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Biomedical subjects

J L Salomon

Publications and source records attributed to J L Salomon.

At least 19 recordsLinked to original sources

[Routine use of gene amplification for pertussis diagnosis in children].

OBJECTIVES: The resurgence of whooping cough observed in France convinced us to develop a specific PCR assay to detect B. pertussis in nasopharyngeal secretions in parallel of the culture. The aim of our study was to show the value of the PCR in routine diagnosis. MATERIAL AND METHODS: From November 1996 to August 2000, in two hospitals located in the Yvelines (France), the children consulting for a cough compatible with the diagnosis of whooping cough were included in this study. A questionnaire including clinical, biological and radiological items was completed for each one of these patients. A culture of Bordetella and a detection by PCR of B. pertussis were carried out on each nasopharyngeal aspirate. The diagnosis of whooping cough was retained if the detection was positive in PCR and/or culture. RESULTS: Among the 215 investigated children with suspected cases of whooping cough, the diagnosis was positive for 45 (20.9%), of which 39 were less than one year old (median: three months). Sixteen (35.5,%) were positive at the same time for both the PCR and the culture, 26 (57.8%) for only PCR and three (6.7%) for only culture. The PCR was positive in 93.3% of the cases. The results were obtained with an average time of 48 hours. The culture was positive in 61.2% of the cases with an average time of six days. The monthly distribution of the cases of whooping cough was very inhomogeneous and of epidemic appearance. The majority of the cases was located between two periods: 42% between November 1996 and September 1997 and 40% between November 1999 and August 2000. Among the infected children, 15 were less than two months old and were not yet vaccinated; among the 24 others infants, a delay in the vaccine calendar was noted in 50% of the cases. Four children between six and 14 years old were correctly vaccinated. The evolution was favourable in all the children. CONCLUSION: The PCR due to its sensitivity, its specificity and its rapidity offers to the clinician a powerful tool for the diagnosis of whooping cough. Nevertheless, the culture must be associated with the PCR, in order to follow the epidemiology and the sensitivity to antibiotics of B. pertussis.

Adolescent↗

Recombinant erythropoietin therapy as an alternative to blood transfusions in infants with hereditary spherocytosis.

INTRODUCTION: In hereditary spherocytosis, erythropoiesis has been described as 'sluggish' during the first months of life. The lack of appropriate erythropoietic response to compensate for increased red cell destruction necessitates blood transfusions in 70-80% of hereditary spherocytosis-affected infants during their first year of life. After this period, less than 30% require regular transfusion support. This transient requirement for transfusion led us to wonder whether anemic hereditary spherocytosis infants, like anemic premature infants, could benefit from recombinant erythropoietin therapy (rHu-Epo). MATERIAL AND METHODS: In 16 hereditary spherocytosis infants (age range 16-119 days) with severe anemia, a compassionate open preliminary study was performed. rHu-Epo treatment (1000 IU/kg/week) was instituted together with iron supplementation. Hemoglobin values and reticulocyte counts were repeatedly assessed. RESULTS: In 13 out of 16 infants, prompt increases in reticulocyte counts were noted after the first week of treatment with 1000 IU/kg/week of rHu-Epo. During treatment with Epo these infants maintained clinically acceptable levels of hemoglobin and did not require blood transfusions. As the infants grew and began to mount an adequate erythropoietic response, the rHu-Epo dose could be tapered and the treatment could be discontinued before the age of nine months. CONCLUSION: Epo treatment in most hereditary spherocytosis infants appears to be effective in the management of anemia and could serve as a valuable alternative to packed RBC transfusions.

Blood Transfusion↗

Human immunodeficiency virus-1 infection in neonates: correlation of plasma and cellular viremia and clinical outcome. French Pediatric Cohort Study Group.

Among human immunodeficiency virus-1 (HIV-1) vertically infected children, two patterns of disease progression have been observed: about 25% develop a severe immunodeficiency within the first 2 years of life; the rest experience a slower progression, like adults. We have assessed infectious viral burden in infected neonates through the French National Prospective Study. Plasma and cell-associated viremia were assayed by endpoint-dilution cultures in samples from 46 infants followed prospectively from birth. Plasma and cell-associated viral burden were found to be significantly higher in rapid progressing infants than in non-progressing infants in the first months of life: before the age of 2 months, between 2 and 4 months of age and by the age of 6 months. Moreover, among the non-progressing children, the infectious viral burden before the age of 4 months was predictive of the viral burden measured after the age of 12 months. In conclusion, this work demonstrates that infectious viral load is a reliable predictive marker for rapid progression to AIDS in infants and could be useful for initiating antiretroviral therapy.

Age Factors↗

Granulomatous cheilitis in two children with sarcoidosis.

Sarcoidosis is rare in children, who usually have only asymptomatic pulmonary involvement. We report granulomatous cheilitis in two children with sarcoidosis. Both children had systemic sarcoidosis with lymph node and pulmonary involvement.

Child↗

[A valve for microsurgical decompression of optic nerve sheets].

The authors describe a valve for optic nerve sheath fenestration. The valve is composed of a silicone tube with one end placed between the two layers of the arachnoïd at the top of the optic nerve head and the other end blocked but laterally fenestrated with two slits, fixed to the sclera with two small silicone wings. The pressure inside the tube can open the two slit openings when greater than ten millimetres of mercury. This valve has been designed in order to obtain a better function for optic nerve sheath fenestration operations.

Humans↗

[Decompression of the optic nerve sheath].

Optic nerve sheath decompression (ONSD) has become a more frequently performed surgical procedure in recent years. We describe the surgical techniques and complications and we detail the indications of ONSD in pseudotumor cerebri, ischemic optic neuropathy due to arteriosclerosis and other etiologies under investigation. With a 2% risk of postoperative blindness, this procedure must be indicated only in case of severe visual loss and performed by surgeon experienced to orbital surgery.

Humans↗

[Hereditary protein S deficiency and recurrent venous thrombosis. Study of a family].

Hereditary protein S deficiency was detected in three subjects belonging to three generations of one single family. The deficiency was heterozygous and was associated with recurrent venous thromboembolism in two of the subjects affected. Plasma analysis by two-dimensional immunoelectrophoresis showed that the protein S fraction bound to C4b-binding protein was quantitatively normal, whereas the free protein S fraction was much reduced. Since it has been well established that only free protein S intervenes in the regulation of blood coagulation, any quantitative deficiency, even moderate, in protein S resulting in redistribution of its free and bound fractions will have major functional repercussions.

Adult↗

[Ciliary disorders of the bronchi in children].

The present study relates to 39 children, 24 boys and 15 girls, aged 1 to 17 (mean age, 6 1/2 years) suffering from chronic airflow obstruction without muco-viscidosis. The search for a ciliary anomaly was motivated either by the coexistence of situs inversus (group I: 12 cases) or by the negative or scanty aetiological history (group II: 27 cases). The investigation of the cilia consists of a study of ciliary motility and an ultrastructural study of biopsy specimens using the electron microscope. Groups I and II were similar as regards severity of disease, assessed by the incidence of bronchiectasis and chronic hypoxia at rest; a higher incidence of neonatal respiratory distress was noted in group I (7 cases against 4 in group II). Ciliary immotility was particularly noted in group I (7 cases) and one case of weak motility in group II. The major ultrastructural anomalies of the axonemal complex were seen as well in both groups I and II; it was noted that all the structures may be totally or partially missing with the exception of the peripheral microtubules. The discussion centred on three points: 1. In the first analysis, the discordance between the existance of major ultrastructural anomalies of the axonemal complex, a priori incompatible with the conservation of ciliary motility on light microscopy; the link seems to lie in the percentage of cilia affected, the existence of ciliary dyskinesia and finally the disorientation of the basal corpuscles. 2. The immunologic abnormalities sometimes associated with a syndrome of ciliary immotility: that is the anomalies of leucokyte migration under the control of microtubular structures in the peri-centriolar region. 3. The practical consequences. Current treatment rests on daily respiratory physiotherapy and antibiotics adapted for cases of superinfection; also there are drugs stimulating ciliary activity perhaps by the effect on their residual motility; some substances carrying ATP and ATP-ase may re-establish ciliary motility anulled by the absence of dynein arms; also the observations of major ultrastructural abnormalities ought to lead to better genetic counselling than at present, where the mode or modes of transmission are uncertain.

Adolescent↗