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Biomedical subjects

D Floret

Publications and source records attributed to D Floret.

At least 145 records · Page 8Linked to original sources

Pharmacokinetics and clinical evaluation of cefotaxime in children suffering with purulent meningitis.

Seventy-five children with bacterial meningitis were included in a multicentre trial for evaluation of cerebrospinal fluid (CSF) pharmacokinetics and clinical efficacy of cefotaxime. Mean age of patients was 4 years. Causative pathogens were Haemophilus influenzae in 28 patients (37%), Neisseria meningitidis in 27 patients (36%), Streptococcus pneumoniae in 10 patients (13%), group B streptococcus in 2 patients (2%) and unknown in 8 patients. All isolated pathogens were susceptible to cefotaxime. Seven ampicillin-resistant H. influenzae (9.4%) were found. Cefotaxime was 50 mg/kg intravenously, 4 times daily. The duration of treatment ranged from 5 to 22 days (mean: 13.8). Blood and CSF concentrations of cefotaxime were performed in 50 patients 3 h after infusion at day one and seven cefotaxime levels were determinated both by microbiological assay procedure and high pressure liquid chromatography. On day 1, CSF levels ranged from 0.39 to 2.0 mg/l by microbiological assay procedure (median 3.6) and from 0.0 to 17.4 mg/l (median 2.2) for cefotaxime and from 0.0 to 11.5 mg/l (median 2.2) for desacetyl-cefotaxime by HPLC. We observed a decrease in CSF levels of cefotaxime on day 7. They ranged from 0.3 to 7.0 mg/l (median 1.1) by microbiological assay and from 0.0 to 3.3 mg/l (median 0.8) for cefotaxime and from 0.0 to 6.0 mg/l (median 1.0) for desacetyl-cefotaxime by HPLC. On day 1 and day 7, CSF levels determined by microbiological assay and HPLC were correlated as follows: day 1:r = 0.59 (P less than 0.001). All children (100%) were cured and efficacy of cefotaxime was excellent in 72 cases (96%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Acro-coxo-mesomelic dwarfism: a new variety of autosomal recessive dwarfism].

Acro-coxo-mesomelic dwarfism seems to be a new autosomal recessive entity, one compatible with survival. This severe, dysmorphic condition is characterized by shortening of median and distal segments of the limbs without anomalies of the spine. Other malformations are clubhand and foot, short malformed fingers, and reduced articular mobility of elbows and hips with radial and femoral dislocations. Skeletal X-rays show the following: delayed bone age; mesomelic shortening of the limbs with cubitus brevus, radius curvus, and mostly fibula agenesis; severe acromelic deformities with clinodactyly of the IIIrd, IVth, and Vth digits and brachyrhizophalangia of the IInd and Vth digits. Brachymetacarpia is diffuse, with a "squashed candle" appearance. The IInd metacarpals and the proximal phalanx of the Vth digits have a peculiar "butterfly wings" appearance. The toes are shortened with a "drumstick" appearance and phalangeal hypoplasia, mostly of the midphalanges; hip dislocation and dysplasia (coxomelic), with hypoplasia of the femoral head and a coxa vara cylindric neck.

Adolescent↗

[Klinefelter's syndrome associated with precocious puberty due to tumoral secretion of chorionic gonadotropins].

A 8 and a half year-old boy presented with precocious puberty related to a malignant thoracic teratoma. He was also shown to have a Klinefelter syndrome. Precocious puberty related mainly to the liver, intracranial or thoracic tumors is rare. It seems to be exclusively observed in boys. The slight testicular enlargement is the main clinical sign. The contrast between high LH and low FSH levels is the most striking biological data. The diagnosis is proved by plasma HCG, beta-HCG and alpha-foetoprotein determination. Our patient is the third one with Klinefelter syndrome; this this association is certainly not fortuitous.

Child↗

[Cutaneous manifestations of McCune-Albright syndrome: report of a case (author's transl)].

An 11-year-old boy with fibrous dysplasia of bone of the McCune-Albright syndrome type led to a search of the literature for reports of cutaneous signs other than hyperpigmented macules. We have found descriptions of patchy alopecia, verrucous nevi, soft tissue tumours and other cutaneous abnormalities associated with the McCune-Albright syndrome. Our patient presented in addition pili torti, dermal cysts and a pre-auricular appendix which are all signs not previously described in this syndrome. Our observation is in keeping with the hypothesis that there is a congenital ectodermal and mesodermal mal development to account for this polydysplasia.

Alopecia↗

Hyperthyroidism, diabetes mellitus and the congenital rubella syndrome.

A male patient born to a mother who developed rubella during the tenth week of gestation presented a typical congenital rubella syndrome with mental retardation, neuro-sensory deafness, hypoplasia of the dental enamel and chorioretinitis. Hyperthyroidism occurred at the age of 3 10/12 years and was treated successfully with propylthiouracil for 4 years. The course was complicated by premature craniosynostosis and a craniectomy was performed at the age of 7 years. Overt diabetes mellitus developed at 17 years and was well controlled by insulin therapy. Histocompatilibity (HLA) antigens were A2, B8, B40. Diabetes mellitus and thyroid disorders have previously been reported after congenital rubella, and recently after congenital cytomegalovirus infection. Our patient had both endocrinopaties. It is possible that HLA B8 antigens might be responsible for increased susceptibility to rubella infection.

Child, Preschool↗

[Diagnostic value of café-au-lait spots (author's transl)].

Clinical, histological and ultrastructural studies of café-au-lait spots (C. L. S.) are summarized, in order to establish a differential diagnosis between isolated C. L. S. and C. L. S. associated with polymalformative syndromes. The differential of C. L. S. include nevus spilus and Becker's melanosis. Distinctive clinical and histological features usually differentiate these entities. The incidence of C. L. S. in the general population is about 10 p. 100. It is significantly increased in neurofibromatosis (90 to 100 p. 100), Albright's syndrome (35 p. 100), ataxia telangiectasia (20 p. 100), Silver-Russel syndrome (45 p. 100), Watson's syndrome (60 p. 100), Léopard syndrome (38 p. 100) and Fanconi's anemia. An increased incidence of C. L. S. has been reported in tuberous sclerosis. C. L. S. have been observed in isolated patients with bony skeletal and muscular abnormalities, in Westerhof's syndrome, Leschke's syndrome, Cowden's disease, nevoid basal cell carcinoma syndrome, epidermal nevus syndrome, Gaucher's disease, Hunter's disease, Turner syndrome. Several clinical features of C. L. S. (number, size, distribution, family history) allow to distinguish isolated C. L. S. from C. L. S. associated with polymalformative syndromes. However, the distinction between "normal" and "abnormal" is not always clear cut. A study on the cellular and subcellular characterization of C. L. S. in a case of neurofibromatosis, tuberous sclerosis, Albright's syndrome and ataxia telangiectasia is reported. In each case, the number of epidermal melanocytes, the size of melanosomes and their mode of distribution within epidermal keratinocytes were evaluated. The authors emphasize that no specific histological or ultrastructural "marker" can be helpful for the differential diagnosis between the various forms of C. L. S. (C. L. S. of neurofibromatosis, Albright...).

Ataxia Telangiectasia↗

[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↗

[Hypomelanosis of Ito: report of one case (author's transl)].

A case of hypomelanosis of Ito in a 22-month-old North African girl is reported. The family history is negative for skin disease. Whorls of depigmentation are present on the trunk and abdomen. Associated defects include: growth retardation, microcephaly, strabismus, bilateral epicanthus, myopia, hypertelorism and abnormalities of external ear. Associated non-cutaneous abnormalities documented in patients with hypomelanosis of Ito are reviewed.

Child, Preschool↗

[Endoscopical measures of length of intramural ureter in children with primary vesico-ureteral reflux (author's transl)].

153 endoscopical measures of the length of the intra-mural ureter in 81 children over 2 years have enabled us to ascertain that this segment of vesical ureter is significantly shorter in most patients with vesico-ureteral reflux. By contrast with other previous studies, we have not been able to use this examination for establishing the prognosis of the reflux, perhaps by lack of sufficiently prolonged follow-up However these data have been helpful to us in two circumstances and were complementary of the determination of aspect and position of ureteral meatus: a - When the child is operated for au unilateral reflux, a controlateral short intra-mural ureter suggests the value of a bilateral reimplantation to avoid the appearance of a controlateral reflux. b - When the child has an urinary infection without demonstrated reflux, a short intra-mural ureter suggests the value of a repeated cystography.

Child↗

[Glutaric aciduria. 1 new case].

A 4 year old girl with mild mental retardation presented with convulsions, coma and hepatomegaly. She died rapidly. The main biochemical findings were hypoglycaemia, metabolic acidosis, generalised aminoaciduria, elevation of the plasma and urine alpha-amino adipic acid, massive urine excretion of glutaric and glutaconic acids with traces of alpha-hydroxyglutaric acid. The diagnosis of glutaric aciduria was confirmed by the low activity of glutaryl CoA dehydrogenase in liver tissue. This diagnosis should be considered in children with progressive neurological disorders (dystonia, choreoathetosis) and in children with an illness similar to Reye's syndrome.

Amino Acid Metabolism, Inborn Errors↗