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Introduction: Blood transfusion is a life-saving treatment for severely anaemic children both in developed and developing countries. In this study we describe transfusions in paediatric settings of Gabon, Africa including clinical indications and subsequent outcomes.Methods: This prospective descriptive study was conducted in the cities of Libreville and Lambaréné from 1 January to 30 September 2016.Children between the ages of 1 month and 15 years, who were hospitalised and transfused were included in the study.Results: We included 287 children who represented 17.1% of all hospitalised children. The male:female ratio was 0.95 and the average age was 3.7 years. Packed red blood cells (PRBC) were administered to 99.3% of anaemic patients. World Health Organization (WHO) defined severe anaemia (haemoglobin (Haemoglobin)

Prolonged fever is an important cause of morbidity in pediatric practice, especially in tropical areas. It is above all a problem of etiological diagnosis given the vast number of etiologies. In sub-Saharan Africa, practitioners more often focus on bacterial infections and malaria at the expense of other infectious diseases such as human African trypanosomiasis (HAT), most often leading to overuse of antibiotics and antimalarials. A dramatic resurgence of HAT, also called sleeping sickness, has been reported during the last few decades in large areas of Central Africa. Furthermore, with the development of air transport, cases of children infected during a trip to Africa can be exported outside endemic areas, making diagnosis even more difficult. This parasitic infection causes a protracted, often initially unrecognized, illness with episodes of fever, headache, and malaise, accompanied by progressive lymphadenopathy, before the development of a progressive meningoencephalitis. These three case reports aim to remind practitioners of clinical and biological signs suggestive of HAT diagnosis in children living in endemic areas or having stayed there during the months prior to visiting the doctor. The prognosis is largely dependent on the precocity of diagnosis and therapeutic support.

Ce travail avait pour but de determiner le type, la frequence, et etablir les facteurs etiologiques, des differents accidents domestiques a Libreville. Etude prospective transversale menee de septembre 2005 a janvier 2006 dans les services d’urgences pediatriques du Centre hospitalier de Libreville et de l’Hopital pediatrique d’Owendo. Etaient inclus des enfants de moins de 15 ans, victimes d’accidents ou traumatismes non intentionnels survenus a domicile ou dans ses abords immediats. Ont ete analyses l’âge, le sexe, l’environnement, les circonstances de survenue et le type d’accident. Les 56 enfants inclus se repartissaient en 31 garcons et 25 filles. Les enfants de moins de 6 ans (62,5%) etaient frequemment touches. La chute etait le principal accident domestique (46%) suivi des intoxications (16%). La cour etait le principal lieu des accidents (47%) suivie de la chambre (13%) et la cuisine (12%). L’environnement de l’enfant, le niveau professionnel des parents etaient lies a la frequence des accidents (p < 0, 01).

High fat containing diets lower VCO 2 in patients with impaired pulmonary function fed at a high level of energy intake. We tested the effect of a high fat enteral nutrition on VCO 2 and substrate oxidation in cystic fibrosis patients fed enterally 130% RDA. VCO 2 and substrate oxidation were studied in a group of eight 6–19 year old patients while receiving for 1 month and in a random order isocaloric (1000 kcal/m 2 ), isonitrogenous enteral diet with a normal fat and a high fat content (40% and 67% of non-protein energy intake). Substrate oxidation and net balance were estimated using indirect calorimetry at the end of each study period. Overnight high fat enteral infusion resulted in no significant change in VCO 2 and VO 2 but lowered RQ (0.84 ± 0.01 vs 0.88 ± 0.01, P = 0.02) and non-protein RQ (0.83 ± 0.01 vs 0.88 ± 0.01). In spite of a higher glucose oxidation rate (8.1 ± 0.5 vs 6.3 ± 0.5 g. h −1 , P = 0.04), glucose net balance was significantly higher during normal fat formula administration (+2.5 ± 0.8 v-0.3 ± 0.7 g/h, P The present study failed to show any benefit of a high fat diet on VCO 2 in non oxygenodependant cystic fibrosis children and adolescents fed slightly above RAID. Normal fat enteral formula led to higher glycogen repletion.

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

Resume Le devenir a long terme des stenoses caustiques de l'aesophage est mal connu chez l'enfant. L'objectif de ce travail etait de l'evaluer sur les plans fonctionnel, organique et psychosocial. Population et methodes . - Trente-quatre enfants, âges en moyenne de 3 any et 7 mois ± 3 ans et 2 mois (extremes : 1 moss-14 any et 3 mois) ont ete suivi au cours d'une etude longitudinale pendant une duree moyenne de 11 ans et 4 mois. L'analyse a porte sur les parametres suivants: treatment utilise (dilatations mecaniques et/ou coloplastie), symptomatologie fonctionnelle (dysphagie, reflux gastro-aesophagien), etat nutritionnel (poids/taille, composition corporelle) et consequences psychosociales. Les tests du ,χ 2 et de Mann-Whitney ont ete utilises pour I'analyse statistique. Resultats . - Vingt et un enfants ont ete traites par des dilatations mecaniques et 12 patients ont beneficie d'une coloplastie. Le nombre moyen de seances de dilatations est plus eleve chez les sujets non operes que chez les enfants operes (21 ± 17 versus 14 ± 16; p p = 0,1) et la survenue de reflux gastro-aesophagien exclusivement chez les enfants dilates. L'etat nutritionnel est peu affecte par la presence d'une stenose caustique ; une obesite est retrouvee chez 15 % des enfants, et une malnutrition legere ou moderee chez 26 %. Les consequences psychosociales sont marquees par un retard scolaire chez 65 % des enfants de plus de 10 ans et une orientation professionnelle a partir de la 3 e chez 91 % des enfants; les troubles psychologiques varient de la simple anxiete a la depression severe. Conclusion . - Cette etude souligne l'interet d'un suivi de long cours des enfants porteurs de stenoses caustiques de l'aesophage et la necessite d'une prise en charge pluridisciplinaire, medicale, psychologique et sociale.

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.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.Weight increased by 1.0 (0.8) kg (p0.001); weight/height increased from 94.4(12.2)% to 98(12.7)% (p0.001), energy intake from 107(32)% to 119(41)% (p0.02), and prealbumin from 183 (63) to 276 (89) mg/l (p0.001). Fat mass increased by 0.8 (1.0) kg (p0.001), without any significant change in fat-free mass. The limits of agreement between BIA and anthropometry were -0.7 kg and +1.1 kg.Antibiotic courses allow an improvement in nutritional status in CF patients, with a gain in fat mass.

Resume L'œsogastroduodenoscopie est une exoploration de plus en plus utilisee en periode neonatale grâce a la mise au point de materiel adapte au nouvcau-ne. L'objectif de ce travail est d'evaluer la frequence des affections. observees et de rappeler les pinicipales indications de l'œsogastroduodenoscopie pendant le periode neonatale. Population et methodes . — Une etude retrospective portant sur 107 dossiers de nourveau-nes explores entre octobre 1986 et avril 1995 a ete realisee au CHU de Lille. Les parametres etudies ont ete le terme, le sexe, les indications de l'œsogastroduodenoscopie et des lesions endoscopiques observees. Trois groupes de nouveaunes ont ete constitues selon les resultats macroscopiques: groupe I: œsogastroduodenoscopie normale (n = 22): groupe II: œsophagite isolee (n = 27); groupe II: œsogastrite ou gastroduodenite ou œgastroduodenite (n = 38). Le test du ξ 2 a ete utilise pour l'analyse statistique. Resultats . — Les signes faisant suspecter une œsophagite (pleurs lors des biberons) etaient plus frequents chez les enfants presentant une œsophagite isolee que chez les enfants ayant une œsogastrite: 37% vs 13 % (p 0.01)_et 59% vs 8% (p −4 / L'œsogastroduodenoscopie a permis globalement un diagnostic precis des lesions digestives dans 80% des cas: le rendement diagnostique atteignait 95% en cas d'hematernese avec ou sans melena. Conclusion . — Une œsogastroduodenoscopie en premiere intention est justifiee chez le nouveau-ne suspect d'œsophagie ou presenant une hematemese avec ou sans melena. En revanche, lors d'un bilan de malaise ou d'une suspicion de stenose hypertrophique dy pylore, elle est complementaire d'autres explorations plus contributives./

Anorexia and weight loss are frequently reported as adverse effects during recombinant interferon alpha (rIFN-alpha) treatment. The aim of the present study was to assess both nutritional status and growth of children and adolescents treated with rIFN-alpha for chronic viral hepatitis. Eleven patients aged 4-16 years with histologically proven chronic active hepatitis (hepatitis B, n = 9; hepatitis C, n = 2) receiving rIFN-alpha subcutaneously thrice a week for 6 months were studied. Weight and height increments were assessed during the 6 months before starting rIFN-alpha. Weight and height were measured every 3 months (M0, M3, M6) during the 6 months of rIFN-alpha treatment, then every 6 months during the follow up period (6-36 months). Weight decreased in every child during rIFN-alpha treatment (weight loss varies from 0.5 to 2.6 kg after 3 months of treatment). Weight/age Z-score decreased from 0.12 at M0 to -0.69 at M3 (P0.01), then increased between M3 and M6 (-0.33) (P0.01), but normalized (0.02) only 6 months after completion of treatment. Nutritional status was significantly impaired during treatment (Z-score for weight/height decreased from 0.18 at M0 to -0.74 at M3, P0.01) and recovered progressively thereafter. Height and height velocity were not modified by rIFN-alpha treatment. A reduction of the caloric intake observed between M0 and M3 might explain these features.Significant but transient abnormalities of the nutritional status are encountered constantly at the beginning of rIFN-alpha therapy without any deleterious effect on growth. Information of the families and nutritional intervention during treatment should be required, in order to limit the importance of weight loss.

Resume Les localisations gastro-intestinales de la maladie de Recklinghausen (MR) sont rares chez l'enfant, longtemps latentes et de pronostic reserve du fait des rectidives locales et du risque de transformation maligne. Observation. — Un garcon de 13 ans a presente une hematernese revellatrice d'un ulcere gastrique. Une recidive hemorragique a permis d'identifier une tumefaction gastrique sessile chez ce garcon, par ailleurs porteur de nombreuses taches cutanees caracteristiques de MR. Un nouvel episode de deglobulisation a fait realiser une laparotomie exploratrice qui decouvre une infiltration etendue de la face anterieure de l'estomac dont l'exerese partielle a permis le diagnostic histologique de neurofibrome. L'absence de compaliance a la surveillance echoendoscopique et une recidive symptomatique ont finalement conduit a une gastrectomie totale; le controle histologique ne montrait pas de signes de degenerescence. Conclusion. — L'endoscopie est un moyen important d'investigation de la tumeur ou de l'infiltration, mais seule l'echoendoscopie permet de faire le bilan de l'extension parietale.

Eleven children with total colonic aganglionosis were studied by esophageal and duodenal manometry. They were separated into two groups: group 1, with ileal involvement, and group 2, without ileal involvement. The results of the digestive motility studies were compared with the extent of ileal involvement of the aganglionosis and with the amount of artificial nutritional support required. All children showed abnormalities on both esophageal and duodenal manometry, suggesting complete digestive tract motility involvement. Recent advances in understanding Hirschsprung's disease and manometrial abnormalities suggest a primary motility disorder in children with total colonic aganglionosis.

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 = 5) 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.

La fievre est le premier motif de consultation en pediatrie. L’objectif de ce travail etait d’identifier la prise en charge a domicile de la fievre chez les enfants de moins de 5 ans. Patients et methode Il s’est agi d’une enquete prospective dans 3 structures hospitalieres de Libreville, du 15 au 26 juillet 2013. Les parents d’enfants de moins de 5 ans ont ete interviewes apres l’obtention de leur consentement oral. Les donnees ont ete collectees a l’aide d’un formulaire preetabli. Resultats Soixante sept parents ont ete inclus. L’âge moyen des enfants etait de 18 mois et celui des meres de 28 ans. Les meres avaient un niveau d’etudes secondaire dans 60,7% des cas. Elle etait mesuree par voie rectale chez 83,9% des enfants. Le thermometre a mercure avait ete utilise dans 62,1% des cas. Parmi les medicaments administres a domicile, on notait du paracetamol (65,1%) ou de l’ibuprofene (11,1%). Les moyens physiques cites etaient entre autres, l’application d’une serviette froide sur le front (83,7%) ou l’hydratation (7%). Conclusion La prise en charge medicamenteuse a ete appropriee dans la plupart des cas. Ce n’etait pas le cas des methodes de mesure. La mise en œuvre de la prise en charge integree des maladies de l’enfant au niveau communautaire est une strategie pour l’ameliorer.

Nutritional support is often necessary in chronic liver disease in childhood, and when enteral nutrition is insufficient, parenteral nutrition (PN) can be envisaged as a last resort. Pediatric experience is still limited in this indication. Seven children with severe liver disease received PN for a mean duration of 105 d, with additional enteral nutrition. Clinical tolerance was assessed and anthropometric and biological data were compared at the beginning and at the end of the study by the paired non-parametric test of Wilcoxon. Weight change, expressed as weight-for-age or weight-for-height Z-scores, increased. Conjugated bilirubin increased significantly. This retrospective study suggests that PN is a well-tolerated method for maintaining nutritional status in pediatric chronic liver disease when enteral nutrition has failed.

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

An aminoglycoside in association with beta-lactam antibiotic are usually the most efficient treatment for Pseudomonas aeruginosa infections in cystic fibrosis patients. Tobramycin has the lower MICs than the other aminoglycosides to Pseudomonas aeruginosa. The aim of the work was to compare pharmacokinetics of tobramycin after once daily (15 mg/kg/day; 11 patients) or thrice daily dose (5 mg/kg/day; 9 patients) in combination ceftazidime (CAZ 200 mg/day in 3 inj. IVD) in sputum and sera for two weeks. No statistical difference in the serum concentration obtained in each group of patients was observed between the first and the 14th day. Serum concentrations were three fold higher when tobramycin was administered in once daily dose. Low through concentrations were quickly obtained, but they were slightly higher after thrice daily doses. Bronchial concentrations were 2 to 2.5 superior and near the critical concentration of tobramycin. The clinical efficacy were comparable in the two regimens.

Long term follow-up of children with esophageal caustic stenosis is not well known. The aim of the present study was to describe functional, organic and psychological, as well as social consequences.Thirty-four children with a mean age of 3 years and 7 months +/- 3 years and 2 months (ranges: 1 month-14 years and 3 months) were included in a longitudinal study. Various parameters have been studied: treatment, functional symptoms, nutritional status (weight/height, body composition) and psychological and social consequences. chi 2 and Mann-Whitney tests were used for statistical analysis.Twenty-one patients have been treated by mechanical dilatations whereas surgery was performed in 12 children; the mean number of dilatations per child was higher in patients treated by dilatations (21 +/- 17 vs 14 +/- 16; P0.05). The frequency of dysphagia was not different in patients with colon interposition or not (69% vs 53%; P = 0.1). Nutritional status was not affected by the presence of esophageal caustic stenosis. Psychological and social consequences were characterized by scholastic difficulties, anxiety and severe depression. One case of suicide was observed.Children with caustic stenosis should be followed for a long period of time. A multidisciplinary approach is necessary, taking into account medical, social and psychological consequences.

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.

Upper gastrointestinal endoscopy is frequently used in the neonatal period. The aim of this study was to assess the frequency of the different lesions occurring as well as to precise indications of upper gastrointestinal endoscopy in neonates.A retrospective study including 107 neonates referred between October 1986 and April 1995 has been achieved in the pediatric gastroenterology unit of the Lille University Hospital. Various factors were analysed: gestational age, sex, reasons for endoscopy and macroscopic lesions observed. Three groups were constituted according to macroscopic findings; group I: normal aspect (n = 22); group II: isolated esophagitis (n = 27); group III: esogastritis or gastroduodenitis or esogastroduodenitis (n = 38). Chi 2 test was performed for statistical analysis.Signs recalling esophagitis (cry during feeding) were more frequent in group II than in group III: 37% vs 13% (P0.03). The neonates undergoing endoscopy for life-threatening events were more frequent in group I than in group II or III, respectively: 59% vs 15% (P0.01) and 59% vs 8% (P10(-4). Upper gastrointestinal endoscopy led to a precise diagnosis in 80% of the neonates. However 95% of those examined for hematemesis presented macroscopic lesions.Hematemesis and suspicion of esophagitis are good indications for upper gastrointestinal endoscopy in neonatal period. In life-threatening events and suspicion of pyloric stenosis, upper gastrointestinal endoscopy is only complementary of more contributive other examinations.

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.

A transversal study allowed to analyse the different features of severe bullous toxicodermatosis, and its relations with HIV infection. Since March 1992 to December 1995, 15 patients were hospitalized in Lome Teaching Hospital for severe bullous toxicodermatosis: 16 cases of Lyell's syndrome, 8 cases of Stevens-Johnson's syndrome and 1 case of ectodermosis. The principal drug which induced these diseases were mostly dominated by the sulfanilamide (n = 7). Nine (9) of these subjects were infected by the HIV (four of whose patients had Lyell's syndrome). Its noted five cases of death, all in the patients with HIV infection.

Twenty cystic fibrosis patients aged 1.8-22 years (mean +/- SD: 9.6 +/- 4.8 years) with Pseudomonas aeruginosa pulmonary exacerbations were treated with amikacin (AM) (35 mg/kg/day in one daily 30 min infusion) associated with either ceftazidime (200 mg/kg/day in 3 i.v. injections) (n = 19) or imipenem (n = 1) at the same dose. Glomerular and tubular functions (creatinine clearance, 24-h proteinuria, beta 2 microglobulinuria, lysozymuria) and audiometry remained within normal ranges from day 0 to day 14. A peak concentration of AM of 83 +/- 19 mg/l and a trough concentration of 0.8 +/- 0.5 mg/l were observed in blood while AM levels in sputum were above the minimal inhibitory concentration 50 from 30 min to 16 h. No serum accumulation of AM was observed during the treatment. From day 0 to day 14, the following changes were observed: weight/height ratio: 96%-100% (P0.001); daily energy intake: 111%-128% of RDA (P0.001); prealbumin: 195-290 mg/l (P0.001); forced vital capacity (FVC): 66%-81% (P0.01); forced expiratory volume in 1 s: 60%-75% (P0.01); forced expiratory flow between 25% and 75% of FVC: 42%-56% (P0.01); nocturnal SaO2 also improved significantly; cardiac rate decreased from 89 +/- 18/min to 76 +/- 16/min (P0.001); respiratory rate decreased from 31 +/- 15/min to 26 +/- 10/min (P0.05); inflammatory parameters (white blood cells, polymorphonuclear cells, erythrocyte sedimentation rate) also improved.Once daily amikacin administration associated with ceftazidime is well tolerated for the treatment of Pseudomonas aeruginosa pulmonary exacerbations in cystic fibrosis patients. Serum peak levels and diffusion in sputum are higher than with a conventional schedule.

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.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.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%).Contrary to surgical gastrostomy, PEG does not worsen GER. Therefore, GER is not a contraindication to PEG.

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.

To demonstrate the value of nasal transepithelial potential difference measurements in the diagnosis and assessment of therapeutic effect in cystic fibrosis.Nasal transepithelial potential difference was measured in 50 patients with cystic fibrosis (mean age 10 years) and 21 controls (mean age 8 years).There was a very significant increase in nasal transepithelial potential in cystic fibrosis patients (-28 +/- 8 mv) compared with controls (-8 +/- 3 mv) (p0.0001). The sensitivity and specificity of this test for the diagnosis of cystic fibrosis were 92% and 95% respectively. Nasal transepithelial potential difference was positively correlated with age (r = 0.43; p0.002) in cystic fibrosis patients but no correlation was found in controls. There was no significant difference in potential differences as a function of sex or Delta F508 genotype (homozygous vs heterozygous; Delta F508 vs non-Delta F508).These findings demonstrated the value of transepithelial potential difference in the diagnosis of cystic fibrosis and emphasized its capacity to assess the effect of new therapeutic protocols aimed at modifying C1- and Na+ ion transport. It could be particularly helpful for the interpretation of doubtful sweat tests.

Several possible diagnoses are proposed to explain the symptomatology observed in a slightly jaundiced 10 month-old suckling infant suffering from fever and anaemia. Major beta-thalassaemia in its most severe form (Cooley's syndrome) proved to be the correct diagnosis. The importance of family background is stressed for these haemolytic cases.