[Turbulence in emergency ward. Agitation of children and adolescents].
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Acute Disease↗
Biomedical subjects
G Champion
Publications and source records attributed to G Champion.
Frozen shoulder.
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Anti-Inflammatory Agents↗
[Terminal and pre-terminal chronic renal insufficiency in newborns in French neonatal intensive care units: survey of the French pediatric nephrologic society of resuscitation and emergency].
OBJECTIVES: The aim of this study was to describe the intensive care unit neonatologists' attitudes about a neonate with terminal or pre-terminal renal failure. METHODS: A questionnaire was sent to all French neonatal intensive care units. Physicians were asked to describe their attitude about neonatal chronic renal failure (Would you agree with dialysis and graft for these children?). Physicians were also presented with two clinical observations involving neonates with varying degrees of renal insufficiency and a complicating comorbidity, including neurological abnormality or socioeconomic circumstances. RESULTS: Responses were obtained from 92% of the university neonatal care units. The will to take care of a neonate with end-stage renal failure till the renal graft, varied greatly from a centre to another one. Three (9%) university-teams said they had a strong will to bring the baby from the neonatal period to the time of renal graft. Eleven other centres (32%) did not have any will for accompanying the baby till the renal graft. Eight centres (24%) would be rather favourable to the idea of dialysis and graft, and 12 others (35%) would be rather unfavourable. CONCLUSION: The results of this study show great differences between French neonatologists when they are faced to newborns with end stage renal failure. Ethical, medical and organisational difficulties are matters of controversy. The epidemiological impact of the perinatal discussion could be a 20% variation of all the renal grafts in children.
Ethics, Medical↗
Photoinduced ferrimagnetic systems in Prussian blue analogues C(I)xCo4[Fe(CN)6]y (C(I) = alkali cation). 4. Characterization of the ferrimagnetism of the photoinduced metastable state in Rb1.8Co4[Fe(CN)6]3.3-13H2O by K edges X-ray magnetic circular dichroism.
In Part 2 of this work, the electronic and local structure of the photoinduced metastable magnetic state of the Prussian blue analogue Rb1.8Co4[Fe(CN)6]3.3-13H2O were characterized. To determine directly the relative orientation of the magnetic moments of Co(II) and Fe(III) ions in the metastable state, and the nature of the exchange interaction between them, we performed X-ray magnetic circular dichroism (XMCD) experiments at the cobalt and iron K edges. We present the first direct experimental evidence of the antiferromagnetic interaction between the cobalt and the iron ions, leading to the ferrimagnetism of the photoinduced metastable state.
Journal Article↗
Shigella infection in a SCID mouse-human intestinal xenograft model: role for neutrophils in containing bacterial dissemination in human intestine.
Shigellae infect human intestine and cause intense inflammation and destruction of colonic and rectal mucosa. To model the interactions of shigella with human intestine in vivo, we have studied shigella infection in human intestinal xenografts in severe combined immunodeficient mice (SCID-HU-INT mice). Inoculation of shigella into human intestinal xenografts caused severe inflammation and mucosal damage, which was apparent as soon as 4 h following infection. Shigella infection was associated with human intestinal production of interleukin-1B (IL-1B) and IL-8 and a marked neutrophil influx into the graft. Depletion of neutrophils from SCID-HU-INT mice reduced inflammation in the human intestinal xenograft in response to shigella infection but failed to significantly alter tissue damage. However, the number of intracellular bacteria was more than 20-fold higher in the human intestinal xenografts from neutrophil-depleted SCID-HU-INT mice. Infection of human intestinal xenografts with an attenuated vaccine strain of shigella (CVD1203) induced lower levels of IL-1B and IL-8 than wild-type shigella and caused only moderate damage to the intestinal permeability barrier. Our studies establish the SCID-HU-INT mouse as a viable model for studying the interactions between shigella and human intestine and indicate that neutrophils are important for controlling the invasion of human intestine by shigella.
Animals↗
HLA class II phenotype controls diversification of the autoantibody response in primary Sjögren's syndrome (pSS).
The coexistence of anti-La (SS-B) and anti-Ro (SS-A) autoantibodies in pSS is probably explained by intermolecular spreading of autoimmunity toward different components of the La/Ro ribonucleoprotein (RNP). In order to evaluate the role of the HLA class II phenotype in controlling diversification of this autoantibody response, 80 patients with pSS were typed by polymerase chain reaction sequence-specific oligonucleotide (PCR-SSO) at the HLA class II loci DRB1, DQA1 and DQB1. Serum samples were examined for anti-La and anti-Ro by counterimmunoelectrophoresis and by ELISA using purified recombinant La and 60-kD Ro proteins. Patient sera were classified according to the extent of diversification of the anti-La, anti-Ro response including the presence or absence of precipitating anti-La antibodies. Immunogenic characteristics of these stratified groups were then studied. All patients with pSS, with or without autoantibodies to Ro and La, were found to have at least one of the HLA-DRB1 types DR2, DR3 or DR5. The HLA DR3-DQA1*0501-DQB1*02 (DR3-DQ2) haplotype was primarily associated with a diversified La/Ro RNP response containing precipitating autoantibodies to La (P<0.001); whereas the haplotype HLA DR2-DQA1*0102-DQB1*0602 (DR2-DQ1) was associated with a less diversified La/Ro RNP response containing non-precipitating (restricted epitope) anti-La autoantibodies (P<0.001). Anti-La-positive patients lacking both HLA-DR2 and HLA-DR3 all expressed the HLA-DQA1*0501 allele, which was present at increasing frequency with greater diversification of the anti-La/Ro autoantibody response. The association of distinct HLA haplotypes with different degrees of autoantibody diversification in patients with pSS suggests a model of HLA-restricted presentation of La/Ro peptide determinants to autoreactive helper T cells. We propose that non-precipitating anti-La responses are driven by limited intermolecular help from DR2-DQ1-restricted T helper cells recognizing Ro determinants. On the other hand, we speculate that the more diversified, precipitating anti-La responses obtain more efficient cognate T help from DR3-DQ2-restricted T helper cells recognizing La determinants, where HLA-DQA1*0501 may be a critical determinant for antigen presentation.
Alleles↗
A second family with XLRH displays the mutation S244L in the CLCN5 gene.
Mutations in the CLCN5 gene, mapped in Xp11.22, have been recently reported to be associated with X-linked nephrolithiasis, X-linked recessive hypophosphataemic rickets and Dent's disease. We report a missense mutation in exon 6 of the CLCN5 gene. The mutation in this pedigree is S244L, the same mutation as has previously been described in an Italian family showing a similar pathology. However, in the family reported here, affected males have developed neither nephrolithiasis nor nephrocalcinosis. The question arises whether we are dealing with a milder phenotype or whether a more severe pathology will develop with ageing.
Adolescent↗
[Urinary tract abnormalities with prenatal diagnosis: neonatal management and outcome of 100 children born 1988-1990 at the Angers CHU (University Hospital)].
UNLABELLED: Malformative uropathies diagnosed in utero are increasing in number. This work describes the decision strategy adopted in Angers concerning the neonatal handling of those abnormalities. PATIENTS AND METHODS: One hundred children born between 1988 and 1990, with prenatally suspected fetal uropathy, were included in the study and followed for a period of 3 years. In every case, an ultrasound scan was performed at birth. In cases with persistent abnormality, a voiding cystourethrography was done in the first week of life. An intravenous urograph and/or a nuclear renography were performed during the second month of life. RESULTS: Twenty-nine children were normal. Seventy-one were affected by 126 isolated or related uropathies; the most frequent ones were the ureteropelvic junction obstruction syndrome (48), ureterovesical junction obstruction (18) and multicystic kidneys (13). A vesicoureteral reflux was associated in 22% of cases. The diagnosis was perfectly correlated with the prenatal diagnosis in 50% of cases. Fourteen of the normal children had a later ultrasound scan control, between 2 and 9 months; later on, three of them showed a moderate ureteropelvic junction obstruction syndrome. Amongst the 48 ureteropelvic junction obstruction syndromes, 22% have been operated on. The others obstructive uropathies remained stable or spontaneously improved. DISCUSSION: These results require us to discuss as matter of priority the large number of spontaneously regressive prenatal hydronephrosis and the necessity to establish a consensus for the pre and postnatal pathological thresholds of the anteroposterior pelvic diameter, the interest in carrying out a voiding cystography after the birth when dilatation is confirmed, the interest of nuclear renography for the diagnosis and follow-up of obstructive uropathies and the absence of urgency for surgery. CONCLUSION: We propose a decision tree specifying the action to take when facing the diagnosis of a prenatal hydronephrosis. It could be a part of the reflection for the medical teams handling these uropathies is the aim of a consensual attitude which is now essential.
Disease Management↗
A curable cause of chronic idiopathic intestinal pseudo-obstruction in children: idiopathic myositis of the small intestine.
We report an intestinal pseudo-obstruction syndrome occurring in a 6-month-old infant girl suffering from acquired major and persistent abdominal distension, which 2 months later required ileostomy. Histologic examination of samples of the small intestine showed considerable inflammatory reaction in the muscular layers of the intestinal wall. Steroid therapy, begun as soon as the histologic results were known, resulted in recovery. In a similar case, reported previously, steroid therapy used after the lesions had already caused fibrosis and atrophy of the intestinal wall, proved ineffective. This particular form of pseudo-obstruction is classified as "idiopathic myositis of the small intestine." It is important to identify the condition because an early course of steroid therapy, before the appearance of fibrotic lesions, could improve the prognosis.
Female↗
Hepatopulmonary syndrome in a child with cystic fibrosis.
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Child↗
[Training protocol for nurses in a hemodialysis unit].
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Curriculum↗
Effects of oral erythromycin on esophageal pH and pressure profiles in patients with gastroesophageal reflux disease.
Erythromycin, a possible motilin agonist, is a potent gastrokinetic agent that may increase the lower esophageal sphincter pressure. Therefore, we assessed the effects of erythromycin in two dosages (250 and 500 mg per os four times a day) on esophageal pH and pressure profiles in reflux patients using prolonged ambulatory monitoring systems. Studies were blinded, placebo-controlled with randomized crossover design. Patients took each drug for three days prior to studies, with erythromycin serum levels obtained the day of esophageal studies. Erythromycin 250 mg four times a day had no effect on esophageal contraction pressures or peristalsis during the day or meal periods. In the supine position, however, erythromycin significantly (P = 0.012) decreased esophageal contraction velocity and showed a strong trend (P = 0.059) towards increasing the percentage of peristaltic waves. Despite these potentially beneficial effects on esophageal clearance, no significant difference in acid exposure times during 24-hr pH studies were observed between placebo and low-dose erythromycin. High-dose erythromycin (500 mg four times a day) was associated with drug levels in the typical antibiotic efficacy range (normal 1-3 micrograms/ml; patients 1.7-7.0 micrograms/ml), but, here again, there was no significant difference in all acid reflux parameters between placebo and erythromycin phases. Therefore, "standard" doses of erythromycin have no important clinical effects on esophageal pressures or acid reflux parameters.
Administration, Oral↗
Duodenogastroesophageal reflux: relationship to pH and importance in Barrett's esophagus.
BACKGROUND/AIMS: Several reports suggest that duodenogastroesophageal reflux may produce esophagitis, Barrett's esophagus, and esophageal adenocarcinoma. The purpose of this study was to understand better the relationship of pH (< 4 and > 7), duodenogastroesophageal reflux, and fasting bile acid concentrations in producing esophageal damage. METHODS: Using a spectrophotometric technique to measure bile reflux, four groups were studied: healthy subjects, reflux patients, patients with Barrett's esophagus, and patients with esophageal symptoms after partial gastrectomy. RESULTS: Simultaneous 24-hour pH and bile monitoring of distal esophagus found close association between total percent of time pH < 4 and duodenogastroesophageal reflux (r = 0.78; P < 0.001) but a poor relationship (r = -0.06) with total percent of time pH > 7, suggesting that the term alkaline reflux is a misnomer. Duodenogastroesophageal reflux increased significantly with the severity of reflux disease, being greatest in patients with Barrett's esophagus and comparable with that in patients with partial gastrectomy. Fasting bile acid concentrations did not distinguish patients with Barrett's esophagus from those with reflux. Rather, increased quantity of acid reflux was the single factor most characterizing patients with Barrett's esophagus. Omeprazole (20 mg twice daily) normalized acid reflux parameters (13.8% +/- 1.6% to 0.8% +/- 0.6%) and significantly (P < 0.001) decreased duodenogastroesophageal reflux (32.8% +/- 6.9% to 4.7% +/- 1.7%). CONCLUSIONS: Acid reflux is the primary factor in the development of Barrett's esophagus. Bile reflux parallels acid reflux and, at best, may have a synergistic role. Aggressive acid suppression with omeprazole markedly decreases both.
Acids↗
Omeprazole versus H2-receptor antagonists in treating patients with peptic stricture and esophagitis.
BACKGROUND/AIMS: Although dysphagia in patients with peptic stricture is attributed to a decreased luminal diameter, coexistent esophagitis may be an equally important cause. The goals of this study were to determine whether medical healing of esophagitis in patients with stricture improves dysphagia and decreases dilatation need and to compare the efficacy and cost-effectiveness of omeprazole versus H2-receptor antagonists (H2RA). METHODS: Thirty-four dysphagic patients with peptic stricture and erosive esophagitis were dilated and randomized to omeprazole 20 mg every day versus H2RA (ranitidine 150 mg twice daily or famotidine 20 mg twice daily). Patients received further dilatations only if dysphagia frequency was greater than or equal to once per week. At 3 and 6 months, patients were assessed for esophagitis healing, dysphagia relief, and bougienage requirements. Cost-effectiveness of omeprazole and H2RA was determined. RESULTS: Patients with healed esophagitis at 3 and 6 months were more likely to dysphagia-free and to require fewer dilatations than patients with persistent esophagitis. At 6 months, omeprazole produced a significantly (P < 0.01) higher rate of esophagitis healing, dysphagia relief, and fewer dilatations compared with H2RA. Omeprazole was also 40%-50% more cost-effective. CONCLUSIONS: Esophagitis healing improves dysphagia and decreases dilatation need in patients with peptic stricture. Omeprazole heals esophagitis and relieves dysphagia more efficaciously than H2RA while decreasing costs to patients.
Aged↗
Treatment of achalasia: the best of both worlds.
OBJECTIVE: To conduct a retrospective analysis of one center's experience with treating achalasia over 4 yr with skilled gastroenterologists using primarily the Rigiflex balloon dilator and with a senior surgeon performing Heller myotomies. METHODS: Newly diagnosed cases of achalasia were identified by a computer search of hospital records. Charts were reviewed for the presence/severity of dysphagia, regurgitation, heartburn, and chest pain. Weight loss was also recorded. Esophageal manometries and barium swallows were reviewed. Choice of treatment was made freely by patients. With an a priori definition of success, follow-up was conducted by telephone interviews. RESULTS: A total of 45 achalasia patients (mean age, 46 yr; 32 females, 13 males) were identified with a symptoms-frequency as follows: dysphagia, 100%; regurgitation, 78%; heartburn, 50%; and chest pain, 42%. Mean weight loss was 17.5 pounds. Primary treatment was pneumatic dilation in 36 patients and surgery in nine patients. In a total of 45 pneumatic dilations, three (6.6%) were complicated by perforation. Five (14%) patients required repeat dilation. Mean duration of follow-up for pneumatic dilation and surgery was 27 months and 20.8 months, respectively. The overall excellent-good success rates were: pneumatic dilation 88% and surgery 89%. In comparing the efficacy of pneumatic dilation versus surgery, all symptoms were improved significantly (p < 0.01) in both groups, except heartburn, which increased postmyotomy. CONCLUSIONS: If both procedures are available by skilled operators, pneumatic dilation and surgery are equally effective in the treatment of achalasia.
Adolescent↗
[Hypothalamic syndromes. Review of clinical and endocrinal semiology].
A case of hypothalamic dysfunction in a girl with a twelve-year follow-up is reported. Onset occurred at the age of three with severe obesity, hypothermia, hypersomnia, and lethargy. Somatotropic, gonadotropic, and thyrotropic hormones were low, whereas prolactin was increased. Imaging techniques failed to disclose any lesion of the hypothalamus or pituitary. Clomipramine improved the vegetative disorders. The literature on clinical and hormonal disorders of hypothalamic dysfunction is reviewed.
Child, Preschool↗
Does steroid pulsing influence the efficacy and toxicity of chrysotherapy? A double blind, placebo controlled study.
To test the hypothesis that early steroid pulsing augments the efficacy and decreases the toxicity of chrysotherapy 40 patients with rheumatoid arthritis were studied in a double blind, placebo controlled study. During the first three months of gold treatment group 1 received monthly intravenous methylprednisolone pulsing (steroid group) while group 2 received placebo (placebo group). All patients were assessed clinically and serologically over a 24 week period. Twelve patients were withdrawn before completion of the study and all but one of the remaining 28 patients reported clinical and serological improvements. Two patients in the steroid group were withdrawn owing to gold induced side effects while four were withdrawn in the placebo group. These small numbers were not significantly different. Minor side effects occurred more commonly in the placebo group. The clinical response was clearly better in the steroid group with statistical significance almost being achieved. In an endeavour to obtain a significant conclusion further patients will now be entered into this study.
Aged↗
[Severe manifestations of non-poliomyelitic enterovirus infections in newborn infants].
The authors report eight cases of severe neonatal infections with Coxsackie and Echo virus. Meningo-encephalitis, aseptic meningitis, myocarditis and sepsis-like state were the most frequent presenting conditions. A high mortality rate was associated with meningo-encephalitis and low birth weight.
Coxsackievirus Infections↗