[Children who swallow air]. / Les enfants avaleurs d'air.

UNLABELLED: Gas in the gut derives from swallowed air, intra-luminal production and diffusion from the blood. Excessive swallowed air may cause pathologic aerophagy. CASE REPORTS: This report describes four children aged from 3 to 12 years with pathologic aerophagy due to excessive air swallowing. One of them had a "Gas Bloat Syndrome". Clinical presentation was very incongruous. However, the clinical features were characteristic: abdominal distention increasing throughout the day, associated with visible and often audible air swallowing and excessive flatus. The questioning and clinical examination helped for diagnosing. Medical symptomatic treatment was associated with a psychotherapy approach. CONCLUSION: A better recognition of this condition might contribute towards earlier diagnosis.

Efficacy, tolerance, and pharmacokinetics of once daily tobramycin for pseudomonas exacerbations in cystic fibrosis.

OBJECTIVE: To compare once daily with thrice daily tobramycin for treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis. DESIGN: 22 patients with cystic fibrosis, mean (SD) age 11 (3.4) years (range 5.6-19.3), with pulmonary pseudomonas exacerbations were randomly assigned to receive a 14 day course of tobramycin (15 mg/kg/day) either in three infusions (group A) (n = 10) or a single daily infusion (group B) (n = 12), combined with ceftazidime (200 mg/kg/day as three intravenous injections). Efficacy was assessed by comparison of pulmonary, nutritional, and inflammatory indices on days 1 and 14. Cochlear and renal tolerance were assessed on days 1 and 14. Tobramycin concentration was measured in serum and sputum 1, 2, 3, 4, 8, and 24 hours after the start of the infusion. Analysis was by non-parametric Wilcoxon test. RESULTS: Variables improving (p < 0.05) in both groups A and B were, respectively: weight/height (+4% and +3.1%), plasma prealbumin (+66 and +63 mg/l), forced vital capacity (FVC) (+14% and +11%), forced expiratory volume in one second (+15% and +14%), and forced expiratory flow between 25% and 75% of FVC (+13% and +21%). Improvement was not significantly different between groups. Renal and cochlear indices remained within the normal range. Serum peak concentration of tobramycin on day 1 was 13.2 (7.1) mg/l in group A and 42.5 (11.2) mg/l in group B (p < 0.001); serum trough was 1.1 (0.8) mg/l in group A and 0.3 (0.2) mg/l in group B (p < 0.01). Tobramycin concentrations in sputum were two to three times higher in group B than group A. CONCLUSIONS: Once daily tobramycin combined with three injections of ceftazidime is safe and effective for the treatment of pseudomonas exacerbations in cystic fibrosis patients.

A longitudinal study of seroreactivity against Mycoplasma penetrans in HIV-infected homosexual men: association with disease progression.

We investigated the relationships between a putative cofactor of HIV infection, Mycoplasma penetrans, and the evolution of HIV disease. The evolution of titers of anti-M. penetrans antibodies in 58 randomly selected HIV-seropositive adult homosexual men was investigated. The median length of follow-up was 38 months. Thirty-six individuals was investigated. The median length of follow-up was 38 months. Thirty-six individuals (62.1%) remained M. penetrans seronegative (group 0). Fourteen patients (24.1%) had consistently low antibody titers or low antibody titer(s) in at least one sample and negative test(s) in the other(s). This pattern was possibly associated with latent or earlier infection (group 1). Eight patients (13.8%) had moderate to high antibody titers for long periods, indicating an active and persistent M. penetrans infection (group 2); four patients in this group presented a serological reactivation and thus probably developed an acute infection during the study; two had a stable and moderate level of antibody throughout the study; in two patients the antibody titers decreased substantially. Interestingly, CD4 cell counts declined more rapidly in group 2 than in group 0 (medians of -4.5 versus -2.1 cells/mm3/month, p < 0.05 and -0.16 versus 0 cell percentage/month, p < 0.05), whereas there was no significant difference between groups 1 and 0 (medians of -2.0 versus -2.1 cells/mm3/month and -0.15 versus 0 cell percentage/month). In patients with serological reactivation, the viral load was higher in sera with higher M. penetrans antibody titers. These findings suggest an association between active M. penetrans infection and progression of HIV disease.

Nutritional impact of antipseudomonas intravenous antibiotic courses in cystic fibrosis.

OBJECTIVE: To evaluate the short term effects on nutritional status of home intravenous anti-pseudomonas antibiotic courses in cystic fibrosis (CF) patients chronically colonised with Pseudomonas aeruginosa. DESIGN: A prospective study involving 38 CF patients, mean age 10.9 (SD 4.3) years (range 4.3 to 22.2 years), presenting with pulmonary exacerbations of P aeruginosa infection. The patients received a 14 day antibiotic course of intravenous ceftazidime (200 mg/kg/day) and either amikacin (35 mg/kg/day) or tobramycin (15 mg/kg/day). Nutritional evaluation on days 1 and 14 involved measurements of weight, weight/height ratio (per cent of predicted value), energy intake (per cent of recommended daily allowances), serum prealbumin, and body composition assessed by two methods: bioelectrical analysis (BIA) and skinfold anthropometry. The non-parametric Wilcoxon t test was used for statistical analysis, with a Bland-Altman plot to assess the degree of agreement between the two methods of evaluating body composition. RESULTS: Weight increased by 1.0 (0.8) kg (p < 0.001); weight/height increased from 94.4(12.2)% to 98(12.7)% (p < 0.001), energy intake from 107(32)% to 119(41)% (p < 0.02), and prealbumin from 183 (63) to 276 (89) mg/l (p < 0.001). Fat mass increased by 0.8 (1.0) kg (p < 0.001), without any significant change in fat-free mass. The limits of agreement between BIA and anthropometry were -0.7 kg and +1.1 kg. CONCLUSIONS: Antibiotic courses allow an improvement in nutritional status in CF patients, with a gain in fat mass.

Pharmacokinetics and bronchial diffusion of single daily dose amikacin in cystic fibrosis patients.

A single daily dose of amikacin 35 mg/kg by i.v. infusion over 30 min in 18 cystic fibrosis patients achieved mean serum peak and trough concentrations of 121.4 mg/L (+/- 37.3) and 0.88 mg/L (+/- 0.62), respectively. Pharmacokinetic parameters and bronchial diffusion of amikacin showed marked inter-patient variability. The highest concentrations in sputum were obtained at 2 h (10.95 +/- 7.55 mg/L) and decreased slowly to reach a mean concentration of 2.14 mg/L (range 0.2-3.8 mg/L) just before the following infusion. An increase in the body clearance of amikacin and a decrease in the volume of distribution according to age were observed.

[Autoimmune hepatitis treated with cyclosporin revealed by acute hepatocellular failure]. / Traitement par la ciclosporine d'une hépatite auto-immune révélée par une insuffisance hépatocellulaire aiguë.

BACKGROUND: Autoimmune hepatitis (AIH), usually chronic hepatitis, can be revealed by an acute episode of hepatic failure not easily treatable. CASE REPORT: A 4 year-old boy presented with severe acute hepatic failure (AHF) without hypergammaglobulinemia, revealing AIH. A triple immunosuppressive treatment (prednisolone, azathioprine, ciclosporine) was started when clinical and biological signs worsened (prothrombin time 18%, factor V 32%). Liver functions significantly improved within 10 days, although complications due to intensive immunosuppression occurred (Candida septicemia). CONCLUSION: AIH must be evocated in each case of AHF. Some observations of AIH treated with ciclosporine are published but protocols of its administration are variable: ciclosporine is used alone or associated with other drugs, in first intention or secondarily, when the classical treatment is contra-indicated or fails. Controlled studies are needed to precise ciclosporine indications in AIH and to propose the best protocol.

High prevalence of antibodies to Mycoplasma penetrans in human immunodeficiency virus-seronegative and -seropositive populations in Brazzaville, Congo.

In industrialized countries, the prevalence of antibodies to Mycoplasma penetrans is higher among human immunodeficiency virus (HIV)-seropositive homosexuals than other HIV-seropositive and HIV-seronegative groups. In an African heterosexual population, we found a higher prevalence of M. penetrans antibodies in HIV-seronegative blood donors (15.5%) than in France (0.9%) or the United States (0.3%) and a prevalence of 13.4% in HIV-seropositive individuals. HIV-seropositive individuals with less than 5% CD4 cells had a higher prevalence of M. penetrans antibodies than individuals with 5% or more CD4 cells (25.0 versus 8.5%).

PIP: Numerous studies in developed countries have revealed a higher prevalence of antibodies to Mycoplasma penetrans in homosexuals infected with HIV than in other HIV-positive and HIV-negative population groups. To confirm whether this association prevails in African countries, a cross-sectional analysis was conducted in Brazzaville, Congo, in 1993-94. Tested were 116 HIV-negative blood donors and 149 HIV-infected hospital patients. The prevalence of antibodies to M. penetrans was 13.4% in the HIV-positive and 15.5% in the HIV-negative group. Among HIV-infected patients, M. penetrans seropositivity was more frequent among patients under 30 years of age, those with CD4 lymphocyte counts below 200 cells/cu. mm, and those with CD4 lymphocyte percentages below 5%. This correlation between the prevalence of antibodies to M. penetrans and the severity of immunosuppression has been documented in studies from France and the US as well. The high prevalence of antibodies to M. penetrans in the late stages of HIV infection in Western homosexuals and Congolese heterosexuals may reflect a cohort effect in which the groups most exposed to HIV at the beginning of the epidemic were also those most exposed to M. penetrans infection. It is also possible that the development of M. penetrans is due to immunosuppression or, alternatively, infection influences HIV multiplication.

[Liver transplantation in an adolescent with cystic fibrosis]. / Transplantation hépatique chez une adolescente atteinte de mucoviscidose.

BACKGROUND: Orthotopic liver transplantation (OLT) is an effective treatment for patients with cystic fibrosis end stage liver disease, especially those with only mild pulmonary involvement. Long-term follow-up in such transplanted patients is still lacking. CASE REPORT: A 15-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aeruginosa for 3 years and had pancreatic insufficiency; she also had mild glucose intolerance. Postoperatively she developed diabetes mellitus requiring insulin therapy for 9 months. Oral cyclosporin was poorly absorbed so that she was given a new emulsion of cyclosporin (Neoral) that was better absorbed. A rapid pubertal catch-up was obtained but the patient remained colonized by Pseudomonas aeruginosa. CONCLUSION: This 3-year postoperative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.

Search for the presence of six Mycoplasma species in peripheral blood mononuclear cells of subjects seropositive and seronegative for human immunodeficiency virus.

The prevalence of Mycoplasma fermentans, Mycoplasma pirum, Mycoplasma genitalium, Mycoplasma pneumoniae, Mycoplasma hominis, and Mycoplasma penetrans was investigated by using specific PCR assays with peripheral blood mononuclear cells from subjects infected or not infected with the human immunodeficiency virus (HIV). Only M. fermentans was detected in 5.8% of 154 HIV-seropositive and 11.1% of 90 HIV-seronegative subjects.

Percutaneous endoscopic gastrostomy in children: influence on gastroesophageal reflux.

OBJECTIVE: Few data exist in the literature about the relationship between percutaneous endoscopic gastrostomy (PEG) and gastroesophageal reflux (GER) in children, and the data that do exist are contradictory. The aim of the present study was to evaluate the effect of PEG on GER. METHODS: Twenty children underwent PEG for enteral nutrition. They were 55 +/- 55 months old and weighed 13 +/- 10 kg. A pH study was performed before and after PEG without treatment when GER status was unknown (n = 10) or under treatment (n = 10) if previous GER was demonstrated. In these cases, the pH study was performed under the same treatment before and after PEG. RESULTS: Six pH studies had abnormal results before PEG. After PEG, the GER of these 6 children significantly improved after the treatment was intensified (n = 50 or spontaneously normalized (n = 1). Results of 13 pH studies that were previously normal remained normal. Only one child with a normal reflux index before PEG had GER after it. For the 20 children, the mean reflux index did not change significantly after PEG (5.5% vs 5.6%). CONCLUSION: Contrary to surgical gastrostomy, PEG does not worsen GER. Therefore, GER is not a contraindication to PEG.

Association of Mycoplasma penetrans with human immunodeficiency virus infection.

A cross-sectional study was done to determine the seroprevalence of Mycoplasma penetrans in human immunodeficiency virus (HIV) type 1-seropositive and -seronegative persons recruited in France. The data were analyzed with respect to the sociodemographic, clinical, and biologic status of the patients. M. penetrans seropositivity was associated with HIV infection (18.2% of HIV-seropositive vs. 1.3% of HIV-seronegative persons were M. penetrans-seropositive; P < .001). M. penetrans infection was predominantly but not exclusively associated with homosexual practices in HIV-seropositive subjects and thus presumably sexually transmitted. M. penetrans seroprevalence increased with progression of HIV-associated disease. No association was found between M. penetrans and Kaposi's sarcoma. Thus, there is an unambiguous association between M. penetrans and HIV, particularly among homosexual persons, but its clinical significance remains to be investigated.

[Frequency of gastroesophageal reflux in infants and in young children with cystic fibrosis]. / Fréquence du reflux gastrooesophagien chez le nourrisson et le jeune enfant atteints de mucoviscidose.

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.

Development of PCR-based assays for the detection of two human mollicute species, Mycoplasma penetrans and M. hominis.

Recently, a 16S rDNA-based polymerase chain reaction (PCR) assay was developed for the selective and sensitive detection of Mycoplasma pirum. In this study, the same procedure was used in order to selectively detect by PCR two human mycoplasmas, M. hominis and M. penetrans, with a high level of sensitivity even in a context of human DNA. For each assay, the specificity was verified by testing DNA from other mollicute species (including those closely related to the corresponding mycoplasma), from bacteria phylogenetically close to mollicutes, from Escherichia coli and from human peripheral blood mononuclear cells (PBMCs). Each assay proved to be highly sensitive since it reliably detected 10 DNA molecules, even in a context of human DNA. The results of this study demonstrate the suitability of our procedure using primers which were designed for the PCR detection of human mollicutes with a high specificity and a low and reproducible threshold of sensitivity.

[Sturge-Weber-Krabbe syndrome. A severe form in a monozygote female twin]. / Syndrome de Sturge-Weber-Krabbe. Forme grave chez une jumelle monozygote.

We report a severe case of Sturge-Weber syndrome in one of two monozygotic twins. This syndrome included a facial portwine stain over the trigeminal ophthalmic V1 area, and ipsilateral vascular anomalies of the eye and of the pia mater. CT scans and MRI were very informative. The cerebral regional blood flow, studied with SPECT, showed a paradoxically high rate.