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The use of transcranial Doppler (TCD) ultrasound in neurocritical patients is reported to be increasingly common in pediatric intensive care units. The objective of this survey was to know about the use and practice of TCD ultrasound in neurocritical care and the training process of staff members performing it.Survey administered to providers from 23 pediatric intensive care units of Argentina.The percentage of response was 86%. TCD ultrasound was used for suspected brain death (n = 20), head injury (n = 16), and stroke (n = 16). Pediatric intensivists perform the test (n = 13/20). Surveyed participants use TCD ultrasound to decide on treatment and management, start brain death assessment, request brain computed tomography, and manage cerebral perfusion pressure with vasopressors.All surveyed participants use TCD ultrasound findings to guide management or treatments. Half of surveyed participants are little satisfied with their training.Introducción. El uso del Doppler transcraneal (DTC) en pacientes neurocríticos se reporta cada vez más en las terapias intensivas pediátricas. El objetivo de esta encuesta es conocer los usos y prácticas del DTC en la atención neurocrítica y el proceso de formación del personal que realiza el estudio. Materiales y métodos. Encuesta a profesionales de 23 unidades de terapia intensiva pediátrica de Argentina. Resultados. Porcentaje de respuesta del 86 %. Se utilizó en sospecha de muerte encefálica (n = 20), trauma de cráneo (n = 16) y accidente cerebrovascular (n = 16). El intensivista pediátrico es quien realiza los estudios (n = 13/20). Los encuestados utilizan el Doppler para decidir conductas y tratamientos, comenzar el proceso de evaluación de muerte encefálica, solicitar tomografía de cerebro y manejar la presión de perfusión cerebral con vasopresores. Conclusión. Todos los encuestados utilizan los hallazgos del DTC para guiar tratamientos o conductas. La mitad de los encuestados está poco conforme con la capacitación recibida.

Resume Des 1994, nous avons debute la rhDNase chez tous les patients atteints de mucoviscidose âges de plus de 5 ans et ayant une CVF ≥ 40 % de la valeur theorique. Population et methodes Nous avons analyse retrospectivement les effets de la rhDNase au cours de 2 ans de traitement chez 69 enfants et adolescents. Les patients ( 35 garcons, 34 filles) ont recu une dose unique journaliere de 2,5 mg de rhDNase. Ils etaient âges initialement de 8,5 ans (5 a 16,4 ans). Les valeurs spirometriques (pourcentage de la valeur theorique) et le statut nutritionnel de base etaient les suivants: CVF = 84,8 ± 21,7, VEMS = 80,8 ± 22,2, DEP = 89,7 ± 34,2, DEM 25-75 = 71, 8 ± 32,8, Z score poids/taille = -0,41 ± 1,14, Z-score poids/âge = −0,48 ± 1,25, index de Quetelet = 15,4 ± 1,8, ration calorique = 107 ± 25 % des apports journaliers recommandes. Les donnees spirometriques et nutritionnelles ont ete analysees apres 1, 3, 6, 12, 18 et 24 mois de traitement et comparees aux valeurs de base (resultats spirometriques exprimes en pourcentage des valeurs de base moyennes). Le score de Shwachman-Kulczycki etait de 87 ± 9 avant le traitement et a ete releve 24 mois plus tard. Resultats Une amelioration de la CVF (+ 10,7 %, p p p ≤ 0,01 ). Ceci a ete note en particulier chez les enfants âges de 5 a 10 ans, chez les garcons et chez les patients dont la CVF de base etait e ( p e mois ( p ≤ 0,02), essentiellement chez les filles. Les Z scores poids/âge et poids/taille se sont ameliores respectivement pendant les 3 premiers mois et apres 2 ans de traitement. Le score de Shwachman-Kulczycki est reste stable. Conclusion La rhDNase ameliore donc la fonction respiratoire et le statut nutritionnel des jeunes patients atteints de mucoviscidose et la reponse au traitement peut etre evaluee des les 3 premiers mois.

Resume La transplantation hepatique est une solution therapeutique proposee chez les patients atteints de mucoviscidose presentant une insuffisance hepatique terminale. Lorsque ces patients sont porteurs chroniques de Pseudomonas aeruginosa , se pose la question du choix entre une transplantation hepatique isolee (TRH) et combinee foie-poumon. Observation. Une adolescente de 15 ans atteinte de mucoviscidose a beneficie d'une TRH en raison d'une cirrhose au stade terminal. Elle presentait depuis 3 ans un portage chronique de pyocyanique traite par cures antibiotiques intravcineuses systematiques. Il existait une insuffisance pancreatique exocrine. Il etait note une intolerance glucidique moderee lors des surinfections. Les suites operatoires immediates ont ete simples. Elle a presente par la suite un diabete necessitant une insulinotherapie pendant 9 mois. Il a ete constate des troubles majeurs de l'absorption de la ciclosporine orale ameliores secondairement par la prise d'une nouvelle forme hydrosoluble (Neoral ® ). Il n'y a pas eu de surinfection pulmonaire severe due au portage chronique du pyocyanique. Il a ete note un rattrapage du developpement pubertaire. Conclusion. Ce recul de 3 ans confirme que la TRH est une bonne alternative therapeutique pour les patients atteints de mucoviscidose presentant une insuffisance hepatique terminale et une atteinte pulmonaire moderee.

Resume Un reflux gastroœsophagien (RGO) est frequemment observe au cours des affections respiratoires chroniques, et peut en aggraver l'evolution. Le but de ce travail etait de rechercher la frequence et les caracteristiques du RGO chez de jeunes enfants atteints de mucoviscidose (CF). Population et methodes. — Vingt-cinq enfants âges de 45 a 954 jours (moyenne: 219 jours), atteints de CF, ont eteetudies par pHmetrie de longue duree (19,45 ± 1,89 h) (moyenne ± DS), en l'absence de signes cliniques de surinfection bronchopulmonaire, et en l'absence de traitement bronchodilatateur. L'atteinte respiratoire a eteevaluee par le nombre de cures d'antibiotiques anterieurement recues et par l'analyse de la radiographic pulmonaire realisee au moment de la pHmetrie. La symptomatologie clinique evocatrice de RGO etait recherchee par l'interrogatoire des parents. Resultats. — Dix-neuf enfants etaient porteurs de RGO (76%); quatre des 19 enfants n'avaient pas de signes cliniques evocateurs de RGO. Tous les enfants avaient une atteinte respiratoire moderee. L'index de reflux (pourcentage du temps total d'enregistrement a pH Conclusions. — Le RGO est frequent au cours de la CF et atteint les jeunes enfants. Il semble primitif, non pas secondaire a l'atteinte respiratoire. Sa frequence et sa gravite potentielle incitent a pratiquer une pHmetrie systematique lors du bilan initial de tout enfant atteint de CF.

The prevalence of urolithiasis is higher in patients with cystic fibrosis than in the normal population. We report on a case of a 12-year-old child with cystic fibrosis who presented flank pain which revealed a calcium oxalate urolithiasis. Evolution was satisfactory with pharmacological and dietary treatment. The principal mechanisms of lithogenesis in patients with cystic fibrosis are described and preventive treatment is discussed.

The Pseudomonas aeruginosa bronchopulmonary infection is, when chronical, a poor prognosis factor for cystic fibrosis children. The higher life expectancy is partly linked to the progresses of the antipseudomonal antibiotherapy: treatment modulated to the stage of infection; possible use of nebulized and oral (ciprofloxacin) antibiotics. But the antipseudomonal strategy does not limit to the antibiotherapy. The preventive approach includes: preservation of a good nutritional status; daily chest physiotherapy with combined use of aerosolized recombinant human DNase for more than 5 years old children with significative bronchorrea; early anti-infammatory treatment with inhaled corticosteroids, despite a poor efficacy proof level; hygiene measures to reduce the environmental and domestic reservoirs and to avoid nosocomial infections. The development of vaccines for the prevention of P. aeruginosa infection is a promising way but stays in the field of clinical research. In case of chronical infection, long-term macrolids reduce the virulence factors expression of the bacteria, leading to antibiofilm properties in spite of subinhibitory concentrations.

The prevalence of urolithiasis is higher in patients with cystic fibrosis than in the normal population. We report on a case of a 12-year-old child with cystic fibrosis who presented flank pain which revealed a calcium oxalate urolithiasis. Evolution was satisfactory with pharmacological and dietary treatment. The principal mechanisms of lithogenesis in patients with cystic fibrosis are described and preventive treatment is discussed.

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.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.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.

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.

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).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.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 pH4) was 12.82%. The GER episodes were more frequent during walking time (p0.001), but longer during sleeping time (p0.001).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.

The aim of the present study was to describe linear growth of infants with celiac disease, using the “ICP-growth model”. Supine length during the first three years of life was studied longitudinally in 63 infants with diagnosed celiac disease. “Undisturbed” linear growth was seen during the first six postnatal months followed by reduced growth during the second half of the first year. After 1.5 years of age a pattern of catch-up growth was observed, leading to an average attained length at 3 years of age similar to that of the controls. According to the “ICP-growth model”, normal linear growth can mathematically be represented during the first 3 years of life by an Infancy component with the addition of a Childhood component, the latter acting from the second half of the first postnatal year. The onset of the Childhood component (assumed to represent the age at which growth hormone begins to influence linear growth significantly) was delayed by about an average of 3 months, which is in agreement with the observed reduction in gain during the second half of the first year of life. Children suffering from celiac disease and with “late” onset of the Childhood component were shorter at 1, 2 and 3 years of age than those with “normal” onset. The results of this investigation show that ICP-based growth charts are helpful in detecting and monitoring growth for children with celiac disease, and indicate a possible mechanism whereby malabsorption (and perhaps secondary malnutrition) leads to reduced growth velocity.