[Cardiorespiratory arrest of obstructive origin].
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Biomedical subjects
Publications and source records attributed to D Floret.
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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.
Three cases of fulminating pneumococcal septicemia are reported in children aged respectively 3 months, 21 months and 6 years 1 month. The third patient only have been previously splenectomized for traumatic rupture of the spleen. This patient recovered when the two others with an expected normal spleen died quickly. The fulminating pneumococcal septicemia is characterized by the association of severe infection state, collapse and hemorrhagic syndrome with often gastric bleeding. Fatal outcome is observed in 50 to 70% of cases. Most cases occur in asplenic patients that can be explained by the role of the spleen in the infectious defense. This can be prevented by vaccination or penicillin but failure has been observed with both methods. Normal spleen, as observed in two of our patients seems to be rare. Functional hyposplenism might explain such facts.
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)
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.
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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.
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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.
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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.
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...).
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.
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