholesterol-lowering drugs seem to offer some hope of success.
Aortic Diseases↗
[Restrictive ostium secundum: a new fetal malformation syndrome].
Echocardiographic examination of a 23 weeks' hydropic fetus disclosed abnormal kinetics of the valve of the foramen ovale. This valve was constantly bulging out, dome-like, into the left atrium, and on TM-mode tracings in transatrial projection the alpha and beta peaks which occur respectively during the opening and closure of the atrioventricular valves throughout fetal life were missing. This anomaly suggested that the ostium secundum was restrictive; the foramen ovale itself was not restrictive. Such abnormal kinetics have not been encountered among 16 other cases of hydrops fetalis of cardiac or other origin, or in a control series of 81 normal fetuses, which clearly shows that the restriction was primitive. At birth, the child presented with aneurysm of the foramen ovale, probably due to the restrictive ostium secundum.
Echocardiography↗
[Value of the immunosorbent agglutination assay (ISAGA) in the early diagnosis of congenital toxoplasmosis].
An IgM immunosorbent agglutination assay (ISAGA) test was evaluated for detection of IgM antibodies in diagnosis of congenital toxoplasma infection, and compared with IgM IFI test. Of 36 sera from infants with congenital infection, the IgM ISAGA test was positive in 19 cases (52%), whereas the IgM IFI test was positive in only 4 cases (11%). Of those sera obtained during the first 90 days of life from the infected infants, 70% were positive in the IgM ISAGA test whereas only 23% in the IgM IFI test. Neonatal sera from 10 infants suspected, but in whom this diagnosis was ruled out, were also evaluated: one false positive was found with the ISAGA test, but without control 15 days later. The ISAGA test is a simple, sensitive and probably highly specific test for diagnosis of congenital toxoplasma infection.
Agglutination Tests↗
[Massive breast involvement in Burkitt's lymphoma].
Massive bilateral breast involvement in the course of a Burkitt's lymphoma is reported as an exceptional occurrence in a 13 year-old girl, in early puberty. It was an extensive form, stage IV, with massive organ involvement but without CNS involvement. Chemotherapy (LMB 84) induced total cure (18 months disease free survival).
Adolescent↗
[Acute chorea, systemic lupus erythematosus and antiphospholipid antibodies. Apropos of a case].
A case of acute chorea in a 10 years old girl complicating a systemic lupus erythematosus associated with antiphospholipid antibodies is reported. The lupus anticoagulant was detected with a coagulation assay and the false serological reaction for syphilis by the RPR test. The child recovered with Prednisone therapy. The place of chorea in the context of neurological complications of SLE and the particularity of its association with anti-phospholipid antibodies are discussed.
Acute Disease↗
[Hydranencephaly and congenital toxoplasmosis. Apropos of 4 cases].
Four cases of congenital toxoplasmosis with hydranencephaly are reported. The anatomic lesions are the consequence of ischemic necrosis and foetal hydrocephalus. The risk of such lesions is highest during the second trimester of pregnancy. The preventive steps against congenital toxoplasmosis are recalled.
Anencephaly↗
[Sleep apnea syndrome combined with an anatomical obstruction of the upper airways].
In patients with sleep apnoea syndrome abnormalities of the upper airways may play a major role in the genesis of apnoea. Three cases are presented which illustrate the part played by hypertrophy of the tonsils in a child and by stenosis of the pharynx in an adult and of the larynx in another adult. All three patients were cured by surgical correction of these abnormalities. The physiopathology of alveolar hypoventilation, present in one of the three patients, is discussed in relation to the effects of pure oxygen inhalation on the patient's ventilation.
Adult↗
Pattern of plasma levels of cortisol, dehydroepiandrosterone and pregnenolone sulphate in normal subjects and in patients with homozygous familial hypercholesterolaemia during ACTH infusion.
The aim of this study was to determine whether patients with homozygous familial hypercholesterolaemia (FH) have impaired adrenal cortical function. Plasma levels of cortisol, dehydroepiandrosterone (DHA), pregnenolone sulphate (PS) and DHA sulphate (DHAS) were measured during and 8 h ACTH infusion in six controls and two patients with homozygous FH. The basal PS levels of both patients and the basal DHA level of one were abnormally low for age and pubertal stage. During ACTH infusion we observed in both patients: (1) a mild impairment of control response after sustained stimulation (P less than 0.002); (2) a clear impairment of PS response (values less than 2 SD of those in controls); (3) a clear impairment of DHA response (values less than 2 SD) until 4 h in the boy who was at pubertal stage 4 and whose response could be compared to controls; no increase at all in the affected girl (pubertal stage 2) at a stage where normal subjects respond with significant increase. These results suggest that patients with FH lack cholesterol for corticosteroid biosynthesis under maximal ACTH stimulation and that mild chronic ACTH stimulation due to a deficit in the cholesterol supply to adrenal cells might increase the conversion of delta 5 to delta 4-steroids. They provide further evidence to support the primordial role of low density lipoprotein (LDL)-cholesterol in adrenal steroidogenesis in vivo.
Adrenal Cortex↗
Long term treatment of male and female precocious puberty by periodic administration of a long-acting preparation of D-Trp6-luteinizing hormone-releasing hormone microcapsules.
The efficacy and safety of a delayed release formulation of the LHRH agonist D-Trp6-LHRH (LHRH-A; im microcapsules) were tested in 16 girls, 0.9-8.8 yr old, and 10 boys, 2.0-10.5 yr old, with precocious puberty. All children had advanced bone age, breast or testis enlargement, and a pubertal LH response to LHRH. Precocious puberty was idiopathic in 19 subjects and secondary to a brain tumor or other central nervous system abnormality in 7. Nine girls and 6 boys had been previously treated unsuccessfully with medroxyprogesterone and/or cyproterone acetate. The microcapsules were made of 2% LHRH-A dispersed in a biocompatible biodegradable polymeric matrix of DL-lactide-coglycolide. Sixty micrograms of LHRH-A/kg BW were given im on days 1 and 21 and thereafter every 4 weeks for 10-27 months. Plasma LHRH-A levels were measured in 13 children by means of a specific RIA. On days 3, 7, 14, and 21, mean concentrations (+/- SEM) were 295 +/- 44, 218 +/- 31, 215 +/- 45, and 224 +/- 39 pg/ml, respectively. In girls, breast enlargement disappeared, and mean uterus size decreased from 44.4 +/- 2.5 to 38.1 +/- 3.1 mm (mean +/- SEM; P less than 0.02) within 6 months. Mean ovary length decreased from 23.0 +/- 1.5 to 16.2 +/- 1.5 mm (P less than 0.01). In boys, mean testis volume decreased from 8.1 +/- 1.2 to 6.7 +/- 1.2 ml (P less than 0.02) within 6 months. In both sexes, growth velocity decreased significantly, and bone maturation was generally reduced. Plasma levels of estradiol or testosterone and FSH levels decreased significantly within 3 weeks. The LH response to LHRH was reduced to normal prepubertal values after 7 weeks. No secondary clinical or biochemical escape occurred. In 1 boy, all biological features of puberty recurred within 1 month after omission of the fifth injection. No side-effects occurred, except for transient vaginal bleeding in girls after the first or second injection. No antibodies to LHRH-A were detected in the patients' sera. This study demonstrates the ability of a delayed release formulation of LHRH-A to achieve stable levels of the drug in plasma for at least 21 days after a single im injection and to suppress pituitary and gonadal secretion and pituitary response to LHRH for as long as 2 yr after therapy. This treatment appears to be more efficient in treating both clinical and biochemical abnormalities than does treatment with inhibitory steroids. Additionally, the method of administration is more practical and ensures better patient compliance.
Antibodies↗
[Treatment of precocious puberty with LH-RH agonists. Multicenter study using D-Trp-6-LH-RH in a programmed-release form].
LH-RH analogs, substituted in position 6 by D-tryptophane, D-serine(tBu), D-leucine or D-alanine induce a strong stimulation of the gonadotrophs, followed by a desensitization of the LH-RH receptors, which leads to a blockade of the gonadotropin secretion and to a hypogonadism. A delayed release preparation of D-Trp-6-LH-RH (Decapeptyl in microcapsules), designed to release the peptide for 28 days after intramuscular injection, was given to 69 girls and 18 boys with precocious puberty. In both, plasma levels of gonadotropins and gonadal steroids were suppressed within 3 weeks, whilst pituitary responses to LH-RH were almost abolished within 7 weeks. A significant improvement of secondary sex characteristics, as well as gonadal size, was obvious within 6 months. Growth velocity was markedly lowered and, more, in most of children, bone maturation was blocked. This study shows that Decapeptyl in microcapsules is more rapidly and more constantly efficient than LH-RH agonists given discontinuously by subcutaneous or intranasal route.
Bone Development↗
[Seriousness of severe acquired aplastic anemia in children. Apropos of 32 cases].
Thirty-two cases of severe aplastic anemia in children less than 16 years of age, observed between 1967 and 1985 in the Rhône-Alpes area were studied retrospectively. A toxic cause was suspected in 7 cases, an hepatitis in 5 cases. Patients presented with 2 of the 3 following cytopenias during the 3 weeks following diagnosis: hemoglobin level below 100 g/l, reticulocyte count lower than 20 G/l, neutrophil polymorphonuclear leukocyte count below 0.5 G/l, platelet count lower than 20 G/l. Whatever the treatment, prognosis was poor: 6 surviving patients (18.7%). Nineteen patients did not undergo bone marrow transplantation: only 2 are surviving after 4 years, one after low dose corticosteroid therapy, the other after antilymphocyte globulins and androgen therapy; 10 of 17 deaths occurred in the first 4 months. Thirteen bone marrow transplantations from sibling donors, of which 10 HLA A, B DR identical and 3 haploidentical allowed for 8 prolonged takes but only 4 survivors of whom 2 lasted for more than 2 years.
Anemia, Aplastic↗
The heterozygous form of 4.1(-) hereditary elliptocytosis [the 4.1(-) trait].
Using clinical, morphological, genetic, and biochemical criteria, we studied ten white and North African families with hereditary elliptocytosis (HE). In four families, elliptocytic individuals displayed a highly significant reduction of band 4.1, which was recorded using two electrophoretic procedures. The 4.1a/4.1b ratio was also significantly reduced, as is usually observed in suspensions enriched in young red cells. This form of HE was invariably associated with the following characteristics: absence of clinical signs; numerous, smooth and well-elongated elliptocytes; dominant transmission; and, when investigated, normal osmotic fragility. Its frequency, among all forms of HE, is about one third as a first estimate, at least in whites and North Africans. In the other six families studied, elliptocytic subjects presented normal 4.1 bands. Again, the 4.1a/4.1b ratio was decreased, reflecting the red cell age-dependent changes in these two components. In three of these families, elliptocytosis was accompanied by clinical signs of variable intensity, and the mode of inheritance could not be unequivocally determined. Therefore, HE with a partially reduced band 4.1 defines a homogeneous variety of HE that can be isolated from other forms of HE. We suggest that it be termed the 4.1 (-) trait, so as to correspond with a previously proposed terminology.
Adolescent↗
[Use of the Heimlich Maneuver on children in the Rhône-Alpes area].
In 27 children suffering from a foreign body in the respiratory tract with asphyxia, the Heimlich manoeuvre, previously described, was successful in saving life of this dramatic situation in all cases, without respiratory complication. While this method is better known by members of first-aid associations than by pediatricians, it may be successfully used in very young children and infants.
Adolescent↗
[Diagnosis and treatment of fetal tachycardia. Apropos of 2 cases].
Fetal echocardiographic studies were performed in 2 patients referred for evaluation of cardiac dysrythmias. Supra-ventricular tachycardia were diagnosed in the 2 patients. Appropriate prenatal treatment can not avoid premature deliveries in one case. Evaluation of in utero cardiac arrhythmias and monitoring of in utero therapy are discussed.
Digoxin↗
[Cranial fasciitis in children. Apropos of a case in a neonate with duramateral involvement].
A case of cranial fasciitis in the skull of a male child is described. At the age of a few weeks was noted a "lump" beneath the scalp of the right parietal region. Physical examination was otherwise normal. Radiographs showed erosion of the skull deep to the palpable mass. At operation, a firm, 2,5 X 2 X 2 cm nodule was found, which had destroyed the underlying bone and invaded the dura matter. This nodule both extra and intracranial was excised en bloc with the surrounding bone. Histological features were those of a fibroblastic proliferation closely resembling nodular fasciitis. The child is well and symptom-free a year after treatment. The authors also include diagnostic, prognostic and histogenetic comments about cranial fasciitis of childhood, new entity described by Lauer and Enzinger in 1980.
Dura Mater↗
[Haemophilus influenzae meningitis in infants. Apropos of 22 recent cases].
In a three years retrospective evaluation, the authors point out an important increase of hemophilus influenzae meningitis. This germ, who seem to be actually the first cause in infants bacterial meningitis, set the problem of his Ampicillin resistance (10-15% of cases). It is therefore necessary to change first treatment of these meningitis. Cefotaxime which has been prescribed to 50% of cases gives satisfactory results.
Amoxicillin↗