[Radiological case of the month. Claude-Bernard-Horner syndrome revealing cervicothoracic neuroblastoma].
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Biomedical subjects
Publications and source records attributed to P Vic.
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BACKGROUND: Vertebral compression is rarely the first manifestation of acute leukemia (AL) in children. CASE REPORT: Three children, 13, 4 and 13 years of age respectively, suffered from recent and persistent back pain. The first patient was considered as having psychosomatic problems despite thrombocytopenia at the first examination. The second patient was also pale with hematoma on his legs so that AL could be rapidly recognised. An initial diagnosis of Scheuermann disease was proposed for the third patient in whom hemogram was performed due to worsening of his general condition. The three patients had extended vertebral demineralisation on the first X-rays. CONCLUSION: Causes of back pain in children are often organic at this age of life. They must be searched by suitable examinations.
BACKGROUND: Polycythemia vera is a very rare disease in childhood; its treatment for this reason is not well established. CASE REPORT: An 11-year-old boy was admitted for polycythemia vera. The three classical major criteria for this condition were present and no cause for secondary polyglobulia could be recognized. The patient was given hydroxyurea. Three years later, he is well with hematocrit below 50%. CONCLUSIONS: Oral continuous myelosuppressive therapy with hydroxyurea may be proposed in children if there is no HLA-matched family member. Long-term follow-up is nevertheless necessary in order to detect relapse or side-effects of the treatment.
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Elastic fibers required to maintain bronchial patency during ventilation may be damaged in asthma as a result of repair following inflammation or stretching during exacerbations. Fifteen normal subjects and 40 asthmatics of variable severity were studied. Bronchial biopsies were obtained from a subsegmental bronchus using a flexible bronchoscope. The elastic fibers were examined using orceine-eosine sustaining and/or immunohistochemistry with two monoclonal antibodies against elastin or transmission electron microscopy (six asthmatics and four control subjects). Orceine-eosine staining revealed that most normal subjects had normal fibers throughout the submucosa whereas of the 21 asthmatics analyzed only three had a normal superficial elastin network. In five patients, elastin had virtually disappeared. In the remaining patients, fibers appeared fragmented. The deeper layer of elastic fibers was abnormal in 17 asthmatics, fibers being patchy, tangled, and thickened. The fragmentation of the superficial network of elastic fibers shown in asthmatics was confirmed by immunohistochemistry. Electron microscopy studies suggested that the elastinolytic process and fragmentation of elastic fibers occurred in asthmatics. Elastinolysis occurs in the airways of asthmatics possibly as a result of repair elicited by chronic inflammation. Mechanical stretch induced by breathing and edema may lead to the fragmentation of fibers in asthmatic airways.
BACKGROUND: Gastro-esophageal reflux (GER) is common in chronic bronchopulmonary diseases, and may aggravate them. The aim of this study was to study frequency and characteristics of GER in infants and toddlers with cystic fibrosis (CF). POPULATION AND METHODS: Twenty-five CF patients, 45 to 954 days-old (mean 219 days) without clinical evidence of bronchopulmonary infection, were studied by prolonged pHmetry (19.45 +/- 1.89 hours). Their pulmonary state was evaluated by the number of previous courses of antibiotic treatment and analysis of the X-rays. RESULTS: Nineteen children had GER (76%); four of them had no clinical sign of GER. All 19 patients had mild or moderate signs of pulmonary impairment. The reflux index (percentage of time with pH < 4) was 12.82%. The GER episodes were more frequent during walking time (p < 0.001), but longer during sleeping time (p < 0.001). CONCLUSIONS: GER is frequent in CF patients, even the youngest. It seems primary, and not related to pulmonary impairment. Its frequency and its potential severity lead to suggesting a pHmetry to every new CF patient.
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Platelet-derived growth factor (PDGF) controls cellular growth, migration, and differentiation. It is secreted by various cell types, including macrophages, and participates in tissue repair and epithelial regeneration. PDGF may therefore be involved in airway remodeling in asthma. This study compared the immunoreactivity of PDGF and its receptors (R alpha and R beta) in bronchial biopsies and the levels of PDGF in bronchoalveolar lavage (BAL) fluid of asthmatics and control subjects. Bronchial biopsies were done in a subsegmental bronchus of 11 asthmatics and 11 control subjects by flexible bronchoscope. PDGF AA and BB, and PDGF receptors R alpha and R beta were studied with monoclonal antibodies and revealed by immunoperoxidase staining. The percentage of subjects presenting positive staining with PDGFs and its receptors was studied in the epithelium and submucosa. PDGF AA, AB, and BB were measured in BAL fluid of 18 asthmatics and 10 controls by specific ELISA. In biopsies, there was no significant difference between asthmatics and controls for PDGF AA, BB, PDGF-R alpha and R beta (Fisher's exact test and Bonferroni's correction). Moreover, the levels of PDGF, AA, AB, and BB were similar in asthmatics and controls. This study does not support a role for PDGF in the repair processes of asthma.
Heat shock proteins (HSPs) appear to be involved in inflammation. Because asthma is an inflammatory disease, HSPs may be expressed. We studied the expression of HSP-70 by immunohistochemistry using the alkaline-phosphatase anti-alkaline phosphatase technique on epithelial cells and alveolar macrophages (AMs) from 19 patients with asthma, eight patients with chronic bronchitis (CB), and 13 control subjects and in bronchial biopsies from 15 asthmatics, 15 CB patients, and nine control subjects. The specificity of the antibody was confirmed by Western blotting on heated AM. The specificity of the immunostaining was confirmed by inhibition experiments. The expression of HSPs in the asthmatics in comparison with the CB patients and control subjects was significantly increased on epithelial cells (P < 0.002 and P < 0.007, respectively) and AMs (P < 0.0004 and P < 0.0002, respectively) and was significantly correlated with the severity of asthma (P < 0.005 for AMs) and the percentage of eosinophils in bronchoalveolar lavage fluid (P < 0.004). In biopsies from the asthmatics but not from the CB patients or control subjects, a positive staining for HSP-70 was observed on epithelium, mononuclear cells, and basement membrane and was correlated with the severity of asthma (P < 0.0005, P < 0.002, and P < 0.007, respectively). This study demonstrates that bronchial inflammation in asthma but not in CB may be linked to the production of HSPs.
The interest of the treatment with a single daily dose of amikacin (AMK) in cystic fibrosis (CF) patients with P. aeruginosa infections has been much debated. The aim of work was to study the efficiency of this treatment on (CF) patients with chronic bronchopulmonary P. aeruginosa infections previously treated for two weeks with the combination ceftazidime (CAZ 200 mg/day in 3 inj. IVD) and AMK (35 mg/day in one IV perf. of 30 minutes). The bacteriological supervision of this treatment was performed 1: by the determination of MICs before and after treatment, 2: by the decrease of P. aeruginosa colonization immediately after this treatment and during 11 months, 3: by the identification of P. aeruginosa strains with phenotypic methods (serotyping and antibiotyping) and with genotypic method (pulsed field gel electrophoresis). The use of AMK in a single daily dose in order to treat chronic lung infections colonized with P. aeruginosa susceptible to this antibiotic shows encouraging results as far as bacteriology is concerned: this treatment has given means to reduce colonization for a month in 15 of 18 patients. For 9 of the 18 patients, no P. aeruginosa strains were isolated for nine months. The serotyping and antibiotyping systems do not enable us to study the P. aeruginosa epidemiology. Genome macrorestriction fingerprinting of P. aeruginosa in pulsed field gel electrophoresis confirms that patient with CF were colonized with one or several clones. In our study no variation of these clones was noticed for the first eleven months. Genome macrorestriction fingerprinting appears to be one of the most effective methods for delineate strains of P. aeruginosa colonizing CF patients.
BACKGROUND: French Polynesia includes several ethnic groups; thus, it is well suited for evaluation of racial incidence of extrahepatic biliary atresia (BA). On the other hand, its geographic localization, far from specialized centers, does not facilitate early referral, indispensable for effective surgery. POPULATION AND METHODS: The files of 17 infants with BA, born in French Polynesia between 1 December 1981 and 1 December 1991, were retrospectively studied. During this period, there were 53,111 live births. RESULTS: The incidence of BA was 1/3,124. There were eight boys and nine girls; all belonged to the Maohi ethnic group (67% of the total population). They were admitted at the mean age of 71 +/- 33 days (range: 15-120 days); 15 of them were transferred to France at a mean age of 80 +/- 38 days (range: 35-150 days) and were operated on at 99 +/- 43 days (range: 48-195 days). The Kasai procedure was effective in four patients, with a follow-up ranging from 7 to 10 years. Among the 11 other patients, two underwent liver transplantation, four are waiting for transplantation and four have died. CONCLUSIONS: The influence of race on the incidence of BA has not been statistically established here even though this incidence appears high in French Polynesia and similar to Chinese babies in Hawaii. Local geographic conditions explain late admission in pediatric Units whereas possibilities for early surgery are improving.
BACKGROUND: Eosinophils but not neutrophils may play a role in the airway inflammation of asthma. In chronic bronchitis (CB) and chronic obstructive pulmonary disease (COPD), neutrophils are present in the airways. To differentiate among the pathology of asthma, CB, and COPD eosinophils and neutrophils were studied in peripheral blood, bronchial biopsy specimens, and bronchoalveolar lavage fluid (BALF). METHODS: We studied nine nonsmoking healthy subjects, 20 nonsmoking patients with asthma, 10 nonatopic smoking patients with CB (forced expiratory volume in 1 second: 98.4% +/- 11.3%) and 17 patients with COPD (forced expiratory volume in 1 second: 51.2% +/- 14.3%). Eosinophils were characterized by their enumeration in biopsy specimens (EG2 monoclonal antibody), peripheral blood, and BALF and by measurement of eosinophil cationic protein in BALF. Neutrophils were characterized by their enumeration in biopsy specimens (anti-elastase monoclonal antibody) and BALF and by measurement of neutrophil-specific myeloperoxidase in BALF. RESULTS: In patients with asthma we found degranulated eosinophils in biopsy specimens and significantly increased eosinophil cationic protein levels in BALF. In patients with CB or COPD, eosinophil numbers in biopsy specimens were not significantly different from those of patients with asthma, but cells were not degranulated and eosinophil cationic protein levels in BALF were similar to those of normal subjects. In patients with CB or COPD neutrophils were not increased in the mucosa, but neutrophil numbers and myeloperoxidase levels in BALF were significantly increased. CONCLUSION: The percentages of neutrophils in BALF were greater in patients with COPD than in those with CB, suggesting a role in the chronic airflow limitation.
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